Outcomes of Research or Clinical Trials Activity Levels Acute Flaccid Paralysis Ageing Anaerobic Threshold Anaesthesia Assistive Technology Brain Cardiorespiratory Cardiovascular Clinical Evaluation Cold Intolerance Complementary Therapies Continence Coping Styles and Strategies Cultural Context Diagnosis and Management Differential Diagnosis Drugs Dysphagia Dysphonia Epidemiology Exercise Falls Fatigue Fractures Gender Differences Immune Response Inflammation Late Effects of Polio Muscle Strength Muscular Atrophy Orthoses Pain Polio Immunisation Post-Polio Motor Unit Psychology Quality of Life Renal Complications Respiratory Complications and Management Restless Legs Syndrome Sleep Analaysis Surgery Vitality Vocational Implications

Title order Author order Journal order Date order
Category: Diagnosis and Management

Title: A positive turning point in life -- how persons with late effects of polio experience the influence of an interdisciplinary rehabilitation programme
Author: Larsson Lund M (1), Lexell J
Affiliation: (1) Department of Community Medicine and Rehabilitation, Occupational Therapy , Umeå University, Umeå, Sweden
Journal: Journal of Rehabilitation Medicine
Citation: J Rehabil Med. 2010 Jun;42(6):559-65. doi: 10.2340/16501977-0559
Publication Year and Month: 2010 06

Abstract: OBJECTIVE: To describe and enhance our understanding of how persons with late effects of polio experience the influence of an interdisciplinary rehabilitation programme.

PARTICIPANTS: Twelve persons with clinically verified late effects of polio who had participated in an individualized, goal-oriented, comprehensive interdisciplinary rehabilitation programme.

METHODS: Qualitative research interviews analysed using the constant comparative method of grounded theory.

RESULTS: The rehabilitation programme was experienced as a turning point in the participants' lives. Before rehabilitation they felt they were on a downward slope without control. Rehabilitation was the start of a process of change whereby they acquired new skills, which, over time, contributed to a different but good life. After approximately a year, they had a sense of control and had accepted life with late effects of polio. They had also established new habits, taken on a changed valued self and could look to the future with confidence.

Conclusions: This qualitative study has shown that persons with late effects of polio can benefit from an individualized, goal-oriented, comprehensive interdisciplinary rehabilitation programme and experience positive changes in their management of daily activities and in their view of their late effects of polio, their future and their self.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Cardiac risk factors in polio survivors
Author: Gawne AC, Wells KR, Wilson KS.
Affiliation: Roosevelt Warm Springs Institute for Rehabilitation, Warm Springs, GA 31830, USA - [email protected]
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 2003 May;84(5):694-6
Publication Year and Month: 2003 05

Abstract: OBJECTIVE: To assess the prevalence of dyslipidemia and other risk factors for coronary heart disease in a sample of polio survivors with and without postpoliomyelitis syndrome.

DESIGN: Retrospective chart review.

SETTING: A multidisciplinary outpatient postpolio clinic.

PARTICIPANTS: Eighty-eight consecutive symptomatic postpolio patients, 50 women (mean age, 59.0y; range, 36-81y) and 38 men (mean age, 61.2y; range, 44-83y).

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Presence of risk factors for coronary heart disease: clinical atherosclerotic disease, male age >or=45 years or female age >or=55 years, history of hypertension (blood pressure >or=140/90mmHg or on antihypertensive medication), diabetes mellitus, cigarette smoking, and high-density lipoprotein (HDL) less than 35mg/dL. Obesity (body mass index [BMI], >25kg/m(2)) was assessed as an intervention target. Laboratory values included fasting total cholesterol, HDL, low-density lipoprotein, triglycerides, and glucose.

RESULTS: Of the total sample, 61.3% had dyslipidemia. Average HDL cholesterol ratio was 4.01 (women, 3.68; men, 4.55). Forty-four patients (50%) had a history of hypertension or had elevated blood pressure. Seven patients (8%) had a history of diabetes or had elevated fasting blood glucose (>110). Eighteen patients (20.4%) were smokers or had a history of smoking; 9 continued to smoke and 9 had quit smoking. Twenty-five patients (28.4%) were overweight (BMI, >25kg/m(2)). Forty-one patients (46.5%) had more than 1 risk factor for coronary heart disease. Nine of the total sample (10.2%) had a history of heart disease ranging from atrial fibrillation to angina. Only 19 patients had a previous diagnosis of dyslipidemia and only 12 were on a lipid-lowering medication.

Conclusions: Polio patients have a high prevalence of dyslipidemia. The study sample supports the National Cholesterol Education Program's Adult Treatment Panel III statements that hypercholesterolemia is underdiagnosed and undertreated. The postpolio population carries a high prevalence of 2 or more coronary heart disease risk factors. Evaluation and rehabilitation of polio patients should include screening for dyslipidemia and education about elimination of controllable risk factors.

Outcome of Research: Not applicable.

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Category: Diagnosis and Management

Title: Characteristics and management of postpolio syndrome
Author: Jubelt B, Agre JC
Affiliation: State University of New York, Upstate Medical University, 750 E Adams St, Syracuse, NY 13210, USA – [email protected]
Journal: The Journal of the American Medical Association
Citation: JAMA. 2000 Jul 26;284(4):412-4
Publication Year and Month: 2000 07

Abstract: Postpolio syndrome (PPS) refers to new, late manifestations occurring many years after acute poliomyelitis infection. Over the last 25 years, PPS has become a relatively common problem encountered by primary care physicians. A 1987 National Health Interview Survey estimated that about half of the 640,000 survivors of paralytic poliomyelitis in the United States had new late manifestations of PPS. Subsequent studies in the 1990s have found the occurrence of PPS among patients with previous poliomyelitis to range from 28.5% to 64%. The average time in various reports from the acute poliomyelitis until the onset of PPS is about 35 years, with a range from 8 to 71 years. However, it is unclear if the occurrence of PPS increases with aging, which may be the case based on the most accepted etiologic hypothesis.

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Category: Diagnosis and Management

Title: Characteristics of Patients at First Visit to a Polio Clinic in Sweden
Author: Skough Vreede, K. and Sunnerhagen, K.S.
Affiliation: Goteborg University
Journal: PLoS One
Citation: https://doi.org/10.1371/journal.pone.0150286
Publication Year and Month: 2016 03

Abstract: Aim
Describe polio patients visiting a polio clinic in Sweden, a country where vaccination was introduced in 1957.

Design
A consecutive cohort study.

Patients
Prior polio patients.

Methods
All patients (n = 865) visiting the polio clinic at Sahlgrenska University Hospital, Gothenburg Sweden, between 1994 and 2012 were included in this study. Data at first visit regarding patient characteristics, polio classification, data of electromyography, origin, assistive devices and gait speed as well as muscle strength were collected for these patients. Twenty-three patients were excluded because no polio diagnosis could be established. A total of 842 patients with confirmed polio remained in the study.

Results
More than twenty percent of the patients were from countries outside the Nordic region and considerably younger than those from the Nordic region. The majority of the emigrants were from Asia and Africa followed by Europe (outside the Nordic region). Of all patients included ninety-seven percent (n = 817) had polio in the lower extremity and almost 53% (n = 444) had polio in the upper extremity while 28% (n = 238) had polio in the trunk, according to clinical classification of polio. Compared with a sample of the normal population, the polio patients walked 61–71% slower, and were 53–77% weaker in muscle strength of the knee and foot as well as grip strength.

Conclusion
The younger patients with polio emigrating from countries with different cultures may lead to a challenge for the multi professional teams working with post-polio rehabilitation and are of importance when planning for the care of polio patients the coming years.

Conclusions: Polio in lower extremities was more common than polio in upper extremities, verified both
by EMG and clinical classification. This is in accordance with earlier studies. Polio in lower
extremities was also classified as clinically unstable or severely atrophic to a higher extent than
polio in upper extremities (as shown in Fig 1). This is in accordance with an earlier study by
Sandberg et al [20] indicating a more pronounced ongoing denervation-reinnervation process
over time in a lower extremity muscle compared to upper extremity muscle (tibialis anterior
and biceps brachii respectively). The same pattern was also seen in patients studied in Minnesota where patients with leg weakness were twice as likely to complain of new problems compared to those with arm weakness [10].

The ongoing denervation-reinnervation process in patients with PPS results in larger motor
units.When motor-unit size has reached an upper limit, further losses of neurons can no longer
be compensated for and this results in increased muscle weakness [21]. The patients
showed to be stronger in isometric endurance compared to normal population. This may be
explained by the fact that the patients were weaker than the normal population in isometric
peak torque, which the measure of isometric endurance was based on. An increase in type I
(slow) muscle fibres has also been described in prior polio patients [22–23] and may be due to
a transition of type II (fast) to type I (slow).

An important study limitation were seen in the classification of polio as polio were classified
for left and right arm and leg, respectively, and not per muscle group. This can explain why a
polio affected leg in some cases was stronger compared to normal values as the muscles
involved in the strength measured i.e. knee flexion and knee extension muscles may not be
affected of polio. And the same is applicable regarding muscle strength of the foot as well as
grip strength. Data of muscle strength from some of the subjects were missing for different reasons i.e. they may have just not had time to participate, or refused to participate and some were too weak to perform the strength test. Some of the patients with muscle strength data missing, tried to perform the test, but were too weak to get a result. In the future, the use of ultrasound may be used to assess muscle function [24]. This would give the possibility to have more information of muscle function since this does not require that the patient has muscle strength to overcome gravity, which is a requirement for isokinetic testing.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Cognitive behavioural therapy for reducing fatigue in post-polio syndrome and in facioscapulohumeral dystrophy: A comparison
Author: Koopman, Fieke S., Merel A. Brehm, Anita Beelen, Nicole Voet, Gijs Bleijenberg, Alexander Geurts, Frans Nollet
Affiliation: Department of Rehabilitation , Academic Medical Center, University of Amsterdam, PO 22660, 1100 DD Amsterdam, The Netherlands. E-mail: [email protected]

Journal: Journal of Rehabilitation Medicine
Citation: 2017; 49: 585–590
Publication Year and Month: 2017

Abstract: Post-polio syndrome (PPS) and facioscapulohumeral dystrophy (FSHD) are two different neuromuscular disorders. Fatigue is a frequent complaint in both disorders. A recent study showed that cognitive behavioral therapy (CBT), which is a type of psychotherapy that helps patients to identify and reshape thoughts and behavior patterns that contribute to the fatigue was effective in alleviating fatigue in FSHD but not in PPS. In this study we investigated whether this difference in effectiveness might be explained by dissimilar fatigue-related thoughts (for example focusing on fatigue) in both conditions. We used questionnaires to measure the fatigue-related thoughts in 21 patients with PPS and 24 patients with FSHD . It appeared that fatigue-related thoughts in PPS were similar to those in FSHD and thus do not explain the difference in effectiveness of CBT.

Conclusions: Fatigue-related thoughts in PPS were similar to those in FSHD and thus do not explain the difference in effectiveness of CBT.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Effect of treatment and noncompliance on post-polio sequelae
Author: Peach PE, Olejnik S
Affiliation: Roosevelt Warm Springs Institute for Rehabilitation, Georgia 31830
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1199-1203
Publication Year and Month: 1991 11

Abstract: In this study of 77 patients with post-polio sequelae (PPS), symptoms and manual test scores on initial evaluation were compared with those at subsequent follow-up evaluations. Patients were divided into three groups based on the degree to which they had complied with clinically recommended interventions: compliers, partial compliers, and noncompliers. At the end of the follow-up period (2.2 +/- 1.2 years), the mean muscle function scores of the entire study group had declined -1.5%, which represented a decline of -0.7% annually. On follow-up evaluations, the complier group had realized an improvement or resolution of post-polio symptoms, and also an improvement in muscle function of +0.6% annually. The partial complier group had realized either no improvement, or improvement in post-polio symptoms, but showed a further decline in muscle function of -3.0%, or an annual decline of -1.3%. The noncomplier group showed either no change, or a worsening of post-polio symptoms, and also showed a further decline in muscle function of -4.1%, which represented an annual decline of -2.0%.

Conclusions: The disparate outcomes among our post-polio patients underscore the need to develop more effective intervention strategies to achieve improved patient compliance, given the favorable outcomes experienced by patients who complied with clinical recommendations.

Outcome of Research:

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Category: Diagnosis and Management

Title: EFNS guideline on diagnosis and management of post-polio syndrome - report of an EFNS task force
Author: Farbu E, Gilhus NE, Barnes MP, Borg K, de Visser M, Driessen A, Howard R, Nollet F, Opara J, Stalberg E
Affiliation: Department of Neurology, Haukeland University Hospital, University of Bergen, Bergen, Norway – [email protected] – European Federation of Neurological Society
Journal: European Journal of Neurology
Citation: Eur J Neurol. 2006 Aug; 13(8):795-801
Publication Year and Month: 2006 08

Abstract: Post-polio syndrome (PPS) is characterized by new or increased muscular weakness, atrophy, muscle pain and fatigue several years after acute polio. The aim of the article is to prepare diagnostic criteria for PPS, and to evaluate the existing evidence for therapeutic interventions. The Medline, EMBASE and ISI databases were searched. Consensus in the group was reached after discussion by e-mail. We recommend Halstead's definition of PPS from 1991 as diagnostic criteria. Supervised, aerobic muscular training, both isokinetic and isometric, is a safe and effective way to prevent further decline for patients with moderate weakness (Level B). Muscular training can also improve muscular fatigue, muscle weakness and pain. Training in a warm climate and non-swimming water exercises are particularly useful (Level B). Respiratory muscle training can improve pulmonary function. Recognition of respiratory impairment and early introduction of non-invasive ventilatory aids prevent or delay further respiratory decline and the need for invasive respiratory aid (Level C). Group training, regular follow-up and patient education are useful for the patients' mental status and well-being. Weight loss, adjustment and introduction of properly fitted assistive devices should be considered (good practice points). A small number of controlled studies of potential-specific treatments for PPS have been completed, but no definitive therapeutic effect has been reported for the agents evaluated (pyridostigmine, corticosteroids, amantadine). Future randomized trials should particularly address the treatment of pain, which is commonly reported by PPS patients. There is also a need for studies evaluating the long-term effects of muscular training.

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Category: Diagnosis and Management

Title: Epidemiology of the post-polio syndrome
Author: Ramlow J, Alexander M, LaPorte R, Kaufmann C, Kuller L
Affiliation: Department of Biostatistics, Graduate School of Public Health, University of Pittsburgh, PA
Journal: American Journal of Epidemiology
Citation: Am J Epidemiol. 1992 Oct 1;136(7):769-86
Publication Year and Month: 1992 10

Abstract: A late-onset syndrome, consisting of muscle weakness, muscle pain, and unaccustomed fatigue, has been reported with increasing frequency among former poliomyelitis patients. A population-based cohort of poliomyelitis patients from Allegheny County, Pennsylvania, was traced and surveyed to estimate the prevalence and incidence and to identify determinants of the post-polio syndrome. A questionnaire validated in clinical examinations of 40 cohort members was used in the survey. The prevalence of the post-polio syndrome was 28.5% of all paralytic cases (95% confidence interval 24.4-32.6). The risk of post-polio syndrome was significantly higher among patients who sustained substantial permanent impairment after polio and among females. The incidence did not vary with age at acute onset, acute severity, or level of physical activity after recovery. The strongest determinant of post-polio syndrome onset was the length of the interval following the acute illness, with incidence peaking at 30-34 years. Of all cases of post-polio syndrome, 79% reported no major change in impairment status since onset. This study demonstrates that poliomyelitis patients are not equally susceptible to post-polio syndrome within the interval of 30-40 years after the original illness. For syndrome cases, the onset was associated with new neuromuscular symptoms and functional changes but not with major new impairment.

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Category: Diagnosis and Management

Title: Estimation of the Direct Cost of Poliomyelitis Rehabilitation Treatment to Pakistani Patients: A 53-Year Retrospective Study
Author: Atta Abbas Naqvi, Syed Baqir Shyum Naqvi, Fatima Zehra, Ashutosh Kumar Verma, Saman Usmani, Sehrish Badar, Rizwan Ahmad, Niyaz Ahmad
Affiliation: 1.Department of Pharmacy Practice, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
2.Faculty of PharmacyHamdard UniversityKarachiPakistan
3.Applied Economics Research CentreUniversity of KarachiKarachiPakistan
4.Discipline of Social and Administrative Pharmacy, School of Pharmaceutical SciencesUniversiti Sains MalaysiaMindenMalaysia
5.Institute of Pharmaceutical Sciences, Jinnah Sindh Medical UniversityKarachiPakistan
6.Natural Products and Alternative Medicines, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
7.Department of Pharmaceutics, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
Journal: Applied Health Economics and Health Policy
Citation: December 2018, Volume 16, Issue 6, pp 871–888
Publication Year and Month: 2018 12

Abstract: Background
Pakistan is one of the last few countries in which poliomyelitis is endemic. Evidence indicates that out-of-pocket expenditures are a barrier to polio rehabilitation treatment, yet there are no reported figures related to the financial burden of this disease on patients in a recently polio-endemic country.

Objective
This study investigated direct costs attributed to rehabilitation treatment of poliomyelitis among Pakistani patients and reported its duration along with the socioeconomic status of poliomyelitis survivors.

Conclusions: Conclusion
The cost of poliomyelitis rehabilitation in Pakistan is high; it has an economic effect on the lives of patients and their families. Despite good education, polio survivors in Pakistan appear to have low socioeconomic status, lower chances of employment and marriage, as well as fewer children. Further research is recommended to explore the burden of disease on society, i.e., indirect costs and suffering.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Frequency and clinical manifestations of post-poliomyelitis syndrome in a Brazilian tertiary care center
Author: Quadros AA (1), Conde MT, Marin LF, Silva HC, Silva TM, Paula MB, Pereira RD, Ramos PE, Abe G, Oliveira AS
Affiliation: (1) Department of Neurology and Neurosurgery, Division of Neuromuscular Disorders, Federal University of São Paulo (Unifesp), São Paulo SP, Brazil - [email protected]
Journal: Arquivos de Neuro-psiquiatria
Citation: Arq Neuropsiquiatr. 2012 Aug;70(8):571-3
Publication Year and Month: 2012 08

Abstract: OBJECTIVE: To determine the frequency and clinical manifestations of patients with post-poliomyelitis syndrome (PPS) in a Brazilian division of neuromuscular disorders.

METHODS: A total of 167 patients with prior history of paralytic poliomyelitis was investigated for PPS, based on international diagnostic criteria. Other variables analyzed were: gender, race, age at poliomyelitis infection, age at PPS onset, and PPS symptoms.

RESULTS: One hundred and twenty-nine patients presented PPS, corresponding to 77.2% of the studied population. 62.8% were women and 37.2% were men. Mean age of patients with PPS at onset of PPS symptoms was 39.9±9.69 years. Their main clinical manifestations were: new weakness in the previously affected limbs (69%) and in the apparently not affected limbs (31%); joint pain (79.8%); fatigue (77.5%); muscle pain (76%); and cold intolerance (69.8%).

Conclusions: Most patients of our sample presented PPS. In Brazil, PPS frequency and clinical features are quite similar to those of other countries.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Histopathologic Basis of Clinical Findings in Poliomyelitis
Author: Bodian, D.
Affiliation: Poliomyelitis Research Center, Department of Epidemiology, Johns Hopkins University, Baltimore
Journal: The American Journal of Medicine
Citation: 1949: 6(5):563–578
Publication Year and Month: 1949 05

Abstract: 1. Experimental evidence indicates that the onset of CNS pathologic changes occurs in the preparalytic period and is closely associated with the earliest evidence of virus activity in any particular region involved.
2. The earliest cytopathologic changes are diffuse chromatolysis of Nissl substance in the cytoplasm of nerve cells and mild cellular exudate consisting of polymorphonuclear and mononuclear leukocytes.
3. Nerve cell changes may be present in the earliest stages without inflammatory reaction in the vicinity and therefore are not necessarily the result of the latter, but rather the result of direct virus action.
4. Nerve cell changes either lead to rapid destruction of the cell or to arrest in the stage of cytoplasmic chromatolysis, following which complete morphologic recovery of the cell generally occurs over a period of about a month or less, depending upon the severity of injury.
5. Virus activity, nerve cell changes and inflammatory reaction are localized only in certain susceptible regions of the CNS, largely due to specific differences of susceptibility of nerve cells. The intensity of the inflammatory reaction, however, may be quite variable in different susceptible centers and in different individuals. Severe inflammatory reaction is usually but not always associated with extensive nerve cell destruction. Severe nerve cell damage may occur without extensive cellular infiltration in the cord.
6. Lesions in the cerebral cortex are usually confined to the motor area of the precentral gyrus and even here the lesions are rarely severe enough to suggest that they may produce clinical symptoms.
7. “Encephalitic” symptoms such as restlessness, stupor, disorientation and coma are associated with severe inflammatory reaction in the brainstem and often with small softenings in this region. They are not associated with unusual involvement of the cerebral cortex.
8. Brainstem centers principally involved in most instances are the reticular formation of the hind-brain, the vestibular nuclei and the roof nuclei of the cerebellum. Resulting functional disturbances are discussed.
9. Widespread dissemination of virus among most motor nerve cells in spinal cord enlargements occurs in experimental poliomyelitis as early as the first day of paralysis. Motor nerve cells which are affected either are destroyed very quickly during the first few days of the disease or undergo slower recovery changes leading to complete morphologic recovery within about a month. After this time it can be shown that the degree of paralysis and atrophy are closely correlated with the number of motor nerve cells destroyed. In the acute stage, however, this correlation is not as high and other factors must also play a role in producing paralysis. An important factor is the reversible injury of motor nerve cells. Less complete evidence from human material suggests that a similar situation obtains in human poliomyelitis.
10. Experimental work suggests three possible factors which may determine the variation in severity of infection. These are, first, variations due to difference in strains of the virus, second, reduction of severity due to previous paralytic or non-paralytic infection, and third, host variation unrelated to previous immunizing experience with the virus.

Conclusions: Poliomyelitis virus has strains that can vary depending on the host, and response to the virus. The virus causes most damage to the anterior horn cells of the cervical and lumbar segments, however there is damage to the brainstem and premotor areas in the brain.

Outcome of Research: More research required

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Comments (if any): David Bodian produced papers that helped enable (effective) availability for vaccinations. His work is still cited and referred to in understanding the effects of the polio virus, especially in relation to the central nervous system pathology.

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Category: Diagnosis and Management

Title: Identification of targets for improving access to care in persons with long term physical disabilities
Author: Jennifer L.Wong, Kevin N.Alschuler, Tracy M.Mroz, Kimberly P.Hreha, Ivan R.Molton
Affiliation: University of Washington, Rehabilitation Medicine, United States
Journal: Disability and Health Journal
Citation: Available online 20 January 2019
Publication Year and Month: 2019 01

Abstract: Background
People with long-term physical disability (LTPD) continue to experience difficulties in accessing health care despite the focus of highlighting disparities in the last two decades.

Objectives
To describe health care utilization, accommodations and barriers experienced while accessing health care, and reasons why individuals delay or skip health care among people with LTPD.

Methods
The current study was a part of a larger longitudinal survey administered to individuals with physical disability associated with one of four long-term conditions (MS; SCI; PPS; MD). Measures included demographics, health care utilization, barriers to health care, and reasons for delaying or skipping medical care from the sixth wave of data from 2015 to 2016.

Results
Roughly 90% of all participants (N = 1159) saw at least one medical provider within 12 months. The most encountered barrier participants reported experiencing within that time was an office that did not have a safe transfer device to move them to an exam table (69%). Participants’ physical function, quality of life, status of living with a spouse, diagnostic condition, and sex (male) were significantly associated with endorsing a barrier in accessing health care. The inability to afford out of pocket expenses was the highest reported reason for delaying health care.

Conclusions: People with LTPD access a variety of health care, including rehabilitation services, and continue to experience barriers when doing so. While understanding barriers individuals experience when accessing health care is important, it is equally important to document the type of care they delay or skip due to barriers.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Inverse Relationship Between Polio Incidence in the US and Colorectal Cancer.
Author: STEVEN LEHRER and PETER H RHEINSTEIN
Affiliation: Department of Radiation Oncology, Icahn School of Medicine at Mount Sinai, New York, U.S.A. [email protected].
Severn Health Solutions, Severna Park, MD, U.S.A.
Journal: IN VIVO
Citation: (Athens, Greece) vol. 32,6 (2018): 1485-1489.
Publication Year and Month: 2018

Abstract: BACKGROUND/AIM:
Polio is predominantly an enteric viral infection that was progressively eradicated in the United States after the introduction of polio vaccine in the early 1950s. U.S. colorectal cancer rates have dropped steadily for individuals born between 1890 and 1950, but have been increasing for every generation born since 1950. Moreover, the lowest worldwide age adjusted rates of colorectal cancer in 2012 were in sub-Saharan Africa, Gambia and Mozambique, where polio has not been eradicated. In the current study, poliomyelitis incidence in US states before the introduction of polio vaccine was analyzed.

MATERIALS AND METHODS:
Reported cases of poliomyelitis per 100,000 population by state 1932-1951 were from Centers for Disease Control. Colorectal cancer deaths per 100,000 in men (2005-2009) by US State are from the American Cancer Society. US state overweight and obesity data are from the Centers for Disease Control and Prevention (CDC). Smoking data are from the CDC.

RESULTS:
By US state, colorectal cancer incidence per 100,000 in men for 2005-2009 was inversely correlated with reported cases of poliomyelitis per 100,000 for 1932-1951 (r=-0.311, p=0.032). Colorectal cancer deaths per 100,000 in men in 2005-2009 were also inversely correlated with reported cases of poliomyelitis per 100,000 by state for 1932-1951 (r=-0.493, p<0.001). The relationship between colorectal cancer deaths and polio incidence was significant (β=-0.196, p=0.028) and independent of the effects of smoking (β=0.289, p=0.012) and overweight (β=0.547, p<0.001). The relationship in females with colorectal cancer was identical.

Conclusions: Polio virus infection of cells of the colon may induce some degree of resistance to the development of colon cancer decades later. The effect of polio virus infection seems to be especially potent in reducing the rate of death from colon cancer.

Outcome of Research: More research required

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Category: Diagnosis and Management, Late Effects of Polio

Title: Knowledge of healthcare professionals about poliomyelitis and postpoliomyelitis: a cross-sectional study
Author: Claudio Andre Barbosa de Lira (I), Douglas Assis Teles Santos (II), Ricardo Borges Viana (III), Juliana Moreira Guimarães (IV), Jéssica Nathalia Soares Oliveira (V), Bolivar Saldanha Sousa (VI), Marcos Gonçalves de Santana (VII), Rodrigo Luiz Vancini (VIII),
Marília Santos Andrade (IX), Pantelis Nikolaidis (X), Thomas Rosemann (XI), Beat Knechtle (XII)
Affiliation: (I) BSc, PhD. Associate Professor, Faculdade de Educação Física e Dança (FEFD), Universidade Federal de Goiás (UFG), Goiânia (GO), Brazil. https://orcid.org/0000-0001-5749-6877

(II) BSc, MSc. Assistent Professor, Colegiado de Educação Física, Universidade do Estado da Bahia (UNEB), Teixeira de Freitas (BA), Brazil. https://orcid.org/0000-0002-7664-5468

(III) BSc, PhD. Professor, Escola Superior de Educação Física e Fisioterapia do Estado de Goiás (ESEFFEGO), Universidade Estadual de Goiás (UEG), Goiânia (GO), Brazil. https://orcid.org/0000-0001-9200-3185

(IV) BSc. Nurse, Unidade Acadêmica Especial de Ciências da Saúde, Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0002-6729-0335

(V) BSc. Biomedic, Unidade Acadêmica Especial de Ciências da Saúde (UA-CISAU),
Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0002-9531-4277

(VI) MD, PhD. Physician, Hospital Israelita Albert Einstein, São Paulo-Brazil, São Paulo (SP), Brazil. https://orcid.org/0000-0001-9682-5987

(VII) BSc, PhD. Associate Professor, Unidade Acadêmica Especial de Ciências da Saúde (UA-CISAU), Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0001-7674-3263

(VIII) BSc, PhD. Adjunct Professor, Centro de Educação Física e Desportos, Universidade Federal do Espírito Santo (UFES), Vitória (ES), Brazil. https://orcid.org/0000-0003-1981-1092

(IX) PhD. Physical Therapist and Associate Professor, Department of Physiology, Universidade Federal de São Paulo (USP), São Paulo (SP), Brazil. https://orcid.org/0000-0002-7004-4565

(X) BSc, PhD. Professor, School of Health and Caring Sciences, University of West Attica, Athens, Greece. https://orcid.org/0000-0001-8030-7122

(XI) MD, PhD. Physician and Professor, Institute of Primary Care, University of Zurich, Zurich, Switzerland. https://orcid.org/0000-0002-6436-6306

(XII) MD, PhD. Physician and Scientific Assistant, Institute of Primary Care, University of Zurich, Zurich, Switzerland; and Researcher, Medbase St. Gallen Am Vadianplatz, St. Gallen, Switzerland https://orcid.org/0000-0002-2412-9103
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Sao Paulo Med. J.

139 (5) • Aug-Sep 2021
https://doi.org/10.1590/1516-3180.2020.0617.16032021
Publication Year and Month: 2021 08

Abstract: BACKGROUND:
Postpoliomyelitis syndrome is a clinical condition that can affect poliomyelitis survivors.

OBJECTIVE:
Our aim was to evaluate knowledge of poliomyelitis and postpoliomyelitis syndrome among Brazilian healthcare professionals.

DESIGN AND SETTING:
Cross-sectional study conducted at a Brazilian public higher education institution located in the state of Goiás.

METHODS:
The participants (n = 578) were Brazilian physicians, physical therapists, nurses, nutritionists and psychologists. A self-administered questionnaire (30 questions) was designed to probe knowledge about poliomyelitis and postpoliomyelitis syndrome. From the questionnaire, we created a structured test to objectively evaluate the knowledge of these professionals. The test was composed of 20 questions and was scored over a range from 0 (totally ill-informed) to 20 (totally well-informed).

RESULTS:
In general, the physicians, physical therapists and nurses demonstrated better understanding of poliomyelitis and postpoliomyelitis syndrome. The healthcare professionals who had received previous information about poliomyelitis and postpoliomyelitis syndrome had significantly higher scores than those who had never received information (P < 0.001). On average, this difference was approximately 28.6%.

CONCLUSIONS:
The findings from the present study indicate that there is a critical need for improvement of knowledge about postpoliomyelitis syndrome among Brazilian healthcare professionals. The services provided by these professionals may therefore become compromised. Furthermore, public healthcare initiatives should be implemented to improve knowledge among healthcare professionals.

KEYWORDS (MeSH terms):
Poliomyelitis; Physicians; Nervous system; Postpoliomyelitis syndrome; Knowledge

AUTHORS’ KEY WORDS:
Medical education; Neuromuscular disease; Late effects of polio

Conclusions: Our study showed that, overall, there is a lack of knowledge about PPS and poliomyelitis, especially among psychologists and nutritionists. Therefore, the services provided by these professionals may become compromised. Furthermore, government initiatives should be implemented to increase knowledge among healthcare professionals.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Life-Long Morbidity Among Danes With Poliomyelitis
Author: Nielsen NM, Rostgaard K, Askgaard D, Skinhøj, & P Aaby P.
Affiliation: Department of Epidemiology Research, Danish Epidemiology Science
Centre, Statens Serum Institut (Nielsen, Rostgaard, Aaby) and Department of Infectious Diseases M, National University Hospital, (Askgaard, Skinhøj), Copenhagen,
Denmark.
Supported by the Danish Medical Research Council, the AP Møller and Chastine
McKinney Møller Foundation, the Danish National Research Foundation, the WedellWedellsborg Foundation, and The National Polio Society
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil 2004; 85:385-91 - doi:10.1016/S0003-9993(03)00474-X
Publication Year and Month: 2004 03

Abstract: Abstract
OBJECTIVE:
To estimate long-term morbidity in a cohort of Danish poliomyelitis patients.

DESIGN:
A historical prospective cohort study of 27,047 persons.

SETTING:
Denmark.

PARTICIPANTS:
A total of 5421 persons hospitalized for poliomyelitis between 1919 to 1954 in Copenhagen, Denmark, and 21,626 age- and gender-matched Danes. Participants were followed up on average for 20.6 years, yielding a total of 555,884 person-years of follow-up.

INTERVENTIONS:
Not applicable.

MAIN OUTCOME MEASURES:
The exposed (poliomyelitis) cohort and the unexposed (control) cohort were followed up for somatic hospitalization from 1977 to 1999 in the Danish Hospital Discharge Register. The incidence rate ratio (IRR) was calculated as the ratio between the incidence rate of disease in the exposed and unexposed cohorts.

RESULTS:
Overall, polio patients had a 1.2- to 1.3-fold increased risk of being hospitalized with pulmonary diseases, heart diseases, gastrointestinal disorders, or diseases of the locomotive apparatus. Among paralytic polio patients, long-term morbidity seems to be associated with the acute severity of poliomyelitis, as well as young age at infection. Paralytic patients, who contracted respiratory polio under the age of 5, had the highest risk of being hospitalized with lung diseases (IRR=7.26; 95% confidence interval [CI], 3.06-18.33), diseases of the locomotive apparatus (IRR=4.05; 95% CI, 1.66-9.86), heart diseases (IRR=1.70; 95% CI, 0.65-3.98), and diseases of the digestive system (IRR= 2.23; 95% CI, 1.03-4.62). Surprisingly, patients without paralyses, especially women, also had an increased morbidity.

CONCLUSIONS:
Overall, a history of poliomyelitis was associated with a slightly increased morbidity measured by hospitalizations. Long-term morbidity was highest among respiratory polio patients; however, patients presumably left without any residual symptoms also had an increased morbidity.

Conclusions: Overall, a history of poliomyelitis was associated with a slightly increased morbidity measured by hospitalizations. Long-term morbidity was highest among respiratory polio patients; however, patients presumably left without any residual symptoms also had an increased morbidity.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Lipid metabolic pathways converge in motor neuron degenerative diseases
Author: Olivia J Rickman, Emma L Baple, and Andrew H Crosby
Affiliation: Medical Research (Level 4), RILD Wellcome Wolfson Centre, University of Exeter Medical School, Royal Devon and Exeter NHS Foundation Trust, Barrack Road, Exeter, EX2 5DW, UK

Correspondence to: Professor Andrew Crosby Professor of Human Genetics University of Exeter Medical School Medical Research (Level 4) RILD Wellcome Wolfson Centre Royal Devon and Exeter NHS Foundation Trust Barrack Road, Exeter, EX2 5DW, UK E-mail: [email protected]
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Brain. 2020 Apr; 143(4): 1073–1087.
Published online 2019 Dec 18. doi: 10.1093/brain/awz382
Publication Year and Month: 2019 12

Abstract: Motor neuron diseases (MNDs) encompass an extensive and heterogeneous group of upper and/or lower motor neuron degenerative disorders, in which the particular clinical outcomes stem from the specific neuronal component involved in each condition. While mutations in a large number of molecules associated with lipid metabolism are known to be implicated in MNDs, there remains a lack of clarity regarding the key functional pathways involved, and their inter-relationships. This review highlights evidence that defines defects within two specific lipid (cholesterol/oxysterol and phosphatidylethanolamine) biosynthetic cascades as being centrally involved in MND, particularly hereditary spastic paraplegia. We also identify how other MND-associated molecules may impact these cascades, in particular through impaired organellar interfacing, to propose ‘subcellular lipidome imbalance’ as a likely common pathomolecular theme in MND. Further exploration of this mechanism has the potential to identify new therapeutic targets and management strategies for modulation of disease progression in hereditary spastic paraplegias and other MNDs.

Keywords: HSP, MND, cholesterol, mitochondria, lipidome imbalance

Conclusions: The development of new methodologies and approaches are important to delineate the specific subcellular biomarker deficits, such as oxysterol and PE imbalance, in HSP and MND. As well as providing potentially powerful biomarkers of disease, such tools may enable monitoring of treatment efficacy of therapeutics to re-address disease-associated lipidome imbalances. Specific genetic subtypes may be more amenable to treatment at targeting (for example) feedback systems, such as CDCA in CTX, or addressing oxysterol imbalance in SPG5. Genetic subtypes of disease leading to more complex subcellular outcomes may require multiple treatment approaches to address the specific mechanistic basis of each condition, and it may be unlikely that one approach will entail a ‘fix all’ treatment. Ultimately, clearer definition of the subcellular lipidome (and other) biological pathways underlying MND and HSP will pave the way for a more elegant approaches for predicting onset and severity of disease, and for designing and monitoring new therapeutic approaches.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Management of post-polio syndrome (1997)
Author: Trojan DA, Finch L
Affiliation: Not stated
Journal: NeuroRehabilitation
Citation: NeuroRehabil. 1997;8:93-105
Publication Year and Month: 1997 08

Abstract: The management of patients presenting with post-poliomyelitis syndrome is discussed. It is essential to identify and treat other medical and neurological conditions which could produce these symptoms. New weakness can be managed with exercise, avoidance of muscular overuse, weight loss, orthoses and assistive devices. Fatigue can be managed with energy conservation techniques. The management of pain is dependent upon its causes. Treatments are reviewed.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Management of postpolio syndrome (2010)
Author: Gonzalez H, Olsson T, Borg K
Affiliation: Division of Rehabilitation Medicine, Department of Clinical Sciences, Danderyd Hospital, Karolinska Institute, Stockholm, Sweden – [email protected]
Journal: The Lancet Neurology
Citation: Lancet Neurol. 2010 Jun; 9(6):634-42 and Comment in: Lancet Neurol. 2010 Jun; 9(6):561-3
Publication Year and Month: 2010 06

Abstract: Postpolio syndrome is characterised by the exacerbation of existing or new health problems, most often muscle weakness and fatigability, general fatigue, and pain, after a period of stability subsequent to acute polio infection. Diagnosis is based on the presence of a lower motor neuron disorder that is supported by neurophysiological findings, with exclusion of other disorders as causes of the new symptoms. The muscle-related effects of postpolio syndrome are possibly associated with an ongoing process of denervation and reinnervation, reaching a point at which denervation is no longer compensated for by reinnervation. The cause of this denervation is unknown, but an inflammatory process is possible. Rehabilitation in patients with postpolio syndrome should take a multiprofessional and multidisciplinary approach, with an emphasis on physiotherapy, including enhanced or individually modified physical activity, and muscle training. Patients with postpolio syndrome should be advised to avoid both inactivity and overuse of weak muscles. Evaluation of the need for orthoses and assistive devices is often required.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Motoneuron Disease and Past Poliomyelitis in England and Wales
Author: Martyn CN, Barker DJP, Osmond C.
Affiliation: MRC Environmental Epidemiology Unit, Southampton Hospital, United Kingdom
Journal: The Lancet Neurology
Citation: The Lancet, 331(8598):1319-1322

Publication Year and Month: 1988 06

Abstract: Past notification rates for poliomyelitis show a close geographical relation with current mortality from motoneuron disease in England and Wales. The increasing rate of poliomyelitis during the first half of this century and its predilection for affluent places and families were unique amongst infectious diseases. The unusual epidemiology of poliomyelitis is now being paralleled by motoneuron disease. These observations provide new evidence for a causal connection between the two conditions.

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Multiple sclerosis and poliomyelitis. A Danish historical cohort study.
Author: Nielsen NM, Wohlfahrt J, Melbye M, Rasmussen S, Mølbak K, Askgaard DS, Aaby P.
Affiliation: Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark
Journal: Acta Neurologica Scandinavica
Citation: Acta Neurol Scand 2000 Jun;101(6):384-7.
Publication Year and Month: 2000 06

Abstract: OBJECTIVE:
To evaluate whether persons with a history of poliomyelitis are at an increased risk of developing multiple sclerosis (MS).

MATERIAL AND METHODS:
All patients diagnosed with acute poliomyelitis in the greater capital area of Copenhagen, Denmark, between 1919 and 1954 were identified and followed with respect to MS. Information on vital status and diagnosis of sclerosis was obtained through linkage with the Danish Civil Registration System and The Danish Multiple Sclerosis Registry, respectively. Follow-up started on the date of the establishment of the Danish Civil Registration System (April 1, 1968) until death, emigration or December 31, 1996, whichever came first. The observed incidence of MS among polio patients was compared with the expected incidence calculated according to national gender, age and period specific rates of MS.

RESULTS:
During 149,364 years of follow-up, 19 cases of multiple sclerosis were observed among 5652 polio patients compared with 11.0 expected (SIR = 1.73 (1.04-2.74)). The increased risk of MS was most pronounced in polio patients hospitalized during adolescence. Neither gender nor the acute severity of poliomyelitis modified the risk of MS.

CONCLUSION:
Our results are based on small numbers of events, however the findings suggest that the polio patients might be at an increased risk of MS.

Conclusions:

Outcome of Research: More research required

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Comments (if any): There appears a higher incidence of Multiple Sclerosis in this study of Danish patients previously diagnosed with Poliomyelitis. This should not be interpreted that previously contracting Poliomyelitis will lead to Multiple Sclerosis.

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Category: Diagnosis and Management

Title: National Rehabilitation Hospital Limb Classification for Exercise, Research, and Clinical Trials in Post-Polio Patients
Author: Lauro S. Halstead, Anne Carrington Gawne, and Bao T. Pham

Affiliation: The Post-Polio Program; National Rehabilitation Hospital, Washington, DC


Journal: Annals of the New York Academy of Sciences
Citation: The Post-Polio Syndrome: Advances in the Pathogenesis and Treatment Volume 753 pp 343-353 of the Annals of the New York Academy of Sciences May 25, 1995.
Publication Year and Month: 1995 05

Abstract:

Conclusions: A need exists for an objective classification of polio patients for clinical and research purposes that takes into account the focal, asymmetric, and frequent subclinical nature of polio lesions. In order to prescribe a safe, effective exercise program, we developed a five-level (Classes I-V) limb-specific classification system based on remote and recent history, physical examination, and a four-extremity electrodiagnostic study (EMG/NCS). Class I limbs have no history of remote or recent weakness, normal strength, and a normal EMG. Class II limbs have no history of remote or recent weakness (or if remote history of weakness, full recovery occurred), normal strength and EMG evidence of prior anterior horn cell disease (AHCD). Class III limbs have a history of remote weakness with variable recovery, no new weakness, decreased strength, and EMG evidence of prior AHCD. Class IV limbs have a history of remote weakness with variable recovery, new clinical weakness, decreased strength, and EMG evidence of AHCD. Class V limbs have a history of severe weakness with little-to-no recovery, severely decreased strength and atrophy, and few-to-no motor units on EMG. In a prospective study of 400 limbs in 100 consecutive post-polio patients attending our clinic, 94 (23%) limbs were Class I, 88 (22%) were Class II, 95 (24%) were Class III, 75 (19%) were Class IV, and 48 (12%) were Class V. Guidelines for the use of this classification in a clinical/research setting are presented along with sample case histories and class-specific exercise recommendations.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Neurological Symptoms in Danes with a History of Poliomyelitis: Lifelong Follow-Up of Late Symptoms, their Association with Initial Symptoms of Polio, and Presence of Postpolio Syndrome
Author: Kay L. Nielsen N.M. Wanscher B. Jennum P.
Affiliation: Specialized Hospitalet for Polio and Accident Patients, Rødovre, Denmark
Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark
Medical Department 3, Næstved, Slagelse and Ringsted Hospital, Slagelse, Denmark
Danish Center for Sleep Medicine, Department of Clinical Neurophysiology, Rigshospitalet, University of Copenhagen, Glostrup, Denmark
Journal: European Neurology
Citation: 2018, Vol.80, No. 5-6
Publication Year and Month: 2018

Abstract: Background: Previous studies suggest that patients with a history of poliomyelitis (PM) later in life experience a variety of symptoms. These studies were carried out in patients who later in life were admitted to hospital or became members of polio societies and may therefore not be representative of all polio patients. Little data have been published concerning patients actually discharged from hospital with a diagnosis of acute paralytic PM. Objectives: The aim of this study was to compare the prevalence of late symptoms in individuals with a history of paralytic PM with that of controls, and to study whether late symptoms in individuals with a history of PM were associated with symptoms at the acute stage of polio, and finally to compare the prevalence of symptoms in polio patients with postpolio syndrome (PPS) with the prevalence of symptoms in polio patients without PPS. Methods: A questionnaire concerning various symptoms was sent to a previously established cohort of patients, who during the polio epidemics were discharged from the Department of Infectious Disease at Blegdamshospitalet, Copenhagen, with a diagnosis of paralytic PM, and to age- and gender-matched controls without PM. Information about symptoms at the acute stage of disease was obtained from hospital records. Logistic regression analysis with adjustment for age and gender was applied to compare the occurrence of late symptoms in cases and controls and within the above-mentioned groups of individuals with a history of PM. Results: (i) Compared with controls, individuals with a history of polio significantly more often reported muscle symptoms, pain, neuropathic sensory symptoms, and bulbar symptoms; (ii) the occurrence of symptoms did not seem to be related to symptoms of the initial PM; and (iii) symptom prevalence was significantly higher in individuals with a history of polio who reported PPS as compared with those who did not.

Conclusions: Conclusion: Our data indicate that individuals with a history of PM late in life experience a variety of symptoms that cannot be attributed to lesions of the anterior horn. Furthermore, late symptoms do not seem to be related to initial symptoms of the acute stage of PM but to reported PPS. The last finding supports the perception that the cause of PPS is not just normal ageing.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Nonparalytic polio and postpolio syndrome
Author: Halstead LS, Silver JK
Affiliation: National Rehabilitation Hospital, Washington, DC 20010, USA
Journal: American Journal of Physical Medicine & Rehabilitation
Citation: Am J Phys Med Rehabil. 2000 Jan-Feb;79(1):13-8
Publication Year and Month: 2000 01

Abstract: We describe four cases of postpolio syndrome with typical histories, physical examination results, and electrodiagnostic evidence of extensive anterior horn cell disease, as well as the putative pathophysiology of postpolio syndrome in persons with histories of nonparalytic polio and the diagnostic implications for individuals older than 40 yr of age who are experiencing unexplained new weakness, fatigue, and muscle or joint pain. Although the diagnosis of postpolio syndrome traditionally has required a remote history of paralytic polio, many persons such as the ones described here with typical symptoms of postpolio syndrome have no clear history of paralytic disease and are being misdiagnosed. With this in mind, we believe that the diagnostic criteria for postpolio syndrome should be modified to include the following: a history of remote paralytic polio or findings on history, physical examination results, and laboratory studies compatible with poliovirus damage of the central nervous system earlier in life.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Outcome of physiotherapy as part of a multidisciplinary rehabilitation in an unselected polio population with one-year follow-up: an uncontrolled study.
Author: Bertelsen M, Broberg S, Madsen E.
Affiliation: Rehabilitation Centre of the Danish Society of Polio and Accident Victims (PTU), Rødovre, Denmark. [email protected]
Journal: Journal of Rehabilitation Medicine
Citation: 2009 Jan;41(1):85-7.
Publication Year and Month: 2009 01

Abstract: OBJECTIVE:
The aim of this study was to evaluate the outcome of physiotherapy as part of a multidisciplinary rehabilitation.

DESIGN:
Prospective uncontrolled intervention study.

SUBJECTS:
Fifty patients with late effects of polio, first time referred to physiotherapy at the Danish Society of Polio and Accident Victims (PTU) Rehabilitation Centre.

METHODS:
The intervention was physiotherapy as an essential part of an individually planned multidisciplinary rehabilitation. The outcome measures Six-Minute Walk Test and Timed-Stands Test were used to assess the functional capacity. Quality of life was evaluated by Medical Outcome Survey Short Form (SF-36) and fatigue by Multidimensional Fatigue Inventory (MFI-20). Patients were tested at baseline; 3 months after the start of rehabilitation and at one-year follow-up.

RESULTS:
The patients showed significantly better functional capacity on all measurements 3 months after start of intervention and at one-year follow-up. The patients showed significant improvement in 3 of the SF-36 dimensions regarding quality of life, but only the improvement in "general health" remained after one year.

Conclusions: This study shows that patients with late effects of polio, who experience new problems related to polio, can benefit from an individually planned multidisciplinary intervention with emphasis on physiotherapy, and the improvement in physical capacity and general health can remain at one-year follow-up.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Paralytic vs "nonparalytic" polio: distinction without a difference?
Author: Bruno RL
Affiliation: The Post-Polio Institute, Englewood Hospital and Medical Center, New Jersey, USA
Journal: American Journal of Physical Medicine & Rehabilitation
Citation: Am J Phys Med Rehabil. 2000 Jan-Feb;79(1):4-12
Publication Year and Month: 2000 01

Abstract: Nonparalytic polio (NPP) is commonly thought to be synonymous with "abortive polio," in which the poliovirus neither entered the central nervous system nor damaged neurons. Described are two epidemic illness-"The Summer Grippe" and Iceland disease-apparently caused by a low virulence but neuropathic type 2 poliovirus. Studies show that neuronal lesions in the brain and spinal cord and muscle weakness were common in NPP, and epidemiologic studies document late-onset weakness and fatigue in 14% to 42% of NPP survivors. These findings indicate that clinicians should not require a history of paralytic polio, electromyographic evidence of denervation, and new muscle weakness for the diagnosis of "Postpolio Syndrome" but should be aware that NPP, and possibly even poliovirus-induced "minor illnesses," can be associated with acute central nervous system damage and late-onset muscle weakness and fatigue.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Pathogenetic mechanisms of post-polio syndrome: morphological, electrophysiological, virological, and immunological correlations.
Author: Dalakas MC
Affiliation: Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1382, USA.
Journal: Annals of the New York Academy of Sciences
Citation: 1995 May 25;753:167-85.
Publication Year and Month: 1995 05

Abstract: To understand the mechanism of post-poliomyelitis muscular atrophy (PPMA) and the post-polio syndrome (PPS) in general, we performed the following studies: (1) histopathology in spinal cord sections from patients who died 9 days to 44 years after acute paralytic poliomyelitis; (2) enzyme histochemistry, immunocytochemistry (for lymphocyte subsets, MHC antigens and N-CAM) and polymerase chain reaction (PCR) for poliovirus RNA in the muscle biopsies from symptomatic or asymptomatic muscles of post-polio patients; (3) determination of lymphocyte subsets and circulating IgG or IgM antibodies against GM1 and poliovirus; (4) virological studies in the spinal fluid for oligoclonal bands and search for poliovirus genome with PCR; (5) electrophysiological studies including single fiber EMG, fiber density and macro-EMG; and (6) [31P] exercise MRS spectroscopy on previously affected muscles to search for a metabolic correlate of fatigue. These studies concluded that in PPS a continuing dysfunction is present in the spinal cord motor neurons, resulting in ongoing muscle denervation and reinnervation first evident at the axonal branch points. Symptoms are related to attrition of the oversprouting motor neurons which after a period of time cannot support all their axonal sprouts, resulting in failure of re-reinnervation. In some patients with PPS there is also an ongoing immune activation and presence of defective viral particles in the spinal fluid. However, their role in the pathogenesis of PPS is presently unknown.

Conclusions: These studies concluded that in PPS a continuing dysfunction is present in the spinal cord motor neurons, resulting in ongoing muscle denervation and reinnervation first evident at the axonal branch points.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Physical therapy management of the patient with post-polio syndrome. A case report.
Author: Donna J. Twist, Dong M. Ma
Affiliation:
Journal: Physical Therapy
Citation: Volume 66, Issue 9, 1 September 1986, Pages 1403–1406
Publication Year and Month: 1986 09

Abstract: This case report documents the treatment of a patient who experienced progressive muscle weakness and a decrease in function over time that did not appear to be related to any secondary neuromuscular disease. We discuss the relationship between age and maximal muscle function in addition to some general guidelines for rehabilitation. This type of patient can represent a challenge for the physical therapist. This case report, however, illustrates the degree of muscular and functional recovery that can result with a physical therapy program aimed at reducing levels and intensity of exercise, daily activity, and stress.

Conclusions: A combination of short-term goals appears to be essential to the successful management of a patient with post-polio syndrome.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Physiotherapy for poliomyelitis: a descriptive study in the Republic of Congo
Author: Mancini S, Coldiron ME, Nicholas S, Llosa AE, Mouniaman-Nara I, Ngala J, Grais RF, Porten K
Affiliation: Epicentre, Paris, France - [email protected].
Journal: BioMedCentral Research Notes
Citation: BMC Res Notes. 2014 Oct 23;7:755. doi: 10.1186/1756-0500-7-755
Publication Year and Month: 2014 10

Abstract: BACKGROUND: A large poliomyelitis outbreak occurred in 2010 in the Republic of Congo. This paper describes the demographic and clinical characteristics of poliomyelitis cases and their outcomes following physiotherapy.

FINDINGS: Demographic and clinical data were collected on 126 individuals between November 23, 2010 and March 23, 2011. The male/female ratio was 2.5 and the median age was 19 years (IQR: 13.5-23). The most severe forms of the disease were more common in older patients, 81 of the 126 patients (64.3%) had multiple evaluations of muscle strength. Among patients with multiple evaluations, 38.1% had improved strength at final evaluation, 48.3% were stable and 13.6% had decreased strength.

Conclusions: Most acute poliomyelitis patients receiving physiotherapy had improved or stable muscle strength at their final evaluation. These descriptive results highlight the need for further research into the potential benefits of physiotherapy in polio affected patients.

Outcome of Research: More research required.

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Category: Diagnosis and Management, Differential Diagnosis

Title: Plasma CHI3L1 in Amyotrophic Lateral Sclerosis: A Potential Differential Diagnostic Biomarker
Author: Alessandro Bombaci*, Umberto Manera, Giovanni De Marco, Federico Casale, Paolina Salamone, Giuseppe Fuda, Giulia Marchese, Barbara Iazzolino, Laura Peotta, Cristina Moglia, Andrea Calvo and Adriano Chiò
Affiliation: “Rita Levi Montalcini” Department of Neuroscience, University of Turin, 10126 Turin, Italy
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: J. Clin. Med. 2023, 12(6), 2367; https://doi.org/10.3390/jcm12062367
Publication Year and Month: 2023 03

Abstract: Background: Motor neuron diseases (MNDs) are fatal neurodegenerative diseases. Biomarkers could help with defining patients’ prognoses and stratifications. Besides neurofilaments, chitinases are a promising family of possible biomarkers which correlate with neuroinflammatory status. We evaluated the plasmatic levels of CHI3L1 in MNDs, MND mimics, and healthy controls (HCs).

Methods: We used a sandwich ELISA to quantify the CHI3L1 in plasma samples from 44 MND patients, 7 hereditary spastic paraplegia (HSP) patients, 9 MND mimics, and 19 HCs. We also collected a ALSFRSr scale, MRC scale, spirometry, mutational status, progression rate (PR), blood sampling, and neuropsychological evaluation.

Results: The plasma levels of the CHI3L1 were different among groups (p = 0.005). Particularly, the MND mimics showed higher CHI3L1 levels compared with the MND patients and HCs. The CHI3L1 levels did not differ among PMA, PLS, and ALS, and we did not find a correlation among the CHI3L1 levels and clinical scores, spirometry parameters, PR, and neuropsychological features. Of note, the red blood cell count and haemoglobin was correlated with the CHI3L1 levels (respectively, p < 0.001, r = 0.63; p = 0.022, and r = 0.52).

Conclusions: The CHI3L1 plasma levels were increased in the MND mimics cohort compared with MNDs group. The increase of CHI3L1 in neuroinflammatory processes could explain our findings. We confirmed that the CHI3L1 plasma levels did not allow for differentiation between ALS and HCs, nor were they correlated with neuropsychological impairment.

Keywords: biomarker; chitinases; cognitive impairment; differential diagnosis; early diagnosis; MND mimics; red blood cells

Conclusions: The measurement of the plasmatic levels of CHI3L1 could be useful in the differential diagnosis between MNDs and MND mimics. This is an important issue, since the early diagnosis of an MND is a determinant in the early starting of neuroprotective therapy and in clinical trial recruitment.
Further multicentre and longitudinal studies on a larger patient cohort, testing alongside other fluid biomarkers, are needed to better explain the role of CHI3L1 in the diagnosis and prognosis of MNDs and, also, of MND mimics.

Outcome of Research: More research required

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any): This article is helpful for any medical/neurologist trying to determine differential diagnosis of ALS vs post-polio.

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Category: Diagnosis and Management, Orthoses, Surgery

Title: Polio revisited: reviving knowledge and skills to meet the challenge of resurgence
Author: Benjamin Joseph (1) and Hugh Watts (2)
Affiliation: (1) Aster Medcity, Kochi, Kerala India, 18 HIG HUDCO Colony, Manipal, Karnataka 576104 India

(2) Shriners Hospital for Children, Los Angeles, CA USA
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Journal of Children's Orthopaedics

9 (5): 325–338, doi: 10.1007/s11832-015-0678-4
Publication Year and Month: 2015 09

Abstract: Purpose
To date, polio has not been eradicated and there appears to be a resurgence of the disease. Hence, there is a need to revive decision-making skills to treat the effects of polio.

Methods
Here, we outline the aspects of treatment of paralysis following polio based on the literature and personal experience of the authors. The surgical treatment of the lower and upper extremities and the spine have been reviewed. The scope of bracing of the lower limb has been defined.

Results
The effects of polio can be mitigated by judicious correction of deformities, restoration of muscle balance, stabilising unstable joints and compensating for limb length inequality.

Conclusions
As polio has not been eradicated and there is a risk of resurgence of the disease, paediatric orthopaedic surgeons need to be prepared to deal with fresh cases of polio. Revival of old techniques for managing the effects of paralysis following polio is needed.

Keywords: Poliomyelitis, Resurgence, Surgical decision-making, Bracing, Paralytic deformity

Conclusions: Polio has not been eradicated and there is a risk of resurgence of the disease. Paediatric orthopaedic surgeons need to be prepared to deal with fresh cases of polio. Revival of old techniques of managing the effects of paralysis following polio is needed.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Polio survivors perceptions of a multi-disciplinary rehabilitation programme
Author: Atwal, A., Duncan, H., Queally, C., Cedar, S.H.
Affiliation: Nil
Journal: Disability and Rehabilitation
Citation: Atwal, A., Duncan, H., Queally, C., Cedar, S.H. (2017) Polio survivors perceptions of a multi-disciplinary rehabilitation programme. Disability and Rehabilitation. DOI: 10.1080/09638288.2017.1381184
Publication Year and Month: 2017 10

Abstract: Purpose: Post-polio syndrome refers to a late complication of the poliovirus infection. Management of post-polio syndrome is complex due to the extensive symptomology. European and United Kingdom guidelines have advised the use of rehabilitation programmes to manage post-polio syndrome. There is a paucity of research in relation to the effectiveness of rehabilitation interventions. The objective of this study is to explore polio survivor’s perceptions of an in-patient multi-disciplinary rehabilitation programme.

Methods: Semi-structured interviews of community dwelling polio survivors who attended in-patient rehabilitation programme in the United Kingdom. Thematic analysis was used to describe and interpret interview data.

Results: Participants’ experiences were influenced by past experiences of polio and their self-concept. Participants generally had a positive experience and valued being with other polio survivors. Positive strategies, such as pacing and reflection changed their mind-sets into their lives after the programme, though they still faced challenges in daily living. Some participants supported others with post-polio syndrome after completing the programme.

Conclusions: The research identified that participants experienced long term positive benefits from attending a rehabilitation programme. Strategies that users found helpful that explored the effectiveness of interventions to manage polio are not cited within a Cochrane review. If we are to recognise the lived experience and service user empowerment within a model of co- production it is essential that patient preferences are evaluated and used as evidence to justify service provision. Further research is required with polio survivors to explore how best rehabilitation programmes can adopt the principles of co-production.

Implications for Rehabilitation
The patients’ expertise and lived experience must be at the centre of a rehabilitation programme.

Strategies such as pacing and reflection are perceived as important strategies to enable self-management of polio and post-polio syndrome despite the limited evidence base to support these interventions.

Polio rehabilitation programmes should not be time limited and commissioners and therapists need to ensure that follow up support is provided.

When measuring outcomes patient preferences and views must be evaluated.

Outcome of Research: More research required

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Comments (if any): This investigation supports feedback from polio survivors in Australia on the benefits of group-based rehabilitation, especially where there is an opportunity to learn and discuss their rehabilitation with fellow participants.

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Category: Diagnosis and Management

Title: Polioencephalitis, stress, and the etiology of post-polio sequelae
Author: Bruno RL, Frick NM, Cohen J
Affiliation: Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation, East Orange, NJ 07018
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1269-76
Publication Year and Month: 1991 11

Abstract: Post-mortem neurohistopathologies that document polio virus-induced lesions in reticular formation and hypothalamic, thalamic, peptidergic, and monoaminergic neurons in the brain are reviewed from 158 individuals who contracted polio before 1950. This polioencephalitis was found to occur in every case of poliomyelitis, even those without evidence of damage to spinal motor neurons. These findings, in combination with data from the 1990 National Post-Polio Survey and new magnetic resonance imaging studies documenting post-encephalitis-like lesions in the brains of polio survivors, are used to present two hypotheses: 1) polioencephalitic damage to aging reticular activating system and monoaminergic neurons is responsible for post-polio fatigue, and 2) polioencephalitic damage to enkephalin-producing neurons is responsible for hypersensitivity to pain in polio survivors. In addition, the antimetabolic action of glucocorticoids on polio-damaged, metabolically vulnerable neurons may be responsible for the fatigue and muscle weakness reported by polio survivors during emotional stress.

Conclusions: The ability of the polio virus to produce symptoms by its destruction of neurons outside of the anterior horn has been accepted for more than 100 years. It is only our recent experience with PPS that has forced us to recognize that both the people who survived the original viral onslaught and their central nervous systems have been operating for decades under extreme stress. This stress now may be combining with the aging of an extensively damaged but here-to-fore remarkably functional central nervous system to reveal the previously hidden symptoms of polioencephalitis.

The above-presented hypotheses concerning the etiology of PPS fatigue suggest that reductions in both emotional and physical stress will reduce PPS. This is the experience of post-polio clinics throughout the world (36,54,55,56). As PPS fatigue continues to be treated with stress-management, work simplification and energy conservation (see 36,56), the hypotheses are being tested by studying the neuroanatomy, neuroendocrinology and neuropsychology of PPS fatigue. In addition, pharmacological means for stimulating the RAS that do not further metabolically stress its remaining aging, polio-damaged neurons are being tested.

Outcome of Research:

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Category: Diagnosis and Management

Title: Poliomyelitis and Parkinson Disease.
Author: Nielsen NM, Rostgaard K, Hjalgrim H, Aaby P, Askgaard D.
Affiliation: Department of Epidemiology Research, National University Hospital, Copenhagen, Denmark
Journal: The Journal of the American Medical Association
Citation: JAMA. 2002;287(13):1650–1651.
doi:10.1001/jama.287.13.1645
Publication Year and Month: 2002 04

Abstract: Parkinson disease (PD), which is due to loss of dopaminergic neurons in the zona compacta of the substantia nigra,1 may involve both genetic and environmental risk factors.2 Poliovirus is believed to cause neuronal damage in the substantia nigra,3 and thus a history of poliovirus infection may be associated with an increased risk of PD

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Poliomyelitis and the postpolio syndrome
Author: Howard, R.S.
Affiliation: Lane-Fox Unit and Department of Neurology, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London
Journal: The British Medical Journal
(The BMJ)
Citation: 330(7503): 1314–1318.
doi: 10.1136/bmj.330.7503.1314
Publication Year and Month: 2005 06

Abstract: Acute poliomyelitis is now rarely encountered in the United Kingdom, but “imported” poliomyelitis still occurs and it is necessary to distinguish acute poliomyelitis from other causes of acute flaccid paralysis. Despite the obvious success of preventive policies, many patients who had poliomyelitis experience late functional deterioration after periods of prolonged stability—the so called postpolio syndrome. The patterns of disability and their management present unique challenges to the multidisciplinary rehabilitation team.

Conclusions: Although some patients who have had poliomyelitis may later develop wasting, pain, and fatigue in isolation, in most there is significant underlying weakness and skeletal deformity predisposing to functional deterioration. The severe physical stresses of postpolio disability contribute to the development of progressive orthopaedic, respiratory, neurological, and general medical abnormalities, often exacerbated by intercurrent events. These abnormalities may present with atypical clinical features because of the extent of underlying atrophy and weakness, but many are potentially treatable and most patients can be helped to understand and manage increasing disability. It is essential to emphasise that the symptoms, disabilities, and impairments of postpolio functional deterioration are often amenable to treatment. It is also important to urge caution before attributing functional deterioration to a primary “postpolio syndrome” or “progressive postpolio muscular atrophy.”

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Post-polio sequelae - differential diagnosis and management
Author: Maynard FM
Affiliation: Not stated
Journal: Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):857-61
Publication Year and Month: 1985 07

Abstract: Forty-two patients with a past history of poliomyelitis were evaluated at a post-polio clinic for new problems or impairments. Evaluation included a complete history, neurological and biomechanical examination and electrodiagnostic studies. Based on this evaluation patients were placed into three groups: 23 patients were considered to have or likely to have Progressive Post-Polio Muscular Atrophy (PPPMA); 17 patients were considered to have other post-polio sequelae; and two patients had problems unrelated to a past history of polio but mistaken for post-polio sequelae. Musculoskeletal pain was a common complaint among all groups of patients. Twenty-two of the 40 patients with post-polio sequelae were advised to alter their method of ambulation and/or decrease their activity pattern in order to decrease strain and/or excessive exertion of involved muscles. The role of chronic overuse and exercise in producing PPPMA or musculoskeletal pain problems is discussed. Characteristic clinical problems and useful management plans are described.

Conclusions:

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Category: Diagnosis and Management

Title: Post-polio sequelae: physiological and psychological overview
Author: Frick NM, Bruno RL
Affiliation: Not stated
Journal: Rehabilitation Literature
Citation: Rehabil Lit. 1986 May-Jun; 47(5-6):106-11
Publication Year and Month: 1986 05

Abstract: When the Salk and Sabin vaccines brought an end to the annual summer nightmare of polio epidemics, most Americans simply forgot about polio. Even many of those who had paralytic poliomyelitis put the disease out of their minds once they had achieved maximum recovery of function. Unfortunately, polio has again forced itself into the nation's consciousness. Over the past five years, many of those who had polio have been experiencing new and unexpected symptoms that range in severity from being merely unpleasant to severely debilitating.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management, Late Effects of Polio

Title: Post-polio syndrome and the phantom of acute previous poliomyelitis: a systemic entity
Author: Jacqueline Stephanie Fernandes do Nascimento (1), Nicolle dos Santos Moraes Nunes (1), Thais de R. Bessa-Guerra (1*), Marco Antonio Alves Azizi (2), Adalgiza Mafra Moreno (2), Antônio Marcos da Silva Catharino (2), Paulo Henrique de Moura (2), Thiago Rodrigues Gonçalves (2), Renata R. T. Castro (2), Acary Souza Bulle Oliveira (3), Marcos RG de Freitas (4) and Marco Orsini (2)
Affiliation: (1) Graduanda de Medicina na Universidade lguaçu (UNIG) - Nova lguaçu, RJ -Brasil

(2) Professor titular na Universidade lguaçu (UNIG). Nova lguaçu, RJ -Brasil

(3) Neurologista, Doutor em Neurologia pela Escola Paulista de Medicina (UNIFESP) - São Paulo (SP) - Brasil

(4) Neurologista, Doutor, Professor Titular e Chefe do Serviço de Neurologia da UFF, Niterói-RJ - Brasil
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: International Journal of Current Research

Vol. 13, Issue, 04
Publication Year and Month: 2021 04

Abstract: Introduction:
Poliomyelitis, often referred to as infantile paralysis or polio, was first described in 1840 by Jakob Von Heine, a German orthopedic physician. It is an acute and infectious disease caused by an enterovirus of worldwide distribution. With the implementation of immunization, the incidence of cases has reduced exponentially worldwide. However, a portion of individuals who have already developed polio, are now manifesting the late effects of polio, called post polio syndrome (PPS).

Case Report:
PRF, male, system analyst. Diagnosis of acute previous polio at the 18th month of age in a hospital in Fortaleza in 1965. At the age of 18 he started to present a new clinic of muscle fatigue, weakness and inability to perform his daily activities.

Discussion:
The Post-polio syndrome (PPS) was first reported in 1875, by Raymond, when reporting the case of a 19-year-old man, previously infected with acute previous polio and at that time had presented a new clinic of muscle weakness and atrophy. Post-poliomyelitis syndrome is defined as a presentation of the delayed effects of polio with the appearance of new neuromuscular symptoms that occur at least 15 years after clinical and functional stability in the natural history of patients with a previous history of acute previous polio. It can be characterized by: new muscle weakness, atrophy and pain, fatigue, sleep disorders, joint pain, cold intolerance, recent weight gain, respiratory distress and dysphagia. Criteria that support the diagnosis are used, such as: (1) Having been affected with acute previous polio; (2) at least 15 years of clinical stability; (3) new clinic of muscle weakness and fatigue; (4) complete or partial recovery of the functions lost in the poliomyelitis period, with a subsequent decline; (5) no other clinical conditions that explain the case. However, effective monitoring by a multidisciplinary team is necessary to assess the degree of functional and motor deterioration, respiratory capacity, as well as the psychological assessment of the patient.

Conclusion:
Although acute previous poliomyelitis has been eradicated in Brazil, as well as in several countries in the world, it is estimated that today there are still about 12 million individuals with poliomyelitis after-effects.

Conclusions: Although acute previous polio has been eradicated in Brazil, as well as in several countries in the world, it is estimated that today there are still about 12 million individuals with poliomyelitis after-effects. These patients overload the health system, especially those diagnosed with post-polio syndrome. Therefore, studies in different areas of health sciences are extremely important for understanding the pathophysiological mechanisms and with the aim of improving the quality of life of these patients.

Studies that address the biochemical and physiological issue can be useful for understanding the neurodegeneration that occurs in PPS, in addition to composing material for the study of the repercussion of a physical / motor after-effect on one or more organic systems and on functional capacity.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Post-polio syndrome and total health status in a prospective hospital study.
Author: Farbu E, Rekand T, Gilhus NE
Affiliation: Department of Neurology, Haukeland University Hospital, Bergen, Norway
Journal: European Journal of Neurology
Citation: 2003 Jul;10(4):407-13.
Publication Year and Month: 2003 07

Abstract: New loss of function among patients with previous polio is frequently reported and has several causes. All patients referred to the Department of Neurology, Haukeland University Hospital, Bergen, for 13 months during 2000-2001 with diagnosis late effects of polio were examined prospectively to identify their symptoms and loss of function. Eighty-five patients aged 47-91 years with mean of 61 years were included. The most common complaints were pain (44%), muscular weakness (27%), and fatigue (16%). Muscular weakness occurred in lower limbs in 75%, in respiratory muscles in only 5%. Walking in stairs was impaired in 72% and outdoor walking in 65%. Seventeen patients (19%) reported no loss of function. Post-polio syndrome was diagnosed in 26% of the patients. Polio-related loss of function including cervical and lumbosacral radiculopathies, mononeuropathies and degenerative joint disease were found in an additional 53%. Eleven patients (13%) had distinct non-polio-related disorders that caused new loss of function. The remaining 8% had a stable condition.

Conclusions: In conclusion, the majority of polio patients who seek hospital, experience a new loss of function because of polio-related disorders. A careful neurological examination is necessary to identify the correct diagnosis and treatment.

Outcome of Research: More research required

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Category: Clinical Evaluation, Diagnosis and Management, Late Effects of Polio

Title: Post-polio Syndrome: More Than Just a Lower Motor Neuron Disease
Author: Stacey Li Hi Shing, Rangariroyashe H. Chipika, Eoin Finegan, Deirdre Murray, Orla Hardiman, and Peter Bede
Affiliation: Computational Neuroimaging Group, Academic Unit of Neurology, Biomedical Sciences Institute, Trinity College Dublin, Dublin, Ireland
Edited by: Francesca Trojsi, University of Campania, Luigi Vanvitelli Caserta, Italy
Reviewed by: Andrea Romigi, Mediterranean Neurological Institute (IRCCS), Italy; Louisa Ng, The University of Melbourne, Australia
*Correspondence: Peter Bede ei.dct@pedeb
Journal: Frontiers in Neurology
Citation: 10, 773. https://doi.org/10.3389/fneur.2019.00773
Publication Year and Month: 2019 07

Abstract: Post-polio syndrome (PPS) is a neurological condition that affects polio survivors decades after their initial infection. Despite its high prevalence, the etiology of PPS remains elusive, mechanisms of progression are poorly understood, and the condition is notoriously under-researched. While motor dysfunction is a hallmark feature of the condition, generalized fatigue, sleep disturbance, decreased endurance, neuropsychological deficits, sensory symptoms, and chronic pain are also often reported and have considerable quality of life implications in PPS. The non-motor aspects of PPS are particularly challenging to evaluate, quantify, and treat. Generalized fatigue is one of the most distressing symptoms of PPS and is likely to be multifactorial due to weight-gain, respiratory compromise, poor sleep, and polypharmacy. No validated diagnostic, monitoring, or prognostic markers have been developed in PPS to date and the mainstay of therapy centers on symptomatic relief and individualized rehabilitation strategies such as energy conservation and muscle strengthening exercise regimes. Despite a number of large clinical trials in PPS, no effective disease-modifying pharmacological treatments are currently available.

Conclusions: Despite being one of the most devastating neurodegenerative conditions in the world, surprisingly limited research is undertaken in post-polio syndrome. Its pathogenesis remains elusive, no sensitive diagnostic tools have been developed, and validated prognostic and monitoring markers are lacking. Non-motor symptoms of PPS have considerable quality of life implications and are notoriously challenging to manage. The etiology of fatigue in PPS is yet to be elucidated and successful individualized management strategies are needed to maintain mobility, independence, and patient autonomy. There is striking a paucity of neuroimaging studies in PPS that could provide anatomical insights into the substrate of extra-motor symptoms. Ultimately, the characterization of PPS-associated pathology may help research efforts in other motor neuron diseases.

Outcome of Research: More research required

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Comments (if any): This is a good overview of Post-Polio conditions particularly for clinicians new to the topic.

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Category: Diagnosis and Management

Title: Post-poliomyelitis syndrome (2005)
Author: Trojan DA, Cashman NR
Affiliation: Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, 3801 rue Université, Montréal, Québec H3A 2B4, Canada – [email protected]
Journal: Muscle & Nerve
Citation: Muscle Nerve. 2005 Jan;31(1):6-19
Publication Year and Month: 2005 01

Abstract: Post-poliomyelitis syndrome (PPS) is a common neurological disorder that occurs in a large proportion of individuals who have recovered from paralytic poliomyelitis. The main clinical features are new weakness, muscular fatigability, general fatigue, and pain. The primary criteria necessary for the diagnosis of PPS are a history of paralytic poliomyelitis, partial or complete recovery of neurological function followed by a period of stability (usually several decades), persistent new muscle weakness or abnormal muscle fatigability, and the exclusion of other causes of new symptoms. The cause of PPS remains unclear, but is likely due to a distal degeneration of enlarged post-poliomyelitis motor units. Contributing factors to PPS may be aging (with motor neuron loss), overuse, and disuse. PPS is usually a slowly progressive neuromuscular disease. Although there is no specific treatment for PPS, an interdisciplinary management program can be useful in controlling symptoms.

Conclusions:

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Category: Diagnosis and Management, Late Effects of Polio

Title: Post-poliomyelitis syndrome (2019)
Author: Eric Chun Pu Chu1 and Kary Ka Wai Lam2
Affiliation: 1New York Chiropractic and Physiotherapy Center, New York Medical Group, Hong Kong, People’s Republic of China
2Downtown Chiropractic Limited, Hong Kong, People’s Republic of China
Correspondence: Eric Chun Pu ChuNew York Chiropractic and Physiotherapy Centre, New York Medical Group, 41/F Langham Place Office Tower, 8 Argyle Street, Mongkok, Hong Kong, People’s Republic of China, Phone: Tel +852 3 594 7844, Fax: Fax +852 3 594 6193, Email [email protected]
Journal: International Medical Case Reports Journal
Citation: 12, 261–264. https://doi.org/10.2147/IMCRJ.S219481

Publication Year and Month: 2019 08

Abstract: Most developed countries eliminated paralytic poliomyelitis (polio) in the 1970s to 1980s. It was believed that after recovery from acute paralytic poliomyelitis, the physical condition of survivors would remain stable for the rest of their lives. However, the elimination of polio does not equate the end of medical management of polio. Hundreds of thousands of polio survivors worldwide are still at risk of developing the late effects of the disease. Here, we report a case of post-polio syndrome who attended our clinic for the presence of new weakness and neuromuscular problems six decades after recovery from paralytic polio. It is essential that health professionals be aware of these conditions and have an understanding of the underlying pathophysiology of the symptoms.

Conclusions: Post-polio syndrome is related to the exhaustion of the motor units that form decades after the polio attack. This case report describes the effectiveness of manual interventions in assisting our patient in restoring the level of function and alleviating pain. The limitation of the current report is that it is just a single case. Further comparison with more existing therapeutic regimens is warranted to clarify these issues.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Post-poliomyelitis syndrome as a possible viral disease
Author: Baj A (1), Colombo M (1), Headley JL (2), McFarlane JR (3), Liethof MA (4), Toniolo A (5)
Affiliation: (1) Laboratory of Clinical Microbiology, University of Insubria Medical School, Viale Borri 57, 21100 Varese, Italy; (2) Post-Polio Health International, Saint Louis, Missouri, USA; (3) European Polio Union, Huldenberg, Belgium; (4) Polio Australia Incorporated, Kew, Victoria, Australia; (5) Laboratory of Clinical Microbiology, University of Insubria Medical School, Viale Borri 57, 21100 Varese, Italy. Electronic address: [email protected]
Journal: International Journal of Infectious Diseases
Citation: Int J Infect Dis. 2015 May 1;35:107-116. doi: 10.1016/j.ijid.2015.04.018
Publication Year and Month: 2015 05

Abstract: This review summarizes current concepts on post-polio syndrome (PPS), a condition that may arise in polio survivors after partial or complete functional recovery followed by a prolonged interval of stable neurological function. PPS affects 15-20 million people worldwide. Epidemiological data are reported, together with the pathogenic pathways that possibly lead to the progressive degeneration and loss of neuromuscular motor units. As a consequence of PPS, polio survivors experience new weakness, generalized fatigue, atrophy of previously unaffected muscles, and a physical decline that may culminate in the loss of independent life. Emphasis is given to the possible pathogenic role of persistent poliovirus infection and chronic inflammation. These factors could contribute to the neurological and physical decline in polio survivors. A perspective is then given on novel anti-poliovirus compounds and monoclonal antibodies that have been developed to contribute to the final phases of polio eradication. These agents could also be useful for the treatment or prevention of PPS. Some of these compounds/antibodies are in early clinical development. Finally, current clinical trials for PPS are reported. In this area, the intravenous infusion of normal human immunoglobulins appears both feasible and promising.

Conclusions:

Outcome of Research: Effective.

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Category: Diagnosis and Management

Title: Postpolio syndrome
Author: Nollet F, de Visser M
Affiliation: Department of Rehabilitation, Academic Medical Center, University of Amsterdam, PO Box 22660, 1100 DD Amsterdam, the Netherlands – [email protected]
Journal: Archives of Neurology
Citation: Arch Neurol. 2004 Jul;61(7):1142-4
Publication Year and Month: 2004 07

Abstract: This paper has no abstract - this is an extract:

Postpolio syndrome (PPS) refers to a decline of muscle function usually occurring 30 to 40 years after the acute polio episode. This syndrome has been widely recognized only during the last decades, when many people affected by the large epidemics of the previous century experienced new muscle weakness as they grew older. However, cases of late-onset weakening following poliomyelitis were already reported at the end of the 19th century.

Conclusions:

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Category: Diagnosis and Management

Title: Postpolio syndrome and the late effects of poliomyelitis. Part 1. pathogenesis, biomechanical considerations, diagnosis, and investigations.
Author: Lo JK, Robinson LR.
Affiliation: Sunnybrook Health Sciences Centre, Division of Physical Medicine and Rehabilitation, Department of Medicine, University of Toronto, 2075 Bayview Avenue, Toronto, Ontario, Canada
Journal: Muscle & Nerve
Citation: https://doi.org/10.1002/mus.26168
Publication Year and Month: 2018 05

Abstract: Postpolio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. Many theories exist regarding the pathogenesis of PPS, which remains incompletely understood. In contrast, the late effects of poliomyelitis are often a consequence of biomechanical alterations that occur as a result of polio‐related surgeries, musculoskeletal deformities, or weakness. Osteoporosis and fractures of the polio‐involved limbs are common. A comprehensive clinical evaluation with appropriate investigations is essential to fulfilling the established PPS diagnostic criteria. PPS is a diagnosis of exclusion in which a key clinical feature required for the diagnosis is new muscle weakness and/or muscle fatigability that is persistent for at least 1 year. Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS.

Conclusions: Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS.

Outcome of Research: More research required

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Category: Diagnosis and Management, Differential Diagnosis, Gender Differences, Late Effects of Polio

Title: Postpolio Syndrome: A Review of Lived Experiences of Patients
Author: Oluwaseyi Jacob Oluwasanmi, Devaunna Andrene Mckenzie, Idowu Oluwasegun Adewole, Christian O Aluka, James Iyasse, Esther Olunu, and Adegbenro Omotuyi Fakoya1
Affiliation: Department of Microbiology, All Saints University School of Medicine, Commonwealth of Dominica, Roseau, Dominica
1Department of Anatomical Sciences, University of Medicine and Health Sciences, Basseterre, St. Kitts and Nevis
Address for correspondence: Dr. Adegbenro Omotuyi Fakoya, University of Medicine and Health Sciences, Basseterre, St. Kitts and Nevis. E-mail: [email protected]
Journal: International Journal of Applied & Basic Medical Research
Citation: 9(3), 129–134. https://doi.org/10.4103/ijabmr.IJABMR_333_18
Publication Year and Month: 2019 07

Abstract: Postpolio syndrome (PPS) refers to a group of conditions that are present in patients, years after recovery from initial acute paralytic poliomyelitis. About 15%–80% of 20 million polio survivors worldwide will experience exacerbation of symptoms which typically appear 15–30 years after the resolution of initial poliomyelitis. Symptoms include new muscle weakness, fatigue, myalgia, joint pain, dysphagia, and difficulty breathing. Other reported symptoms include cold intolerance, sleep disorder, dysphonia, loss of stamina, musculoskeletal deformities, cardiovascular disorders, psychosocial problems, and restless legs syndrome. These symptoms are attributed to the superimposed neuronal loss of aging with inflammatory mechanisms, but without any convincing evidence of viral reactivation. Risk factors include female gender, respiratory symptoms, normal aging, permanent disability caused by motor neuron damage, muscle overuse and disuse, aging, and immunologic mechanisms. Hypothyroidism-induced myopathy and fibromyalgia are a differential diagnosis for PPS, and exclusion diagnosis is required as confirmatory criteria for PPS. The symptoms of PPS presented determine the course of management.

Keywords: Fatigue, muscle weakness, myalgia, poliomyelitis, postpolio syndrome

Conclusions: PPS is a sequela of acute poliomyelitis which occurs decades after resolution of the initial paralytic or nonparalytic polio. Fatigue, muscle weakness, myalgia, and joint pain are the major symptoms of PPS. Diagnosis of PPS is made based on the exclusion of other conditions that can cause similar symptoms. The quality of life of patients with PPS is significantly affected by the reoccurrence of the constellations of symptoms they experience. Proper assessment and treatment should be performed to prevent severe impairment of function in patients.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Postpolio syndrome: unanswered questions regarding cause, course, risk factors, and therapies
Author: Nollet F
Affiliation: Not stated
Journal: The Lancet Neurology
Citation: Lancet Neurol. 2010 Jun;9(6):561-3 - Comment on: Lancet Neurol. 2010 Jun;9(6):634-42
Publication Year and Month: 2010 06

Abstract: Living with the consequences of poliomyelitis is not recognised as an important health issue at present. However, millions of people worldwide have lasting impairments caused by polio infection, many of whom also had a decline in muscle function and decline in activities of daily living after years of stable functioning.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Post‐polio syndrome and the late effects of poliomyelitis: Part 2. treatment, management, and prognosis.
Author: Lo JK, Robinson LR.
Affiliation: Sunnybrook Health Sciences Centre, Division of Physical Medicine and Rehabilitation, Department of Medicine, University of Toronto, 2075 Bayview Avenue, Toronto, Ontario, M4N 3M5, Canada
Journal: Muscle & Nerve
Citation: https://doi.org/10.1002/mus.26167
Publication Year and Month: 2018 05

Abstract: Post‐polio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. An individualized approach to rehabilitation management is critical. Interventions may include rehabilitation management strategies, adaptive equipment, orthotic equipment, gait/mobility aids, and a variety of therapeutic exercises. The progression of muscle weakness in PPS is typically slow and gradual; however, there is also variability in both the natural history of weakness and functional prognosis. Further research is required to determine the effectiveness of selected medical treatment. Muscle Nerve 58:760–769, 2018

Conclusions: The progression of muscle weakness in PPS is typically slow and gradual; however, there is also variability in both the natural history of weakness and functional prognosis.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Predictive Factors for Post-Poliomyelitis Syndrome
Author: Daria A. Trojan, MD, MSc, Neil R. Cashman, MD, Stanley Shapiro, PhD, Catherine M. Tansey, MSc, John M. Esdaile, MD
Affiliation: From the Department of Neurology (Drs. Trojan, Cashman), Montreal Neurological Institute and Hospital, the Department of Medicine (Dr. Esdaile), Montreal General Hospital, and the Department of Epidemiology and Biostatistics (Dr. Shapiro, Ms. Tansey), McGill University, Montreal, Quebec, Canada.
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil Vol 75, July 1994, 770-777
Publication Year and Month: 1994 07

Abstract: Post-poliomyelitis syndrome (PPS) is generally defined as a clinical syndrome of new weakness, fatigue, and pain in individuals who have previously recovered from acute paralytic poliomyelitis. The purpose of this study was to identify, through a case-control study design, factors that predict subsequent PPS in patients with prior paralytic poliomyelitis. Among patients attending a university-affiliate hospital post-polio clinic, "cases" were patients with new weakness and fatigue, and "controls" were patients without these complaints. A chart review of 353 patients identified 127 cases and 39 controls. Logistic regression modeling was used to calculate adjusted and unadjusted odds ratios. In univariate analyses, significant risk factors for PPS were a greater age at time of presentation to clinic (p = 0.01), a longer time since acute polio (p = 0.01), and more weakness at acute polio (p = 0.02). Other significant associated, but not necessarily causal factors were a recent weight gain (p = 0.005), muscle pain (p = 0.01) particularly that associated with exercise (p = 0.005), and joint pain (p = 0.04). Multivariate analyses revealed that a model containing age at presentation to clinic, severity of weakness at acute polio, muscle pain with exercise, recent weight gain, and joint pain best distinguished cases from controls. Age at acute polio, degree of recovery after polio, weakness at best point after polio, physical activity, and sex were not contributing factors. These findings suggest that the degree of initial motor unit involvement as measured by weakness at acute polio, and possibly the aging process and overuse are important in predicting PPS.

Conclusions: In conclusion, the results from this study provide insight on predictive factors for PPS, and can be applied in the clinical management of patients who have recovered from paralytic poliomyelitis. Our findings support the hypothesis that the severity of initial motor unit involvement as estimated by weakness at acute polio, and possibly the normal ageing process and overuse are important in predicting PPS. Even though patients have no control over the severity of weakness as a result of acute polio, they do have control over some predictive factors for PPS. Patients can be advised that they should avoid gaining weight and exercising to the point of muscle pain because these variables have been found to be strongly associated with PPS. The exact role of physical activity will still need further evaluation; however, the usual recommendations of low-level aerobic exercise with avoidance of muscle pain and fatigue appear valid. Thus, this study can provide the basis for physiologically reasonable and practical advice to post-polio patients to minimize or delay the risk of PPS.

Outcome of Research: Not applicable

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Comments (if any): Paul Cavendish (Clinical Health Educator): The best multivariate model for predicting who will develop PPS indicates that patients who had a greater weakness at acute polio, are currently older, have muscle pain with exercise, a recent weight gain, and joint pain are those most likely to develop PPS. Other factors shown to be important in univariate analyses are a longer time since acute polio, and muscle pain (at rest or with exercise). Age at acute polio, recovery after polio, weakness at "best point" after polio, physical activity, and sex were not contributing factors.

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Category: Diagnosis and Management

Title: Repetitive transcranial magnetic stimulation in treatment of post polio syndrome
Author: Pastuszak, Z., Piusaska-Macoch, R., Stepieri, A., Czernicki, Z.,
Affiliation: Department of Neurosurgery, Mossakowski Medical Research Centre, Polish Academy of Sciences, A. Pawińskiego 5, 02-106 Warsaw, Poland

Department of Neurology, Military Institute of Medicine, Szaserów 128, 04-141 Warsaw, Poland

Department of Neurosurgery, Warsaw University of Medicine, Cegłowska 80, 01-809 Warsaw, Poland
Journal: Neurologia i Neurochirurgia Polska
Citation: Volume 52 (2): 2018, Pages 281-284
Publication Year and Month: 2018 03

Abstract: Post polio syndrome is a rare disease that occurs decades after polio virus infection. Repetitive transcranial magnetic stimulation (rTMS) is a treatment option with proved effectiveness in drug resistant depression. Possibly it can be helpful in therapy of other neurological diseases including post polio syndrome.

Conclusions: rTMS can be an effective method in treatment of post polio syndrome but further studies with larger group need to be done to confirm that data.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Risk factors for post-polio syndrome among an Italian population: a case-control study.
Author: Bertolasi L, Acler M, dall'Ora E, Gajofatto A, Frasson E, Tocco P, Turri M, Ferlisi M, Fiorini M, Pimazzoni F, Squintani G, Martini M, Danzi B, Monaco S.
Affiliation: Section of Neurology, Department of Neurological, Neuropsychological, Morphological and Motor Sciences, University of Verona, Piazzale L.A. Scuro 10, 37134, Verona, Italy. [email protected]
Journal: Neurological sciences: official journal of the Italian neurological society
Citation: 2012 Dec;33(6):1271-5. doi: 10.1007/s10072-012-0931-2. Epub 2012 Jan 14.
Publication Year and Month: 2012 01

Abstract: Post-polio syndrome (PPS) is a clinical syndrome of new weakness, fatigue and musculoskeletal pain occurring in a variable proportion of polio survivors decades after acute disease. To date, several risk factors for PPS development have been reported, although the etiology of this disorder remains elusive. Using a case-control design, we aimed to assess risk indicators for PPS in a group of Italian polio survivors. Subjects with prior poliomyelitis attending the rehabilitation hospital of Malcesine, Italy, were the target population. Patients with PPS, diagnosed according to the European Federation of Neurological Societies criteria, served as cases, while patients not meeting diagnostic criteria for PPS were used as controls. All subjects were assessed through a structured questionnaire made of 82 questions and neurological examination. The association with investigated risk factors (sex, age at polio onset, age at onset of symptoms, extension and severity of polio, employment) was analyzed by the calculation of the odds ratio. A total of 161 out of 391 eligible patients met the adopted diagnostic criteria for PPS, giving a frequency of 41.2%. Symptoms most frequently complained by PPS patients were loss of muscle strength, loss of resistance, loss of muscle volume and generalized fatigue. Female gender, the presence of respiratory disturbance during the acute phase of polio and the use of orthoses and aids during the recovery and stabilization represented independent risk factors for PPS in the studied population.

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Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Safety and feasibility of transcranial direct current stimulation for patients with post-polio syndrome
Author: Y. Matsushima, A. Hachisuka, H. Itoh, K. Sugimoto, S. Saeki
Affiliation: Department of rehabilitation medicine, University of occupational and environmental health, Japan
Journal: Brain Stimulation
Citation: (2019) 385-592
Publication Year and Month: 2019

Abstract: Post-polio syndrome (PPS) is generally defined as a clinical syndrome consisting of new muscle weakness, fatigue, and pain in poliomyelitis survivors. In PPS, there is no definitively validated treatment option, although Acler M et al. reported that anodal transcranial direct current stimulation (tDCS) over pre-motor cortex for 15 days improved sleep and fatigue symptoms in patients with PPS. tDCS may be a valuable, non-invasive new tool for managing patients with PPS.

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Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Sense of Coherence in persons with late effects of polio
Author: Nolvi M, Brogardh C, Jacobsson L, Lexell J
Affiliation: Maria Nolvi, MD, Department of Health Sciences, PO Box 157, Lund University, SE-221 00 Lund, Sweden
Journal: NeuroRehabilitation
Citation: Vol. 42, no. 1, pp. 103-111, 2018
Publication Year and Month: 2018 01

Abstract: BACKGROUND:Sense of Coherence (SOC) is important for successful adaptation and mental well-being in people with life-long medical conditions. Late effects of polio (LEoP) often lead to a life-long disability, but no study has assessed SOC in this population. OBJECTIVE:To assess SOC in persons with LEoP and to explore the association between SOC, demographics (age, gender, marital status and level of education) and variables related to LEoP (age at polio onset, number of years from polio until onset of LEoP and self-rated disability). METHOD:Ninety-three community-dwelling persons with clinically verified LEoP responded to a postal survey with the Sense of Coherence Scale (SOC-13). A hierarchical multiple regression analysis was performed to explore the associations with SOC. RESULTS:SOC varied considerably among the participants. The mean and median SOC-13 total sum score was 71.8 and 76 points, which is similar to age-matched non-disabled people. The number of years before onset of LEoP and self-rated disability together with the participants’ marital status and level of education explained 37% (p < 0.001) of the variance in SOC.

Conclusions: CONCLUSION:Persons with LEoP have a level of sense of coherence (SOC) indicating that they generally have the ability to understand, handle and being motivated when dealing with stressful events and problems arising in their lives as a result of their disability. Being married and having a higher education, living many years before onset of LEoP and perceiving a mild to moderate disability contributed to a strong SOC.

Outcome of Research: More research required

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Category: Brain, Diagnosis and Management, Late Effects of Polio, Post-Polio Motor Unit

Title: Spinal cord gray matter atrophy is associated with functional decline in post-polio syndrome
Author: Maria Janina Wendebourg (1,2), Matthias Weigel (1,2,3,4,5), Laura Richter (1), Vanya Gocheva (6), Patricia Hafner (6), Anna-Lena Orsini (6), Valentina Crepulja (1,2), Simone Schmidt (6), Antal Huck (4), Johanna Oechtering (1), Maria Blatow (7), Tanja Haas (3,4), Cristina Granziera (1,2,5), Ludwig Kappos (1,2,5), Philippe Cattin (4), Oliver Bieri (3,4) Dirk Fischer (6), Regina Schlaeger (1,2,5)
Affiliation: 1. Neurology Clinic and Policlinic, Department of Clinical Research, University Hospital Basel, University of Basel, Basel, Switzerland

2. Translational Imaging in Neurology (ThINk), Department of Biomedical Engineering, University of Basel, Basel, Switzerland

3. Division of Radiological Physics, Department of Radiology, University Hospital Basel, Basel, Switzerland

4. Department of Biomedical Engineering, University of Basel, Basel, Switzerland

5. MS Center and Research Center for Clinical Neuroimmunology and Neuroscience Basel (RC2NB), University Hospital Basel and University of Basel, Basel, Switzerland

6. Division of Pediatric Neurology, University of Basel Children's Hospital, Basel, Switzerland

7. Department of Neuroradiology, Clinical Neuroscience Center, University Hospital Zurich, University of Zurich, Zurich,
Journal: European Journal of Neurology
Citation: Eur J Neurol. 2022;00:1–11.
DOI: 10.1111/ene.15261
Publication Year and Month: 2022 01

Abstract: Objective: To determine if patients with post- polio syndrome (PPS) show spinal cord gray matter (SCGM) atrophy and to assess associations between SCGM atrophy, muscle strength and patient- reported functional decline.

Methods: Twenty patients diagnosed with PPS (March of Dimes criteria) and 20 age- and sex- matched healthy controls (HC) underwent 3T axial 2D- rAMIRA magnetic resonance imaging at the intervertebral disc levels C2/C3–C6/C7, T9/T10 and the lumbar enlarge-ment level (Tmax) (0.5 × 0.5 mm2 in- plane resolution). SCGM areas were segmented manu-ally by two independent raters. Muscle strength, self-reported fatigue, depression and pain measures were assessed.

Results: Post- polio syndrome patients showed significantly and preferentially re-duced SCGM areas at C2/C3 (p= 0.048), C3/C4 (p= 0.001), C4/C5 (p< 0.001), C5/C6 (p= 0.004) and Tmax (p= 0.041) compared to HC. SCGM areas were significantly associated with muscle strength in corresponding myotomes even after adjustment for fatigue, pain and depression. SCGM areaTmax together with age and sex explained 68% of ankle dorsiflexion strength variance. No associations were found with age at or time since infection. Patients reporting PPS- related decline in arm function showed significant cervical SCGM atrophy compared to stable patients adjusted for initial disease severity.

Conclusions: Patients with PPS show significant SCGM atrophy that correlates with mus-cle strength and is associated with PPS- related functional decline. Our findings suggest a secondary neurodegenerative process underlying SCGM atrophy in PPS that is not ex-plained by aging or residua of the initial infection alone. Confirmation by longitudinal studies is needed. The described imaging methodology is promising for developing novel imaging surrogates for SCGM diseases.

Conclusions: The rAMIRA approach is a novel, promising, clinically feasible and sensitive method for segment-wise quantitation of GM atrophy in the cervical and thoracic SC in patients with lower motor neuron disorders. This study demonstrated its clinical applicability and vali-dated it in patients with PPS, a presumed pure, lower motor neuron disorder, which can serve as a model for other neurodegenerative, genetic or autoimmune diseases of the SCGM.

Patients with PPS show significant SCGM atrophy, particularly at levels close to the cervical and lumbar enlargements. Even after adjustment for the level of depression, fatigue and pain, potential confounding symptoms frequently observed in PPS, SCGM atrophy is significantly and segment-wise associated with muscle strength in corresponding myotomes. Moreover, SCGM atrophy is associated with patient-reported PPS-related functional decline. Secondary analyses suggest that SCGM atrophy is rather due to a second dis-ease phase than being a sole residuum of the initial infection or a pure aging effect. These observations support the hypothesis of a focally accentuated neurodegenerative process in the SC underlying PPS. Larger, ideally multicentric, longitudinal studies conducted over a sufficiently long timespan are an important next step to confirm our results and gain more insights into the development of SCGM atrophy over time and its correlation to clinical symptom evolution in patients with PPS.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Stroke Risk in Poliomyelitis Survivors: A Nationwide Population-Based Study
Author: Wu C-H, Liou T-H, Chen H-H, Sun T-Y, Chen K-H, Chang K-H
Affiliation: Wu, Sun, KH Chang: Chung-Yuan Christian University
Liou: Shuang Ho Hospital and Taipei Medical University, Taipei
HH Chen; Chang: Wan Fang Hospital
Chang: Taipei Medical University, Taipei
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Volume 93, Issue 12, Pages 2184–2188
Publication Year and Month: 2012 12

Abstract: Objectives
To assess the prevalence and risk of stroke among adults with polio and controls.

Design
A prospective, probability-sampling, 6-year population-based cohort study.

Setting
A National Health Insurance Research Database consisting of 316,355 randomly selected enrollees. The database is related to a National Health Insurance program with more than 22 million participants.

Participants
After excluding patients under 40 years of age, polio patients (N=212) (mean age ± SD, 54.0±10.2y; 57.1% men) were identified from the database from January 1, 2003 to December 31, 2008. For each polio patient, 2 age- and sex-matched patients were recruited as controls. Control patients did not have any neuromuscular diseases commonly found in childhood. The frequencies of patients with potential risk factors for stroke were assessed.

Intervention
None.

Main Outcome Measure
The prevalence and the adjusted odds ratio of ischemic stroke among polio patients and the controls were estimated.

Results
Polio patients had a higher prevalence of stroke (10.8% vs 2.4%, P<.001) than the controls. Polio patients with hypertension had a much higher prevalence of stroke (23.0%). The risk of stroke was higher for polio patients compared with the controls, yielding an adjusted odds ratio of 4.17 (95% confidence interval, 1.84–9.45, P<.001). Polio was a significant risk factor for stroke independent from hypertension, diabetes mellitus, hyperlipidemia, and cardiac diseases.

Conclusions
Adults with polio had a high prevalence of ischemic stroke. Polio was an additional risk factor for stroke. Polio patients with hypertension might potentiate the risk of stroke. Developing a health promotion program, suitable for polio patients, to increase participation in activities and exercises may be essential, especially for polio patients with hypertension.

Conclusions:

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: The cultural context of polio biographies
Author: Scheer J, Luborsky ML
Affiliation: National Rehabilitation Hospital Research Center, Washington, DC 20010
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1173-81
Publication Year and Month: 1991 11

Abstract: Cultural contexts influence the ways individuals interpret and experience functional losses associated with post-polio sequelae. Using in-depth multiple interview case studies from two National Institute on Aging projects, the concept of “biographies” is presented to place the individuals’ polio-related experiences within the context of their lives. Two major cultural contexts shape the construction of polio biographies: normative life course expectations and developmental tasks; and traditions associated with polio recovery and rehabilitation. The authors identify key dimensions of personal concern among polio survivors that can be used as entrance points for effective clinical intervention and to promote treatment compliance.

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Category: Diagnosis and Management

Title: The post-polio syndrome as an evolved clinical entity. Definition and clinical description.
Author: Dalakas MC
Affiliation: Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
Journal: Annals of the New York Academy of Sciences
Citation: Ann N Y Acad Sci. 1995 May 25;753:68-80
Publication Year and Month: 1995 05

Abstract: Post-polio syndrome (PPS) refers to the new neuromuscular symptoms that occur at least 15 years after stability in patients with prior acute paralytic polio-myelitis. They include: (1) new muscle weakness and atrophy in the limbs, the bulbar or the respiratory muscles [post-poliomyelitis muscular atrophy (PPMA)] and (2) excessive muscle fatigue and diminished physical endurance. PPS is a clinical diagnosis that requires exclusion of all other medical, neurological, orthopedic or psychiatric diseases that could explain the cause of the new symptoms. Routine electromyography is useful to confirm chronic and ongoing denervation and exclude neuropathies. Muscle biopsy, single fiber electromyography (EMG), macro-EMG, serum antibody titers to polio virus, and spinal fluid studies are very useful research tools but they are rarely needed to establish the clinical diagnosis. PPS is a slowly progressive phenomenon with periods of stability that vary from 3 to 10 years. Current evidence indicates that PPS is the evolution of a subclinically ongoing motor neuron dysfunction that begins after the time of the acute polio. It is clinically manifested as PPS when the well-compensated reinnervating process crosses a critical threshold beyond which the remaining motor neurons cannot maintain the innervation to all the muscle fibers within their motor unit territory.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: The split hand syndrome in ALS and post-polio-syndrome
Author: M.Hoheisel, L.Burmeister, M.Tesch
Affiliation: Schlosspark-Klinik Berlin, Neurology, Berlin, Germany
Journal: Clinical Neurophysiology
Citation: Volume 129, Issue 8, August 2018, Pages e82-e83

Publication Year and Month: 2018 08

Abstract: Introduction
Electrodiagnostic evaluation for amyotrophic lateral sclerosis (ALS) relies on extensive measurements. As one diagnostic clue, the split-hand-index (SHI) was proposed. It compares the compound muscle action potential (CMAP) of the abductor pollicis brevis (APB) muscle with the CMAP of the abductor digiti minimi (ADM) muscle.

In ALS, asymmetric atrophy of APB and ADM results in the index being reduced compared to the healthy population. This holds true despite the fact, that there is the same segmental innervation C8 for both examined muscles, as was previously discussed. Several studies have shown a diagnostic value in differentiating ALS from other motorneuron-diseases by means of the SHI, claiming a specific form of neurodegeneration in ALS, which is less marked for example in lower motor neuron disease (LMND), spinal muscle atrophy (SMA) or Hirayama disease.

In our study, we aimed to compare the SHI of ALS-patients with our cohort of patients with post-polio-syndrome (PPS) to find out, whether it has a discriminative value in these patients too and to add knowledge to the proposed neuroscientific explanations of asymmetric thenar/hypothenar-atrophy.

Methods
We conducted a retrospective analysis of our post-polio cohort since 1997. All patients were screened whether CMAPs of APB and ADM were collected. For comparison, we screened electrodiagnostic reports of all patients with a diagnosis of ALS for collected CMAPs of APB and ADM. We excluded patients with neuropathy of the median nerve (NMN) by means of a prolonged distal motoric latency (>4,4 ms). Finally we randomly chose the same number of patients from our reports in that time period with normal results (NR), by excluding diagnosis of NMN, acute polyneuropathies and radiculo- and plexopathies of the arm. We calculated the SHI by dividing the CMAP of APB by the CMAP of ADM.

Results
We found a significant difference (p = 0,01) of the SHI between ALS patients (0,97 ± 0,84) and the NR-group (1,26 ± 0,72). The SHI of the PPS-group (0,91 ± 0,55) was not significantly different compared to the NR group (p = 0,08), but showed a trend. Comparing the ALS-group with the PPS-patients, we found no statistically relevant difference (p = 0,83).

Conclusions: As expected, we could reproduce a significant decrease of the SHI in ALS patients. However our results showed no statistically relevant difference between ALS-patients and PPS-patients when comparing the SHI. There is a broader distribution of values in the PPS-group, including cases of very high grade asymmetric atrophy of APB and ADM. A low SHI therefore is of no help in differentiating PPS from ALS-patients.

As a limitation of our study it is important to acknowledge the retrospective study type and a possible selection bias of patients who suffer from an already clinically visible asymmetry, possible increasing electrodiagnostic evaluation numbers of the hand.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: The treatment of fatigue by non-invasive brain stimulation.
Author: Lefaucheur JP, Chalah MA, Mhalla A, Palm U, Ayache SS, Mylius V.
Affiliation: Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Service de physiologie-Explorations fonctionnelles, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, Créteil, France. Electronic address: [email protected].

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Service de physiologie-Explorations fonctionnelles, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, Créteil, France.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Department of Psychiatry and Psychotherapy, Klinikum der Universität München, Munich, Germany.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Department of Neurology, Philipps University, Marburg, Germany; Department of Neurology, Center for Neurorehabilitation, Valens, Switzerland.
Journal: Clinical Neurophysiology
Citation: 2017 Apr;47(2):173-184.
Publication Year and Month: 2017 04

Abstract: The use of non-invasive brain neurostimulation (NIBS) techniques to treat neurological or psychiatric diseases is currently under development. Fatigue is a commonly observed symptom in the field of potentially treatable pathologies by NIBS, yet very little data has been published regarding its treatment. We conducted a review of the literature until the end of February 2017 to analyze all the studies that reported a clinical assessment of the effects of NIBS techniques on fatigue. We have limited our analysis to repetitive transcranial magnetic stimulation (rTMS) and transcranial direct current stimulation (tDCS). We found only 15 studies on this subject, including 8 tDCS studies and 7 rTMS studies. Of the tDCS studies, 6 concerned patients with multiple sclerosis while 6 rTMS studies concerned fibromyalgia or chronic fatigue syndrome. The remaining 3 studies included patients with post-polio syndrome, Parkinson's disease and amyotrophic lateral sclerosis. Three cortical regions were targeted: the primary sensorimotor cortex, the dorsolateral prefrontal cortex and the posterior parietal cortex. In all cases, tDCS protocols were performed according to a bipolar montage with the anode over the cortical target. On the other hand, rTMS protocols consisted of either high-frequency phasic stimulation or low-frequency tonic stimulation. The results available to date are still too few, partial and heterogeneous as to the methods applied, the clinical profile of the patients and the variables studied (different fatigue scores) in order to draw any conclusion. However, the effects obtained, especially in multiple sclerosis and fibromyalgia, are really carriers of therapeutic hope.

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Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Treatment for postpolio syndrome
Author: Koopman FS, Beelen A, Gilhus NE, de Visser M, Nollet F
Affiliation: Department of Rehabilitation, University of Amsterdam Academic Medical Center, PO Box 22660, Amsterdam, North Holland, Netherlands, 1100 DD
Journal: Cochrane Database of Systematic Reviews
Citation: Cochrane Database Syst Rev. 2015 May 18;5:CD007818
Publication Year and Month: 2015 05

Abstract: BACKGROUND: Postpolio syndrome (PPS) may affect survivors of paralytic poliomyelitis and is characterised by a complex of neuromuscular symptoms leading to a decline in physical functioning. The effectiveness of pharmacological treatment and rehabilitation management in PPS is not yet established. This is an update of a review first published in 2011.

OBJECTIVES: To systematically review the evidence from randomised and quasi-randomised controlled trials for the effect of any pharmacological or non-pharmacological treatment for PPS compared to placebo, usual care or no treatment.

SEARCH METHODS: We searched the following databases on 21 July 2014: Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, PsycINFO and CINAHL Plus. We also checked reference lists of all relevant articles, searched the Database of Abstracts of Reviews of Effects (DARE), the Health Technology Assessment (HTA) Database and trial registers and contacted investigators known to be involved in research in this area.

SELECTION CRITERIA: Randomised and quasi-randomised trials of any form of pharmacological or non-pharmacological treatment for people with PPS. The primary outcome was self perceived activity limitations and secondary outcomes were muscle strength, muscle endurance, fatigue, pain and adverse events.

DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by The Cochrane Collaboration.

MAIN RESULTS: We included 10 pharmacological (modafinil, intravenous immunoglobulin (IVIg), pyridostigmine, lamotrigine, amantadine, prednisone) and three non-pharmacological (muscle strengthening, rehabilitation in a warm climate (that is temperature ± 25°C, dry and sunny) and a cold climate (that is temperature ± 0°C, rainy or snowy), static magnetic fields) studies with a total of 675 participants with PPS in this review. None of the included studies were completely free from any risk of bias, the most prevalent risk of bias being lack of blinding.There was moderate- and low-quality evidence that IVIg has no beneficial effect on activity limitations in the short term and long term, respectively, and inconsistency in the evidence for effectiveness on muscle strength. IVIg caused minor adverse events in a substantial proportion of the participants. Results of one trial provided very low-quality evidence that lamotrigine might be effective in reducing pain and fatigue, resulting in fewer activity limitations without generating adverse events. Data from two single trials suggested that muscle strengthening of thumb muscles (very low-quality evidence) and static magnetic fields (moderate-quality evidence) are safe and beneficial for improving muscle strength and pain, respectively, with unknown effects on activity limitations. Finally, there was evidence varying from very low quality to high quality that modafinil, pyridostigmine, amantadine, prednisone and rehabilitation in a warm or cold climate are not beneficial in PPS.

Conclusions: Due to insufficient good-quality data and lack of randomised studies, it was impossible to draw definite conclusions about the effectiveness of interventions for PPS. Results indicated that IVIg, lamotrigine, muscle strengthening exercises and static magnetic fields may be beneficial but need further investigation to clarify whether any real and meaningful effect exists.

Outcome of Research: More research required.

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Category: Diagnosis and Management

Title: Unraveling the transmission ecology of polio
Author: Martinez-Bakker M (1), King AA (1,2), Rohani P (1,2)
Affiliation: (1) Department of Ecology & Evolutionary Biology, University of Michigan, Ann Arbor, Michigan, United States of America; (2) Center for the Study of Complex Systems, University of Michigan, Ann Arbor, Michigan, United States of America; Fogarty International Center, National Institutes of Health, Bethesda, Maryland, United States of America
Journal: Public Library of Science
Citation: PLoS Biol. 2015 Jun;13(6): e1002172. doi:10.1371/journal.pbio.1002172
Publication Year and Month: 2015 06

Abstract: Sustained and coordinated vaccination efforts have brought polio eradication within reach. Anticipating the eradication of wild poliovirus (WPV) and the subsequent challenges in preventing its re-emergence, we look to the past to identify why polio rose to epidemic levels in the mid-20th century, and how WPV persisted over large geographic scales. We analyzed an extensive epidemiological dataset, spanning the 1930s to the 1950s and spatially replicated across each state in the United States, to glean insight into the drivers of polio’s historical expansion and the ecological mode of its persistence prior to vaccine introduction. We document a latitudinal gradient in polio’s seasonality. Additionally, we fitted and validated mechanistic transmission models to data from each US state independently. The fitted models revealed that: (1) polio persistence was the product of a dynamic mosaic of source and sink populations; (2) geographic heterogeneity of seasonal transmission conditions account for the latitudinal structure of polio epidemics; (3) contrary to the prevailing “disease of development” hypothesis, our analyses demonstrate that polio’s historical expansion was straightforwardly explained by demographic trends rather than improvements in sanitation and hygiene; and (4) the absence of clinical disease is not a reliable indicator of polio transmission, because widespread polio transmission was likely in the multiyear absence of clinical disease. As the world edges closer to global polio eradication and continues the strategic withdrawal of the Oral Polio Vaccine (OPV), the regular identification of, and rapid response to, these silent chains of transmission is of the utmost importance.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Update on current and emerging treatment options for post-polio syndrome
Author: Farbu E
Affiliation: Neurocenter and National Competence Center for Movement Disorders, Stavanger University Hospital, Stavanger, Norway
Journal: Therapeutics and Clinical Risk Management
Citation: Ther Clin Risk Manag. 2010 Jul 21;6:307-13
Publication Year and Month: 2010 07

Abstract: Post-polio syndrome (PPS) refers to the clinical deterioration experienced by many polio survivors several decades after their acute illness. The symptoms are new muscle weakness, decreased muscle endurance, fatigue, muscle pain, joint pain, cold intolerance, and this typical clinical entity is reported from different parts of the world. The pathophysiology behind PPS is not fully understood, but a combination of distal degeneration of enlarged motor units caused by increased metabolic demands and the normal aging process, in addition to inflammatory mechanisms, are thought to be involved. There is no diagnostic test for PPS, and the diagnosis is based on a proper clinical workup where all other possible explanations for the new symptoms are ruled out. The basic principle of management of PPS lies in physical activity, individually tailored training programs, and lifestyle modification. Muscle weakness and muscle pain may be helped with specific training programs, in which training in warm water seems to be particularly helpful. Properly fitted orthoses can improve the biomechanical movement pattern and be energy-saving. Fatigue can be relieved with lifestyle changes, assistive devices, and training programs. Respiratory insufficiency can be controlled with noninvasive respiratory aids including biphasic positive pressure ventilators. Pharmacologic agents like prednisone, amantadine, pyridostigmine, and coenzyme Q10 are of no benefit in PPS. Intravenous immunoglobulin (IVIG) has been tried in three studies, all having positive results. IVIG could probably be a therapeutic alternative, but the potential benefit is modest, and some important questions are still unanswered, in particular to which patients this treatment is useful, the dose, and the therapeutic interval.

Conclusions:

Outcome of Research: More research required.

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There are currently 61 papers in this category.

Category: Diagnosis and Management, Differential Diagnosis

Title: Plasma CHI3L1 in Amyotrophic Lateral Sclerosis: A Potential Differential Diagnostic Biomarker
Author: Alessandro Bombaci*, Umberto Manera, Giovanni De Marco, Federico Casale, Paolina Salamone, Giuseppe Fuda, Giulia Marchese, Barbara Iazzolino, Laura Peotta, Cristina Moglia, Andrea Calvo and Adriano Chiò
Affiliation: “Rita Levi Montalcini” Department of Neuroscience, University of Turin, 10126 Turin, Italy
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: J. Clin. Med. 2023, 12(6), 2367; https://doi.org/10.3390/jcm12062367
Publication Year and Month: 2023 03

Abstract: Background: Motor neuron diseases (MNDs) are fatal neurodegenerative diseases. Biomarkers could help with defining patients’ prognoses and stratifications. Besides neurofilaments, chitinases are a promising family of possible biomarkers which correlate with neuroinflammatory status. We evaluated the plasmatic levels of CHI3L1 in MNDs, MND mimics, and healthy controls (HCs).

Methods: We used a sandwich ELISA to quantify the CHI3L1 in plasma samples from 44 MND patients, 7 hereditary spastic paraplegia (HSP) patients, 9 MND mimics, and 19 HCs. We also collected a ALSFRSr scale, MRC scale, spirometry, mutational status, progression rate (PR), blood sampling, and neuropsychological evaluation.

Results: The plasma levels of the CHI3L1 were different among groups (p = 0.005). Particularly, the MND mimics showed higher CHI3L1 levels compared with the MND patients and HCs. The CHI3L1 levels did not differ among PMA, PLS, and ALS, and we did not find a correlation among the CHI3L1 levels and clinical scores, spirometry parameters, PR, and neuropsychological features. Of note, the red blood cell count and haemoglobin was correlated with the CHI3L1 levels (respectively, p < 0.001, r = 0.63; p = 0.022, and r = 0.52).

Conclusions: The CHI3L1 plasma levels were increased in the MND mimics cohort compared with MNDs group. The increase of CHI3L1 in neuroinflammatory processes could explain our findings. We confirmed that the CHI3L1 plasma levels did not allow for differentiation between ALS and HCs, nor were they correlated with neuropsychological impairment.

Keywords: biomarker; chitinases; cognitive impairment; differential diagnosis; early diagnosis; MND mimics; red blood cells

Conclusions: The measurement of the plasmatic levels of CHI3L1 could be useful in the differential diagnosis between MNDs and MND mimics. This is an important issue, since the early diagnosis of an MND is a determinant in the early starting of neuroprotective therapy and in clinical trial recruitment.
Further multicentre and longitudinal studies on a larger patient cohort, testing alongside other fluid biomarkers, are needed to better explain the role of CHI3L1 in the diagnosis and prognosis of MNDs and, also, of MND mimics.

Outcome of Research: More research required

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any): This article is helpful for any medical/neurologist trying to determine differential diagnosis of ALS vs post-polio.

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Category: Diagnosis and Management

Title: Estimation of the Direct Cost of Poliomyelitis Rehabilitation Treatment to Pakistani Patients: A 53-Year Retrospective Study
Author: Atta Abbas Naqvi, Syed Baqir Shyum Naqvi, Fatima Zehra, Ashutosh Kumar Verma, Saman Usmani, Sehrish Badar, Rizwan Ahmad, Niyaz Ahmad
Affiliation: 1.Department of Pharmacy Practice, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
2.Faculty of PharmacyHamdard UniversityKarachiPakistan
3.Applied Economics Research CentreUniversity of KarachiKarachiPakistan
4.Discipline of Social and Administrative Pharmacy, School of Pharmaceutical SciencesUniversiti Sains MalaysiaMindenMalaysia
5.Institute of Pharmaceutical Sciences, Jinnah Sindh Medical UniversityKarachiPakistan
6.Natural Products and Alternative Medicines, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
7.Department of Pharmaceutics, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
Journal: Applied Health Economics and Health Policy
Citation: December 2018, Volume 16, Issue 6, pp 871–888
Publication Year and Month: 2018 12

Abstract: Background
Pakistan is one of the last few countries in which poliomyelitis is endemic. Evidence indicates that out-of-pocket expenditures are a barrier to polio rehabilitation treatment, yet there are no reported figures related to the financial burden of this disease on patients in a recently polio-endemic country.

Objective
This study investigated direct costs attributed to rehabilitation treatment of poliomyelitis among Pakistani patients and reported its duration along with the socioeconomic status of poliomyelitis survivors.

Conclusions: Conclusion
The cost of poliomyelitis rehabilitation in Pakistan is high; it has an economic effect on the lives of patients and their families. Despite good education, polio survivors in Pakistan appear to have low socioeconomic status, lower chances of employment and marriage, as well as fewer children. Further research is recommended to explore the burden of disease on society, i.e., indirect costs and suffering.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Polio survivors perceptions of a multi-disciplinary rehabilitation programme
Author: Atwal, A., Duncan, H., Queally, C., Cedar, S.H.
Affiliation: Nil
Journal: Disability and Rehabilitation
Citation: Atwal, A., Duncan, H., Queally, C., Cedar, S.H. (2017) Polio survivors perceptions of a multi-disciplinary rehabilitation programme. Disability and Rehabilitation. DOI: 10.1080/09638288.2017.1381184
Publication Year and Month: 2017 10

Abstract: Purpose: Post-polio syndrome refers to a late complication of the poliovirus infection. Management of post-polio syndrome is complex due to the extensive symptomology. European and United Kingdom guidelines have advised the use of rehabilitation programmes to manage post-polio syndrome. There is a paucity of research in relation to the effectiveness of rehabilitation interventions. The objective of this study is to explore polio survivor’s perceptions of an in-patient multi-disciplinary rehabilitation programme.

Methods: Semi-structured interviews of community dwelling polio survivors who attended in-patient rehabilitation programme in the United Kingdom. Thematic analysis was used to describe and interpret interview data.

Results: Participants’ experiences were influenced by past experiences of polio and their self-concept. Participants generally had a positive experience and valued being with other polio survivors. Positive strategies, such as pacing and reflection changed their mind-sets into their lives after the programme, though they still faced challenges in daily living. Some participants supported others with post-polio syndrome after completing the programme.

Conclusions: The research identified that participants experienced long term positive benefits from attending a rehabilitation programme. Strategies that users found helpful that explored the effectiveness of interventions to manage polio are not cited within a Cochrane review. If we are to recognise the lived experience and service user empowerment within a model of co- production it is essential that patient preferences are evaluated and used as evidence to justify service provision. Further research is required with polio survivors to explore how best rehabilitation programmes can adopt the principles of co-production.

Implications for Rehabilitation
The patients’ expertise and lived experience must be at the centre of a rehabilitation programme.

Strategies such as pacing and reflection are perceived as important strategies to enable self-management of polio and post-polio syndrome despite the limited evidence base to support these interventions.

Polio rehabilitation programmes should not be time limited and commissioners and therapists need to ensure that follow up support is provided.

When measuring outcomes patient preferences and views must be evaluated.

Outcome of Research: More research required

Availability of Paper: Paid subscription required to view or download full text.

Comments (if any): This investigation supports feedback from polio survivors in Australia on the benefits of group-based rehabilitation, especially where there is an opportunity to learn and discuss their rehabilitation with fellow participants.

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Category: Diagnosis and Management

Title: Post-poliomyelitis syndrome as a possible viral disease
Author: Baj A (1), Colombo M (1), Headley JL (2), McFarlane JR (3), Liethof MA (4), Toniolo A (5)
Affiliation: (1) Laboratory of Clinical Microbiology, University of Insubria Medical School, Viale Borri 57, 21100 Varese, Italy; (2) Post-Polio Health International, Saint Louis, Missouri, USA; (3) European Polio Union, Huldenberg, Belgium; (4) Polio Australia Incorporated, Kew, Victoria, Australia; (5) Laboratory of Clinical Microbiology, University of Insubria Medical School, Viale Borri 57, 21100 Varese, Italy. Electronic address: [email protected]
Journal: International Journal of Infectious Diseases
Citation: Int J Infect Dis. 2015 May 1;35:107-116. doi: 10.1016/j.ijid.2015.04.018
Publication Year and Month: 2015 05

Abstract: This review summarizes current concepts on post-polio syndrome (PPS), a condition that may arise in polio survivors after partial or complete functional recovery followed by a prolonged interval of stable neurological function. PPS affects 15-20 million people worldwide. Epidemiological data are reported, together with the pathogenic pathways that possibly lead to the progressive degeneration and loss of neuromuscular motor units. As a consequence of PPS, polio survivors experience new weakness, generalized fatigue, atrophy of previously unaffected muscles, and a physical decline that may culminate in the loss of independent life. Emphasis is given to the possible pathogenic role of persistent poliovirus infection and chronic inflammation. These factors could contribute to the neurological and physical decline in polio survivors. A perspective is then given on novel anti-poliovirus compounds and monoclonal antibodies that have been developed to contribute to the final phases of polio eradication. These agents could also be useful for the treatment or prevention of PPS. Some of these compounds/antibodies are in early clinical development. Finally, current clinical trials for PPS are reported. In this area, the intravenous infusion of normal human immunoglobulins appears both feasible and promising.

Conclusions:

Outcome of Research: Effective.

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Category: Diagnosis and Management, Orthoses, Surgery

Title: Polio revisited: reviving knowledge and skills to meet the challenge of resurgence
Author: Benjamin Joseph (1) and Hugh Watts (2)
Affiliation: (1) Aster Medcity, Kochi, Kerala India, 18 HIG HUDCO Colony, Manipal, Karnataka 576104 India

(2) Shriners Hospital for Children, Los Angeles, CA USA
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Journal of Children's Orthopaedics

9 (5): 325–338, doi: 10.1007/s11832-015-0678-4
Publication Year and Month: 2015 09

Abstract: Purpose
To date, polio has not been eradicated and there appears to be a resurgence of the disease. Hence, there is a need to revive decision-making skills to treat the effects of polio.

Methods
Here, we outline the aspects of treatment of paralysis following polio based on the literature and personal experience of the authors. The surgical treatment of the lower and upper extremities and the spine have been reviewed. The scope of bracing of the lower limb has been defined.

Results
The effects of polio can be mitigated by judicious correction of deformities, restoration of muscle balance, stabilising unstable joints and compensating for limb length inequality.

Conclusions
As polio has not been eradicated and there is a risk of resurgence of the disease, paediatric orthopaedic surgeons need to be prepared to deal with fresh cases of polio. Revival of old techniques for managing the effects of paralysis following polio is needed.

Keywords: Poliomyelitis, Resurgence, Surgical decision-making, Bracing, Paralytic deformity

Conclusions: Polio has not been eradicated and there is a risk of resurgence of the disease. Paediatric orthopaedic surgeons need to be prepared to deal with fresh cases of polio. Revival of old techniques of managing the effects of paralysis following polio is needed.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Outcome of physiotherapy as part of a multidisciplinary rehabilitation in an unselected polio population with one-year follow-up: an uncontrolled study.
Author: Bertelsen M, Broberg S, Madsen E.
Affiliation: Rehabilitation Centre of the Danish Society of Polio and Accident Victims (PTU), Rødovre, Denmark. [email protected]
Journal: Journal of Rehabilitation Medicine
Citation: 2009 Jan;41(1):85-7.
Publication Year and Month: 2009 01

Abstract: OBJECTIVE:
The aim of this study was to evaluate the outcome of physiotherapy as part of a multidisciplinary rehabilitation.

DESIGN:
Prospective uncontrolled intervention study.

SUBJECTS:
Fifty patients with late effects of polio, first time referred to physiotherapy at the Danish Society of Polio and Accident Victims (PTU) Rehabilitation Centre.

METHODS:
The intervention was physiotherapy as an essential part of an individually planned multidisciplinary rehabilitation. The outcome measures Six-Minute Walk Test and Timed-Stands Test were used to assess the functional capacity. Quality of life was evaluated by Medical Outcome Survey Short Form (SF-36) and fatigue by Multidimensional Fatigue Inventory (MFI-20). Patients were tested at baseline; 3 months after the start of rehabilitation and at one-year follow-up.

RESULTS:
The patients showed significantly better functional capacity on all measurements 3 months after start of intervention and at one-year follow-up. The patients showed significant improvement in 3 of the SF-36 dimensions regarding quality of life, but only the improvement in "general health" remained after one year.

Conclusions: This study shows that patients with late effects of polio, who experience new problems related to polio, can benefit from an individually planned multidisciplinary intervention with emphasis on physiotherapy, and the improvement in physical capacity and general health can remain at one-year follow-up.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Risk factors for post-polio syndrome among an Italian population: a case-control study.
Author: Bertolasi L, Acler M, dall'Ora E, Gajofatto A, Frasson E, Tocco P, Turri M, Ferlisi M, Fiorini M, Pimazzoni F, Squintani G, Martini M, Danzi B, Monaco S.
Affiliation: Section of Neurology, Department of Neurological, Neuropsychological, Morphological and Motor Sciences, University of Verona, Piazzale L.A. Scuro 10, 37134, Verona, Italy. [email protected]
Journal: Neurological sciences: official journal of the Italian neurological society
Citation: 2012 Dec;33(6):1271-5. doi: 10.1007/s10072-012-0931-2. Epub 2012 Jan 14.
Publication Year and Month: 2012 01

Abstract: Post-polio syndrome (PPS) is a clinical syndrome of new weakness, fatigue and musculoskeletal pain occurring in a variable proportion of polio survivors decades after acute disease. To date, several risk factors for PPS development have been reported, although the etiology of this disorder remains elusive. Using a case-control design, we aimed to assess risk indicators for PPS in a group of Italian polio survivors. Subjects with prior poliomyelitis attending the rehabilitation hospital of Malcesine, Italy, were the target population. Patients with PPS, diagnosed according to the European Federation of Neurological Societies criteria, served as cases, while patients not meeting diagnostic criteria for PPS were used as controls. All subjects were assessed through a structured questionnaire made of 82 questions and neurological examination. The association with investigated risk factors (sex, age at polio onset, age at onset of symptoms, extension and severity of polio, employment) was analyzed by the calculation of the odds ratio. A total of 161 out of 391 eligible patients met the adopted diagnostic criteria for PPS, giving a frequency of 41.2%. Symptoms most frequently complained by PPS patients were loss of muscle strength, loss of resistance, loss of muscle volume and generalized fatigue. Female gender, the presence of respiratory disturbance during the acute phase of polio and the use of orthoses and aids during the recovery and stabilization represented independent risk factors for PPS in the studied population.

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Histopathologic Basis of Clinical Findings in Poliomyelitis
Author: Bodian, D.
Affiliation: Poliomyelitis Research Center, Department of Epidemiology, Johns Hopkins University, Baltimore
Journal: The American Journal of Medicine
Citation: 1949: 6(5):563–578
Publication Year and Month: 1949 05

Abstract: 1. Experimental evidence indicates that the onset of CNS pathologic changes occurs in the preparalytic period and is closely associated with the earliest evidence of virus activity in any particular region involved.
2. The earliest cytopathologic changes are diffuse chromatolysis of Nissl substance in the cytoplasm of nerve cells and mild cellular exudate consisting of polymorphonuclear and mononuclear leukocytes.
3. Nerve cell changes may be present in the earliest stages without inflammatory reaction in the vicinity and therefore are not necessarily the result of the latter, but rather the result of direct virus action.
4. Nerve cell changes either lead to rapid destruction of the cell or to arrest in the stage of cytoplasmic chromatolysis, following which complete morphologic recovery of the cell generally occurs over a period of about a month or less, depending upon the severity of injury.
5. Virus activity, nerve cell changes and inflammatory reaction are localized only in certain susceptible regions of the CNS, largely due to specific differences of susceptibility of nerve cells. The intensity of the inflammatory reaction, however, may be quite variable in different susceptible centers and in different individuals. Severe inflammatory reaction is usually but not always associated with extensive nerve cell destruction. Severe nerve cell damage may occur without extensive cellular infiltration in the cord.
6. Lesions in the cerebral cortex are usually confined to the motor area of the precentral gyrus and even here the lesions are rarely severe enough to suggest that they may produce clinical symptoms.
7. “Encephalitic” symptoms such as restlessness, stupor, disorientation and coma are associated with severe inflammatory reaction in the brainstem and often with small softenings in this region. They are not associated with unusual involvement of the cerebral cortex.
8. Brainstem centers principally involved in most instances are the reticular formation of the hind-brain, the vestibular nuclei and the roof nuclei of the cerebellum. Resulting functional disturbances are discussed.
9. Widespread dissemination of virus among most motor nerve cells in spinal cord enlargements occurs in experimental poliomyelitis as early as the first day of paralysis. Motor nerve cells which are affected either are destroyed very quickly during the first few days of the disease or undergo slower recovery changes leading to complete morphologic recovery within about a month. After this time it can be shown that the degree of paralysis and atrophy are closely correlated with the number of motor nerve cells destroyed. In the acute stage, however, this correlation is not as high and other factors must also play a role in producing paralysis. An important factor is the reversible injury of motor nerve cells. Less complete evidence from human material suggests that a similar situation obtains in human poliomyelitis.
10. Experimental work suggests three possible factors which may determine the variation in severity of infection. These are, first, variations due to difference in strains of the virus, second, reduction of severity due to previous paralytic or non-paralytic infection, and third, host variation unrelated to previous immunizing experience with the virus.

Conclusions: Poliomyelitis virus has strains that can vary depending on the host, and response to the virus. The virus causes most damage to the anterior horn cells of the cervical and lumbar segments, however there is damage to the brainstem and premotor areas in the brain.

Outcome of Research: More research required

Availability of Paper: Paid subscription required to view or download full text.

Comments (if any): David Bodian produced papers that helped enable (effective) availability for vaccinations. His work is still cited and referred to in understanding the effects of the polio virus, especially in relation to the central nervous system pathology.

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Category: Diagnosis and Management

Title: Paralytic vs "nonparalytic" polio: distinction without a difference?
Author: Bruno RL
Affiliation: The Post-Polio Institute, Englewood Hospital and Medical Center, New Jersey, USA
Journal: American Journal of Physical Medicine & Rehabilitation
Citation: Am J Phys Med Rehabil. 2000 Jan-Feb;79(1):4-12
Publication Year and Month: 2000 01

Abstract: Nonparalytic polio (NPP) is commonly thought to be synonymous with "abortive polio," in which the poliovirus neither entered the central nervous system nor damaged neurons. Described are two epidemic illness-"The Summer Grippe" and Iceland disease-apparently caused by a low virulence but neuropathic type 2 poliovirus. Studies show that neuronal lesions in the brain and spinal cord and muscle weakness were common in NPP, and epidemiologic studies document late-onset weakness and fatigue in 14% to 42% of NPP survivors. These findings indicate that clinicians should not require a history of paralytic polio, electromyographic evidence of denervation, and new muscle weakness for the diagnosis of "Postpolio Syndrome" but should be aware that NPP, and possibly even poliovirus-induced "minor illnesses," can be associated with acute central nervous system damage and late-onset muscle weakness and fatigue.

Conclusions:

Outcome of Research: Not applicable

Availability of Paper: Paid subscription required to view or download full text.

Comments (if any): The full paper is available from Polio Australia for private study purposes.

Link to Paper (if available): Click here to view Abstract


Category: Diagnosis and Management

Title: Polioencephalitis, stress, and the etiology of post-polio sequelae
Author: Bruno RL, Frick NM, Cohen J
Affiliation: Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation, East Orange, NJ 07018
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1269-76
Publication Year and Month: 1991 11

Abstract: Post-mortem neurohistopathologies that document polio virus-induced lesions in reticular formation and hypothalamic, thalamic, peptidergic, and monoaminergic neurons in the brain are reviewed from 158 individuals who contracted polio before 1950. This polioencephalitis was found to occur in every case of poliomyelitis, even those without evidence of damage to spinal motor neurons. These findings, in combination with data from the 1990 National Post-Polio Survey and new magnetic resonance imaging studies documenting post-encephalitis-like lesions in the brains of polio survivors, are used to present two hypotheses: 1) polioencephalitic damage to aging reticular activating system and monoaminergic neurons is responsible for post-polio fatigue, and 2) polioencephalitic damage to enkephalin-producing neurons is responsible for hypersensitivity to pain in polio survivors. In addition, the antimetabolic action of glucocorticoids on polio-damaged, metabolically vulnerable neurons may be responsible for the fatigue and muscle weakness reported by polio survivors during emotional stress.

Conclusions: The ability of the polio virus to produce symptoms by its destruction of neurons outside of the anterior horn has been accepted for more than 100 years. It is only our recent experience with PPS that has forced us to recognize that both the people who survived the original viral onslaught and their central nervous systems have been operating for decades under extreme stress. This stress now may be combining with the aging of an extensively damaged but here-to-fore remarkably functional central nervous system to reveal the previously hidden symptoms of polioencephalitis.

The above-presented hypotheses concerning the etiology of PPS fatigue suggest that reductions in both emotional and physical stress will reduce PPS. This is the experience of post-polio clinics throughout the world (36,54,55,56). As PPS fatigue continues to be treated with stress-management, work simplification and energy conservation (see 36,56), the hypotheses are being tested by studying the neuroanatomy, neuroendocrinology and neuropsychology of PPS fatigue. In addition, pharmacological means for stimulating the RAS that do not further metabolically stress its remaining aging, polio-damaged neurons are being tested.

Outcome of Research:

Availability of Paper: The full text of this paper has been generously made available by Dr Bruno.

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Category: Diagnosis and Management, Late Effects of Polio

Title: Knowledge of healthcare professionals about poliomyelitis and postpoliomyelitis: a cross-sectional study
Author: Claudio Andre Barbosa de Lira (I), Douglas Assis Teles Santos (II), Ricardo Borges Viana (III), Juliana Moreira Guimarães (IV), Jéssica Nathalia Soares Oliveira (V), Bolivar Saldanha Sousa (VI), Marcos Gonçalves de Santana (VII), Rodrigo Luiz Vancini (VIII),
Marília Santos Andrade (IX), Pantelis Nikolaidis (X), Thomas Rosemann (XI), Beat Knechtle (XII)

Affiliation: (I) BSc, PhD. Associate Professor, Faculdade de Educação Física e Dança (FEFD), Universidade Federal de Goiás (UFG), Goiânia (GO), Brazil. https://orcid.org/0000-0001-5749-6877

(II) BSc, MSc. Assistent Professor, Colegiado de Educação Física, Universidade do Estado da Bahia (UNEB), Teixeira de Freitas (BA), Brazil. https://orcid.org/0000-0002-7664-5468

(III) BSc, PhD. Professor, Escola Superior de Educação Física e Fisioterapia do Estado de Goiás (ESEFFEGO), Universidade Estadual de Goiás (UEG), Goiânia (GO), Brazil. https://orcid.org/0000-0001-9200-3185

(IV) BSc. Nurse, Unidade Acadêmica Especial de Ciências da Saúde, Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0002-6729-0335

(V) BSc. Biomedic, Unidade Acadêmica Especial de Ciências da Saúde (UA-CISAU),
Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0002-9531-4277

(VI) MD, PhD. Physician, Hospital Israelita Albert Einstein, São Paulo-Brazil, São Paulo (SP), Brazil. https://orcid.org/0000-0001-9682-5987

(VII) BSc, PhD. Associate Professor, Unidade Acadêmica Especial de Ciências da Saúde (UA-CISAU), Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0001-7674-3263

(VIII) BSc, PhD. Adjunct Professor, Centro de Educação Física e Desportos, Universidade Federal do Espírito Santo (UFES), Vitória (ES), Brazil. https://orcid.org/0000-0003-1981-1092

(IX) PhD. Physical Therapist and Associate Professor, Department of Physiology, Universidade Federal de São Paulo (USP), São Paulo (SP), Brazil. https://orcid.org/0000-0002-7004-4565

(X) BSc, PhD. Professor, School of Health and Caring Sciences, University of West Attica, Athens, Greece. https://orcid.org/0000-0001-8030-7122

(XI) MD, PhD. Physician and Professor, Institute of Primary Care, University of Zurich, Zurich, Switzerland. https://orcid.org/0000-0002-6436-6306

(XII) MD, PhD. Physician and Scientific Assistant, Institute of Primary Care, University of Zurich, Zurich, Switzerland; and Researcher, Medbase St. Gallen Am Vadianplatz, St. Gallen, Switzerland https://orcid.org/0000-0002-2412-9103
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Sao Paulo Med. J.

139 (5) • Aug-Sep 2021
https://doi.org/10.1590/1516-3180.2020.0617.16032021
Publication Year and Month: 2021 08

Abstract: BACKGROUND:
Postpoliomyelitis syndrome is a clinical condition that can affect poliomyelitis survivors.

OBJECTIVE:
Our aim was to evaluate knowledge of poliomyelitis and postpoliomyelitis syndrome among Brazilian healthcare professionals.

DESIGN AND SETTING:
Cross-sectional study conducted at a Brazilian public higher education institution located in the state of Goiás.

METHODS:
The participants (n = 578) were Brazilian physicians, physical therapists, nurses, nutritionists and psychologists. A self-administered questionnaire (30 questions) was designed to probe knowledge about poliomyelitis and postpoliomyelitis syndrome. From the questionnaire, we created a structured test to objectively evaluate the knowledge of these professionals. The test was composed of 20 questions and was scored over a range from 0 (totally ill-informed) to 20 (totally well-informed).

RESULTS:
In general, the physicians, physical therapists and nurses demonstrated better understanding of poliomyelitis and postpoliomyelitis syndrome. The healthcare professionals who had received previous information about poliomyelitis and postpoliomyelitis syndrome had significantly higher scores than those who had never received information (P < 0.001). On average, this difference was approximately 28.6%.

CONCLUSIONS:
The findings from the present study indicate that there is a critical need for improvement of knowledge about postpoliomyelitis syndrome among Brazilian healthcare professionals. The services provided by these professionals may therefore become compromised. Furthermore, public healthcare initiatives should be implemented to improve knowledge among healthcare professionals.

KEYWORDS (MeSH terms):
Poliomyelitis; Physicians; Nervous system; Postpoliomyelitis syndrome; Knowledge

AUTHORS’ KEY WORDS:
Medical education; Neuromuscular disease; Late effects of polio

Conclusions: Our study showed that, overall, there is a lack of knowledge about PPS and poliomyelitis, especially among psychologists and nutritionists. Therefore, the services provided by these professionals may become compromised. Furthermore, government initiatives should be implemented to increase knowledge among healthcare professionals.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: The post-polio syndrome as an evolved clinical entity. Definition and clinical description.
Author: Dalakas MC
Affiliation: Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
Journal: Annals of the New York Academy of Sciences
Citation: Ann N Y Acad Sci. 1995 May 25;753:68-80
Publication Year and Month: 1995 05

Abstract: Post-polio syndrome (PPS) refers to the new neuromuscular symptoms that occur at least 15 years after stability in patients with prior acute paralytic polio-myelitis. They include: (1) new muscle weakness and atrophy in the limbs, the bulbar or the respiratory muscles [post-poliomyelitis muscular atrophy (PPMA)] and (2) excessive muscle fatigue and diminished physical endurance. PPS is a clinical diagnosis that requires exclusion of all other medical, neurological, orthopedic or psychiatric diseases that could explain the cause of the new symptoms. Routine electromyography is useful to confirm chronic and ongoing denervation and exclude neuropathies. Muscle biopsy, single fiber electromyography (EMG), macro-EMG, serum antibody titers to polio virus, and spinal fluid studies are very useful research tools but they are rarely needed to establish the clinical diagnosis. PPS is a slowly progressive phenomenon with periods of stability that vary from 3 to 10 years. Current evidence indicates that PPS is the evolution of a subclinically ongoing motor neuron dysfunction that begins after the time of the acute polio. It is clinically manifested as PPS when the well-compensated reinnervating process crosses a critical threshold beyond which the remaining motor neurons cannot maintain the innervation to all the muscle fibers within their motor unit territory.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Pathogenetic mechanisms of post-polio syndrome: morphological, electrophysiological, virological, and immunological correlations.
Author: Dalakas MC
Affiliation: Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1382, USA.
Journal: Annals of the New York Academy of Sciences
Citation: 1995 May 25;753:167-85.
Publication Year and Month: 1995 05

Abstract: To understand the mechanism of post-poliomyelitis muscular atrophy (PPMA) and the post-polio syndrome (PPS) in general, we performed the following studies: (1) histopathology in spinal cord sections from patients who died 9 days to 44 years after acute paralytic poliomyelitis; (2) enzyme histochemistry, immunocytochemistry (for lymphocyte subsets, MHC antigens and N-CAM) and polymerase chain reaction (PCR) for poliovirus RNA in the muscle biopsies from symptomatic or asymptomatic muscles of post-polio patients; (3) determination of lymphocyte subsets and circulating IgG or IgM antibodies against GM1 and poliovirus; (4) virological studies in the spinal fluid for oligoclonal bands and search for poliovirus genome with PCR; (5) electrophysiological studies including single fiber EMG, fiber density and macro-EMG; and (6) [31P] exercise MRS spectroscopy on previously affected muscles to search for a metabolic correlate of fatigue. These studies concluded that in PPS a continuing dysfunction is present in the spinal cord motor neurons, resulting in ongoing muscle denervation and reinnervation first evident at the axonal branch points. Symptoms are related to attrition of the oversprouting motor neurons which after a period of time cannot support all their axonal sprouts, resulting in failure of re-reinnervation. In some patients with PPS there is also an ongoing immune activation and presence of defective viral particles in the spinal fluid. However, their role in the pathogenesis of PPS is presently unknown.

Conclusions: These studies concluded that in PPS a continuing dysfunction is present in the spinal cord motor neurons, resulting in ongoing muscle denervation and reinnervation first evident at the axonal branch points.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Predictive Factors for Post-Poliomyelitis Syndrome
Author: Daria A. Trojan, MD, MSc, Neil R. Cashman, MD, Stanley Shapiro, PhD, Catherine M. Tansey, MSc, John M. Esdaile, MD
Affiliation: From the Department of Neurology (Drs. Trojan, Cashman), Montreal Neurological Institute and Hospital, the Department of Medicine (Dr. Esdaile), Montreal General Hospital, and the Department of Epidemiology and Biostatistics (Dr. Shapiro, Ms. Tansey), McGill University, Montreal, Quebec, Canada.
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil Vol 75, July 1994, 770-777
Publication Year and Month: 1994 07

Abstract: Post-poliomyelitis syndrome (PPS) is generally defined as a clinical syndrome of new weakness, fatigue, and pain in individuals who have previously recovered from acute paralytic poliomyelitis. The purpose of this study was to identify, through a case-control study design, factors that predict subsequent PPS in patients with prior paralytic poliomyelitis. Among patients attending a university-affiliate hospital post-polio clinic, "cases" were patients with new weakness and fatigue, and "controls" were patients without these complaints. A chart review of 353 patients identified 127 cases and 39 controls. Logistic regression modeling was used to calculate adjusted and unadjusted odds ratios. In univariate analyses, significant risk factors for PPS were a greater age at time of presentation to clinic (p = 0.01), a longer time since acute polio (p = 0.01), and more weakness at acute polio (p = 0.02). Other significant associated, but not necessarily causal factors were a recent weight gain (p = 0.005), muscle pain (p = 0.01) particularly that associated with exercise (p = 0.005), and joint pain (p = 0.04). Multivariate analyses revealed that a model containing age at presentation to clinic, severity of weakness at acute polio, muscle pain with exercise, recent weight gain, and joint pain best distinguished cases from controls. Age at acute polio, degree of recovery after polio, weakness at best point after polio, physical activity, and sex were not contributing factors. These findings suggest that the degree of initial motor unit involvement as measured by weakness at acute polio, and possibly the aging process and overuse are important in predicting PPS.

Conclusions: In conclusion, the results from this study provide insight on predictive factors for PPS, and can be applied in the clinical management of patients who have recovered from paralytic poliomyelitis. Our findings support the hypothesis that the severity of initial motor unit involvement as estimated by weakness at acute polio, and possibly the normal ageing process and overuse are important in predicting PPS. Even though patients have no control over the severity of weakness as a result of acute polio, they do have control over some predictive factors for PPS. Patients can be advised that they should avoid gaining weight and exercising to the point of muscle pain because these variables have been found to be strongly associated with PPS. The exact role of physical activity will still need further evaluation; however, the usual recommendations of low-level aerobic exercise with avoidance of muscle pain and fatigue appear valid. Thus, this study can provide the basis for physiologically reasonable and practical advice to post-polio patients to minimize or delay the risk of PPS.

Outcome of Research: Not applicable

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Comments (if any): Paul Cavendish (Clinical Health Educator): The best multivariate model for predicting who will develop PPS indicates that patients who had a greater weakness at acute polio, are currently older, have muscle pain with exercise, a recent weight gain, and joint pain are those most likely to develop PPS. Other factors shown to be important in univariate analyses are a longer time since acute polio, and muscle pain (at rest or with exercise). Age at acute polio, recovery after polio, weakness at "best point" after polio, physical activity, and sex were not contributing factors.

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Category: Diagnosis and Management

Title: Physical therapy management of the patient with post-polio syndrome. A case report.
Author: Donna J. Twist, Dong M. Ma
Affiliation:
Journal: Physical Therapy
Citation: Volume 66, Issue 9, 1 September 1986, Pages 1403–1406
Publication Year and Month: 1986 09

Abstract: This case report documents the treatment of a patient who experienced progressive muscle weakness and a decrease in function over time that did not appear to be related to any secondary neuromuscular disease. We discuss the relationship between age and maximal muscle function in addition to some general guidelines for rehabilitation. This type of patient can represent a challenge for the physical therapist. This case report, however, illustrates the degree of muscular and functional recovery that can result with a physical therapy program aimed at reducing levels and intensity of exercise, daily activity, and stress.

Conclusions: A combination of short-term goals appears to be essential to the successful management of a patient with post-polio syndrome.

Outcome of Research: Not applicable

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Category: Diagnosis and Management, Late Effects of Polio

Title: Post-poliomyelitis syndrome (2019)
Author: Eric Chun Pu Chu1 and Kary Ka Wai Lam2
Affiliation: 1New York Chiropractic and Physiotherapy Center, New York Medical Group, Hong Kong, People’s Republic of China
2Downtown Chiropractic Limited, Hong Kong, People’s Republic of China
Correspondence: Eric Chun Pu ChuNew York Chiropractic and Physiotherapy Centre, New York Medical Group, 41/F Langham Place Office Tower, 8 Argyle Street, Mongkok, Hong Kong, People’s Republic of China, Phone: Tel +852 3 594 7844, Fax: Fax +852 3 594 6193, Email [email protected]
Journal: International Medical Case Reports Journal
Citation: 12, 261–264. https://doi.org/10.2147/IMCRJ.S219481

Publication Year and Month: 2019 08

Abstract: Most developed countries eliminated paralytic poliomyelitis (polio) in the 1970s to 1980s. It was believed that after recovery from acute paralytic poliomyelitis, the physical condition of survivors would remain stable for the rest of their lives. However, the elimination of polio does not equate the end of medical management of polio. Hundreds of thousands of polio survivors worldwide are still at risk of developing the late effects of the disease. Here, we report a case of post-polio syndrome who attended our clinic for the presence of new weakness and neuromuscular problems six decades after recovery from paralytic polio. It is essential that health professionals be aware of these conditions and have an understanding of the underlying pathophysiology of the symptoms.

Conclusions: Post-polio syndrome is related to the exhaustion of the motor units that form decades after the polio attack. This case report describes the effectiveness of manual interventions in assisting our patient in restoring the level of function and alleviating pain. The limitation of the current report is that it is just a single case. Further comparison with more existing therapeutic regimens is warranted to clarify these issues.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Update on current and emerging treatment options for post-polio syndrome
Author: Farbu E
Affiliation: Neurocenter and National Competence Center for Movement Disorders, Stavanger University Hospital, Stavanger, Norway
Journal: Therapeutics and Clinical Risk Management
Citation: Ther Clin Risk Manag. 2010 Jul 21;6:307-13
Publication Year and Month: 2010 07

Abstract: Post-polio syndrome (PPS) refers to the clinical deterioration experienced by many polio survivors several decades after their acute illness. The symptoms are new muscle weakness, decreased muscle endurance, fatigue, muscle pain, joint pain, cold intolerance, and this typical clinical entity is reported from different parts of the world. The pathophysiology behind PPS is not fully understood, but a combination of distal degeneration of enlarged motor units caused by increased metabolic demands and the normal aging process, in addition to inflammatory mechanisms, are thought to be involved. There is no diagnostic test for PPS, and the diagnosis is based on a proper clinical workup where all other possible explanations for the new symptoms are ruled out. The basic principle of management of PPS lies in physical activity, individually tailored training programs, and lifestyle modification. Muscle weakness and muscle pain may be helped with specific training programs, in which training in warm water seems to be particularly helpful. Properly fitted orthoses can improve the biomechanical movement pattern and be energy-saving. Fatigue can be relieved with lifestyle changes, assistive devices, and training programs. Respiratory insufficiency can be controlled with noninvasive respiratory aids including biphasic positive pressure ventilators. Pharmacologic agents like prednisone, amantadine, pyridostigmine, and coenzyme Q10 are of no benefit in PPS. Intravenous immunoglobulin (IVIG) has been tried in three studies, all having positive results. IVIG could probably be a therapeutic alternative, but the potential benefit is modest, and some important questions are still unanswered, in particular to which patients this treatment is useful, the dose, and the therapeutic interval.

Conclusions:

Outcome of Research: More research required.

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Category: Diagnosis and Management

Title: EFNS guideline on diagnosis and management of post-polio syndrome - report of an EFNS task force
Author: Farbu E, Gilhus NE, Barnes MP, Borg K, de Visser M, Driessen A, Howard R, Nollet F, Opara J, Stalberg E
Affiliation: Department of Neurology, Haukeland University Hospital, University of Bergen, Bergen, Norway – [email protected] – European Federation of Neurological Society
Journal: European Journal of Neurology
Citation: Eur J Neurol. 2006 Aug; 13(8):795-801
Publication Year and Month: 2006 08

Abstract: Post-polio syndrome (PPS) is characterized by new or increased muscular weakness, atrophy, muscle pain and fatigue several years after acute polio. The aim of the article is to prepare diagnostic criteria for PPS, and to evaluate the existing evidence for therapeutic interventions. The Medline, EMBASE and ISI databases were searched. Consensus in the group was reached after discussion by e-mail. We recommend Halstead's definition of PPS from 1991 as diagnostic criteria. Supervised, aerobic muscular training, both isokinetic and isometric, is a safe and effective way to prevent further decline for patients with moderate weakness (Level B). Muscular training can also improve muscular fatigue, muscle weakness and pain. Training in a warm climate and non-swimming water exercises are particularly useful (Level B). Respiratory muscle training can improve pulmonary function. Recognition of respiratory impairment and early introduction of non-invasive ventilatory aids prevent or delay further respiratory decline and the need for invasive respiratory aid (Level C). Group training, regular follow-up and patient education are useful for the patients' mental status and well-being. Weight loss, adjustment and introduction of properly fitted assistive devices should be considered (good practice points). A small number of controlled studies of potential-specific treatments for PPS have been completed, but no definitive therapeutic effect has been reported for the agents evaluated (pyridostigmine, corticosteroids, amantadine). Future randomized trials should particularly address the treatment of pain, which is commonly reported by PPS patients. There is also a need for studies evaluating the long-term effects of muscular training.

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Category: Diagnosis and Management

Title: Post-polio syndrome and total health status in a prospective hospital study.
Author: Farbu E, Rekand T, Gilhus NE
Affiliation: Department of Neurology, Haukeland University Hospital, Bergen, Norway
Journal: European Journal of Neurology
Citation: 2003 Jul;10(4):407-13.
Publication Year and Month: 2003 07

Abstract: New loss of function among patients with previous polio is frequently reported and has several causes. All patients referred to the Department of Neurology, Haukeland University Hospital, Bergen, for 13 months during 2000-2001 with diagnosis late effects of polio were examined prospectively to identify their symptoms and loss of function. Eighty-five patients aged 47-91 years with mean of 61 years were included. The most common complaints were pain (44%), muscular weakness (27%), and fatigue (16%). Muscular weakness occurred in lower limbs in 75%, in respiratory muscles in only 5%. Walking in stairs was impaired in 72% and outdoor walking in 65%. Seventeen patients (19%) reported no loss of function. Post-polio syndrome was diagnosed in 26% of the patients. Polio-related loss of function including cervical and lumbosacral radiculopathies, mononeuropathies and degenerative joint disease were found in an additional 53%. Eleven patients (13%) had distinct non-polio-related disorders that caused new loss of function. The remaining 8% had a stable condition.

Conclusions: In conclusion, the majority of polio patients who seek hospital, experience a new loss of function because of polio-related disorders. A careful neurological examination is necessary to identify the correct diagnosis and treatment.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Post-polio sequelae: physiological and psychological overview
Author: Frick NM, Bruno RL
Affiliation: Not stated
Journal: Rehabilitation Literature
Citation: Rehabil Lit. 1986 May-Jun; 47(5-6):106-11
Publication Year and Month: 1986 05

Abstract: When the Salk and Sabin vaccines brought an end to the annual summer nightmare of polio epidemics, most Americans simply forgot about polio. Even many of those who had paralytic poliomyelitis put the disease out of their minds once they had achieved maximum recovery of function. Unfortunately, polio has again forced itself into the nation's consciousness. Over the past five years, many of those who had polio have been experiencing new and unexpected symptoms that range in severity from being merely unpleasant to severely debilitating.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Cardiac risk factors in polio survivors
Author: Gawne AC, Wells KR, Wilson KS.
Affiliation: Roosevelt Warm Springs Institute for Rehabilitation, Warm Springs, GA 31830, USA - [email protected]
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 2003 May;84(5):694-6
Publication Year and Month: 2003 05

Abstract: OBJECTIVE: To assess the prevalence of dyslipidemia and other risk factors for coronary heart disease in a sample of polio survivors with and without postpoliomyelitis syndrome.

DESIGN: Retrospective chart review.

SETTING: A multidisciplinary outpatient postpolio clinic.

PARTICIPANTS: Eighty-eight consecutive symptomatic postpolio patients, 50 women (mean age, 59.0y; range, 36-81y) and 38 men (mean age, 61.2y; range, 44-83y).

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Presence of risk factors for coronary heart disease: clinical atherosclerotic disease, male age >or=45 years or female age >or=55 years, history of hypertension (blood pressure >or=140/90mmHg or on antihypertensive medication), diabetes mellitus, cigarette smoking, and high-density lipoprotein (HDL) less than 35mg/dL. Obesity (body mass index [BMI], >25kg/m(2)) was assessed as an intervention target. Laboratory values included fasting total cholesterol, HDL, low-density lipoprotein, triglycerides, and glucose.

RESULTS: Of the total sample, 61.3% had dyslipidemia. Average HDL cholesterol ratio was 4.01 (women, 3.68; men, 4.55). Forty-four patients (50%) had a history of hypertension or had elevated blood pressure. Seven patients (8%) had a history of diabetes or had elevated fasting blood glucose (>110). Eighteen patients (20.4%) were smokers or had a history of smoking; 9 continued to smoke and 9 had quit smoking. Twenty-five patients (28.4%) were overweight (BMI, >25kg/m(2)). Forty-one patients (46.5%) had more than 1 risk factor for coronary heart disease. Nine of the total sample (10.2%) had a history of heart disease ranging from atrial fibrillation to angina. Only 19 patients had a previous diagnosis of dyslipidemia and only 12 were on a lipid-lowering medication.

Conclusions: Polio patients have a high prevalence of dyslipidemia. The study sample supports the National Cholesterol Education Program's Adult Treatment Panel III statements that hypercholesterolemia is underdiagnosed and undertreated. The postpolio population carries a high prevalence of 2 or more coronary heart disease risk factors. Evaluation and rehabilitation of polio patients should include screening for dyslipidemia and education about elimination of controllable risk factors.

Outcome of Research: Not applicable.

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Category: Diagnosis and Management

Title: Management of postpolio syndrome (2010)
Author: Gonzalez H, Olsson T, Borg K
Affiliation: Division of Rehabilitation Medicine, Department of Clinical Sciences, Danderyd Hospital, Karolinska Institute, Stockholm, Sweden – [email protected]
Journal: The Lancet Neurology
Citation: Lancet Neurol. 2010 Jun; 9(6):634-42 and Comment in: Lancet Neurol. 2010 Jun; 9(6):561-3
Publication Year and Month: 2010 06

Abstract: Postpolio syndrome is characterised by the exacerbation of existing or new health problems, most often muscle weakness and fatigability, general fatigue, and pain, after a period of stability subsequent to acute polio infection. Diagnosis is based on the presence of a lower motor neuron disorder that is supported by neurophysiological findings, with exclusion of other disorders as causes of the new symptoms. The muscle-related effects of postpolio syndrome are possibly associated with an ongoing process of denervation and reinnervation, reaching a point at which denervation is no longer compensated for by reinnervation. The cause of this denervation is unknown, but an inflammatory process is possible. Rehabilitation in patients with postpolio syndrome should take a multiprofessional and multidisciplinary approach, with an emphasis on physiotherapy, including enhanced or individually modified physical activity, and muscle training. Patients with postpolio syndrome should be advised to avoid both inactivity and overuse of weak muscles. Evaluation of the need for orthoses and assistive devices is often required.

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Category: Diagnosis and Management

Title: Nonparalytic polio and postpolio syndrome
Author: Halstead LS, Silver JK
Affiliation: National Rehabilitation Hospital, Washington, DC 20010, USA
Journal: American Journal of Physical Medicine & Rehabilitation
Citation: Am J Phys Med Rehabil. 2000 Jan-Feb;79(1):13-8
Publication Year and Month: 2000 01

Abstract: We describe four cases of postpolio syndrome with typical histories, physical examination results, and electrodiagnostic evidence of extensive anterior horn cell disease, as well as the putative pathophysiology of postpolio syndrome in persons with histories of nonparalytic polio and the diagnostic implications for individuals older than 40 yr of age who are experiencing unexplained new weakness, fatigue, and muscle or joint pain. Although the diagnosis of postpolio syndrome traditionally has required a remote history of paralytic polio, many persons such as the ones described here with typical symptoms of postpolio syndrome have no clear history of paralytic disease and are being misdiagnosed. With this in mind, we believe that the diagnostic criteria for postpolio syndrome should be modified to include the following: a history of remote paralytic polio or findings on history, physical examination results, and laboratory studies compatible with poliovirus damage of the central nervous system earlier in life.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Poliomyelitis and the postpolio syndrome
Author: Howard, R.S.
Affiliation: Lane-Fox Unit and Department of Neurology, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London
Journal: The British Medical Journal
(The BMJ)
Citation: 330(7503): 1314–1318.
doi: 10.1136/bmj.330.7503.1314
Publication Year and Month: 2005 06

Abstract: Acute poliomyelitis is now rarely encountered in the United Kingdom, but “imported” poliomyelitis still occurs and it is necessary to distinguish acute poliomyelitis from other causes of acute flaccid paralysis. Despite the obvious success of preventive policies, many patients who had poliomyelitis experience late functional deterioration after periods of prolonged stability—the so called postpolio syndrome. The patterns of disability and their management present unique challenges to the multidisciplinary rehabilitation team.

Conclusions: Although some patients who have had poliomyelitis may later develop wasting, pain, and fatigue in isolation, in most there is significant underlying weakness and skeletal deformity predisposing to functional deterioration. The severe physical stresses of postpolio disability contribute to the development of progressive orthopaedic, respiratory, neurological, and general medical abnormalities, often exacerbated by intercurrent events. These abnormalities may present with atypical clinical features because of the extent of underlying atrophy and weakness, but many are potentially treatable and most patients can be helped to understand and manage increasing disability. It is essential to emphasise that the symptoms, disabilities, and impairments of postpolio functional deterioration are often amenable to treatment. It is also important to urge caution before attributing functional deterioration to a primary “postpolio syndrome” or “progressive postpolio muscular atrophy.”

Outcome of Research: Not applicable

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Category: Diagnosis and Management, Late Effects of Polio

Title: Post-polio syndrome and the phantom of acute previous poliomyelitis: a systemic entity
Author: Jacqueline Stephanie Fernandes do Nascimento (1), Nicolle dos Santos Moraes Nunes (1), Thais de R. Bessa-Guerra (1*), Marco Antonio Alves Azizi (2), Adalgiza Mafra Moreno (2), Antônio Marcos da Silva Catharino (2), Paulo Henrique de Moura (2), Thiago Rodrigues Gonçalves (2), Renata R. T. Castro (2), Acary Souza Bulle Oliveira (3), Marcos RG de Freitas (4) and Marco Orsini (2)
Affiliation: (1) Graduanda de Medicina na Universidade lguaçu (UNIG) - Nova lguaçu, RJ -Brasil

(2) Professor titular na Universidade lguaçu (UNIG). Nova lguaçu, RJ -Brasil

(3) Neurologista, Doutor em Neurologia pela Escola Paulista de Medicina (UNIFESP) - São Paulo (SP) - Brasil

(4) Neurologista, Doutor, Professor Titular e Chefe do Serviço de Neurologia da UFF, Niterói-RJ - Brasil
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: International Journal of Current Research

Vol. 13, Issue, 04
Publication Year and Month: 2021 04

Abstract: Introduction:
Poliomyelitis, often referred to as infantile paralysis or polio, was first described in 1840 by Jakob Von Heine, a German orthopedic physician. It is an acute and infectious disease caused by an enterovirus of worldwide distribution. With the implementation of immunization, the incidence of cases has reduced exponentially worldwide. However, a portion of individuals who have already developed polio, are now manifesting the late effects of polio, called post polio syndrome (PPS).

Case Report:
PRF, male, system analyst. Diagnosis of acute previous polio at the 18th month of age in a hospital in Fortaleza in 1965. At the age of 18 he started to present a new clinic of muscle fatigue, weakness and inability to perform his daily activities.

Discussion:
The Post-polio syndrome (PPS) was first reported in 1875, by Raymond, when reporting the case of a 19-year-old man, previously infected with acute previous polio and at that time had presented a new clinic of muscle weakness and atrophy. Post-poliomyelitis syndrome is defined as a presentation of the delayed effects of polio with the appearance of new neuromuscular symptoms that occur at least 15 years after clinical and functional stability in the natural history of patients with a previous history of acute previous polio. It can be characterized by: new muscle weakness, atrophy and pain, fatigue, sleep disorders, joint pain, cold intolerance, recent weight gain, respiratory distress and dysphagia. Criteria that support the diagnosis are used, such as: (1) Having been affected with acute previous polio; (2) at least 15 years of clinical stability; (3) new clinic of muscle weakness and fatigue; (4) complete or partial recovery of the functions lost in the poliomyelitis period, with a subsequent decline; (5) no other clinical conditions that explain the case. However, effective monitoring by a multidisciplinary team is necessary to assess the degree of functional and motor deterioration, respiratory capacity, as well as the psychological assessment of the patient.

Conclusion:
Although acute previous poliomyelitis has been eradicated in Brazil, as well as in several countries in the world, it is estimated that today there are still about 12 million individuals with poliomyelitis after-effects.

Conclusions: Although acute previous polio has been eradicated in Brazil, as well as in several countries in the world, it is estimated that today there are still about 12 million individuals with poliomyelitis after-effects. These patients overload the health system, especially those diagnosed with post-polio syndrome. Therefore, studies in different areas of health sciences are extremely important for understanding the pathophysiological mechanisms and with the aim of improving the quality of life of these patients.

Studies that address the biochemical and physiological issue can be useful for understanding the neurodegeneration that occurs in PPS, in addition to composing material for the study of the repercussion of a physical / motor after-effect on one or more organic systems and on functional capacity.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Identification of targets for improving access to care in persons with long term physical disabilities
Author: Jennifer L.Wong, Kevin N.Alschuler, Tracy M.Mroz, Kimberly P.Hreha, Ivan R.Molton
Affiliation: University of Washington, Rehabilitation Medicine, United States
Journal: Disability and Health Journal
Citation: Available online 20 January 2019
Publication Year and Month: 2019 01

Abstract: Background
People with long-term physical disability (LTPD) continue to experience difficulties in accessing health care despite the focus of highlighting disparities in the last two decades.

Objectives
To describe health care utilization, accommodations and barriers experienced while accessing health care, and reasons why individuals delay or skip health care among people with LTPD.

Methods
The current study was a part of a larger longitudinal survey administered to individuals with physical disability associated with one of four long-term conditions (MS; SCI; PPS; MD). Measures included demographics, health care utilization, barriers to health care, and reasons for delaying or skipping medical care from the sixth wave of data from 2015 to 2016.

Results
Roughly 90% of all participants (N = 1159) saw at least one medical provider within 12 months. The most encountered barrier participants reported experiencing within that time was an office that did not have a safe transfer device to move them to an exam table (69%). Participants’ physical function, quality of life, status of living with a spouse, diagnostic condition, and sex (male) were significantly associated with endorsing a barrier in accessing health care. The inability to afford out of pocket expenses was the highest reported reason for delaying health care.

Conclusions: People with LTPD access a variety of health care, including rehabilitation services, and continue to experience barriers when doing so. While understanding barriers individuals experience when accessing health care is important, it is equally important to document the type of care they delay or skip due to barriers.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Characteristics and management of postpolio syndrome
Author: Jubelt B, Agre JC
Affiliation: State University of New York, Upstate Medical University, 750 E Adams St, Syracuse, NY 13210, USA – [email protected]
Journal: The Journal of the American Medical Association
Citation: JAMA. 2000 Jul 26;284(4):412-4
Publication Year and Month: 2000 07

Abstract: Postpolio syndrome (PPS) refers to new, late manifestations occurring many years after acute poliomyelitis infection. Over the last 25 years, PPS has become a relatively common problem encountered by primary care physicians. A 1987 National Health Interview Survey estimated that about half of the 640,000 survivors of paralytic poliomyelitis in the United States had new late manifestations of PPS. Subsequent studies in the 1990s have found the occurrence of PPS among patients with previous poliomyelitis to range from 28.5% to 64%. The average time in various reports from the acute poliomyelitis until the onset of PPS is about 35 years, with a range from 8 to 71 years. However, it is unclear if the occurrence of PPS increases with aging, which may be the case based on the most accepted etiologic hypothesis.

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Category: Diagnosis and Management

Title: Neurological Symptoms in Danes with a History of Poliomyelitis: Lifelong Follow-Up of Late Symptoms, their Association with Initial Symptoms of Polio, and Presence of Postpolio Syndrome
Author: Kay L. Nielsen N.M. Wanscher B. Jennum P.
Affiliation: Specialized Hospitalet for Polio and Accident Patients, Rødovre, Denmark
Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark
Medical Department 3, Næstved, Slagelse and Ringsted Hospital, Slagelse, Denmark
Danish Center for Sleep Medicine, Department of Clinical Neurophysiology, Rigshospitalet, University of Copenhagen, Glostrup, Denmark
Journal: European Neurology
Citation: 2018, Vol.80, No. 5-6
Publication Year and Month: 2018

Abstract: Background: Previous studies suggest that patients with a history of poliomyelitis (PM) later in life experience a variety of symptoms. These studies were carried out in patients who later in life were admitted to hospital or became members of polio societies and may therefore not be representative of all polio patients. Little data have been published concerning patients actually discharged from hospital with a diagnosis of acute paralytic PM. Objectives: The aim of this study was to compare the prevalence of late symptoms in individuals with a history of paralytic PM with that of controls, and to study whether late symptoms in individuals with a history of PM were associated with symptoms at the acute stage of polio, and finally to compare the prevalence of symptoms in polio patients with postpolio syndrome (PPS) with the prevalence of symptoms in polio patients without PPS. Methods: A questionnaire concerning various symptoms was sent to a previously established cohort of patients, who during the polio epidemics were discharged from the Department of Infectious Disease at Blegdamshospitalet, Copenhagen, with a diagnosis of paralytic PM, and to age- and gender-matched controls without PM. Information about symptoms at the acute stage of disease was obtained from hospital records. Logistic regression analysis with adjustment for age and gender was applied to compare the occurrence of late symptoms in cases and controls and within the above-mentioned groups of individuals with a history of PM. Results: (i) Compared with controls, individuals with a history of polio significantly more often reported muscle symptoms, pain, neuropathic sensory symptoms, and bulbar symptoms; (ii) the occurrence of symptoms did not seem to be related to symptoms of the initial PM; and (iii) symptom prevalence was significantly higher in individuals with a history of polio who reported PPS as compared with those who did not.

Conclusions: Conclusion: Our data indicate that individuals with a history of PM late in life experience a variety of symptoms that cannot be attributed to lesions of the anterior horn. Furthermore, late symptoms do not seem to be related to initial symptoms of the acute stage of PM but to reported PPS. The last finding supports the perception that the cause of PPS is not just normal ageing.

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Category: Diagnosis and Management

Title: Treatment for postpolio syndrome
Author: Koopman FS, Beelen A, Gilhus NE, de Visser M, Nollet F
Affiliation: Department of Rehabilitation, University of Amsterdam Academic Medical Center, PO Box 22660, Amsterdam, North Holland, Netherlands, 1100 DD
Journal: Cochrane Database of Systematic Reviews
Citation: Cochrane Database Syst Rev. 2015 May 18;5:CD007818
Publication Year and Month: 2015 05

Abstract: BACKGROUND: Postpolio syndrome (PPS) may affect survivors of paralytic poliomyelitis and is characterised by a complex of neuromuscular symptoms leading to a decline in physical functioning. The effectiveness of pharmacological treatment and rehabilitation management in PPS is not yet established. This is an update of a review first published in 2011.

OBJECTIVES: To systematically review the evidence from randomised and quasi-randomised controlled trials for the effect of any pharmacological or non-pharmacological treatment for PPS compared to placebo, usual care or no treatment.

SEARCH METHODS: We searched the following databases on 21 July 2014: Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, PsycINFO and CINAHL Plus. We also checked reference lists of all relevant articles, searched the Database of Abstracts of Reviews of Effects (DARE), the Health Technology Assessment (HTA) Database and trial registers and contacted investigators known to be involved in research in this area.

SELECTION CRITERIA: Randomised and quasi-randomised trials of any form of pharmacological or non-pharmacological treatment for people with PPS. The primary outcome was self perceived activity limitations and secondary outcomes were muscle strength, muscle endurance, fatigue, pain and adverse events.

DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by The Cochrane Collaboration.

MAIN RESULTS: We included 10 pharmacological (modafinil, intravenous immunoglobulin (IVIg), pyridostigmine, lamotrigine, amantadine, prednisone) and three non-pharmacological (muscle strengthening, rehabilitation in a warm climate (that is temperature ± 25°C, dry and sunny) and a cold climate (that is temperature ± 0°C, rainy or snowy), static magnetic fields) studies with a total of 675 participants with PPS in this review. None of the included studies were completely free from any risk of bias, the most prevalent risk of bias being lack of blinding.There was moderate- and low-quality evidence that IVIg has no beneficial effect on activity limitations in the short term and long term, respectively, and inconsistency in the evidence for effectiveness on muscle strength. IVIg caused minor adverse events in a substantial proportion of the participants. Results of one trial provided very low-quality evidence that lamotrigine might be effective in reducing pain and fatigue, resulting in fewer activity limitations without generating adverse events. Data from two single trials suggested that muscle strengthening of thumb muscles (very low-quality evidence) and static magnetic fields (moderate-quality evidence) are safe and beneficial for improving muscle strength and pain, respectively, with unknown effects on activity limitations. Finally, there was evidence varying from very low quality to high quality that modafinil, pyridostigmine, amantadine, prednisone and rehabilitation in a warm or cold climate are not beneficial in PPS.

Conclusions: Due to insufficient good-quality data and lack of randomised studies, it was impossible to draw definite conclusions about the effectiveness of interventions for PPS. Results indicated that IVIg, lamotrigine, muscle strengthening exercises and static magnetic fields may be beneficial but need further investigation to clarify whether any real and meaningful effect exists.

Outcome of Research: More research required.

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Category: Diagnosis and Management

Title: Cognitive behavioural therapy for reducing fatigue in post-polio syndrome and in facioscapulohumeral dystrophy: A comparison
Author: Koopman, Fieke S., Merel A. Brehm, Anita Beelen, Nicole Voet, Gijs Bleijenberg, Alexander Geurts, Frans Nollet
Affiliation: Department of Rehabilitation , Academic Medical Center, University of Amsterdam, PO 22660, 1100 DD Amsterdam, The Netherlands. E-mail: [email protected]

Journal: Journal of Rehabilitation Medicine
Citation: 2017; 49: 585–590
Publication Year and Month: 2017

Abstract: Post-polio syndrome (PPS) and facioscapulohumeral dystrophy (FSHD) are two different neuromuscular disorders. Fatigue is a frequent complaint in both disorders. A recent study showed that cognitive behavioral therapy (CBT), which is a type of psychotherapy that helps patients to identify and reshape thoughts and behavior patterns that contribute to the fatigue was effective in alleviating fatigue in FSHD but not in PPS. In this study we investigated whether this difference in effectiveness might be explained by dissimilar fatigue-related thoughts (for example focusing on fatigue) in both conditions. We used questionnaires to measure the fatigue-related thoughts in 21 patients with PPS and 24 patients with FSHD . It appeared that fatigue-related thoughts in PPS were similar to those in FSHD and thus do not explain the difference in effectiveness of CBT.

Conclusions: Fatigue-related thoughts in PPS were similar to those in FSHD and thus do not explain the difference in effectiveness of CBT.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: A positive turning point in life -- how persons with late effects of polio experience the influence of an interdisciplinary rehabilitation programme
Author: Larsson Lund M (1), Lexell J
Affiliation: (1) Department of Community Medicine and Rehabilitation, Occupational Therapy , Umeå University, Umeå, Sweden
Journal: Journal of Rehabilitation Medicine
Citation: J Rehabil Med. 2010 Jun;42(6):559-65. doi: 10.2340/16501977-0559
Publication Year and Month: 2010 06

Abstract: OBJECTIVE: To describe and enhance our understanding of how persons with late effects of polio experience the influence of an interdisciplinary rehabilitation programme.

PARTICIPANTS: Twelve persons with clinically verified late effects of polio who had participated in an individualized, goal-oriented, comprehensive interdisciplinary rehabilitation programme.

METHODS: Qualitative research interviews analysed using the constant comparative method of grounded theory.

RESULTS: The rehabilitation programme was experienced as a turning point in the participants' lives. Before rehabilitation they felt they were on a downward slope without control. Rehabilitation was the start of a process of change whereby they acquired new skills, which, over time, contributed to a different but good life. After approximately a year, they had a sense of control and had accepted life with late effects of polio. They had also established new habits, taken on a changed valued self and could look to the future with confidence.

Conclusions: This qualitative study has shown that persons with late effects of polio can benefit from an individualized, goal-oriented, comprehensive interdisciplinary rehabilitation programme and experience positive changes in their management of daily activities and in their view of their late effects of polio, their future and their self.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: National Rehabilitation Hospital Limb Classification for Exercise, Research, and Clinical Trials in Post-Polio Patients
Author: Lauro S. Halstead, Anne Carrington Gawne, and Bao T. Pham

Affiliation: The Post-Polio Program; National Rehabilitation Hospital, Washington, DC


Journal: Annals of the New York Academy of Sciences
Citation: The Post-Polio Syndrome: Advances in the Pathogenesis and Treatment Volume 753 pp 343-353 of the Annals of the New York Academy of Sciences May 25, 1995.
Publication Year and Month: 1995 05

Abstract:

Conclusions: A need exists for an objective classification of polio patients for clinical and research purposes that takes into account the focal, asymmetric, and frequent subclinical nature of polio lesions. In order to prescribe a safe, effective exercise program, we developed a five-level (Classes I-V) limb-specific classification system based on remote and recent history, physical examination, and a four-extremity electrodiagnostic study (EMG/NCS). Class I limbs have no history of remote or recent weakness, normal strength, and a normal EMG. Class II limbs have no history of remote or recent weakness (or if remote history of weakness, full recovery occurred), normal strength and EMG evidence of prior anterior horn cell disease (AHCD). Class III limbs have a history of remote weakness with variable recovery, no new weakness, decreased strength, and EMG evidence of prior AHCD. Class IV limbs have a history of remote weakness with variable recovery, new clinical weakness, decreased strength, and EMG evidence of AHCD. Class V limbs have a history of severe weakness with little-to-no recovery, severely decreased strength and atrophy, and few-to-no motor units on EMG. In a prospective study of 400 limbs in 100 consecutive post-polio patients attending our clinic, 94 (23%) limbs were Class I, 88 (22%) were Class II, 95 (24%) were Class III, 75 (19%) were Class IV, and 48 (12%) were Class V. Guidelines for the use of this classification in a clinical/research setting are presented along with sample case histories and class-specific exercise recommendations.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: The treatment of fatigue by non-invasive brain stimulation.
Author: Lefaucheur JP, Chalah MA, Mhalla A, Palm U, Ayache SS, Mylius V.
Affiliation: Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Service de physiologie-Explorations fonctionnelles, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, Créteil, France. Electronic address: [email protected].

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Service de physiologie-Explorations fonctionnelles, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, Créteil, France.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Department of Psychiatry and Psychotherapy, Klinikum der Universität München, Munich, Germany.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Department of Neurology, Philipps University, Marburg, Germany; Department of Neurology, Center for Neurorehabilitation, Valens, Switzerland.
Journal: Clinical Neurophysiology
Citation: 2017 Apr;47(2):173-184.
Publication Year and Month: 2017 04

Abstract: The use of non-invasive brain neurostimulation (NIBS) techniques to treat neurological or psychiatric diseases is currently under development. Fatigue is a commonly observed symptom in the field of potentially treatable pathologies by NIBS, yet very little data has been published regarding its treatment. We conducted a review of the literature until the end of February 2017 to analyze all the studies that reported a clinical assessment of the effects of NIBS techniques on fatigue. We have limited our analysis to repetitive transcranial magnetic stimulation (rTMS) and transcranial direct current stimulation (tDCS). We found only 15 studies on this subject, including 8 tDCS studies and 7 rTMS studies. Of the tDCS studies, 6 concerned patients with multiple sclerosis while 6 rTMS studies concerned fibromyalgia or chronic fatigue syndrome. The remaining 3 studies included patients with post-polio syndrome, Parkinson's disease and amyotrophic lateral sclerosis. Three cortical regions were targeted: the primary sensorimotor cortex, the dorsolateral prefrontal cortex and the posterior parietal cortex. In all cases, tDCS protocols were performed according to a bipolar montage with the anode over the cortical target. On the other hand, rTMS protocols consisted of either high-frequency phasic stimulation or low-frequency tonic stimulation. The results available to date are still too few, partial and heterogeneous as to the methods applied, the clinical profile of the patients and the variables studied (different fatigue scores) in order to draw any conclusion. However, the effects obtained, especially in multiple sclerosis and fibromyalgia, are really carriers of therapeutic hope.

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Category: Diagnosis and Management

Title: Postpolio syndrome and the late effects of poliomyelitis. Part 1. pathogenesis, biomechanical considerations, diagnosis, and investigations.
Author: Lo JK, Robinson LR.
Affiliation: Sunnybrook Health Sciences Centre, Division of Physical Medicine and Rehabilitation, Department of Medicine, University of Toronto, 2075 Bayview Avenue, Toronto, Ontario, Canada
Journal: Muscle & Nerve
Citation: https://doi.org/10.1002/mus.26168
Publication Year and Month: 2018 05

Abstract: Postpolio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. Many theories exist regarding the pathogenesis of PPS, which remains incompletely understood. In contrast, the late effects of poliomyelitis are often a consequence of biomechanical alterations that occur as a result of polio‐related surgeries, musculoskeletal deformities, or weakness. Osteoporosis and fractures of the polio‐involved limbs are common. A comprehensive clinical evaluation with appropriate investigations is essential to fulfilling the established PPS diagnostic criteria. PPS is a diagnosis of exclusion in which a key clinical feature required for the diagnosis is new muscle weakness and/or muscle fatigability that is persistent for at least 1 year. Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS.

Conclusions: Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Post‐polio syndrome and the late effects of poliomyelitis: Part 2. treatment, management, and prognosis.
Author: Lo JK, Robinson LR.
Affiliation: Sunnybrook Health Sciences Centre, Division of Physical Medicine and Rehabilitation, Department of Medicine, University of Toronto, 2075 Bayview Avenue, Toronto, Ontario, M4N 3M5, Canada
Journal: Muscle & Nerve
Citation: https://doi.org/10.1002/mus.26167
Publication Year and Month: 2018 05

Abstract: Post‐polio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. An individualized approach to rehabilitation management is critical. Interventions may include rehabilitation management strategies, adaptive equipment, orthotic equipment, gait/mobility aids, and a variety of therapeutic exercises. The progression of muscle weakness in PPS is typically slow and gradual; however, there is also variability in both the natural history of weakness and functional prognosis. Further research is required to determine the effectiveness of selected medical treatment. Muscle Nerve 58:760–769, 2018

Conclusions: The progression of muscle weakness in PPS is typically slow and gradual; however, there is also variability in both the natural history of weakness and functional prognosis.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: The split hand syndrome in ALS and post-polio-syndrome
Author: M.Hoheisel, L.Burmeister, M.Tesch
Affiliation: Schlosspark-Klinik Berlin, Neurology, Berlin, Germany
Journal: Clinical Neurophysiology
Citation: Volume 129, Issue 8, August 2018, Pages e82-e83

Publication Year and Month: 2018 08

Abstract: Introduction
Electrodiagnostic evaluation for amyotrophic lateral sclerosis (ALS) relies on extensive measurements. As one diagnostic clue, the split-hand-index (SHI) was proposed. It compares the compound muscle action potential (CMAP) of the abductor pollicis brevis (APB) muscle with the CMAP of the abductor digiti minimi (ADM) muscle.

In ALS, asymmetric atrophy of APB and ADM results in the index being reduced compared to the healthy population. This holds true despite the fact, that there is the same segmental innervation C8 for both examined muscles, as was previously discussed. Several studies have shown a diagnostic value in differentiating ALS from other motorneuron-diseases by means of the SHI, claiming a specific form of neurodegeneration in ALS, which is less marked for example in lower motor neuron disease (LMND), spinal muscle atrophy (SMA) or Hirayama disease.

In our study, we aimed to compare the SHI of ALS-patients with our cohort of patients with post-polio-syndrome (PPS) to find out, whether it has a discriminative value in these patients too and to add knowledge to the proposed neuroscientific explanations of asymmetric thenar/hypothenar-atrophy.

Methods
We conducted a retrospective analysis of our post-polio cohort since 1997. All patients were screened whether CMAPs of APB and ADM were collected. For comparison, we screened electrodiagnostic reports of all patients with a diagnosis of ALS for collected CMAPs of APB and ADM. We excluded patients with neuropathy of the median nerve (NMN) by means of a prolonged distal motoric latency (>4,4 ms). Finally we randomly chose the same number of patients from our reports in that time period with normal results (NR), by excluding diagnosis of NMN, acute polyneuropathies and radiculo- and plexopathies of the arm. We calculated the SHI by dividing the CMAP of APB by the CMAP of ADM.

Results
We found a significant difference (p = 0,01) of the SHI between ALS patients (0,97 ± 0,84) and the NR-group (1,26 ± 0,72). The SHI of the PPS-group (0,91 ± 0,55) was not significantly different compared to the NR group (p = 0,08), but showed a trend. Comparing the ALS-group with the PPS-patients, we found no statistically relevant difference (p = 0,83).

Conclusions: As expected, we could reproduce a significant decrease of the SHI in ALS patients. However our results showed no statistically relevant difference between ALS-patients and PPS-patients when comparing the SHI. There is a broader distribution of values in the PPS-group, including cases of very high grade asymmetric atrophy of APB and ADM. A low SHI therefore is of no help in differentiating PPS from ALS-patients.

As a limitation of our study it is important to acknowledge the retrospective study type and a possible selection bias of patients who suffer from an already clinically visible asymmetry, possible increasing electrodiagnostic evaluation numbers of the hand.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Physiotherapy for poliomyelitis: a descriptive study in the Republic of Congo
Author: Mancini S, Coldiron ME, Nicholas S, Llosa AE, Mouniaman-Nara I, Ngala J, Grais RF, Porten K
Affiliation: Epicentre, Paris, France - [email protected].
Journal: BioMedCentral Research Notes
Citation: BMC Res Notes. 2014 Oct 23;7:755. doi: 10.1186/1756-0500-7-755
Publication Year and Month: 2014 10

Abstract: BACKGROUND: A large poliomyelitis outbreak occurred in 2010 in the Republic of Congo. This paper describes the demographic and clinical characteristics of poliomyelitis cases and their outcomes following physiotherapy.

FINDINGS: Demographic and clinical data were collected on 126 individuals between November 23, 2010 and March 23, 2011. The male/female ratio was 2.5 and the median age was 19 years (IQR: 13.5-23). The most severe forms of the disease were more common in older patients, 81 of the 126 patients (64.3%) had multiple evaluations of muscle strength. Among patients with multiple evaluations, 38.1% had improved strength at final evaluation, 48.3% were stable and 13.6% had decreased strength.

Conclusions: Most acute poliomyelitis patients receiving physiotherapy had improved or stable muscle strength at their final evaluation. These descriptive results highlight the need for further research into the potential benefits of physiotherapy in polio affected patients.

Outcome of Research: More research required.

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Category: Brain, Diagnosis and Management, Late Effects of Polio, Post-Polio Motor Unit

Title: Spinal cord gray matter atrophy is associated with functional decline in post-polio syndrome
Author: Maria Janina Wendebourg (1,2), Matthias Weigel (1,2,3,4,5), Laura Richter (1), Vanya Gocheva (6), Patricia Hafner (6), Anna-Lena Orsini (6), Valentina Crepulja (1,2), Simone Schmidt (6), Antal Huck (4), Johanna Oechtering (1), Maria Blatow (7), Tanja Haas (3,4), Cristina Granziera (1,2,5), Ludwig Kappos (1,2,5), Philippe Cattin (4), Oliver Bieri (3,4) Dirk Fischer (6), Regina Schlaeger (1,2,5)
Affiliation: 1. Neurology Clinic and Policlinic, Department of Clinical Research, University Hospital Basel, University of Basel, Basel, Switzerland

2. Translational Imaging in Neurology (ThINk), Department of Biomedical Engineering, University of Basel, Basel, Switzerland

3. Division of Radiological Physics, Department of Radiology, University Hospital Basel, Basel, Switzerland

4. Department of Biomedical Engineering, University of Basel, Basel, Switzerland

5. MS Center and Research Center for Clinical Neuroimmunology and Neuroscience Basel (RC2NB), University Hospital Basel and University of Basel, Basel, Switzerland

6. Division of Pediatric Neurology, University of Basel Children's Hospital, Basel, Switzerland

7. Department of Neuroradiology, Clinical Neuroscience Center, University Hospital Zurich, University of Zurich, Zurich,
Journal: European Journal of Neurology
Citation: Eur J Neurol. 2022;00:1–11.
DOI: 10.1111/ene.15261
Publication Year and Month: 2022 01

Abstract: Objective: To determine if patients with post- polio syndrome (PPS) show spinal cord gray matter (SCGM) atrophy and to assess associations between SCGM atrophy, muscle strength and patient- reported functional decline.

Methods: Twenty patients diagnosed with PPS (March of Dimes criteria) and 20 age- and sex- matched healthy controls (HC) underwent 3T axial 2D- rAMIRA magnetic resonance imaging at the intervertebral disc levels C2/C3–C6/C7, T9/T10 and the lumbar enlarge-ment level (Tmax) (0.5 × 0.5 mm2 in- plane resolution). SCGM areas were segmented manu-ally by two independent raters. Muscle strength, self-reported fatigue, depression and pain measures were assessed.

Results: Post- polio syndrome patients showed significantly and preferentially re-duced SCGM areas at C2/C3 (p= 0.048), C3/C4 (p= 0.001), C4/C5 (p< 0.001), C5/C6 (p= 0.004) and Tmax (p= 0.041) compared to HC. SCGM areas were significantly associated with muscle strength in corresponding myotomes even after adjustment for fatigue, pain and depression. SCGM areaTmax together with age and sex explained 68% of ankle dorsiflexion strength variance. No associations were found with age at or time since infection. Patients reporting PPS- related decline in arm function showed significant cervical SCGM atrophy compared to stable patients adjusted for initial disease severity.

Conclusions: Patients with PPS show significant SCGM atrophy that correlates with mus-cle strength and is associated with PPS- related functional decline. Our findings suggest a secondary neurodegenerative process underlying SCGM atrophy in PPS that is not ex-plained by aging or residua of the initial infection alone. Confirmation by longitudinal studies is needed. The described imaging methodology is promising for developing novel imaging surrogates for SCGM diseases.

Conclusions: The rAMIRA approach is a novel, promising, clinically feasible and sensitive method for segment-wise quantitation of GM atrophy in the cervical and thoracic SC in patients with lower motor neuron disorders. This study demonstrated its clinical applicability and vali-dated it in patients with PPS, a presumed pure, lower motor neuron disorder, which can serve as a model for other neurodegenerative, genetic or autoimmune diseases of the SCGM.

Patients with PPS show significant SCGM atrophy, particularly at levels close to the cervical and lumbar enlargements. Even after adjustment for the level of depression, fatigue and pain, potential confounding symptoms frequently observed in PPS, SCGM atrophy is significantly and segment-wise associated with muscle strength in corresponding myotomes. Moreover, SCGM atrophy is associated with patient-reported PPS-related functional decline. Secondary analyses suggest that SCGM atrophy is rather due to a second dis-ease phase than being a sole residuum of the initial infection or a pure aging effect. These observations support the hypothesis of a focally accentuated neurodegenerative process in the SC underlying PPS. Larger, ideally multicentric, longitudinal studies conducted over a sufficiently long timespan are an important next step to confirm our results and gain more insights into the development of SCGM atrophy over time and its correlation to clinical symptom evolution in patients with PPS.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Unraveling the transmission ecology of polio
Author: Martinez-Bakker M (1), King AA (1,2), Rohani P (1,2)
Affiliation: (1) Department of Ecology & Evolutionary Biology, University of Michigan, Ann Arbor, Michigan, United States of America; (2) Center for the Study of Complex Systems, University of Michigan, Ann Arbor, Michigan, United States of America; Fogarty International Center, National Institutes of Health, Bethesda, Maryland, United States of America
Journal: Public Library of Science
Citation: PLoS Biol. 2015 Jun;13(6): e1002172. doi:10.1371/journal.pbio.1002172
Publication Year and Month: 2015 06

Abstract: Sustained and coordinated vaccination efforts have brought polio eradication within reach. Anticipating the eradication of wild poliovirus (WPV) and the subsequent challenges in preventing its re-emergence, we look to the past to identify why polio rose to epidemic levels in the mid-20th century, and how WPV persisted over large geographic scales. We analyzed an extensive epidemiological dataset, spanning the 1930s to the 1950s and spatially replicated across each state in the United States, to glean insight into the drivers of polio’s historical expansion and the ecological mode of its persistence prior to vaccine introduction. We document a latitudinal gradient in polio’s seasonality. Additionally, we fitted and validated mechanistic transmission models to data from each US state independently. The fitted models revealed that: (1) polio persistence was the product of a dynamic mosaic of source and sink populations; (2) geographic heterogeneity of seasonal transmission conditions account for the latitudinal structure of polio epidemics; (3) contrary to the prevailing “disease of development” hypothesis, our analyses demonstrate that polio’s historical expansion was straightforwardly explained by demographic trends rather than improvements in sanitation and hygiene; and (4) the absence of clinical disease is not a reliable indicator of polio transmission, because widespread polio transmission was likely in the multiyear absence of clinical disease. As the world edges closer to global polio eradication and continues the strategic withdrawal of the Oral Polio Vaccine (OPV), the regular identification of, and rapid response to, these silent chains of transmission is of the utmost importance.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Motoneuron Disease and Past Poliomyelitis in England and Wales
Author: Martyn CN, Barker DJP, Osmond C.
Affiliation: MRC Environmental Epidemiology Unit, Southampton Hospital, United Kingdom
Journal: The Lancet Neurology
Citation: The Lancet, 331(8598):1319-1322

Publication Year and Month: 1988 06

Abstract: Past notification rates for poliomyelitis show a close geographical relation with current mortality from motoneuron disease in England and Wales. The increasing rate of poliomyelitis during the first half of this century and its predilection for affluent places and families were unique amongst infectious diseases. The unusual epidemiology of poliomyelitis is now being paralleled by motoneuron disease. These observations provide new evidence for a causal connection between the two conditions.

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Post-polio sequelae - differential diagnosis and management
Author: Maynard FM
Affiliation: Not stated
Journal: Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):857-61
Publication Year and Month: 1985 07

Abstract: Forty-two patients with a past history of poliomyelitis were evaluated at a post-polio clinic for new problems or impairments. Evaluation included a complete history, neurological and biomechanical examination and electrodiagnostic studies. Based on this evaluation patients were placed into three groups: 23 patients were considered to have or likely to have Progressive Post-Polio Muscular Atrophy (PPPMA); 17 patients were considered to have other post-polio sequelae; and two patients had problems unrelated to a past history of polio but mistaken for post-polio sequelae. Musculoskeletal pain was a common complaint among all groups of patients. Twenty-two of the 40 patients with post-polio sequelae were advised to alter their method of ambulation and/or decrease their activity pattern in order to decrease strain and/or excessive exertion of involved muscles. The role of chronic overuse and exercise in producing PPPMA or musculoskeletal pain problems is discussed. Characteristic clinical problems and useful management plans are described.

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Category: Diagnosis and Management

Title: Life-Long Morbidity Among Danes With Poliomyelitis
Author: Nielsen NM, Rostgaard K, Askgaard D, Skinhøj, & P Aaby P.
Affiliation: Department of Epidemiology Research, Danish Epidemiology Science
Centre, Statens Serum Institut (Nielsen, Rostgaard, Aaby) and Department of Infectious Diseases M, National University Hospital, (Askgaard, Skinhøj), Copenhagen,
Denmark.
Supported by the Danish Medical Research Council, the AP Møller and Chastine
McKinney Møller Foundation, the Danish National Research Foundation, the WedellWedellsborg Foundation, and The National Polio Society
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil 2004; 85:385-91 - doi:10.1016/S0003-9993(03)00474-X
Publication Year and Month: 2004 03

Abstract: Abstract
OBJECTIVE:
To estimate long-term morbidity in a cohort of Danish poliomyelitis patients.

DESIGN:
A historical prospective cohort study of 27,047 persons.

SETTING:
Denmark.

PARTICIPANTS:
A total of 5421 persons hospitalized for poliomyelitis between 1919 to 1954 in Copenhagen, Denmark, and 21,626 age- and gender-matched Danes. Participants were followed up on average for 20.6 years, yielding a total of 555,884 person-years of follow-up.

INTERVENTIONS:
Not applicable.

MAIN OUTCOME MEASURES:
The exposed (poliomyelitis) cohort and the unexposed (control) cohort were followed up for somatic hospitalization from 1977 to 1999 in the Danish Hospital Discharge Register. The incidence rate ratio (IRR) was calculated as the ratio between the incidence rate of disease in the exposed and unexposed cohorts.

RESULTS:
Overall, polio patients had a 1.2- to 1.3-fold increased risk of being hospitalized with pulmonary diseases, heart diseases, gastrointestinal disorders, or diseases of the locomotive apparatus. Among paralytic polio patients, long-term morbidity seems to be associated with the acute severity of poliomyelitis, as well as young age at infection. Paralytic patients, who contracted respiratory polio under the age of 5, had the highest risk of being hospitalized with lung diseases (IRR=7.26; 95% confidence interval [CI], 3.06-18.33), diseases of the locomotive apparatus (IRR=4.05; 95% CI, 1.66-9.86), heart diseases (IRR=1.70; 95% CI, 0.65-3.98), and diseases of the digestive system (IRR= 2.23; 95% CI, 1.03-4.62). Surprisingly, patients without paralyses, especially women, also had an increased morbidity.

CONCLUSIONS:
Overall, a history of poliomyelitis was associated with a slightly increased morbidity measured by hospitalizations. Long-term morbidity was highest among respiratory polio patients; however, patients presumably left without any residual symptoms also had an increased morbidity.

Conclusions: Overall, a history of poliomyelitis was associated with a slightly increased morbidity measured by hospitalizations. Long-term morbidity was highest among respiratory polio patients; however, patients presumably left without any residual symptoms also had an increased morbidity.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Poliomyelitis and Parkinson Disease.
Author: Nielsen NM, Rostgaard K, Hjalgrim H, Aaby P, Askgaard D.
Affiliation: Department of Epidemiology Research, National University Hospital, Copenhagen, Denmark
Journal: The Journal of the American Medical Association
Citation: JAMA. 2002;287(13):1650–1651.
doi:10.1001/jama.287.13.1645
Publication Year and Month: 2002 04

Abstract: Parkinson disease (PD), which is due to loss of dopaminergic neurons in the zona compacta of the substantia nigra,1 may involve both genetic and environmental risk factors.2 Poliovirus is believed to cause neuronal damage in the substantia nigra,3 and thus a history of poliovirus infection may be associated with an increased risk of PD

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Multiple sclerosis and poliomyelitis. A Danish historical cohort study.
Author: Nielsen NM, Wohlfahrt J, Melbye M, Rasmussen S, Mølbak K, Askgaard DS, Aaby P.
Affiliation: Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark
Journal: Acta Neurologica Scandinavica
Citation: Acta Neurol Scand 2000 Jun;101(6):384-7.
Publication Year and Month: 2000 06

Abstract: OBJECTIVE:
To evaluate whether persons with a history of poliomyelitis are at an increased risk of developing multiple sclerosis (MS).

MATERIAL AND METHODS:
All patients diagnosed with acute poliomyelitis in the greater capital area of Copenhagen, Denmark, between 1919 and 1954 were identified and followed with respect to MS. Information on vital status and diagnosis of sclerosis was obtained through linkage with the Danish Civil Registration System and The Danish Multiple Sclerosis Registry, respectively. Follow-up started on the date of the establishment of the Danish Civil Registration System (April 1, 1968) until death, emigration or December 31, 1996, whichever came first. The observed incidence of MS among polio patients was compared with the expected incidence calculated according to national gender, age and period specific rates of MS.

RESULTS:
During 149,364 years of follow-up, 19 cases of multiple sclerosis were observed among 5652 polio patients compared with 11.0 expected (SIR = 1.73 (1.04-2.74)). The increased risk of MS was most pronounced in polio patients hospitalized during adolescence. Neither gender nor the acute severity of poliomyelitis modified the risk of MS.

CONCLUSION:
Our results are based on small numbers of events, however the findings suggest that the polio patients might be at an increased risk of MS.

Conclusions:

Outcome of Research: More research required

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Comments (if any): There appears a higher incidence of Multiple Sclerosis in this study of Danish patients previously diagnosed with Poliomyelitis. This should not be interpreted that previously contracting Poliomyelitis will lead to Multiple Sclerosis.

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Category: Diagnosis and Management

Title: Postpolio syndrome: unanswered questions regarding cause, course, risk factors, and therapies
Author: Nollet F
Affiliation: Not stated
Journal: The Lancet Neurology
Citation: Lancet Neurol. 2010 Jun;9(6):561-3 - Comment on: Lancet Neurol. 2010 Jun;9(6):634-42
Publication Year and Month: 2010 06

Abstract: Living with the consequences of poliomyelitis is not recognised as an important health issue at present. However, millions of people worldwide have lasting impairments caused by polio infection, many of whom also had a decline in muscle function and decline in activities of daily living after years of stable functioning.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Postpolio syndrome
Author: Nollet F, de Visser M
Affiliation: Department of Rehabilitation, Academic Medical Center, University of Amsterdam, PO Box 22660, 1100 DD Amsterdam, the Netherlands – [email protected]
Journal: Archives of Neurology
Citation: Arch Neurol. 2004 Jul;61(7):1142-4
Publication Year and Month: 2004 07

Abstract: This paper has no abstract - this is an extract:

Postpolio syndrome (PPS) refers to a decline of muscle function usually occurring 30 to 40 years after the acute polio episode. This syndrome has been widely recognized only during the last decades, when many people affected by the large epidemics of the previous century experienced new muscle weakness as they grew older. However, cases of late-onset weakening following poliomyelitis were already reported at the end of the 19th century.

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Category: Diagnosis and Management

Title: Sense of Coherence in persons with late effects of polio
Author: Nolvi M, Brogardh C, Jacobsson L, Lexell J
Affiliation: Maria Nolvi, MD, Department of Health Sciences, PO Box 157, Lund University, SE-221 00 Lund, Sweden
Journal: NeuroRehabilitation
Citation: Vol. 42, no. 1, pp. 103-111, 2018
Publication Year and Month: 2018 01

Abstract: BACKGROUND:Sense of Coherence (SOC) is important for successful adaptation and mental well-being in people with life-long medical conditions. Late effects of polio (LEoP) often lead to a life-long disability, but no study has assessed SOC in this population. OBJECTIVE:To assess SOC in persons with LEoP and to explore the association between SOC, demographics (age, gender, marital status and level of education) and variables related to LEoP (age at polio onset, number of years from polio until onset of LEoP and self-rated disability). METHOD:Ninety-three community-dwelling persons with clinically verified LEoP responded to a postal survey with the Sense of Coherence Scale (SOC-13). A hierarchical multiple regression analysis was performed to explore the associations with SOC. RESULTS:SOC varied considerably among the participants. The mean and median SOC-13 total sum score was 71.8 and 76 points, which is similar to age-matched non-disabled people. The number of years before onset of LEoP and self-rated disability together with the participants’ marital status and level of education explained 37% (p < 0.001) of the variance in SOC.

Conclusions: CONCLUSION:Persons with LEoP have a level of sense of coherence (SOC) indicating that they generally have the ability to understand, handle and being motivated when dealing with stressful events and problems arising in their lives as a result of their disability. Being married and having a higher education, living many years before onset of LEoP and perceiving a mild to moderate disability contributed to a strong SOC.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Lipid metabolic pathways converge in motor neuron degenerative diseases
Author: Olivia J Rickman, Emma L Baple, and Andrew H Crosby
Affiliation: Medical Research (Level 4), RILD Wellcome Wolfson Centre, University of Exeter Medical School, Royal Devon and Exeter NHS Foundation Trust, Barrack Road, Exeter, EX2 5DW, UK

Correspondence to: Professor Andrew Crosby Professor of Human Genetics University of Exeter Medical School Medical Research (Level 4) RILD Wellcome Wolfson Centre Royal Devon and Exeter NHS Foundation Trust Barrack Road, Exeter, EX2 5DW, UK E-mail: [email protected]
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Brain. 2020 Apr; 143(4): 1073–1087.
Published online 2019 Dec 18. doi: 10.1093/brain/awz382
Publication Year and Month: 2019 12

Abstract: Motor neuron diseases (MNDs) encompass an extensive and heterogeneous group of upper and/or lower motor neuron degenerative disorders, in which the particular clinical outcomes stem from the specific neuronal component involved in each condition. While mutations in a large number of molecules associated with lipid metabolism are known to be implicated in MNDs, there remains a lack of clarity regarding the key functional pathways involved, and their inter-relationships. This review highlights evidence that defines defects within two specific lipid (cholesterol/oxysterol and phosphatidylethanolamine) biosynthetic cascades as being centrally involved in MND, particularly hereditary spastic paraplegia. We also identify how other MND-associated molecules may impact these cascades, in particular through impaired organellar interfacing, to propose ‘subcellular lipidome imbalance’ as a likely common pathomolecular theme in MND. Further exploration of this mechanism has the potential to identify new therapeutic targets and management strategies for modulation of disease progression in hereditary spastic paraplegias and other MNDs.

Keywords: HSP, MND, cholesterol, mitochondria, lipidome imbalance

Conclusions: The development of new methodologies and approaches are important to delineate the specific subcellular biomarker deficits, such as oxysterol and PE imbalance, in HSP and MND. As well as providing potentially powerful biomarkers of disease, such tools may enable monitoring of treatment efficacy of therapeutics to re-address disease-associated lipidome imbalances. Specific genetic subtypes may be more amenable to treatment at targeting (for example) feedback systems, such as CDCA in CTX, or addressing oxysterol imbalance in SPG5. Genetic subtypes of disease leading to more complex subcellular outcomes may require multiple treatment approaches to address the specific mechanistic basis of each condition, and it may be unlikely that one approach will entail a ‘fix all’ treatment. Ultimately, clearer definition of the subcellular lipidome (and other) biological pathways underlying MND and HSP will pave the way for a more elegant approaches for predicting onset and severity of disease, and for designing and monitoring new therapeutic approaches.

Outcome of Research: More research required

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Category: Diagnosis and Management, Differential Diagnosis, Gender Differences, Late Effects of Polio

Title: Postpolio Syndrome: A Review of Lived Experiences of Patients
Author: Oluwaseyi Jacob Oluwasanmi, Devaunna Andrene Mckenzie, Idowu Oluwasegun Adewole, Christian O Aluka, James Iyasse, Esther Olunu, and Adegbenro Omotuyi Fakoya1
Affiliation: Department of Microbiology, All Saints University School of Medicine, Commonwealth of Dominica, Roseau, Dominica
1Department of Anatomical Sciences, University of Medicine and Health Sciences, Basseterre, St. Kitts and Nevis
Address for correspondence: Dr. Adegbenro Omotuyi Fakoya, University of Medicine and Health Sciences, Basseterre, St. Kitts and Nevis. E-mail: [email protected]
Journal: International Journal of Applied & Basic Medical Research
Citation: 9(3), 129–134. https://doi.org/10.4103/ijabmr.IJABMR_333_18
Publication Year and Month: 2019 07

Abstract: Postpolio syndrome (PPS) refers to a group of conditions that are present in patients, years after recovery from initial acute paralytic poliomyelitis. About 15%–80% of 20 million polio survivors worldwide will experience exacerbation of symptoms which typically appear 15–30 years after the resolution of initial poliomyelitis. Symptoms include new muscle weakness, fatigue, myalgia, joint pain, dysphagia, and difficulty breathing. Other reported symptoms include cold intolerance, sleep disorder, dysphonia, loss of stamina, musculoskeletal deformities, cardiovascular disorders, psychosocial problems, and restless legs syndrome. These symptoms are attributed to the superimposed neuronal loss of aging with inflammatory mechanisms, but without any convincing evidence of viral reactivation. Risk factors include female gender, respiratory symptoms, normal aging, permanent disability caused by motor neuron damage, muscle overuse and disuse, aging, and immunologic mechanisms. Hypothyroidism-induced myopathy and fibromyalgia are a differential diagnosis for PPS, and exclusion diagnosis is required as confirmatory criteria for PPS. The symptoms of PPS presented determine the course of management.

Keywords: Fatigue, muscle weakness, myalgia, poliomyelitis, postpolio syndrome

Conclusions: PPS is a sequela of acute poliomyelitis which occurs decades after resolution of the initial paralytic or nonparalytic polio. Fatigue, muscle weakness, myalgia, and joint pain are the major symptoms of PPS. Diagnosis of PPS is made based on the exclusion of other conditions that can cause similar symptoms. The quality of life of patients with PPS is significantly affected by the reoccurrence of the constellations of symptoms they experience. Proper assessment and treatment should be performed to prevent severe impairment of function in patients.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Repetitive transcranial magnetic stimulation in treatment of post polio syndrome
Author: Pastuszak, Z., Piusaska-Macoch, R., Stepieri, A., Czernicki, Z.,
Affiliation: Department of Neurosurgery, Mossakowski Medical Research Centre, Polish Academy of Sciences, A. Pawińskiego 5, 02-106 Warsaw, Poland

Department of Neurology, Military Institute of Medicine, Szaserów 128, 04-141 Warsaw, Poland

Department of Neurosurgery, Warsaw University of Medicine, Cegłowska 80, 01-809 Warsaw, Poland
Journal: Neurologia i Neurochirurgia Polska
Citation: Volume 52 (2): 2018, Pages 281-284
Publication Year and Month: 2018 03

Abstract: Post polio syndrome is a rare disease that occurs decades after polio virus infection. Repetitive transcranial magnetic stimulation (rTMS) is a treatment option with proved effectiveness in drug resistant depression. Possibly it can be helpful in therapy of other neurological diseases including post polio syndrome.

Conclusions: rTMS can be an effective method in treatment of post polio syndrome but further studies with larger group need to be done to confirm that data.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Effect of treatment and noncompliance on post-polio sequelae
Author: Peach PE, Olejnik S
Affiliation: Roosevelt Warm Springs Institute for Rehabilitation, Georgia 31830
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1199-1203
Publication Year and Month: 1991 11

Abstract: In this study of 77 patients with post-polio sequelae (PPS), symptoms and manual test scores on initial evaluation were compared with those at subsequent follow-up evaluations. Patients were divided into three groups based on the degree to which they had complied with clinically recommended interventions: compliers, partial compliers, and noncompliers. At the end of the follow-up period (2.2 +/- 1.2 years), the mean muscle function scores of the entire study group had declined -1.5%, which represented a decline of -0.7% annually. On follow-up evaluations, the complier group had realized an improvement or resolution of post-polio symptoms, and also an improvement in muscle function of +0.6% annually. The partial complier group had realized either no improvement, or improvement in post-polio symptoms, but showed a further decline in muscle function of -3.0%, or an annual decline of -1.3%. The noncomplier group showed either no change, or a worsening of post-polio symptoms, and also showed a further decline in muscle function of -4.1%, which represented an annual decline of -2.0%.

Conclusions: The disparate outcomes among our post-polio patients underscore the need to develop more effective intervention strategies to achieve improved patient compliance, given the favorable outcomes experienced by patients who complied with clinical recommendations.

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Category: Diagnosis and Management

Title: Frequency and clinical manifestations of post-poliomyelitis syndrome in a Brazilian tertiary care center
Author: Quadros AA (1), Conde MT, Marin LF, Silva HC, Silva TM, Paula MB, Pereira RD, Ramos PE, Abe G, Oliveira AS
Affiliation: (1) Department of Neurology and Neurosurgery, Division of Neuromuscular Disorders, Federal University of São Paulo (Unifesp), São Paulo SP, Brazil - [email protected]
Journal: Arquivos de Neuro-psiquiatria
Citation: Arq Neuropsiquiatr. 2012 Aug;70(8):571-3
Publication Year and Month: 2012 08

Abstract: OBJECTIVE: To determine the frequency and clinical manifestations of patients with post-poliomyelitis syndrome (PPS) in a Brazilian division of neuromuscular disorders.

METHODS: A total of 167 patients with prior history of paralytic poliomyelitis was investigated for PPS, based on international diagnostic criteria. Other variables analyzed were: gender, race, age at poliomyelitis infection, age at PPS onset, and PPS symptoms.

RESULTS: One hundred and twenty-nine patients presented PPS, corresponding to 77.2% of the studied population. 62.8% were women and 37.2% were men. Mean age of patients with PPS at onset of PPS symptoms was 39.9±9.69 years. Their main clinical manifestations were: new weakness in the previously affected limbs (69%) and in the apparently not affected limbs (31%); joint pain (79.8%); fatigue (77.5%); muscle pain (76%); and cold intolerance (69.8%).

Conclusions: Most patients of our sample presented PPS. In Brazil, PPS frequency and clinical features are quite similar to those of other countries.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Epidemiology of the post-polio syndrome
Author: Ramlow J, Alexander M, LaPorte R, Kaufmann C, Kuller L
Affiliation: Department of Biostatistics, Graduate School of Public Health, University of Pittsburgh, PA
Journal: American Journal of Epidemiology
Citation: Am J Epidemiol. 1992 Oct 1;136(7):769-86
Publication Year and Month: 1992 10

Abstract: A late-onset syndrome, consisting of muscle weakness, muscle pain, and unaccustomed fatigue, has been reported with increasing frequency among former poliomyelitis patients. A population-based cohort of poliomyelitis patients from Allegheny County, Pennsylvania, was traced and surveyed to estimate the prevalence and incidence and to identify determinants of the post-polio syndrome. A questionnaire validated in clinical examinations of 40 cohort members was used in the survey. The prevalence of the post-polio syndrome was 28.5% of all paralytic cases (95% confidence interval 24.4-32.6). The risk of post-polio syndrome was significantly higher among patients who sustained substantial permanent impairment after polio and among females. The incidence did not vary with age at acute onset, acute severity, or level of physical activity after recovery. The strongest determinant of post-polio syndrome onset was the length of the interval following the acute illness, with incidence peaking at 30-34 years. Of all cases of post-polio syndrome, 79% reported no major change in impairment status since onset. This study demonstrates that poliomyelitis patients are not equally susceptible to post-polio syndrome within the interval of 30-40 years after the original illness. For syndrome cases, the onset was associated with new neuromuscular symptoms and functional changes but not with major new impairment.

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Category: Diagnosis and Management

Title: The cultural context of polio biographies
Author: Scheer J, Luborsky ML
Affiliation: National Rehabilitation Hospital Research Center, Washington, DC 20010
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1173-81
Publication Year and Month: 1991 11

Abstract: Cultural contexts influence the ways individuals interpret and experience functional losses associated with post-polio sequelae. Using in-depth multiple interview case studies from two National Institute on Aging projects, the concept of “biographies” is presented to place the individuals’ polio-related experiences within the context of their lives. Two major cultural contexts shape the construction of polio biographies: normative life course expectations and developmental tasks; and traditions associated with polio recovery and rehabilitation. The authors identify key dimensions of personal concern among polio survivors that can be used as entrance points for effective clinical intervention and to promote treatment compliance.

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Category: Diagnosis and Management

Title: Characteristics of Patients at First Visit to a Polio Clinic in Sweden
Author: Skough Vreede, K. and Sunnerhagen, K.S.
Affiliation: Goteborg University
Journal: PLoS One
Citation: https://doi.org/10.1371/journal.pone.0150286
Publication Year and Month: 2016 03

Abstract: Aim
Describe polio patients visiting a polio clinic in Sweden, a country where vaccination was introduced in 1957.

Design
A consecutive cohort study.

Patients
Prior polio patients.

Methods
All patients (n = 865) visiting the polio clinic at Sahlgrenska University Hospital, Gothenburg Sweden, between 1994 and 2012 were included in this study. Data at first visit regarding patient characteristics, polio classification, data of electromyography, origin, assistive devices and gait speed as well as muscle strength were collected for these patients. Twenty-three patients were excluded because no polio diagnosis could be established. A total of 842 patients with confirmed polio remained in the study.

Results
More than twenty percent of the patients were from countries outside the Nordic region and considerably younger than those from the Nordic region. The majority of the emigrants were from Asia and Africa followed by Europe (outside the Nordic region). Of all patients included ninety-seven percent (n = 817) had polio in the lower extremity and almost 53% (n = 444) had polio in the upper extremity while 28% (n = 238) had polio in the trunk, according to clinical classification of polio. Compared with a sample of the normal population, the polio patients walked 61–71% slower, and were 53–77% weaker in muscle strength of the knee and foot as well as grip strength.

Conclusion
The younger patients with polio emigrating from countries with different cultures may lead to a challenge for the multi professional teams working with post-polio rehabilitation and are of importance when planning for the care of polio patients the coming years.

Conclusions: Polio in lower extremities was more common than polio in upper extremities, verified both
by EMG and clinical classification. This is in accordance with earlier studies. Polio in lower
extremities was also classified as clinically unstable or severely atrophic to a higher extent than
polio in upper extremities (as shown in Fig 1). This is in accordance with an earlier study by
Sandberg et al [20] indicating a more pronounced ongoing denervation-reinnervation process
over time in a lower extremity muscle compared to upper extremity muscle (tibialis anterior
and biceps brachii respectively). The same pattern was also seen in patients studied in Minnesota where patients with leg weakness were twice as likely to complain of new problems compared to those with arm weakness [10].

The ongoing denervation-reinnervation process in patients with PPS results in larger motor
units.When motor-unit size has reached an upper limit, further losses of neurons can no longer
be compensated for and this results in increased muscle weakness [21]. The patients
showed to be stronger in isometric endurance compared to normal population. This may be
explained by the fact that the patients were weaker than the normal population in isometric
peak torque, which the measure of isometric endurance was based on. An increase in type I
(slow) muscle fibres has also been described in prior polio patients [22–23] and may be due to
a transition of type II (fast) to type I (slow).

An important study limitation were seen in the classification of polio as polio were classified
for left and right arm and leg, respectively, and not per muscle group. This can explain why a
polio affected leg in some cases was stronger compared to normal values as the muscles
involved in the strength measured i.e. knee flexion and knee extension muscles may not be
affected of polio. And the same is applicable regarding muscle strength of the foot as well as
grip strength. Data of muscle strength from some of the subjects were missing for different reasons i.e. they may have just not had time to participate, or refused to participate and some were too weak to perform the strength test. Some of the patients with muscle strength data missing, tried to perform the test, but were too weak to get a result. In the future, the use of ultrasound may be used to assess muscle function [24]. This would give the possibility to have more information of muscle function since this does not require that the patient has muscle strength to overcome gravity, which is a requirement for isokinetic testing.

Outcome of Research: Effective

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Category: Clinical Evaluation, Diagnosis and Management, Late Effects of Polio

Title: Post-polio Syndrome: More Than Just a Lower Motor Neuron Disease
Author: Stacey Li Hi Shing, Rangariroyashe H. Chipika, Eoin Finegan, Deirdre Murray, Orla Hardiman, and Peter Bede
Affiliation: Computational Neuroimaging Group, Academic Unit of Neurology, Biomedical Sciences Institute, Trinity College Dublin, Dublin, Ireland
Edited by: Francesca Trojsi, University of Campania, Luigi Vanvitelli Caserta, Italy
Reviewed by: Andrea Romigi, Mediterranean Neurological Institute (IRCCS), Italy; Louisa Ng, The University of Melbourne, Australia
*Correspondence: Peter Bede ei.dct@pedeb
Journal: Frontiers in Neurology
Citation: 10, 773. https://doi.org/10.3389/fneur.2019.00773
Publication Year and Month: 2019 07

Abstract: Post-polio syndrome (PPS) is a neurological condition that affects polio survivors decades after their initial infection. Despite its high prevalence, the etiology of PPS remains elusive, mechanisms of progression are poorly understood, and the condition is notoriously under-researched. While motor dysfunction is a hallmark feature of the condition, generalized fatigue, sleep disturbance, decreased endurance, neuropsychological deficits, sensory symptoms, and chronic pain are also often reported and have considerable quality of life implications in PPS. The non-motor aspects of PPS are particularly challenging to evaluate, quantify, and treat. Generalized fatigue is one of the most distressing symptoms of PPS and is likely to be multifactorial due to weight-gain, respiratory compromise, poor sleep, and polypharmacy. No validated diagnostic, monitoring, or prognostic markers have been developed in PPS to date and the mainstay of therapy centers on symptomatic relief and individualized rehabilitation strategies such as energy conservation and muscle strengthening exercise regimes. Despite a number of large clinical trials in PPS, no effective disease-modifying pharmacological treatments are currently available.

Conclusions: Despite being one of the most devastating neurodegenerative conditions in the world, surprisingly limited research is undertaken in post-polio syndrome. Its pathogenesis remains elusive, no sensitive diagnostic tools have been developed, and validated prognostic and monitoring markers are lacking. Non-motor symptoms of PPS have considerable quality of life implications and are notoriously challenging to manage. The etiology of fatigue in PPS is yet to be elucidated and successful individualized management strategies are needed to maintain mobility, independence, and patient autonomy. There is striking a paucity of neuroimaging studies in PPS that could provide anatomical insights into the substrate of extra-motor symptoms. Ultimately, the characterization of PPS-associated pathology may help research efforts in other motor neuron diseases.

Outcome of Research: More research required

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Comments (if any): This is a good overview of Post-Polio conditions particularly for clinicians new to the topic.

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Category: Diagnosis and Management

Title: Inverse Relationship Between Polio Incidence in the US and Colorectal Cancer.
Author: STEVEN LEHRER and PETER H RHEINSTEIN
Affiliation: Department of Radiation Oncology, Icahn School of Medicine at Mount Sinai, New York, U.S.A. [email protected].
Severn Health Solutions, Severna Park, MD, U.S.A.
Journal: IN VIVO
Citation: (Athens, Greece) vol. 32,6 (2018): 1485-1489.
Publication Year and Month: 2018

Abstract: BACKGROUND/AIM:
Polio is predominantly an enteric viral infection that was progressively eradicated in the United States after the introduction of polio vaccine in the early 1950s. U.S. colorectal cancer rates have dropped steadily for individuals born between 1890 and 1950, but have been increasing for every generation born since 1950. Moreover, the lowest worldwide age adjusted rates of colorectal cancer in 2012 were in sub-Saharan Africa, Gambia and Mozambique, where polio has not been eradicated. In the current study, poliomyelitis incidence in US states before the introduction of polio vaccine was analyzed.

MATERIALS AND METHODS:
Reported cases of poliomyelitis per 100,000 population by state 1932-1951 were from Centers for Disease Control. Colorectal cancer deaths per 100,000 in men (2005-2009) by US State are from the American Cancer Society. US state overweight and obesity data are from the Centers for Disease Control and Prevention (CDC). Smoking data are from the CDC.

RESULTS:
By US state, colorectal cancer incidence per 100,000 in men for 2005-2009 was inversely correlated with reported cases of poliomyelitis per 100,000 for 1932-1951 (r=-0.311, p=0.032). Colorectal cancer deaths per 100,000 in men in 2005-2009 were also inversely correlated with reported cases of poliomyelitis per 100,000 by state for 1932-1951 (r=-0.493, p<0.001). The relationship between colorectal cancer deaths and polio incidence was significant (β=-0.196, p=0.028) and independent of the effects of smoking (β=0.289, p=0.012) and overweight (β=0.547, p<0.001). The relationship in females with colorectal cancer was identical.

Conclusions: Polio virus infection of cells of the colon may induce some degree of resistance to the development of colon cancer decades later. The effect of polio virus infection seems to be especially potent in reducing the rate of death from colon cancer.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Post-poliomyelitis syndrome (2005)
Author: Trojan DA, Cashman NR
Affiliation: Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, 3801 rue Université, Montréal, Québec H3A 2B4, Canada – [email protected]
Journal: Muscle & Nerve
Citation: Muscle Nerve. 2005 Jan;31(1):6-19
Publication Year and Month: 2005 01

Abstract: Post-poliomyelitis syndrome (PPS) is a common neurological disorder that occurs in a large proportion of individuals who have recovered from paralytic poliomyelitis. The main clinical features are new weakness, muscular fatigability, general fatigue, and pain. The primary criteria necessary for the diagnosis of PPS are a history of paralytic poliomyelitis, partial or complete recovery of neurological function followed by a period of stability (usually several decades), persistent new muscle weakness or abnormal muscle fatigability, and the exclusion of other causes of new symptoms. The cause of PPS remains unclear, but is likely due to a distal degeneration of enlarged post-poliomyelitis motor units. Contributing factors to PPS may be aging (with motor neuron loss), overuse, and disuse. PPS is usually a slowly progressive neuromuscular disease. Although there is no specific treatment for PPS, an interdisciplinary management program can be useful in controlling symptoms.

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Category: Diagnosis and Management

Title: Management of post-polio syndrome (1997)
Author: Trojan DA, Finch L
Affiliation: Not stated
Journal: NeuroRehabilitation
Citation: NeuroRehabil. 1997;8:93-105
Publication Year and Month: 1997 08

Abstract: The management of patients presenting with post-poliomyelitis syndrome is discussed. It is essential to identify and treat other medical and neurological conditions which could produce these symptoms. New weakness can be managed with exercise, avoidance of muscular overuse, weight loss, orthoses and assistive devices. Fatigue can be managed with energy conservation techniques. The management of pain is dependent upon its causes. Treatments are reviewed.

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Category: Diagnosis and Management

Title: Stroke Risk in Poliomyelitis Survivors: A Nationwide Population-Based Study
Author: Wu C-H, Liou T-H, Chen H-H, Sun T-Y, Chen K-H, Chang K-H
Affiliation: Wu, Sun, KH Chang: Chung-Yuan Christian University
Liou: Shuang Ho Hospital and Taipei Medical University, Taipei
HH Chen; Chang: Wan Fang Hospital
Chang: Taipei Medical University, Taipei
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Volume 93, Issue 12, Pages 2184–2188
Publication Year and Month: 2012 12

Abstract: Objectives
To assess the prevalence and risk of stroke among adults with polio and controls.

Design
A prospective, probability-sampling, 6-year population-based cohort study.

Setting
A National Health Insurance Research Database consisting of 316,355 randomly selected enrollees. The database is related to a National Health Insurance program with more than 22 million participants.

Participants
After excluding patients under 40 years of age, polio patients (N=212) (mean age ± SD, 54.0±10.2y; 57.1% men) were identified from the database from January 1, 2003 to December 31, 2008. For each polio patient, 2 age- and sex-matched patients were recruited as controls. Control patients did not have any neuromuscular diseases commonly found in childhood. The frequencies of patients with potential risk factors for stroke were assessed.

Intervention
None.

Main Outcome Measure
The prevalence and the adjusted odds ratio of ischemic stroke among polio patients and the controls were estimated.

Results
Polio patients had a higher prevalence of stroke (10.8% vs 2.4%, P<.001) than the controls. Polio patients with hypertension had a much higher prevalence of stroke (23.0%). The risk of stroke was higher for polio patients compared with the controls, yielding an adjusted odds ratio of 4.17 (95% confidence interval, 1.84–9.45, P<.001). Polio was a significant risk factor for stroke independent from hypertension, diabetes mellitus, hyperlipidemia, and cardiac diseases.

Conclusions
Adults with polio had a high prevalence of ischemic stroke. Polio was an additional risk factor for stroke. Polio patients with hypertension might potentiate the risk of stroke. Developing a health promotion program, suitable for polio patients, to increase participation in activities and exercises may be essential, especially for polio patients with hypertension.

Conclusions:

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Safety and feasibility of transcranial direct current stimulation for patients with post-polio syndrome
Author: Y. Matsushima, A. Hachisuka, H. Itoh, K. Sugimoto, S. Saeki
Affiliation: Department of rehabilitation medicine, University of occupational and environmental health, Japan
Journal: Brain Stimulation
Citation: (2019) 385-592
Publication Year and Month: 2019

Abstract: Post-polio syndrome (PPS) is generally defined as a clinical syndrome consisting of new muscle weakness, fatigue, and pain in poliomyelitis survivors. In PPS, there is no definitively validated treatment option, although Acler M et al. reported that anodal transcranial direct current stimulation (tDCS) over pre-motor cortex for 15 days improved sleep and fatigue symptoms in patients with PPS. tDCS may be a valuable, non-invasive new tool for managing patients with PPS.

Conclusions:

Outcome of Research: More research required

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There are currently 61 papers in this category.

Category: Diagnosis and Management

Title: Multiple sclerosis and poliomyelitis. A Danish historical cohort study.
Author: Nielsen NM, Wohlfahrt J, Melbye M, Rasmussen S, Mølbak K, Askgaard DS, Aaby P.
Affiliation: Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark
Journal: Acta Neurologica Scandinavica
Citation: Acta Neurol Scand 2000 Jun;101(6):384-7.
Publication Year and Month: 2000 06

Abstract: OBJECTIVE:
To evaluate whether persons with a history of poliomyelitis are at an increased risk of developing multiple sclerosis (MS).

MATERIAL AND METHODS:
All patients diagnosed with acute poliomyelitis in the greater capital area of Copenhagen, Denmark, between 1919 and 1954 were identified and followed with respect to MS. Information on vital status and diagnosis of sclerosis was obtained through linkage with the Danish Civil Registration System and The Danish Multiple Sclerosis Registry, respectively. Follow-up started on the date of the establishment of the Danish Civil Registration System (April 1, 1968) until death, emigration or December 31, 1996, whichever came first. The observed incidence of MS among polio patients was compared with the expected incidence calculated according to national gender, age and period specific rates of MS.

RESULTS:
During 149,364 years of follow-up, 19 cases of multiple sclerosis were observed among 5652 polio patients compared with 11.0 expected (SIR = 1.73 (1.04-2.74)). The increased risk of MS was most pronounced in polio patients hospitalized during adolescence. Neither gender nor the acute severity of poliomyelitis modified the risk of MS.

CONCLUSION:
Our results are based on small numbers of events, however the findings suggest that the polio patients might be at an increased risk of MS.

Conclusions:

Outcome of Research: More research required

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Comments (if any): There appears a higher incidence of Multiple Sclerosis in this study of Danish patients previously diagnosed with Poliomyelitis. This should not be interpreted that previously contracting Poliomyelitis will lead to Multiple Sclerosis.

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Category: Diagnosis and Management

Title: Epidemiology of the post-polio syndrome
Author: Ramlow J, Alexander M, LaPorte R, Kaufmann C, Kuller L
Affiliation: Department of Biostatistics, Graduate School of Public Health, University of Pittsburgh, PA
Journal: American Journal of Epidemiology
Citation: Am J Epidemiol. 1992 Oct 1;136(7):769-86
Publication Year and Month: 1992 10

Abstract: A late-onset syndrome, consisting of muscle weakness, muscle pain, and unaccustomed fatigue, has been reported with increasing frequency among former poliomyelitis patients. A population-based cohort of poliomyelitis patients from Allegheny County, Pennsylvania, was traced and surveyed to estimate the prevalence and incidence and to identify determinants of the post-polio syndrome. A questionnaire validated in clinical examinations of 40 cohort members was used in the survey. The prevalence of the post-polio syndrome was 28.5% of all paralytic cases (95% confidence interval 24.4-32.6). The risk of post-polio syndrome was significantly higher among patients who sustained substantial permanent impairment after polio and among females. The incidence did not vary with age at acute onset, acute severity, or level of physical activity after recovery. The strongest determinant of post-polio syndrome onset was the length of the interval following the acute illness, with incidence peaking at 30-34 years. Of all cases of post-polio syndrome, 79% reported no major change in impairment status since onset. This study demonstrates that poliomyelitis patients are not equally susceptible to post-polio syndrome within the interval of 30-40 years after the original illness. For syndrome cases, the onset was associated with new neuromuscular symptoms and functional changes but not with major new impairment.

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Category: Diagnosis and Management

Title: Nonparalytic polio and postpolio syndrome
Author: Halstead LS, Silver JK
Affiliation: National Rehabilitation Hospital, Washington, DC 20010, USA
Journal: American Journal of Physical Medicine & Rehabilitation
Citation: Am J Phys Med Rehabil. 2000 Jan-Feb;79(1):13-8
Publication Year and Month: 2000 01

Abstract: We describe four cases of postpolio syndrome with typical histories, physical examination results, and electrodiagnostic evidence of extensive anterior horn cell disease, as well as the putative pathophysiology of postpolio syndrome in persons with histories of nonparalytic polio and the diagnostic implications for individuals older than 40 yr of age who are experiencing unexplained new weakness, fatigue, and muscle or joint pain. Although the diagnosis of postpolio syndrome traditionally has required a remote history of paralytic polio, many persons such as the ones described here with typical symptoms of postpolio syndrome have no clear history of paralytic disease and are being misdiagnosed. With this in mind, we believe that the diagnostic criteria for postpolio syndrome should be modified to include the following: a history of remote paralytic polio or findings on history, physical examination results, and laboratory studies compatible with poliovirus damage of the central nervous system earlier in life.

Conclusions:

Outcome of Research: Not applicable

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Comments (if any): The full paper is available from Polio Australia for private study purposes.

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Category: Diagnosis and Management

Title: Paralytic vs "nonparalytic" polio: distinction without a difference?
Author: Bruno RL
Affiliation: The Post-Polio Institute, Englewood Hospital and Medical Center, New Jersey, USA
Journal: American Journal of Physical Medicine & Rehabilitation
Citation: Am J Phys Med Rehabil. 2000 Jan-Feb;79(1):4-12
Publication Year and Month: 2000 01

Abstract: Nonparalytic polio (NPP) is commonly thought to be synonymous with "abortive polio," in which the poliovirus neither entered the central nervous system nor damaged neurons. Described are two epidemic illness-"The Summer Grippe" and Iceland disease-apparently caused by a low virulence but neuropathic type 2 poliovirus. Studies show that neuronal lesions in the brain and spinal cord and muscle weakness were common in NPP, and epidemiologic studies document late-onset weakness and fatigue in 14% to 42% of NPP survivors. These findings indicate that clinicians should not require a history of paralytic polio, electromyographic evidence of denervation, and new muscle weakness for the diagnosis of "Postpolio Syndrome" but should be aware that NPP, and possibly even poliovirus-induced "minor illnesses," can be associated with acute central nervous system damage and late-onset muscle weakness and fatigue.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: The post-polio syndrome as an evolved clinical entity. Definition and clinical description.
Author: Dalakas MC
Affiliation: Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
Journal: Annals of the New York Academy of Sciences
Citation: Ann N Y Acad Sci. 1995 May 25;753:68-80
Publication Year and Month: 1995 05

Abstract: Post-polio syndrome (PPS) refers to the new neuromuscular symptoms that occur at least 15 years after stability in patients with prior acute paralytic polio-myelitis. They include: (1) new muscle weakness and atrophy in the limbs, the bulbar or the respiratory muscles [post-poliomyelitis muscular atrophy (PPMA)] and (2) excessive muscle fatigue and diminished physical endurance. PPS is a clinical diagnosis that requires exclusion of all other medical, neurological, orthopedic or psychiatric diseases that could explain the cause of the new symptoms. Routine electromyography is useful to confirm chronic and ongoing denervation and exclude neuropathies. Muscle biopsy, single fiber electromyography (EMG), macro-EMG, serum antibody titers to polio virus, and spinal fluid studies are very useful research tools but they are rarely needed to establish the clinical diagnosis. PPS is a slowly progressive phenomenon with periods of stability that vary from 3 to 10 years. Current evidence indicates that PPS is the evolution of a subclinically ongoing motor neuron dysfunction that begins after the time of the acute polio. It is clinically manifested as PPS when the well-compensated reinnervating process crosses a critical threshold beyond which the remaining motor neurons cannot maintain the innervation to all the muscle fibers within their motor unit territory.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: National Rehabilitation Hospital Limb Classification for Exercise, Research, and Clinical Trials in Post-Polio Patients
Author: Lauro S. Halstead, Anne Carrington Gawne, and Bao T. Pham

Affiliation: The Post-Polio Program; National Rehabilitation Hospital, Washington, DC


Journal: Annals of the New York Academy of Sciences
Citation: The Post-Polio Syndrome: Advances in the Pathogenesis and Treatment Volume 753 pp 343-353 of the Annals of the New York Academy of Sciences May 25, 1995.
Publication Year and Month: 1995 05

Abstract:

Conclusions: A need exists for an objective classification of polio patients for clinical and research purposes that takes into account the focal, asymmetric, and frequent subclinical nature of polio lesions. In order to prescribe a safe, effective exercise program, we developed a five-level (Classes I-V) limb-specific classification system based on remote and recent history, physical examination, and a four-extremity electrodiagnostic study (EMG/NCS). Class I limbs have no history of remote or recent weakness, normal strength, and a normal EMG. Class II limbs have no history of remote or recent weakness (or if remote history of weakness, full recovery occurred), normal strength and EMG evidence of prior anterior horn cell disease (AHCD). Class III limbs have a history of remote weakness with variable recovery, no new weakness, decreased strength, and EMG evidence of prior AHCD. Class IV limbs have a history of remote weakness with variable recovery, new clinical weakness, decreased strength, and EMG evidence of AHCD. Class V limbs have a history of severe weakness with little-to-no recovery, severely decreased strength and atrophy, and few-to-no motor units on EMG. In a prospective study of 400 limbs in 100 consecutive post-polio patients attending our clinic, 94 (23%) limbs were Class I, 88 (22%) were Class II, 95 (24%) were Class III, 75 (19%) were Class IV, and 48 (12%) were Class V. Guidelines for the use of this classification in a clinical/research setting are presented along with sample case histories and class-specific exercise recommendations.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Pathogenetic mechanisms of post-polio syndrome: morphological, electrophysiological, virological, and immunological correlations.
Author: Dalakas MC
Affiliation: Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1382, USA.
Journal: Annals of the New York Academy of Sciences
Citation: 1995 May 25;753:167-85.
Publication Year and Month: 1995 05

Abstract: To understand the mechanism of post-poliomyelitis muscular atrophy (PPMA) and the post-polio syndrome (PPS) in general, we performed the following studies: (1) histopathology in spinal cord sections from patients who died 9 days to 44 years after acute paralytic poliomyelitis; (2) enzyme histochemistry, immunocytochemistry (for lymphocyte subsets, MHC antigens and N-CAM) and polymerase chain reaction (PCR) for poliovirus RNA in the muscle biopsies from symptomatic or asymptomatic muscles of post-polio patients; (3) determination of lymphocyte subsets and circulating IgG or IgM antibodies against GM1 and poliovirus; (4) virological studies in the spinal fluid for oligoclonal bands and search for poliovirus genome with PCR; (5) electrophysiological studies including single fiber EMG, fiber density and macro-EMG; and (6) [31P] exercise MRS spectroscopy on previously affected muscles to search for a metabolic correlate of fatigue. These studies concluded that in PPS a continuing dysfunction is present in the spinal cord motor neurons, resulting in ongoing muscle denervation and reinnervation first evident at the axonal branch points. Symptoms are related to attrition of the oversprouting motor neurons which after a period of time cannot support all their axonal sprouts, resulting in failure of re-reinnervation. In some patients with PPS there is also an ongoing immune activation and presence of defective viral particles in the spinal fluid. However, their role in the pathogenesis of PPS is presently unknown.

Conclusions: These studies concluded that in PPS a continuing dysfunction is present in the spinal cord motor neurons, resulting in ongoing muscle denervation and reinnervation first evident at the axonal branch points.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Estimation of the Direct Cost of Poliomyelitis Rehabilitation Treatment to Pakistani Patients: A 53-Year Retrospective Study
Author: Atta Abbas Naqvi, Syed Baqir Shyum Naqvi, Fatima Zehra, Ashutosh Kumar Verma, Saman Usmani, Sehrish Badar, Rizwan Ahmad, Niyaz Ahmad
Affiliation: 1.Department of Pharmacy Practice, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
2.Faculty of PharmacyHamdard UniversityKarachiPakistan
3.Applied Economics Research CentreUniversity of KarachiKarachiPakistan
4.Discipline of Social and Administrative Pharmacy, School of Pharmaceutical SciencesUniversiti Sains MalaysiaMindenMalaysia
5.Institute of Pharmaceutical Sciences, Jinnah Sindh Medical UniversityKarachiPakistan
6.Natural Products and Alternative Medicines, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
7.Department of Pharmaceutics, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
Journal: Applied Health Economics and Health Policy
Citation: December 2018, Volume 16, Issue 6, pp 871–888
Publication Year and Month: 2018 12

Abstract: Background
Pakistan is one of the last few countries in which poliomyelitis is endemic. Evidence indicates that out-of-pocket expenditures are a barrier to polio rehabilitation treatment, yet there are no reported figures related to the financial burden of this disease on patients in a recently polio-endemic country.

Objective
This study investigated direct costs attributed to rehabilitation treatment of poliomyelitis among Pakistani patients and reported its duration along with the socioeconomic status of poliomyelitis survivors.

Conclusions: Conclusion
The cost of poliomyelitis rehabilitation in Pakistan is high; it has an economic effect on the lives of patients and their families. Despite good education, polio survivors in Pakistan appear to have low socioeconomic status, lower chances of employment and marriage, as well as fewer children. Further research is recommended to explore the burden of disease on society, i.e., indirect costs and suffering.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Postpolio syndrome
Author: Nollet F, de Visser M
Affiliation: Department of Rehabilitation, Academic Medical Center, University of Amsterdam, PO Box 22660, 1100 DD Amsterdam, the Netherlands – [email protected]
Journal: Archives of Neurology
Citation: Arch Neurol. 2004 Jul;61(7):1142-4
Publication Year and Month: 2004 07

Abstract: This paper has no abstract - this is an extract:

Postpolio syndrome (PPS) refers to a decline of muscle function usually occurring 30 to 40 years after the acute polio episode. This syndrome has been widely recognized only during the last decades, when many people affected by the large epidemics of the previous century experienced new muscle weakness as they grew older. However, cases of late-onset weakening following poliomyelitis were already reported at the end of the 19th century.

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Category: Diagnosis and Management

Title: Cardiac risk factors in polio survivors
Author: Gawne AC, Wells KR, Wilson KS.
Affiliation: Roosevelt Warm Springs Institute for Rehabilitation, Warm Springs, GA 31830, USA - [email protected]
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 2003 May;84(5):694-6
Publication Year and Month: 2003 05

Abstract: OBJECTIVE: To assess the prevalence of dyslipidemia and other risk factors for coronary heart disease in a sample of polio survivors with and without postpoliomyelitis syndrome.

DESIGN: Retrospective chart review.

SETTING: A multidisciplinary outpatient postpolio clinic.

PARTICIPANTS: Eighty-eight consecutive symptomatic postpolio patients, 50 women (mean age, 59.0y; range, 36-81y) and 38 men (mean age, 61.2y; range, 44-83y).

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Presence of risk factors for coronary heart disease: clinical atherosclerotic disease, male age >or=45 years or female age >or=55 years, history of hypertension (blood pressure >or=140/90mmHg or on antihypertensive medication), diabetes mellitus, cigarette smoking, and high-density lipoprotein (HDL) less than 35mg/dL. Obesity (body mass index [BMI], >25kg/m(2)) was assessed as an intervention target. Laboratory values included fasting total cholesterol, HDL, low-density lipoprotein, triglycerides, and glucose.

RESULTS: Of the total sample, 61.3% had dyslipidemia. Average HDL cholesterol ratio was 4.01 (women, 3.68; men, 4.55). Forty-four patients (50%) had a history of hypertension or had elevated blood pressure. Seven patients (8%) had a history of diabetes or had elevated fasting blood glucose (>110). Eighteen patients (20.4%) were smokers or had a history of smoking; 9 continued to smoke and 9 had quit smoking. Twenty-five patients (28.4%) were overweight (BMI, >25kg/m(2)). Forty-one patients (46.5%) had more than 1 risk factor for coronary heart disease. Nine of the total sample (10.2%) had a history of heart disease ranging from atrial fibrillation to angina. Only 19 patients had a previous diagnosis of dyslipidemia and only 12 were on a lipid-lowering medication.

Conclusions: Polio patients have a high prevalence of dyslipidemia. The study sample supports the National Cholesterol Education Program's Adult Treatment Panel III statements that hypercholesterolemia is underdiagnosed and undertreated. The postpolio population carries a high prevalence of 2 or more coronary heart disease risk factors. Evaluation and rehabilitation of polio patients should include screening for dyslipidemia and education about elimination of controllable risk factors.

Outcome of Research: Not applicable.

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Category: Diagnosis and Management

Title: Predictive Factors for Post-Poliomyelitis Syndrome
Author: Daria A. Trojan, MD, MSc, Neil R. Cashman, MD, Stanley Shapiro, PhD, Catherine M. Tansey, MSc, John M. Esdaile, MD
Affiliation: From the Department of Neurology (Drs. Trojan, Cashman), Montreal Neurological Institute and Hospital, the Department of Medicine (Dr. Esdaile), Montreal General Hospital, and the Department of Epidemiology and Biostatistics (Dr. Shapiro, Ms. Tansey), McGill University, Montreal, Quebec, Canada.
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil Vol 75, July 1994, 770-777
Publication Year and Month: 1994 07

Abstract: Post-poliomyelitis syndrome (PPS) is generally defined as a clinical syndrome of new weakness, fatigue, and pain in individuals who have previously recovered from acute paralytic poliomyelitis. The purpose of this study was to identify, through a case-control study design, factors that predict subsequent PPS in patients with prior paralytic poliomyelitis. Among patients attending a university-affiliate hospital post-polio clinic, "cases" were patients with new weakness and fatigue, and "controls" were patients without these complaints. A chart review of 353 patients identified 127 cases and 39 controls. Logistic regression modeling was used to calculate adjusted and unadjusted odds ratios. In univariate analyses, significant risk factors for PPS were a greater age at time of presentation to clinic (p = 0.01), a longer time since acute polio (p = 0.01), and more weakness at acute polio (p = 0.02). Other significant associated, but not necessarily causal factors were a recent weight gain (p = 0.005), muscle pain (p = 0.01) particularly that associated with exercise (p = 0.005), and joint pain (p = 0.04). Multivariate analyses revealed that a model containing age at presentation to clinic, severity of weakness at acute polio, muscle pain with exercise, recent weight gain, and joint pain best distinguished cases from controls. Age at acute polio, degree of recovery after polio, weakness at best point after polio, physical activity, and sex were not contributing factors. These findings suggest that the degree of initial motor unit involvement as measured by weakness at acute polio, and possibly the aging process and overuse are important in predicting PPS.

Conclusions: In conclusion, the results from this study provide insight on predictive factors for PPS, and can be applied in the clinical management of patients who have recovered from paralytic poliomyelitis. Our findings support the hypothesis that the severity of initial motor unit involvement as estimated by weakness at acute polio, and possibly the normal ageing process and overuse are important in predicting PPS. Even though patients have no control over the severity of weakness as a result of acute polio, they do have control over some predictive factors for PPS. Patients can be advised that they should avoid gaining weight and exercising to the point of muscle pain because these variables have been found to be strongly associated with PPS. The exact role of physical activity will still need further evaluation; however, the usual recommendations of low-level aerobic exercise with avoidance of muscle pain and fatigue appear valid. Thus, this study can provide the basis for physiologically reasonable and practical advice to post-polio patients to minimize or delay the risk of PPS.

Outcome of Research: Not applicable

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Comments (if any): Paul Cavendish (Clinical Health Educator): The best multivariate model for predicting who will develop PPS indicates that patients who had a greater weakness at acute polio, are currently older, have muscle pain with exercise, a recent weight gain, and joint pain are those most likely to develop PPS. Other factors shown to be important in univariate analyses are a longer time since acute polio, and muscle pain (at rest or with exercise). Age at acute polio, recovery after polio, weakness at "best point" after polio, physical activity, and sex were not contributing factors.

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Category: Diagnosis and Management

Title: Life-Long Morbidity Among Danes With Poliomyelitis
Author: Nielsen NM, Rostgaard K, Askgaard D, Skinhøj, & P Aaby P.
Affiliation: Department of Epidemiology Research, Danish Epidemiology Science
Centre, Statens Serum Institut (Nielsen, Rostgaard, Aaby) and Department of Infectious Diseases M, National University Hospital, (Askgaard, Skinhøj), Copenhagen,
Denmark.
Supported by the Danish Medical Research Council, the AP Møller and Chastine
McKinney Møller Foundation, the Danish National Research Foundation, the WedellWedellsborg Foundation, and The National Polio Society
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil 2004; 85:385-91 - doi:10.1016/S0003-9993(03)00474-X
Publication Year and Month: 2004 03

Abstract: Abstract
OBJECTIVE:
To estimate long-term morbidity in a cohort of Danish poliomyelitis patients.

DESIGN:
A historical prospective cohort study of 27,047 persons.

SETTING:
Denmark.

PARTICIPANTS:
A total of 5421 persons hospitalized for poliomyelitis between 1919 to 1954 in Copenhagen, Denmark, and 21,626 age- and gender-matched Danes. Participants were followed up on average for 20.6 years, yielding a total of 555,884 person-years of follow-up.

INTERVENTIONS:
Not applicable.

MAIN OUTCOME MEASURES:
The exposed (poliomyelitis) cohort and the unexposed (control) cohort were followed up for somatic hospitalization from 1977 to 1999 in the Danish Hospital Discharge Register. The incidence rate ratio (IRR) was calculated as the ratio between the incidence rate of disease in the exposed and unexposed cohorts.

RESULTS:
Overall, polio patients had a 1.2- to 1.3-fold increased risk of being hospitalized with pulmonary diseases, heart diseases, gastrointestinal disorders, or diseases of the locomotive apparatus. Among paralytic polio patients, long-term morbidity seems to be associated with the acute severity of poliomyelitis, as well as young age at infection. Paralytic patients, who contracted respiratory polio under the age of 5, had the highest risk of being hospitalized with lung diseases (IRR=7.26; 95% confidence interval [CI], 3.06-18.33), diseases of the locomotive apparatus (IRR=4.05; 95% CI, 1.66-9.86), heart diseases (IRR=1.70; 95% CI, 0.65-3.98), and diseases of the digestive system (IRR= 2.23; 95% CI, 1.03-4.62). Surprisingly, patients without paralyses, especially women, also had an increased morbidity.

CONCLUSIONS:
Overall, a history of poliomyelitis was associated with a slightly increased morbidity measured by hospitalizations. Long-term morbidity was highest among respiratory polio patients; however, patients presumably left without any residual symptoms also had an increased morbidity.

Conclusions: Overall, a history of poliomyelitis was associated with a slightly increased morbidity measured by hospitalizations. Long-term morbidity was highest among respiratory polio patients; however, patients presumably left without any residual symptoms also had an increased morbidity.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Stroke Risk in Poliomyelitis Survivors: A Nationwide Population-Based Study
Author: Wu C-H, Liou T-H, Chen H-H, Sun T-Y, Chen K-H, Chang K-H
Affiliation: Wu, Sun, KH Chang: Chung-Yuan Christian University
Liou: Shuang Ho Hospital and Taipei Medical University, Taipei
HH Chen; Chang: Wan Fang Hospital
Chang: Taipei Medical University, Taipei
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Volume 93, Issue 12, Pages 2184–2188
Publication Year and Month: 2012 12

Abstract: Objectives
To assess the prevalence and risk of stroke among adults with polio and controls.

Design
A prospective, probability-sampling, 6-year population-based cohort study.

Setting
A National Health Insurance Research Database consisting of 316,355 randomly selected enrollees. The database is related to a National Health Insurance program with more than 22 million participants.

Participants
After excluding patients under 40 years of age, polio patients (N=212) (mean age ± SD, 54.0±10.2y; 57.1% men) were identified from the database from January 1, 2003 to December 31, 2008. For each polio patient, 2 age- and sex-matched patients were recruited as controls. Control patients did not have any neuromuscular diseases commonly found in childhood. The frequencies of patients with potential risk factors for stroke were assessed.

Intervention
None.

Main Outcome Measure
The prevalence and the adjusted odds ratio of ischemic stroke among polio patients and the controls were estimated.

Results
Polio patients had a higher prevalence of stroke (10.8% vs 2.4%, P<.001) than the controls. Polio patients with hypertension had a much higher prevalence of stroke (23.0%). The risk of stroke was higher for polio patients compared with the controls, yielding an adjusted odds ratio of 4.17 (95% confidence interval, 1.84–9.45, P<.001). Polio was a significant risk factor for stroke independent from hypertension, diabetes mellitus, hyperlipidemia, and cardiac diseases.

Conclusions
Adults with polio had a high prevalence of ischemic stroke. Polio was an additional risk factor for stroke. Polio patients with hypertension might potentiate the risk of stroke. Developing a health promotion program, suitable for polio patients, to increase participation in activities and exercises may be essential, especially for polio patients with hypertension.

Conclusions:

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Frequency and clinical manifestations of post-poliomyelitis syndrome in a Brazilian tertiary care center
Author: Quadros AA (1), Conde MT, Marin LF, Silva HC, Silva TM, Paula MB, Pereira RD, Ramos PE, Abe G, Oliveira AS
Affiliation: (1) Department of Neurology and Neurosurgery, Division of Neuromuscular Disorders, Federal University of São Paulo (Unifesp), São Paulo SP, Brazil - [email protected]
Journal: Arquivos de Neuro-psiquiatria
Citation: Arq Neuropsiquiatr. 2012 Aug;70(8):571-3
Publication Year and Month: 2012 08

Abstract: OBJECTIVE: To determine the frequency and clinical manifestations of patients with post-poliomyelitis syndrome (PPS) in a Brazilian division of neuromuscular disorders.

METHODS: A total of 167 patients with prior history of paralytic poliomyelitis was investigated for PPS, based on international diagnostic criteria. Other variables analyzed were: gender, race, age at poliomyelitis infection, age at PPS onset, and PPS symptoms.

RESULTS: One hundred and twenty-nine patients presented PPS, corresponding to 77.2% of the studied population. 62.8% were women and 37.2% were men. Mean age of patients with PPS at onset of PPS symptoms was 39.9±9.69 years. Their main clinical manifestations were: new weakness in the previously affected limbs (69%) and in the apparently not affected limbs (31%); joint pain (79.8%); fatigue (77.5%); muscle pain (76%); and cold intolerance (69.8%).

Conclusions: Most patients of our sample presented PPS. In Brazil, PPS frequency and clinical features are quite similar to those of other countries.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Physiotherapy for poliomyelitis: a descriptive study in the Republic of Congo
Author: Mancini S, Coldiron ME, Nicholas S, Llosa AE, Mouniaman-Nara I, Ngala J, Grais RF, Porten K
Affiliation: Epicentre, Paris, France - [email protected].
Journal: BioMedCentral Research Notes
Citation: BMC Res Notes. 2014 Oct 23;7:755. doi: 10.1186/1756-0500-7-755
Publication Year and Month: 2014 10

Abstract: BACKGROUND: A large poliomyelitis outbreak occurred in 2010 in the Republic of Congo. This paper describes the demographic and clinical characteristics of poliomyelitis cases and their outcomes following physiotherapy.

FINDINGS: Demographic and clinical data were collected on 126 individuals between November 23, 2010 and March 23, 2011. The male/female ratio was 2.5 and the median age was 19 years (IQR: 13.5-23). The most severe forms of the disease were more common in older patients, 81 of the 126 patients (64.3%) had multiple evaluations of muscle strength. Among patients with multiple evaluations, 38.1% had improved strength at final evaluation, 48.3% were stable and 13.6% had decreased strength.

Conclusions: Most acute poliomyelitis patients receiving physiotherapy had improved or stable muscle strength at their final evaluation. These descriptive results highlight the need for further research into the potential benefits of physiotherapy in polio affected patients.

Outcome of Research: More research required.

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Category: Diagnosis and Management

Title: Safety and feasibility of transcranial direct current stimulation for patients with post-polio syndrome
Author: Y. Matsushima, A. Hachisuka, H. Itoh, K. Sugimoto, S. Saeki
Affiliation: Department of rehabilitation medicine, University of occupational and environmental health, Japan
Journal: Brain Stimulation
Citation: (2019) 385-592
Publication Year and Month: 2019

Abstract: Post-polio syndrome (PPS) is generally defined as a clinical syndrome consisting of new muscle weakness, fatigue, and pain in poliomyelitis survivors. In PPS, there is no definitively validated treatment option, although Acler M et al. reported that anodal transcranial direct current stimulation (tDCS) over pre-motor cortex for 15 days improved sleep and fatigue symptoms in patients with PPS. tDCS may be a valuable, non-invasive new tool for managing patients with PPS.

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: The split hand syndrome in ALS and post-polio-syndrome
Author: M.Hoheisel, L.Burmeister, M.Tesch
Affiliation: Schlosspark-Klinik Berlin, Neurology, Berlin, Germany
Journal: Clinical Neurophysiology
Citation: Volume 129, Issue 8, August 2018, Pages e82-e83

Publication Year and Month: 2018 08

Abstract: Introduction
Electrodiagnostic evaluation for amyotrophic lateral sclerosis (ALS) relies on extensive measurements. As one diagnostic clue, the split-hand-index (SHI) was proposed. It compares the compound muscle action potential (CMAP) of the abductor pollicis brevis (APB) muscle with the CMAP of the abductor digiti minimi (ADM) muscle.

In ALS, asymmetric atrophy of APB and ADM results in the index being reduced compared to the healthy population. This holds true despite the fact, that there is the same segmental innervation C8 for both examined muscles, as was previously discussed. Several studies have shown a diagnostic value in differentiating ALS from other motorneuron-diseases by means of the SHI, claiming a specific form of neurodegeneration in ALS, which is less marked for example in lower motor neuron disease (LMND), spinal muscle atrophy (SMA) or Hirayama disease.

In our study, we aimed to compare the SHI of ALS-patients with our cohort of patients with post-polio-syndrome (PPS) to find out, whether it has a discriminative value in these patients too and to add knowledge to the proposed neuroscientific explanations of asymmetric thenar/hypothenar-atrophy.

Methods
We conducted a retrospective analysis of our post-polio cohort since 1997. All patients were screened whether CMAPs of APB and ADM were collected. For comparison, we screened electrodiagnostic reports of all patients with a diagnosis of ALS for collected CMAPs of APB and ADM. We excluded patients with neuropathy of the median nerve (NMN) by means of a prolonged distal motoric latency (>4,4 ms). Finally we randomly chose the same number of patients from our reports in that time period with normal results (NR), by excluding diagnosis of NMN, acute polyneuropathies and radiculo- and plexopathies of the arm. We calculated the SHI by dividing the CMAP of APB by the CMAP of ADM.

Results
We found a significant difference (p = 0,01) of the SHI between ALS patients (0,97 ± 0,84) and the NR-group (1,26 ± 0,72). The SHI of the PPS-group (0,91 ± 0,55) was not significantly different compared to the NR group (p = 0,08), but showed a trend. Comparing the ALS-group with the PPS-patients, we found no statistically relevant difference (p = 0,83).

Conclusions: As expected, we could reproduce a significant decrease of the SHI in ALS patients. However our results showed no statistically relevant difference between ALS-patients and PPS-patients when comparing the SHI. There is a broader distribution of values in the PPS-group, including cases of very high grade asymmetric atrophy of APB and ADM. A low SHI therefore is of no help in differentiating PPS from ALS-patients.

As a limitation of our study it is important to acknowledge the retrospective study type and a possible selection bias of patients who suffer from an already clinically visible asymmetry, possible increasing electrodiagnostic evaluation numbers of the hand.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: The treatment of fatigue by non-invasive brain stimulation.
Author: Lefaucheur JP, Chalah MA, Mhalla A, Palm U, Ayache SS, Mylius V.
Affiliation: Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Service de physiologie-Explorations fonctionnelles, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, Créteil, France. Electronic address: [email protected].

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Service de physiologie-Explorations fonctionnelles, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, Créteil, France.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Department of Psychiatry and Psychotherapy, Klinikum der Universität München, Munich, Germany.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Department of Neurology, Philipps University, Marburg, Germany; Department of Neurology, Center for Neurorehabilitation, Valens, Switzerland.
Journal: Clinical Neurophysiology
Citation: 2017 Apr;47(2):173-184.
Publication Year and Month: 2017 04

Abstract: The use of non-invasive brain neurostimulation (NIBS) techniques to treat neurological or psychiatric diseases is currently under development. Fatigue is a commonly observed symptom in the field of potentially treatable pathologies by NIBS, yet very little data has been published regarding its treatment. We conducted a review of the literature until the end of February 2017 to analyze all the studies that reported a clinical assessment of the effects of NIBS techniques on fatigue. We have limited our analysis to repetitive transcranial magnetic stimulation (rTMS) and transcranial direct current stimulation (tDCS). We found only 15 studies on this subject, including 8 tDCS studies and 7 rTMS studies. Of the tDCS studies, 6 concerned patients with multiple sclerosis while 6 rTMS studies concerned fibromyalgia or chronic fatigue syndrome. The remaining 3 studies included patients with post-polio syndrome, Parkinson's disease and amyotrophic lateral sclerosis. Three cortical regions were targeted: the primary sensorimotor cortex, the dorsolateral prefrontal cortex and the posterior parietal cortex. In all cases, tDCS protocols were performed according to a bipolar montage with the anode over the cortical target. On the other hand, rTMS protocols consisted of either high-frequency phasic stimulation or low-frequency tonic stimulation. The results available to date are still too few, partial and heterogeneous as to the methods applied, the clinical profile of the patients and the variables studied (different fatigue scores) in order to draw any conclusion. However, the effects obtained, especially in multiple sclerosis and fibromyalgia, are really carriers of therapeutic hope.

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Treatment for postpolio syndrome
Author: Koopman FS, Beelen A, Gilhus NE, de Visser M, Nollet F
Affiliation: Department of Rehabilitation, University of Amsterdam Academic Medical Center, PO Box 22660, Amsterdam, North Holland, Netherlands, 1100 DD
Journal: Cochrane Database of Systematic Reviews
Citation: Cochrane Database Syst Rev. 2015 May 18;5:CD007818
Publication Year and Month: 2015 05

Abstract: BACKGROUND: Postpolio syndrome (PPS) may affect survivors of paralytic poliomyelitis and is characterised by a complex of neuromuscular symptoms leading to a decline in physical functioning. The effectiveness of pharmacological treatment and rehabilitation management in PPS is not yet established. This is an update of a review first published in 2011.

OBJECTIVES: To systematically review the evidence from randomised and quasi-randomised controlled trials for the effect of any pharmacological or non-pharmacological treatment for PPS compared to placebo, usual care or no treatment.

SEARCH METHODS: We searched the following databases on 21 July 2014: Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, PsycINFO and CINAHL Plus. We also checked reference lists of all relevant articles, searched the Database of Abstracts of Reviews of Effects (DARE), the Health Technology Assessment (HTA) Database and trial registers and contacted investigators known to be involved in research in this area.

SELECTION CRITERIA: Randomised and quasi-randomised trials of any form of pharmacological or non-pharmacological treatment for people with PPS. The primary outcome was self perceived activity limitations and secondary outcomes were muscle strength, muscle endurance, fatigue, pain and adverse events.

DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by The Cochrane Collaboration.

MAIN RESULTS: We included 10 pharmacological (modafinil, intravenous immunoglobulin (IVIg), pyridostigmine, lamotrigine, amantadine, prednisone) and three non-pharmacological (muscle strengthening, rehabilitation in a warm climate (that is temperature ± 25°C, dry and sunny) and a cold climate (that is temperature ± 0°C, rainy or snowy), static magnetic fields) studies with a total of 675 participants with PPS in this review. None of the included studies were completely free from any risk of bias, the most prevalent risk of bias being lack of blinding.There was moderate- and low-quality evidence that IVIg has no beneficial effect on activity limitations in the short term and long term, respectively, and inconsistency in the evidence for effectiveness on muscle strength. IVIg caused minor adverse events in a substantial proportion of the participants. Results of one trial provided very low-quality evidence that lamotrigine might be effective in reducing pain and fatigue, resulting in fewer activity limitations without generating adverse events. Data from two single trials suggested that muscle strengthening of thumb muscles (very low-quality evidence) and static magnetic fields (moderate-quality evidence) are safe and beneficial for improving muscle strength and pain, respectively, with unknown effects on activity limitations. Finally, there was evidence varying from very low quality to high quality that modafinil, pyridostigmine, amantadine, prednisone and rehabilitation in a warm or cold climate are not beneficial in PPS.

Conclusions: Due to insufficient good-quality data and lack of randomised studies, it was impossible to draw definite conclusions about the effectiveness of interventions for PPS. Results indicated that IVIg, lamotrigine, muscle strengthening exercises and static magnetic fields may be beneficial but need further investigation to clarify whether any real and meaningful effect exists.

Outcome of Research: More research required.

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Category: Diagnosis and Management

Title: Identification of targets for improving access to care in persons with long term physical disabilities
Author: Jennifer L.Wong, Kevin N.Alschuler, Tracy M.Mroz, Kimberly P.Hreha, Ivan R.Molton
Affiliation: University of Washington, Rehabilitation Medicine, United States
Journal: Disability and Health Journal
Citation: Available online 20 January 2019
Publication Year and Month: 2019 01

Abstract: Background
People with long-term physical disability (LTPD) continue to experience difficulties in accessing health care despite the focus of highlighting disparities in the last two decades.

Objectives
To describe health care utilization, accommodations and barriers experienced while accessing health care, and reasons why individuals delay or skip health care among people with LTPD.

Methods
The current study was a part of a larger longitudinal survey administered to individuals with physical disability associated with one of four long-term conditions (MS; SCI; PPS; MD). Measures included demographics, health care utilization, barriers to health care, and reasons for delaying or skipping medical care from the sixth wave of data from 2015 to 2016.

Results
Roughly 90% of all participants (N = 1159) saw at least one medical provider within 12 months. The most encountered barrier participants reported experiencing within that time was an office that did not have a safe transfer device to move them to an exam table (69%). Participants’ physical function, quality of life, status of living with a spouse, diagnostic condition, and sex (male) were significantly associated with endorsing a barrier in accessing health care. The inability to afford out of pocket expenses was the highest reported reason for delaying health care.

Conclusions: People with LTPD access a variety of health care, including rehabilitation services, and continue to experience barriers when doing so. While understanding barriers individuals experience when accessing health care is important, it is equally important to document the type of care they delay or skip due to barriers.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Polio survivors perceptions of a multi-disciplinary rehabilitation programme
Author: Atwal, A., Duncan, H., Queally, C., Cedar, S.H.
Affiliation: Nil
Journal: Disability and Rehabilitation
Citation: Atwal, A., Duncan, H., Queally, C., Cedar, S.H. (2017) Polio survivors perceptions of a multi-disciplinary rehabilitation programme. Disability and Rehabilitation. DOI: 10.1080/09638288.2017.1381184
Publication Year and Month: 2017 10

Abstract: Purpose: Post-polio syndrome refers to a late complication of the poliovirus infection. Management of post-polio syndrome is complex due to the extensive symptomology. European and United Kingdom guidelines have advised the use of rehabilitation programmes to manage post-polio syndrome. There is a paucity of research in relation to the effectiveness of rehabilitation interventions. The objective of this study is to explore polio survivor’s perceptions of an in-patient multi-disciplinary rehabilitation programme.

Methods: Semi-structured interviews of community dwelling polio survivors who attended in-patient rehabilitation programme in the United Kingdom. Thematic analysis was used to describe and interpret interview data.

Results: Participants’ experiences were influenced by past experiences of polio and their self-concept. Participants generally had a positive experience and valued being with other polio survivors. Positive strategies, such as pacing and reflection changed their mind-sets into their lives after the programme, though they still faced challenges in daily living. Some participants supported others with post-polio syndrome after completing the programme.

Conclusions: The research identified that participants experienced long term positive benefits from attending a rehabilitation programme. Strategies that users found helpful that explored the effectiveness of interventions to manage polio are not cited within a Cochrane review. If we are to recognise the lived experience and service user empowerment within a model of co- production it is essential that patient preferences are evaluated and used as evidence to justify service provision. Further research is required with polio survivors to explore how best rehabilitation programmes can adopt the principles of co-production.

Implications for Rehabilitation
The patients’ expertise and lived experience must be at the centre of a rehabilitation programme.

Strategies such as pacing and reflection are perceived as important strategies to enable self-management of polio and post-polio syndrome despite the limited evidence base to support these interventions.

Polio rehabilitation programmes should not be time limited and commissioners and therapists need to ensure that follow up support is provided.

When measuring outcomes patient preferences and views must be evaluated.

Outcome of Research: More research required

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Comments (if any): This investigation supports feedback from polio survivors in Australia on the benefits of group-based rehabilitation, especially where there is an opportunity to learn and discuss their rehabilitation with fellow participants.

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Category: Diagnosis and Management

Title: EFNS guideline on diagnosis and management of post-polio syndrome - report of an EFNS task force
Author: Farbu E, Gilhus NE, Barnes MP, Borg K, de Visser M, Driessen A, Howard R, Nollet F, Opara J, Stalberg E
Affiliation: Department of Neurology, Haukeland University Hospital, University of Bergen, Bergen, Norway – [email protected] – European Federation of Neurological Society
Journal: European Journal of Neurology
Citation: Eur J Neurol. 2006 Aug; 13(8):795-801
Publication Year and Month: 2006 08

Abstract: Post-polio syndrome (PPS) is characterized by new or increased muscular weakness, atrophy, muscle pain and fatigue several years after acute polio. The aim of the article is to prepare diagnostic criteria for PPS, and to evaluate the existing evidence for therapeutic interventions. The Medline, EMBASE and ISI databases were searched. Consensus in the group was reached after discussion by e-mail. We recommend Halstead's definition of PPS from 1991 as diagnostic criteria. Supervised, aerobic muscular training, both isokinetic and isometric, is a safe and effective way to prevent further decline for patients with moderate weakness (Level B). Muscular training can also improve muscular fatigue, muscle weakness and pain. Training in a warm climate and non-swimming water exercises are particularly useful (Level B). Respiratory muscle training can improve pulmonary function. Recognition of respiratory impairment and early introduction of non-invasive ventilatory aids prevent or delay further respiratory decline and the need for invasive respiratory aid (Level C). Group training, regular follow-up and patient education are useful for the patients' mental status and well-being. Weight loss, adjustment and introduction of properly fitted assistive devices should be considered (good practice points). A small number of controlled studies of potential-specific treatments for PPS have been completed, but no definitive therapeutic effect has been reported for the agents evaluated (pyridostigmine, corticosteroids, amantadine). Future randomized trials should particularly address the treatment of pain, which is commonly reported by PPS patients. There is also a need for studies evaluating the long-term effects of muscular training.

Conclusions:

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Category: Diagnosis and Management

Title: Post-polio syndrome and total health status in a prospective hospital study.
Author: Farbu E, Rekand T, Gilhus NE
Affiliation: Department of Neurology, Haukeland University Hospital, Bergen, Norway
Journal: European Journal of Neurology
Citation: 2003 Jul;10(4):407-13.
Publication Year and Month: 2003 07

Abstract: New loss of function among patients with previous polio is frequently reported and has several causes. All patients referred to the Department of Neurology, Haukeland University Hospital, Bergen, for 13 months during 2000-2001 with diagnosis late effects of polio were examined prospectively to identify their symptoms and loss of function. Eighty-five patients aged 47-91 years with mean of 61 years were included. The most common complaints were pain (44%), muscular weakness (27%), and fatigue (16%). Muscular weakness occurred in lower limbs in 75%, in respiratory muscles in only 5%. Walking in stairs was impaired in 72% and outdoor walking in 65%. Seventeen patients (19%) reported no loss of function. Post-polio syndrome was diagnosed in 26% of the patients. Polio-related loss of function including cervical and lumbosacral radiculopathies, mononeuropathies and degenerative joint disease were found in an additional 53%. Eleven patients (13%) had distinct non-polio-related disorders that caused new loss of function. The remaining 8% had a stable condition.

Conclusions: In conclusion, the majority of polio patients who seek hospital, experience a new loss of function because of polio-related disorders. A careful neurological examination is necessary to identify the correct diagnosis and treatment.

Outcome of Research: More research required

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Category: Brain, Diagnosis and Management, Late Effects of Polio, Post-Polio Motor Unit

Title: Spinal cord gray matter atrophy is associated with functional decline in post-polio syndrome
Author: Maria Janina Wendebourg (1,2), Matthias Weigel (1,2,3,4,5), Laura Richter (1), Vanya Gocheva (6), Patricia Hafner (6), Anna-Lena Orsini (6), Valentina Crepulja (1,2), Simone Schmidt (6), Antal Huck (4), Johanna Oechtering (1), Maria Blatow (7), Tanja Haas (3,4), Cristina Granziera (1,2,5), Ludwig Kappos (1,2,5), Philippe Cattin (4), Oliver Bieri (3,4) Dirk Fischer (6), Regina Schlaeger (1,2,5)
Affiliation: 1. Neurology Clinic and Policlinic, Department of Clinical Research, University Hospital Basel, University of Basel, Basel, Switzerland

2. Translational Imaging in Neurology (ThINk), Department of Biomedical Engineering, University of Basel, Basel, Switzerland

3. Division of Radiological Physics, Department of Radiology, University Hospital Basel, Basel, Switzerland

4. Department of Biomedical Engineering, University of Basel, Basel, Switzerland

5. MS Center and Research Center for Clinical Neuroimmunology and Neuroscience Basel (RC2NB), University Hospital Basel and University of Basel, Basel, Switzerland

6. Division of Pediatric Neurology, University of Basel Children's Hospital, Basel, Switzerland

7. Department of Neuroradiology, Clinical Neuroscience Center, University Hospital Zurich, University of Zurich, Zurich,
Journal: European Journal of Neurology
Citation: Eur J Neurol. 2022;00:1–11.
DOI: 10.1111/ene.15261
Publication Year and Month: 2022 01

Abstract: Objective: To determine if patients with post- polio syndrome (PPS) show spinal cord gray matter (SCGM) atrophy and to assess associations between SCGM atrophy, muscle strength and patient- reported functional decline.

Methods: Twenty patients diagnosed with PPS (March of Dimes criteria) and 20 age- and sex- matched healthy controls (HC) underwent 3T axial 2D- rAMIRA magnetic resonance imaging at the intervertebral disc levels C2/C3–C6/C7, T9/T10 and the lumbar enlarge-ment level (Tmax) (0.5 × 0.5 mm2 in- plane resolution). SCGM areas were segmented manu-ally by two independent raters. Muscle strength, self-reported fatigue, depression and pain measures were assessed.

Results: Post- polio syndrome patients showed significantly and preferentially re-duced SCGM areas at C2/C3 (p= 0.048), C3/C4 (p= 0.001), C4/C5 (p< 0.001), C5/C6 (p= 0.004) and Tmax (p= 0.041) compared to HC. SCGM areas were significantly associated with muscle strength in corresponding myotomes even after adjustment for fatigue, pain and depression. SCGM areaTmax together with age and sex explained 68% of ankle dorsiflexion strength variance. No associations were found with age at or time since infection. Patients reporting PPS- related decline in arm function showed significant cervical SCGM atrophy compared to stable patients adjusted for initial disease severity.

Conclusions: Patients with PPS show significant SCGM atrophy that correlates with mus-cle strength and is associated with PPS- related functional decline. Our findings suggest a secondary neurodegenerative process underlying SCGM atrophy in PPS that is not ex-plained by aging or residua of the initial infection alone. Confirmation by longitudinal studies is needed. The described imaging methodology is promising for developing novel imaging surrogates for SCGM diseases.

Conclusions: The rAMIRA approach is a novel, promising, clinically feasible and sensitive method for segment-wise quantitation of GM atrophy in the cervical and thoracic SC in patients with lower motor neuron disorders. This study demonstrated its clinical applicability and vali-dated it in patients with PPS, a presumed pure, lower motor neuron disorder, which can serve as a model for other neurodegenerative, genetic or autoimmune diseases of the SCGM.

Patients with PPS show significant SCGM atrophy, particularly at levels close to the cervical and lumbar enlargements. Even after adjustment for the level of depression, fatigue and pain, potential confounding symptoms frequently observed in PPS, SCGM atrophy is significantly and segment-wise associated with muscle strength in corresponding myotomes. Moreover, SCGM atrophy is associated with patient-reported PPS-related functional decline. Secondary analyses suggest that SCGM atrophy is rather due to a second dis-ease phase than being a sole residuum of the initial infection or a pure aging effect. These observations support the hypothesis of a focally accentuated neurodegenerative process in the SC underlying PPS. Larger, ideally multicentric, longitudinal studies conducted over a sufficiently long timespan are an important next step to confirm our results and gain more insights into the development of SCGM atrophy over time and its correlation to clinical symptom evolution in patients with PPS.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Neurological Symptoms in Danes with a History of Poliomyelitis: Lifelong Follow-Up of Late Symptoms, their Association with Initial Symptoms of Polio, and Presence of Postpolio Syndrome
Author: Kay L. Nielsen N.M. Wanscher B. Jennum P.
Affiliation: Specialized Hospitalet for Polio and Accident Patients, Rødovre, Denmark
Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark
Medical Department 3, Næstved, Slagelse and Ringsted Hospital, Slagelse, Denmark
Danish Center for Sleep Medicine, Department of Clinical Neurophysiology, Rigshospitalet, University of Copenhagen, Glostrup, Denmark
Journal: European Neurology
Citation: 2018, Vol.80, No. 5-6
Publication Year and Month: 2018

Abstract: Background: Previous studies suggest that patients with a history of poliomyelitis (PM) later in life experience a variety of symptoms. These studies were carried out in patients who later in life were admitted to hospital or became members of polio societies and may therefore not be representative of all polio patients. Little data have been published concerning patients actually discharged from hospital with a diagnosis of acute paralytic PM. Objectives: The aim of this study was to compare the prevalence of late symptoms in individuals with a history of paralytic PM with that of controls, and to study whether late symptoms in individuals with a history of PM were associated with symptoms at the acute stage of polio, and finally to compare the prevalence of symptoms in polio patients with postpolio syndrome (PPS) with the prevalence of symptoms in polio patients without PPS. Methods: A questionnaire concerning various symptoms was sent to a previously established cohort of patients, who during the polio epidemics were discharged from the Department of Infectious Disease at Blegdamshospitalet, Copenhagen, with a diagnosis of paralytic PM, and to age- and gender-matched controls without PM. Information about symptoms at the acute stage of disease was obtained from hospital records. Logistic regression analysis with adjustment for age and gender was applied to compare the occurrence of late symptoms in cases and controls and within the above-mentioned groups of individuals with a history of PM. Results: (i) Compared with controls, individuals with a history of polio significantly more often reported muscle symptoms, pain, neuropathic sensory symptoms, and bulbar symptoms; (ii) the occurrence of symptoms did not seem to be related to symptoms of the initial PM; and (iii) symptom prevalence was significantly higher in individuals with a history of polio who reported PPS as compared with those who did not.

Conclusions: Conclusion: Our data indicate that individuals with a history of PM late in life experience a variety of symptoms that cannot be attributed to lesions of the anterior horn. Furthermore, late symptoms do not seem to be related to initial symptoms of the acute stage of PM but to reported PPS. The last finding supports the perception that the cause of PPS is not just normal ageing.

Outcome of Research: More research required

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Category: Clinical Evaluation, Diagnosis and Management, Late Effects of Polio

Title: Post-polio Syndrome: More Than Just a Lower Motor Neuron Disease
Author: Stacey Li Hi Shing, Rangariroyashe H. Chipika, Eoin Finegan, Deirdre Murray, Orla Hardiman, and Peter Bede
Affiliation: Computational Neuroimaging Group, Academic Unit of Neurology, Biomedical Sciences Institute, Trinity College Dublin, Dublin, Ireland
Edited by: Francesca Trojsi, University of Campania, Luigi Vanvitelli Caserta, Italy
Reviewed by: Andrea Romigi, Mediterranean Neurological Institute (IRCCS), Italy; Louisa Ng, The University of Melbourne, Australia
*Correspondence: Peter Bede ei.dct@pedeb
Journal: Frontiers in Neurology
Citation: 10, 773. https://doi.org/10.3389/fneur.2019.00773
Publication Year and Month: 2019 07

Abstract: Post-polio syndrome (PPS) is a neurological condition that affects polio survivors decades after their initial infection. Despite its high prevalence, the etiology of PPS remains elusive, mechanisms of progression are poorly understood, and the condition is notoriously under-researched. While motor dysfunction is a hallmark feature of the condition, generalized fatigue, sleep disturbance, decreased endurance, neuropsychological deficits, sensory symptoms, and chronic pain are also often reported and have considerable quality of life implications in PPS. The non-motor aspects of PPS are particularly challenging to evaluate, quantify, and treat. Generalized fatigue is one of the most distressing symptoms of PPS and is likely to be multifactorial due to weight-gain, respiratory compromise, poor sleep, and polypharmacy. No validated diagnostic, monitoring, or prognostic markers have been developed in PPS to date and the mainstay of therapy centers on symptomatic relief and individualized rehabilitation strategies such as energy conservation and muscle strengthening exercise regimes. Despite a number of large clinical trials in PPS, no effective disease-modifying pharmacological treatments are currently available.

Conclusions: Despite being one of the most devastating neurodegenerative conditions in the world, surprisingly limited research is undertaken in post-polio syndrome. Its pathogenesis remains elusive, no sensitive diagnostic tools have been developed, and validated prognostic and monitoring markers are lacking. Non-motor symptoms of PPS have considerable quality of life implications and are notoriously challenging to manage. The etiology of fatigue in PPS is yet to be elucidated and successful individualized management strategies are needed to maintain mobility, independence, and patient autonomy. There is striking a paucity of neuroimaging studies in PPS that could provide anatomical insights into the substrate of extra-motor symptoms. Ultimately, the characterization of PPS-associated pathology may help research efforts in other motor neuron diseases.

Outcome of Research: More research required

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Comments (if any): This is a good overview of Post-Polio conditions particularly for clinicians new to the topic.

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Category: Diagnosis and Management

Title: Inverse Relationship Between Polio Incidence in the US and Colorectal Cancer.
Author: STEVEN LEHRER and PETER H RHEINSTEIN
Affiliation: Department of Radiation Oncology, Icahn School of Medicine at Mount Sinai, New York, U.S.A. [email protected].
Severn Health Solutions, Severna Park, MD, U.S.A.
Journal: IN VIVO
Citation: (Athens, Greece) vol. 32,6 (2018): 1485-1489.
Publication Year and Month: 2018

Abstract: BACKGROUND/AIM:
Polio is predominantly an enteric viral infection that was progressively eradicated in the United States after the introduction of polio vaccine in the early 1950s. U.S. colorectal cancer rates have dropped steadily for individuals born between 1890 and 1950, but have been increasing for every generation born since 1950. Moreover, the lowest worldwide age adjusted rates of colorectal cancer in 2012 were in sub-Saharan Africa, Gambia and Mozambique, where polio has not been eradicated. In the current study, poliomyelitis incidence in US states before the introduction of polio vaccine was analyzed.

MATERIALS AND METHODS:
Reported cases of poliomyelitis per 100,000 population by state 1932-1951 were from Centers for Disease Control. Colorectal cancer deaths per 100,000 in men (2005-2009) by US State are from the American Cancer Society. US state overweight and obesity data are from the Centers for Disease Control and Prevention (CDC). Smoking data are from the CDC.

RESULTS:
By US state, colorectal cancer incidence per 100,000 in men for 2005-2009 was inversely correlated with reported cases of poliomyelitis per 100,000 for 1932-1951 (r=-0.311, p=0.032). Colorectal cancer deaths per 100,000 in men in 2005-2009 were also inversely correlated with reported cases of poliomyelitis per 100,000 by state for 1932-1951 (r=-0.493, p<0.001). The relationship between colorectal cancer deaths and polio incidence was significant (β=-0.196, p=0.028) and independent of the effects of smoking (β=0.289, p=0.012) and overweight (β=0.547, p<0.001). The relationship in females with colorectal cancer was identical.

Conclusions: Polio virus infection of cells of the colon may induce some degree of resistance to the development of colon cancer decades later. The effect of polio virus infection seems to be especially potent in reducing the rate of death from colon cancer.

Outcome of Research: More research required

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Category: Diagnosis and Management, Differential Diagnosis, Gender Differences, Late Effects of Polio

Title: Postpolio Syndrome: A Review of Lived Experiences of Patients
Author: Oluwaseyi Jacob Oluwasanmi, Devaunna Andrene Mckenzie, Idowu Oluwasegun Adewole, Christian O Aluka, James Iyasse, Esther Olunu, and Adegbenro Omotuyi Fakoya1
Affiliation: Department of Microbiology, All Saints University School of Medicine, Commonwealth of Dominica, Roseau, Dominica
1Department of Anatomical Sciences, University of Medicine and Health Sciences, Basseterre, St. Kitts and Nevis
Address for correspondence: Dr. Adegbenro Omotuyi Fakoya, University of Medicine and Health Sciences, Basseterre, St. Kitts and Nevis. E-mail: [email protected]
Journal: International Journal of Applied & Basic Medical Research
Citation: 9(3), 129–134. https://doi.org/10.4103/ijabmr.IJABMR_333_18
Publication Year and Month: 2019 07

Abstract: Postpolio syndrome (PPS) refers to a group of conditions that are present in patients, years after recovery from initial acute paralytic poliomyelitis. About 15%–80% of 20 million polio survivors worldwide will experience exacerbation of symptoms which typically appear 15–30 years after the resolution of initial poliomyelitis. Symptoms include new muscle weakness, fatigue, myalgia, joint pain, dysphagia, and difficulty breathing. Other reported symptoms include cold intolerance, sleep disorder, dysphonia, loss of stamina, musculoskeletal deformities, cardiovascular disorders, psychosocial problems, and restless legs syndrome. These symptoms are attributed to the superimposed neuronal loss of aging with inflammatory mechanisms, but without any convincing evidence of viral reactivation. Risk factors include female gender, respiratory symptoms, normal aging, permanent disability caused by motor neuron damage, muscle overuse and disuse, aging, and immunologic mechanisms. Hypothyroidism-induced myopathy and fibromyalgia are a differential diagnosis for PPS, and exclusion diagnosis is required as confirmatory criteria for PPS. The symptoms of PPS presented determine the course of management.

Keywords: Fatigue, muscle weakness, myalgia, poliomyelitis, postpolio syndrome

Conclusions: PPS is a sequela of acute poliomyelitis which occurs decades after resolution of the initial paralytic or nonparalytic polio. Fatigue, muscle weakness, myalgia, and joint pain are the major symptoms of PPS. Diagnosis of PPS is made based on the exclusion of other conditions that can cause similar symptoms. The quality of life of patients with PPS is significantly affected by the reoccurrence of the constellations of symptoms they experience. Proper assessment and treatment should be performed to prevent severe impairment of function in patients.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Post-poliomyelitis syndrome as a possible viral disease
Author: Baj A (1), Colombo M (1), Headley JL (2), McFarlane JR (3), Liethof MA (4), Toniolo A (5)
Affiliation: (1) Laboratory of Clinical Microbiology, University of Insubria Medical School, Viale Borri 57, 21100 Varese, Italy; (2) Post-Polio Health International, Saint Louis, Missouri, USA; (3) European Polio Union, Huldenberg, Belgium; (4) Polio Australia Incorporated, Kew, Victoria, Australia; (5) Laboratory of Clinical Microbiology, University of Insubria Medical School, Viale Borri 57, 21100 Varese, Italy. Electronic address: [email protected]
Journal: International Journal of Infectious Diseases
Citation: Int J Infect Dis. 2015 May 1;35:107-116. doi: 10.1016/j.ijid.2015.04.018
Publication Year and Month: 2015 05

Abstract: This review summarizes current concepts on post-polio syndrome (PPS), a condition that may arise in polio survivors after partial or complete functional recovery followed by a prolonged interval of stable neurological function. PPS affects 15-20 million people worldwide. Epidemiological data are reported, together with the pathogenic pathways that possibly lead to the progressive degeneration and loss of neuromuscular motor units. As a consequence of PPS, polio survivors experience new weakness, generalized fatigue, atrophy of previously unaffected muscles, and a physical decline that may culminate in the loss of independent life. Emphasis is given to the possible pathogenic role of persistent poliovirus infection and chronic inflammation. These factors could contribute to the neurological and physical decline in polio survivors. A perspective is then given on novel anti-poliovirus compounds and monoclonal antibodies that have been developed to contribute to the final phases of polio eradication. These agents could also be useful for the treatment or prevention of PPS. Some of these compounds/antibodies are in early clinical development. Finally, current clinical trials for PPS are reported. In this area, the intravenous infusion of normal human immunoglobulins appears both feasible and promising.

Conclusions:

Outcome of Research: Effective.

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Category: Diagnosis and Management, Late Effects of Polio

Title: Post-poliomyelitis syndrome (2019)
Author: Eric Chun Pu Chu1 and Kary Ka Wai Lam2
Affiliation: 1New York Chiropractic and Physiotherapy Center, New York Medical Group, Hong Kong, People’s Republic of China
2Downtown Chiropractic Limited, Hong Kong, People’s Republic of China
Correspondence: Eric Chun Pu ChuNew York Chiropractic and Physiotherapy Centre, New York Medical Group, 41/F Langham Place Office Tower, 8 Argyle Street, Mongkok, Hong Kong, People’s Republic of China, Phone: Tel +852 3 594 7844, Fax: Fax +852 3 594 6193, Email [email protected]
Journal: International Medical Case Reports Journal
Citation: 12, 261–264. https://doi.org/10.2147/IMCRJ.S219481

Publication Year and Month: 2019 08

Abstract: Most developed countries eliminated paralytic poliomyelitis (polio) in the 1970s to 1980s. It was believed that after recovery from acute paralytic poliomyelitis, the physical condition of survivors would remain stable for the rest of their lives. However, the elimination of polio does not equate the end of medical management of polio. Hundreds of thousands of polio survivors worldwide are still at risk of developing the late effects of the disease. Here, we report a case of post-polio syndrome who attended our clinic for the presence of new weakness and neuromuscular problems six decades after recovery from paralytic polio. It is essential that health professionals be aware of these conditions and have an understanding of the underlying pathophysiology of the symptoms.

Conclusions: Post-polio syndrome is related to the exhaustion of the motor units that form decades after the polio attack. This case report describes the effectiveness of manual interventions in assisting our patient in restoring the level of function and alleviating pain. The limitation of the current report is that it is just a single case. Further comparison with more existing therapeutic regimens is warranted to clarify these issues.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: A positive turning point in life -- how persons with late effects of polio experience the influence of an interdisciplinary rehabilitation programme
Author: Larsson Lund M (1), Lexell J
Affiliation: (1) Department of Community Medicine and Rehabilitation, Occupational Therapy , Umeå University, Umeå, Sweden
Journal: Journal of Rehabilitation Medicine
Citation: J Rehabil Med. 2010 Jun;42(6):559-65. doi: 10.2340/16501977-0559
Publication Year and Month: 2010 06

Abstract: OBJECTIVE: To describe and enhance our understanding of how persons with late effects of polio experience the influence of an interdisciplinary rehabilitation programme.

PARTICIPANTS: Twelve persons with clinically verified late effects of polio who had participated in an individualized, goal-oriented, comprehensive interdisciplinary rehabilitation programme.

METHODS: Qualitative research interviews analysed using the constant comparative method of grounded theory.

RESULTS: The rehabilitation programme was experienced as a turning point in the participants' lives. Before rehabilitation they felt they were on a downward slope without control. Rehabilitation was the start of a process of change whereby they acquired new skills, which, over time, contributed to a different but good life. After approximately a year, they had a sense of control and had accepted life with late effects of polio. They had also established new habits, taken on a changed valued self and could look to the future with confidence.

Conclusions: This qualitative study has shown that persons with late effects of polio can benefit from an individualized, goal-oriented, comprehensive interdisciplinary rehabilitation programme and experience positive changes in their management of daily activities and in their view of their late effects of polio, their future and their self.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Cognitive behavioural therapy for reducing fatigue in post-polio syndrome and in facioscapulohumeral dystrophy: A comparison
Author: Koopman, Fieke S., Merel A. Brehm, Anita Beelen, Nicole Voet, Gijs Bleijenberg, Alexander Geurts, Frans Nollet
Affiliation: Department of Rehabilitation , Academic Medical Center, University of Amsterdam, PO 22660, 1100 DD Amsterdam, The Netherlands. E-mail: [email protected]

Journal: Journal of Rehabilitation Medicine
Citation: 2017; 49: 585–590
Publication Year and Month: 2017

Abstract: Post-polio syndrome (PPS) and facioscapulohumeral dystrophy (FSHD) are two different neuromuscular disorders. Fatigue is a frequent complaint in both disorders. A recent study showed that cognitive behavioral therapy (CBT), which is a type of psychotherapy that helps patients to identify and reshape thoughts and behavior patterns that contribute to the fatigue was effective in alleviating fatigue in FSHD but not in PPS. In this study we investigated whether this difference in effectiveness might be explained by dissimilar fatigue-related thoughts (for example focusing on fatigue) in both conditions. We used questionnaires to measure the fatigue-related thoughts in 21 patients with PPS and 24 patients with FSHD . It appeared that fatigue-related thoughts in PPS were similar to those in FSHD and thus do not explain the difference in effectiveness of CBT.

Conclusions: Fatigue-related thoughts in PPS were similar to those in FSHD and thus do not explain the difference in effectiveness of CBT.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Outcome of physiotherapy as part of a multidisciplinary rehabilitation in an unselected polio population with one-year follow-up: an uncontrolled study.
Author: Bertelsen M, Broberg S, Madsen E.
Affiliation: Rehabilitation Centre of the Danish Society of Polio and Accident Victims (PTU), Rødovre, Denmark. [email protected]
Journal: Journal of Rehabilitation Medicine
Citation: 2009 Jan;41(1):85-7.
Publication Year and Month: 2009 01

Abstract: OBJECTIVE:
The aim of this study was to evaluate the outcome of physiotherapy as part of a multidisciplinary rehabilitation.

DESIGN:
Prospective uncontrolled intervention study.

SUBJECTS:
Fifty patients with late effects of polio, first time referred to physiotherapy at the Danish Society of Polio and Accident Victims (PTU) Rehabilitation Centre.

METHODS:
The intervention was physiotherapy as an essential part of an individually planned multidisciplinary rehabilitation. The outcome measures Six-Minute Walk Test and Timed-Stands Test were used to assess the functional capacity. Quality of life was evaluated by Medical Outcome Survey Short Form (SF-36) and fatigue by Multidimensional Fatigue Inventory (MFI-20). Patients were tested at baseline; 3 months after the start of rehabilitation and at one-year follow-up.

RESULTS:
The patients showed significantly better functional capacity on all measurements 3 months after start of intervention and at one-year follow-up. The patients showed significant improvement in 3 of the SF-36 dimensions regarding quality of life, but only the improvement in "general health" remained after one year.

Conclusions: This study shows that patients with late effects of polio, who experience new problems related to polio, can benefit from an individually planned multidisciplinary intervention with emphasis on physiotherapy, and the improvement in physical capacity and general health can remain at one-year follow-up.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Post-poliomyelitis syndrome (2005)
Author: Trojan DA, Cashman NR
Affiliation: Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, 3801 rue Université, Montréal, Québec H3A 2B4, Canada – [email protected]
Journal: Muscle & Nerve
Citation: Muscle Nerve. 2005 Jan;31(1):6-19
Publication Year and Month: 2005 01

Abstract: Post-poliomyelitis syndrome (PPS) is a common neurological disorder that occurs in a large proportion of individuals who have recovered from paralytic poliomyelitis. The main clinical features are new weakness, muscular fatigability, general fatigue, and pain. The primary criteria necessary for the diagnosis of PPS are a history of paralytic poliomyelitis, partial or complete recovery of neurological function followed by a period of stability (usually several decades), persistent new muscle weakness or abnormal muscle fatigability, and the exclusion of other causes of new symptoms. The cause of PPS remains unclear, but is likely due to a distal degeneration of enlarged post-poliomyelitis motor units. Contributing factors to PPS may be aging (with motor neuron loss), overuse, and disuse. PPS is usually a slowly progressive neuromuscular disease. Although there is no specific treatment for PPS, an interdisciplinary management program can be useful in controlling symptoms.

Conclusions:

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Category: Diagnosis and Management

Title: Postpolio syndrome and the late effects of poliomyelitis. Part 1. pathogenesis, biomechanical considerations, diagnosis, and investigations.
Author: Lo JK, Robinson LR.
Affiliation: Sunnybrook Health Sciences Centre, Division of Physical Medicine and Rehabilitation, Department of Medicine, University of Toronto, 2075 Bayview Avenue, Toronto, Ontario, Canada
Journal: Muscle & Nerve
Citation: https://doi.org/10.1002/mus.26168
Publication Year and Month: 2018 05

Abstract: Postpolio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. Many theories exist regarding the pathogenesis of PPS, which remains incompletely understood. In contrast, the late effects of poliomyelitis are often a consequence of biomechanical alterations that occur as a result of polio‐related surgeries, musculoskeletal deformities, or weakness. Osteoporosis and fractures of the polio‐involved limbs are common. A comprehensive clinical evaluation with appropriate investigations is essential to fulfilling the established PPS diagnostic criteria. PPS is a diagnosis of exclusion in which a key clinical feature required for the diagnosis is new muscle weakness and/or muscle fatigability that is persistent for at least 1 year. Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS.

Conclusions: Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Post‐polio syndrome and the late effects of poliomyelitis: Part 2. treatment, management, and prognosis.
Author: Lo JK, Robinson LR.
Affiliation: Sunnybrook Health Sciences Centre, Division of Physical Medicine and Rehabilitation, Department of Medicine, University of Toronto, 2075 Bayview Avenue, Toronto, Ontario, M4N 3M5, Canada
Journal: Muscle & Nerve
Citation: https://doi.org/10.1002/mus.26167
Publication Year and Month: 2018 05

Abstract: Post‐polio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. An individualized approach to rehabilitation management is critical. Interventions may include rehabilitation management strategies, adaptive equipment, orthotic equipment, gait/mobility aids, and a variety of therapeutic exercises. The progression of muscle weakness in PPS is typically slow and gradual; however, there is also variability in both the natural history of weakness and functional prognosis. Further research is required to determine the effectiveness of selected medical treatment. Muscle Nerve 58:760–769, 2018

Conclusions: The progression of muscle weakness in PPS is typically slow and gradual; however, there is also variability in both the natural history of weakness and functional prognosis.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Repetitive transcranial magnetic stimulation in treatment of post polio syndrome
Author: Pastuszak, Z., Piusaska-Macoch, R., Stepieri, A., Czernicki, Z.,
Affiliation: Department of Neurosurgery, Mossakowski Medical Research Centre, Polish Academy of Sciences, A. Pawińskiego 5, 02-106 Warsaw, Poland

Department of Neurology, Military Institute of Medicine, Szaserów 128, 04-141 Warsaw, Poland

Department of Neurosurgery, Warsaw University of Medicine, Cegłowska 80, 01-809 Warsaw, Poland
Journal: Neurologia i Neurochirurgia Polska
Citation: Volume 52 (2): 2018, Pages 281-284
Publication Year and Month: 2018 03

Abstract: Post polio syndrome is a rare disease that occurs decades after polio virus infection. Repetitive transcranial magnetic stimulation (rTMS) is a treatment option with proved effectiveness in drug resistant depression. Possibly it can be helpful in therapy of other neurological diseases including post polio syndrome.

Conclusions: rTMS can be an effective method in treatment of post polio syndrome but further studies with larger group need to be done to confirm that data.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Risk factors for post-polio syndrome among an Italian population: a case-control study.
Author: Bertolasi L, Acler M, dall'Ora E, Gajofatto A, Frasson E, Tocco P, Turri M, Ferlisi M, Fiorini M, Pimazzoni F, Squintani G, Martini M, Danzi B, Monaco S.
Affiliation: Section of Neurology, Department of Neurological, Neuropsychological, Morphological and Motor Sciences, University of Verona, Piazzale L.A. Scuro 10, 37134, Verona, Italy. [email protected]
Journal: Neurological sciences: official journal of the Italian neurological society
Citation: 2012 Dec;33(6):1271-5. doi: 10.1007/s10072-012-0931-2. Epub 2012 Jan 14.
Publication Year and Month: 2012 01

Abstract: Post-polio syndrome (PPS) is a clinical syndrome of new weakness, fatigue and musculoskeletal pain occurring in a variable proportion of polio survivors decades after acute disease. To date, several risk factors for PPS development have been reported, although the etiology of this disorder remains elusive. Using a case-control design, we aimed to assess risk indicators for PPS in a group of Italian polio survivors. Subjects with prior poliomyelitis attending the rehabilitation hospital of Malcesine, Italy, were the target population. Patients with PPS, diagnosed according to the European Federation of Neurological Societies criteria, served as cases, while patients not meeting diagnostic criteria for PPS were used as controls. All subjects were assessed through a structured questionnaire made of 82 questions and neurological examination. The association with investigated risk factors (sex, age at polio onset, age at onset of symptoms, extension and severity of polio, employment) was analyzed by the calculation of the odds ratio. A total of 161 out of 391 eligible patients met the adopted diagnostic criteria for PPS, giving a frequency of 41.2%. Symptoms most frequently complained by PPS patients were loss of muscle strength, loss of resistance, loss of muscle volume and generalized fatigue. Female gender, the presence of respiratory disturbance during the acute phase of polio and the use of orthoses and aids during the recovery and stabilization represented independent risk factors for PPS in the studied population.

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Management of post-polio syndrome (1997)
Author: Trojan DA, Finch L
Affiliation: Not stated
Journal: NeuroRehabilitation
Citation: NeuroRehabil. 1997;8:93-105
Publication Year and Month: 1997 08

Abstract: The management of patients presenting with post-poliomyelitis syndrome is discussed. It is essential to identify and treat other medical and neurological conditions which could produce these symptoms. New weakness can be managed with exercise, avoidance of muscular overuse, weight loss, orthoses and assistive devices. Fatigue can be managed with energy conservation techniques. The management of pain is dependent upon its causes. Treatments are reviewed.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Sense of Coherence in persons with late effects of polio
Author: Nolvi M, Brogardh C, Jacobsson L, Lexell J
Affiliation: Maria Nolvi, MD, Department of Health Sciences, PO Box 157, Lund University, SE-221 00 Lund, Sweden
Journal: NeuroRehabilitation
Citation: Vol. 42, no. 1, pp. 103-111, 2018
Publication Year and Month: 2018 01

Abstract: BACKGROUND:Sense of Coherence (SOC) is important for successful adaptation and mental well-being in people with life-long medical conditions. Late effects of polio (LEoP) often lead to a life-long disability, but no study has assessed SOC in this population. OBJECTIVE:To assess SOC in persons with LEoP and to explore the association between SOC, demographics (age, gender, marital status and level of education) and variables related to LEoP (age at polio onset, number of years from polio until onset of LEoP and self-rated disability). METHOD:Ninety-three community-dwelling persons with clinically verified LEoP responded to a postal survey with the Sense of Coherence Scale (SOC-13). A hierarchical multiple regression analysis was performed to explore the associations with SOC. RESULTS:SOC varied considerably among the participants. The mean and median SOC-13 total sum score was 71.8 and 76 points, which is similar to age-matched non-disabled people. The number of years before onset of LEoP and self-rated disability together with the participants’ marital status and level of education explained 37% (p < 0.001) of the variance in SOC.

Conclusions: CONCLUSION:Persons with LEoP have a level of sense of coherence (SOC) indicating that they generally have the ability to understand, handle and being motivated when dealing with stressful events and problems arising in their lives as a result of their disability. Being married and having a higher education, living many years before onset of LEoP and perceiving a mild to moderate disability contributed to a strong SOC.

Outcome of Research: More research required

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Category: Diagnosis and Management, Late Effects of Polio

Title: Knowledge of healthcare professionals about poliomyelitis and postpoliomyelitis: a cross-sectional study
Author: Claudio Andre Barbosa de Lira (I), Douglas Assis Teles Santos (II), Ricardo Borges Viana (III), Juliana Moreira Guimarães (IV), Jéssica Nathalia Soares Oliveira (V), Bolivar Saldanha Sousa (VI), Marcos Gonçalves de Santana (VII), Rodrigo Luiz Vancini (VIII),
Marília Santos Andrade (IX), Pantelis Nikolaidis (X), Thomas Rosemann (XI), Beat Knechtle (XII)
Affiliation: (I) BSc, PhD. Associate Professor, Faculdade de Educação Física e Dança (FEFD), Universidade Federal de Goiás (UFG), Goiânia (GO), Brazil. https://orcid.org/0000-0001-5749-6877

(II) BSc, MSc. Assistent Professor, Colegiado de Educação Física, Universidade do Estado da Bahia (UNEB), Teixeira de Freitas (BA), Brazil. https://orcid.org/0000-0002-7664-5468

(III) BSc, PhD. Professor, Escola Superior de Educação Física e Fisioterapia do Estado de Goiás (ESEFFEGO), Universidade Estadual de Goiás (UEG), Goiânia (GO), Brazil. https://orcid.org/0000-0001-9200-3185

(IV) BSc. Nurse, Unidade Acadêmica Especial de Ciências da Saúde, Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0002-6729-0335

(V) BSc. Biomedic, Unidade Acadêmica Especial de Ciências da Saúde (UA-CISAU),
Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0002-9531-4277

(VI) MD, PhD. Physician, Hospital Israelita Albert Einstein, São Paulo-Brazil, São Paulo (SP), Brazil. https://orcid.org/0000-0001-9682-5987

(VII) BSc, PhD. Associate Professor, Unidade Acadêmica Especial de Ciências da Saúde (UA-CISAU), Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0001-7674-3263

(VIII) BSc, PhD. Adjunct Professor, Centro de Educação Física e Desportos, Universidade Federal do Espírito Santo (UFES), Vitória (ES), Brazil. https://orcid.org/0000-0003-1981-1092

(IX) PhD. Physical Therapist and Associate Professor, Department of Physiology, Universidade Federal de São Paulo (USP), São Paulo (SP), Brazil. https://orcid.org/0000-0002-7004-4565

(X) BSc, PhD. Professor, School of Health and Caring Sciences, University of West Attica, Athens, Greece. https://orcid.org/0000-0001-8030-7122

(XI) MD, PhD. Physician and Professor, Institute of Primary Care, University of Zurich, Zurich, Switzerland. https://orcid.org/0000-0002-6436-6306

(XII) MD, PhD. Physician and Scientific Assistant, Institute of Primary Care, University of Zurich, Zurich, Switzerland; and Researcher, Medbase St. Gallen Am Vadianplatz, St. Gallen, Switzerland https://orcid.org/0000-0002-2412-9103
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Sao Paulo Med. J.

139 (5) • Aug-Sep 2021
https://doi.org/10.1590/1516-3180.2020.0617.16032021
Publication Year and Month: 2021 08

Abstract: BACKGROUND:
Postpoliomyelitis syndrome is a clinical condition that can affect poliomyelitis survivors.

OBJECTIVE:
Our aim was to evaluate knowledge of poliomyelitis and postpoliomyelitis syndrome among Brazilian healthcare professionals.

DESIGN AND SETTING:
Cross-sectional study conducted at a Brazilian public higher education institution located in the state of Goiás.

METHODS:
The participants (n = 578) were Brazilian physicians, physical therapists, nurses, nutritionists and psychologists. A self-administered questionnaire (30 questions) was designed to probe knowledge about poliomyelitis and postpoliomyelitis syndrome. From the questionnaire, we created a structured test to objectively evaluate the knowledge of these professionals. The test was composed of 20 questions and was scored over a range from 0 (totally ill-informed) to 20 (totally well-informed).

RESULTS:
In general, the physicians, physical therapists and nurses demonstrated better understanding of poliomyelitis and postpoliomyelitis syndrome. The healthcare professionals who had received previous information about poliomyelitis and postpoliomyelitis syndrome had significantly higher scores than those who had never received information (P < 0.001). On average, this difference was approximately 28.6%.

CONCLUSIONS:
The findings from the present study indicate that there is a critical need for improvement of knowledge about postpoliomyelitis syndrome among Brazilian healthcare professionals. The services provided by these professionals may therefore become compromised. Furthermore, public healthcare initiatives should be implemented to improve knowledge among healthcare professionals.

KEYWORDS (MeSH terms):
Poliomyelitis; Physicians; Nervous system; Postpoliomyelitis syndrome; Knowledge

AUTHORS’ KEY WORDS:
Medical education; Neuromuscular disease; Late effects of polio

Conclusions: Our study showed that, overall, there is a lack of knowledge about PPS and poliomyelitis, especially among psychologists and nutritionists. Therefore, the services provided by these professionals may become compromised. Furthermore, government initiatives should be implemented to increase knowledge among healthcare professionals.

Outcome of Research: Not applicable

Availability of Paper: The full text of this paper has been generously made available by the publisher.

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Category: Diagnosis and Management, Orthoses, Surgery

Title: Polio revisited: reviving knowledge and skills to meet the challenge of resurgence
Author: Benjamin Joseph (1) and Hugh Watts (2)
Affiliation: (1) Aster Medcity, Kochi, Kerala India, 18 HIG HUDCO Colony, Manipal, Karnataka 576104 India

(2) Shriners Hospital for Children, Los Angeles, CA USA
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Journal of Children's Orthopaedics

9 (5): 325–338, doi: 10.1007/s11832-015-0678-4
Publication Year and Month: 2015 09

Abstract: Purpose
To date, polio has not been eradicated and there appears to be a resurgence of the disease. Hence, there is a need to revive decision-making skills to treat the effects of polio.

Methods
Here, we outline the aspects of treatment of paralysis following polio based on the literature and personal experience of the authors. The surgical treatment of the lower and upper extremities and the spine have been reviewed. The scope of bracing of the lower limb has been defined.

Results
The effects of polio can be mitigated by judicious correction of deformities, restoration of muscle balance, stabilising unstable joints and compensating for limb length inequality.

Conclusions
As polio has not been eradicated and there is a risk of resurgence of the disease, paediatric orthopaedic surgeons need to be prepared to deal with fresh cases of polio. Revival of old techniques for managing the effects of paralysis following polio is needed.

Keywords: Poliomyelitis, Resurgence, Surgical decision-making, Bracing, Paralytic deformity

Conclusions: Polio has not been eradicated and there is a risk of resurgence of the disease. Paediatric orthopaedic surgeons need to be prepared to deal with fresh cases of polio. Revival of old techniques of managing the effects of paralysis following polio is needed.

Outcome of Research: Not applicable

Availability of Paper: The full text of this paper has been generously made available by the publisher.

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Category: Diagnosis and Management, Late Effects of Polio

Title: Post-polio syndrome and the phantom of acute previous poliomyelitis: a systemic entity
Author: Jacqueline Stephanie Fernandes do Nascimento (1), Nicolle dos Santos Moraes Nunes (1), Thais de R. Bessa-Guerra (1*), Marco Antonio Alves Azizi (2), Adalgiza Mafra Moreno (2), Antônio Marcos da Silva Catharino (2), Paulo Henrique de Moura (2), Thiago Rodrigues Gonçalves (2), Renata R. T. Castro (2), Acary Souza Bulle Oliveira (3), Marcos RG de Freitas (4) and Marco Orsini (2)
Affiliation: (1) Graduanda de Medicina na Universidade lguaçu (UNIG) - Nova lguaçu, RJ -Brasil

(2) Professor titular na Universidade lguaçu (UNIG). Nova lguaçu, RJ -Brasil

(3) Neurologista, Doutor em Neurologia pela Escola Paulista de Medicina (UNIFESP) - São Paulo (SP) - Brasil

(4) Neurologista, Doutor, Professor Titular e Chefe do Serviço de Neurologia da UFF, Niterói-RJ - Brasil
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: International Journal of Current Research

Vol. 13, Issue, 04
Publication Year and Month: 2021 04

Abstract: Introduction:
Poliomyelitis, often referred to as infantile paralysis or polio, was first described in 1840 by Jakob Von Heine, a German orthopedic physician. It is an acute and infectious disease caused by an enterovirus of worldwide distribution. With the implementation of immunization, the incidence of cases has reduced exponentially worldwide. However, a portion of individuals who have already developed polio, are now manifesting the late effects of polio, called post polio syndrome (PPS).

Case Report:
PRF, male, system analyst. Diagnosis of acute previous polio at the 18th month of age in a hospital in Fortaleza in 1965. At the age of 18 he started to present a new clinic of muscle fatigue, weakness and inability to perform his daily activities.

Discussion:
The Post-polio syndrome (PPS) was first reported in 1875, by Raymond, when reporting the case of a 19-year-old man, previously infected with acute previous polio and at that time had presented a new clinic of muscle weakness and atrophy. Post-poliomyelitis syndrome is defined as a presentation of the delayed effects of polio with the appearance of new neuromuscular symptoms that occur at least 15 years after clinical and functional stability in the natural history of patients with a previous history of acute previous polio. It can be characterized by: new muscle weakness, atrophy and pain, fatigue, sleep disorders, joint pain, cold intolerance, recent weight gain, respiratory distress and dysphagia. Criteria that support the diagnosis are used, such as: (1) Having been affected with acute previous polio; (2) at least 15 years of clinical stability; (3) new clinic of muscle weakness and fatigue; (4) complete or partial recovery of the functions lost in the poliomyelitis period, with a subsequent decline; (5) no other clinical conditions that explain the case. However, effective monitoring by a multidisciplinary team is necessary to assess the degree of functional and motor deterioration, respiratory capacity, as well as the psychological assessment of the patient.

Conclusion:
Although acute previous poliomyelitis has been eradicated in Brazil, as well as in several countries in the world, it is estimated that today there are still about 12 million individuals with poliomyelitis after-effects.

Conclusions: Although acute previous polio has been eradicated in Brazil, as well as in several countries in the world, it is estimated that today there are still about 12 million individuals with poliomyelitis after-effects. These patients overload the health system, especially those diagnosed with post-polio syndrome. Therefore, studies in different areas of health sciences are extremely important for understanding the pathophysiological mechanisms and with the aim of improving the quality of life of these patients.

Studies that address the biochemical and physiological issue can be useful for understanding the neurodegeneration that occurs in PPS, in addition to composing material for the study of the repercussion of a physical / motor after-effect on one or more organic systems and on functional capacity.

Outcome of Research: More research required

Availability of Paper: The full text of this paper has been generously made available by the publisher.

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Category: Diagnosis and Management, Differential Diagnosis

Title: Plasma CHI3L1 in Amyotrophic Lateral Sclerosis: A Potential Differential Diagnostic Biomarker
Author: Alessandro Bombaci*, Umberto Manera, Giovanni De Marco, Federico Casale, Paolina Salamone, Giuseppe Fuda, Giulia Marchese, Barbara Iazzolino, Laura Peotta, Cristina Moglia, Andrea Calvo and Adriano Chiò
Affiliation: “Rita Levi Montalcini” Department of Neuroscience, University of Turin, 10126 Turin, Italy
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: J. Clin. Med. 2023, 12(6), 2367; https://doi.org/10.3390/jcm12062367
Publication Year and Month: 2023 03

Abstract: Background: Motor neuron diseases (MNDs) are fatal neurodegenerative diseases. Biomarkers could help with defining patients’ prognoses and stratifications. Besides neurofilaments, chitinases are a promising family of possible biomarkers which correlate with neuroinflammatory status. We evaluated the plasmatic levels of CHI3L1 in MNDs, MND mimics, and healthy controls (HCs).

Methods: We used a sandwich ELISA to quantify the CHI3L1 in plasma samples from 44 MND patients, 7 hereditary spastic paraplegia (HSP) patients, 9 MND mimics, and 19 HCs. We also collected a ALSFRSr scale, MRC scale, spirometry, mutational status, progression rate (PR), blood sampling, and neuropsychological evaluation.

Results: The plasma levels of the CHI3L1 were different among groups (p = 0.005). Particularly, the MND mimics showed higher CHI3L1 levels compared with the MND patients and HCs. The CHI3L1 levels did not differ among PMA, PLS, and ALS, and we did not find a correlation among the CHI3L1 levels and clinical scores, spirometry parameters, PR, and neuropsychological features. Of note, the red blood cell count and haemoglobin was correlated with the CHI3L1 levels (respectively, p < 0.001, r = 0.63; p = 0.022, and r = 0.52).

Conclusions: The CHI3L1 plasma levels were increased in the MND mimics cohort compared with MNDs group. The increase of CHI3L1 in neuroinflammatory processes could explain our findings. We confirmed that the CHI3L1 plasma levels did not allow for differentiation between ALS and HCs, nor were they correlated with neuropsychological impairment.

Keywords: biomarker; chitinases; cognitive impairment; differential diagnosis; early diagnosis; MND mimics; red blood cells

Conclusions: The measurement of the plasmatic levels of CHI3L1 could be useful in the differential diagnosis between MNDs and MND mimics. This is an important issue, since the early diagnosis of an MND is a determinant in the early starting of neuroprotective therapy and in clinical trial recruitment.
Further multicentre and longitudinal studies on a larger patient cohort, testing alongside other fluid biomarkers, are needed to better explain the role of CHI3L1 in the diagnosis and prognosis of MNDs and, also, of MND mimics.

Outcome of Research: More research required

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any): This article is helpful for any medical/neurologist trying to determine differential diagnosis of ALS vs post-polio.

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Category: Diagnosis and Management

Title: Lipid metabolic pathways converge in motor neuron degenerative diseases
Author: Olivia J Rickman, Emma L Baple, and Andrew H Crosby
Affiliation: Medical Research (Level 4), RILD Wellcome Wolfson Centre, University of Exeter Medical School, Royal Devon and Exeter NHS Foundation Trust, Barrack Road, Exeter, EX2 5DW, UK

Correspondence to: Professor Andrew Crosby Professor of Human Genetics University of Exeter Medical School Medical Research (Level 4) RILD Wellcome Wolfson Centre Royal Devon and Exeter NHS Foundation Trust Barrack Road, Exeter, EX2 5DW, UK E-mail: [email protected]
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Brain. 2020 Apr; 143(4): 1073–1087.
Published online 2019 Dec 18. doi: 10.1093/brain/awz382
Publication Year and Month: 2019 12

Abstract: Motor neuron diseases (MNDs) encompass an extensive and heterogeneous group of upper and/or lower motor neuron degenerative disorders, in which the particular clinical outcomes stem from the specific neuronal component involved in each condition. While mutations in a large number of molecules associated with lipid metabolism are known to be implicated in MNDs, there remains a lack of clarity regarding the key functional pathways involved, and their inter-relationships. This review highlights evidence that defines defects within two specific lipid (cholesterol/oxysterol and phosphatidylethanolamine) biosynthetic cascades as being centrally involved in MND, particularly hereditary spastic paraplegia. We also identify how other MND-associated molecules may impact these cascades, in particular through impaired organellar interfacing, to propose ‘subcellular lipidome imbalance’ as a likely common pathomolecular theme in MND. Further exploration of this mechanism has the potential to identify new therapeutic targets and management strategies for modulation of disease progression in hereditary spastic paraplegias and other MNDs.

Keywords: HSP, MND, cholesterol, mitochondria, lipidome imbalance

Conclusions: The development of new methodologies and approaches are important to delineate the specific subcellular biomarker deficits, such as oxysterol and PE imbalance, in HSP and MND. As well as providing potentially powerful biomarkers of disease, such tools may enable monitoring of treatment efficacy of therapeutics to re-address disease-associated lipidome imbalances. Specific genetic subtypes may be more amenable to treatment at targeting (for example) feedback systems, such as CDCA in CTX, or addressing oxysterol imbalance in SPG5. Genetic subtypes of disease leading to more complex subcellular outcomes may require multiple treatment approaches to address the specific mechanistic basis of each condition, and it may be unlikely that one approach will entail a ‘fix all’ treatment. Ultimately, clearer definition of the subcellular lipidome (and other) biological pathways underlying MND and HSP will pave the way for a more elegant approaches for predicting onset and severity of disease, and for designing and monitoring new therapeutic approaches.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: The cultural context of polio biographies
Author: Scheer J, Luborsky ML
Affiliation: National Rehabilitation Hospital Research Center, Washington, DC 20010
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1173-81
Publication Year and Month: 1991 11

Abstract: Cultural contexts influence the ways individuals interpret and experience functional losses associated with post-polio sequelae. Using in-depth multiple interview case studies from two National Institute on Aging projects, the concept of “biographies” is presented to place the individuals’ polio-related experiences within the context of their lives. Two major cultural contexts shape the construction of polio biographies: normative life course expectations and developmental tasks; and traditions associated with polio recovery and rehabilitation. The authors identify key dimensions of personal concern among polio survivors that can be used as entrance points for effective clinical intervention and to promote treatment compliance.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Post-polio sequelae - differential diagnosis and management
Author: Maynard FM
Affiliation: Not stated
Journal: Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):857-61
Publication Year and Month: 1985 07

Abstract: Forty-two patients with a past history of poliomyelitis were evaluated at a post-polio clinic for new problems or impairments. Evaluation included a complete history, neurological and biomechanical examination and electrodiagnostic studies. Based on this evaluation patients were placed into three groups: 23 patients were considered to have or likely to have Progressive Post-Polio Muscular Atrophy (PPPMA); 17 patients were considered to have other post-polio sequelae; and two patients had problems unrelated to a past history of polio but mistaken for post-polio sequelae. Musculoskeletal pain was a common complaint among all groups of patients. Twenty-two of the 40 patients with post-polio sequelae were advised to alter their method of ambulation and/or decrease their activity pattern in order to decrease strain and/or excessive exertion of involved muscles. The role of chronic overuse and exercise in producing PPPMA or musculoskeletal pain problems is discussed. Characteristic clinical problems and useful management plans are described.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Effect of treatment and noncompliance on post-polio sequelae
Author: Peach PE, Olejnik S
Affiliation: Roosevelt Warm Springs Institute for Rehabilitation, Georgia 31830
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1199-1203
Publication Year and Month: 1991 11

Abstract: In this study of 77 patients with post-polio sequelae (PPS), symptoms and manual test scores on initial evaluation were compared with those at subsequent follow-up evaluations. Patients were divided into three groups based on the degree to which they had complied with clinically recommended interventions: compliers, partial compliers, and noncompliers. At the end of the follow-up period (2.2 +/- 1.2 years), the mean muscle function scores of the entire study group had declined -1.5%, which represented a decline of -0.7% annually. On follow-up evaluations, the complier group had realized an improvement or resolution of post-polio symptoms, and also an improvement in muscle function of +0.6% annually. The partial complier group had realized either no improvement, or improvement in post-polio symptoms, but showed a further decline in muscle function of -3.0%, or an annual decline of -1.3%. The noncomplier group showed either no change, or a worsening of post-polio symptoms, and also showed a further decline in muscle function of -4.1%, which represented an annual decline of -2.0%.

Conclusions: The disparate outcomes among our post-polio patients underscore the need to develop more effective intervention strategies to achieve improved patient compliance, given the favorable outcomes experienced by patients who complied with clinical recommendations.

Outcome of Research:

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Category: Diagnosis and Management

Title: Polioencephalitis, stress, and the etiology of post-polio sequelae
Author: Bruno RL, Frick NM, Cohen J
Affiliation: Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation, East Orange, NJ 07018
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1269-76
Publication Year and Month: 1991 11

Abstract: Post-mortem neurohistopathologies that document polio virus-induced lesions in reticular formation and hypothalamic, thalamic, peptidergic, and monoaminergic neurons in the brain are reviewed from 158 individuals who contracted polio before 1950. This polioencephalitis was found to occur in every case of poliomyelitis, even those without evidence of damage to spinal motor neurons. These findings, in combination with data from the 1990 National Post-Polio Survey and new magnetic resonance imaging studies documenting post-encephalitis-like lesions in the brains of polio survivors, are used to present two hypotheses: 1) polioencephalitic damage to aging reticular activating system and monoaminergic neurons is responsible for post-polio fatigue, and 2) polioencephalitic damage to enkephalin-producing neurons is responsible for hypersensitivity to pain in polio survivors. In addition, the antimetabolic action of glucocorticoids on polio-damaged, metabolically vulnerable neurons may be responsible for the fatigue and muscle weakness reported by polio survivors during emotional stress.

Conclusions: The ability of the polio virus to produce symptoms by its destruction of neurons outside of the anterior horn has been accepted for more than 100 years. It is only our recent experience with PPS that has forced us to recognize that both the people who survived the original viral onslaught and their central nervous systems have been operating for decades under extreme stress. This stress now may be combining with the aging of an extensively damaged but here-to-fore remarkably functional central nervous system to reveal the previously hidden symptoms of polioencephalitis.

The above-presented hypotheses concerning the etiology of PPS fatigue suggest that reductions in both emotional and physical stress will reduce PPS. This is the experience of post-polio clinics throughout the world (36,54,55,56). As PPS fatigue continues to be treated with stress-management, work simplification and energy conservation (see 36,56), the hypotheses are being tested by studying the neuroanatomy, neuroendocrinology and neuropsychology of PPS fatigue. In addition, pharmacological means for stimulating the RAS that do not further metabolically stress its remaining aging, polio-damaged neurons are being tested.

Outcome of Research:

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Category: Diagnosis and Management

Title: Physical therapy management of the patient with post-polio syndrome. A case report.
Author: Donna J. Twist, Dong M. Ma
Affiliation:
Journal: Physical Therapy
Citation: Volume 66, Issue 9, 1 September 1986, Pages 1403–1406
Publication Year and Month: 1986 09

Abstract: This case report documents the treatment of a patient who experienced progressive muscle weakness and a decrease in function over time that did not appear to be related to any secondary neuromuscular disease. We discuss the relationship between age and maximal muscle function in addition to some general guidelines for rehabilitation. This type of patient can represent a challenge for the physical therapist. This case report, however, illustrates the degree of muscular and functional recovery that can result with a physical therapy program aimed at reducing levels and intensity of exercise, daily activity, and stress.

Conclusions: A combination of short-term goals appears to be essential to the successful management of a patient with post-polio syndrome.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Characteristics of Patients at First Visit to a Polio Clinic in Sweden
Author: Skough Vreede, K. and Sunnerhagen, K.S.
Affiliation: Goteborg University
Journal: PLoS One
Citation: https://doi.org/10.1371/journal.pone.0150286
Publication Year and Month: 2016 03

Abstract: Aim
Describe polio patients visiting a polio clinic in Sweden, a country where vaccination was introduced in 1957.

Design
A consecutive cohort study.

Patients
Prior polio patients.

Methods
All patients (n = 865) visiting the polio clinic at Sahlgrenska University Hospital, Gothenburg Sweden, between 1994 and 2012 were included in this study. Data at first visit regarding patient characteristics, polio classification, data of electromyography, origin, assistive devices and gait speed as well as muscle strength were collected for these patients. Twenty-three patients were excluded because no polio diagnosis could be established. A total of 842 patients with confirmed polio remained in the study.

Results
More than twenty percent of the patients were from countries outside the Nordic region and considerably younger than those from the Nordic region. The majority of the emigrants were from Asia and Africa followed by Europe (outside the Nordic region). Of all patients included ninety-seven percent (n = 817) had polio in the lower extremity and almost 53% (n = 444) had polio in the upper extremity while 28% (n = 238) had polio in the trunk, according to clinical classification of polio. Compared with a sample of the normal population, the polio patients walked 61–71% slower, and were 53–77% weaker in muscle strength of the knee and foot as well as grip strength.

Conclusion
The younger patients with polio emigrating from countries with different cultures may lead to a challenge for the multi professional teams working with post-polio rehabilitation and are of importance when planning for the care of polio patients the coming years.

Conclusions: Polio in lower extremities was more common than polio in upper extremities, verified both
by EMG and clinical classification. This is in accordance with earlier studies. Polio in lower
extremities was also classified as clinically unstable or severely atrophic to a higher extent than
polio in upper extremities (as shown in Fig 1). This is in accordance with an earlier study by
Sandberg et al [20] indicating a more pronounced ongoing denervation-reinnervation process
over time in a lower extremity muscle compared to upper extremity muscle (tibialis anterior
and biceps brachii respectively). The same pattern was also seen in patients studied in Minnesota where patients with leg weakness were twice as likely to complain of new problems compared to those with arm weakness [10].

The ongoing denervation-reinnervation process in patients with PPS results in larger motor
units.When motor-unit size has reached an upper limit, further losses of neurons can no longer
be compensated for and this results in increased muscle weakness [21]. The patients
showed to be stronger in isometric endurance compared to normal population. This may be
explained by the fact that the patients were weaker than the normal population in isometric
peak torque, which the measure of isometric endurance was based on. An increase in type I
(slow) muscle fibres has also been described in prior polio patients [22–23] and may be due to
a transition of type II (fast) to type I (slow).

An important study limitation were seen in the classification of polio as polio were classified
for left and right arm and leg, respectively, and not per muscle group. This can explain why a
polio affected leg in some cases was stronger compared to normal values as the muscles
involved in the strength measured i.e. knee flexion and knee extension muscles may not be
affected of polio. And the same is applicable regarding muscle strength of the foot as well as
grip strength. Data of muscle strength from some of the subjects were missing for different reasons i.e. they may have just not had time to participate, or refused to participate and some were too weak to perform the strength test. Some of the patients with muscle strength data missing, tried to perform the test, but were too weak to get a result. In the future, the use of ultrasound may be used to assess muscle function [24]. This would give the possibility to have more information of muscle function since this does not require that the patient has muscle strength to overcome gravity, which is a requirement for isokinetic testing.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Unraveling the transmission ecology of polio
Author: Martinez-Bakker M (1), King AA (1,2), Rohani P (1,2)
Affiliation: (1) Department of Ecology & Evolutionary Biology, University of Michigan, Ann Arbor, Michigan, United States of America; (2) Center for the Study of Complex Systems, University of Michigan, Ann Arbor, Michigan, United States of America; Fogarty International Center, National Institutes of Health, Bethesda, Maryland, United States of America
Journal: Public Library of Science
Citation: PLoS Biol. 2015 Jun;13(6): e1002172. doi:10.1371/journal.pbio.1002172
Publication Year and Month: 2015 06

Abstract: Sustained and coordinated vaccination efforts have brought polio eradication within reach. Anticipating the eradication of wild poliovirus (WPV) and the subsequent challenges in preventing its re-emergence, we look to the past to identify why polio rose to epidemic levels in the mid-20th century, and how WPV persisted over large geographic scales. We analyzed an extensive epidemiological dataset, spanning the 1930s to the 1950s and spatially replicated across each state in the United States, to glean insight into the drivers of polio’s historical expansion and the ecological mode of its persistence prior to vaccine introduction. We document a latitudinal gradient in polio’s seasonality. Additionally, we fitted and validated mechanistic transmission models to data from each US state independently. The fitted models revealed that: (1) polio persistence was the product of a dynamic mosaic of source and sink populations; (2) geographic heterogeneity of seasonal transmission conditions account for the latitudinal structure of polio epidemics; (3) contrary to the prevailing “disease of development” hypothesis, our analyses demonstrate that polio’s historical expansion was straightforwardly explained by demographic trends rather than improvements in sanitation and hygiene; and (4) the absence of clinical disease is not a reliable indicator of polio transmission, because widespread polio transmission was likely in the multiyear absence of clinical disease. As the world edges closer to global polio eradication and continues the strategic withdrawal of the Oral Polio Vaccine (OPV), the regular identification of, and rapid response to, these silent chains of transmission is of the utmost importance.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Post-polio sequelae: physiological and psychological overview
Author: Frick NM, Bruno RL
Affiliation: Not stated
Journal: Rehabilitation Literature
Citation: Rehabil Lit. 1986 May-Jun; 47(5-6):106-11
Publication Year and Month: 1986 05

Abstract: When the Salk and Sabin vaccines brought an end to the annual summer nightmare of polio epidemics, most Americans simply forgot about polio. Even many of those who had paralytic poliomyelitis put the disease out of their minds once they had achieved maximum recovery of function. Unfortunately, polio has again forced itself into the nation's consciousness. Over the past five years, many of those who had polio have been experiencing new and unexpected symptoms that range in severity from being merely unpleasant to severely debilitating.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Histopathologic Basis of Clinical Findings in Poliomyelitis
Author: Bodian, D.
Affiliation: Poliomyelitis Research Center, Department of Epidemiology, Johns Hopkins University, Baltimore
Journal: The American Journal of Medicine
Citation: 1949: 6(5):563–578
Publication Year and Month: 1949 05

Abstract: 1. Experimental evidence indicates that the onset of CNS pathologic changes occurs in the preparalytic period and is closely associated with the earliest evidence of virus activity in any particular region involved.
2. The earliest cytopathologic changes are diffuse chromatolysis of Nissl substance in the cytoplasm of nerve cells and mild cellular exudate consisting of polymorphonuclear and mononuclear leukocytes.
3. Nerve cell changes may be present in the earliest stages without inflammatory reaction in the vicinity and therefore are not necessarily the result of the latter, but rather the result of direct virus action.
4. Nerve cell changes either lead to rapid destruction of the cell or to arrest in the stage of cytoplasmic chromatolysis, following which complete morphologic recovery of the cell generally occurs over a period of about a month or less, depending upon the severity of injury.
5. Virus activity, nerve cell changes and inflammatory reaction are localized only in certain susceptible regions of the CNS, largely due to specific differences of susceptibility of nerve cells. The intensity of the inflammatory reaction, however, may be quite variable in different susceptible centers and in different individuals. Severe inflammatory reaction is usually but not always associated with extensive nerve cell destruction. Severe nerve cell damage may occur without extensive cellular infiltration in the cord.
6. Lesions in the cerebral cortex are usually confined to the motor area of the precentral gyrus and even here the lesions are rarely severe enough to suggest that they may produce clinical symptoms.
7. “Encephalitic” symptoms such as restlessness, stupor, disorientation and coma are associated with severe inflammatory reaction in the brainstem and often with small softenings in this region. They are not associated with unusual involvement of the cerebral cortex.
8. Brainstem centers principally involved in most instances are the reticular formation of the hind-brain, the vestibular nuclei and the roof nuclei of the cerebellum. Resulting functional disturbances are discussed.
9. Widespread dissemination of virus among most motor nerve cells in spinal cord enlargements occurs in experimental poliomyelitis as early as the first day of paralysis. Motor nerve cells which are affected either are destroyed very quickly during the first few days of the disease or undergo slower recovery changes leading to complete morphologic recovery within about a month. After this time it can be shown that the degree of paralysis and atrophy are closely correlated with the number of motor nerve cells destroyed. In the acute stage, however, this correlation is not as high and other factors must also play a role in producing paralysis. An important factor is the reversible injury of motor nerve cells. Less complete evidence from human material suggests that a similar situation obtains in human poliomyelitis.
10. Experimental work suggests three possible factors which may determine the variation in severity of infection. These are, first, variations due to difference in strains of the virus, second, reduction of severity due to previous paralytic or non-paralytic infection, and third, host variation unrelated to previous immunizing experience with the virus.

Conclusions: Poliomyelitis virus has strains that can vary depending on the host, and response to the virus. The virus causes most damage to the anterior horn cells of the cervical and lumbar segments, however there is damage to the brainstem and premotor areas in the brain.

Outcome of Research: More research required

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Comments (if any): David Bodian produced papers that helped enable (effective) availability for vaccinations. His work is still cited and referred to in understanding the effects of the polio virus, especially in relation to the central nervous system pathology.

Link to Paper (if available): Click here to view Abstract


Category: Diagnosis and Management

Title: Poliomyelitis and the postpolio syndrome
Author: Howard, R.S.
Affiliation: Lane-Fox Unit and Department of Neurology, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London
Journal: The British Medical Journal
(The BMJ)

Citation: 330(7503): 1314–1318.
doi: 10.1136/bmj.330.7503.1314
Publication Year and Month: 2005 06

Abstract: Acute poliomyelitis is now rarely encountered in the United Kingdom, but “imported” poliomyelitis still occurs and it is necessary to distinguish acute poliomyelitis from other causes of acute flaccid paralysis. Despite the obvious success of preventive policies, many patients who had poliomyelitis experience late functional deterioration after periods of prolonged stability—the so called postpolio syndrome. The patterns of disability and their management present unique challenges to the multidisciplinary rehabilitation team.

Conclusions: Although some patients who have had poliomyelitis may later develop wasting, pain, and fatigue in isolation, in most there is significant underlying weakness and skeletal deformity predisposing to functional deterioration. The severe physical stresses of postpolio disability contribute to the development of progressive orthopaedic, respiratory, neurological, and general medical abnormalities, often exacerbated by intercurrent events. These abnormalities may present with atypical clinical features because of the extent of underlying atrophy and weakness, but many are potentially treatable and most patients can be helped to understand and manage increasing disability. It is essential to emphasise that the symptoms, disabilities, and impairments of postpolio functional deterioration are often amenable to treatment. It is also important to urge caution before attributing functional deterioration to a primary “postpolio syndrome” or “progressive postpolio muscular atrophy.”

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Characteristics and management of postpolio syndrome
Author: Jubelt B, Agre JC
Affiliation: State University of New York, Upstate Medical University, 750 E Adams St, Syracuse, NY 13210, USA – [email protected]
Journal: The Journal of the American Medical Association
Citation: JAMA. 2000 Jul 26;284(4):412-4
Publication Year and Month: 2000 07

Abstract: Postpolio syndrome (PPS) refers to new, late manifestations occurring many years after acute poliomyelitis infection. Over the last 25 years, PPS has become a relatively common problem encountered by primary care physicians. A 1987 National Health Interview Survey estimated that about half of the 640,000 survivors of paralytic poliomyelitis in the United States had new late manifestations of PPS. Subsequent studies in the 1990s have found the occurrence of PPS among patients with previous poliomyelitis to range from 28.5% to 64%. The average time in various reports from the acute poliomyelitis until the onset of PPS is about 35 years, with a range from 8 to 71 years. However, it is unclear if the occurrence of PPS increases with aging, which may be the case based on the most accepted etiologic hypothesis.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Poliomyelitis and Parkinson Disease.
Author: Nielsen NM, Rostgaard K, Hjalgrim H, Aaby P, Askgaard D.
Affiliation: Department of Epidemiology Research, National University Hospital, Copenhagen, Denmark
Journal: The Journal of the American Medical Association
Citation: JAMA. 2002;287(13):1650–1651.
doi:10.1001/jama.287.13.1645
Publication Year and Month: 2002 04

Abstract: Parkinson disease (PD), which is due to loss of dopaminergic neurons in the zona compacta of the substantia nigra,1 may involve both genetic and environmental risk factors.2 Poliovirus is believed to cause neuronal damage in the substantia nigra,3 and thus a history of poliovirus infection may be associated with an increased risk of PD

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Management of postpolio syndrome (2010)
Author: Gonzalez H, Olsson T, Borg K
Affiliation: Division of Rehabilitation Medicine, Department of Clinical Sciences, Danderyd Hospital, Karolinska Institute, Stockholm, Sweden – [email protected]
Journal: The Lancet Neurology
Citation: Lancet Neurol. 2010 Jun; 9(6):634-42 and Comment in: Lancet Neurol. 2010 Jun; 9(6):561-3
Publication Year and Month: 2010 06

Abstract: Postpolio syndrome is characterised by the exacerbation of existing or new health problems, most often muscle weakness and fatigability, general fatigue, and pain, after a period of stability subsequent to acute polio infection. Diagnosis is based on the presence of a lower motor neuron disorder that is supported by neurophysiological findings, with exclusion of other disorders as causes of the new symptoms. The muscle-related effects of postpolio syndrome are possibly associated with an ongoing process of denervation and reinnervation, reaching a point at which denervation is no longer compensated for by reinnervation. The cause of this denervation is unknown, but an inflammatory process is possible. Rehabilitation in patients with postpolio syndrome should take a multiprofessional and multidisciplinary approach, with an emphasis on physiotherapy, including enhanced or individually modified physical activity, and muscle training. Patients with postpolio syndrome should be advised to avoid both inactivity and overuse of weak muscles. Evaluation of the need for orthoses and assistive devices is often required.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Postpolio syndrome: unanswered questions regarding cause, course, risk factors, and therapies
Author: Nollet F
Affiliation: Not stated
Journal: The Lancet Neurology
Citation: Lancet Neurol. 2010 Jun;9(6):561-3 - Comment on: Lancet Neurol. 2010 Jun;9(6):634-42
Publication Year and Month: 2010 06

Abstract: Living with the consequences of poliomyelitis is not recognised as an important health issue at present. However, millions of people worldwide have lasting impairments caused by polio infection, many of whom also had a decline in muscle function and decline in activities of daily living after years of stable functioning.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Motoneuron Disease and Past Poliomyelitis in England and Wales
Author: Martyn CN, Barker DJP, Osmond C.
Affiliation: MRC Environmental Epidemiology Unit, Southampton Hospital, United Kingdom
Journal: The Lancet Neurology
Citation: The Lancet, 331(8598):1319-1322

Publication Year and Month: 1988 06

Abstract: Past notification rates for poliomyelitis show a close geographical relation with current mortality from motoneuron disease in England and Wales. The increasing rate of poliomyelitis during the first half of this century and its predilection for affluent places and families were unique amongst infectious diseases. The unusual epidemiology of poliomyelitis is now being paralleled by motoneuron disease. These observations provide new evidence for a causal connection between the two conditions.

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Update on current and emerging treatment options for post-polio syndrome
Author: Farbu E
Affiliation: Neurocenter and National Competence Center for Movement Disorders, Stavanger University Hospital, Stavanger, Norway
Journal: Therapeutics and Clinical Risk Management
Citation: Ther Clin Risk Manag. 2010 Jul 21;6:307-13
Publication Year and Month: 2010 07

Abstract: Post-polio syndrome (PPS) refers to the clinical deterioration experienced by many polio survivors several decades after their acute illness. The symptoms are new muscle weakness, decreased muscle endurance, fatigue, muscle pain, joint pain, cold intolerance, and this typical clinical entity is reported from different parts of the world. The pathophysiology behind PPS is not fully understood, but a combination of distal degeneration of enlarged motor units caused by increased metabolic demands and the normal aging process, in addition to inflammatory mechanisms, are thought to be involved. There is no diagnostic test for PPS, and the diagnosis is based on a proper clinical workup where all other possible explanations for the new symptoms are ruled out. The basic principle of management of PPS lies in physical activity, individually tailored training programs, and lifestyle modification. Muscle weakness and muscle pain may be helped with specific training programs, in which training in warm water seems to be particularly helpful. Properly fitted orthoses can improve the biomechanical movement pattern and be energy-saving. Fatigue can be relieved with lifestyle changes, assistive devices, and training programs. Respiratory insufficiency can be controlled with noninvasive respiratory aids including biphasic positive pressure ventilators. Pharmacologic agents like prednisone, amantadine, pyridostigmine, and coenzyme Q10 are of no benefit in PPS. Intravenous immunoglobulin (IVIG) has been tried in three studies, all having positive results. IVIG could probably be a therapeutic alternative, but the potential benefit is modest, and some important questions are still unanswered, in particular to which patients this treatment is useful, the dose, and the therapeutic interval.

Conclusions:

Outcome of Research: More research required.

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There are currently 61 papers in this category.

Category: Diagnosis and Management, Differential Diagnosis

Title: Plasma CHI3L1 in Amyotrophic Lateral Sclerosis: A Potential Differential Diagnostic Biomarker
Author: Alessandro Bombaci*, Umberto Manera, Giovanni De Marco, Federico Casale, Paolina Salamone, Giuseppe Fuda, Giulia Marchese, Barbara Iazzolino, Laura Peotta, Cristina Moglia, Andrea Calvo and Adriano Chiò
Affiliation: “Rita Levi Montalcini” Department of Neuroscience, University of Turin, 10126 Turin, Italy
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: J. Clin. Med. 2023, 12(6), 2367; https://doi.org/10.3390/jcm12062367
Publication Year and Month: 2023 03

Abstract: Background: Motor neuron diseases (MNDs) are fatal neurodegenerative diseases. Biomarkers could help with defining patients’ prognoses and stratifications. Besides neurofilaments, chitinases are a promising family of possible biomarkers which correlate with neuroinflammatory status. We evaluated the plasmatic levels of CHI3L1 in MNDs, MND mimics, and healthy controls (HCs).

Methods: We used a sandwich ELISA to quantify the CHI3L1 in plasma samples from 44 MND patients, 7 hereditary spastic paraplegia (HSP) patients, 9 MND mimics, and 19 HCs. We also collected a ALSFRSr scale, MRC scale, spirometry, mutational status, progression rate (PR), blood sampling, and neuropsychological evaluation.

Results: The plasma levels of the CHI3L1 were different among groups (p = 0.005). Particularly, the MND mimics showed higher CHI3L1 levels compared with the MND patients and HCs. The CHI3L1 levels did not differ among PMA, PLS, and ALS, and we did not find a correlation among the CHI3L1 levels and clinical scores, spirometry parameters, PR, and neuropsychological features. Of note, the red blood cell count and haemoglobin was correlated with the CHI3L1 levels (respectively, p < 0.001, r = 0.63; p = 0.022, and r = 0.52).

Conclusions: The CHI3L1 plasma levels were increased in the MND mimics cohort compared with MNDs group. The increase of CHI3L1 in neuroinflammatory processes could explain our findings. We confirmed that the CHI3L1 plasma levels did not allow for differentiation between ALS and HCs, nor were they correlated with neuropsychological impairment.

Keywords: biomarker; chitinases; cognitive impairment; differential diagnosis; early diagnosis; MND mimics; red blood cells

Conclusions: The measurement of the plasmatic levels of CHI3L1 could be useful in the differential diagnosis between MNDs and MND mimics. This is an important issue, since the early diagnosis of an MND is a determinant in the early starting of neuroprotective therapy and in clinical trial recruitment.
Further multicentre and longitudinal studies on a larger patient cohort, testing alongside other fluid biomarkers, are needed to better explain the role of CHI3L1 in the diagnosis and prognosis of MNDs and, also, of MND mimics.

Outcome of Research: More research required

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any): This article is helpful for any medical/neurologist trying to determine differential diagnosis of ALS vs post-polio.

Link to Paper (if available): Click here to view full text or to download


Category: Brain, Diagnosis and Management, Late Effects of Polio, Post-Polio Motor Unit

Title: Spinal cord gray matter atrophy is associated with functional decline in post-polio syndrome
Author: Maria Janina Wendebourg (1,2), Matthias Weigel (1,2,3,4,5), Laura Richter (1), Vanya Gocheva (6), Patricia Hafner (6), Anna-Lena Orsini (6), Valentina Crepulja (1,2), Simone Schmidt (6), Antal Huck (4), Johanna Oechtering (1), Maria Blatow (7), Tanja Haas (3,4), Cristina Granziera (1,2,5), Ludwig Kappos (1,2,5), Philippe Cattin (4), Oliver Bieri (3,4) Dirk Fischer (6), Regina Schlaeger (1,2,5)
Affiliation: 1. Neurology Clinic and Policlinic, Department of Clinical Research, University Hospital Basel, University of Basel, Basel, Switzerland

2. Translational Imaging in Neurology (ThINk), Department of Biomedical Engineering, University of Basel, Basel, Switzerland

3. Division of Radiological Physics, Department of Radiology, University Hospital Basel, Basel, Switzerland

4. Department of Biomedical Engineering, University of Basel, Basel, Switzerland

5. MS Center and Research Center for Clinical Neuroimmunology and Neuroscience Basel (RC2NB), University Hospital Basel and University of Basel, Basel, Switzerland

6. Division of Pediatric Neurology, University of Basel Children's Hospital, Basel, Switzerland

7. Department of Neuroradiology, Clinical Neuroscience Center, University Hospital Zurich, University of Zurich, Zurich,
Journal: European Journal of Neurology
Citation: Eur J Neurol. 2022;00:1–11.
DOI: 10.1111/ene.15261
Publication Year and Month: 2022 01

Abstract: Objective: To determine if patients with post- polio syndrome (PPS) show spinal cord gray matter (SCGM) atrophy and to assess associations between SCGM atrophy, muscle strength and patient- reported functional decline.

Methods: Twenty patients diagnosed with PPS (March of Dimes criteria) and 20 age- and sex- matched healthy controls (HC) underwent 3T axial 2D- rAMIRA magnetic resonance imaging at the intervertebral disc levels C2/C3–C6/C7, T9/T10 and the lumbar enlarge-ment level (Tmax) (0.5 × 0.5 mm2 in- plane resolution). SCGM areas were segmented manu-ally by two independent raters. Muscle strength, self-reported fatigue, depression and pain measures were assessed.

Results: Post- polio syndrome patients showed significantly and preferentially re-duced SCGM areas at C2/C3 (p= 0.048), C3/C4 (p= 0.001), C4/C5 (p< 0.001), C5/C6 (p= 0.004) and Tmax (p= 0.041) compared to HC. SCGM areas were significantly associated with muscle strength in corresponding myotomes even after adjustment for fatigue, pain and depression. SCGM areaTmax together with age and sex explained 68% of ankle dorsiflexion strength variance. No associations were found with age at or time since infection. Patients reporting PPS- related decline in arm function showed significant cervical SCGM atrophy compared to stable patients adjusted for initial disease severity.

Conclusions: Patients with PPS show significant SCGM atrophy that correlates with mus-cle strength and is associated with PPS- related functional decline. Our findings suggest a secondary neurodegenerative process underlying SCGM atrophy in PPS that is not ex-plained by aging or residua of the initial infection alone. Confirmation by longitudinal studies is needed. The described imaging methodology is promising for developing novel imaging surrogates for SCGM diseases.

Conclusions: The rAMIRA approach is a novel, promising, clinically feasible and sensitive method for segment-wise quantitation of GM atrophy in the cervical and thoracic SC in patients with lower motor neuron disorders. This study demonstrated its clinical applicability and vali-dated it in patients with PPS, a presumed pure, lower motor neuron disorder, which can serve as a model for other neurodegenerative, genetic or autoimmune diseases of the SCGM.

Patients with PPS show significant SCGM atrophy, particularly at levels close to the cervical and lumbar enlargements. Even after adjustment for the level of depression, fatigue and pain, potential confounding symptoms frequently observed in PPS, SCGM atrophy is significantly and segment-wise associated with muscle strength in corresponding myotomes. Moreover, SCGM atrophy is associated with patient-reported PPS-related functional decline. Secondary analyses suggest that SCGM atrophy is rather due to a second dis-ease phase than being a sole residuum of the initial infection or a pure aging effect. These observations support the hypothesis of a focally accentuated neurodegenerative process in the SC underlying PPS. Larger, ideally multicentric, longitudinal studies conducted over a sufficiently long timespan are an important next step to confirm our results and gain more insights into the development of SCGM atrophy over time and its correlation to clinical symptom evolution in patients with PPS.

Outcome of Research: More research required

Availability of Paper: The full text of this paper has been generously made available by the publisher.

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Category: Diagnosis and Management, Late Effects of Polio

Title: Knowledge of healthcare professionals about poliomyelitis and postpoliomyelitis: a cross-sectional study
Author: Claudio Andre Barbosa de Lira (I), Douglas Assis Teles Santos (II), Ricardo Borges Viana (III), Juliana Moreira Guimarães (IV), Jéssica Nathalia Soares Oliveira (V), Bolivar Saldanha Sousa (VI), Marcos Gonçalves de Santana (VII), Rodrigo Luiz Vancini (VIII),
Marília Santos Andrade (IX), Pantelis Nikolaidis (X), Thomas Rosemann (XI), Beat Knechtle (XII)
Affiliation: (I) BSc, PhD. Associate Professor, Faculdade de Educação Física e Dança (FEFD), Universidade Federal de Goiás (UFG), Goiânia (GO), Brazil. https://orcid.org/0000-0001-5749-6877

(II) BSc, MSc. Assistent Professor, Colegiado de Educação Física, Universidade do Estado da Bahia (UNEB), Teixeira de Freitas (BA), Brazil. https://orcid.org/0000-0002-7664-5468

(III) BSc, PhD. Professor, Escola Superior de Educação Física e Fisioterapia do Estado de Goiás (ESEFFEGO), Universidade Estadual de Goiás (UEG), Goiânia (GO), Brazil. https://orcid.org/0000-0001-9200-3185

(IV) BSc. Nurse, Unidade Acadêmica Especial de Ciências da Saúde, Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0002-6729-0335

(V) BSc. Biomedic, Unidade Acadêmica Especial de Ciências da Saúde (UA-CISAU),
Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0002-9531-4277

(VI) MD, PhD. Physician, Hospital Israelita Albert Einstein, São Paulo-Brazil, São Paulo (SP), Brazil. https://orcid.org/0000-0001-9682-5987

(VII) BSc, PhD. Associate Professor, Unidade Acadêmica Especial de Ciências da Saúde (UA-CISAU), Universidade Federal de Jataí (UFJ), Jataí (GO), Brazil. https://orcid.org/0000-0001-7674-3263

(VIII) BSc, PhD. Adjunct Professor, Centro de Educação Física e Desportos, Universidade Federal do Espírito Santo (UFES), Vitória (ES), Brazil. https://orcid.org/0000-0003-1981-1092

(IX) PhD. Physical Therapist and Associate Professor, Department of Physiology, Universidade Federal de São Paulo (USP), São Paulo (SP), Brazil. https://orcid.org/0000-0002-7004-4565

(X) BSc, PhD. Professor, School of Health and Caring Sciences, University of West Attica, Athens, Greece. https://orcid.org/0000-0001-8030-7122

(XI) MD, PhD. Physician and Professor, Institute of Primary Care, University of Zurich, Zurich, Switzerland. https://orcid.org/0000-0002-6436-6306

(XII) MD, PhD. Physician and Scientific Assistant, Institute of Primary Care, University of Zurich, Zurich, Switzerland; and Researcher, Medbase St. Gallen Am Vadianplatz, St. Gallen, Switzerland https://orcid.org/0000-0002-2412-9103
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Sao Paulo Med. J.

139 (5) • Aug-Sep 2021
https://doi.org/10.1590/1516-3180.2020.0617.16032021
Publication Year and Month: 2021 08

Abstract: BACKGROUND:
Postpoliomyelitis syndrome is a clinical condition that can affect poliomyelitis survivors.

OBJECTIVE:
Our aim was to evaluate knowledge of poliomyelitis and postpoliomyelitis syndrome among Brazilian healthcare professionals.

DESIGN AND SETTING:
Cross-sectional study conducted at a Brazilian public higher education institution located in the state of Goiás.

METHODS:
The participants (n = 578) were Brazilian physicians, physical therapists, nurses, nutritionists and psychologists. A self-administered questionnaire (30 questions) was designed to probe knowledge about poliomyelitis and postpoliomyelitis syndrome. From the questionnaire, we created a structured test to objectively evaluate the knowledge of these professionals. The test was composed of 20 questions and was scored over a range from 0 (totally ill-informed) to 20 (totally well-informed).

RESULTS:
In general, the physicians, physical therapists and nurses demonstrated better understanding of poliomyelitis and postpoliomyelitis syndrome. The healthcare professionals who had received previous information about poliomyelitis and postpoliomyelitis syndrome had significantly higher scores than those who had never received information (P < 0.001). On average, this difference was approximately 28.6%.

CONCLUSIONS:
The findings from the present study indicate that there is a critical need for improvement of knowledge about postpoliomyelitis syndrome among Brazilian healthcare professionals. The services provided by these professionals may therefore become compromised. Furthermore, public healthcare initiatives should be implemented to improve knowledge among healthcare professionals.

KEYWORDS (MeSH terms):
Poliomyelitis; Physicians; Nervous system; Postpoliomyelitis syndrome; Knowledge

AUTHORS’ KEY WORDS:
Medical education; Neuromuscular disease; Late effects of polio

Conclusions: Our study showed that, overall, there is a lack of knowledge about PPS and poliomyelitis, especially among psychologists and nutritionists. Therefore, the services provided by these professionals may become compromised. Furthermore, government initiatives should be implemented to increase knowledge among healthcare professionals.

Outcome of Research: Not applicable

Availability of Paper: The full text of this paper has been generously made available by the publisher.

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Category: Diagnosis and Management, Late Effects of Polio

Title: Post-polio syndrome and the phantom of acute previous poliomyelitis: a systemic entity
Author: Jacqueline Stephanie Fernandes do Nascimento (1), Nicolle dos Santos Moraes Nunes (1), Thais de R. Bessa-Guerra (1*), Marco Antonio Alves Azizi (2), Adalgiza Mafra Moreno (2), Antônio Marcos da Silva Catharino (2), Paulo Henrique de Moura (2), Thiago Rodrigues Gonçalves (2), Renata R. T. Castro (2), Acary Souza Bulle Oliveira (3), Marcos RG de Freitas (4) and Marco Orsini (2)
Affiliation: (1) Graduanda de Medicina na Universidade lguaçu (UNIG) - Nova lguaçu, RJ -Brasil

(2) Professor titular na Universidade lguaçu (UNIG). Nova lguaçu, RJ -Brasil

(3) Neurologista, Doutor em Neurologia pela Escola Paulista de Medicina (UNIFESP) - São Paulo (SP) - Brasil

(4) Neurologista, Doutor, Professor Titular e Chefe do Serviço de Neurologia da UFF, Niterói-RJ - Brasil
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: International Journal of Current Research

Vol. 13, Issue, 04
Publication Year and Month: 2021 04

Abstract: Introduction:
Poliomyelitis, often referred to as infantile paralysis or polio, was first described in 1840 by Jakob Von Heine, a German orthopedic physician. It is an acute and infectious disease caused by an enterovirus of worldwide distribution. With the implementation of immunization, the incidence of cases has reduced exponentially worldwide. However, a portion of individuals who have already developed polio, are now manifesting the late effects of polio, called post polio syndrome (PPS).

Case Report:
PRF, male, system analyst. Diagnosis of acute previous polio at the 18th month of age in a hospital in Fortaleza in 1965. At the age of 18 he started to present a new clinic of muscle fatigue, weakness and inability to perform his daily activities.

Discussion:
The Post-polio syndrome (PPS) was first reported in 1875, by Raymond, when reporting the case of a 19-year-old man, previously infected with acute previous polio and at that time had presented a new clinic of muscle weakness and atrophy. Post-poliomyelitis syndrome is defined as a presentation of the delayed effects of polio with the appearance of new neuromuscular symptoms that occur at least 15 years after clinical and functional stability in the natural history of patients with a previous history of acute previous polio. It can be characterized by: new muscle weakness, atrophy and pain, fatigue, sleep disorders, joint pain, cold intolerance, recent weight gain, respiratory distress and dysphagia. Criteria that support the diagnosis are used, such as: (1) Having been affected with acute previous polio; (2) at least 15 years of clinical stability; (3) new clinic of muscle weakness and fatigue; (4) complete or partial recovery of the functions lost in the poliomyelitis period, with a subsequent decline; (5) no other clinical conditions that explain the case. However, effective monitoring by a multidisciplinary team is necessary to assess the degree of functional and motor deterioration, respiratory capacity, as well as the psychological assessment of the patient.

Conclusion:
Although acute previous poliomyelitis has been eradicated in Brazil, as well as in several countries in the world, it is estimated that today there are still about 12 million individuals with poliomyelitis after-effects.

Conclusions: Although acute previous polio has been eradicated in Brazil, as well as in several countries in the world, it is estimated that today there are still about 12 million individuals with poliomyelitis after-effects. These patients overload the health system, especially those diagnosed with post-polio syndrome. Therefore, studies in different areas of health sciences are extremely important for understanding the pathophysiological mechanisms and with the aim of improving the quality of life of these patients.

Studies that address the biochemical and physiological issue can be useful for understanding the neurodegeneration that occurs in PPS, in addition to composing material for the study of the repercussion of a physical / motor after-effect on one or more organic systems and on functional capacity.

Outcome of Research: More research required

Availability of Paper: The full text of this paper has been generously made available by the publisher.

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Category: Diagnosis and Management

Title: Lipid metabolic pathways converge in motor neuron degenerative diseases
Author: Olivia J Rickman, Emma L Baple, and Andrew H Crosby
Affiliation: Medical Research (Level 4), RILD Wellcome Wolfson Centre, University of Exeter Medical School, Royal Devon and Exeter NHS Foundation Trust, Barrack Road, Exeter, EX2 5DW, UK

Correspondence to: Professor Andrew Crosby Professor of Human Genetics University of Exeter Medical School Medical Research (Level 4) RILD Wellcome Wolfson Centre Royal Devon and Exeter NHS Foundation Trust Barrack Road, Exeter, EX2 5DW, UK E-mail: [email protected]
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Brain. 2020 Apr; 143(4): 1073–1087.
Published online 2019 Dec 18. doi: 10.1093/brain/awz382
Publication Year and Month: 2019 12

Abstract: Motor neuron diseases (MNDs) encompass an extensive and heterogeneous group of upper and/or lower motor neuron degenerative disorders, in which the particular clinical outcomes stem from the specific neuronal component involved in each condition. While mutations in a large number of molecules associated with lipid metabolism are known to be implicated in MNDs, there remains a lack of clarity regarding the key functional pathways involved, and their inter-relationships. This review highlights evidence that defines defects within two specific lipid (cholesterol/oxysterol and phosphatidylethanolamine) biosynthetic cascades as being centrally involved in MND, particularly hereditary spastic paraplegia. We also identify how other MND-associated molecules may impact these cascades, in particular through impaired organellar interfacing, to propose ‘subcellular lipidome imbalance’ as a likely common pathomolecular theme in MND. Further exploration of this mechanism has the potential to identify new therapeutic targets and management strategies for modulation of disease progression in hereditary spastic paraplegias and other MNDs.

Keywords: HSP, MND, cholesterol, mitochondria, lipidome imbalance

Conclusions: The development of new methodologies and approaches are important to delineate the specific subcellular biomarker deficits, such as oxysterol and PE imbalance, in HSP and MND. As well as providing potentially powerful biomarkers of disease, such tools may enable monitoring of treatment efficacy of therapeutics to re-address disease-associated lipidome imbalances. Specific genetic subtypes may be more amenable to treatment at targeting (for example) feedback systems, such as CDCA in CTX, or addressing oxysterol imbalance in SPG5. Genetic subtypes of disease leading to more complex subcellular outcomes may require multiple treatment approaches to address the specific mechanistic basis of each condition, and it may be unlikely that one approach will entail a ‘fix all’ treatment. Ultimately, clearer definition of the subcellular lipidome (and other) biological pathways underlying MND and HSP will pave the way for a more elegant approaches for predicting onset and severity of disease, and for designing and monitoring new therapeutic approaches.

Outcome of Research: More research required

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Category: Diagnosis and Management, Late Effects of Polio

Title: Post-poliomyelitis syndrome (2019)
Author: Eric Chun Pu Chu1 and Kary Ka Wai Lam2
Affiliation: 1New York Chiropractic and Physiotherapy Center, New York Medical Group, Hong Kong, People’s Republic of China
2Downtown Chiropractic Limited, Hong Kong, People’s Republic of China
Correspondence: Eric Chun Pu ChuNew York Chiropractic and Physiotherapy Centre, New York Medical Group, 41/F Langham Place Office Tower, 8 Argyle Street, Mongkok, Hong Kong, People’s Republic of China, Phone: Tel +852 3 594 7844, Fax: Fax +852 3 594 6193, Email [email protected]
Journal: International Medical Case Reports Journal
Citation: 12, 261–264. https://doi.org/10.2147/IMCRJ.S219481

Publication Year and Month: 2019 08

Abstract: Most developed countries eliminated paralytic poliomyelitis (polio) in the 1970s to 1980s. It was believed that after recovery from acute paralytic poliomyelitis, the physical condition of survivors would remain stable for the rest of their lives. However, the elimination of polio does not equate the end of medical management of polio. Hundreds of thousands of polio survivors worldwide are still at risk of developing the late effects of the disease. Here, we report a case of post-polio syndrome who attended our clinic for the presence of new weakness and neuromuscular problems six decades after recovery from paralytic polio. It is essential that health professionals be aware of these conditions and have an understanding of the underlying pathophysiology of the symptoms.

Conclusions: Post-polio syndrome is related to the exhaustion of the motor units that form decades after the polio attack. This case report describes the effectiveness of manual interventions in assisting our patient in restoring the level of function and alleviating pain. The limitation of the current report is that it is just a single case. Further comparison with more existing therapeutic regimens is warranted to clarify these issues.

Outcome of Research: Not applicable

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Category: Clinical Evaluation, Diagnosis and Management, Late Effects of Polio

Title: Post-polio Syndrome: More Than Just a Lower Motor Neuron Disease
Author: Stacey Li Hi Shing, Rangariroyashe H. Chipika, Eoin Finegan, Deirdre Murray, Orla Hardiman, and Peter Bede
Affiliation: Computational Neuroimaging Group, Academic Unit of Neurology, Biomedical Sciences Institute, Trinity College Dublin, Dublin, Ireland
Edited by: Francesca Trojsi, University of Campania, Luigi Vanvitelli Caserta, Italy
Reviewed by: Andrea Romigi, Mediterranean Neurological Institute (IRCCS), Italy; Louisa Ng, The University of Melbourne, Australia
*Correspondence: Peter Bede ei.dct@pedeb
Journal: Frontiers in Neurology
Citation: 10, 773. https://doi.org/10.3389/fneur.2019.00773
Publication Year and Month: 2019 07

Abstract: Post-polio syndrome (PPS) is a neurological condition that affects polio survivors decades after their initial infection. Despite its high prevalence, the etiology of PPS remains elusive, mechanisms of progression are poorly understood, and the condition is notoriously under-researched. While motor dysfunction is a hallmark feature of the condition, generalized fatigue, sleep disturbance, decreased endurance, neuropsychological deficits, sensory symptoms, and chronic pain are also often reported and have considerable quality of life implications in PPS. The non-motor aspects of PPS are particularly challenging to evaluate, quantify, and treat. Generalized fatigue is one of the most distressing symptoms of PPS and is likely to be multifactorial due to weight-gain, respiratory compromise, poor sleep, and polypharmacy. No validated diagnostic, monitoring, or prognostic markers have been developed in PPS to date and the mainstay of therapy centers on symptomatic relief and individualized rehabilitation strategies such as energy conservation and muscle strengthening exercise regimes. Despite a number of large clinical trials in PPS, no effective disease-modifying pharmacological treatments are currently available.

Conclusions: Despite being one of the most devastating neurodegenerative conditions in the world, surprisingly limited research is undertaken in post-polio syndrome. Its pathogenesis remains elusive, no sensitive diagnostic tools have been developed, and validated prognostic and monitoring markers are lacking. Non-motor symptoms of PPS have considerable quality of life implications and are notoriously challenging to manage. The etiology of fatigue in PPS is yet to be elucidated and successful individualized management strategies are needed to maintain mobility, independence, and patient autonomy. There is striking a paucity of neuroimaging studies in PPS that could provide anatomical insights into the substrate of extra-motor symptoms. Ultimately, the characterization of PPS-associated pathology may help research efforts in other motor neuron diseases.

Outcome of Research: More research required

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Comments (if any): This is a good overview of Post-Polio conditions particularly for clinicians new to the topic.

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Category: Diagnosis and Management, Differential Diagnosis, Gender Differences, Late Effects of Polio

Title: Postpolio Syndrome: A Review of Lived Experiences of Patients
Author: Oluwaseyi Jacob Oluwasanmi, Devaunna Andrene Mckenzie, Idowu Oluwasegun Adewole, Christian O Aluka, James Iyasse, Esther Olunu, and Adegbenro Omotuyi Fakoya1
Affiliation: Department of Microbiology, All Saints University School of Medicine, Commonwealth of Dominica, Roseau, Dominica
1Department of Anatomical Sciences, University of Medicine and Health Sciences, Basseterre, St. Kitts and Nevis
Address for correspondence: Dr. Adegbenro Omotuyi Fakoya, University of Medicine and Health Sciences, Basseterre, St. Kitts and Nevis. E-mail: [email protected]
Journal: International Journal of Applied & Basic Medical Research
Citation: 9(3), 129–134. https://doi.org/10.4103/ijabmr.IJABMR_333_18
Publication Year and Month: 2019 07

Abstract: Postpolio syndrome (PPS) refers to a group of conditions that are present in patients, years after recovery from initial acute paralytic poliomyelitis. About 15%–80% of 20 million polio survivors worldwide will experience exacerbation of symptoms which typically appear 15–30 years after the resolution of initial poliomyelitis. Symptoms include new muscle weakness, fatigue, myalgia, joint pain, dysphagia, and difficulty breathing. Other reported symptoms include cold intolerance, sleep disorder, dysphonia, loss of stamina, musculoskeletal deformities, cardiovascular disorders, psychosocial problems, and restless legs syndrome. These symptoms are attributed to the superimposed neuronal loss of aging with inflammatory mechanisms, but without any convincing evidence of viral reactivation. Risk factors include female gender, respiratory symptoms, normal aging, permanent disability caused by motor neuron damage, muscle overuse and disuse, aging, and immunologic mechanisms. Hypothyroidism-induced myopathy and fibromyalgia are a differential diagnosis for PPS, and exclusion diagnosis is required as confirmatory criteria for PPS. The symptoms of PPS presented determine the course of management.

Keywords: Fatigue, muscle weakness, myalgia, poliomyelitis, postpolio syndrome

Conclusions: PPS is a sequela of acute poliomyelitis which occurs decades after resolution of the initial paralytic or nonparalytic polio. Fatigue, muscle weakness, myalgia, and joint pain are the major symptoms of PPS. Diagnosis of PPS is made based on the exclusion of other conditions that can cause similar symptoms. The quality of life of patients with PPS is significantly affected by the reoccurrence of the constellations of symptoms they experience. Proper assessment and treatment should be performed to prevent severe impairment of function in patients.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Identification of targets for improving access to care in persons with long term physical disabilities
Author: Jennifer L.Wong, Kevin N.Alschuler, Tracy M.Mroz, Kimberly P.Hreha, Ivan R.Molton
Affiliation: University of Washington, Rehabilitation Medicine, United States
Journal: Disability and Health Journal
Citation: Available online 20 January 2019
Publication Year and Month: 2019 01

Abstract: Background
People with long-term physical disability (LTPD) continue to experience difficulties in accessing health care despite the focus of highlighting disparities in the last two decades.

Objectives
To describe health care utilization, accommodations and barriers experienced while accessing health care, and reasons why individuals delay or skip health care among people with LTPD.

Methods
The current study was a part of a larger longitudinal survey administered to individuals with physical disability associated with one of four long-term conditions (MS; SCI; PPS; MD). Measures included demographics, health care utilization, barriers to health care, and reasons for delaying or skipping medical care from the sixth wave of data from 2015 to 2016.

Results
Roughly 90% of all participants (N = 1159) saw at least one medical provider within 12 months. The most encountered barrier participants reported experiencing within that time was an office that did not have a safe transfer device to move them to an exam table (69%). Participants’ physical function, quality of life, status of living with a spouse, diagnostic condition, and sex (male) were significantly associated with endorsing a barrier in accessing health care. The inability to afford out of pocket expenses was the highest reported reason for delaying health care.

Conclusions: People with LTPD access a variety of health care, including rehabilitation services, and continue to experience barriers when doing so. While understanding barriers individuals experience when accessing health care is important, it is equally important to document the type of care they delay or skip due to barriers.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Safety and feasibility of transcranial direct current stimulation for patients with post-polio syndrome
Author: Y. Matsushima, A. Hachisuka, H. Itoh, K. Sugimoto, S. Saeki
Affiliation: Department of rehabilitation medicine, University of occupational and environmental health, Japan
Journal: Brain Stimulation
Citation: (2019) 385-592
Publication Year and Month: 2019

Abstract: Post-polio syndrome (PPS) is generally defined as a clinical syndrome consisting of new muscle weakness, fatigue, and pain in poliomyelitis survivors. In PPS, there is no definitively validated treatment option, although Acler M et al. reported that anodal transcranial direct current stimulation (tDCS) over pre-motor cortex for 15 days improved sleep and fatigue symptoms in patients with PPS. tDCS may be a valuable, non-invasive new tool for managing patients with PPS.

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Estimation of the Direct Cost of Poliomyelitis Rehabilitation Treatment to Pakistani Patients: A 53-Year Retrospective Study
Author: Atta Abbas Naqvi, Syed Baqir Shyum Naqvi, Fatima Zehra, Ashutosh Kumar Verma, Saman Usmani, Sehrish Badar, Rizwan Ahmad, Niyaz Ahmad
Affiliation: 1.Department of Pharmacy Practice, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
2.Faculty of PharmacyHamdard UniversityKarachiPakistan
3.Applied Economics Research CentreUniversity of KarachiKarachiPakistan
4.Discipline of Social and Administrative Pharmacy, School of Pharmaceutical SciencesUniversiti Sains MalaysiaMindenMalaysia
5.Institute of Pharmaceutical Sciences, Jinnah Sindh Medical UniversityKarachiPakistan
6.Natural Products and Alternative Medicines, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
7.Department of Pharmaceutics, College of Clinical PharmacyImam Abdulrahman Bin Faisal UniversityDammamSaudi Arabia
Journal: Applied Health Economics and Health Policy
Citation: December 2018, Volume 16, Issue 6, pp 871–888
Publication Year and Month: 2018 12

Abstract: Background
Pakistan is one of the last few countries in which poliomyelitis is endemic. Evidence indicates that out-of-pocket expenditures are a barrier to polio rehabilitation treatment, yet there are no reported figures related to the financial burden of this disease on patients in a recently polio-endemic country.

Objective
This study investigated direct costs attributed to rehabilitation treatment of poliomyelitis among Pakistani patients and reported its duration along with the socioeconomic status of poliomyelitis survivors.

Conclusions: Conclusion
The cost of poliomyelitis rehabilitation in Pakistan is high; it has an economic effect on the lives of patients and their families. Despite good education, polio survivors in Pakistan appear to have low socioeconomic status, lower chances of employment and marriage, as well as fewer children. Further research is recommended to explore the burden of disease on society, i.e., indirect costs and suffering.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: The split hand syndrome in ALS and post-polio-syndrome
Author: M.Hoheisel, L.Burmeister, M.Tesch
Affiliation: Schlosspark-Klinik Berlin, Neurology, Berlin, Germany
Journal: Clinical Neurophysiology
Citation: Volume 129, Issue 8, August 2018, Pages e82-e83

Publication Year and Month: 2018 08

Abstract: Introduction
Electrodiagnostic evaluation for amyotrophic lateral sclerosis (ALS) relies on extensive measurements. As one diagnostic clue, the split-hand-index (SHI) was proposed. It compares the compound muscle action potential (CMAP) of the abductor pollicis brevis (APB) muscle with the CMAP of the abductor digiti minimi (ADM) muscle.

In ALS, asymmetric atrophy of APB and ADM results in the index being reduced compared to the healthy population. This holds true despite the fact, that there is the same segmental innervation C8 for both examined muscles, as was previously discussed. Several studies have shown a diagnostic value in differentiating ALS from other motorneuron-diseases by means of the SHI, claiming a specific form of neurodegeneration in ALS, which is less marked for example in lower motor neuron disease (LMND), spinal muscle atrophy (SMA) or Hirayama disease.

In our study, we aimed to compare the SHI of ALS-patients with our cohort of patients with post-polio-syndrome (PPS) to find out, whether it has a discriminative value in these patients too and to add knowledge to the proposed neuroscientific explanations of asymmetric thenar/hypothenar-atrophy.

Methods
We conducted a retrospective analysis of our post-polio cohort since 1997. All patients were screened whether CMAPs of APB and ADM were collected. For comparison, we screened electrodiagnostic reports of all patients with a diagnosis of ALS for collected CMAPs of APB and ADM. We excluded patients with neuropathy of the median nerve (NMN) by means of a prolonged distal motoric latency (>4,4 ms). Finally we randomly chose the same number of patients from our reports in that time period with normal results (NR), by excluding diagnosis of NMN, acute polyneuropathies and radiculo- and plexopathies of the arm. We calculated the SHI by dividing the CMAP of APB by the CMAP of ADM.

Results
We found a significant difference (p = 0,01) of the SHI between ALS patients (0,97 ± 0,84) and the NR-group (1,26 ± 0,72). The SHI of the PPS-group (0,91 ± 0,55) was not significantly different compared to the NR group (p = 0,08), but showed a trend. Comparing the ALS-group with the PPS-patients, we found no statistically relevant difference (p = 0,83).

Conclusions: As expected, we could reproduce a significant decrease of the SHI in ALS patients. However our results showed no statistically relevant difference between ALS-patients and PPS-patients when comparing the SHI. There is a broader distribution of values in the PPS-group, including cases of very high grade asymmetric atrophy of APB and ADM. A low SHI therefore is of no help in differentiating PPS from ALS-patients.

As a limitation of our study it is important to acknowledge the retrospective study type and a possible selection bias of patients who suffer from an already clinically visible asymmetry, possible increasing electrodiagnostic evaluation numbers of the hand.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Postpolio syndrome and the late effects of poliomyelitis. Part 1. pathogenesis, biomechanical considerations, diagnosis, and investigations.
Author: Lo JK, Robinson LR.
Affiliation: Sunnybrook Health Sciences Centre, Division of Physical Medicine and Rehabilitation, Department of Medicine, University of Toronto, 2075 Bayview Avenue, Toronto, Ontario, Canada
Journal: Muscle & Nerve
Citation: https://doi.org/10.1002/mus.26168
Publication Year and Month: 2018 05

Abstract: Postpolio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. Many theories exist regarding the pathogenesis of PPS, which remains incompletely understood. In contrast, the late effects of poliomyelitis are often a consequence of biomechanical alterations that occur as a result of polio‐related surgeries, musculoskeletal deformities, or weakness. Osteoporosis and fractures of the polio‐involved limbs are common. A comprehensive clinical evaluation with appropriate investigations is essential to fulfilling the established PPS diagnostic criteria. PPS is a diagnosis of exclusion in which a key clinical feature required for the diagnosis is new muscle weakness and/or muscle fatigability that is persistent for at least 1 year. Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS.

Conclusions: Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Post‐polio syndrome and the late effects of poliomyelitis: Part 2. treatment, management, and prognosis.
Author: Lo JK, Robinson LR.
Affiliation: Sunnybrook Health Sciences Centre, Division of Physical Medicine and Rehabilitation, Department of Medicine, University of Toronto, 2075 Bayview Avenue, Toronto, Ontario, M4N 3M5, Canada
Journal: Muscle & Nerve
Citation: https://doi.org/10.1002/mus.26167
Publication Year and Month: 2018 05

Abstract: Post‐polio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. An individualized approach to rehabilitation management is critical. Interventions may include rehabilitation management strategies, adaptive equipment, orthotic equipment, gait/mobility aids, and a variety of therapeutic exercises. The progression of muscle weakness in PPS is typically slow and gradual; however, there is also variability in both the natural history of weakness and functional prognosis. Further research is required to determine the effectiveness of selected medical treatment. Muscle Nerve 58:760–769, 2018

Conclusions: The progression of muscle weakness in PPS is typically slow and gradual; however, there is also variability in both the natural history of weakness and functional prognosis.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Repetitive transcranial magnetic stimulation in treatment of post polio syndrome
Author: Pastuszak, Z., Piusaska-Macoch, R., Stepieri, A., Czernicki, Z.,
Affiliation: Department of Neurosurgery, Mossakowski Medical Research Centre, Polish Academy of Sciences, A. Pawińskiego 5, 02-106 Warsaw, Poland

Department of Neurology, Military Institute of Medicine, Szaserów 128, 04-141 Warsaw, Poland

Department of Neurosurgery, Warsaw University of Medicine, Cegłowska 80, 01-809 Warsaw, Poland
Journal: Neurologia i Neurochirurgia Polska
Citation: Volume 52 (2): 2018, Pages 281-284
Publication Year and Month: 2018 03

Abstract: Post polio syndrome is a rare disease that occurs decades after polio virus infection. Repetitive transcranial magnetic stimulation (rTMS) is a treatment option with proved effectiveness in drug resistant depression. Possibly it can be helpful in therapy of other neurological diseases including post polio syndrome.

Conclusions: rTMS can be an effective method in treatment of post polio syndrome but further studies with larger group need to be done to confirm that data.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Sense of Coherence in persons with late effects of polio
Author: Nolvi M, Brogardh C, Jacobsson L, Lexell J
Affiliation: Maria Nolvi, MD, Department of Health Sciences, PO Box 157, Lund University, SE-221 00 Lund, Sweden
Journal: NeuroRehabilitation
Citation: Vol. 42, no. 1, pp. 103-111, 2018
Publication Year and Month: 2018 01

Abstract: BACKGROUND:Sense of Coherence (SOC) is important for successful adaptation and mental well-being in people with life-long medical conditions. Late effects of polio (LEoP) often lead to a life-long disability, but no study has assessed SOC in this population. OBJECTIVE:To assess SOC in persons with LEoP and to explore the association between SOC, demographics (age, gender, marital status and level of education) and variables related to LEoP (age at polio onset, number of years from polio until onset of LEoP and self-rated disability). METHOD:Ninety-three community-dwelling persons with clinically verified LEoP responded to a postal survey with the Sense of Coherence Scale (SOC-13). A hierarchical multiple regression analysis was performed to explore the associations with SOC. RESULTS:SOC varied considerably among the participants. The mean and median SOC-13 total sum score was 71.8 and 76 points, which is similar to age-matched non-disabled people. The number of years before onset of LEoP and self-rated disability together with the participants’ marital status and level of education explained 37% (p < 0.001) of the variance in SOC.

Conclusions: CONCLUSION:Persons with LEoP have a level of sense of coherence (SOC) indicating that they generally have the ability to understand, handle and being motivated when dealing with stressful events and problems arising in their lives as a result of their disability. Being married and having a higher education, living many years before onset of LEoP and perceiving a mild to moderate disability contributed to a strong SOC.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Inverse Relationship Between Polio Incidence in the US and Colorectal Cancer.
Author: STEVEN LEHRER and PETER H RHEINSTEIN
Affiliation: Department of Radiation Oncology, Icahn School of Medicine at Mount Sinai, New York, U.S.A. [email protected].
Severn Health Solutions, Severna Park, MD, U.S.A.
Journal: IN VIVO
Citation: (Athens, Greece) vol. 32,6 (2018): 1485-1489.
Publication Year and Month: 2018

Abstract: BACKGROUND/AIM:
Polio is predominantly an enteric viral infection that was progressively eradicated in the United States after the introduction of polio vaccine in the early 1950s. U.S. colorectal cancer rates have dropped steadily for individuals born between 1890 and 1950, but have been increasing for every generation born since 1950. Moreover, the lowest worldwide age adjusted rates of colorectal cancer in 2012 were in sub-Saharan Africa, Gambia and Mozambique, where polio has not been eradicated. In the current study, poliomyelitis incidence in US states before the introduction of polio vaccine was analyzed.

MATERIALS AND METHODS:
Reported cases of poliomyelitis per 100,000 population by state 1932-1951 were from Centers for Disease Control. Colorectal cancer deaths per 100,000 in men (2005-2009) by US State are from the American Cancer Society. US state overweight and obesity data are from the Centers for Disease Control and Prevention (CDC). Smoking data are from the CDC.

RESULTS:
By US state, colorectal cancer incidence per 100,000 in men for 2005-2009 was inversely correlated with reported cases of poliomyelitis per 100,000 for 1932-1951 (r=-0.311, p=0.032). Colorectal cancer deaths per 100,000 in men in 2005-2009 were also inversely correlated with reported cases of poliomyelitis per 100,000 by state for 1932-1951 (r=-0.493, p<0.001). The relationship between colorectal cancer deaths and polio incidence was significant (β=-0.196, p=0.028) and independent of the effects of smoking (β=0.289, p=0.012) and overweight (β=0.547, p<0.001). The relationship in females with colorectal cancer was identical.

Conclusions: Polio virus infection of cells of the colon may induce some degree of resistance to the development of colon cancer decades later. The effect of polio virus infection seems to be especially potent in reducing the rate of death from colon cancer.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Neurological Symptoms in Danes with a History of Poliomyelitis: Lifelong Follow-Up of Late Symptoms, their Association with Initial Symptoms of Polio, and Presence of Postpolio Syndrome
Author: Kay L. Nielsen N.M. Wanscher B. Jennum P.
Affiliation: Specialized Hospitalet for Polio and Accident Patients, Rødovre, Denmark
Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark
Medical Department 3, Næstved, Slagelse and Ringsted Hospital, Slagelse, Denmark
Danish Center for Sleep Medicine, Department of Clinical Neurophysiology, Rigshospitalet, University of Copenhagen, Glostrup, Denmark
Journal: European Neurology
Citation: 2018, Vol.80, No. 5-6
Publication Year and Month: 2018

Abstract: Background: Previous studies suggest that patients with a history of poliomyelitis (PM) later in life experience a variety of symptoms. These studies were carried out in patients who later in life were admitted to hospital or became members of polio societies and may therefore not be representative of all polio patients. Little data have been published concerning patients actually discharged from hospital with a diagnosis of acute paralytic PM. Objectives: The aim of this study was to compare the prevalence of late symptoms in individuals with a history of paralytic PM with that of controls, and to study whether late symptoms in individuals with a history of PM were associated with symptoms at the acute stage of polio, and finally to compare the prevalence of symptoms in polio patients with postpolio syndrome (PPS) with the prevalence of symptoms in polio patients without PPS. Methods: A questionnaire concerning various symptoms was sent to a previously established cohort of patients, who during the polio epidemics were discharged from the Department of Infectious Disease at Blegdamshospitalet, Copenhagen, with a diagnosis of paralytic PM, and to age- and gender-matched controls without PM. Information about symptoms at the acute stage of disease was obtained from hospital records. Logistic regression analysis with adjustment for age and gender was applied to compare the occurrence of late symptoms in cases and controls and within the above-mentioned groups of individuals with a history of PM. Results: (i) Compared with controls, individuals with a history of polio significantly more often reported muscle symptoms, pain, neuropathic sensory symptoms, and bulbar symptoms; (ii) the occurrence of symptoms did not seem to be related to symptoms of the initial PM; and (iii) symptom prevalence was significantly higher in individuals with a history of polio who reported PPS as compared with those who did not.

Conclusions: Conclusion: Our data indicate that individuals with a history of PM late in life experience a variety of symptoms that cannot be attributed to lesions of the anterior horn. Furthermore, late symptoms do not seem to be related to initial symptoms of the acute stage of PM but to reported PPS. The last finding supports the perception that the cause of PPS is not just normal ageing.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Polio survivors perceptions of a multi-disciplinary rehabilitation programme
Author: Atwal, A., Duncan, H., Queally, C., Cedar, S.H.
Affiliation: Nil
Journal: Disability and Rehabilitation
Citation: Atwal, A., Duncan, H., Queally, C., Cedar, S.H. (2017) Polio survivors perceptions of a multi-disciplinary rehabilitation programme. Disability and Rehabilitation. DOI: 10.1080/09638288.2017.1381184
Publication Year and Month: 2017 10

Abstract: Purpose: Post-polio syndrome refers to a late complication of the poliovirus infection. Management of post-polio syndrome is complex due to the extensive symptomology. European and United Kingdom guidelines have advised the use of rehabilitation programmes to manage post-polio syndrome. There is a paucity of research in relation to the effectiveness of rehabilitation interventions. The objective of this study is to explore polio survivor’s perceptions of an in-patient multi-disciplinary rehabilitation programme.

Methods: Semi-structured interviews of community dwelling polio survivors who attended in-patient rehabilitation programme in the United Kingdom. Thematic analysis was used to describe and interpret interview data.

Results: Participants’ experiences were influenced by past experiences of polio and their self-concept. Participants generally had a positive experience and valued being with other polio survivors. Positive strategies, such as pacing and reflection changed their mind-sets into their lives after the programme, though they still faced challenges in daily living. Some participants supported others with post-polio syndrome after completing the programme.

Conclusions: The research identified that participants experienced long term positive benefits from attending a rehabilitation programme. Strategies that users found helpful that explored the effectiveness of interventions to manage polio are not cited within a Cochrane review. If we are to recognise the lived experience and service user empowerment within a model of co- production it is essential that patient preferences are evaluated and used as evidence to justify service provision. Further research is required with polio survivors to explore how best rehabilitation programmes can adopt the principles of co-production.

Implications for Rehabilitation
The patients’ expertise and lived experience must be at the centre of a rehabilitation programme.

Strategies such as pacing and reflection are perceived as important strategies to enable self-management of polio and post-polio syndrome despite the limited evidence base to support these interventions.

Polio rehabilitation programmes should not be time limited and commissioners and therapists need to ensure that follow up support is provided.

When measuring outcomes patient preferences and views must be evaluated.

Outcome of Research: More research required

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Comments (if any): This investigation supports feedback from polio survivors in Australia on the benefits of group-based rehabilitation, especially where there is an opportunity to learn and discuss their rehabilitation with fellow participants.

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Category: Diagnosis and Management

Title: The treatment of fatigue by non-invasive brain stimulation.
Author: Lefaucheur JP, Chalah MA, Mhalla A, Palm U, Ayache SS, Mylius V.
Affiliation: Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Service de physiologie-Explorations fonctionnelles, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, Créteil, France. Electronic address: [email protected].

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Service de physiologie-Explorations fonctionnelles, hôpital Henri-Mondor, Assistance publique-Hôpitaux de Paris, Créteil, France.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Department of Psychiatry and Psychotherapy, Klinikum der Universität München, Munich, Germany.

Faculté de médecine de Créteil, université Paris Est Créteil, Créteil, France; Department of Neurology, Philipps University, Marburg, Germany; Department of Neurology, Center for Neurorehabilitation, Valens, Switzerland.
Journal: Clinical Neurophysiology
Citation: 2017 Apr;47(2):173-184.
Publication Year and Month: 2017 04

Abstract: The use of non-invasive brain neurostimulation (NIBS) techniques to treat neurological or psychiatric diseases is currently under development. Fatigue is a commonly observed symptom in the field of potentially treatable pathologies by NIBS, yet very little data has been published regarding its treatment. We conducted a review of the literature until the end of February 2017 to analyze all the studies that reported a clinical assessment of the effects of NIBS techniques on fatigue. We have limited our analysis to repetitive transcranial magnetic stimulation (rTMS) and transcranial direct current stimulation (tDCS). We found only 15 studies on this subject, including 8 tDCS studies and 7 rTMS studies. Of the tDCS studies, 6 concerned patients with multiple sclerosis while 6 rTMS studies concerned fibromyalgia or chronic fatigue syndrome. The remaining 3 studies included patients with post-polio syndrome, Parkinson's disease and amyotrophic lateral sclerosis. Three cortical regions were targeted: the primary sensorimotor cortex, the dorsolateral prefrontal cortex and the posterior parietal cortex. In all cases, tDCS protocols were performed according to a bipolar montage with the anode over the cortical target. On the other hand, rTMS protocols consisted of either high-frequency phasic stimulation or low-frequency tonic stimulation. The results available to date are still too few, partial and heterogeneous as to the methods applied, the clinical profile of the patients and the variables studied (different fatigue scores) in order to draw any conclusion. However, the effects obtained, especially in multiple sclerosis and fibromyalgia, are really carriers of therapeutic hope.

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Cognitive behavioural therapy for reducing fatigue in post-polio syndrome and in facioscapulohumeral dystrophy: A comparison
Author: Koopman, Fieke S., Merel A. Brehm, Anita Beelen, Nicole Voet, Gijs Bleijenberg, Alexander Geurts, Frans Nollet
Affiliation: Department of Rehabilitation , Academic Medical Center, University of Amsterdam, PO 22660, 1100 DD Amsterdam, The Netherlands. E-mail: [email protected]

Journal: Journal of Rehabilitation Medicine
Citation: 2017; 49: 585–590
Publication Year and Month: 2017

Abstract: Post-polio syndrome (PPS) and facioscapulohumeral dystrophy (FSHD) are two different neuromuscular disorders. Fatigue is a frequent complaint in both disorders. A recent study showed that cognitive behavioral therapy (CBT), which is a type of psychotherapy that helps patients to identify and reshape thoughts and behavior patterns that contribute to the fatigue was effective in alleviating fatigue in FSHD but not in PPS. In this study we investigated whether this difference in effectiveness might be explained by dissimilar fatigue-related thoughts (for example focusing on fatigue) in both conditions. We used questionnaires to measure the fatigue-related thoughts in 21 patients with PPS and 24 patients with FSHD . It appeared that fatigue-related thoughts in PPS were similar to those in FSHD and thus do not explain the difference in effectiveness of CBT.

Conclusions: Fatigue-related thoughts in PPS were similar to those in FSHD and thus do not explain the difference in effectiveness of CBT.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Characteristics of Patients at First Visit to a Polio Clinic in Sweden
Author: Skough Vreede, K. and Sunnerhagen, K.S.
Affiliation: Goteborg University
Journal: PLoS One
Citation: https://doi.org/10.1371/journal.pone.0150286
Publication Year and Month: 2016 03

Abstract: Aim
Describe polio patients visiting a polio clinic in Sweden, a country where vaccination was introduced in 1957.

Design
A consecutive cohort study.

Patients
Prior polio patients.

Methods
All patients (n = 865) visiting the polio clinic at Sahlgrenska University Hospital, Gothenburg Sweden, between 1994 and 2012 were included in this study. Data at first visit regarding patient characteristics, polio classification, data of electromyography, origin, assistive devices and gait speed as well as muscle strength were collected for these patients. Twenty-three patients were excluded because no polio diagnosis could be established. A total of 842 patients with confirmed polio remained in the study.

Results
More than twenty percent of the patients were from countries outside the Nordic region and considerably younger than those from the Nordic region. The majority of the emigrants were from Asia and Africa followed by Europe (outside the Nordic region). Of all patients included ninety-seven percent (n = 817) had polio in the lower extremity and almost 53% (n = 444) had polio in the upper extremity while 28% (n = 238) had polio in the trunk, according to clinical classification of polio. Compared with a sample of the normal population, the polio patients walked 61–71% slower, and were 53–77% weaker in muscle strength of the knee and foot as well as grip strength.

Conclusion
The younger patients with polio emigrating from countries with different cultures may lead to a challenge for the multi professional teams working with post-polio rehabilitation and are of importance when planning for the care of polio patients the coming years.

Conclusions: Polio in lower extremities was more common than polio in upper extremities, verified both
by EMG and clinical classification. This is in accordance with earlier studies. Polio in lower
extremities was also classified as clinically unstable or severely atrophic to a higher extent than
polio in upper extremities (as shown in Fig 1). This is in accordance with an earlier study by
Sandberg et al [20] indicating a more pronounced ongoing denervation-reinnervation process
over time in a lower extremity muscle compared to upper extremity muscle (tibialis anterior
and biceps brachii respectively). The same pattern was also seen in patients studied in Minnesota where patients with leg weakness were twice as likely to complain of new problems compared to those with arm weakness [10].

The ongoing denervation-reinnervation process in patients with PPS results in larger motor
units.When motor-unit size has reached an upper limit, further losses of neurons can no longer
be compensated for and this results in increased muscle weakness [21]. The patients
showed to be stronger in isometric endurance compared to normal population. This may be
explained by the fact that the patients were weaker than the normal population in isometric
peak torque, which the measure of isometric endurance was based on. An increase in type I
(slow) muscle fibres has also been described in prior polio patients [22–23] and may be due to
a transition of type II (fast) to type I (slow).

An important study limitation were seen in the classification of polio as polio were classified
for left and right arm and leg, respectively, and not per muscle group. This can explain why a
polio affected leg in some cases was stronger compared to normal values as the muscles
involved in the strength measured i.e. knee flexion and knee extension muscles may not be
affected of polio. And the same is applicable regarding muscle strength of the foot as well as
grip strength. Data of muscle strength from some of the subjects were missing for different reasons i.e. they may have just not had time to participate, or refused to participate and some were too weak to perform the strength test. Some of the patients with muscle strength data missing, tried to perform the test, but were too weak to get a result. In the future, the use of ultrasound may be used to assess muscle function [24]. This would give the possibility to have more information of muscle function since this does not require that the patient has muscle strength to overcome gravity, which is a requirement for isokinetic testing.

Outcome of Research: Effective

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Category: Diagnosis and Management, Orthoses, Surgery

Title: Polio revisited: reviving knowledge and skills to meet the challenge of resurgence
Author: Benjamin Joseph (1) and Hugh Watts (2)
Affiliation: (1) Aster Medcity, Kochi, Kerala India, 18 HIG HUDCO Colony, Manipal, Karnataka 576104 India

(2) Shriners Hospital for Children, Los Angeles, CA USA
Journal: NEW - PUT DETAILS IN CITATION FIELD
Citation: Journal of Children's Orthopaedics

9 (5): 325–338, doi: 10.1007/s11832-015-0678-4
Publication Year and Month: 2015 09

Abstract: Purpose
To date, polio has not been eradicated and there appears to be a resurgence of the disease. Hence, there is a need to revive decision-making skills to treat the effects of polio.

Methods
Here, we outline the aspects of treatment of paralysis following polio based on the literature and personal experience of the authors. The surgical treatment of the lower and upper extremities and the spine have been reviewed. The scope of bracing of the lower limb has been defined.

Results
The effects of polio can be mitigated by judicious correction of deformities, restoration of muscle balance, stabilising unstable joints and compensating for limb length inequality.

Conclusions
As polio has not been eradicated and there is a risk of resurgence of the disease, paediatric orthopaedic surgeons need to be prepared to deal with fresh cases of polio. Revival of old techniques for managing the effects of paralysis following polio is needed.

Keywords: Poliomyelitis, Resurgence, Surgical decision-making, Bracing, Paralytic deformity

Conclusions: Polio has not been eradicated and there is a risk of resurgence of the disease. Paediatric orthopaedic surgeons need to be prepared to deal with fresh cases of polio. Revival of old techniques of managing the effects of paralysis following polio is needed.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Unraveling the transmission ecology of polio
Author: Martinez-Bakker M (1), King AA (1,2), Rohani P (1,2)
Affiliation: (1) Department of Ecology & Evolutionary Biology, University of Michigan, Ann Arbor, Michigan, United States of America; (2) Center for the Study of Complex Systems, University of Michigan, Ann Arbor, Michigan, United States of America; Fogarty International Center, National Institutes of Health, Bethesda, Maryland, United States of America
Journal: Public Library of Science
Citation: PLoS Biol. 2015 Jun;13(6): e1002172. doi:10.1371/journal.pbio.1002172
Publication Year and Month: 2015 06

Abstract: Sustained and coordinated vaccination efforts have brought polio eradication within reach. Anticipating the eradication of wild poliovirus (WPV) and the subsequent challenges in preventing its re-emergence, we look to the past to identify why polio rose to epidemic levels in the mid-20th century, and how WPV persisted over large geographic scales. We analyzed an extensive epidemiological dataset, spanning the 1930s to the 1950s and spatially replicated across each state in the United States, to glean insight into the drivers of polio’s historical expansion and the ecological mode of its persistence prior to vaccine introduction. We document a latitudinal gradient in polio’s seasonality. Additionally, we fitted and validated mechanistic transmission models to data from each US state independently. The fitted models revealed that: (1) polio persistence was the product of a dynamic mosaic of source and sink populations; (2) geographic heterogeneity of seasonal transmission conditions account for the latitudinal structure of polio epidemics; (3) contrary to the prevailing “disease of development” hypothesis, our analyses demonstrate that polio’s historical expansion was straightforwardly explained by demographic trends rather than improvements in sanitation and hygiene; and (4) the absence of clinical disease is not a reliable indicator of polio transmission, because widespread polio transmission was likely in the multiyear absence of clinical disease. As the world edges closer to global polio eradication and continues the strategic withdrawal of the Oral Polio Vaccine (OPV), the regular identification of, and rapid response to, these silent chains of transmission is of the utmost importance.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Post-poliomyelitis syndrome as a possible viral disease
Author: Baj A (1), Colombo M (1), Headley JL (2), McFarlane JR (3), Liethof MA (4), Toniolo A (5)
Affiliation: (1) Laboratory of Clinical Microbiology, University of Insubria Medical School, Viale Borri 57, 21100 Varese, Italy; (2) Post-Polio Health International, Saint Louis, Missouri, USA; (3) European Polio Union, Huldenberg, Belgium; (4) Polio Australia Incorporated, Kew, Victoria, Australia; (5) Laboratory of Clinical Microbiology, University of Insubria Medical School, Viale Borri 57, 21100 Varese, Italy. Electronic address: [email protected]
Journal: International Journal of Infectious Diseases
Citation: Int J Infect Dis. 2015 May 1;35:107-116. doi: 10.1016/j.ijid.2015.04.018
Publication Year and Month: 2015 05

Abstract: This review summarizes current concepts on post-polio syndrome (PPS), a condition that may arise in polio survivors after partial or complete functional recovery followed by a prolonged interval of stable neurological function. PPS affects 15-20 million people worldwide. Epidemiological data are reported, together with the pathogenic pathways that possibly lead to the progressive degeneration and loss of neuromuscular motor units. As a consequence of PPS, polio survivors experience new weakness, generalized fatigue, atrophy of previously unaffected muscles, and a physical decline that may culminate in the loss of independent life. Emphasis is given to the possible pathogenic role of persistent poliovirus infection and chronic inflammation. These factors could contribute to the neurological and physical decline in polio survivors. A perspective is then given on novel anti-poliovirus compounds and monoclonal antibodies that have been developed to contribute to the final phases of polio eradication. These agents could also be useful for the treatment or prevention of PPS. Some of these compounds/antibodies are in early clinical development. Finally, current clinical trials for PPS are reported. In this area, the intravenous infusion of normal human immunoglobulins appears both feasible and promising.

Conclusions:

Outcome of Research: Effective.

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Category: Diagnosis and Management

Title: Treatment for postpolio syndrome
Author: Koopman FS, Beelen A, Gilhus NE, de Visser M, Nollet F
Affiliation: Department of Rehabilitation, University of Amsterdam Academic Medical Center, PO Box 22660, Amsterdam, North Holland, Netherlands, 1100 DD
Journal: Cochrane Database of Systematic Reviews
Citation: Cochrane Database Syst Rev. 2015 May 18;5:CD007818
Publication Year and Month: 2015 05

Abstract: BACKGROUND: Postpolio syndrome (PPS) may affect survivors of paralytic poliomyelitis and is characterised by a complex of neuromuscular symptoms leading to a decline in physical functioning. The effectiveness of pharmacological treatment and rehabilitation management in PPS is not yet established. This is an update of a review first published in 2011.

OBJECTIVES: To systematically review the evidence from randomised and quasi-randomised controlled trials for the effect of any pharmacological or non-pharmacological treatment for PPS compared to placebo, usual care or no treatment.

SEARCH METHODS: We searched the following databases on 21 July 2014: Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, EMBASE, PsycINFO and CINAHL Plus. We also checked reference lists of all relevant articles, searched the Database of Abstracts of Reviews of Effects (DARE), the Health Technology Assessment (HTA) Database and trial registers and contacted investigators known to be involved in research in this area.

SELECTION CRITERIA: Randomised and quasi-randomised trials of any form of pharmacological or non-pharmacological treatment for people with PPS. The primary outcome was self perceived activity limitations and secondary outcomes were muscle strength, muscle endurance, fatigue, pain and adverse events.

DATA COLLECTION AND ANALYSIS: We used standard methodological procedures expected by The Cochrane Collaboration.

MAIN RESULTS: We included 10 pharmacological (modafinil, intravenous immunoglobulin (IVIg), pyridostigmine, lamotrigine, amantadine, prednisone) and three non-pharmacological (muscle strengthening, rehabilitation in a warm climate (that is temperature ± 25°C, dry and sunny) and a cold climate (that is temperature ± 0°C, rainy or snowy), static magnetic fields) studies with a total of 675 participants with PPS in this review. None of the included studies were completely free from any risk of bias, the most prevalent risk of bias being lack of blinding.There was moderate- and low-quality evidence that IVIg has no beneficial effect on activity limitations in the short term and long term, respectively, and inconsistency in the evidence for effectiveness on muscle strength. IVIg caused minor adverse events in a substantial proportion of the participants. Results of one trial provided very low-quality evidence that lamotrigine might be effective in reducing pain and fatigue, resulting in fewer activity limitations without generating adverse events. Data from two single trials suggested that muscle strengthening of thumb muscles (very low-quality evidence) and static magnetic fields (moderate-quality evidence) are safe and beneficial for improving muscle strength and pain, respectively, with unknown effects on activity limitations. Finally, there was evidence varying from very low quality to high quality that modafinil, pyridostigmine, amantadine, prednisone and rehabilitation in a warm or cold climate are not beneficial in PPS.

Conclusions: Due to insufficient good-quality data and lack of randomised studies, it was impossible to draw definite conclusions about the effectiveness of interventions for PPS. Results indicated that IVIg, lamotrigine, muscle strengthening exercises and static magnetic fields may be beneficial but need further investigation to clarify whether any real and meaningful effect exists.

Outcome of Research: More research required.

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Category: Diagnosis and Management

Title: Physiotherapy for poliomyelitis: a descriptive study in the Republic of Congo
Author: Mancini S, Coldiron ME, Nicholas S, Llosa AE, Mouniaman-Nara I, Ngala J, Grais RF, Porten K
Affiliation: Epicentre, Paris, France - [email protected].
Journal: BioMedCentral Research Notes
Citation: BMC Res Notes. 2014 Oct 23;7:755. doi: 10.1186/1756-0500-7-755
Publication Year and Month: 2014 10

Abstract: BACKGROUND: A large poliomyelitis outbreak occurred in 2010 in the Republic of Congo. This paper describes the demographic and clinical characteristics of poliomyelitis cases and their outcomes following physiotherapy.

FINDINGS: Demographic and clinical data were collected on 126 individuals between November 23, 2010 and March 23, 2011. The male/female ratio was 2.5 and the median age was 19 years (IQR: 13.5-23). The most severe forms of the disease were more common in older patients, 81 of the 126 patients (64.3%) had multiple evaluations of muscle strength. Among patients with multiple evaluations, 38.1% had improved strength at final evaluation, 48.3% were stable and 13.6% had decreased strength.

Conclusions: Most acute poliomyelitis patients receiving physiotherapy had improved or stable muscle strength at their final evaluation. These descriptive results highlight the need for further research into the potential benefits of physiotherapy in polio affected patients.

Outcome of Research: More research required.

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Category: Diagnosis and Management

Title: Stroke Risk in Poliomyelitis Survivors: A Nationwide Population-Based Study
Author: Wu C-H, Liou T-H, Chen H-H, Sun T-Y, Chen K-H, Chang K-H
Affiliation: Wu, Sun, KH Chang: Chung-Yuan Christian University
Liou: Shuang Ho Hospital and Taipei Medical University, Taipei
HH Chen; Chang: Wan Fang Hospital
Chang: Taipei Medical University, Taipei
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Volume 93, Issue 12, Pages 2184–2188
Publication Year and Month: 2012 12

Abstract: Objectives
To assess the prevalence and risk of stroke among adults with polio and controls.

Design
A prospective, probability-sampling, 6-year population-based cohort study.

Setting
A National Health Insurance Research Database consisting of 316,355 randomly selected enrollees. The database is related to a National Health Insurance program with more than 22 million participants.

Participants
After excluding patients under 40 years of age, polio patients (N=212) (mean age ± SD, 54.0±10.2y; 57.1% men) were identified from the database from January 1, 2003 to December 31, 2008. For each polio patient, 2 age- and sex-matched patients were recruited as controls. Control patients did not have any neuromuscular diseases commonly found in childhood. The frequencies of patients with potential risk factors for stroke were assessed.

Intervention
None.

Main Outcome Measure
The prevalence and the adjusted odds ratio of ischemic stroke among polio patients and the controls were estimated.

Results
Polio patients had a higher prevalence of stroke (10.8% vs 2.4%, P<.001) than the controls. Polio patients with hypertension had a much higher prevalence of stroke (23.0%). The risk of stroke was higher for polio patients compared with the controls, yielding an adjusted odds ratio of 4.17 (95% confidence interval, 1.84–9.45, P<.001). Polio was a significant risk factor for stroke independent from hypertension, diabetes mellitus, hyperlipidemia, and cardiac diseases.

Conclusions
Adults with polio had a high prevalence of ischemic stroke. Polio was an additional risk factor for stroke. Polio patients with hypertension might potentiate the risk of stroke. Developing a health promotion program, suitable for polio patients, to increase participation in activities and exercises may be essential, especially for polio patients with hypertension.

Conclusions:

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Frequency and clinical manifestations of post-poliomyelitis syndrome in a Brazilian tertiary care center
Author: Quadros AA (1), Conde MT, Marin LF, Silva HC, Silva TM, Paula MB, Pereira RD, Ramos PE, Abe G, Oliveira AS
Affiliation: (1) Department of Neurology and Neurosurgery, Division of Neuromuscular Disorders, Federal University of São Paulo (Unifesp), São Paulo SP, Brazil - [email protected]
Journal: Arquivos de Neuro-psiquiatria
Citation: Arq Neuropsiquiatr. 2012 Aug;70(8):571-3
Publication Year and Month: 2012 08

Abstract: OBJECTIVE: To determine the frequency and clinical manifestations of patients with post-poliomyelitis syndrome (PPS) in a Brazilian division of neuromuscular disorders.

METHODS: A total of 167 patients with prior history of paralytic poliomyelitis was investigated for PPS, based on international diagnostic criteria. Other variables analyzed were: gender, race, age at poliomyelitis infection, age at PPS onset, and PPS symptoms.

RESULTS: One hundred and twenty-nine patients presented PPS, corresponding to 77.2% of the studied population. 62.8% were women and 37.2% were men. Mean age of patients with PPS at onset of PPS symptoms was 39.9±9.69 years. Their main clinical manifestations were: new weakness in the previously affected limbs (69%) and in the apparently not affected limbs (31%); joint pain (79.8%); fatigue (77.5%); muscle pain (76%); and cold intolerance (69.8%).

Conclusions: Most patients of our sample presented PPS. In Brazil, PPS frequency and clinical features are quite similar to those of other countries.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Risk factors for post-polio syndrome among an Italian population: a case-control study.
Author: Bertolasi L, Acler M, dall'Ora E, Gajofatto A, Frasson E, Tocco P, Turri M, Ferlisi M, Fiorini M, Pimazzoni F, Squintani G, Martini M, Danzi B, Monaco S.
Affiliation: Section of Neurology, Department of Neurological, Neuropsychological, Morphological and Motor Sciences, University of Verona, Piazzale L.A. Scuro 10, 37134, Verona, Italy. [email protected]
Journal: Neurological sciences: official journal of the Italian neurological society
Citation: 2012 Dec;33(6):1271-5. doi: 10.1007/s10072-012-0931-2. Epub 2012 Jan 14.
Publication Year and Month: 2012 01

Abstract: Post-polio syndrome (PPS) is a clinical syndrome of new weakness, fatigue and musculoskeletal pain occurring in a variable proportion of polio survivors decades after acute disease. To date, several risk factors for PPS development have been reported, although the etiology of this disorder remains elusive. Using a case-control design, we aimed to assess risk indicators for PPS in a group of Italian polio survivors. Subjects with prior poliomyelitis attending the rehabilitation hospital of Malcesine, Italy, were the target population. Patients with PPS, diagnosed according to the European Federation of Neurological Societies criteria, served as cases, while patients not meeting diagnostic criteria for PPS were used as controls. All subjects were assessed through a structured questionnaire made of 82 questions and neurological examination. The association with investigated risk factors (sex, age at polio onset, age at onset of symptoms, extension and severity of polio, employment) was analyzed by the calculation of the odds ratio. A total of 161 out of 391 eligible patients met the adopted diagnostic criteria for PPS, giving a frequency of 41.2%. Symptoms most frequently complained by PPS patients were loss of muscle strength, loss of resistance, loss of muscle volume and generalized fatigue. Female gender, the presence of respiratory disturbance during the acute phase of polio and the use of orthoses and aids during the recovery and stabilization represented independent risk factors for PPS in the studied population.

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Update on current and emerging treatment options for post-polio syndrome
Author: Farbu E
Affiliation: Neurocenter and National Competence Center for Movement Disorders, Stavanger University Hospital, Stavanger, Norway
Journal: Therapeutics and Clinical Risk Management
Citation: Ther Clin Risk Manag. 2010 Jul 21;6:307-13
Publication Year and Month: 2010 07

Abstract: Post-polio syndrome (PPS) refers to the clinical deterioration experienced by many polio survivors several decades after their acute illness. The symptoms are new muscle weakness, decreased muscle endurance, fatigue, muscle pain, joint pain, cold intolerance, and this typical clinical entity is reported from different parts of the world. The pathophysiology behind PPS is not fully understood, but a combination of distal degeneration of enlarged motor units caused by increased metabolic demands and the normal aging process, in addition to inflammatory mechanisms, are thought to be involved. There is no diagnostic test for PPS, and the diagnosis is based on a proper clinical workup where all other possible explanations for the new symptoms are ruled out. The basic principle of management of PPS lies in physical activity, individually tailored training programs, and lifestyle modification. Muscle weakness and muscle pain may be helped with specific training programs, in which training in warm water seems to be particularly helpful. Properly fitted orthoses can improve the biomechanical movement pattern and be energy-saving. Fatigue can be relieved with lifestyle changes, assistive devices, and training programs. Respiratory insufficiency can be controlled with noninvasive respiratory aids including biphasic positive pressure ventilators. Pharmacologic agents like prednisone, amantadine, pyridostigmine, and coenzyme Q10 are of no benefit in PPS. Intravenous immunoglobulin (IVIG) has been tried in three studies, all having positive results. IVIG could probably be a therapeutic alternative, but the potential benefit is modest, and some important questions are still unanswered, in particular to which patients this treatment is useful, the dose, and the therapeutic interval.

Conclusions:

Outcome of Research: More research required.

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Category: Diagnosis and Management

Title: Management of postpolio syndrome (2010)
Author: Gonzalez H, Olsson T, Borg K
Affiliation: Division of Rehabilitation Medicine, Department of Clinical Sciences, Danderyd Hospital, Karolinska Institute, Stockholm, Sweden – [email protected]
Journal: The Lancet Neurology
Citation: Lancet Neurol. 2010 Jun; 9(6):634-42 and Comment in: Lancet Neurol. 2010 Jun; 9(6):561-3
Publication Year and Month: 2010 06

Abstract: Postpolio syndrome is characterised by the exacerbation of existing or new health problems, most often muscle weakness and fatigability, general fatigue, and pain, after a period of stability subsequent to acute polio infection. Diagnosis is based on the presence of a lower motor neuron disorder that is supported by neurophysiological findings, with exclusion of other disorders as causes of the new symptoms. The muscle-related effects of postpolio syndrome are possibly associated with an ongoing process of denervation and reinnervation, reaching a point at which denervation is no longer compensated for by reinnervation. The cause of this denervation is unknown, but an inflammatory process is possible. Rehabilitation in patients with postpolio syndrome should take a multiprofessional and multidisciplinary approach, with an emphasis on physiotherapy, including enhanced or individually modified physical activity, and muscle training. Patients with postpolio syndrome should be advised to avoid both inactivity and overuse of weak muscles. Evaluation of the need for orthoses and assistive devices is often required.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: Postpolio syndrome: unanswered questions regarding cause, course, risk factors, and therapies
Author: Nollet F
Affiliation: Not stated
Journal: The Lancet Neurology
Citation: Lancet Neurol. 2010 Jun;9(6):561-3 - Comment on: Lancet Neurol. 2010 Jun;9(6):634-42
Publication Year and Month: 2010 06

Abstract: Living with the consequences of poliomyelitis is not recognised as an important health issue at present. However, millions of people worldwide have lasting impairments caused by polio infection, many of whom also had a decline in muscle function and decline in activities of daily living after years of stable functioning.

Conclusions:

Outcome of Research:

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Category: Diagnosis and Management

Title: A positive turning point in life -- how persons with late effects of polio experience the influence of an interdisciplinary rehabilitation programme
Author: Larsson Lund M (1), Lexell J
Affiliation: (1) Department of Community Medicine and Rehabilitation, Occupational Therapy , Umeå University, Umeå, Sweden
Journal: Journal of Rehabilitation Medicine
Citation: J Rehabil Med. 2010 Jun;42(6):559-65. doi: 10.2340/16501977-0559
Publication Year and Month: 2010 06

Abstract: OBJECTIVE: To describe and enhance our understanding of how persons with late effects of polio experience the influence of an interdisciplinary rehabilitation programme.

PARTICIPANTS: Twelve persons with clinically verified late effects of polio who had participated in an individualized, goal-oriented, comprehensive interdisciplinary rehabilitation programme.

METHODS: Qualitative research interviews analysed using the constant comparative method of grounded theory.

RESULTS: The rehabilitation programme was experienced as a turning point in the participants' lives. Before rehabilitation they felt they were on a downward slope without control. Rehabilitation was the start of a process of change whereby they acquired new skills, which, over time, contributed to a different but good life. After approximately a year, they had a sense of control and had accepted life with late effects of polio. They had also established new habits, taken on a changed valued self and could look to the future with confidence.

Conclusions: This qualitative study has shown that persons with late effects of polio can benefit from an individualized, goal-oriented, comprehensive interdisciplinary rehabilitation programme and experience positive changes in their management of daily activities and in their view of their late effects of polio, their future and their self.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Outcome of physiotherapy as part of a multidisciplinary rehabilitation in an unselected polio population with one-year follow-up: an uncontrolled study.
Author: Bertelsen M, Broberg S, Madsen E.
Affiliation: Rehabilitation Centre of the Danish Society of Polio and Accident Victims (PTU), Rødovre, Denmark. [email protected]
Journal: Journal of Rehabilitation Medicine
Citation: 2009 Jan;41(1):85-7.
Publication Year and Month: 2009 01

Abstract: OBJECTIVE:
The aim of this study was to evaluate the outcome of physiotherapy as part of a multidisciplinary rehabilitation.

DESIGN:
Prospective uncontrolled intervention study.

SUBJECTS:
Fifty patients with late effects of polio, first time referred to physiotherapy at the Danish Society of Polio and Accident Victims (PTU) Rehabilitation Centre.

METHODS:
The intervention was physiotherapy as an essential part of an individually planned multidisciplinary rehabilitation. The outcome measures Six-Minute Walk Test and Timed-Stands Test were used to assess the functional capacity. Quality of life was evaluated by Medical Outcome Survey Short Form (SF-36) and fatigue by Multidimensional Fatigue Inventory (MFI-20). Patients were tested at baseline; 3 months after the start of rehabilitation and at one-year follow-up.

RESULTS:
The patients showed significantly better functional capacity on all measurements 3 months after start of intervention and at one-year follow-up. The patients showed significant improvement in 3 of the SF-36 dimensions regarding quality of life, but only the improvement in "general health" remained after one year.

Conclusions: This study shows that patients with late effects of polio, who experience new problems related to polio, can benefit from an individually planned multidisciplinary intervention with emphasis on physiotherapy, and the improvement in physical capacity and general health can remain at one-year follow-up.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: EFNS guideline on diagnosis and management of post-polio syndrome - report of an EFNS task force
Author: Farbu E, Gilhus NE, Barnes MP, Borg K, de Visser M, Driessen A, Howard R, Nollet F, Opara J, Stalberg E
Affiliation: Department of Neurology, Haukeland University Hospital, University of Bergen, Bergen, Norway – [email protected] – European Federation of Neurological Society
Journal: European Journal of Neurology
Citation: Eur J Neurol. 2006 Aug; 13(8):795-801
Publication Year and Month: 2006 08

Abstract: Post-polio syndrome (PPS) is characterized by new or increased muscular weakness, atrophy, muscle pain and fatigue several years after acute polio. The aim of the article is to prepare diagnostic criteria for PPS, and to evaluate the existing evidence for therapeutic interventions. The Medline, EMBASE and ISI databases were searched. Consensus in the group was reached after discussion by e-mail. We recommend Halstead's definition of PPS from 1991 as diagnostic criteria. Supervised, aerobic muscular training, both isokinetic and isometric, is a safe and effective way to prevent further decline for patients with moderate weakness (Level B). Muscular training can also improve muscular fatigue, muscle weakness and pain. Training in a warm climate and non-swimming water exercises are particularly useful (Level B). Respiratory muscle training can improve pulmonary function. Recognition of respiratory impairment and early introduction of non-invasive ventilatory aids prevent or delay further respiratory decline and the need for invasive respiratory aid (Level C). Group training, regular follow-up and patient education are useful for the patients' mental status and well-being. Weight loss, adjustment and introduction of properly fitted assistive devices should be considered (good practice points). A small number of controlled studies of potential-specific treatments for PPS have been completed, but no definitive therapeutic effect has been reported for the agents evaluated (pyridostigmine, corticosteroids, amantadine). Future randomized trials should particularly address the treatment of pain, which is commonly reported by PPS patients. There is also a need for studies evaluating the long-term effects of muscular training.

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Category: Diagnosis and Management

Title: Poliomyelitis and the postpolio syndrome
Author: Howard, R.S.
Affiliation: Lane-Fox Unit and Department of Neurology, St Thomas' Hospital, Guy's and St Thomas' NHS Foundation Trust, London
Journal: The British Medical Journal
(The BMJ)
Citation: 330(7503): 1314–1318.
doi: 10.1136/bmj.330.7503.1314
Publication Year and Month: 2005 06

Abstract: Acute poliomyelitis is now rarely encountered in the United Kingdom, but “imported” poliomyelitis still occurs and it is necessary to distinguish acute poliomyelitis from other causes of acute flaccid paralysis. Despite the obvious success of preventive policies, many patients who had poliomyelitis experience late functional deterioration after periods of prolonged stability—the so called postpolio syndrome. The patterns of disability and their management present unique challenges to the multidisciplinary rehabilitation team.

Conclusions: Although some patients who have had poliomyelitis may later develop wasting, pain, and fatigue in isolation, in most there is significant underlying weakness and skeletal deformity predisposing to functional deterioration. The severe physical stresses of postpolio disability contribute to the development of progressive orthopaedic, respiratory, neurological, and general medical abnormalities, often exacerbated by intercurrent events. These abnormalities may present with atypical clinical features because of the extent of underlying atrophy and weakness, but many are potentially treatable and most patients can be helped to understand and manage increasing disability. It is essential to emphasise that the symptoms, disabilities, and impairments of postpolio functional deterioration are often amenable to treatment. It is also important to urge caution before attributing functional deterioration to a primary “postpolio syndrome” or “progressive postpolio muscular atrophy.”

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Post-poliomyelitis syndrome (2005)
Author: Trojan DA, Cashman NR
Affiliation: Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, 3801 rue Université, Montréal, Québec H3A 2B4, Canada – [email protected]
Journal: Muscle & Nerve
Citation: Muscle Nerve. 2005 Jan;31(1):6-19
Publication Year and Month: 2005 01

Abstract: Post-poliomyelitis syndrome (PPS) is a common neurological disorder that occurs in a large proportion of individuals who have recovered from paralytic poliomyelitis. The main clinical features are new weakness, muscular fatigability, general fatigue, and pain. The primary criteria necessary for the diagnosis of PPS are a history of paralytic poliomyelitis, partial or complete recovery of neurological function followed by a period of stability (usually several decades), persistent new muscle weakness or abnormal muscle fatigability, and the exclusion of other causes of new symptoms. The cause of PPS remains unclear, but is likely due to a distal degeneration of enlarged post-poliomyelitis motor units. Contributing factors to PPS may be aging (with motor neuron loss), overuse, and disuse. PPS is usually a slowly progressive neuromuscular disease. Although there is no specific treatment for PPS, an interdisciplinary management program can be useful in controlling symptoms.

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Category: Diagnosis and Management

Title: Postpolio syndrome
Author: Nollet F, de Visser M
Affiliation: Department of Rehabilitation, Academic Medical Center, University of Amsterdam, PO Box 22660, 1100 DD Amsterdam, the Netherlands – [email protected]
Journal: Archives of Neurology
Citation: Arch Neurol. 2004 Jul;61(7):1142-4
Publication Year and Month: 2004 07

Abstract: This paper has no abstract - this is an extract:

Postpolio syndrome (PPS) refers to a decline of muscle function usually occurring 30 to 40 years after the acute polio episode. This syndrome has been widely recognized only during the last decades, when many people affected by the large epidemics of the previous century experienced new muscle weakness as they grew older. However, cases of late-onset weakening following poliomyelitis were already reported at the end of the 19th century.

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Category: Diagnosis and Management

Title: Life-Long Morbidity Among Danes With Poliomyelitis
Author: Nielsen NM, Rostgaard K, Askgaard D, Skinhøj, & P Aaby P.
Affiliation: Department of Epidemiology Research, Danish Epidemiology Science
Centre, Statens Serum Institut (Nielsen, Rostgaard, Aaby) and Department of Infectious Diseases M, National University Hospital, (Askgaard, Skinhøj), Copenhagen,
Denmark.
Supported by the Danish Medical Research Council, the AP Møller and Chastine
McKinney Møller Foundation, the Danish National Research Foundation, the WedellWedellsborg Foundation, and The National Polio Society
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil 2004; 85:385-91 - doi:10.1016/S0003-9993(03)00474-X
Publication Year and Month: 2004 03

Abstract: Abstract
OBJECTIVE:
To estimate long-term morbidity in a cohort of Danish poliomyelitis patients.

DESIGN:
A historical prospective cohort study of 27,047 persons.

SETTING:
Denmark.

PARTICIPANTS:
A total of 5421 persons hospitalized for poliomyelitis between 1919 to 1954 in Copenhagen, Denmark, and 21,626 age- and gender-matched Danes. Participants were followed up on average for 20.6 years, yielding a total of 555,884 person-years of follow-up.

INTERVENTIONS:
Not applicable.

MAIN OUTCOME MEASURES:
The exposed (poliomyelitis) cohort and the unexposed (control) cohort were followed up for somatic hospitalization from 1977 to 1999 in the Danish Hospital Discharge Register. The incidence rate ratio (IRR) was calculated as the ratio between the incidence rate of disease in the exposed and unexposed cohorts.

RESULTS:
Overall, polio patients had a 1.2- to 1.3-fold increased risk of being hospitalized with pulmonary diseases, heart diseases, gastrointestinal disorders, or diseases of the locomotive apparatus. Among paralytic polio patients, long-term morbidity seems to be associated with the acute severity of poliomyelitis, as well as young age at infection. Paralytic patients, who contracted respiratory polio under the age of 5, had the highest risk of being hospitalized with lung diseases (IRR=7.26; 95% confidence interval [CI], 3.06-18.33), diseases of the locomotive apparatus (IRR=4.05; 95% CI, 1.66-9.86), heart diseases (IRR=1.70; 95% CI, 0.65-3.98), and diseases of the digestive system (IRR= 2.23; 95% CI, 1.03-4.62). Surprisingly, patients without paralyses, especially women, also had an increased morbidity.

CONCLUSIONS:
Overall, a history of poliomyelitis was associated with a slightly increased morbidity measured by hospitalizations. Long-term morbidity was highest among respiratory polio patients; however, patients presumably left without any residual symptoms also had an increased morbidity.

Conclusions: Overall, a history of poliomyelitis was associated with a slightly increased morbidity measured by hospitalizations. Long-term morbidity was highest among respiratory polio patients; however, patients presumably left without any residual symptoms also had an increased morbidity.

Outcome of Research: Effective

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Category: Diagnosis and Management

Title: Post-polio syndrome and total health status in a prospective hospital study.
Author: Farbu E, Rekand T, Gilhus NE
Affiliation: Department of Neurology, Haukeland University Hospital, Bergen, Norway
Journal: European Journal of Neurology
Citation: 2003 Jul;10(4):407-13.
Publication Year and Month: 2003 07

Abstract: New loss of function among patients with previous polio is frequently reported and has several causes. All patients referred to the Department of Neurology, Haukeland University Hospital, Bergen, for 13 months during 2000-2001 with diagnosis late effects of polio were examined prospectively to identify their symptoms and loss of function. Eighty-five patients aged 47-91 years with mean of 61 years were included. The most common complaints were pain (44%), muscular weakness (27%), and fatigue (16%). Muscular weakness occurred in lower limbs in 75%, in respiratory muscles in only 5%. Walking in stairs was impaired in 72% and outdoor walking in 65%. Seventeen patients (19%) reported no loss of function. Post-polio syndrome was diagnosed in 26% of the patients. Polio-related loss of function including cervical and lumbosacral radiculopathies, mononeuropathies and degenerative joint disease were found in an additional 53%. Eleven patients (13%) had distinct non-polio-related disorders that caused new loss of function. The remaining 8% had a stable condition.

Conclusions: In conclusion, the majority of polio patients who seek hospital, experience a new loss of function because of polio-related disorders. A careful neurological examination is necessary to identify the correct diagnosis and treatment.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Cardiac risk factors in polio survivors
Author: Gawne AC, Wells KR, Wilson KS.
Affiliation: Roosevelt Warm Springs Institute for Rehabilitation, Warm Springs, GA 31830, USA - [email protected]
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil. 2003 May;84(5):694-6
Publication Year and Month: 2003 05

Abstract: OBJECTIVE: To assess the prevalence of dyslipidemia and other risk factors for coronary heart disease in a sample of polio survivors with and without postpoliomyelitis syndrome.

DESIGN: Retrospective chart review.

SETTING: A multidisciplinary outpatient postpolio clinic.

PARTICIPANTS: Eighty-eight consecutive symptomatic postpolio patients, 50 women (mean age, 59.0y; range, 36-81y) and 38 men (mean age, 61.2y; range, 44-83y).

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Presence of risk factors for coronary heart disease: clinical atherosclerotic disease, male age >or=45 years or female age >or=55 years, history of hypertension (blood pressure >or=140/90mmHg or on antihypertensive medication), diabetes mellitus, cigarette smoking, and high-density lipoprotein (HDL) less than 35mg/dL. Obesity (body mass index [BMI], >25kg/m(2)) was assessed as an intervention target. Laboratory values included fasting total cholesterol, HDL, low-density lipoprotein, triglycerides, and glucose.

RESULTS: Of the total sample, 61.3% had dyslipidemia. Average HDL cholesterol ratio was 4.01 (women, 3.68; men, 4.55). Forty-four patients (50%) had a history of hypertension or had elevated blood pressure. Seven patients (8%) had a history of diabetes or had elevated fasting blood glucose (>110). Eighteen patients (20.4%) were smokers or had a history of smoking; 9 continued to smoke and 9 had quit smoking. Twenty-five patients (28.4%) were overweight (BMI, >25kg/m(2)). Forty-one patients (46.5%) had more than 1 risk factor for coronary heart disease. Nine of the total sample (10.2%) had a history of heart disease ranging from atrial fibrillation to angina. Only 19 patients had a previous diagnosis of dyslipidemia and only 12 were on a lipid-lowering medication.

Conclusions: Polio patients have a high prevalence of dyslipidemia. The study sample supports the National Cholesterol Education Program's Adult Treatment Panel III statements that hypercholesterolemia is underdiagnosed and undertreated. The postpolio population carries a high prevalence of 2 or more coronary heart disease risk factors. Evaluation and rehabilitation of polio patients should include screening for dyslipidemia and education about elimination of controllable risk factors.

Outcome of Research: Not applicable.

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Category: Diagnosis and Management

Title: Poliomyelitis and Parkinson Disease.
Author: Nielsen NM, Rostgaard K, Hjalgrim H, Aaby P, Askgaard D.
Affiliation: Department of Epidemiology Research, National University Hospital, Copenhagen, Denmark
Journal: The Journal of the American Medical Association
Citation: JAMA. 2002;287(13):1650–1651.
doi:10.1001/jama.287.13.1645
Publication Year and Month: 2002 04

Abstract: Parkinson disease (PD), which is due to loss of dopaminergic neurons in the zona compacta of the substantia nigra,1 may involve both genetic and environmental risk factors.2 Poliovirus is believed to cause neuronal damage in the substantia nigra,3 and thus a history of poliovirus infection may be associated with an increased risk of PD

Conclusions:

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Characteristics and management of postpolio syndrome
Author: Jubelt B, Agre JC
Affiliation: State University of New York, Upstate Medical University, 750 E Adams St, Syracuse, NY 13210, USA – [email protected]
Journal: The Journal of the American Medical Association
Citation: JAMA. 2000 Jul 26;284(4):412-4
Publication Year and Month: 2000 07

Abstract: Postpolio syndrome (PPS) refers to new, late manifestations occurring many years after acute poliomyelitis infection. Over the last 25 years, PPS has become a relatively common problem encountered by primary care physicians. A 1987 National Health Interview Survey estimated that about half of the 640,000 survivors of paralytic poliomyelitis in the United States had new late manifestations of PPS. Subsequent studies in the 1990s have found the occurrence of PPS among patients with previous poliomyelitis to range from 28.5% to 64%. The average time in various reports from the acute poliomyelitis until the onset of PPS is about 35 years, with a range from 8 to 71 years. However, it is unclear if the occurrence of PPS increases with aging, which may be the case based on the most accepted etiologic hypothesis.

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Category: Diagnosis and Management

Title: Multiple sclerosis and poliomyelitis. A Danish historical cohort study.
Author: Nielsen NM, Wohlfahrt J, Melbye M, Rasmussen S, Mølbak K, Askgaard DS, Aaby P.
Affiliation: Department of Epidemiology Research, Statens Serum Institut, Copenhagen, Denmark
Journal: Acta Neurologica Scandinavica
Citation: Acta Neurol Scand 2000 Jun;101(6):384-7.
Publication Year and Month: 2000 06

Abstract: OBJECTIVE:
To evaluate whether persons with a history of poliomyelitis are at an increased risk of developing multiple sclerosis (MS).

MATERIAL AND METHODS:
All patients diagnosed with acute poliomyelitis in the greater capital area of Copenhagen, Denmark, between 1919 and 1954 were identified and followed with respect to MS. Information on vital status and diagnosis of sclerosis was obtained through linkage with the Danish Civil Registration System and The Danish Multiple Sclerosis Registry, respectively. Follow-up started on the date of the establishment of the Danish Civil Registration System (April 1, 1968) until death, emigration or December 31, 1996, whichever came first. The observed incidence of MS among polio patients was compared with the expected incidence calculated according to national gender, age and period specific rates of MS.

RESULTS:
During 149,364 years of follow-up, 19 cases of multiple sclerosis were observed among 5652 polio patients compared with 11.0 expected (SIR = 1.73 (1.04-2.74)). The increased risk of MS was most pronounced in polio patients hospitalized during adolescence. Neither gender nor the acute severity of poliomyelitis modified the risk of MS.

CONCLUSION:
Our results are based on small numbers of events, however the findings suggest that the polio patients might be at an increased risk of MS.

Conclusions:

Outcome of Research: More research required

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Comments (if any): There appears a higher incidence of Multiple Sclerosis in this study of Danish patients previously diagnosed with Poliomyelitis. This should not be interpreted that previously contracting Poliomyelitis will lead to Multiple Sclerosis.

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Category: Diagnosis and Management

Title: Nonparalytic polio and postpolio syndrome
Author: Halstead LS, Silver JK
Affiliation: National Rehabilitation Hospital, Washington, DC 20010, USA
Journal: American Journal of Physical Medicine & Rehabilitation
Citation: Am J Phys Med Rehabil. 2000 Jan-Feb;79(1):13-8
Publication Year and Month: 2000 01

Abstract: We describe four cases of postpolio syndrome with typical histories, physical examination results, and electrodiagnostic evidence of extensive anterior horn cell disease, as well as the putative pathophysiology of postpolio syndrome in persons with histories of nonparalytic polio and the diagnostic implications for individuals older than 40 yr of age who are experiencing unexplained new weakness, fatigue, and muscle or joint pain. Although the diagnosis of postpolio syndrome traditionally has required a remote history of paralytic polio, many persons such as the ones described here with typical symptoms of postpolio syndrome have no clear history of paralytic disease and are being misdiagnosed. With this in mind, we believe that the diagnostic criteria for postpolio syndrome should be modified to include the following: a history of remote paralytic polio or findings on history, physical examination results, and laboratory studies compatible with poliovirus damage of the central nervous system earlier in life.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Paralytic vs "nonparalytic" polio: distinction without a difference?
Author: Bruno RL
Affiliation: The Post-Polio Institute, Englewood Hospital and Medical Center, New Jersey, USA
Journal: American Journal of Physical Medicine & Rehabilitation
Citation: Am J Phys Med Rehabil. 2000 Jan-Feb;79(1):4-12
Publication Year and Month: 2000 01

Abstract: Nonparalytic polio (NPP) is commonly thought to be synonymous with "abortive polio," in which the poliovirus neither entered the central nervous system nor damaged neurons. Described are two epidemic illness-"The Summer Grippe" and Iceland disease-apparently caused by a low virulence but neuropathic type 2 poliovirus. Studies show that neuronal lesions in the brain and spinal cord and muscle weakness were common in NPP, and epidemiologic studies document late-onset weakness and fatigue in 14% to 42% of NPP survivors. These findings indicate that clinicians should not require a history of paralytic polio, electromyographic evidence of denervation, and new muscle weakness for the diagnosis of "Postpolio Syndrome" but should be aware that NPP, and possibly even poliovirus-induced "minor illnesses," can be associated with acute central nervous system damage and late-onset muscle weakness and fatigue.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Management of post-polio syndrome (1997)
Author: Trojan DA, Finch L
Affiliation: Not stated
Journal: NeuroRehabilitation
Citation: NeuroRehabil. 1997;8:93-105
Publication Year and Month: 1997 08

Abstract: The management of patients presenting with post-poliomyelitis syndrome is discussed. It is essential to identify and treat other medical and neurological conditions which could produce these symptoms. New weakness can be managed with exercise, avoidance of muscular overuse, weight loss, orthoses and assistive devices. Fatigue can be managed with energy conservation techniques. The management of pain is dependent upon its causes. Treatments are reviewed.

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Category: Diagnosis and Management

Title: The post-polio syndrome as an evolved clinical entity. Definition and clinical description.
Author: Dalakas MC
Affiliation: Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892, USA
Journal: Annals of the New York Academy of Sciences
Citation: Ann N Y Acad Sci. 1995 May 25;753:68-80
Publication Year and Month: 1995 05

Abstract: Post-polio syndrome (PPS) refers to the new neuromuscular symptoms that occur at least 15 years after stability in patients with prior acute paralytic polio-myelitis. They include: (1) new muscle weakness and atrophy in the limbs, the bulbar or the respiratory muscles [post-poliomyelitis muscular atrophy (PPMA)] and (2) excessive muscle fatigue and diminished physical endurance. PPS is a clinical diagnosis that requires exclusion of all other medical, neurological, orthopedic or psychiatric diseases that could explain the cause of the new symptoms. Routine electromyography is useful to confirm chronic and ongoing denervation and exclude neuropathies. Muscle biopsy, single fiber electromyography (EMG), macro-EMG, serum antibody titers to polio virus, and spinal fluid studies are very useful research tools but they are rarely needed to establish the clinical diagnosis. PPS is a slowly progressive phenomenon with periods of stability that vary from 3 to 10 years. Current evidence indicates that PPS is the evolution of a subclinically ongoing motor neuron dysfunction that begins after the time of the acute polio. It is clinically manifested as PPS when the well-compensated reinnervating process crosses a critical threshold beyond which the remaining motor neurons cannot maintain the innervation to all the muscle fibers within their motor unit territory.

Conclusions:

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: National Rehabilitation Hospital Limb Classification for Exercise, Research, and Clinical Trials in Post-Polio Patients
Author: Lauro S. Halstead, Anne Carrington Gawne, and Bao T. Pham

Affiliation: The Post-Polio Program; National Rehabilitation Hospital, Washington, DC


Journal: Annals of the New York Academy of Sciences
Citation: The Post-Polio Syndrome: Advances in the Pathogenesis and Treatment Volume 753 pp 343-353 of the Annals of the New York Academy of Sciences May 25, 1995.
Publication Year and Month: 1995 05

Abstract:

Conclusions: A need exists for an objective classification of polio patients for clinical and research purposes that takes into account the focal, asymmetric, and frequent subclinical nature of polio lesions. In order to prescribe a safe, effective exercise program, we developed a five-level (Classes I-V) limb-specific classification system based on remote and recent history, physical examination, and a four-extremity electrodiagnostic study (EMG/NCS). Class I limbs have no history of remote or recent weakness, normal strength, and a normal EMG. Class II limbs have no history of remote or recent weakness (or if remote history of weakness, full recovery occurred), normal strength and EMG evidence of prior anterior horn cell disease (AHCD). Class III limbs have a history of remote weakness with variable recovery, no new weakness, decreased strength, and EMG evidence of prior AHCD. Class IV limbs have a history of remote weakness with variable recovery, new clinical weakness, decreased strength, and EMG evidence of AHCD. Class V limbs have a history of severe weakness with little-to-no recovery, severely decreased strength and atrophy, and few-to-no motor units on EMG. In a prospective study of 400 limbs in 100 consecutive post-polio patients attending our clinic, 94 (23%) limbs were Class I, 88 (22%) were Class II, 95 (24%) were Class III, 75 (19%) were Class IV, and 48 (12%) were Class V. Guidelines for the use of this classification in a clinical/research setting are presented along with sample case histories and class-specific exercise recommendations.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Pathogenetic mechanisms of post-polio syndrome: morphological, electrophysiological, virological, and immunological correlations.
Author: Dalakas MC
Affiliation: Medical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1382, USA.
Journal: Annals of the New York Academy of Sciences
Citation: 1995 May 25;753:167-85.
Publication Year and Month: 1995 05

Abstract: To understand the mechanism of post-poliomyelitis muscular atrophy (PPMA) and the post-polio syndrome (PPS) in general, we performed the following studies: (1) histopathology in spinal cord sections from patients who died 9 days to 44 years after acute paralytic poliomyelitis; (2) enzyme histochemistry, immunocytochemistry (for lymphocyte subsets, MHC antigens and N-CAM) and polymerase chain reaction (PCR) for poliovirus RNA in the muscle biopsies from symptomatic or asymptomatic muscles of post-polio patients; (3) determination of lymphocyte subsets and circulating IgG or IgM antibodies against GM1 and poliovirus; (4) virological studies in the spinal fluid for oligoclonal bands and search for poliovirus genome with PCR; (5) electrophysiological studies including single fiber EMG, fiber density and macro-EMG; and (6) [31P] exercise MRS spectroscopy on previously affected muscles to search for a metabolic correlate of fatigue. These studies concluded that in PPS a continuing dysfunction is present in the spinal cord motor neurons, resulting in ongoing muscle denervation and reinnervation first evident at the axonal branch points. Symptoms are related to attrition of the oversprouting motor neurons which after a period of time cannot support all their axonal sprouts, resulting in failure of re-reinnervation. In some patients with PPS there is also an ongoing immune activation and presence of defective viral particles in the spinal fluid. However, their role in the pathogenesis of PPS is presently unknown.

Conclusions: These studies concluded that in PPS a continuing dysfunction is present in the spinal cord motor neurons, resulting in ongoing muscle denervation and reinnervation first evident at the axonal branch points.

Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Predictive Factors for Post-Poliomyelitis Syndrome
Author: Daria A. Trojan, MD, MSc, Neil R. Cashman, MD, Stanley Shapiro, PhD, Catherine M. Tansey, MSc, John M. Esdaile, MD
Affiliation: From the Department of Neurology (Drs. Trojan, Cashman), Montreal Neurological Institute and Hospital, the Department of Medicine (Dr. Esdaile), Montreal General Hospital, and the Department of Epidemiology and Biostatistics (Dr. Shapiro, Ms. Tansey), McGill University, Montreal, Quebec, Canada.
Journal: Archives of Physical Medicine and Rehabilitation
Citation: Arch Phys Med Rehabil Vol 75, July 1994, 770-777
Publication Year and Month: 1994 07

Abstract: Post-poliomyelitis syndrome (PPS) is generally defined as a clinical syndrome of new weakness, fatigue, and pain in individuals who have previously recovered from acute paralytic poliomyelitis. The purpose of this study was to identify, through a case-control study design, factors that predict subsequent PPS in patients with prior paralytic poliomyelitis. Among patients attending a university-affiliate hospital post-polio clinic, "cases" were patients with new weakness and fatigue, and "controls" were patients without these complaints. A chart review of 353 patients identified 127 cases and 39 controls. Logistic regression modeling was used to calculate adjusted and unadjusted odds ratios. In univariate analyses, significant risk factors for PPS were a greater age at time of presentation to clinic (p = 0.01), a longer time since acute polio (p = 0.01), and more weakness at acute polio (p = 0.02). Other significant associated, but not necessarily causal factors were a recent weight gain (p = 0.005), muscle pain (p = 0.01) particularly that associated with exercise (p = 0.005), and joint pain (p = 0.04). Multivariate analyses revealed that a model containing age at presentation to clinic, severity of weakness at acute polio, muscle pain with exercise, recent weight gain, and joint pain best distinguished cases from controls. Age at acute polio, degree of recovery after polio, weakness at best point after polio, physical activity, and sex were not contributing factors. These findings suggest that the degree of initial motor unit involvement as measured by weakness at acute polio, and possibly the aging process and overuse are important in predicting PPS.

Conclusions: In conclusion, the results from this study provide insight on predictive factors for PPS, and can be applied in the clinical management of patients who have recovered from paralytic poliomyelitis. Our findings support the hypothesis that the severity of initial motor unit involvement as estimated by weakness at acute polio, and possibly the normal ageing process and overuse are important in predicting PPS. Even though patients have no control over the severity of weakness as a result of acute polio, they do have control over some predictive factors for PPS. Patients can be advised that they should avoid gaining weight and exercising to the point of muscle pain because these variables have been found to be strongly associated with PPS. The exact role of physical activity will still need further evaluation; however, the usual recommendations of low-level aerobic exercise with avoidance of muscle pain and fatigue appear valid. Thus, this study can provide the basis for physiologically reasonable and practical advice to post-polio patients to minimize or delay the risk of PPS.

Outcome of Research: Not applicable

Availability of Paper: The full text of this paper has been generously made available by the publisher.

Comments (if any): Paul Cavendish (Clinical Health Educator): The best multivariate model for predicting who will develop PPS indicates that patients who had a greater weakness at acute polio, are currently older, have muscle pain with exercise, a recent weight gain, and joint pain are those most likely to develop PPS. Other factors shown to be important in univariate analyses are a longer time since acute polio, and muscle pain (at rest or with exercise). Age at acute polio, recovery after polio, weakness at "best point" after polio, physical activity, and sex were not contributing factors.

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Category: Diagnosis and Management

Title: Epidemiology of the post-polio syndrome
Author: Ramlow J, Alexander M, LaPorte R, Kaufmann C, Kuller L
Affiliation: Department of Biostatistics, Graduate School of Public Health, University of Pittsburgh, PA
Journal: American Journal of Epidemiology
Citation: Am J Epidemiol. 1992 Oct 1;136(7):769-86
Publication Year and Month: 1992 10

Abstract: A late-onset syndrome, consisting of muscle weakness, muscle pain, and unaccustomed fatigue, has been reported with increasing frequency among former poliomyelitis patients. A population-based cohort of poliomyelitis patients from Allegheny County, Pennsylvania, was traced and surveyed to estimate the prevalence and incidence and to identify determinants of the post-polio syndrome. A questionnaire validated in clinical examinations of 40 cohort members was used in the survey. The prevalence of the post-polio syndrome was 28.5% of all paralytic cases (95% confidence interval 24.4-32.6). The risk of post-polio syndrome was significantly higher among patients who sustained substantial permanent impairment after polio and among females. The incidence did not vary with age at acute onset, acute severity, or level of physical activity after recovery. The strongest determinant of post-polio syndrome onset was the length of the interval following the acute illness, with incidence peaking at 30-34 years. Of all cases of post-polio syndrome, 79% reported no major change in impairment status since onset. This study demonstrates that poliomyelitis patients are not equally susceptible to post-polio syndrome within the interval of 30-40 years after the original illness. For syndrome cases, the onset was associated with new neuromuscular symptoms and functional changes but not with major new impairment.

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Category: Diagnosis and Management

Title: The cultural context of polio biographies
Author: Scheer J, Luborsky ML
Affiliation: National Rehabilitation Hospital Research Center, Washington, DC 20010
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1173-81
Publication Year and Month: 1991 11

Abstract: Cultural contexts influence the ways individuals interpret and experience functional losses associated with post-polio sequelae. Using in-depth multiple interview case studies from two National Institute on Aging projects, the concept of “biographies” is presented to place the individuals’ polio-related experiences within the context of their lives. Two major cultural contexts shape the construction of polio biographies: normative life course expectations and developmental tasks; and traditions associated with polio recovery and rehabilitation. The authors identify key dimensions of personal concern among polio survivors that can be used as entrance points for effective clinical intervention and to promote treatment compliance.

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Category: Diagnosis and Management

Title: Effect of treatment and noncompliance on post-polio sequelae
Author: Peach PE, Olejnik S
Affiliation: Roosevelt Warm Springs Institute for Rehabilitation, Georgia 31830
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1199-1203
Publication Year and Month: 1991 11

Abstract: In this study of 77 patients with post-polio sequelae (PPS), symptoms and manual test scores on initial evaluation were compared with those at subsequent follow-up evaluations. Patients were divided into three groups based on the degree to which they had complied with clinically recommended interventions: compliers, partial compliers, and noncompliers. At the end of the follow-up period (2.2 +/- 1.2 years), the mean muscle function scores of the entire study group had declined -1.5%, which represented a decline of -0.7% annually. On follow-up evaluations, the complier group had realized an improvement or resolution of post-polio symptoms, and also an improvement in muscle function of +0.6% annually. The partial complier group had realized either no improvement, or improvement in post-polio symptoms, but showed a further decline in muscle function of -3.0%, or an annual decline of -1.3%. The noncomplier group showed either no change, or a worsening of post-polio symptoms, and also showed a further decline in muscle function of -4.1%, which represented an annual decline of -2.0%.

Conclusions: The disparate outcomes among our post-polio patients underscore the need to develop more effective intervention strategies to achieve improved patient compliance, given the favorable outcomes experienced by patients who complied with clinical recommendations.

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Category: Diagnosis and Management

Title: Polioencephalitis, stress, and the etiology of post-polio sequelae
Author: Bruno RL, Frick NM, Cohen J
Affiliation: Post-Polio Rehabilitation and Research Service, Kessler Institute for Rehabilitation, East Orange, NJ 07018
Journal: Orthopedics
Citation: Orthopedics. 1991 Nov; 14(11):1269-76
Publication Year and Month: 1991 11

Abstract: Post-mortem neurohistopathologies that document polio virus-induced lesions in reticular formation and hypothalamic, thalamic, peptidergic, and monoaminergic neurons in the brain are reviewed from 158 individuals who contracted polio before 1950. This polioencephalitis was found to occur in every case of poliomyelitis, even those without evidence of damage to spinal motor neurons. These findings, in combination with data from the 1990 National Post-Polio Survey and new magnetic resonance imaging studies documenting post-encephalitis-like lesions in the brains of polio survivors, are used to present two hypotheses: 1) polioencephalitic damage to aging reticular activating system and monoaminergic neurons is responsible for post-polio fatigue, and 2) polioencephalitic damage to enkephalin-producing neurons is responsible for hypersensitivity to pain in polio survivors. In addition, the antimetabolic action of glucocorticoids on polio-damaged, metabolically vulnerable neurons may be responsible for the fatigue and muscle weakness reported by polio survivors during emotional stress.

Conclusions: The ability of the polio virus to produce symptoms by its destruction of neurons outside of the anterior horn has been accepted for more than 100 years. It is only our recent experience with PPS that has forced us to recognize that both the people who survived the original viral onslaught and their central nervous systems have been operating for decades under extreme stress. This stress now may be combining with the aging of an extensively damaged but here-to-fore remarkably functional central nervous system to reveal the previously hidden symptoms of polioencephalitis.

The above-presented hypotheses concerning the etiology of PPS fatigue suggest that reductions in both emotional and physical stress will reduce PPS. This is the experience of post-polio clinics throughout the world (36,54,55,56). As PPS fatigue continues to be treated with stress-management, work simplification and energy conservation (see 36,56), the hypotheses are being tested by studying the neuroanatomy, neuroendocrinology and neuropsychology of PPS fatigue. In addition, pharmacological means for stimulating the RAS that do not further metabolically stress its remaining aging, polio-damaged neurons are being tested.

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Category: Diagnosis and Management

Title: Motoneuron Disease and Past Poliomyelitis in England and Wales
Author: Martyn CN, Barker DJP, Osmond C.
Affiliation: MRC Environmental Epidemiology Unit, Southampton Hospital, United Kingdom
Journal: The Lancet Neurology
Citation: The Lancet, 331(8598):1319-1322

Publication Year and Month: 1988 06

Abstract: Past notification rates for poliomyelitis show a close geographical relation with current mortality from motoneuron disease in England and Wales. The increasing rate of poliomyelitis during the first half of this century and its predilection for affluent places and families were unique amongst infectious diseases. The unusual epidemiology of poliomyelitis is now being paralleled by motoneuron disease. These observations provide new evidence for a causal connection between the two conditions.

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Outcome of Research: More research required

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Category: Diagnosis and Management

Title: Physical therapy management of the patient with post-polio syndrome. A case report.
Author: Donna J. Twist, Dong M. Ma
Affiliation:
Journal: Physical Therapy
Citation: Volume 66, Issue 9, 1 September 1986, Pages 1403–1406
Publication Year and Month: 1986 09

Abstract: This case report documents the treatment of a patient who experienced progressive muscle weakness and a decrease in function over time that did not appear to be related to any secondary neuromuscular disease. We discuss the relationship between age and maximal muscle function in addition to some general guidelines for rehabilitation. This type of patient can represent a challenge for the physical therapist. This case report, however, illustrates the degree of muscular and functional recovery that can result with a physical therapy program aimed at reducing levels and intensity of exercise, daily activity, and stress.

Conclusions: A combination of short-term goals appears to be essential to the successful management of a patient with post-polio syndrome.

Outcome of Research: Not applicable

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Category: Diagnosis and Management

Title: Post-polio sequelae: physiological and psychological overview
Author: Frick NM, Bruno RL
Affiliation: Not stated
Journal: Rehabilitation Literature
Citation: Rehabil Lit. 1986 May-Jun; 47(5-6):106-11
Publication Year and Month: 1986 05

Abstract: When the Salk and Sabin vaccines brought an end to the annual summer nightmare of polio epidemics, most Americans simply forgot about polio. Even many of those who had paralytic poliomyelitis put the disease out of their minds once they had achieved maximum recovery of function. Unfortunately, polio has again forced itself into the nation's consciousness. Over the past five years, many of those who had polio have been experiencing new and unexpected symptoms that range in severity from being merely unpleasant to severely debilitating.

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Category: Diagnosis and Management

Title: Post-polio sequelae - differential diagnosis and management
Author: Maynard FM
Affiliation: Not stated
Journal: Orthopedics
Citation: Orthopedics. 1985 Jul; 8(7):857-61
Publication Year and Month: 1985 07

Abstract: Forty-two patients with a past history of poliomyelitis were evaluated at a post-polio clinic for new problems or impairments. Evaluation included a complete history, neurological and biomechanical examination and electrodiagnostic studies. Based on this evaluation patients were placed into three groups: 23 patients were considered to have or likely to have Progressive Post-Polio Muscular Atrophy (PPPMA); 17 patients were considered to have other post-polio sequelae; and two patients had problems unrelated to a past history of polio but mistaken for post-polio sequelae. Musculoskeletal pain was a common complaint among all groups of patients. Twenty-two of the 40 patients with post-polio sequelae were advised to alter their method of ambulation and/or decrease their activity pattern in order to decrease strain and/or excessive exertion of involved muscles. The role of chronic overuse and exercise in producing PPPMA or musculoskeletal pain problems is discussed. Characteristic clinical problems and useful management plans are described.

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Category: Diagnosis and Management

Title: Histopathologic Basis of Clinical Findings in Poliomyelitis
Author: Bodian, D.
Affiliation: Poliomyelitis Research Center, Department of Epidemiology, Johns Hopkins University, Baltimore
Journal: The American Journal of Medicine
Citation: 1949: 6(5):563–578
Publication Year and Month: 1949 05

Abstract: 1. Experimental evidence indicates that the onset of CNS pathologic changes occurs in the preparalytic period and is closely associated with the earliest evidence of virus activity in any particular region involved.
2. The earliest cytopathologic changes are diffuse chromatolysis of Nissl substance in the cytoplasm of nerve cells and mild cellular exudate consisting of polymorphonuclear and mononuclear leukocytes.
3. Nerve cell changes may be present in the earliest stages without inflammatory reaction in the vicinity and therefore are not necessarily the result of the latter, but rather the result of direct virus action.
4. Nerve cell changes either lead to rapid destruction of the cell or to arrest in the stage of cytoplasmic chromatolysis, following which complete morphologic recovery of the cell generally occurs over a period of about a month or less, depending upon the severity of injury.
5. Virus activity, nerve cell changes and inflammatory reaction are localized only in certain susceptible regions of the CNS, largely due to specific differences of susceptibility of nerve cells. The intensity of the inflammatory reaction, however, may be quite variable in different susceptible centers and in different individuals. Severe inflammatory reaction is usually but not always associated with extensive nerve cell destruction. Severe nerve cell damage may occur without extensive cellular infiltration in the cord.
6. Lesions in the cerebral cortex are usually confined to the motor area of the precentral gyrus and even here the lesions are rarely severe enough to suggest that they may produce clinical symptoms.
7. “Encephalitic” symptoms such as restlessness, stupor, disorientation and coma are associated with severe inflammatory reaction in the brainstem and often with small softenings in this region. They are not associated with unusual involvement of the cerebral cortex.
8. Brainstem centers principally involved in most instances are the reticular formation of the hind-brain, the vestibular nuclei and the roof nuclei of the cerebellum. Resulting functional disturbances are discussed.
9. Widespread dissemination of virus among most motor nerve cells in spinal cord enlargements occurs in experimental poliomyelitis as early as the first day of paralysis. Motor nerve cells which are affected either are destroyed very quickly during the first few days of the disease or undergo slower recovery changes leading to complete morphologic recovery within about a month. After this time it can be shown that the degree of paralysis and atrophy are closely correlated with the number of motor nerve cells destroyed. In the acute stage, however, this correlation is not as high and other factors must also play a role in producing paralysis. An important factor is the reversible injury of motor nerve cells. Less complete evidence from human material suggests that a similar situation obtains in human poliomyelitis.
10. Experimental work suggests three possible factors which may determine the variation in severity of infection. These are, first, variations due to difference in strains of the virus, second, reduction of severity due to previous paralytic or non-paralytic infection, and third, host variation unrelated to previous immunizing experience with the virus.

Conclusions: Poliomyelitis virus has strains that can vary depending on the host, and response to the virus. The virus causes most damage to the anterior horn cells of the cervical and lumbar segments, however there is damage to the brainstem and premotor areas in the brain.

Outcome of Research: More research required

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Comments (if any): David Bodian produced papers that helped enable (effective) availability for vaccinations. His work is still cited and referred to in understanding the effects of the polio virus, especially in relation to the central nervous system pathology.

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Outcomes of Research or Clinical Trials Activity Levels Acute Flaccid Paralysis Ageing Anaerobic Threshold Anaesthesia Assistive Technology Brain Cardiorespiratory Cardiovascular Clinical Evaluation Cold Intolerance Complementary Therapies Continence Coping Styles and Strategies Cultural Context Diagnosis and Management Differential Diagnosis Drugs Dysphagia Dysphonia Epidemiology Exercise Falls Fatigue Fractures Gender Differences Immune Response Inflammation Late Effects of Polio Muscle Strength Muscular Atrophy Orthoses Pain Polio Immunisation Post-Polio Motor Unit Psychology Quality of Life Renal Complications Respiratory Complications and Management Restless Legs Syndrome Sleep Analaysis Surgery Vitality Vocational Implications