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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

Activity Levels
Energy conservation, occupational therapy, and the treatment of post-polio sequelae
Individuals experiencing post-polio sequelae (PPS) are usually advised to make significant lifestyle changes to lessen symptoms and prevent further decline in function. These individuals have spent most of their lives equating success with over-achievement and find it difficult to implement such recommendations. As specialists in energy conservation and work simplification, occupational therapists increasingly are being called on to evaluate and treat these patients. Over the past 2 years, an occupational therapy educational program has been developed to educate patients about their condition and about ways to implement lifestyle changes while preserving the ability to do valued activities. This article describes the components of a thorough occupational therapy evaluation and the design and functional outcomes of a successful occupational therapy educational program to treat PPS.
Activity Levels
Neuromuscular function in polio survivors
Although there is no documented, objective evidence that symptomatic post-polio subjects are rapidly losing strength, they have a number of neuromuscular deficits related to a more severe poliomyelitis illness that may explain why they complain of problems with strength, endurance, and local muscle fatigue. Symptomatic post-polio subjects were hospitalized longer during the acute poliomyelitis, recovered more slowly, and had electromyographic evidence of greater loss of anterior horn cells. Additionally, recent assessment demonstrated that they were weaker, had a reduced work capacity, and recovered strength less readily after activity in the quadriceps muscles as compared to asymptomatic subjects. Of great clinical importance, rating of perceived exertion in the muscle during exercise was the same in symptomatic and asymptomatic post-polio and control subjects, indicating that symptomatic subjects have a mechanism to monitor local muscle fatigue that could be used to avoid exhaustion. A study of pacing (interspersing activity with rest breaks) showed that symptomatic subjects had less local muscle fatigue and greater strength recovery when they paced their activity than when they worked at a constant rate to exhaustion. We recommend that post-polio individuals pace their daily activity to avoid excessive fatigue.
Activity Levels
Actual and perceived activity levels in polio survivors and older controls: a longitudinal study
OBJECTIVE: To examine factors associated with daily step activity, perceived activity, maximum walking speed, and walking speed reserve over time in polio survivors and older adults with no history of polio.

DESIGN: Longitudinal study.

SETTING: A research clinic and the community.

PARTICIPANTS: Polio survivors (n=96; 65 in postpolio syndrome [PPS] group, 31 in non-PPS group) and older adults (n=112) with no history of polio.

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Daily step activity, perceived activity, maximum walking speed, and walking speed reserve.

RESULTS: Results showed decreases in perceived activity over time in the PPS group. However, there was no change in average daily walking activity. Overall, polio survivors walk less and have a smaller walking speed reserve than controls. Knee strength was positively associated with maximum walking speed and walking speed reserve in all groups. Weight and age were associated with daily step activity in controls but not polio survivors.
Activity Levels
The postpolio syndrome – an overuse phenomenon
Persons with good recovery of function following their initial poliomyelitis are now, more than 30 years later, experiencing new weakness, fatigue, and muscle pain. The likelihood of muscle overuse being the cause of this late functional loss was investigated by dynamic electromyography (EMG) and foot-switch stride analysis in 34 symptomatic patients. Manual testing grouped the muscles, with strong (S) encompassing Grades Good (G) and Normal (N) while weak (W) included Fair plus (F+) to zero (0). After testing quadriceps and calf strength, the patients fell into one of four classes: strong quadriceps and calf (SQ/SC), strong quadriceps and weak calf (SQ/WC), weak quadriceps and strong calf (WQ/SC), or combined weak quadriceps and calf (WQ/WC). Quantified EMG (normalized by the manual muscle test EMG) defined the mean duration and intensity of the quadriceps, soleus, lower gluteus maximus, and long head of the biceps femoris during walking. Overuse was defined as values greater than the laboratory normal (mean + 1 SD). Each muscle exhibited instances of overuse, normalcy, and sparing. The biceps femoris was the only muscle with dominant overuse (82%). Quadriceps overuse was next in frequency (53%). Soleus activity infrequently exceeded normal function (34%), but this still represented more than twice the intensity and duration of the other muscles. Gluteus maximus action was also seldom excessive (34%). The patients averaged two muscles with excessive use during walking. Gait velocity of the SQ/SC strong group was highest (71% of normal) while the three categories that included weak muscles had walking speeds in the range of 50% of normal. The finding of muscle overuse during a single free-speed walking test that does not attain normal velocity supports the concept of muscle overuse being the cause of the patient's dysfunction.
Activity Levels
Walking in postpoliomyelitis syndrome: the relationships between time‐scored tests, walking in daily life and perceived mobility problems
OBJECTIVE: To compare walking test results with walking in daily life, and to investigate the relationships between walking tests, walking activity in daily life, and perceived mobility problems in patients with post-poliomyelitis syndrome.

SUBJECTS: Twenty-four ambulant patients with post-poliomyelitis syndrome.

METHODS: Walking tests were performed at self-preferred and maximal speed. Walking activity was measured with an ambulatory activity monitor. Heart rate, step cadence and walking speed in the test and in daily life were compared. Walking speed in daily life was represented by the intensity of walking. Perceived mobility problems were assessed with the Nottingham Health Profile.

RESULTS: Heart rate during walking was lower in the test at self-preferred speed than in daily life (mean difference: 11.3+/-10.4; p=0.001). Self-preferred walking speed in the test and in daily life correlated significantly (r=0.55; p=0.04). In a sub-group with a test performance below the median value, test performance correlated significantly with walking activity. No significant correlation was found between perceived mobility problems and walking activity.
Activity Levels
Change in physical mobility over 10 years in post-polio syndrome
Post-polio syndrome is characterised by progressive muscle weakness and other symptoms which can limit physical mobility. We assessed the rate of decline in mobility over 10 years in relation to strength decline; and investigated potential predictors for the rate of decline of walking capacity, a measure of mobility, in 48 patients with post-polio syndrome and proven quadriceps dysfunction at baseline. Average walking capacity and self-reported physical mobility declined over 10 years, by 6 and 14%, respectively. Concomitantly people lost an average of 15% of isometric quadriceps strength. Significantly more people used walking aids offering greater support at follow-up. Notably, there was much individual variation, with 18% of participants losing a substantial amount of walking capacity (27% decline) and concomitant self-reported physical mobility (38% decline). Loss of quadriceps strength only explained a small proportion of the variance of the decline in walking capacity (R = 11%) and the rate of decline could not be predicted from baseline values for strength, walking capacity, self-reported physical mobility or basic demographics. The individual variability, yet lack of predictive factors, underscores the need for personally tailored care based on actual functional decline in patients with post-polio syndrome.
Activity Levels
Reproducibility of walking at self-preferred and maximal speed in patients with postpoliomyelitis syndrome
To assess the reproducibility of walking performance, heart rate, and perceived exertion at self-preferred speed and maximal walking speed in patients with the postpoliomyelitis syndrome (PPS).

DESIGN: Repeated measurement at a 3-week interval.

SETTING: University hospital.

PARTICIPANTS: Convenience sample of 65 patients with PPS.

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Walking performance: the distance walked in 2 minutes at a self-preferred speed and the time needed to walk 75 m at maximal speed, heart rate, and rating of perceived exertion (RPE) on an 11-point scale.

RESULTS: Test-retest reliability of walking performance was excellent for both tests (intraclass correlation coefficient [ICC] range, .94-.97). No systematic differences existed between test and retest. The smallest detectable change for an individual was 15% for both tests. Test-retest reliability for heart rate was good (ICC=.86) but moderate for RPE (Spearman rho range, .67-.70). The smallest detectable change for RPE was between 4 and 6 scale points. The variability in walking performance was significantly correlated with the variability in heart rate at self-preferred speed (r =.36, P <.01) but not with the variability in RPE (r =.20, P =.11).
Activity Levels
Change in physical mobility over 10 years in post-polio syndrome.
Post-polio syndrome is characterised by progressive muscle weakness and other symptoms which can limit physical mobility. We assessed the rate of decline in mobility over 10 years in relation to strength decline; and investigated potential predictors for the rate of decline of walking capacity, a measure of mobility, in 48 patients with post-polio syndrome and proven quadriceps dysfunction at baseline. Average walking capacity and self-reported physical mobility declined over 10 years, by 6 and 14%, respectively. Concomitantly people lost an average of 15% of isometric quadriceps strength. Significantly more people used walking aids offering greater support at follow-up. Notably, there was much individual variation, with 18% of participants losing a substantial amount of walking capacity (27% decline) and concomitant self-reported physical mobility (38% decline). Loss of quadriceps strength only explained a small proportion of the variance of the decline in walking capacity (R = 11%) and the rate of decline could not be predicted from baseline values for strength, walking capacity, self-reported physical mobility or basic demographics. The individual variability, yet lack of predictive factors, underscores the need for personally tailored care based on actual functional decline in patients with post-polio syndrome.
Activity Levels, Psychology
Exercise therapy and cognitive behavioural therapy to improve fatigue, daily activity performance and quality of life in postpoliomyelitis syndrome: the protocol of the FACTS-2-PPS trial
BACKGROUND: Postpoliomyelitis Syndrome (PPS) is a complex of late onset neuromuscular symptoms with new or increased muscle weakness and muscle fatigability as key symptoms. Main clinical complaints are severe fatigue, deterioration in functional abilities and health related quality of life. Rehabilitation management is the mainstay of treatment. Two different therapeutic interventions may be prescribed (1) exercise therapy or (2) cognitive behavioural therapy (CBT). However, the evidence on the effectiveness of both interventions is limited. The primary aim of the FACTS-2-PPS trial is to study the efficacy of exercise therapy and CBT for reducing fatigue and improving activities and quality of life in patients with PPS. Additionally, the working mechanisms, patients' and therapists' expectations of and experiences with both interventions and cost-effectiveness will be evaluated.

METHODS/DESIGN: A multi-centre, single-blinded, randomized controlled trial will be conducted. A sample of 81 severely fatigued patients with PPS will be recruited from 3 different university hospitals and their affiliate rehabilitation centres. Patients will be randomized to one of three groups i.e. (1) exercise therapy + usual care, (2) CBT + usual care, (3) usual care. At baseline, immediately post-intervention and at 3- and 6-months follow-up, fatigue, activities, quality of life and secondary outcomes will be assessed. Costs will be based on a cost questionnaire, and statistical analyses on GEE (generalized estimated equations). Analysis will also consider mechanisms of change during therapy. A responsive evaluation will be conducted to monitor the implementation process and to investigate the perspectives of patients and therapists on both interventions.

DISCUSSION: A major strength of the FACTS-2-PPS study is the use of a mixed methods design in which a responsive and economic evaluation runs parallel to the trial. The results of this study will generate new evidence for the rehabilitation treatment of persons with PPS.

TRIAL REGISTRATION: Dutch Trial Register NTR1371.
Activity Levels, Psychology,
No reduction of severe fatigue in patients with postpolio syndrome by exercise therapy or cognitive behavioral therapy: results of an RCT
BACKGROUND: People with postpolio syndrome (PPS) commonly experience severe fatigue that persists over time and negatively affects functioning and health-related quality of life (HRQoL).

OBJECTIVES: To study the efficacy of exercise therapy (ET) and cognitive behavioral therapy (CBT) on reducing fatigue and improving activities and HRQoL in patients with PPS.

METHODS: We conducted a multicenter, single-blinded, randomized controlled trial. Over 4 months, severely fatigued patients with PPS received ET, CBT, or usual care (UC). The primary end point (fatigue) was assessed using the subscale fatigue severity of the Checklist Individual Strength (CIS20-F). Secondary end points included activities and HRQoL, which were assessed with the Sickness Impact Profile and the 36-Item Short-Form, respectively. End points were measured at baseline and at 4, 7, and 10 months.

RESULTS: A total of 68 patients were randomized. No differences were observed between the intervention groups and UC group for fatigue (mean differences in CIS20-F score = 1.47, 95%CI = -2.84 to 5.79, for ET versus UC; and 1.87, 95%CI = -2.24 to 5.98, for CBT versus UC), activities, or HRQoL.
Acute Flaccid Paralysis
Acute Flaccid Paralysis Associated with Novel Enterovirus C105
An outbreak of acute flaccid paralysis among children in the United States during summer 2014 was tentatively associated with enterovirus D68 infection. This syndrome in a child in fall 2014 was associated with enterovirus C105 infection. The presence of this virus strain in North America may pose a diagnostic challenge.
Acute Flaccid Paralysis
Acute Flaccid paralysis in adults: Our experience
Acute flaccid paralysis (AFP) is a complex clinical syndrome with a broad array of potential etiologies that vary with age. We present our experience of acute onset lower motor neuron paralysis.

MATERIALS AND METHODS: One hundred and thirty-three consecutive adult patients presenting with weakness of duration less than four weeks over 12 months period were enrolled. Detailed history, clinical examination, and relevant investigations according to a pre-defined diagnostic algorithm were carried out. The patients were followed through their hospital stay till discharge or death.

RESULTS: The mean age was 33.27 (range 13-89) years with male preponderance (67.7%). The most common etiology was neuroparalytic snake envenomation (51.9%), followed by Guillain Barre syndrome (33.1%), constituting 85% of all patients. Hypokalemic paralysis (7.5%) and acute intermittent porphyria (4.5%) were the other important conditions. We did not encounter any case of acute poliomylitis in adults. In-hospital mortality due to respiratory paralysis was 9%.
Acute Flaccid Paralysis
Potential for the Australian and New Zealand paediatric intensive care registry to enhance acute flaccid paralysis surveillance in Australia: a data-linkage study
BACKGROUND: Australia uses acute flaccid paralysis (AFP) surveillance to monitor its polio-free status. The World Health Organization criterion for a sensitive AFP surveillance system is the annual detection of at least one non-polio AFP case per 100,000 children aged less than 15 years, a target Australia has not consistently achieved. Children exhibiting AFP are likely to be hospitalised and may be admitted to an intensive care unit. This provides a potential opportunity for active AFP surveillance.

METHODS: A data-linkage study for the period from 1 January 2005 to 31 December 2008 compared 165 non-polio AFP cases classified by the Polio Expert Panel with 880 acute neurological presentations potentially compatible with AFP documented in the Australian and New Zealand Paediatric Intensive Care (ANZPIC) Registry.

RESULTS: Forty-two (25%) AFP cases classified by the Polio Expert Panel were matched to case records in the ANZPIC Registry. Of these, nineteen (45%) cases were classified as Guillain-Barré syndrome on both registries. Ten additional Guillain-Barré syndrome cases recorded in the ANZPIC Registry were not notified to the national AFP surveillance system.
Acute Flaccid Paralysis
Human Enteroviruses isolated during acute flaccid paralysis surveillance in Ghana: implications for the post eradication era
INTRODUCTION: Surveillance of acute flaccid surveillance (AFP) has been used world-wide to monitor the control and eradication of circulating wild polioviruses. The Polio Laboratory since its accreditation in 1996 has supported the Disease Surveillance Department for AFP surveillance. This study aims to isolate and characterize human enteroviruses from patients with AFP in Ghana.

METHOD: Stool suspension was prepared from 308 samples received in 2009 from the surveillance activities throughout the country and inoculated on both RD and L20B cell lines. Isolates that showed growth on L20B were selected for real-time RT-PCR using degenerate and non-degenerate primers and probes. RD isolates were however characterized by microneutralisation technique with antisera pools from RIVM, The Netherlands and viruses that were untypable subjected to neutralization assay using antibodies specific for E71.

RESULTS: Of the 308 samples processed, 17 (5.5%) grew on both L20B and RD cells while 32 (10.4%) grew on RD only. All 28 isolates from L20B were characterized by rRT-PCR as Sabin-like polioviruses. No wild poliovirus or VDPV was found. However from the microneutralisation assay, six different enteroviruses were characterized. Among these, Coxsackie B viruses were most predominant followed by Echovirus. Three children from whom non-polio enteroviruses were isolated had residual paralysis while one child with VAPP found. The non-polio enteroviruses circulated throughout the country with the majority (20.7%) from Ashanti region.
Aging and sequelae of poliomyelitis
OBJECTIVE: We estimate that there are about 50,000 persons who survived poliomyelitis in their childhood in France (mean age estimated between 50 and 65 years). After a few decades of stability, 30 to 65% of individuals who had been infected and recovered from polio begin to experience new signs and symptoms.

METHOD: Review of the literature on Pubmed with the following keywords "Poliomyelitis" and "Post-Polio Syndrome (PPS)".

RESULTS: These new signs and symptoms are characterized by muscular atrophy (decreased muscle mass), muscle weakness and fatigue, muscle and/or joint pain. All these symptoms lead to significant changes in mobility with falls and inability to carry on with daily life activities. There are several intricate causes. The normal aging process and weight gain are regularly blamed. Respiratory disorders and sleep disorders must be looked for: respiratory insufficiency, sleep-related breathing disorders such as sleep apnea, restless legs syndrome. Orthopedics complications are quite common: soft-tissue pathologies of the upper limbs, degenerative pathologies of the large joints or spinal cord, fall-related fractures. Finally, the onset of an authentic PPS is possible.
Review of secondary health conditions in postpolio syndrome: prevalence and effects of aging
OBJECTIVE: This study sought to better understand the prevalence and the severity of secondary health conditions in individuals with postpolio syndrome (PPS) as well as the association between these conditions and aging.

DESIGN: A scoping literature review was conducted searching electronic databases for studies published from 1986 to 2011. The scoping review provided information regarding the prevalence and associations of secondary health conditions in PPS with age or other duration-related variables.

RESULTS: The findings indicate that (1) individuals with PPS experience a number of serious secondary health conditions; (2) the most common conditions or symptoms are fatigue, pain, respiratory and sleep complaints, and increased risk for falls; (3) reports of the associations between the frequency or the severity of conditions and age-related factors are variable, perhaps because of methodological inconsistencies between studies; and (4) there is a marked lack of longitudinal research examining the natural course of health conditions in people aging with PPS
Symptom profiles in individuals aging with post-polio syndrome
This paper does not have an abstract.
Changes in Post-Polio Survivors Over Five Years: Symptoms and Reactions to Treatments.
A group of 176 people with post-polio syndrome, identified using Ramlow et al's (1992) criteria, took part in a 5 year follow-up survey. Most reported increased muscle weakness (91%), fatigue (91%), muscle pain (80%), joint pain (64%) and changes in walking (60%). Increases in other symptoms occurred in less than half the group. Cramps, sensitivity to cold, muscle atrophy and muscle twitching were the symptoms most likely to have stabilised. The average respondent reported greater difficulty in carrying out 4 of the 8 activities of daily living investigated. Respondents were significantly less anxious and depressed about their condition at follow-up. Degree of post-polio changes reported at the time of the first survey was a better predictor of decline during the five years than were initial polio histories or psycho-social variables. Health practitioners most likely to have been consulted were general practitioners and physiotherapists. Specialists in rehabilitation medicine were rated as providing more beneficial treatment than other medical practitioners. Treatments reported to provide good symptom relief included massage and water activities but not exercise. Life style modifications associated with pacing, reduced activity and rest were particularly effective. Overall 68% of respondents considered there was much they could do to control post-polio symptoms.
Assistive Technology, Orthoses
Evaluation of gait symmetry in poliomyelitis subjects: Comparison of a conventional knee-ankle-foot orthosis and a new powered knee-ankle-foot orthosis
BACKGROUND: Compared to able-bodied subjects, subjects with post-polio syndrome and poliomyelitis demonstrate a preference for weight-bearing on the non-paretic limb, causing gait asymmetry.

OBJECTIVES: The purpose of this study was to evaluate the gait symmetry of the poliomyelitis subjects when ambulating with either a drop-locked knee-ankle-foot orthosis or a newly developed powered knee-ankle-foot orthosis.

STUDY DESIGN: Quasi experimental study.

METHODS: Seven subjects with poliomyelitis who routinely wore conventional knee-ankle-foot orthoses participated in this study and received training to enable them to ambulate with the powered knee-ankle-foot orthosis on level ground, prior to gait analysis.

RESULTS: There were no significant differences in the gait symmetry index of step length (p = 0.085), stance time (p = 0.082), double-limb support time (p = 0.929), or speed of walking (p = 0.325) between the two test conditions. However, using the new powered knee-ankle-foot orthosis improved the symmetry index in step width (p = 0.037), swing time (p = 0.014), stance phase percentage (p = 0.008), and knee flexion during swing phase (p ⩽ 0.001) compared to wearing the drop-locked knee-ankle-foot orthosis.
Assistive Technology, Orthoses
Wearable monitoring devices for assistive technology: case studies in post-polio syndrome
The correct choice and customization of an orthosis are crucial to obtain the best comfort and efficiency. This study explored the feasibility of a multivariate quantitative assessment of the functional efficiency of lower limb orthosis through a novel wearable system. Gait basographic parameters and energetic indexes were analysed during a Six-Minute Walking Test (6-MWT) through a cost-effective, non-invasive polygraph device, with a multichannel wireless transmission, that carried out electro-cardiograph (ECG); impedance-cardiograph (ICG); and lower-limb accelerations detection. Four subjects affected by Post-Polio Syndrome (PPS) were recruited. The wearable device and the semi-automatic post-processing software provided a novel set of objective data to assess the overall efficiency of the patient-orthosis system. Despite the small number of examined subjects, the results obtained with this new approach encourage the application of the method thus enlarging the dataset to validate this promising protocol and measuring system in supporting clinical decisions and out of a laboratory environment.
Assistive Technology, Quality of Life
Assistive technology and prediction of happiness in people with post-polio syndrome
PURPOSE: To explore the relationship between level of happiness in people with post-polio syndrome (PPS) and assistive technology (AT) by taking into account confounding factors such as age, gender and house composition.

METHOD: Existing data from 218 adults with PPS, who had completed a cross-sectional survey conducted by the British Polio Fellowship in 2007, were used for a secondary quantitative analysis. Ordinal logistic regression was applied to determine whether ownership of or the need for AT predicted happiness in people with PPS.

RESULTS: Ownership of AT did not predict happiness, whereas the perceived need for AT was a significant predictor of feeling less happy (p = 0.028). Among the different types of AT needed, only need of home adaptations combined with major equipment was close to being significantly associated with less happiness (p = 0.078). Being older (p < 0.001) and living with a partner (p < 0.001) significantly increased the likelihood of feeling happier.
Parallels between Post Polio fatigue and chronic fatigue syndrome: a common pathophysiology?
Fatigue is the most commonly reported and most debilitating of post-polio sequelae affecting the >1.8 million North American polio survivors. Post-polio fatigue is characterized by subjective reports of difficulty with attention, cognition, and maintaining wakefulness. These symptoms resemble those reported in nearly 2 dozen outbreaks of post-viral fatigue syndromes (PVFS) that have recurred during this century and that are related clinically, historically, anatomically, or physiologically to poliovirus infections. This article reviews recent studies that relate the symptoms of post-polio fatigue and chronic fatigue syndrome (CFS) to clinically significant deficits on neuropsychologic tests of attention, histopathologic and neuroradiologic evidence of brain lesions, impaired activation of the hypothalamic-pituitary-adrenal axis, increased prolactin secretion, and electroencephalogram (EEG) slow-wave activity. A possible common pathophysiology for post-polio fatigue and CFS, based on the Brain Fatigue Generator Model of PVFS, and a possible pharmacotherapy for PVFS based on replacement of depleted brain dopamine, will be described
Clinical Evaluation
Quantitative muscle ultrasound and quadriceps strength in patients with post-polio syndrome.
We investigated whether muscle ultrasound can distinguish muscles affected by post-polio syndrome (PPS) from healthy muscles and whether severity of ultrasound abnormalities is associated with muscle strength.
Echo intensity, muscle thickness, and isometric strength of the quadriceps muscles were measured in 48 patients with PPS and 12 healthy controls.
Patients with PPS had significantly higher echo intensity and lower muscle thickness than healthy controls. In patients, both echo intensity and muscle thickness were associated independently with muscle strength. A combined measure of echo intensity and muscle thickness was more strongly related to muscle strength than either parameter alone.
Quantitative ultrasound distinguishes healthy muscles from those affected by PPS, and measures of muscle quality and quantity are associated with muscle strength. Hence, ultrasound could be a useful tool for assessing disease severity and monitoring changes resulting from disease progression or clinical intervention in patients with PPS.
Complementary Therapies
Hatha yoga and meditation in patients with post-polio syndrome
This paper does not have an abstract. The following is an extract:
Conemaugh Health System has completed a preliminary outcome study evaluating the benefits of Hatha yoga and meditation in patients with post-polio syndrome (PPS). This research integrates clinical trials investigating the application of Hatha yoga with ongoing patient care and education. The results of this clinical trial will be used to develop a longitudinal data collection effort integrating research and clinical trials investigating the applications of Hatha yoga in ongoing patient care and education.
Complementary Therapies
A randomized controlled trial of coenzyme Q10 for fatigue in the late-onset sequelae of poliomyelitis
OBJECTIVE: To determine if coenzyme Q10 alleviates fatigue in the late-onset sequelae of poliomyelitis.

DESIGN: Parallel-group, randomized, placebo-controlled trial.

BACKGROUND SETTING: Coenzyme Q10 has been shown to boost muscle energy metabolism in post-polio subjects but it does not promote muscle strength, endurance or function in polio survivors with post-poliomyelitis syndrome. However, the collective increased energy metabolism might contribute to a reduction in post-polio fatigue.

PARTICIPANTS: Polio survivors from the Australian post-polio networks in Queensland and New South Wales who attribute a moderate to high level of fatigue to their diagnosed late-onset sequelae of poliomyelitis. Those with fatigue-associated comorbidities of diabetes, anaemia, hypothyroidism and fibromyalgia were excluded.

METHOD: Participants were assigned (1:1), with stratification of those who use energy-saving mobility aids, to receive 100 mg coenzyme Q10 or matching placebo daily for 60 days. Participants and investigators were blinded to group allocation. Fatigue was assessed by the Multidimensional Assessment of Fatigue as the primary outcome and the Fatigue Severity Scale as secondary outcome.

RESULTS: Of 103 participants, 54 were assigned to receive coenzyme Q10 and 49 to receive the placebo. The difference in the mean score reductions between the two groups was not statistically significant for either fatigue measure. Oral supplementation with coenzyme Q10 was safe and well-tolerated.

The registration number for the clinical trial is ACTRN 12612000552886.
Urologic manifestations of postpolio syndrome
To determine the character and prevalence of bladder dysfunction among persons with a history of polio, we conducted a survey of randomly selected polio survivors. The survey solicited information on the acute episode of polio and the nature of late-onset neurologic symptoms that could be attributed to postpolio syndrome. There were 242 female and 88 male respondents to the study. Symptoms attributable to postpolio syndrome were present in 87.2% of female subjects and 73.9% of male subjects. Respondents with postpolio syndrome had a significantly greater prevalence of urologic symptoms than seen among respondents without postpolio syndrome, although no dominant pattern of voiding dysfunction was noted. The early onset of erectile dysfunction was more common among male subjects with postpolio syndrome than among male subjects without postpolio syndrome. Genuine stress incontinence was seen in 36.3% of the survey population. Sixteen women with postpolio syndrome underwent surgical repair for urinary incontinence, with a success rate of 60.5%. Bladder disorders are common among persons with PPS, but further clinical and urodynamic data are necessary to define the nature and magnitude of this dysfunction.
Bladder symptoms among polio survivors
OBJECTIVE: To describe bladder symptoms among polio survivors and the inconvenience they cause.

DESIGN: A survey using the validated Danish Prostatic Symptom Score questionnaire concerning bladder symptoms.

SUBJECTS: A random age- and gender-stratified sample of polio survivors drawn from members of the Danish Society of Polio and Accident Victims.

METHODS: Eligible subjects were sent the questionnaire by post.

RESULTS: Of 453 eligible subjects 272 responded. Within 2 weeks 87.5% of respondents had experienced at least one bladder symptom, and 76.5% reported that they had been bothered by a bladder symptom. Out of the 12 symptoms in the questionnaire we found a significant gender difference, with straining and weak stream reported more often among men, and urge incontinence reported more often among women. Incontinence occurred significantly more often among women (73.3%) than among men (40.9%). Compared with a similar study of the background population, bladder symptoms overall occurred approximately twice as often among polio survivors.
Diagnosis and Management
The cultural context of polio biographies
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.
Diagnosis and Management
Post-polio sequelae - differential diagnosis and management
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.
Diagnosis and Management
Effect of treatment and noncompliance on post-polio sequelae
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%.
Diagnosis and Management
Polioencephalitis, stress, and the etiology of post-polio sequelae
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.
Diagnosis and Management
EFNS guideline on diagnosis and management of post-polio syndrome - report of an EFNS task force
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.
Diagnosis and Management
Post-polio sequelae: physiological and psychological overview
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.
Diagnosis and Management
Management of postpolio syndrome
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.
Diagnosis and Management
Characteristics and management of postpolio syndrome
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.
Diagnosis and Management
Postpolio syndrome: unanswered questions regarding cause, course, risk factors, and therapies
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.
Diagnosis and Management
Postpolio syndrome
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.
Diagnosis and Management
Epidemiology of the post-polio syndrome
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.
Diagnosis and Management
Post-poliomyelitis syndrome
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.
Diagnosis and Management
Management of post-polio syndrome
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.
Diagnosis and Management
Cardiac risk factors in polio survivors
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.
Diagnosis and Management
Update on current and emerging treatment options for post-polio syndrome
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.
Diagnosis and Management
Physiotherapy for poliomyelitis: a descriptive study in the Republic of Congo
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.
Diagnosis and Management
Post-poliomyelitis syndrome as a possible viral disease
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.
Diagnosis and Management
Treatment for postpolio syndrome
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.
Diagnosis and Management
Unraveling the transmission ecology of polio
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.
Diagnosis and Management
Nonparalytic polio and postpolio syndrome
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.
Diagnosis and Management
Paralytic vs "nonparalytic" polio: distinction without a difference?
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.
Diagnosis and Management
Predictive factors for post-poliomyelitis syndrome
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-affiliated 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.
Diagnosis and Management
Frequency and clinical manifestations of post-poliomyelitis syndrome in a Brazilian tertiary care center
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%).
Diagnosis and Management
A positive turning point in life -- how persons with late effects of polio experience the influence of an interdisciplinary rehabilitation programme
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.
Diagnosis and Management
The post-polio syndrome as an evolved clinical entity. Definition and clinical description.
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.
Diagnosis and Management
Predictive Factors for Post-Poliomyelitis Syndrome
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.
Diagnosis and Management
Effect of Treatment and Noncompliance on Post-Polio Sequelae
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 followup period (2.2 ± 1.2 years), the mean muscle function scores of the entire study group had declined - l.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%.
Diagnosis and Management
National Rehabilitation Hospital Limb Classification for Exercise, Research, and Clinical Trials in Post-Polio Patients
Diagnosis and Management
Polio survivors perceptions of a multi-disciplinary rehabilitation programme
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.

Diagnosis and Management
Characteristics of Patients at First Visit to a Polio Clinic in Sweden
Describe polio patients visiting a polio clinic in Sweden, a country where vaccination was introduced in 1957.

A consecutive cohort study.

Prior polio patients.

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.

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.

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.
Prior poliomyelitis – IVIg treatment reduces proinflammatory cytokine production
The postpolio syndrome (PPS) is characterized by progressive disabilities decades after recovery from the acute paralytic disease. There are reports on intrathecal inflammatory reactions in PPS, including increased expression of cytokines by cerebrospinal fluid (CSF) mononuclear cells (CSF-MC). This is potentially of relevance for the clinical condition. We here explored if cytokine expression in the CSF of PPS patients could be modulated by high-dose intravenous immunoglobulins (IvIg). The expression of TNF-alpha, IFN-gamma, IL-10 and IL-4 mRNAs was measured by real-time RT-PCR in CSF and peripheral blood mononuclear cells (PBMC) of 16 PPS patients before, and 6-8 weeks after IvIg treatment, and in 26 patients with noninflammatory other neurological diseases (OND). TNF-alpha, IFN-gamma and IL-10 CSF mRNA levels were elevated in samples from untreated persons with PPS compared to OND. Upon IvIg treatment, IFN-gamma and TNF-alpha mRNA levels were dramatically reduced, while IL-10 remained unchanged. Placebo-controlled studies are now warranted to evaluate if IvIg treatment also has any effects on the clinical manifestations of PPS.
Intravenous immunoglobulin treatment of the post-polio syndrome: sustained effects on quality of life variables and cytokine expression after one year follow up
BACKGROUND: Expression of inflammatory cytokines in cerebrospinal fluid (CSF) has led to the hypothesis of intrathecal chronic inflammation to explain the denervation observed in post-polio syndrome (PPS). It has been shown that therapy with intravenous immunoglobulin (IVIG) improves physical performance and dampens down the inflammatory process at 6 months in PPS patients. We here examined the effects of IVIG on cytokine expression and clinical outcome one year after IVIG treatment.

METHODS: From a previous study with 135 PPS patients included, 41 patients were further evaluated before un-blinding for one year (21 placebo and 20 treated with IVIG, Xepol® 50 mg/ml), and were assessed for clinical variables by performing the Short Form-36 survey (SF-36) questionnaire assessment, the 6 minute walk distance test (6MWT) and registering pain level by Visual Analogue Scale (VAS) after IVIG treatment. A separate cohort of 37 PPS patients went through lumbar puncture (LP) at baseline and 20 patients, treated with IVIG, repeated the LP one year later. Thirty patients affected with other neurological diseases (OND) were used as control group. Inflammatory cytokines TNF, TGFβ, IFNγ, IL-23, IL-13 and IL-10 were measured in blood cells and CSF cells with RT-PCR.

RESULTS: Scores of the physical components of SF-36 were significantly higher at the one year follow up time-point in the IVIG-treated patients when compared to baseline as well as to the control subjects. Pain VAS score and 6MWT improved significantly in the IVIG-treated patients when compared with baseline Relative expression of TNF and IFN-γ in both PBMCs and CSF from PPS patients were increased compared to OND subjects at baseline (p < 0.05). One year after IVIG-treatment a decreased expression of IFN-γ and IL23 was found in CSF of PPS patients, while anti-inflammatory IL-13 was increased (p < 0.05).
Pyridostigmine in postpolio syndrome: no decline in fatigue and limited functional improvement
OBJECTIVES: To investigate the effect of pyridostigmine on fatigue, physical performance, and muscle function in subjects with postpoliomyelitis syndrome.

METHODS: 67 subjects with increased fatigue and new weakness in one quadriceps muscle showing neuromuscular transmission defects, were included in a randomised, double blind, placebo controlled trial of 60 mg pyridostigmine four times a day for 14 weeks. Primary outcome was fatigue (on the "energy" category of the Nottingham health profile). Secondary outcomes included two minute walking distance and quadriceps strength and jitter. Motor unit size of the quadriceps was studied as a potential effect modifier. The primary data analysis compared the changes from baseline in the outcomes in the last week of treatment between groups.

RESULTS: 31 subjects treated with pyridostigmine and 31 subjects treated with placebo completed the trial. No significant effect of pyridostigmine was found on fatigue. The walking distance improved more in the pyridostigmine group than in the placebo group (by 7.2 m (6.0%); p<0.01). Subgroup analysis showed that a significant improvement in walking performance was only found in subjects with normal sized motor units. Quadriceps strength improved more in the pyridostigmine group than in the placebo group (by 6.7 Nm (7.2%); p = 0.15). No effect of pyridostigmine was found on jitter.
Effect of intravenous immunoglobulin on pain in patients with post-polio syndrome
OBJECTIVE: Pain is a common symptom that affects quality of life in patients with post-polio syndrome. An increase in cytokine in the cerebrospinal fluid suggests that inflammation is pathophysiologically important in post-polio syndrome. Intravenous immunoglobulin might therefore be a therapeutic option. The aim of this study was to analyse the effect of intravenous immunoglobulin treatment on pain in post-polio syndrome.

METHODS: An uncontrolled clinical study. Patients with post-polio syndrome and pain (n = 45) underwent a neurological examination and were interviewed about pain before and 6 months after treatment with intravenous immunoglobulin. Pain intensity was measured on a visual analogue scale. The pain was classified according to the International Association for the Study of Pain criteria as neuropathic when it occurred in an area with decreased sensibility, or nociceptive when signs of inflammation and/or painful joints movements were present.

RESULTS: After treatment 31/45 (69%) patients were improved, with a mean visual analogue scale decrease from 53 to 42 (p = 0.001). Eighteen patients (40%) had a decrease of 20 or more points on the visual analogue scale. The effect of treatment did not differ regarding age, gender and severity of disability.
Efficacy of Modafinil on fatigue and excessive daytime sleepiness associated with neurological disorders: a systematic review and meta-analysis
BACKGROUND: Modafinil is a novel wake-promoting agent approved by the FDA ameliorating excessive daytime sleepiness (EDS) in three disorders: narcolepsy, shift work sleep disorder and obstructive sleep apnea. Existing trials of modafinil for fatigue and EDS associated with neurological disorders provided inconsistent results. This meta-analysis was aimed to assess drug safety and effects of modafinil on fatigue and EDS associated with neurological disorders.

METHODS: A comprehensive literature review was conducted in order to identify published studies assessing the effects of modafinil on fatigue and EDS associated with neurological disorders. Primary outcomes included fatigue and EDS. Secondary outcomes included depression and adverse effects.

FINDINGS: Ten randomized controlled trials were identified including 4 studies of Parkinson's disease (PD), 3 of multiple sclerosis (MS), 2 of traumatic brain injury (TBI) and 1 of post-polio syndrome (PPS). A total of 535 patients were enrolled. Our results suggested a therapeutic effect of modafinil on fatigue in TBI (MD -0.82 95% CI -1.54 - -0.11 p=0.02, I(2)=0%), while a beneficial effect of modafinil on fatigue was not confirmed in the pooled studies of PD or MS. Treatment results demonstrated a clear beneficial effect of modafinil on EDS in patients with PD (MD -2.45 95% CI -4.00 - -0.91 p=0.002 I(2)=14%), but not with MS and TBI. No difference was seen between modafinil and placebo treatments in patients with PPS. Modafinil seemed to have no therapeutic effect on depression. Adverse events were similar between modafinil and placebo groups except that more patients were found with insomnia and nausea in modafinil group.
Effect of intravenous immunoglobulin in patients with post-polio syndrome - an uncontrolled pilot study
OBJECTIVE: To analyse changes in muscle strength, physical performance and quality of life during intravenous immunoglobulin (IVIg) treatment in patients with post-polio syndrome.

DESIGN: Open clinical trial.

PATIENTS: A total of 14 patients (6 women, 8 men; mean age 57 years, range 43-67 years) were included in the study.

INTERVENTION: Treatment with 90 g IVIg (30 g daily for 3 days).

MAIN OUTCOME: Muscle strength, measured with dynamic dynamometry, muscle function, by means of performing the 6-minute walk test, and quality of life, analysed by means of the SF-36 questionnaire, were performed before and after treatment.

RESULTS: For quality of life there was a statistically significant improvement for all but one of the 8 multi-item scales of SF-36 when comparing data before and after treatment with IVIg. The multi-item scale most improved was Vitality. There was no significant increase in muscle strength and physical performance.
Response of postpoliomyelitis patients to bisphosphonate treatment
OBJECTIVE: To evaluate (1) the rate of change of bone mineral density (BMD) at the hip in postpolio patients treated with bisphosphonates compared with the rate of change in BMD in (a) postpolio patients not treated with bisphosphonates and (b) non-postpolio patients treated with bisphosphonates; and (2) to compare the fracture rate in postpolio patients before and after treatment.

DESIGN: Retrospective chart review.

SETTING: University-affiliated hospital postpolio clinic and bone metabolism clinic.

PARTICIPANTS: Patients with at least 2 BMD assessments. We included 144 postpolio patients and 112 non-postpolio patients. For the fracture analysis, 32 postpolio patients with a history of fractures and treatment with bisphosphonates were included.

METHODS: The effect of treatment on BMD in postpolio patients was analyzed with use of a multiple linear regression model and a mixed effects model, with the rate of change in hip BMD and the change in BMD from baseline, respectively, as the dependent variables. The effect of treatment on occurrence of fractures in postpolio patients was analyzed with use of conditional logistic regression and Poisson regression.

MAIN OUTCOME MEASURES: BMD measurements at the femoral neck (g/cm²) and occurrence of fractures before and after initiation of treatment.

RESULTS: In an adjusted model, postpolio patients treated with bisphosphonates (54/144) had a greater rate of change in BMD (0.031 g/cm²/year; 95% confidence interval 0.010-0.052) compared with nontreated postpolio patients. The effect of treatment in postpolio patients was similar to that in non-postpolio patients. Evidence indicated that treated postpolio patients have a lower risk of fracture after treatment (odds ratio 0.3, P = .046; rate ratio 0.4, P = .183).
Intravenous immunoglobulin for postpolio syndrome: a systematic review and meta-analysis
BACKGROUND: Postpolio syndrome (PPS) is characterized by progressive disabilities that develop decades after prior paralytic poliomyelitis. Because chronic inflammation may be the process underlying the development of PPS, immunomodulatory management, such as intravenous immunoglobulin (IVIg) administration, may be beneficial.

METHODS: We performed a systematic review and meta-analysis of published randomized controlled trials (RCTs) and prospective studies that evaluated the efficacy of IVIg in managing PPS. Electronic databases, including PubMed, EMBASE, CINAHL, and the Cochrane Central Register of Controlled Trials, were searched for articles on PPS published before December 2014. The primary outcomes were pain severity, fatigue scores, and muscle strength. The secondary outcomes were physical performance, quality of life (QoL), and cytokine expression levels.

RESULTS: We identified 3 RCTs involving 241 patients and 5 prospective studies involving 267 patients. The meta-analysis of pain severity (weighted mean difference [WMD] = −1.02, 95% confidence interval [CI] = −2.51 to 0.47), fatigue scores (WMD = 0.28, 95% CI −0.56 to 1.12), and muscle strength revealed no significant differences between the IVIg and the placebo group. Regarding QoL, the RCTs yielded controversial outcomes, with improvement in only certain domains of the Short Form 36 (SF-36). Moreover, one prospective study reported significant improvement on SF-36, particularly in patients aged younger than 65 years, those with paresis of the lower limbs, and high pain intensity.
Immunoglobulin treatment in post-polio syndrome: Identification of responders and non-responders
OBJECTIVE: To define and characterize responders and non-responders in a group of 124 patients with post-polio syndrome who received a single treatment with intravenous immunoglobulin.

DESIGN: Open trial, prospective follow-up study.

METHODS: Clinical examination and data from medical records. Short Form 36 (SF-36), Physical Activity Scale for the Elderly (PASE) and visual analogue scale (VAS) measured quality of life, physical activity and intensity of pain, respectively. Data were obtained before treatment and at 6-month follow-up.

RESULTS: Two responder groups were identified with the outcome SF-36 Vitality and 3 with Bodily pain, respectively. Forty-five percent were positive-responders, identified before treatment by reduced physical function, muscle atrophy in the lower extremities, higher levels of fatigue and pain, and a VAS pain score above 20. Negative-responders were identified by good physical function and mental health, lesser muscle atrophy in the lower extremities, and low levels of fatigue and pain.
IVIG treatment in post-polio patients: evaluation of responders
The aim of this work is to evaluate the outcome of IVIG treatment in patients with post-polio syndrome (PPS) and to identify responders. The study included 113 PPS patients who had received one IVIG treatment in an open trial, prospective follow-up study. Clinical examination was performed and clinical data were retrieved from medical records. The short form 36 (SF-36), physical activity scale for the elderly (PASE), and the visual analogue scale (VAS) were used as measurements of quality of life, physical activity, and the intensity of pain. Data before treatment and at 6-month follow-up were collected. Analysis was performed in subgroups based on demographic and medical parameters. A statistically significant increase of the SF-36 sub domains bodily pain, vitality, social function, role emotional, and the mental compound score (MCS) was found at the 6-month follow-up. A significant decrease of pain was found in patients who reported pain intensity over VAS of 20 mm, in patients younger than 65 years of age and in patients who had paresis in the lower extremities. A trend was found in patients with PPS as the only diagnosis. IVIG leads to increase of quality of life at 6-month follow-up for SF-36 regarding sub domains of bodily pain, vitality, social function, role emotional, as well as for pain. Age below 65 years, paresis in the lower extremities, and lack of concomitant disorders may be the main indicators for a future identification of responders.
Immunoglobulin g for the treatment of chronic pain: report of an expert workshop
BACKGROUND: The treatment of chronic pain is still unsatisfactory. Despite the availability of different drugs, most patients with chronic pain do not receive satisfactory pain relief or report side effects. Converging evidence implicates involvement of the immune system in the pathogenesis of different types of nociceptive and neuropathic chronic pain.

DESIGN: At a workshop in Liverpool, UK (October 2012), experts presented evidence suggesting immunological involvement in chronic pain and recent data supporting the concept that the established immune-modulating drug, polyvalent immunoglobulin G (IgG), either given intravenously (IVIg) or subcutaneously (SCIg), may reduce pain in some peripheral neuropathies and a range of other pain disorders. Workshop's attendees discussed the practicalities of using IVIg and SCIg in these disorders, including indications, cost-effectiveness, and side effects.

RESULTS: IgG may reduce pain in a range of nociceptive and neuropathic chronic pain conditions, including diabetes mellitus, Sjögren's syndrome, fibromyalgia, complex regional pain syndrome, post-polio syndrome, and pain secondary to pathological autoantibodies.
Anticholinesterase-responsive neuromuscular junction transmission defects in post-poliomyelitis fatigue
Disabling generalized fatigue and muscle fatiguability are common features of post-poliomyelitis syndrome (PPS). In 17 fatigued PPS patients, we measured jitter on stimulation single-fiber electromyography (S-SFEMG) for at least 3.5 min before and after i.v. injection of 10 mg edrophonium. We observed reduction in jitter (defined as a significant difference in jitter means before and after edrophonium, unpaired t-test P < 0.05) in 7 patients, no change in 8, and a significant increase in 2 patients. Blinded to their edrophonium results, the 17 patients were treated with pyridostigmine 180 mg/day for 1 month, with a subjective improvement of fatigue in 9 patients, and with a significant reduction in mean Hare fatigue scores in the entire group of 17 patients (pre = 2.71, and post = 1.71; Wilcoxan signed rank sum test, P < 0.05). Edrophonium-induced reduction of jitter on S-SFEMG was significantly associated with pyridostigmine-induced subjective improvement of fatigue (Fisher's exact test, P < 0.04). A significant reduction in fatigue with pyridostigmine was observed only in the 7 patients who experienced a significant reduction in jitter with edrophonium (Wilcoxan signed rank sum test, P = 0.03). In addition, the 9 pyridostigmine responders experienced a significant reduction in jitter means pre- and post-edrophonium (100% vs. 88%, Bonferroni corrected, P < 0.01). We conclude that neuromuscular transmission as measured by jitter on S-SFEMG can improve with edrophonium in a proportion of PPS patients, and that generalized fatigue and muscle fatiguability in some patients with PPS may be due to anticholinesterase-responsive NMJ transmission defects.
Anticholinesterases in Post-Poliomyelitis Syndrome
New weakness, fatigue, and pain after decades of functional stability in those who have recovered from acute paralytic poliomyelitis constitutes post-poliomyelitis syndrome (PPS).[1-7] The cause of PPS is unknown, but it is thought to be due to a distal degeneration of enlarged post-polio motor units produced by terminal axonal sprouting during the recovery process after acute polio.[8,9] The symptoms of weakness and fatigue may be a direct result of this distal motor unit degeneration;[2,7-13] however, it is presently unclear how pain relates to disease of the motor unit. PPS is a slowly progressive motor neuron disease for which there is currently no specific treatment.[4]
An Open Trial of Pyridostigmine in Post-poliomyelitis Syndrome

Background: One of the major symptoms of postpoliomyelitis syndrome (PPS) is disabling generalized fatigue. Subjects with PPS also report muscle fatiguability and display electrophysiologic evidence of anticholinesterase-responsive neuromuscular junction transmission defects, suggesting that anticholinesterase therapy may be useful in the management of disabling fatigue. Methods: We initiated an open trial of the oral anticholinesterase pyridostigmine, up to 180 mg per day, in 27 PPS patients with generalized fatigue and muscle fatiguability. Response to Pyridostigmine was assessed with the Hare fatigue scale, the modified Barthel index for activities of daily living, and a modified Klingman mobility index. Results: Two patients could not tolerate the medication. After one month of therapy, 16 patients (64%) reported a reduction in fatigue on the Hare fatigue scale; three of 16 showed improvement on the modified Barthel index for activities of daily living, and two of 16 experienced improvement on a modified Klingman mobility index. Pyridostigmine responders were significantly more fatigued than non-responders on the pre-treatment Hare score, but were not significantly different with regard to age, sex, age at acute poliomyelitis, or severity of acute poliomyelitis. Conclusions: Pyridostigmine may be useful in the management of fatigue in selected patients with PPS. Response to pyridostigmine may be predicted by severity of pre-treatment fatigue.
Bromocriptine In The Treatment Of Post-Polio Fatigue: A pilot study with implications for the pathophysiology of fatigue
Objective: Determine the effectiveness of bromocriptine in the treatment of severe and disabling post-polio fatigue.
Design: Placebo-controlled drug trial in a pilot series of patients.
Setting: Outpatient rehabilitation hospital.
Patients: Of 83 patients without comorbidities who completed treatment with the Post-Polio Service, 5 of 8 patients who had paralytic polio and continued to report moderate to severe daily fatigue after complying with conservative treatments for post-polio fatigue agreed to be studied.
Intervention: Placebo was given for four weeks followed by increasing doses of bromocriptine mesylate (Parlodel®) administered at noon for 28 days reaching a total dose of 12.5 mg/day.
Main Outcome Measures: Daily logs of subjective fatigue and cognitive difficulties.
Results: Three of the subjects reported symptom improvement on bromocriptine but not on placebo. However, all subjects experienced nausea on bromocriptine, likely eliminating blinding. Drug responders had clinically impaired performance on neuropsychological tests of attention and information processing speed. Logged daily difficulty with attention, cognition, word finding memory, staying awake and fatigue on awakening were significantly negatively correlated with days on bromocriptine, but not with days on placebo, in drug responders.
Conclusions: A double-blind, placebo-controlled multicenter study will be needed to confirm bromocriptine's effectiveness in treating attentionally-impaired polio survivors whose severe and disabling fatigue does not responded to conservative treatment.
Polio residuals clinic: conditioning exercise program
The additional disability experienced by individuals who had poliomyelitis many years earlier has a variety of expressions and a variety of interacting origins. Undertraining and deconditioning are addressed in this article. Weakened musculature often fatigues before a conditioning level of activity is reached. An adapted exercise program for cardiac endurance will reduce symptoms of fatigue and pain. An intentional training program for muscles weakened further by disuse or underutilization will supplement the conditioning program. The clinical assessment and exercise prescription is described.
The use of strengthening exercises in post-polio sequelae - methods and results
Some individuals who had poliomyelitis 20 to 30 years ago are now reporting a recurrence of symptoms of weakness in the same muscle groups that were weakened during the initial onset of the disease. Electrophysiological findings on EMG and repetitive stimulation studies identify changes peculiar to this disease. Non-fatiguing progressive resistive exercises have been used to strengthen muscle groups demonstrating this secondary weakness after the muscles have been identified by electrophysiological studies. Favorable results are reported after non-fatiguing exercises which, combined with Occupational Therapy and appropriate orthotic management, have resulted in improvement in function of ambulation and activities of daily living. The causes of muscle atrophy and pain seen in these individuals are also discussed.
Effect of modified aerobic training on movement energetics in polio survivors
Given that individuals with disabilities may be unable to achieve maximal oxygen uptake in an exercise test and that maximal exercise testing may cause increased fatigue, pain, and muscle weakness, we examined the role of submaximal exercise testing and training based on objective as well as subjective parameters in polio survivors. Experimental (N = 7) and control subjects (N = 13) were tested before and after a 6-week period. The experimental subjects participated in a 6-week exercise training program for 30 to 40 minutes, three times a week. The program consisted of treadmill walking at 55% to 70% of age-predicted maximum heart rates; however, exercise intensity was modified to minimize discomfort/pain and fatigue. Neither objective nor subjective exercise responses were significantly different in the control group over the 6 weeks. No change was observed in cardiorespiratory conditioning in the experimental group. However, movement economy, which is related to the energy cost of walking, was significantly improved; and walking duration was significantly increased at the end of training. Modified aerobic training may have a role in enhancing endurance and reducing fatigue during activities of daily living in polio survivors.
The effects of long-term non-fatiguing resistance exercise in subjects with post-polio syndrome
Measures of torque were used to evaluate changes in muscle strength and endurance in 17 patients with post-polio syndrome who did prescribed resistance exercise for up to 2 years. Exercise compliance averaged 75%, with 16 subjects increasing the weight lifted in training. Maximum torque was significantly increased in the exercised muscle compared to the control muscle; no difference was seen in muscle endurance. Individuals with post-polio syndrome can increase muscle strength by doing non-fatiguing resistance exercise, but they should undergo quantitative testing of muscle strength a minimum of every 3 months to guard against overwork weakness.
Post-polio fatigue: a 31P magnetic resonance spectroscopy investigation
Changes in high energy phosphates (HEP) and intramuscular pH during exercise were measured in 17 patients with post-polio fatigue and in 28 healthy controls using 31P magnetic resonance spectroscopy (MRS). Subjects performed a dynamic hand grip exercise at low and high intensity. Mean changes in the HEP and pH showed no significant differences between the groups, although the post-polio group's response was highly variable. Six patients showed evidence of a lower lactate accumulation during the high intensity exercise when compared with controls. These data suggest that the whole body fatigue experienced by polio survivors is not related to any systemic metabolic abnormality.
Cardiorespiratory responses to aerobic training by patients with postpoliomyelitis sequelae
We examined the cardiorespiratory responses of 16 patients with postpoliomyelitis sequelae to a 16-week aerobic exercise program. The patients exercised at 70% of maximal heart rate. Dependent variables were resting and maximal heart rates, systolic and diastolic blood pressures, maximum oxygen consumption, maximum carbon dioxide consumption, respiratory quotient, and maximum expired volume per unit time. The exercise group was superior to the control group in watts, exercise time, maximum expired volume per unit time, and maximum oxygen consumption. No untoward events or loss of leg strength occurred as a result of the exercise regimen. We conclude that the aerobic training program employed in this study is a safe, short-term procedure and that patients with postpolio sequelae respond to training in a manner similar to healthy adults.
Gait characteristics and influence of fatigue during the 6-minute walk test in patients with post-polio syndrome
OBJECTIVE: To evaluate gait in patients with post-polio syndrome, using the 6-minute walk test (6MWT) combined with three-dimensional kinematic analysis.

DESIGN: Descriptive study.

SUBJECTS: Eighteen patients and 11 healthy controls.

METHODS: Kinematic data were obtained during a 6MWT by a Vicon motion capture system. Distance, heart rate, leg tiredness, dyspnoea and exertion were also recorded.

RESULTS: Patients with post-polio syndrome showed larger increases in leg tiredness (p < 0.001) and dyspnoea (p < 0.05) as a result of the 6MWT than did controls. Walking speed decreased by 14.1% in patients vs 4.7% in controls (p < 0.05). Fourteen out of 18 patients displayed plantar-flexed ankle at initial contact (1/11 controls). At foot-off, the patients had a flexed hip (extended in controls) and a more flexed knee. Walking speed in patients correlated with hip angle at footoff, at the start (r = –0.60, p < 0.001) and the end of the 6MWT (r = –0.74, p < 0.001), being higher the more the hip was extended.

CONCLUSION: The 6MWT is fatiguing for patients with post polio syndrome, and this was reflected in the kinematic data. Walking speed was negatively correlated with the increased hip flexion, but not with the ankle plantar-flexion at foot-off in the patients with post-polio syndrome. The three-dimensional results underscore the importance of hip function in this patient group.
Postpolio syndrome and cardiopulmonary conditioning
Postpolio syndrome is a group of related signs and symptoms occurring in people who had paralytic poliomyelitis years earlier. New weakness, fatigue, poor endurance, pain, reduced mobility, increased breathing difficulty, intolerance to cold, and sleep disturbance in various degrees and expressions make up the syndrome. The reported incidence is between 25% and 80%. The origins are multifactorial and can be associated with underexertion, overexertion, inactivity due to intercurrent illness or injury, hypo-oxygenation, sleep apnea, deconditioning, and the failure of sprouted, compensatory large motor units. The exercise question in postpolio syndrome is related to the experience of new weakness or loss of muscle function due to overuse, which is often associated with injudicious repeated challenges to weakened musculature. Carefully prescribed exercise can be used for increasing strength and endurance and improving cardiopulmonary conditioning.
Dynamic water exercise in individuals with late poliomyelitis
OBJECTIVE: To evaluate the specific effects of general dynamic water exercise in individuals with late effects of poliomyelitis.

DESIGN: Before-after tests.

SETTING: A university hospital department.

PARTICIPANTS: Twenty-eight individuals with late effects of polio, 15 assigned to the training group (TG) and 13 to the control group (CG).

INTERVENTION: The TG completed a 40-minute general fitness training session in warm water twice weekly. Assessment instruments included the bicycle ergometer test, isokinetic muscle strength, a 30-meter walk indoors, Berg balance scale, a pain drawing, a visual analog scale, the Physical Activity Scale for the Elderly, and the Nottingham Health Profile (NHP).

MAIN OUTCOME MEASURES: Peak load, peak work load, peak oxygen uptake, peak heart rate (HR), muscle function in knee extensors and flexors, and pain dimension of the NHP.

RESULTS: The average training period was 5 months; compliance was 75% (range, 55-98). No negative effects were seen. The exercise did not influence the peak work load, peak oxygen uptake, or muscle function in knee extensors compared with the controls. However, a decreased HR at the same individual work load was seen, as well as a significantly lower distress in the dimension pain of the NHP. Qualitative aspects such as increased well-being, pain relief, and increased physical fitness were reported.
Determining the anaerobic threshold in postpolio syndrome: comparison with current guidelines for training intensity prescription
OBJECTIVES: To determine whether the anaerobic threshold (AT) can be identified in individuals with postpolio syndrome (PPS) using submaximal incremental exercise testing, and to compare current guidelines for intensity prescription in PPS with the AT.

DESIGN: Cohort study.

SETTING: Research laboratory.

PARTICIPANTS: Individuals with PPS (N=82).

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Power output, gas exchange variables, heart rate, and rating of perceived exertion (RPE) were measured in an incremental submaximal cycle ergometry test. Two independent observers identified the AT. Comparison of current guidelines for training intensity prescription in PPS (40%-60% heart rate reserve [HRR] or RPE of 12) with the AT was based on correlations between recommended heart rate and the heart rate at the AT. In addition, we determined the proportion of individuals that would have been recommended to train at an intensity corresponding to their AT.

RESULTS: The AT was identified in 63 (77%) of the participants. Pearson correlation coefficients between the recommended heart rate and the heart rate at the AT were lower in cases of 40% HRR (r=.56) and 60% HRR (r=.50) than in cases of prescription based on the RPE (r=.86). Based on the RPE, 55% of the individuals would have been recommended to train at an intensity corresponding to their AT. This proportion was higher compared with 40% HRR (41%) or 60% HRR (18%) as criterion.
Submaximal exercise capacity and maximal power output in polio subjects
OBJECTIVES: To compare the submaximal exercise capacity of polio subjects with postpoliomyelitis syndrome (PPS) and without (non-PPS) with that of healthy control subjects, to investigate the relationship of this capacity with maximal short-term power and quadriceps strength, and to evaluate movement economy.

DESIGN: Cross-sectional survey.

SETTING: University hospital.

PARTICIPANTS: Forty-three polio subjects (25 PPS, 18 non-PPS) and 12 control subjects.

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Power output, oxygen uptake, and heart rate were measured in an incremental submaximal cycle ergometry test. Maximal short-term power was measured in 5-second all-out efforts. Knee extensor strength was measured on a chair dynamometer.

RESULTS: The mean submaximal power +/- standard deviation at 80% of heart rate reserve of 83.8 +/- 29.9 watts in the polio subjects was significantly less than the mean submaximal power of 142.1 +/- 30.4 watts in the control group. However, expressed as a percentage of the maximal short-term power, submaximal power did not differ between the groups. Strength and maximal short-term power correlated significantly (p < .005) with submaximal power (r = .64 and .76, respectively). The oxygen uptake was higher than theoretically expected for the given submaximal power output in polio subjects, and appeared to increase with increasing asymmetry in strength and power between legs. No differences were found between PPS and non-PPS subjects.
Previous Acute Polio and Post-Polio Syndrome: Recognizing the Pathophysiology for the Establishment of Rehabilitation Programs
Previous acute poliomyelitis (PAP) can be defined as an endemic human disease caused by an enterovirus of worldwide distribution, which compromises the anterior horn cells of the spinal cord. Poliovirus has infected and victimized thousands of people all over the world. Only after the development of the inactivated virus vaccine by Jonas Salk, in 1955, and then with the attenuated virus vaccine, by Albert Bruce Sabin, in 1961, we saw a reduction in the number of poliomyelitis cases in the world.

The patients present clinical status characterized by muscle atrophy and paresis, especially in the lower limbs, under asymmetrical and disproportional form. There is a second form, bulbar, which compromises the motor neurons of the medulla, resulting in impairments in speech, swallowing and breathing. The purpose of this letter to the Editor is to alert readers about the risks of therapeutic exercise for this group of patients.
Endurance Training Effect on Individuals With Postpoliomyelitis
Objective: To determine the effects of an endurance training program on the exercise capacity and muscle structure and function in individuals with postpolio syndrome.

Design: Preexercise and postexercise testing was performed with muscle strength evaluations using isokinetic testing as well as hand-held Myometer. Muscle fatigue was determined by use of isokinetic testing, and endurance was determined by exercise testing. Enzymatic evaluation was performed with muscle biopsies taken at the same site; preexercise and postexercise muscle cross-sectional area was measured by computed tomography. Disability and psychosocial evaluation was performed by a Functional Status Questionnaire.

Setting: A university.

Subjects: Seventeen postpolio subjects ranging in age from 39 to 49 years volunteered for a 6-month combined endurance and strength training program. They had a history of acute poliomyelitis at least 25 years earlier and were able to walk with or without aid.

Intervention: Twelve of the subjects (mean age 42 years) completed the program, attending an average of 29 sessions, which were offered for 60 minutes twice a week.

Main Outcome Measures: Strength, endurance, enzymatic activity, and cross-sectional area were measured 3 months before the beginning of training, just before training, and at the completion of the exercise program.
The Postpolio Syndrome: An Overuse Phenomenon

Persons with good recovery of function following their initial poliomyelitis are now, more than 30 years later, experiencing new weakness, fatigue, and muscle pain. The likelihood of muscle overuse being the cause of this late functional loss was investigated by dynamic electromyography (EMG) and foot-switch stride analysis in 34 symptomatic patients. Manual testing grouped the muscles, with strong (S) encompassing Grades Good (G) and Normal (N) while weak (W) included Fair plus (F+) to zero (0). After testing quadriceps and calf strength, the patients fell into one of four classes: strong quadriceps and calf (SQ/SC) strong quadriceps and weak calf (SQ/WC) weak quadriceps and strong calf (WQ/SC) or combined weak quadriceps and calf (WQ/WC). Quantified EMG; (normalized by the manual muscle test EMG) defined the mean duration and intensity of the quadriceps soleus, lower gluteus maximus, and long head of the biceps femoris during walking. Overuse was defined as values greater than the laboratory normal (mean·+ 1 SD). Each muscle exhibited instances of overuse, normalcy, and sparing. The biceps femoris was the only muscle with dominant overuse (82%). Quadriceps overuse was next in frequency (53%). Soleus activity infrequently exceeded normal function (34%), but this still represented more than twice the intensity and duration of the other muscles. Gluteus maximus action was also seldom excessive (34%). The patients averaged two muscles with excessive use during walking. Gait velocity of the SQ/SC strong group was highest (71% of normal) while the three categories that included weak muscles had walking speeds in the range of 50% of normal. The finding of muscle overuse during a single free-speed walking test that does not attain normal velocity supports the concept of muscle overuse being the cause of the patient's dysfunction.
Comparison of two 6-minute walk tests to assess walking capacity in polio survivors
Objective: To compare walking dynamics and test-retest reliability for 2 frequently applied walk tests in polio survivors: the 6-minute walk test (6MWT) to walk as far as possible; and the 6-minute walking energy cost test (WECT) at comfortable speed.

Design: Observational study.

Participants: Thirty-three polio survivors, able to walk ≥ 150 m.

Methods: On the same day participants performed a 6MWT and a WECT, which were repeated 1–3 weeks later. For each test, distance walked, heart rate and reduction in speed were assessed.

Results: The mean distance walked and mean heart rate were significantly higher in the 6MWT (441 m (standard deviation) (SD 79.7); 118 bpm (SD 19.2)) compared with the WECT (366 m (SD 67.3); 103 bpm (SD 14.3)); p < 0.001. Furthermore, during the 6MWT, patients continuously slowed down (–6%), while during the WECT speed dropped only slightly during the first 2 min, by –1.8% in total. Test-retest reliability of both tests was excellent (intraclass correlation coefficient (ICC) ≥ 0.95; lower bound 95% confidence interval (95% CI) ≥ 0.87). The smallest detectable change for the walked distance was 42 m (9.7% change from the mean) and 50 m (13.7%) on the 6MWT and WECT, respectively.

Conclusion: Both the 6MWT and the WECT are reliable to assess walking capacity in polio survivors, with slightly superior sensitivity to detect change for the 6MWT. Differences in walking dynamics confirm that the tests cannot be used interchangeably. The 6MWT is recommended for measuring maximal walking capacity and the WECT for measuring submaximal walking capacity.
Cardiorespiratory responses to upper extremity aerobic training by postpolio subjects
The cardiorespiratory responses of ten postpolio subjects participating in a 16-week upper extremity aerobic exercise program were compared to ten non-exercised controls. The subjects trained three times a week for 20 minutes per session. Exercise intensity was prescribed at 70% to 75% of heart rate reserve plus resting heart rate. Dependent variables were resting heart rate, maximal heart rate, resting and immediate-post-exercise systolic and diastolic blood pressures, maximal oxygen consumption, maximal carbon dioxide production, minute ventilation, respiratory exchange ratio, power, and exercise time. After training, the exercise group was superior to the control group in oxygen consumption, carbon dioxide production, minute ventilation, power, and exercise time. There was no reported loss of muscle strength. It was concluded that postpolio subjects can safely achieve an increase in aerobic capacity with a properly modified upper extremity exercise program. This improvement is comparable to that demonstrated by able-bodied adults.
Effect of aquatic exercise training in persons with poliomyelitis disability
Aquatic exercise, including swimming, reduces the effect of body weight on limbs and joints. A combination of swimming and specific activities involving resistive devices was used in an attempt to improve strength in persons who had symptomatic weakness related to poliomyelitis. Dynamic muscular force application in selected limb movements and range of motion were measured before and after an 8‐week aquatic exercise intervention. Peak (PF) and average force (AF) were determined in the water using a differential pressure transducer attached to either the hand, foot, or a resistive device. Arm flexion, extension, adduction, abduction, and horizontal adduction and abduction along with combined hip flexion and knee extension were tested for both PF and AF Subjects were randomly assigned to experimental and control groups; complete data were available on nine experimental and four control subjects. PF and AF changes were greater (p ≤ 0.05) for experimental compared with control for right arm flexion (PF, 96 versus 6%) and extension (PR 105 versus ‐15%; AF, 76 versus ‐30%), respectively. Changes were greater (p ≤0.05) in experimental than control for left arm extension (PF, 88% versus 19%) and horizontal abduction (PF, 127% versus ‐21%; AF, 122% versus ‐17%). Aquatic exercise training in subjects with poliomyelitis disability resulted in significant dynamic strength changes of the upper body while appearing not to exacerbate symptomatic fatigue or pain.
Falls and Bone Density
Fractures in an aging population of poliomyelitis survivors: a community-based study in Olmsted County, Minnesota
OBJECTIVE: To determine the incidence of fractures in a cohort of survivors of paralytic poliomyelitis (“polio”).

DESIGN: We conducted a population-based retrospective cohort study of residents of Olmsted County, Minnesota, who had an initial diagnosis of polio between 1935 and 1959 and survived the acute illness.

MATERIALS AND METHODS: A comprehensive medical records-linkage system was used to retrieve all diagnoses of poliomyelitis in residents of the county for the specified 25-year period. For the 277 study subjects, the medical records were searched for the occurrence of fracture from the time of initial diagnosis of polio until death or the most recent clinical contact. All fractures were classified on the basis of type of associated injury and skeletal site of involvement. The influence of polio on the incidence of fractures was evaluated by estimating the cumulative incidence of new fractures after the diagnosis of polio and the standardized morbidity ratio. The relative influence of various factors on the risk of fracture was determined.

RESULTS: Of the 277 Olmsted County residents with polio, 87 experienced 161 fractures, and the estimated cumulative incidence of any fracture after 40 years was 48%. The cumulative incidence of any limb fracture was 41% and was less than the expected value of 44% (P = 0.001). Only the risk of distal femoral and proximal humeral fractures was significantly higher than that among Olmsted County residents in general. The increased risk seemed to be associated with weakness and disuse of the involved limbs rather than with generalized osteoporosis. Theoretically, a greater capacity for bone remodeling in response to changes in muscle use might have protected patients with polio in childhood, but such persons were as likely to sustain a fracture as those with adult-onset poliomyelitis.
Falls and Bone Density
High incidence of osteoporosis and fractures in an aging post-polio population
BACKGROUND/AIMS: Since the polio epidemic in Ireland in the 1950s, most polio survivors are approaching into the 6th and 7th decade of their lives. There is little data about bone density and risk of fractures in these patients. In 2006, we undertook an audit of post-polio patients attending rheumatology and neurology outpatient clinics in a university teaching hospital. Our aim was to determine the prevalence of osteoporosis (OP), falls and fractures and to evaluate the association of bone density with other potential contributing factors to OP.

METHODS: Over a 6-month period, 50 post-polio patients attending outpatient clinics completed a questionnaire, and subsequently their medical records were reviewed. Demographic data and details of treatment were extracted. The patients underwent a dual-energy X-ray absorptiometry scanning to quantify bone mineral density. Results: Thirty subjects (60%) were females (26 were postmenopausal). The average age of females was 60 ± 13.4 years and of men 59 ± 16.8 years. Overall, 41 (82%) of the patients had experienced falls in the last 5 years and 32 (64%) in the last 6 months. Nineteen (38%) of the patients had experienced a bone fracture in the last 5 years. Based on the bone mineral density data, 28 (56%) of the patients were diagnosed with OP and 20 (40%) had osteopenia, but only 8 (16%) received anti-resorptive therapy. Of the 19 patients who had a fracture, 14 (74%) had OP and 5 (26%) had osteopenia, of whom only 6 (32%) received anti-resorptive therapy. Eight out of 9 fractures of the neck of femur occurred in the weaker leg.
Falls and Bone Density
Circumstances and consequences of falls in polio survivors
OBJECTIVES: Many polio survivors have symptoms that are known risk factors for falls in elderly people. This study aims to determine the: (i) frequency; (ii) consequences; (iii) circumstances; and (iv) factors associated with falls in polio survivors.

METHODS: A survey was conducted among 376 polio survivors. Participants completed a falls history questionnaire and additional information was obtained from their medical files.

RESULTS: Of the 305 respondents, 74% reported at least one fall in the past year and 60% two or more. Sixteen percent of fallers described a major injury after a fall in the last year and 69% reported fear of falling. One-third of fallers had reduced the amount they walked because of their fear of falling. Most reported falls in a familiar environment (86%), during ambulation (72%) and in the afternoon (50%). Quadriceps weakness of the weakest leg (Medical Research Council (MRC) ≤ 3), fear of falling and complaints of problems maintaining balance were independently associated with both falls and recurrent falls, while increasing age and medication use were not.
Falls and Bone Density
Polio survivors: falls and subsequent injuries
OBJECTIVE: This study examines the frequency of falls in polio survivors and their resulting morbidity.

DESIGN: Two groups, fallers vs. nonfallers, were investigated in this descriptive study. A total of 233 polio survivors volunteered to complete a structured questionnaire on fall history and sequelae.

RESULTS: Of the study participants, 64% had fallen within the previous year, and 61% had falls for which they received medical attention, including 35% who had at least one fracture. There was not a correlation between age and falling, but there was a strong correlation between tripping and falling.
Falls and Bone Density
Falls among adults aging with disability
OBJECTIVE: To investigate the prevalence of and risk factors for falling among individuals aging with multiple sclerosis (MS), muscular dystrophy (MD), postpolio syndrome (PPS), and spinal cord injury (SCI).

DESIGN: Cross-sectional survey data from 2009 to 2010 were analyzed. We used forward logistic regression models to examine whether risk factors such as age, sex, mobility level, years since diagnosis, vision, balance, weakness, number of comorbid conditions, and physical activity could distinguish participants who reported falling from those who did not.

SETTING: Surveys were mailed to community-dwelling individuals who had 1 of 4 diagnoses (MS, MD, PPS, or SCI). The survey response rate was 91%.

PARTICIPANTS: A convenience sample of community-dwelling individuals (N=1862; age, 18-94y) with MS, MD, PPS, or SCI in the United States.

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURE: Self-reported fall within the last 6 months.

RESULTS: Fall prevalence for people with MS (54%), MD (70%), PPS (55%), and SCI (40%). Across all 4 groups, fall rates peaked in middle age (45-64y) and among people with moderate mobility limitations. Seven risk factors differentiated participants who fell from those who did not: mobility level, imbalance, age, curvilinear age (age(2)), number of comorbid conditions, duration of diagnosis, and sex. The models differed across diagnostic groups.
Falls and Bone Density
Osteoporosis in a postpolio clinic population
OBJECTIVES: To determine (1) the frequency of osteoporosis at the hip and lumbar spine in a postpolio clinic population and (2) the association of lower-extremity muscle strength and other potential contributing factors to osteoporosis with bone density measured at the hip.

DESIGN: Cross-sectional study involving a chart review.

SETTING: A university-affiliated hospital postpolio clinic.

PARTICIPANTS: Patient charts (N=379) were reviewed; 164 (26%) were included, and 215 (74%) were not included primarily (74%) because of the unavailability of bone density results.

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Bone density (in g/cm(2)) and T score were assessed at the femoral neck and lumbar spine. Muscle strength was evaluated by manual muscle testing in 7 bilateral lower-extremity muscles.

RESULTS: The occurrence of osteoporosis at the hip and lumbar spine was 20 (32%) of 62 and 6 (10%) of 61 in men, 3 (9%) of 33 and 2 (6%) of 32 in premenopausal women, and 18 (27%) of 67 and 7 (11%) of 65 in postmenopausal women, respectively. In a logistic regression model, the presence of osteoporosis at the hip was significantly associated with strength sum score in the same extremity in which the bone density was performed after adjusting for other important risk factors (age, body mass index, time since polio).
Activity and post-polio fatigue
Fatigue was studied in 12 subjects with post-polio sequelae (PPS). Results of the Fatigue Severity Scale (FSS) demonstrated a mean score of 4.8 +/- 1.6 (non-disabled scores = 2.3 +/- 0.7). The Human Activity Profile (HAP) was not sensitive enough to measure fatigue. Fifty percent of subjects scored below the first percentile based on age and sex matched norms. The Activity Record (ACTRE) results revealed that subjects spent 5% of their time resting and 1% in planning or preparation activities. Fatigue peaked in the late morning or early afternoon and was relieved by rest periods. Use of energy conservation and work simplification skills along with frequent rest periods was suggested as a possible method for managing PPS fatigue.
Cognitive functioning in post‐polio patients with and without general fatigue
OBJECTIVE AND DESIGN: This study examined 2 main hypotheses. First, whether patients with post-polio suffering from general fatigue (n=10) demonstrate cognitive deficits compared with patients with post-polio without general fatigue (n=10). Secondly, by systematically varied test order administration we examined whether such differences varied as a function of increasing cognitive load during cognitive testing.

SUBJECTS: Twenty patients diagnosed with post-polio syndrome, 10 with general fatigue and 10 without fatigue.

RESULTS: Neither of the 2 hypotheses were confirmed. The group with general fatigue reported elevated levels of depression. However, no systematic association between level of depression and cognitive performance could be detected.
Multidimensional fatigue inventory and post-polio syndrome - a Rasch analysis
BACKGROUND: Fatigue is a common symptom in post-polio syndrome (PPS) and can have a substantial impact on patients. There is a need for validated questionnaires to assess fatigue in PPS for use in clinical practice and research. The aim with this study was to assess the validity and reliability of the Swedish version of Multidimensional Fatigue Inventory (MFI-20) in patients with PPS using the Rasch model.

METHODS: A total of 231 patients diagnosed with PPS completed the Swedish MFI-20 questionnaire at post-polio out-patient clinics in Sweden. The mean age of participants was 62 years and 61% were females. Data were tested against assumptions of the Rasch measurement model (i.e. unidimensionality of the scale, good item fit, independency of items and absence of differential item functioning). Reliability was tested with the person separation index (PSI). A transformation of the ordinal total scale scores into an interval scale for use in parametric analysis was performed. Dummy cases with minimum and maximum scoring were used for the transformation table to achieve interval scores between 20 and 100, which are comprehensive limits for the MFI-20 scale.

RESULTS: An initial Rasch analysis of the full scale with 20 items showed misfit to the Rasch model (p < 0.001). Seven items showed slightly disordered thresholds and person estimates were not significantly improved by rescoring items. Analysis of MFI-20 scale with the 5 MFI-20 subscales as testlets showed good fit with a non-significant x (2) value (p = 0.089). PSI for the testlet solution was 0.86. Local dependency was present in all subscales and fit to the Rasch model was solved with testlets within each subscale. PSI ranged from 0.52 to 0.82 in the subscales.
Circadian fatigue or unrecognized restless legs syndrome? The post-polio syndrome model
This paper does not have an abstract.
Vitality among Swedish patients with post-polio: a physiological phenomenon
OBJECTIVE: To evaluate vitality and fatigue in post-polio patients, and the relative contributions of physiological and psychological parameters to the level of vitality.

DESIGN: Multi-centre study.

SUBJECTS: One hundred and forty-three patients with post-polio syndrome.

METHODS: Inventories of background, quality of life, fatigue and sleep quality were used. Pain was evaluated using a visual analogue scale. Descriptive statistics and correlations were used for all selected parameters. Hierarchical regression models were constructed to examine predictors of variations in vitality, pain, reduced activity and physical fatigue.

RESULTS: General fatigue accounted for 68% of the variation in vitality. Of this, 91% was accounted for by physiological indicators. After controlling for age, physiological para notmeters accounted for 56.6% and 25%, if entered before and after the psychological parameters, respectively. The impact of the psychological parameters decreased after accounting for the physiological parameters. Physical fatigue, age and sleep quality were associated with variation in pain. Body mass index, pain and sleep quality accounted for differences in reduced activity and physical fatigue.
Measuring fatigue in polio survivors: content comparison and reliability of the Fatigue Severity Scale and the Checklist Individual Strength
OBJECTIVES: To compare the content of the Fatigue Severity Scale and the subscale "subjective experience of fatigue" of the Checklist Individual Strength, and to assess the reliability of both questionnaires in polio survivors.

DESIGN: Repeated-measures at a 3-week interval.

SUBJECTS: Consecutive series of 61 polio survivors.

METHODS: Concepts contained in the questionnaire items were linked to the International Classification of Functioning, Disability and Health (ICF), using standardized linking rules. Reliability analyses included tests of internal consistency, test-retest reliability and measurement error.

RESULTS: Questionnaires differed in the extent to which they measured other than fatigue-related aspects of functioning (represented ICF components: "Body functions": 50% and 80%, "Activities and Participation": 30% and 0%, for the Fatigue Severity Scale and Checklist Individual Strength, respectively). Internal consistency and test-retest reliability were considered acceptable, while measurement error was large (Cronbach's α: 0.90 and 0.93, intraclass correlation coefficient: 0.80 and 0.85, smallest detectable change: 28.7% and 29.4% for the Fatigue Severity Scale and Checklist Individual Strength, respectively).
Brain volume and fatigue in patients with postpoliomyelitis syndrome
BACKGROUND: Acute paralytic poliomyelitis is associated with encephalitis. Early brain inflammation may produce permanent neuronal injury with brain atrophy, which may result in symptoms such as fatigue. Brain volume has not been assessed in postpoliomyelitis syndrome (PPS).

OBJECTIVE: To determine whether brain volume is decreased compared with that in normal controls, and whether brain volume is associated with fatigue in patients with PPS.

DESIGN: A cross-sectional study.

SETTING: Tertiary university-affiliated hospital postpolio and multiple sclerosis (MS) clinics.

PARTICIPANTS: Forty-nine ambulatory patients with PPS, 28 normal controls, and 53 ambulatory patients with MS.

METHODS: We studied the brains of all study subjects with magnetic resonance imaging by using a 1.5 T Siemens Sonata machine. The subjects completed the Fatigue Severity Scale. Multivariable linear regression models were computed to evaluate the contribution of PPS and MS compared with controls to explain brain volume.

MAIN OUTCOME MEASUREMENTS: Normalized brain volume (NBV) was assessed with the automated program Structured Image Evaluation, using Normalization, of Atrophy method from the acquired magnetic resonance images. This method may miss brainstem atrophy.

RESULTS: Technically adequate NBV measurements were available for 42 patients with PPS, 27 controls, and 49 patients with MS. The mean (standard deviation) age was 60.9 ± 7.6 years for patients with PPS, 47.0 ± 14.6 years for controls, and 46.2 ± 9.4 years for patients with MS. In a multivariable model adjusted for age and gender, NBV was not significantly different in patients with PPS compared with that in controls (P = .28). As expected, when using a similar model for patients with MS, NBV was significantly decreased compared with that in controls (P = .006). There was no significant association between NBV and fatigue in subjects with PPS (Spearman ρ = 0.23; P = .19).
Psychometric properties of fatigue severity and fatigue impact scales in postpolio patients
We evaluate the reliability, validity, and responsiveness of the Fatigue Severity Scale (FSS) and the Fatigue Impact Scale (FIS) and to determine whether these scales are potentially applicable for measuring fatigue in postpolio patients (PPS). After the Turkish adaptation of FSS and FIS using a forward-backward procedure, the scales were administered to 48 PPS patients without additional health problems that may induce fatigue. Reliability studies were carried out by determination of intraclass correlation coefficient and internal consistency by the Cronbach-α coefficient. Validity was tested by within-scale analyses and analyses against the external criteria including convergent validity and discriminant validity. Correlations with the Notthingham Health Profile (NHP), fatigue, pain and cramp severity (visual analog scale), and manual muscle testing were performed. Sensitivity to changes was determined by standardized response mean values. All patients completed scales, suggesting their satisfactory acceptance. Reliability studies were satisfactory, with higher Cronbach-α values and intraclass correlation coefficients than 0.80. The FSS score was correlated moderately with visual analog scale-fatigue (r=0.41) and the NHP-energy dimension (r=0.29). All FIS scores except cognitive scores were moderately related to the NHP-social isolation score (r=0.40, 0.37, and 0.43 for FIS-physical, social, and total scores, respectively). There was also a significant correlation between the FIS-physical score and the NHP-energy score (r=0.31). On the basis of the standardized response mean values, response to treatment for these two questionnaires was satisfactory (P=0.00). The Turkish versions of FSS and FIS were reliable, sensitive to clinical changes, and also well accepted by patients with PPS. Although they had somewhat satisfactory convergent validity, the absence of strong correlations did not support the validity entirely.
Fatigue in post-poliomyelitis syndrome: association with disease-related, behavioral, and psychosocial factors
OBJECTIVE: To determine the biopsychosocial correlates of general, physical, and mental fatigue in patients with postpoliomyelitis syndrome (PPS) by assessing the additional contribution of potentially modifiable factors after accounting for important nonmodifiable disease-related factors. It was hypothesized that disease-related, behavioral, and psychosocial factors would contribute in different ways to general, physical, and mental fatigue in PPS and that a portion of fatigue would be determined by potentially modifiable factors.

DESIGN: Cross-sectional study.

SETTING: A tertiary university-affiliated hospital post-polio clinic.

PATIENTS: Fifty-two ambulatory patients with PPS who were not severely depressed were included.

ASSESSMENT OF RISK FACTORS: Potential correlates for fatigue included disease-related factors (acute polio weakness, time since acute polio, PPS duration, muscle strength, pain, forced vital capacity, maximum inspiratory pressure, maximum expiratory pressure, body mass index, disability, fibromyalgia), behavioral factors (physical activity, sleep quality), and psychosocial factors (depression, stress, self-efficacy).

MAIN OUTCOME MEASUREMENTS: Fatigue was assessed with the Multidimensional Fatigue Inventory (MFI; assesses fatigue on 5 subscales) and the Fatigue Severity Scale (FSS).

RESULTS: Multivariate models were computed for MFI General, Physical, and Mental Fatigue. Age-adjusted multivariate models with nonmodifiable factors included the following predictors of (1) MFI General Fatigue: maximum inspiratory pressure, fibromyalgia, muscle strength; (2) MFI Physical Fatigue: maximum expiratory pressure, muscle strength, age, time since acute polio; and (3) MFI Mental Fatigue: none. The following potentially modifiable predictors made an additional contribution to the models: (1) MFI General Fatigue: stress, depression; (2) MFI Physical Fatigue: physical activity, pain; and (3) MFI Mental Fatigue: stress.
Disability and functional assessment in former polio patients with and without postpolio syndrome
OBJECTIVES: To compare perceived health problems and disability in former polio subjects with postpolio syndrome (PPS) and those without postpolio syndrome (non-PPS), and to evaluate perceived health problems, disability, physical performance, and muscle strength.

DESIGN: Cross-sectional survey; partially blinded data collection.

SUBJECTS: One hundred three former polio subjects, aged 32 to 60yrs. This volunteer sample came from referrals and patient contacts. Criterion for PPS: new muscle weakness among symptoms.

MAIN OUTCOME MEASURES: Nottingham Health Profile (NHP), adapted D-code of the International Classification of Impairments, Disabilities and Handicaps, performance test, and muscle strength assessment.

RESULTS: PPS subjects (n = 76) showed higher scores (p < .001) than non-PPS subjects (n = 27) within the NHP categories of physical mobility, energy, and pain. On a 16-item Polio Problems List, 78% of PPS subjects selected fatigue as their major problem, followed by walking outdoors (46%) and climbing stairs (41%). The disabilities of PPS subjects were mainly seen in physical and social functioning. No differences in manually tested strength were found between patient groups. PPS subjects needed significantly more time for the performance test than non-PPS subjects and their perceived exertion was higher. Perceived health problems (NHP-PhysMobility) correlated significantly with physical disability (r = .66), performance-time (r = .54), and muscle strength (r = .38). With linear regression analysis, 54% of the NHP-PhysMobility score could be explained by the performance test (time and exertion), presence of PPS, and muscle strength, whereas strength itself explained only 14% of the NHP-PhysMobility score.
A 5-year longitudinal study of fatigue in patients with late-onset sequelae of poliomyelitis
OBJECTIVES: To study the severity and 5-year course of fatigue in patients with late-onset sequelae of poliomyelitis (LOSP) and to identify physical and psychosocial determinants of fatigue.

DESIGN: Prospective cohort study with 5 measurements over 5 years.

SETTING: University hospital.

PARTICIPANTS: Patients with LOSP (N=168); 89% of the subjects completed the study.

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Fatigue assessed with the Fatigue Severity Scale (FSS). Potential determinants were perceived physical functioning, bodily pain and mental health, extent of paresis, walking capacity, comorbidity, sleeping disorders, coping, and social support. Associations were investigated by multivariable longitudinal analysis using generalized estimating equations.

RESULTS: The mean FSS score ± SD at baseline was 5.1±1.4, which did not change significantly during the 5-year follow-up. Reduced physical functioning, increased bodily pain, reduced sleep quality, more psychologic distress, and higher task-oriented coping were independently associated with fatigue. The extent of paresis and walking capacity were strongly associated with physical functioning.
Post polio syndrome: fatigued patients a specific subgroup?
OBJECTIVES: To examine the characteristics of fatigued and non-fatigued post-polio patients and to define potential subgroups across the fatigue continuum.

DESIGN: Multi-centre study.

SUBJECTS: A total of 143 post-polio patients were subdivided on the basis of percentile distribution into a fatigue group, a intermediate group, and a non-fatigue group, using the Multi Fatigue Inventory 20 general fatigue ratings.

METHODS: Data on background, quality of life, fatigue and pain were collected. Descriptive statistics and correlations in each group and analysis of variance and χ2 for group comparisons were performed. Non-linear regressions were employed to evaluate differences in the strength of associations between physical and mental fatigue, on the one hand, and vitality on the other.

RESULTS: The fatigued group was younger, had shorter polio duration, more pain, higher body mass index, lower quality of life and was more physically and mentally fatigued. A higher proportion of this group had contracted polio after 1956 and was under 65 years of age. Mental fatigue had a relatively higher explanatory value than physical fatigue for differences in vitality in the fatigued group, whereas reversed patterns were seen in the other groups.
A comparison of 4 questionnaires to measure fatigue in postpoliomyelitis syndrome
OBJECTIVE: To assess the comparability and reproducibility of 4 questionnaires used to measure fatigue in postpoliomyelitis syndrome (PPS).

DESIGN: Repeated-measures at a 3-week interval.

SETTING: University hospital.

PARTICIPANTS: Convenience sample of 65 patients with PPS.

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: The Fatigue Severity Scale (FSS), the Nottingham Health Profile (NHP) energy category, the Polio Problem List (PPL) fatigue item, and the Dutch Short Fatigue Questionnaire (SFQ).

RESULTS: Correlations of scores between questionnaires were all significant (P<.01) and ranged from .43 (between the NHP energy category and the PPL fatigue item) to .68 (between the PPL fatigue item and the SFQ). Scores on the second visit, normalized to a 0 to 100 scale, were: FSS, 78+/-15; NHP energy category, 47+/-35; PPL fatigue item, 81+/-17; and SFQ, 65+/-22. Except for the difference between the FSS and the PPL fatigue item, the differences in scores between the questionnaires were significant (P<.01). Scale analysis indicated that all questionnaires measured the same unidimensional construct. The reproducibility of the FSS, the PPL fatigue item, and the SFQ was moderate. The smallest detectable change was 1.5 points for the FSS, 2.0 points for the PPL fatigue item, and 1.9 points for the SFQ.
The Neuropsychology of Post-Polio Fatigue
To test the hypothesis that post-polio fatigue and its concomitant cognitive deficits are associated with an impairment of attention and not of higher-level cognitive processes, six carefully screened polio survivors were administered a battery of neuropsychological tests. Only subjects reporting severe fatigue, and not those with mild fatigue, demonstrated clinically significant deficits on all tests of attention, concentration and information processing speed while showing no impairments of cognitive ability or verbal memory. These findings suggest that an impaired ability to maintain attention and rapidly process complex information appears to be a characteristic in polio survivors reporting severe fatigue, since these deficits were documented even when their subjective rating of fatigue was low. This finding supports the hypothesis that a polio-related impairment of selective attention underlies polio survivors' subjective experience of fatigue and cognitive problems.
The Pathophysiology Of Post-Polio Fatigue: A Role for the Basal Ganglia in the Generation of Fatigue
Fatigue is the most commonly reported, most debilitating and least studied Post-Polio Sequelae (PPS) affecting the more than 1.63 million American polio survivors. Post-polio fatigue is characterized by subjective reports of problems with attention, cognition and maintaining wakefulness, symptoms reminiscent of nearly two dozen outbreaks during this century of post-viral fatigue syndromes that are related clinically, historically or anatomically to poliovirus infections. These relationships, and recent studies that associate post-polio fatigue with clinically significant deficits on neuropsychologic tests of attention, histopathologic and neuroradiologic evidence of brain lesions and impaired activation of the hypothalamic-pituitary-adrenal axis, will be reviewed to described a role for the reticular activating system and basal ganglia in the pathophysiology of post-polio fatigue. The possibility of pharmacologic therapy for PPS is also discussed.
Fainting and Fatigue: Causation or Coincidence?
As the former autonomic nervous system fellow at New York's Columbia-Presbyterian Medical Center, and in my current incarnation studying chronic fatigue in polio survivors, I have read with special interest the reports from Johns Hopkins University describing neurally mediated hypotension (NMH) in adults and adolescents with CFIDS.[1] In June 1995, we presented a paper to the American Congress of Rehabilitation Medicine describing several of our post-polio patients who have had episodes of vasovagal syncope.[2] One patient with a 10-year history of severe, chronic and disabling post-polio fatigue had a history of frequent fainting 35 years before she ever experienced fatigue.
Polioencephalitis, Stress And The Etiology Of Post-Polio Sequelae
Post-mortum neurohistopathology from 158 individuals who contracted polio before 1950 are reviewed that document polio virus-induced lesions in reticular formation, hypothalamic, thalamic, peptidergic and monoaminergic neurons in the brain. 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 hypotheses that polioencephalitic damage 1) to aging reticular activating system and monoaminergic neurons is responsible for post-polio fatigue and 2) to enkephalin-producing neurons is responsible for hypersensitivity to pain in polio survivors. Hypotheses are also presented that the anti-metabolic action of glucocorticoids on polio-damaged, metabolically vulnerable neurons is responsible for the fatigue and muscle weakness reported by polio survivors during emotional stress. Suggestions for the treatment of Post-Polio Sequelae based on these hypotheses are also presented.
Muscular effects in late polio.
New or increased muscular weakness, fatigue and muscle and joint pain with neuropathic electromyography (EMG) changes in a person with a confirmed history of polio constitute the cardinal symptoms of the post-polio syndrome. Unusual tiredness or fatigue is a common complaint in late polio subjects as is intolerance to cold. Fatigue in polio subjects can have several explanations: emotional fatigue, central nervous system fatigue, ‘general’ fatigue and/or neuromuscular fatigue. Some studies indicate central fatigue, but it is unclear how often and to which degree there will be a central muscular fatigue. Polio patients are known to be deconditioned (reduced function because of low activity level), and aerobic power is reduced. Defects in the neuromuscular transmission may be present but are not seen in all post-polio subjects with reduction in force and increased fatigability. The fatigue experienced by late polio patients is most likely an augmented peripheral muscle fatigue. Possible explanations may be an imperfection in the sarcoplasmatic reticulum with altered calcium release mechanisms (activation) or in sliding filament function (contractile properties). This may be a secondary effect to the enlarged muscle fibres. However, the prolonged subjective feeling of fatigue reported despite unchanged maximal voluntary contraction (MVC) remains unexplained.
Immune Response
Intrathecal immune response in patients with the post-polio syndrome
BACKGROUND: The syndrome of progressive muscular atrophy decades after acute paralytic poliomyelitis (post-polio syndrome) is not well understood. The theory that physiologic changes and aging cause the new weakness does not explain the immunologic abnormalities reported in some patients. An alternative explanation is persistent or recurrent poliovirus infection.

METHODS: We assessed the intrathecal antibody response to poliovirus and intrathecal production of interleukin-2 and soluble interleukin-2 receptors in 36 patients with the post-polio syndrome and 67 controls (including 13 who had had poliomyelitis but had no new symptoms and 18 with amyotrophic lateral sclerosis). Intrathecal antibody responses to measles, mumps, herpes simplex, and varicella zoster viruses were also determined.

RESULTS: Oligoclonal IgM bands specific to poliovirus were detected in the cerebrospinal fluid of 21 of the 36 patients with the post-polio syndrome (58 percent) but in none of the control group (P less than 0.0001). In quantitative studies there was evidence of increased intrathecal synthesis of IgM antibodies to poliovirus only among the patients with the post-polio syndrome; there was no increased synthesis of IgM to measles, mumps, herpes simplex, or varicella zoster viruses. The patients with post-polio syndrome had significantly higher mean (+/- SD) (cerebrospinal fluid levels of interleukin-2 and soluble interleukin-2 receptors than the controls (8.1 +/- 5.3 vs. 1.4 +/- 0.8 U per milliliter and 159.6 +/- 102.9 vs. 10.7 +/- 6.2 U per milliliter, respectively). The intrathecal synthesis of IgM antibodies to poliovirus correlated with the cerebrospinal fluid concentrations of interleukin-2 (P less than 0.0005) and soluble interleukin-2 receptors (P less than 0.001).
Immune Response
Normal serum levels of immune complexes in postpolio patients
OBJECTIVE: The pathophysiology of the postpolio syndrome is not fully understood. Increased cytokine levels in cerebrospinal fluid and peripheral blood indicate a systemic inflammatory process. Decreased cytokine levels and the clinical effect of intravenous immunoglobulin treatment further indicate an inflammatory/immunological pathogenesis. The aim of the present study was to evaluate whether an autoimmune process follows the initial infection, by means of analyzing immune complexes.

PATIENTS AND METHODS: Circulating immune complexes were analyzed from blood samples of 20 postpolio patients and 95 healthy controls. To compensate for differences in age between patients and controls, a sub-analysis was performed using only the 30 oldest controls. Tumor necrosis factor-inducing properties of polyethylene glycol-precipitated immune complexes were compared between the postpolio patients and 10 healthy controls.

RESULTS: When comparing levels in postpolio patients to the whole control group, including the 30 oldest investigated, there were no statistically significant differences. No difference was found in tumor necrosis factor levels induced by immune complexes when comparing patients and controls.
Immune Response
Leukocyte myeloperoxidase and pathogenesis of the post-polio syndrome
Letter to the Editor - does not have an abstract.
Prior poliomyelitis - evidence of cytokine production in the central nervous system
In order to study the role of a possible inflammatory reaction in the post-polio syndrome (PPS) four key cytokines were determined by means of mRNA expression in mononuclear cells from cerebrospinal fluid (CSF) and peripheral blood of 13 patients. Data were compared with those of samples from eight non-inflammatory control persons. The PPS-patients displayed increased numbers of CSF cells expressing mRNA for TNF-alpha (p<0.02), IFN-gamma (p<0.02), IL-4 (p<0.001) and IL-10 (p<0.05), in comparison to the non-inflammatory controls. As positive controls, samples from patients with Multiple Sclerosis (MS) were examined. We conclude that there is a chronic intra CNS expression of inflammatory cytokines in PPS, in the range of that in MS, a well known neuroinflammatory disease. However, the pathogenic significance of this is unclear.
Elevated blood lipids are uncommon in patients with post-polio syndrome - a cross sectional study.
BACKGROUND: The post-polio syndrome occurs in people who previously have had poliomyelitis. After the initial recovery, new or increasing neurologic symptoms occur. Inflammation and dyslipidaemia may play an important role in the development of atherosclerotic complications, for example myocardial infarction and angina pectoris. Previous studies on cardiovascular risk factors in the post-polio syndrome have found a higher prevalence of hypertension, ischemic heart disease, hyperlipidaemia, and stroke in these patients. The present study was undertaken in order to evaluate whether post-polio patients have elevated lipid values, and if blood lipid abnormalities could be correlated to signs of inflammation.

METHODS: Cross-sectional study of 89 consecutive post-polio patients, (53 women, mean age 65 years) from the Post-Polio Outpatient Clinic, Danderyd University Hospital, Stockholm, Sweden. The lipid profiles of post-polio patients were compared to age and sex matched reference values from two earlier studies. Statistical analyses were performed by the Student's t-test, and linear regression analyses were assessed by Pearson's correlation coefficient.

RESULTS: Mean total cholesterol levels (5.7 mmol/L) were low or normal in post-polio patients, whereas low density lipoprotein levels (3.6 mmol/L) were normal, and high density lipoprotein (1.5 mmol/L) and triglycerides (1.4 mmol/L) lower than reference values. The prevalence of diabetes (7%), hypertension (38%), concomitant cardiovascular disease, (including angina pectoris, myocardial infarction, heart failure, atrial fibrillation and stroke) (7%), and calculated 10 year risk of coronary heart disease according to Framingham risk score algorithm (8%) was not increased in post-polio patients.
Elevated expression of prostaglandin E2 synthetic pathway in skeletal muscle of prior polio patients
OBJECTIVE: The aim of this study was to investigate signs of inflammation in muscle of patients with prior polio, since the main symptoms in these patients are muscle pain, weakness and fatigue. In the context of pain and inflammation, the prostaglandin E2 pathway is of interest. Prostaglandin E2 has many biological actions and is a mediator of inflammation and pain.

PATIENTS AND METHODS: Skeletal muscle biopsies from 8 patients with prior polio and post-polio symptoms, presenting with pain and muscular weakness, and from 6 healthy controls were studied. Immunohistochemistry, conventional microscopy, and computerized image analysis were performed.

RESULTS: There was statistically significant higher expression of enzymes of the prostaglandin E2 synthetic pathway, in muscle from patients, compared with controls. Expression of prostaglandin enzymes was mainly in scattered cells and blood vessels, and may indicate an inflammatory process of the muscle, which could be secondary to systemic inflammation.
Elevated plasma inflammatory mediators in post-polio syndrome: No association with long-term functional decline
A key feature of post-polio syndrome (PPS) is progressive loss of muscle strength. In other chronic diseases systemic inflammation has been linked to muscle wasting. In this study plasma TNF-α, IL-6, IL-8, and leptin levels were significantly increased in PPS-patients compared to healthy controls. There was however no association between these raised systemic levels of inflammatory mediators and long-term decline in quadriceps strength or other clinical parameters. In conclusion, there is evidence for systemic inflammation in PPS, yet the relationship with clinical deterioration remains tenuous.
Late Effects of Polio
New problems in old polio patients: results of a survey of 539 polio survivors
Presented are the results of a questionnaire survey on new health problems in 539 polio survivors. The most common new problems were fatigue, weakness in previously affected and unaffected muscles, muscle pain, and joint pain. The median time from polio to the onset of these problems ranged from 30 to 40 years. Factors at onset of polio most strongly associated with developing these new health problems were: being hospitalized, being over 10 years old, being on a ventilator, and having paralytic involvement of all four limbs. The differential diagnoses of these new problems, implications for treatment and areas for future research are discussed.
Late Effects of Polio
Polio: long-term problems
The most common long-term problems seen in polio are brace problems, knee recurvatum, increasing weakness due to overuse and ankle equinus. A definite increased incidence of problems is seen after the patient is more than 30 years post-polio. The basis for most of these problems is chronic mechanical strain of weak musculature and substituting ligaments. Overuse can cause increasing weakness resulting in pain and decreasing function. It is therefore important to follow polio patients closely, especially those that are more than 30 years post-polio. If signs of overuse or chronic mechanical strain are noted, treatment should not be delayed.
Late Effects of Polio
Psoriasis sparing the lower limb with postpoliomyelitis residual paralysis
This paper does not have an abstract.
Late Effects of Polio
Risk of symptoms related to late effects of poliomyelitis
OBJECTIVE: To compare the risk of developing symptoms related to late effects of poliomyelitis between polio patients and persons of similar age and sex without history of poliomyelitis.

MATERIAL AND METHODS: The study comprised information on 148 patients with prior poliomyelitis and 115 persons with no history of poliomyelitis. Information was obtained by questionnaire and analyzed by multiple logistic regression method.

RESULTS: The risk of experiencing two or more symptoms was significantly higher among the polio patients than among the persons without history of poliomyelitis. The elevation in risk was less pronounced in the nonparalytic group (OR = 2.35; 95% CI = 0.92-5.97) than the group with permanent muscular weakness (OR = 8.84; 95% CI =4.32-18.09).
Late Effects of Polio
Living with polio and postpolio syndrome in the United Kingdom
The term Postpolio Syndrome (PPS) is used to describe new and late manifestations of poliomyelitis that occur later in life in polio survivors. Polio had been eradicated in the United Kingdom (UK) and most of Europe, although this is not the case in all countries. Research in this area has tended to focus upon the impact of polio and PPS on health status and functional health rather than its overall effect on people's lives. This study's two main aims were to explore the ways in which polio and PPS in the UK has affected the respondents' lives and to ascertain their views about how the quality of life could be improved. The two questions were as follows: (1) How has the health of people with polio and PPS affected their quality of life? (2) What would people with polio and PPS change to improve their quality of life? Deductive content analysis using existing qualitative data from a cross-sectional survey of 336 returned questionnaires from persons with polio and PPS was carried out. The average age of the participants was 54 years. Our research found that polio survivors valued social occupations and participation in family life. Our research has also shown that healthcare professionals still do not understand polio and PPS and this lack of understanding influences their clients' quality of life. Finances and accessibility of environments also influence participation in chosen occupations. Rehabilitation programmes for people with polio and PPS need to be targeted towards maintaining and improving accessible environments and participation in chosen occupations, and healthcare professionals need to ensure that persons with polio and PPS are referred to persons with specific expertise in this area.
Late Effects of Polio
Post-polio syndrome and risk factors in Korean polio survivors: a baseline survey by telephone interview
OBJECTIVE: To obtain information on the socioeconomic, medical, and functional status of polio survivors, and to use these results as the preliminary data for establishing the middle-aged cohort of polio survivors.

METHODS: The subjects were recruited based on the medical records of multiple hospitals and centers. They were assessed through a structured questionnaire over the phone. Post-poliomyelitis syndrome (PPS) was identified according to the specified diagnostic criteria. Differences between polio survivors with or without PPS were evaluated, and the risk factors for PPS were analyzed by the odds ratio (OR).

RESULTS: Majority of polio survivors were middle-aged and mean age was 51.2±8.3 years. A total of 188 out of 313 polio survivors met the adopted criteria for PPS based on the symptoms, yielding a prevalence of 61.6%. Mean interval between acute poliomyelitis and the development of PPS was 38.5±11.6 years. Female gender (OR 1.82; confidence interval [CI] 1.09-3.06), the age at onset of poliomyelitis (OR 1.75; CI 1.05-2.94), the use of orthoses or walking aids (OR 2.46; CI 1.44-4.20), and the history of medical treatment for paralysis, pain or gait disturbance (OR 2.62; CI 1.52-4.51) represented independent risk factors for PPS.
Late Effects of Polio
People living with post-polio syndrome must not be forgotten
As research conducted by YouGov reveals, only 7% of people in Britain are aware of the neurological condition post-polio syndrome (PPS). The British Polio Fellowship is keen to highlight what is now an urgent need for more support for those living with PPS, from both the public and the nursing and medical community.
Late Effects of Polio
Late functional deterioration following paralytic poliomyelitis
Many patients with previous poliomyelitis develop 'post-polio syndrome' (PPS) in which late functional deterioration follows a period of relative stability. The frequency with which PPS can be attributed to clearly defined causes remains uncertain. We reviewed 283 newly-referred patients with previous poliomyelitis seen consecutively over a 4-year period; 239 patients developed symptoms of functional deterioration at a mean of 35 (5-65) years after the paralytic illness. Functional deterioration was associated with orthopaedic disorders in 170 cases, neurological disorders in 35, respiratory disorders in 19 and other disorders in 15. Progressive post-polio muscular atrophy was not observed. Functional deterioration following paralytic poliomyelitis is common, and associated with orthopaedic, neurological, respiratory and general medical factors which are potentially treatable.
Muscle Strength
Strength, endurance, and work capacity after muscle strengthening exercise in postpolio subjects
OBJECTIVE: To determine whether a 12-week home quadriceps muscle strengthening exercise program would increase muscle strength, isometric endurance, and tension time index (TTI) in postpolio syndrome subjects without adversely affecting the surviving motor units or the muscle.

DESIGN: A longitudinal study to investigate the effect of a 12-week exercise program on neuromuscular function and electromyographic variables.

SETTING: Neuromuscular laboratory of a university hospital.

SUBJECTS: Seven subjects were recruited from a cohort of 12 subjects who had participated in a previous exercise study. All subjects had greater than antigravity strength of the quadriceps. Upon completion of a postpolio questionnaire, all acknowledged common postpolio syndrome symptoms such as new fatigue, pain, and weakness; 6 of the 7 acknowledged new strength decline.

INTERVENTION: On Mondays and Thursdays subjects performed three sets of four maximal isometric contractions of the quadriceps held for 5 seconds each. On Tuesdays and Fridays subjects performed three sets of 12 dynamic knee extension exercises with ankle weights.

MAIN OUTCOME MEASURES: Neuromuscular variables of the quadriceps muscles were measured at the beginning and completion of the exercise program and included: isokinetic peak torque (ISOKPT, at 60 degrees/sec angular velocity) and total work performed of four contractions (ISOKTW), isometric peak torque (MVC), endurance (EDUR, time subject could hold isometric contraction at 40% of the initial MVC), isometric tension time index (TTI, product of endurance time and torque at 40% of MVC), and initial and final ankle weight (WGT, kg) lifted. Electromyographic variables included: fiber density (FD), jitter (MCD), and blocking (BLK) from single fiber assessment and median macro amplitude (MACRO). Serum creatine kinase (CK) was also measured initially and at 4-week intervals throughout the study.

RESULTS: The following variables significantly (p < .05) increased: WGT by 47%, ISOKPT, 15%, ISOKTW, 15%; MVC, 36%; EDUR, 21%; TTI, 18%. The following variables did not significantly (p > .05) change: FD, MCD, BLK, MACRO, and CK.
Muscle Strength
Neuromuscular function: comparison of symptomatic and asymptomatic polio subjects to control subjects
The purpose of this study was to determine if there were any differences between symptomatic and asymptomatic polio survivors by history of acute poliomyelitis illness, electromyographic evidence of terminal motor unit reorganization, and neuromuscular function of the quadriceps femoris muscle. Thirty-four symptomatic postpolio subjects, 16 asymptomatic postpolio subjects, and 41 controls were studied. A questionnaire assessed polio history. Peak knee extension torque was measured isokinetically and isometrically. Endurance (time to exhaustion) was measured at 40% of maximal isometric torque. Work capacity was determined as the product of torque and duration. Recovery of isometric strength was measured at regular intervals for ten minutes after exhaustion. Quantitative electromyography was also performed on the quadriceps to determine motor unit action potential duration and amplitude. It was found that symptomatic subjects had evidence of more severe original polio involvement by history (documented electromyographically), were weaker and capable of performing less work than asymptomatic subjects, and recovered strength less readily than controls.
Muscle Strength
Reduction in thigh muscle cross-sectional area and strength in a 4-year follow-up in late polio
OBJECTIVE: To study changes in cross-sectional thigh muscle area and muscle strength in late polio subjects over a 4-year period.

DESIGN: Longitudinal study of a cohort of polio survivors, comparing subjects who acknowledge (unstable) with those who do not acknowledge (stable) new muscle weakness.

SETTING: University hospital.

SUBJECTS: Eighteen subjects (6 men, 12 women) with polio-myelitis sequelae (39 to 46 years of age) were studied on two occasions 4 years apart; the first examination was 37 to 44 years after onset of polio. Subjects were recruited through hospital registers, newspaper advertisement, and a patient organization.

OUTCOME MEASUREMENTS: Thigh muscle and intermuscular and intramuscular adipose tissue (AT) cross-sectional areas were measured by computed tomography. Isometric muscle strength for knee extension and flexion was measured using a Kin-Com dynamometer.

RESULTS: Cross-sectional muscle area decreased on average 1.3 +/- 3.6 cm2 (1.4%, p < .05); the intermuscular and intramuscular AT area increased 1.8 +/- 3.4 cm2 (12.1%, p < .05). When divided by legs in which subjects reported (unstable) or did not report (unstable) or did not report (stable) increased muscle weakness, unstable legs showed significant reduction (p < .05) in muscle area, whereas stable legs did not. Estimated total thigh muscle strength decreased 7.8% +/- 2.9% (p < .01), with a significant (p < .001) reduction in unstable legs (13.4% +/- 4.3%) but not in stable legs. The reduction in strength appears to be greater than the reduction in cross-sectional muscle area, but there is still a significant correlation (r = .44, p < .05).
Muscle Strength
An 8-year longitudinal study of muscle strength, muscle fiber size, and dynamic electromyogram in individuals with late polio
Twenty-one subjects with polio 24 to 51 years prior to the first examination were studied on three occasions, each 4 years apart with measurements of muscle strength and endurance for knee extension, macro EMG, and muscle biopsy from vastus lateralis. On average the muscle strength decreased during the 8-year follow-up by 9-15%. Endurance decreased during the observation period. The muscle fiber area was markedly increased in most subjects. There was a decrease in the capillarization during the follow-up. Macro EMG was increased in all subjects (range 3-42 times control) and increased in 20 legs during the 8-year follow-up, but showed a decrease in 8 of 9 legs with an approximative breakpoint when macro MUPs were around 20 times the normal size. Thus, evidence of on-going denervation/reinnervation as well as of failing capacity to maintain large motor units was demonstrated. SFEMG showed a moderate degree of disturbed neuromuscular transmission.
Muscle Strength
The course of functional status and muscle strength in patients with late-onset sequelae of poliomyelitis: a systematic review
OBJECTIVES: To review systematically studies of late-onset polio sequelae on the course of functional status and muscle strength over time and to identify prognostic factors of change.

DATA SOURCES: We conducted a computerized literature search up to July 2004 in MEDLINE, EMBASE, CINAHL, Web of Science, PsychInfo, and the Cochrane controlled trial register using the key words: postpolio, postpoliomyelitis, postpoliomyelitis syndrome, post poliomyelitis muscular atrophy, and poliomyelitis.

STUDY SELECTION: Reports were selected by 1 reviewer if the study involved subjects with a history of poliomyelitis, the outcome measures described functional status or muscle strength, and follow-up was for at least 6 months.

DATA EXTRACTION: Studies were summarized with regard to population, design, sample size, outcome measures, results, and methodologic scores. Overlap in populations between studies was checked.

DATA SYNTHESIS: Of 71 potentially relevant studies, 19 were included (2 on functional status, 15 on muscle strength, 2 on both muscle strength and functional status). Two studies on the course of functional status had sufficient quality and reported inconsistent results. Four studies on the course of muscle strength had sufficient quality. Two studies reported a decline in strength and 2 reported no change. Decline in strength was only reported in studies with a follow-up period longer than 2 years. One study reported extent of paresis as a prognostic factor for change in perceived physical mobility.
Muscle Strength
Disability in a 4-year follow-up study of people with post-polio syndrome
OBJECTIVE: To evaluate changes over time in a clinically based cohort of individuals with post-polio syndrome.

DESIGN: A prospective longitudinal study.

SUBJECTS: A total of 106 individuals with poliomyelitis sequelae were included in the study. They were self-referred or had been referred to the post-polio clinic. After 4 years subjects were called for a follow-up and underwent the same measurements as at the initial assessment.

METHODS: The following measurements were conducted at both the initial assessment, and the follow-up: questionnaires including Nottingham Health Profile, muscle strength and walking speed.

RESULTS: Minor changes in disability during a 4-year period were shown. A significant reduction in muscle strength was only seen for 60° flexion in the left leg and for right and left dorsal flexion. No change could be seen in the total Nottingham Health Profile score.
Muscle Strength
Reliability of knee extensor and flexor muscle strength measurements in persons with late effects of polio
OBJECTIVE: To assess the reliability of knee extensor and flexor muscle strength measurements in persons with late effects of polio.

DESIGN: A test-retest reliability study.

SUBJECTS: Thirty men and women (mean age 63 (standard deviation 6.4) years) with verified late effects of polio.

METHODS: Knee extensor and flexor muscle strength in both lower limbs were measured twice 7 days apart using a Biodex dynamometer (isokinetic concentric contractions at 60°/s and isometric contractions with knee flexion angle 90º) and a Leg Extension/Curl Rehab exercise machine with pneumatic resistance (HUR) (isotonic contractions). Reliability was assessed with the intraclass correlation coefficient (ICC1,1), the mean difference between the test sessions (đ) together with the 95% confidence intervals for đ, the standard error of measurement (SEM and SEM%), the smallest real difference (SRD and SRD%) and Bland-Altman graphs.

RESULTS: Test-retest agreements were high, (ICC1,1 0.93–0.99) and measurement errors generally small. The SEM% was 4–14% and the SRD% 11–39%, with the highest values for the isokinetic measurements.
Muscle Strength
Reliability of contractile properties of the knee extensor muscles in individuals with post-polio syndrome.
OBJECTIVE: To assess the reliability of contractile properties of the knee extensor muscles in 23 individuals with post-polio syndrome (PPS) and 18 age-matched healthy individuals.

METHODS: Contractile properties of the knee extensors were assessed from repeated electrically evoked contractions on 2 separate days, with the use of a fixed dynamometer. Reliability was determined for fatigue resistance, rate of torque development (MRTD), and early and late relaxation time (RT50 and RT25), using the intraclass correlation coefficient (ICC) and standard error of measurement (SEM, expressed as % of the mean).

RESULTS: In both groups, reliability for fatigue resistance was good, with high ICCs (>0.90) and small SEM values (PPS: 7.1%, healthy individuals: 7.0%). Reliability for contractile speed indices varied, with the best values found for RT50 (ICCs>0.82, SEM values <2.8%). We found no systematic differences between test and retest occasions, except for RT50 in healthy subjects (p = 0.016).

SIGNIFICANCE: This was the first study to examine the reliability of electrically evoked contractile properties in individuals with PPS. Our results demonstrate its potential to study mechanisms underlying muscle fatigue in PPS and to evaluate changes in contractile properties over time in response to interventions or from natural course.
Muscle Strength
Perceived disability, fatigue, pain and measured isometric muscle strength in patients with post-polio symptoms
BACKGROUND AND PURPOSE: Several years after the acute polio illness, patients may develop new post-polio symptoms. The purpose of the present study was to evaluate patients with post-polio symptoms with regard to perceived fatigue, functional ability, muscle strength, pain and with regard to measured physical fitness and isometric muscle strength. In addition, the relationship between the results of these subjective and objective measurements was investigated.

METHOD: This was a prospective cross-sectional study in which 32 patients with post-polio symptoms were included. Main outcome measures were the Fatigue Severity Scale (FSS), the Disability Rating Index (DRI), pain intensity, pain distribution, self-reported and measured muscle strength and oxygen uptake.

RESULTS: A marked reduction in isometric muscle strength compared to normal data, high scores in fatigue, widespread pain, low oxygen uptake and difficulties in performing some daily activities were found. Self-reported general muscle strength, pain intensity and pain distribution correlated significantly with patients' perceived fatigue and function at the activity level. There was no significant correlation between self-reported and measured results except for that found between isometric muscle strength in the legs and patients' perceived general muscle strength and oxygen uptake.
Muscle Strength
Men With Late Effects of Polio Decline More Than Women in Lower Limb Muscle Strength: A 4-Year Longitudinal Study
BACKGROUND: In persons with prior paralytic poliomyelitis, progressive muscle weakness can occur after a stable period of at least 15 years. Knowledge is limited about which factors influence changes in lower limb muscle strength in these persons.

OBJECTIVE: To assess changes in lower limb muscle strength annually over 4 years in persons with late effects of polio and to identify prognostic factors for changes in muscle strength.

DESIGN: A prospective, longitudinal study.

SETTING: University hospital outpatient program.

PARTICIPANTS: Fifty-two ambulant persons (mean age ± standard deviation: 64 ± 6 years) with verified late effects of polio.

METHODS: Mixed linear models were used to analyze changes in muscle strength and to identify determinants among the following covariates: gender, age, age at acute polio infection, time with late effects of polio, body mass index, and estimated baseline muscle weakness.

MAIN OUTCOME MEASUREMENTS: Knee extensor and flexor and ankle dorsiflexor muscle strength were measured annually with a Biodex dynamometer.

RESULTS: The men (n = 28) had significant linear change over time for all knee muscle strength measurements, from -1.4% (P < .05) per year for isokinetic knee flexion in the less-affected lower limb to -4.2% (P < .001) for isokinetic knee extension in the more-affected lower limb, and for 2 ankle dorsiflexor muscle strength measurements (-3.3%-1.4% per year [P < .05]). The women (n = 24) had a significant linear change over time only for ankle dorsiflexor measurements (4.0%-5.5% per year [P < .01]). Gender was the strongest factor that predicted a change in muscle strength over time.
Muscle Strength
Quantitative muscle ultrasound and quadriceps strength in patients with post-polio syndrome
INTRODUCTION: We investigated whether muscle ultrasound can distinguish muscles affected by post-polio syndrome (PPS) from healthy muscles and whether severity of ultrasound abnormalities is associated with muscle strength.

METHODS: Echo intensity, muscle thickness, and isometric strength of the quadriceps muscles were measured in 48 patients with PPS and 12 healthy controls.

RESULTS: Patients with PPS had significantly higher echo intensity and lower muscle thickness than healthy controls. In patients, both echo intensity and muscle thickness were associated independently with muscle strength. A combined measure of echo intensity and muscle thickness was more strongly related to muscle strength than either parameter alone.
Muscle Strength
Reliability of contractile properties of the knee extensor muscles in individuals with post-polio syndrome.
To assess the reliability of contractile properties of the knee extensor muscles in 23 individuals with post-polio syndrome (PPS) and 18 age-matched healthy individuals.

Contractile properties of the knee extensors were assessed from repeated electrically evoked contractions on 2 separate days, with the use of a fixed dynamometer. Reliability was determined for fatigue resistance, rate of torque development (MRTD), and early and late relaxation time (RT50 and RT25), using the intraclass correlation coefficient (ICC) and standard error of measurement (SEM, expressed as % of the mean).

In both groups, reliability for fatigue resistance was good, with high ICCs (>0.90) and small SEM values (PPS: 7.1%, healthy individuals: 7.0%). Reliability for contractile speed indices varied, with the best values found for RT50 (ICCs>0.82, SEM values <2.8%). We found no systematic differences between test and retest occasions, except for RT50 in healthy subjects (p = 0.016).

In PPS and healthy individuals, the reliability of fatigue resistance, as obtained from electrically evoked contractions is high. The reliability of contractile speed is only moderate, except for RT50 in PPS, demonstrating high reliability.

This was the first study to examine the reliability of electrically evoked contractile properties in individuals with PPS. Our results demonstrate its potential to study mechanisms underlying muscle fatigue in PPS and to evaluate changes in contractile properties over time in response to interventions or from natural course.
Muscle Strength
Randomized controlled trial of strength training in post-polio patients
Many post-polio patients develop new muscle weakness decades after the initial illness. However, its mechanism and treatment are controversial. The purpose of this study was to test the hypotheses that: (1) after strength training, post-polio patients show strength improvement comparable to that seen in the healthy elderly; (2) such training does not have a deleterious effect on motor unit (MU) survival; and (3) part of the strength improvement is due to an increase in voluntary motor drive. After baseline measures including maximum voluntary contraction force, voluntary activation index, motor unit number estimate, and the tetanic tension of the thumb muscles had been determined, 10 post-polio patients with hand involvement were randomized to either the training or control group. The progressive resistance training program consisted of three sets of eight isometric contractions, three times weekly for 12 weeks. Seven healthy elderly were also randomized and trained in a similar manner. Changes in the baseline parameters were monitored once every 4 weeks throughout the training period. The trained post-polio patients showed a significant improvement in their strength (P < 0.05). The magnitude of gain was greater than that seen in the healthy elderly (mean ± SE, 41 ± 16% vs. 29 ± 8%). The training did not adversely affect MU survival and the improvement was largely attributable to an increase in voluntary motor drive. We therefore conclude that moderate intensity strength training is safe and effective in post-polio patients.
Muscle Strength
Strength gains without muscle injury after strength training in patients with postpolio muscular atrophy.
We evaluated changes in the dynamic and isometric strength in the newly weakened quadriceps muscles and asymptomatic triceps muscles of 6 patients with postpolio muscular atrophy (PPMA) after 10 weeks of progressive resistance strength training. Alterations in muscle size were determined with magnetic resonance imaging. Serum creatine kinase levels were measured throughout training, and histological signs of muscle injury and changes in muscle fiber size and types were assessed with muscle biopsies before and after training. Exercise training led to an increase in dynamic strength of 41% and 61% for the two knee extensor tests, and 54% and 71% for the two elbow extensor tests. Up to 20% of the improvement was maintained 5 months after cessation of training. Isometric strength, whole muscle cross-sectional areas of quadriceps and triceps muscles, and serum muscle enzymes did not change. No destructive histopathological changes were noted in the repeat muscle biopsies, and no consistent changes in muscle fiber size or fiber type percentages were observed. These results demonstrate that a supervised resistance training program can lead to significant gains in dynamic strength of both symptomatic and asymptomatic muscles of PPMA patients without serological or histological evidence of muscular damage.
Muscular Atrophy
A case of post-poliomyelitis muscular atrophy with cranial nerve signs and widespread muscular atrophy of extremities
Here we report a case of a 56-year-old male with post-poliomyelitis muscular atrophy (PPMA), who presented with cranial nerve signs and widespread atrophy of the extremities. He had suffered from poliomyelitis at the age of 2 years. After recovery from the acute stage, the paralysis remained in his left arm. He noticed muscle weakness of the right upper and lower extremities at the age of 45 years and the muscle atrophy progressed to his arms, hip and thigh at the age of 55 years. Neurological examination revealed muscle atrophy of the neck and disturbance of left V, VIII, IX, X and bilateral XI cranial nerves. We diagnosed this case as PPMA from his history and electromyographic and muscle biopsy findings which suggested chronic denervation. Among the 21 PPMA cases in the past in which the acute poliomyelitis had resulted in paralysis of the only one limb, ours was the only case that had muscle atrophy of all the limbs. Cranial nerve involvement is known to occur in acute poliomyelitis; therefore, there is a possibility that the involvement of the cranial nerves in our case might be a delayed progressive symptoms.
Muscular Atrophy
Late postpoliomyelitis muscular atrophy: clinical, virologic, and immunologic studies
Seventeen relatively young patients, ages 31-65 years (average, 45) with prior poliomyelitis, who after a number of years of stability had experienced new neuromuscular symptoms, were studied. Seven patients had deterioration of functional capacity and then stabilization without new muscular weakness. The other 10 had late postpoliomyelitis muscular atrophy (late PPMA) characterized by focal progressive muscle weakness, wasting, fasciculations, and muscle pains affecting previously spared muscles or muscles previously affected but recovered. Four patients with late PPMA had lymphorrhages or lymphocytic infiltrates in their biopsied muscle; three of three patients had oligoclonal IgG bands in their spinal fluid, and five had variable peripheral T lymphocyte-subset ratios. In one patient with late PPMA, antibodies to poliovirus were specifically elevated in the cerebrospinal fluid. Our findings indicate that new motor-neuron disease can occur in patients with prior poliomyelitis and that immunopathologic mechanisms may play a role.
Muscular Atrophy, Post-Polio Motor Unit
An autopsy case of progressive generalized muscle atrophy over 14 years due to post-polio syndrome
We report the case of a 72-year-old man who had contracted acute paralytic poliomyelitis in his childhood. Thereafter, he had suffered from paresis involving the left lower limb, with no relapse or progression of the disease. He began noticing slowly progressive muscle weakness and atrophy in the upper and lower extremities in his 60s. At the age of 72, muscle weakness developed rapidly, and he demonstrated dyspnea on exertion and dysphagia. He died after about 14 years from the onset of muscle weakness symptoms. Autopsy findings demonstrated motoneuron loss and glial scars not only in the plaque-like lesions in the anterior horns, which were sequelae of old poliomyelitis, but also throughout the spine. No Bunina bodies, TDP-43, and ubiquitin inclusions were found. Post-polio syndrome is rarely fatal due to rapid progressive dyspnea and dysphagia. Thus, the pathological findings in the patient are considered to be related to the development of muscle weakness.
Gait Analysis Techniques
In the gait laboratory at Rancho Los Amigos Hospital, the emphasis is on patient testing to identify functional problems and determine the effectiveness of treatment programs. Footswitch stride analysis, dynamic EMG, energy-cost measurements, force plate, and instrumented motion analysis are the techniques most often used. Stride data define the temporal and distance factors of gait. We use this information to classify the patient's ability to walk and measure response to treatment programs. Inappropriate muscle action in the patient disabled by an upper motor neuron lesion is identified with dynamic EMG. Intramuscular wire electrodes are used to differentiate the action of adjacent muscles. We use the information to localize the source of abnormal function so that selection of treatment procedures is more precise. Force and motion data aid in determining the functional requirement and the muscular response necessary to meet the demand. Determining the optimum mode of locomotion and developing criteria for program planning have become more realistic with the aid of energy-cost measurements. Microprocessors and personal computer systems have made compact and reliable single-concept instrumentation available for basic gait analysis in the standard clinical environment at a modest cost. The more elaborate composite systems, however, still require custom instrumentation and engineering support.
Findings in Post-Poliomyelitis Syndrome
The purpose of this study was to identify overuse of muscles and other alterations in the mechanics of gait in twenty-one patients who had muscular dysfunction as a late consequence of poliomyelitis. All of the patients had good or normal strength (grade 4 or 5) of the vastus lateralis and zero to fair strength (grade 0 to 3) of the calf, as determined by manual testing.

Dynamic electromyography was used, while the patients were walking, to quantify the intensity and duration of contraction of the inferior part of the gluteus maximus, the long head of the biceps femoris. the vastus lateralis, and the soleus muscles. Patterns of contact of the foot with the floor, temporal-spatial parameters, and motion of the knee and ankle were recorded.

The principal mechanisms of substitution for a weak calf muscle fell into three groups: overuse of the quadriceps (twelve patients) or a hip extensor (the inferior part of the gluteus maximus in eight patients and the long head of the biceps femoris in four), or both; equinus contracture (twelve patients); and avoidance of loading-response flexion of the knee (five patients). Most patients used more than one method of substitution.

These obervations support the theory that postpoliomyelitis syndrome results from long-term substitutions for muscular weakness that place increased demands on joints, ligaments, and muscles and that treatment -- based on the early identification of overuse of muscles and ligamentous strain -- should aim at modification of lifestyle and include use of a brace.
Gait patterns in association with underlying impairments in polio survivors with calf muscle weakness
The objective was to identify gait patterns in polio survivors with calf muscle weakness and associate them to underlying lower extremity impairments, which are expected to help in the search for an optimal orthosis.

Unilaterally affected patients underwent barefoot 3D-gait analyses. Gait pattern clusters were created based on the ankle and knee angle and ankle moment shown in midstance of the affected limb. Impairment clusters were created based on plantarflexor and knee-extensor strength, and ankle and knee joint range-of-motion. The association between gait patterns and underlying impairments were examined descriptively. The Random Forest Algorithm and regression analyses were used to predict gait patterns and parameters.

Seven gait patterns in 73 polio survivors were identified, with two dominant patterns: one with a mildly/non-deviant ankle angle, ankle moment and knee angle (n = 23), and one with a strongly deviant ankle angle and a mildly/non-deviant ankle moment and knee angle (n = 18). Gait pattern prediction from underlying impairments was 49% accurate with best prediction performance for the second dominant gait pattern (sensitivity 78% and positive predictive value 74%). The underlying impairments explained between 20 and 32% of the variance in individual gait parameters.

Polio survivors with calf muscle weakness who present a similar impairment profile do not necessarily walk the same. From physical examination alone, the gait pattern nor the individual gait parameters could be accurately predicted. The patient’s gait should therefore be measured to help in the prescription and evaluation of orthoses for these patients.
Pain in persons with post-polio syndrome: frequency, intensity, and impact
OBJECTIVE: To describe the frequency, intensity, and impact of pain in persons with postpoliomyelitis syndrome (PPS).

DESIGN: Retrospective, cross-sectional survey.

SETTING: Community-based survey.

PARTICIPANTS: Convenience sample of people with PPS.

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Overall intensity and duration of pain, pain sites, pain interference, pain treatments, and relief provided by pain treatments.

RESULTS: A total of 91% (n=57) of the study participants (N=63) reported pain. The most frequently reported pain sites were the shoulders, lower back, legs, and hips. Participants reported pain intensity to be the greatest in the knees, legs, wrists, lower back, and head. Pain interfered most with sleep and with activities requiring a high level of musculoskeletal involvement. Respondents also reported pain problems that were more severe than those of the general population and than those of a sample of people with multiple sclerosis. Many treatments had been tried previously for pain, but continued use of treatments was reported by relatively few participants at the time of the survey.
Psychosocial factors and adjustment to pain in individuals with postpolio syndrome
OBJECTIVE: The purpose of the current study was to examine the associations among measures of psychosocial factors, pain, and adjustment in persons with postpoliomyelitis syndrome.

DESIGN: A cross-sectional survey design was used. Sixty-three community-dwelling individuals with postpoliomyelitis syndrome returned completed questionnaires (71% response rate) that included measures of pain intensity and interference, psychological functioning, pain catastrophizing, social support, and pain-related beliefs and coping.

RESULTS: After controlling for demographic variables, the group of psychosocial variables accounted for an additional 23% of the variance in pain intensity. These variables explained an additional 35% and 50% of the variance in pain interference and psychological functioning, respectively, after accounting for demographic variables and pain intensity. Social support was associated with both psychological functioning and pain interference, whereas catastrophizing was most closely related to psychological functioning. Individual pain beliefs and coping strategies were variably related to the three criterion measures.
Analysis of long-standing nociceptive and neuropathic pain in patients with post-polio syndrome
The purpose of this study was to analyze pain, both nociceptive and neuropathic, in patients with post-polio syndrome (PPS) and relate the pain to age at the initial polio infection, age at examination, to gender and disability. The study was conducted in a university hospital department. Patients with PPS were interviewed at their regular visits about pain, its character, intensity and localization. A clinical examination, including a thorough neurological examination, was performed. Data included age at time of polio infection, age at time of examination and gender. Pain intensity was measured with the VAS-scale and walking capability by the WISCI-scale. One hundred sixty-three (88 women, 75 men) patients were included in the study. Pain was present in 109 (67%). Pain was more frequently reported by women (82%) than by men (49%). 96 patients experienced nociceptive pain, 10 patients both neuropathic and nociceptive pain and three experienced pure neuropathic pain. Half of the patients with pain experienced pain in more than one body region. When neuropathic pain was present, another additional neurological disorder was diagnosed. Pain was more often found in younger patients (around 70%) than in older patients (around 50%). In summary pain is common in patients with PPS and most patients experienced nociceptive pain. Women have pain more often than men. Older patients experience pain more seldom than younger patients. Age at time of primary polio infection is important for the development of pain. When neuropathic pain is present, it is important to proceed with neurological examination to find an adequate diagnosis.
Predictive factors and correlates for pain in postpoliomyelitis syndrome patients
OBJECTIVE: To identify predictive and associated factors for muscle and joint pain in postpoliomyelitis syndrome (PPS).

DESIGN: Cross-sectional study design.

SETTING: Postpolio clinics.

PARTICIPANTS: Baseline data on 126 PPS patients entered into a multicentered clinical trial.

INTERVENTIONS: Not applicable.

MAIN OUTCOME MEASURES: Adjusted odds ratios were computed by using logistic regression modeling. Patients with or without muscle or joint pain were compared with regard to predictors and associated factors.

RESULTS: In multivariate analyses for muscle pain, significant predictive and associated factors were female gender (P=.0006), longer duration of general fatigue (P=.019), and a lower score on the general health scale (P=.009) of the Medical Outcomes Study 36-Item Short-From Health Survey (SF-36). In multivariate analyses for joint pain, significant predictive and associated factors were female gender (P<.003), longer latency (duration of stability after polio; P=.008), younger age at interview (P<.002), greater weakness at acute polio (P<.07), weaker lower-extremity muscle strength (P<.04), and a lower SF-36 general health scale score (P<.02).
Modeling subjective well-being in individuals with chronic pain and a physical disability: the role of pain control and pain catastrophizing.
To investigate the associations between subjective well-being and pain intensity, pain interference, and depression in individuals with physical disabilities. We hypothesized that (1) pain control and (2) pain catastrophizing mediate the effects of subjective well-being on pain intensity, pain interference, and depression.

Analyses of cross-sectional data from 96 individuals diagnosed with spinal cord injury, multiple sclerosis, neuromuscular disease, or post-polio syndrome, with average pain intensity of ≥4 (0-10) on at least half the days in the past month. Two models tested study hypotheses using structural equation.

Both models showed acceptable model fit. Pain catastrophizing significantly mediated the effect of subjective well-being on pain intensity and pain interference, but not on depression. Pain control did not significantly mediate the effect of subjective well-being on pain intensity, pain interference, or depression. Path coefficients showed significant direct effects of subjective well-being on pain control (β = 0.39), pain catastrophizing (β = -0.61), pain interference (β = -0.48; -0.42), and depression (β = -0.75; -0.78).

This study supports the potential of enhancing subjective well-being and lowering pain catastrophizing for reducing pain intensity, pain interference, and depressive symptoms in individuals with chronic pain and a physical disability. The findings indicate that true experiments to test for causal associations are warranted. Implications for rehabilitation The majority of individuals with physical disabilities report having persistent moderate-to-severe pain that may negatively limit daily activities and quality of life. The present cross-sectional study indicates that individuals who reported greater subjective well-being showed significantly lower pain intensity via the mediating effect of lower pain catastrophizing. Since sample size and respective power are low, these findings should be taken as first indications of potential underlying mechanisms between subjective well-being and pain outcomes that need further confirmation in longitudinal research. However, the findings suggest that treatments which enhance subjective well-being (increasing positive affect and life satisfaction, and decreasing negative affect, e.g., via positive psychology exercises) and reducing pain catastrophizing (via e.g., cognitive-behavioral therapy) may have the highest potential for benefiting individuals with disability-associated chronic pain.
Pain, Quality of Life
Impact of pain on quality of life in patients with post-polio syndrome
OBJECTIVE: Post-polio syndrome is a neurological disorder occurring several years after an acute polio infection. The main symptoms are increased muscular weakness and atrophy, fatigue and pain. Pain is present more often in younger individuals and in females and, according to the visual analogue scale (VAS), the intensity of pain is relatively high. The aim of the present study was to analyse the impact of pain on quality of life in patients with post-polio syndrome.

DESIGN: Transversal study.

PATIENTS AND METHODS: Patients with post-polio syndrome underwent a thorough neurological and general examination. They were interviewed about the presence and intensity of pain during the previous 3 months, then completed the quality of life inventory Short-Form 36 (SF-36), which included questions about pain during the previous 4 weeks, and rated their pain intensity during the previous 24 h according to the VAS.

RESULTS: Seventy-seven of the patients (68%) experienced pain at the examination. Pain was found to have a significant impact on the SF-36 subdomains Vitality and General health. A correlation was found between pain during the previous 3 months, the previous 4 weeks, and the previous 24 h.

DISCUSSION: Pain is common in patients with post-polio syndrome. Although patients have a high mean VAS score the pain only affects quality of life for Vitality and General Health, but not for other physical and mental domains.
Polio Immunisation
Preparation for global introduction of inactivated poliovirus vaccine: safety evidence from the US Vaccine Adverse Event Reporting System, 2000–12
BACKGROUND: Safety data from countries with experience in the use of inactivated poliovirus vaccine (IPV) are important for the global polio eradication strategy to introduce IPV into the immunisation schedules of all countries. In the USA, IPV has been included in the routine immunisation schedule since 1997. We aimed to analyse adverse events after IPV administration reported to the US Vaccine Adverse Event Reporting System (VAERS).

METHODS: We analysed all VAERS data associated with IPV submitted between Jan 1, 2000, and Dec 31, 2012, either as individual or as combination vaccines, for all age and sex groups. We analysed the number and event type (non-serious, non-fatal serious, and death reports) of individual reports, and explored the most commonly coded event terms to describe the adverse event. We classified death reports according to previously published body-system categories (respiratory, cardiovascular, neurological, gastrointestinal, other infectious, and other non-infectious) and reviewed death reports to identify the cause of death. We classified sudden infant death syndrome as a separate cause of death considering previous concerns about sudden infant syndrome after vaccines. We used empirical Bayesian data mining methods to identify disproportionate reporting of adverse events for IPV compared with other vaccines. Additional VAERS data from 1991 to 2000 were analysed to compare the safety profiles of IPV and oral poliovirus vaccine (OPV).

FINDINGS: Of the 41 792 adverse event reports submitted, 39 568 (95%) were for children younger than 7 years. 38 381 of the reports for children in this age group (97%) were for simultaneous vaccination with IPV and other vaccines (most commonly pneumococcal and acellular pertussis vaccines), whereas standalone IPV vaccines accounted for 0·5% of all reports. 34 880 reports were for non-serious events (88%), 3905 reports were for non-fatal serious events (10%), and 783 reports were death reports (2%). Injection-site erythema was the most commonly coded term for non-serious events (29%), and pyrexia for non-fatal serious events (38%). Most deaths (96%) were in children aged 12 months or younger; most (52%) had sudden infant death syndrome as the reported cause of death. The safely profiles of combined IPV and whole-cell pertussis vaccines, OPV and whole-cell pertussis vaccines, and OPV and acellular pertussis vaccines were similar. We noted no indication of disproportionate reporting of adverse events after immunisation with IPV-containing vaccines compared with other vaccines between 1990 and 2013.

INTERPRETATION: Fairly few adverse events were reported for the more than 250 million IPV doses distributed between 2000 and 2012. Sudden infant death syndrome reports after IPV were consistent with reporting patterns for other vaccines. No new or unexpected vaccine safety problems were identified for fatal, non-fatal serious, and non-serious reports in this assessment of adverse events after IPV.
Polio Immunisation
New vaccine strategies to finish polio eradication
The Global Polio Eradication Initiative (GPEI) currently faces two specific challenges. First, all the cases in the past 9 months caused by ongoing wild-virus transmission were in Afghanistan and Pakistan—Africa has had a remarkable 9 months without detection of the disease. Second, circulating vaccine-derived polioviruses are continuing to cause poliomyelitis in a few countries, a rare outcome associated with continued use of the live-attenuated oral poliovirus vaccine (OPV). In The Lancet Infectious Diseases, the results of two clinical trials of OPV that address these challenges are reported by Fatima Mir and colleagues and Concepción Estívariz and colleagues.
Polio Immunisation
Next generation inactivated polio vaccine manufacturing to support post polio-eradication biosafety goals
Worldwide efforts to eradicate polio caused a tipping point in polio vaccination strategies. A switch from the oral polio vaccine, which can cause circulating and virulent vaccine derived polioviruses, to inactivated polio vaccines (IPV) is scheduled. Moreover, a manufacturing process, using attenuated virus strains instead of wild-type polioviruses, is demanded to enhance worldwide production of IPV, especially in low- and middle income countries. Therefore, development of an IPV from attenuated (Sabin) poliovirus strains (sIPV) was pursued. Starting from the current IPV production process based on wild type Salk strains, adaptations, such as lower virus cultivation temperature, were implemented. sIPV was produced at industrial scale followed by formulation of both plain and aluminium adjuvanted sIPV. The final products met the quality criteria, were immunogenic in rats, showed no toxicity in rabbits and could be released for testing in the clinic. Concluding, sIPV was developed to manufacturing scale. The technology can be transferred worldwide to support post polio-eradication biosafety goals.
Polio Immunisation
Immunogenicity of two different sequential schedules of inactivated polio vaccine followed by oral polio vaccine versus oral polio vaccine alone in healthy infants in China
BACKGROUND: Two vaccination schedules where inactivated polio vaccine (IPV) was followed by oral polio vaccine (OPV) were compared to an OPV-only schedule.

METHODS: Healthy Chinese infants received a 3-dose primary series of IPV-OPV-OPV (Group A), IPV-IPV-OPV (Group B), or OPV-OPV-OPV (Group C) at 2, 3, and 4 months of age. At pre-Dose 1, 1-month, and 14-months post-Dose 3, polio 1, 2, and 3 antibody titers were assessed by virus-neutralizing antibody assay with Sabin or wild-type strains. Adverse events were monitored.

RESULTS: Anti-polio 1, 2, and 3 titers were ≥8 (1/dil) in >99% of participants, and Group A and Group B were noninferior to Group C at 1-month post-Dose 3 as assessed by Sabin strain-based assay (SSBA). In Group A 1-month post-Dose 3, there was no geometric mean antibody titers (GMT) differences for types 1 and 3; type 2 GMTs were ≈3-fold higher by wild-type strain-based assay (WTBA) versus SSBA. For Group B, GMTs were ≈1.7- and 3.6-fold higher for types 1 and 2 via WTBA, while type 3 GMTs were similar. For Group C, GMTs were ≈6.3- and 2-fold higher for types 1 and 3 with SSBA, and type 2 GMTs were similar. Antibodies persisted in >96.6% of participants. Adverse event incidence in each group was similar.
Polio Immunisation
A cross-sectional survey of healthcare workers on the knowledge and attitudes towards polio vaccination in Pakistan
INTRODUCTION: Pakistan accounts for 85.2% of the total polio cases reported worldwide. Healthcare workers (HCWs) are an integral part of immunization campaigns and source of education for the general public. This study aimed to assess the knowledge and attitudes towards polio vaccination among HCWs providing immunisation and education to general public in Quetta and Peshawar divisions of Pakistan.

METHODS: A cross-sectional survey of 490 HCWs was conducted in two major referral public teaching hospitals of Quetta and Peshawar divisions. During February to April, 2015, a random sample of 490 HCWs was invited to participate in this study. Knowledge and attitudes were assessed by using self-administered, anonymous and pretested questionnaire. Descriptive and logistic regression analyses were used to express the results.

RESULTS: A total of 468 participants responded to the questionnaire, giving a response rate of 95.5%. Overall, participants demonstrated good knowledge and positive attitudes towards polio vaccination. The mean knowledge score of HCWs about polio was 13.42±2.39 (based on 18 knowledge questions) while the mean attitude score was 28.75±5.5 (based on 9 attitudes statements). Knowledge gaps were identified about the incubation period of poliovirus (19.5%), management issues (31.9%), use of polio vaccine in mild illnesses (34.7%) and the consequences of the polio virus (36.9%). The majority of participants agreed that all children should be vaccinated for polio (95.1%), while reservations were noted about the need of a booster (38.9%), and sterility issues associated with polio vaccines (43.6%). Internet (n = 167, 37%) and Posters (n = 158, 35%) were the main sources used by HCWs to educate themselves about polio.
Polio Immunisation
Applying the concept of peptide uniqueness to anti-polio vaccination
BACKGROUND: Although rare, adverse events may associate with anti-poliovirus vaccination thus possibly hampering global polio eradication worldwide.

OBJECTIVE: To design peptide-based anti-polio vaccines exempt from potential cross-reactivity risks and possibly able to reduce rare potential adverse events such as the postvaccine paralytic poliomyelitis due to the tendency of the poliovirus genome to mutate.

METHODS: Proteins from poliovirus type 1, strain Mahoney, were analyzed for amino acid sequence identity to the human proteome at the pentapeptide level, searching for sequences that (1) have zero percent of identity to human proteins, (2) are potentially endowed with an immunologic potential, and (3) are highly conserved among poliovirus strains.

RESULTS: Sequence analyses produced a set of consensus epitopic peptides potentially able to generate specific anti-polio immune responses exempt from cross-reactivity with the human host.
Polio Immunisation
Review of the health consequences of SV40 contamination of poliomyelitis vaccines, and in particular a possible association with cancers
The published papers concerning the human health risk of vaccines contaminated with SV40 virus falls into three groups: those published in the 1960s when the virus was discovered, a second group dating from the period when the two related human viruses BK and JC were described and the third recent period when molecular techniques were applied to the problem.

GROUP 1: SV40 was discovered in 1960 and shown
~ To be a common infection in healthy rhesus monkeys
~ To belong to the polyoma virus family
~ To cause tumours (especially ependymomas, osteosarcomas, mesotheliomas and lymphomas) when injected into baby hamsters
~ To be incompletely killed by the heat and formalin treatment used to inactivate polioviruses during "Salk" vaccine manufacture
~ To be capable of infecting human recipients of contaminated vaccine
~ To be capable of transforming human cells into cancer cells in the laboratory

Immediate steps were taken to free the vaccine seed cultures of SV40 and to ensure that all future batches of vaccine (both the inactivated "Salk" and the then prototype attenuated "Sabin" types) were made in monkey kidney cultures free of SV40. This was accomplished in 1963.

Many millions of children and adults had already been inoculated with polio vaccine before these measures were fully effective. It is not known which of the early batches actually contained infectious doses of SV40, but tests of recipients showed that many produced SV40 antibodies. This could be the result of either SV40 infection or "immunisation" by the killed SV40 in the vaccine.

Concern focussed on the risk to very young children but no increased risk of cancer was found in follow up of over 1000 vaccinees. As the tumour types induced in hamsters are rare these studies were supplemented with much larger studies comparing cancer registry data for children born (and presumably mostly immunised) during the period between introduction of polio vaccine and eradication of SV40 from manufacture (ie 1957-63) and children born within the preceding or subsequent five year periods. These studies were reassuring, although it was recognised that the follow up was not sufficiently long term to detect a risk of the cancers such as mesothelioma which occur in middle age and beyond. There were also some discrepant reports but in retrospect these (including the single Australian study) have significant design limitations.

GROUP 2: The issue was revived in the 1970s when two new human polyomaviruses (BK and JC) were discovered. These cause tumours and degenerative neurological disease in humans. They also share antigens and DNA sequences with SV40 which may cause cross reactions leading to false positive results in diagnostic tests. Surveys showed that serological evidence of infection with the two new agents was common in healthy people and that disease emerged almost exclusively in immune deficient individuals. Attempts to isolate SV40 from human tumours, even by explanting the cells in culture, were generally unsuccessful, but one typical SV40 strain was obtained from a melanoma and two others from diseased brain tissue. Serological surveys showed that earlier findings that up to 5% of the population had low titre SV40 antibody were mostly, if not entirely, due to cross reactions with the much commoner human polyoma viruses.

It was thus concluded that while SV40 involvement in human tumours could not be absolutely denied it must be very rare indeed.

GROUP 3 : The most recent group of publications has reported the use of molecular techniques to detect SV40 DNA in tumours. The results have been conflicting, some studies showing no positives
others a high proportion. Most workers have focussed on detection of the viral oncogene (T antigen) and/or its expression. Persistence of these sequences integrated into the host cell genome would be expected from experimental studies of polyomavirus induced tumours of other species. Unfortunately the SV40 sequences of interest are widely used as tools in molecular laboratories creating a very substantial risk of cross contamination when testing tumour samples. This casts doubt on these studies which has not yet been resolved. Another new avenue of research has revealed that the SV40 oncogene (Tag) acts through complexing with p53 and affects the pRb pathway of cell cycle control. Genetic mutations of these control elements makes the cell exquisitely sensitive to SV40 transformation. These mutations occur naturally in the population, and confer cancer susceptibility on individuals who often develop tumours of similar type to those associated with SV40. This may need to be taken into account in future epidemiological studies.
Polio Immunisation
Effect of substituting IPV for tOPV on immunity to poliovirus in Bangladeshi infants: An open-label randomized controlled trial
BACKGROUND: The Polio Endgame strategy includes phased withdrawal of oral poliovirus vaccines (OPV) coordinated with introduction of inactivated poliovirus vaccine (IPV) to ensure population immunity. The impact of IPV introduction into a primary OPV series of immunizations in a developing country is uncertain.

METHODS: Between May 2011 and November 2012, we enrolled 700 Bangladeshi infant-mother dyads from Dhaka slums into an open-label randomized controlled trial to test whether substituting an injected IPV dose for the standard Expanded Program on Immunization (EPI) fourth tOPV dose at infant age 39 weeks would reduce fecal shedding and enhance systemic immunity. The primary endpoint was mucosal immunity to poliovirus at age one year, measured by fecal excretion of any Sabin virus at five time points up to 25 days post-52 week tOPV challenge, analyzed by the intention to treat principle.

FINDINGS: We randomized 350 families to the tOPV and IPV vaccination arms. Neither study arm resulted in superior intestinal protection at 52 weeks measured by the prevalence of infants shedding any of three poliovirus serotypes, but the IPV dose induced significantly higher seroprevalence and seroconversion rates. This result was identical for poliovirus detection by cell culture or RT-qPCR. The non-significant estimated culture-based shedding risk difference was -3% favoring IPV, and the two vaccination schedules were inferred to be equivalent within a 95% confidence margin of -10% to +4%. Results for shedding analyses stratified by poliovirus type were similar.
Polio Immunisation
Field study of fecal excretion as a decision support tool in response to silent reintroduction of wild-type poliovirus 1 into Israel
BACKGROUND: Israel has used an inactivated polio vaccine (IPV)-only schedule since 2005 (95% coverage). Silent reintroduction of wild type poliovirus 1 (WPV1) into Israel in early 2013 was detected in Southern Israel via routine environmental surveillance without clinical cases.

OBJECTIVES: To estimate the rate of WPV1 excretion by age and residence and inform decision-making regarding supplemental immunization with OPV.

STUDY DESIGN: A convenience sample of Bedouin and Jewish residential areas in the epicenter of the incident, focusing on under 8 year-olds who not previously given OPV. Fecal samples were directly tested for WPV1 RNA using a novel qRT-PCR assay. Positive samples were confirmed by gold standard cell culture and subject to genotyping.

RESULTS: Overall, 2196 non-duplicate fecal samples were collected and analyzed. WPV1 was detected in 61 samples (2.8%), 55 of which (90.2%) were from Bedouins. WPV1 excretion rates were 5.4% among Bedouins and 0.6% among Jewish individuals. Respective age-specific rates among Bedouin and Jewish children were 4.9% and 0.2% for 0-2 years and 7.2% and 1.7% for 2-8 years. Molecular testing had 89.5% sensitivity (higher than culture) and 100% specificity.
Polio Immunisation
Rotary’s PolioPlus Program: Lessons Learned, Transition Planning, and Legacy
Hundreds of thousands of Rotary volunteers have provided support for polio eradication activities and continue to this day by making financial contributions to the Rotary PolioPlus program, participating in national immunization days, assisting with surveillance, working on local, national, and international advocacy programs for polio eradication, assisting at immunization posts and clinics, and mobilizing their communities for immunization activities (including poliovirus and other vaccines) and other health benefits. Rotary has contributed more than $1.61 billion for the global eradication of polio and has committed to provide an additional $35 million each year until 2018 (all dollar amounts represent US dollars). Its unwavering commitment to eradicate polio has been vital to the success of the program. Rotary is providing additional support for routine immunization and healthcare. When polio is finally gone, we will have the knowledge from the lessons learned with PolioPlus, such as the value of direct involvement by local Rotarians, the program for emergency funding, innovative tactics, and additional approaches for tackling other global issues, even those beyond public health. Rotary has already transitioned its grants program to include 6 areas of focus: disease prevention and treatment, water and sanitation, maternal and child health, basic education and literacy, economic and community development, and peace and conflict prevention/resolution. Funding for these grants in 2015–2016 was $71 million. The legacy of the polio program will be the complete eradication of poliovirus and the elimination of polio for all time.
Polio Immunisation
Patients with Primary Immunodeficiencies Are a Reservoir of Poliovirus and a Risk to Polio Eradication
Immunodeficiency-associated vaccine-derived polioviruses (iVDPVs) have been isolated from primary immunodeficiency (PID) patients exposed to oral poliovirus vaccine (OPV). Patients may excrete poliovirus strains for months or years; the excreted viruses are frequently highly divergent from the parental OPV and have been shown to be as neurovirulent as wild virus. Thus, these patients represent a potential reservoir for transmission of neurovirulent polioviruses in the post-eradication era. In support of WHO recommendations to better estimate the prevalence of poliovirus excreters among PIDs and characterize genetic evolution of these strains, 635 patients including 570 with primary antibody deficiencies and 65 combined immunodeficiencies were studied from 13 OPV-using countries. Two stool samples were collected over 4 days, tested for enterovirus, and the poliovirus positive samples were sequenced. Thirteen patients (2%) excreted polioviruses, most for less than 2 months following identification of infection. Five (0.8%) were classified as iVDPVs (only in combined immunodeficiencies and mostly poliovirus serotype 2). Non-polio enteroviruses were detected in 30 patients (4.7%). Patients with combined immunodeficiencies had increased risk of delayed poliovirus clearance compared to primary antibody deficiencies. Usually, iVDPV was detected in subjects with combined immunodeficiencies in a short period of time after OPV exposure, most for less than 6 months. Surveillance for poliovirus excretion among PID patients should be reinforced until polio eradication is certified and the use of OPV is stopped. Survival rates among PID patients are improving in lower and middle income countries, and iVDPV excreters are identified more frequently. Antivirals or enhanced immunotherapies presently in development represent the only potential means to manage the treatment of prolonged excreters and the risk they present to the polio endgame.
Polio Immunisation
Genetically Thermo-Stabilised, Immunogenic Poliovirus Empty Capsids; a Strategy for Non-replicating Vaccines
While wild type polio has been nearly eradicated there will be a need to continue immunisation programmes for some time because of the possibility of re-emergence and the existence of long term excreters of poliovirus. All vaccines in current use depend on growth of virus and most of the non-replicating (inactivated) vaccines involve wild type viruses known to cause poliomyelitis. The attenuated vaccine strains involved in the eradication programme have been used to develop new inactivated vaccines as production is thought safer. However it is known that the Sabin vaccine strains are genetically unstable and can revert to a virulent transmissible form. A possible solution to the need for virus growth would be to generate empty viral capsids by recombinant technology, but hitherto such particles are so unstable as to be unusable. We report here the genetic manipulation of the virus to generate stable empty capsids for all three serotypes. The particles are shown to be extremely stable and to generate high levels of protective antibodies in animal models.
Post-Polio Motor Unit
Vasomotor abnormalities as post-polio sequelae: functional and clinical implications
Persons who had poliomyelitis report cold and discolored extremities and decreased muscle strength when exposed to mildly cool ambient temperatures. Bilateral digital cutaneous blood flow, skin temperature and median nerve latencies and amplitudes were measured at 30 degrees C, 25 degrees C and 20 degrees C in five post-polio subjects and age-matched controls. Cutaneous blood flow was lowest on the more affected side in the post-polio subjects but vasoconstriction with decreasing Ta was equal bilaterally in both groups. With decreasing Ta, median motor nerve latencies became clinically abnormal and “giant” sensory nerve potential amplitudes were seen in a majority of the post-polio subjects. It is concluded that an impairment of sympathetic vasoconstrictor outflow in post-polios allows passive dilatation and engorgement of the cutaneous venous capacitance beds. This promotes uncontrolled heat loss and causes cooling of nerve and muscle that is responsible for the impairment of muscle functioning and the abnormal electrophysiological findings.
Post-Polio Motor Unit
Acute and latent effect of poliomyelitis on the motor unit as revealed by electromyography
When polio virus attacks the motor neuron it may be completely destroyed, damaged, or unaffected. Muscle fibers of a destroyed motor neuron are orphaned or reinnervated. Nearby functioning motor units will then send terminal axon sprouts to reinnervate the orphaned muscle fibers. If there are too many orphaned muscle fibers and not enough surviving motor units to reinnervate them, the orphaned muscle fibers will continue to fibrillate until they atrophy and die. The resultant effect of poliomyelitis upon the affected muscle is an overall loss of motor units with the remaining units innervating many more muscle fibers than they originally did. There appears to be a late effect of polio upon these larger reinnervated motor units. After approximately 20 to 30 years, impulse transmission to the muscle fibers of the large reinnervated motor unit begins to fail. These transmission difficulties increase with age and time from recovery. These late onset transmission abnormalities may be factors in patient complaints of fatigue and progressive weakness.
Post-Polio Motor Unit
A rat model of the post-polio motor unit
We examined the long-term effects of muscle usage on a rat model of the post-polio motor unit. Isometric tensions, type I and type II muscle fiber areas, the incidence of collateral sprouting, and motor endplate morphology were examined following 1, 3, 6, and 9 months of partial denervation in rat plantaris muscle. Full morphologic and functional stability of the expanded motor units occurred at 6 months post-partial denervation. Fiber hypertrophy was observed, possibly the result of compensatory work hypertrophy due to muscle overuse. Following 9 months of partial denervation and muscle overuse, the twitch and tetanic tensions and type I and type II muscle fiber areas were significantly reduced as compared to sham controls; angulated myofibers and group atrophy also were seen. The percent collateral sprouting, the number of terminal branches per endplate, and the endplate area were all increased, possibly a compensatory response to a decreased synthesis of neurotrophic factor(s) and/or transmitter-related components. These aging-like changes seem to occur earlier in chronically stressed, overenlarged, and overworked motor units.
Post-Polio Motor Unit
Late effects of polio: critical review of the literature on neuromuscular function
Many individuals who have had poliomyelitis are now complaining of several new problems attributed to their former illness including muscle atrophy; fatigue; progressive weakness; and muscle, back, and joint pain. This paper critically examines the literature regarding the neuromuscular effects of poliomyelitis. Weakness resulting from poliomyelitis was due to destruction of anterior horn cells. After the illness, muscle strength was partially recovered as a result of several physiologic adaptive mechanisms including terminal sprouting and reinnervation, myofiber hypertrophy, and, possibly, myofiber type transformation. Several pathophysiologic and functional etiologies have been proposed for late neuromuscular deterioration, but none has been proven. In fact, to date, there is no objective evidence documenting progressive loss of strength in polio survivors. Studies attempting to differentiate polio survivors with and without symptoms of deterioration have resulted in conflicting results; however, it appears reasonable to conclude that symptomatic postpolio subjects had a more severe illness with greater loss of neuromuscular function. Exercise may be helpful for many postpolio patients, but the prescription must be tailored to the individual to avoid problems of overuse or excessive fatigue.
Post-Polio Motor Unit
Late denervation in patients with antecedent paralytic poliomyelitis
The development of new weakness, fatigue, and pain decades after acute paralytic poliomyelitis is a recognized syndrome. We conducted a controlled study of this syndrome by analyzing clinical, electromyographic, and muscle-biopsy features in 18 patients with a history of poliomyelitis--13 reporting 1 to 20 years of new weakness and 5 without new symptoms. The patients with new weakness also reported new muscle atrophy (9 of 13) and fatigue (10 of 13), symptoms not reported by the controls. The age at the time of acute poliomyelitis, severity of poliomyelitis, residual disability, number of years since acute poliomyelitis, and age at the time of study were comparable in the weakening and control groups. Evidence of remote denervation consistent with antecedent poliomyelitis was demonstrated in all patients by electromyography or muscle biopsy or both. In addition, active denervation (as evidenced by spontaneous activity on conventional electromyography, increased jitter on single-fiber electromyography, or atrophic myofibers) was found in 12 patients in the weakening group and in all 5 controls. Immunohistochemical detection of myofibers expressing the neural-cell adhesion molecule corroborated ongoing denervation in both patient groups. When muscle data from both groups were pooled, correlations were observed between the extent of past reinnervation and the degree of ongoing motor-unit instability. We conclude that the extensive reinnervation of denervated muscle that occurs in paralytic poliomyelitis may be followed by late denervation of the previously reinnervated muscle fibers. Electromyographic and muscle-biopsy evidence of ongoing denervation does not distinguish between stable patients with prior paralytic poliomyelitis and those with new weakness.
Post-Polio Motor Unit
A long-term follow-up study of patients with post-poliomyelitis neuromuscular symptoms
A “post-polio” syndrome characterized by new neuromuscular symptoms, including muscle weakness, may develop years after recovery from acute paralytic poliomyelitis. We studied 27 patients (mean age, 50.6 years) in whom new muscle weakness developed a mean of 28.8 years after recovery from acute polio. We reevaluated these patients during a mean follow-up period of 8.2 years (range, 4.5 to 20) after they were originally studied at the National Institutes of Health. The total mean follow-up period after the onset of new weakness was 12.2 years (range, 6 to 29). The patients were assessed with quantitative muscle testing, muscle biopsy, electromyography, and virologic and immunologic examination of the cerebrospinal fluid. Muscle strength had declined in all patients. The rate of decline averaged 1 percent per year. The decrease was irregular, with subjective plateau periods that ranged from 1 to 10 years. None of the patients had amyotrophic lateral sclerosis. Oligoclonal bands (IgG) were found in the cerebrospinal fluid of 7 of 13 patients studied, but no specific elevation of antibodies to poliovirus was observed in the cerebrospinal fluid. The newly affected muscles that were evaluated longitudinally with follow-up muscle biopsies and electromyography showed signs of chronic and new denervation. Groups of atrophic muscle fibers (group atrophy) and "neurogenic jitter" were not present. New post-polio muscle weakness is not a life-threatening form of motor-neuron deterioration. It appears that this weakness is not due to a loss of whole motor neurons, as in amyotrophic lateral sclerosis, but that it is due to a dysfunction of the surviving motor neurons that causes a slow disintegration of the terminals of individual nerve axons.
Post-Polio Motor Unit
Electromyographic and morphological functional compensation in late poliomyelitis
Patients with prior poliomyelitis may experience muscle function deterioration decades after onset of disease. The present study is aimed at describing electromyographic and morphometric evidence of muscular compensation and of on-going muscular instability. Ten subjects 42-62 years of age with onset of polio 25-52 years earlier were studied with macro EMG, single-fiber EMG (SFEMG), muscle strength measurement, and morphometrical analysis of muscle biopsies from the vastus lateralis muscle. SFEMG revealed increased fiber density (FD) and large macro-MUP potentials indicating pronounced reinnervation as compensation to loss of motor neurons. From electrophysiological data of motor unit size, morphometric measures of fiber size, and muscle strength data, the minimal degree of motor neuron loss was estimated to be greater than 70%.
Post-Polio Motor Unit
Neurological manifestations of the post-polio syndrome
Patients with late effects of poliomyelitis, ie PPS, are being seen at an ever increasing frequency by general physicians, neurologists, and orthopedists. An appropriate time interval for the onset of late manifestations has elapsed since the major epidemics of poliomyelitis in the 1940s and 1950s. Post-polio neurological manifestations primarily include new weakness, atrophy, muscle pain, and fasciculations. Fortunately, the weakness is of a very slow, progressive nature. Abnormal laboratory studies include routine EMG, demonstrating chronic denervation; SFEMG, demonstrating increased fiber density, increased jitter, and blocking; and muscle biopsy most often revealing fiber-type grouping of chronic denervation and small isolated angular (or angulated) fibers and group atrophy in some series, both suggestive of active denervation. Unfortunately, both EMG and muscle biopsy studies suffer from a lack of specificity as they do not appear to distinguish asymptomatic from symptomatic (new weakness, PPMA) patients with prior poliomyelitis. Although the cause of PPMA is unknown, electrophysiological (SFEMG) and muscle biopsy studies suggest that the process involves a loss or dropout of axon terminals of reinnervated motor units. The axons terminal dropout could be due to dysfunction in the cell soma, the axon, or the terminals themselves. Whether motor neuron exhaustion, a persistent viral infection, or immune-mediated mechanisms play a role in the pathogenesis of the late weakness is unclear at present and will require further investigation. Treatment at this time is of a supportive nature. A major controversy involves the role of strengthening exercises in these patients since experimental animal studies suggest that excessive exercise of denervated muscles leads to increased weakness. Clearly, a better understanding of PPS and PPMA will allow more effective management of these patients’ problems and might also provide insight into other motor neuron and neuromuscular junction diseases.
Post-Polio Motor Unit
Functional recovery – a major risk factor for the development of postpoliomyelitis muscular atrophy
A retrospective study was undertaken to identify potential risk factors for the development of progressive postpoliomyelitis muscular atrophy (PPMA). Patients with PPMA (n = 57) were compared with patients with a history of poliomyelitis but without a history of progressive weakness (n = 49). Patients who later developed PPMA had histories of more widespread acute paralysis, but relatively greater functional recovery. They were less disabled, and reported higher recent activity levels. Seventy-nine percent of the total variance between the PPMA and control groups could be accounted for by recovery alone (ie, severity minus disability). Functional recovery is generally attributed to reinnervation of sarcomeres by collateral sprouting from surviving lower motor neurons. Since degree of recovery predicts the risk of developing PPMA, our findings suggest that enlarged motor units may carry an increased susceptibility for dysfunction and/or degeneration.
Post-Polio Motor Unit
Dynamic electromyography and muscle biopsy changes in a 4-year follow-up: study of patients with a history of polio
Eighteen patients who had had polio 29-56 years prior to the first investigation were studied on two occasions, 4 years apart. Isokinetic and isometric strength measurements and Macro EMG were performed in 28 legs. Muscle biopsy specimens were obtained on both occasions from 11 legs. On average the muscle strength was 56% of control values at the first examination, and decreased by another 8% during the observation period. The muscle fiber area was increased compared to that of controls and did not change significantly. Macro EMG, comprising muscle fiber area and number of muscle fibers, and/or single fiber EMG showed clear signs of reinnervation in all legs. The motor units at the first examination were increased 11-fold, on average, compared with age-matched control values. During the observation period, reinnervation continued and the size of motor units increased by another 56% as a result of ongoing denervation, that is, loss of neurons. This compensation was particularly pronounced in patients with stable conditions. The parameters studied did not reveal any definite pattern predicting future development of new muscle weakness in individual subjects.
Post-Polio Motor Unit
Loss of motor unit size and quadriceps strength over 10 years in post-polio syndrome
OBJECTIVE: To investigate whether strength decline in post-polio syndrome (PPS) results from excessive distal axonal degeneration of enlarged motor units.

METHODS: We assessed changes over 10 years in isometric quadriceps strength, mean motor unit action potential (MUAP) size, root mean squared (RMS) amplitude, and level of interference (LOI) in 47 patients with PPS and 12 healthy controls, using high density surface EMG. At baseline, all patients had symptomatic quadriceps dysfunction, evidenced by transmission defects on single-fibre EMG.

RESULTS: MU size and strength declined significantly by 20% and 15%, respectively in patients with PPS. Those with the largest initial MU sizes exhibited the greatest losses of mean MU size (27%) and proportional decreases in quadriceps strength (23%). Initial strength, change in LOI and change in RMS amplitude together explained 35% of the variability in strength changes in patients. MU size of controls did not change, although they lost 29% strength.

SIGNIFICANCE: This long term follow-up study provides evidence that size diminution of enlarged MUs combined with a reduced number of active MUs contributes to the gradual strength decline in PPS.
Post-Polio Motor Unit
Repeater F-waves are signs of motor unit pathology in polio survivors
INTRODUCTION: The purpose of this study was to determine whether F-waves reveal electrophysiological features of anterior horn cells in polio survivors.

METHODS: Forty-three polio survivors and 20 healthy controls underwent motor nerve conduction studies of the median and tibial nerves bilaterally, including sampling of F-waves elicited by 100 stimuli and the determination of motor unit number estimation (MUNE).

RESULTS: A significant increase in abnormally stereotyped ("repeater") F-waves and a reduction of F-wave persistence were observed in both nerves in the polio group as compared with the control group. Repeater F-waves had a negative correlation with MUNE.
Post-Polio Motor Unit
Polioencephalitis and the Brain Fatigue Generator Model of Post-Viral Fatigue Syndromes
Fatigue is the most commonly reported and most debilitating Post-Polio Sequelae (PPS) affecting millions of polio survivors world-wide. Post-polio fatigue is associated with: 1) subjective reports of difficulty with attention, cognition, word-finding and maintaining wakefulness; 2) clinically significant deficits on neuropsychological tests of information processing speed and attention; 3) gray and white matter hyperintensities in the reticular activating system on magnetic resonance imaging of the brain; 4) neuroendocrine evidence of impaired activation of the HPA axis. Many of these findings are identical to those documented following a variety of viral encephalitides, including acute poliovirus infection, lethargic encephalitis, Iceland Disease, myalgic encephalomyelitis, and, most recently, Chronic Fatigue Syndrome. The clinical, historic, neuropsychologic, neuroanatomic and physiologic parallels between poliovirus infection, post-polio fatigue and post-viral fatigue syndromes (PVFS) will be explored in an attempt to describe the pathophysiology of PVFS. The disinhibition of a putative Brain Fatigue Generator will be implicated as a cause of the subjective symptoms and objective signs that accompany PVFS. The results of a pilot placebo-controlled study of a dopamine 2 receptor agonist to treat post-polio fatigue will also be described.
Post-Polio Motor Unit
Correlation of Electrophysiology with Pathology, Pathogenesis, and Anticholinesterase Therapy in Post-Polio Syndrome
Post-Polio Motor Unit
Stimulation frequency-dependent neuromuscular junction transmission defects in patients with prior poliomyelitis
Generalized fatigue and muscle fatiguability are major symptoms of post-poliomyelitis syndrome (PPS), and may be due to neuromuscular junction transmission defects, as suggested by increased jitter on single fiber electromyography (SFEMG). To determine the etiology of this defect, we studied jitter at low (1, 5 Hz) and high (10, 15, 20 Hz) frequency stimulation with stimulation SFEMG in 17 post-polio patients with muscle fatiguability, and in 9 normal controls. In 5 of 17 PPS patients and in 1 of 9 controls, jitter was significantly higher (unpaired t-test, P < 0.05) at high frequency stimulation (HFS). In the remaining PPS patients and controls there was no significant difference in jitter at high and low stimulation frequencies. PPS patients with increased jitter at HFS had a significantly longer time interval since acute polio (mean 48.5 years) than PPS patients without increased jitter at HFS (mean 40 years; P < 0.05), but were not distinguished by other historical or clinical criteria. We conclude that the neuromuscular junction defect in post-polio patients is similar to that observed in amyotrophic lateral sclerosis, and is probably due to ineffective conduction along immature nerve sprouts and exhaustion of acetylcholine stores. The appearance of an increase in jitter with HFS in post-polio patients may be dependent upon time after acute polio.
Post-Polio Motor Unit
Electrophysiology and Electrodiagnosis of the Post-Polio Motor Unit
Post-poliomyelitis syndrome refers to new symptoms that may occur years after recovery from poliomyelitis. The most common of these symptoms are new weakness, fatigue, and pain. This article describes electrodiagnostic studies -- conventional electromyography (EMG), single fiber electromyography (SFEMG), and macroelectromyography (macro-EMG) -- that have provided information on the post-polio motor unit and on the possible etiology of some post-polio syndrome symptoms. Muscular fatigue, and indirectly, general fatigue, may be due to neuromuscular junction transmission defects in some post-polio individuals, as suggested by reduction of the compound motor action potentials on repetitive stimulation, and increased jitter and blocking on SFEMG. Progressive weakness and atrophy in post-polio syndrome is probably due to a distal degeneration of post-polio motor units with resultant irreversible muscle fiber denervation. Electrodiagnostic evidence of ongoing denervation includes fibrillation and fasciculation potentials on conventional EMG, increased jitter and blocking on SFEMG, and smaller macro-EMG amplitudes in newly weakened postpolio muscles. However, even though electrodiagnostic studies have provided insight into the possible causes of some postpolio syndrome symptoms, no specific electrodiagnostic test for the syndrome is currently available.
Post-Polio Motor Unit
Electrodiagnostic Findings in 108 Consecutive Patients Referred to a Post-Polio Clinic – The Value of Routine Electrodiagnostic Studies
Many patients with a history of polio develop new symptoms including weakness, pain, fatigue, and changes in function, or post-polio syndrome (PPS).[1] Before a diagnosis of PPS is made, other diagnoses must first be ruled out. Assessment must be done in a comprehensive and coordinated manner.[2] Therefore, as part of our routine evaluation, we do an electromyogram/nerve conduction study (EMG/NCS) on every patient. During examinations on our clinic patients we began to notice (1) electrodiagnostic evidence of polio in limbs not previously felt to be involved; (2) a normal EMG, or evidence of another disease; and (3) EMG evidence consistent with additional neurological lesions, including compression neuropathies, peripheral neuropathies, and radiculopathies. A prospective study using a routine, standardized four-extremity electrodiagnostic protocol was done to quantify the frequency of these occurrences.
Post-Polio Motor Unit
Muscle Function, Muscle Structure, and Electrophysiology in a Dynamic Perspective in Late Polio
The muscular impairment in patients with a history of polio varies from none to severe. The relationship between the degree of initial involvement and the effect of various compensatory mechanisms determines the clinical picture, which changes dynamically. Early and late recovery after poliomyelitis depend on a number of factors. Clinical improvement that appears within a few weeks after the acute phase is probably due to recovery in the excitability of functional, but not degenerated, motor neurons. Degeneration of neurons, causing peripheral denervation, is compensated by collateral sprouting, i.e., by nerve twigs branching off from surviving motor units overlapping with the denervated ones. This is most likely the main factor explaining recovery within the first 6-12 months. Another late compensatory process is the increase in size of the muscle fibers. As a result of these processes, normal muscle strength and presumably normal muscle volume can be seen despite a calculated loss exceeding 50% of the number of motor neurons.
Post-Polio Motor Unit
How long does denervation take in poliomyelitis? Or is it a lifetime?
Background and Objective: This study aims to determine the period of reinnervation in patients with poliomyelitis. This research was conducted to identify the appearance of denervation potentials in patients with poliomyelitis as indicators for reinnervation.

Materials and Methods: A total of 246 male patients with poliomyelitis were assessed electrophysiologically between 1988 and 2007. The mean age was 22.8 (18–42). It has been an average of 19.9 ± 4.9 years since the beginning of complaints from the patients.

Results: The patients had no complaints of newly developing muscle weakness, fatigue, muscle and joint pain, and difficulties in breathing and swallowing. Neurological examinations revealed the absence of myotomal pain and sensory loss. Upon assessment of the patients' limbs, the following findings were revealed: two patients had left upper and lower limb involvement, two patients had left upper and right lower limb involvement, 6 patients had left upper limb involvement, 12 patients had both lower limb involvement, 105 patients had left lower limb involvement, 1 patient had both upper limb involvement, 2 patients had right lower and upper limb involvement, 12 patients had right upper limb involvement, 6 patients had both lower limb involvement, 95 patients had right lower limb involvement, and 3 had all the three extremities affected. The needle electromyography revealed the presence of denervation potentials in 25.2% (62) of the patients.

Conclusion: When poliovirus attacks the motor neuron, this neuron may be completely destroyed, damaged, or unaffected. Reinnervation occurs when nearby functioning motor units send out terminal axon sprouts to reinnervate the damaged muscle fibers. As a consequence of poliomyelitis, several muscle fibers become atrophic and fibrotic, but others continue to survive. This study showed that patients with a history of poliomyelitis experienced denervation with subsequent reinnervation for many years.
Post-polio sequelae and the psychology of second disability
Thousands of persons who had poliomyelitis are reporting new physical symptoms that are eroding physical abilities, regained only after strenuous and lengthy rehabilitation, that were thought to have been permanently restored. These symptoms are causing persons to feel they are becoming disabled for a second time by the same disease. These new symptoms are psychologically traumatic also because they are unexpected, their cause is unknown and there is a lack of knowledge and understanding concerning them within the medical community. Society's negative and pejorative attitude toward the disabled is discussed to explain the psychological trauma associated with any first or second disability. Psychological processes that promote acceptance of disability are outlined with special emphasis on the post-polio experience. Post-polio support groups are described as one means to obtain the resources necessary to surmount the physical and psychological difficulties associated with post-polio sequelae.
The psychology of polio as prelude to post-polio sequelae: behavior modification and psychotherapy
Even as the physical causes and treatments for post-polio sequelae (PPS) are being identified, psychological symptoms – chronic stress, anxiety, depression, and compulsive, Type A behavior – are becoming evident in polio survivors. Importantly, these symptoms are not only causing marked distress but are preventing patients from making the lifestyle changes necessary to treat their PPS. Neither clinicians nor polio survivors have paid sufficient attention to the acute polio experience, its conditioning of life-long patterns of behavior, its relationship to the development of PPS, and its effect on the ability of individuals to cope with and treat their new symptoms. We describe the acute polio and post-polio experiences on the basis of patient histories, relate the experience of polio to the development of compulsive, Type A behavior, link these behaviors to the physical and psychological symptoms reported in the National Post-Polio Surveys, and present a multimodal behavioral approach to treatment.
Recognizing typical coping styles of polio survivors can improve re-rehabilitation – a commentary
This paper does not have an abstract
Coping with the late effects: differences between depressed and nondepressed polio survivors
This study examined differences between depressed and nondepressed individuals with a history of paralytic poliomyelitis in terms of demographics, health status and coping strategies. The prevalence of distress and depression in this group of 116 polio survivors was determined. Subjects completed the Brief Symptom Inventory, the Coping with Disability Inventory and a questionnaire concerning their polio histories and self-perceptions of health. Medical assessments were performed by physicians. Only 15.8% of the sample had scores indicating depression and elevated distress. Depressed/distressed polio survivors were more likely to: be living alone, be experiencing further health status deterioration, seek professional help, view their health as poor, report greater pain, be less satisfied with their occupational status and their lives in general and exhibit poorer coping outcome behaviors in relation to their disability. Three factors in coping with the late effects of polio were identified through a factor analysis of the Coping with Disability Inventory: positive self-acceptance, information seeking/sharing about the disability and social activism. Differences between depressed/distressed and other polio survivors were found across these three factors, with depressed/distressed subjects having significantly lower coping scores. These and other results are discussed.
Prevalence and associated features of depression and psychological distress in polio survivors
This cross-sectional study examines the prevalence of psychological distress and depression among 116 polio survivors. It investigates demographic, medical, and coping differences between subjects with (n = 17) and without (n = 99) these symptoms. Subjects were administered the Brief Symptom Inventory (BSI), the Coping with Disability Inventory (CDI), and a questionnaire about their polio histories. The BSI provided measures of psychological distress and depression that defined the subgroups. The CDI assessed coping behaviors. BSI scores for the overall sample were within the normal range indicating no major distress, depression, nor elevated somatic complaints. Several significant differences were found between the two subgroups. On average, depressed/distressed subjects reported an increase in pain (p < .01) and further deterioration of their medical status since the time of their physical best subsequent to the onset of polio (p < .01). They consistently rated their health as poorer than did nondepressed/nondistressed subjects (p < .001). They also reported less satisfaction with life and their occupational status (p < .001) and displayed poorer coping behaviors combined (p < .001). Selected variables such as life satisfaction, pain, decrease in activity, and current living situation accounted for 51% of the variance when predicting distress and depression among this group of polio survivors.
Living with the late effects of disability: a five-year follow-up survey of coping among post-polio survivors
A follow-up survey of 176 people aged 33–77 yrs with postpolio syndrome found that while they were experiencing significantly less anxiety, uncertainty, depression, and helplessness concerning their increasing disability than 5 yrs ago, their feelings of anger persisted. Relationships were found between coping styles, breadth of coping attempted, and emotions experienced. Specifically, a coping style of focusing on symptoms while attempting to maintain previous activity levels characterized people who felt more helpless, depressed, and angry. Those who coped by accommodating to their symptoms had adopted more strategies. Coping strategies involving lifestyle and personal changes were rated as more effective than many treatments and interpersonal strategies. Findings highlight the need for health practitioners to tap into clients' knowledge when designing and evaluating appropriate rehabilitation programs.
Psychological resilience and depressive symptoms in older adults diagnosed with post-polio syndrome
Depression is a serious comorbidity in people with disabilities; however, few studies have focused on depressive symptoms in older adults with post-polio syndrome (PPS). This study used a resilience conceptual framework that focused on patient psychosocial strengths to investigate the relationship between psychological resilience factors (e.g., acceptance, self-efficacy, personal resources, interpersonal relationships, self-rated health, spiritual growth, stress management) and depressive symptoms in a large sample (N = 630) of people older than 65 years who were diagnosed with PPS. Forty percent of the sample scored > or = 10 on the Center for Epidemiologic Studies Short Depression Scale (CES-D10), which is a higher percentage than what has been previously cited in other studies; however, 53% of the sample had good or excellent self-rated health, suggesting psychological resilience. Depression scores were regressed on seven selected resilience factors after controlling for functional limitations. Four of the seven variables accounted for 30% of the variance in depressive symptoms, with spiritual growth representing the main predictor (beta = -.26). The implications for rehabilitation nurses in developing a patient-strengths perspective in the assessment and counseling of older adults with PPS are discussed.
Long-standing poliomyelitis and psychological health
OBJECTIVE: To compare the psychological health of the individuals with long-standing poliomyelitis, with or without post-polio syndrome (PPS), to the general population and to identify the role of work as well as other variables with regard to their psychological health.

DESIGN: A cross-sectional study.

SUBJECTS: One hundred and ninety-five polio patients attending postpolio clinic in Jerusalem.

METHODS: Emotional distress (ED) was measured using the general health questionnaire (GHQ-12). Demographic, medical, social and functional data were recorded using a specific structured questionnaire. Each polio patient was compared to four age- and sex-matched controls.

RESULTS: ED was higher in the polio population as compared to the general population. Within the polio population ED was inversely correlated with work status. No correlation was found between ED and the functional level of polio participants and no difference was found in GHQ score between polio participants with or without post-polio. In addition, ED was less affected by subjective perception of physical health among polio patients as compared to the general population.
Illness narratives of persons with post-polio syndrome
This qualitative study investigated the lifetime illness experience of individuals with the 'late effects' of polio or post-polio syndrome. Fifteen individuals were interviewed twice about their illness experience and the interviews were transcribed verbatim. The empirical material first underwent a categorization process. The preliminary categories generated through this analysis were then condensed into broader categories which in the final analysis gave rise to the following temporal pattern or stages of the illness experience: (1) the acute phase of polio and subsequent treatment and care; (2) rehabilitation and care at institutions for the disabled; (3) adaptation to a new life; (4) living with the post-polio syndrome today, and finally, (5) memories of the past and apprehensions concerning the future. In spite of the difficult experiences of falling ill and slowly recovering from a life-threatening disease, these individuals have had a good life and accomplished most of their ambitions in the areas of work and family life. Their present psychosocial situation is complicated by the symptoms of the post-polio syndrome which make them more vulnerable to stress, but they are able to handle this burden except when any added strain makes it overwhelming. This potential vulnerability may sometimes express itself as a sudden flashback to traumatic polio experiences and it is therefore important that nurses are aware of the illness history of this patient group.
Postpoliomyelitis Syndrome: Assessment of Behavioral Features
Postpoliomyelitis syndrome (PPS) is an increasingly recognized phenomenon characterized by late-onset weakness, pain, and fatigue. Psychiatric and cognitive disturbances have been noted in postpoliomyelitis patients, but the relationship of these symptoms to PPS is unknown. We examined postpoliomyelitis patients with and without PPS using objective neuropsychological and neuropsychiatric procedures. Our results suggest that disturbances of mood were common and that subtle cognitive deficits also occured in postpoliomyelitis patients. However, patients with PPS did not have greater depression or cognitive deficits compared to postpoliomyelitis patients without PPS. Key Words: Postpoliomyelitis syndrome, Mood disturbances, Cognitive deficits.
Stress and "Type A" Behavior as Precipitants of Post-Polio Sequelae: The Felician/Columbia Survey
A behavioral profile has begun to emerge from studies of persons who survived acute poliomyelitis and are now experiencing post-polio sequelae. Persons who had polio have been shown to be employed full time at four times the rate of the general disabled population (1,2). Persons who had polio have more years of formal education on average than the general population (3), and marry at approximately the same rate as those who are not disabled (4). These data, combined with our own experience with thousands of persons who had polio, indicated that "polio survivors" are competent, hard-driving and time-conscious overachievers who demand perfection in all aspects of their personal, professional, and social lives. It appeared that those who survived polio exhibit "Type A" behavior and would therefore experience chronic emotional stress.

The notion that individuals who had polio exhibit "Type A" behavior and experience chronic stress was thought to be extremely important for an understanding of the pathophysiology and treatment of post-polio sequelae (PPS). Animal studies have demonstrated that stress accelerates the onset of muscle fatigue (5), augments age-related decreases in the terminal axon branch number (6), and accelerates age-related losses of neurons (7). Therefore, this study was designed to test 2 hypotheses: 1) persons who had poliomyelitis exhibit "Type A" behavior and symptoms pathognomonic of chronic stress, and 2) "Type A" behavior and stress precipitate or exacerbate PPS.
Quality of Life
The association between post-polio symptoms as measured by the Index of Post-Polio Sequelae and self-reported functional status
OBJECTIVE: To evaluate the association between self-reported severity of polio sequelae and current functional status among polio survivors.

METHODS: This was a cross sectional study of 195 polio survivors attending a polio outpatient clinic at a university hospital. The main outcome measures of demographic, medical, social, and functional data were gleaned from a questionnaire adapted for the polio population. The severity of polio sequelae was evaluated with the self-reported Index of Post-Polio Sequelae (IPPS).

RESULTS: The mean age of our sample was 57.6 ± 10.5 years, 53% were men, 38% had acquired higher education and 37% were employed. We found significant correlations between the total IPPS score and independence in activity of daily living (P<0.05), the use of walking aids (P<0.005) and mobility in and out-of-doors (P<0.0001). A positive correlation was also found between the total IPPS score and subjective assessment of physical and mental health (P<0.0001).
Quality of Life
Quality of life in Swedish patients with post-polio syndrome with a focus on age and sex
To investigate the health-related quality of life (QOL) in Swedish patients with post-polio syndrome (PPS), with a focus on sex and age. A total of 364 patients were recruited from five Swedish post-polio clinics. Analysis was carried out using SF-36 and data were compared with those of a normal population. QOL was significantly lower in PPS patients for all eight subdomains and the two main scores (physical compound score and mental compound score) when compared with the controls. Male patients had a significantly higher QOL than female patients for all subdomains and also for mental compound score and physical compound score, a phenomenon also observed in the normal population. There was a decrease in QOL in the physical domains and an increase in vitality with age. PPS decreases health-related QOL in both sexes, more in female patients. QOL for physical domains decreases whereas vitality increases with age in both sexes.
Quality of Life
Post-polio syndrome: impact of hope on quality of life
PURPOSE: To determine the effect of future-oriented coping strategies on the quality of life (QOL) of individuals with post-polio syndrome (PPS).

METHODS: A correlative study, in which a cohort of 61 patients was surveyed and a group of 40 healthy, age-matched individuals served as controls. Patients were surveyed as to their QOL, levels of hope and utilization of proactive coping, employment status and degree of functionality.

RESULTS: PPS patients had lower total, physical and mental QOL indices compared to controls. Future-oriented coping strategies associated with hope were positively associated with physical and mental QOL in the PPS group, but not in the controls. In a multivariate analysis, hope and employment status predicted higher QOL among those with PPS.
Quality of Life
Impact of post-polio-related fatigue on quality of life
OBJECTIVE: To assess the impact of post-polio-related fatigue on quality of life.

DESIGN: Cross-sectional case control study.

SUBJECTS: Patients without additional health problems that may induce fatigue were selected from among 82 polio survivors. Twenty-six patients with post-polio syndrome and 10 without post-polio syndrome were included. Control group consisted of 30 healthy volunteers.

METHODS: We assessed presence and severity of fatigue by Fatigue Severity Scale, quality of life by Nottingham Health Profile, and impact of fatigue on quality of life by Fatigue Impact Scale. Leg muscle strength was measured by manual muscle testing.

RESULTS: Strength of leg muscles showed no differences between the patients with and without post-polio syndrome. Patients with post-polio syndrome reported significantly higher levels of fatigue and reduced quality of life compared with both patients without post-polio syndrome and control group. Fatigue Impact Scale revealed that fatigue did not significantly impair mental health, but had a negative impact especially on physical and psychosocial functioning of the patients with post-polio syndrome.
Quality of Life
Health related quality of life in Turkish polio survivors: impact of post-polio on the health related quality of life in terms of functional status, severity of pain, fatigue, and social, and emotional functioning
OBJECTIVE: To determine the impact of postpolio-syndrome on quality of life in polio survivors.

METHODS: Forty polio survivors were included in the study. Twenty-one patients fulfilling the Halstead's postpolio-syndrome criteria participated in postpolio-syndrome group. The remaining nineteen patients formed non-postpolio-syndrome group. Control group was composed of forty healthy subjects. Quality of life was evaluated by Nottingham Health Profile, depression by Beck Depression Scale and fatigue by Fatigue Symptom Inventory. Isometric muscle strength was measured by manual muscle testing.

RESULTS: Total manual muscle testing score was 26.19 ± 13.24 (median: 29) in postpolio-syndrome group and 30.08 ± 8.9 (median: 32) in non-postpolio-syndrome group. Total manual muscle testing scores of non-postpolio-syndrome group were significantly higher than that of postpolio-syndrome group. Patients with postpolio-syndrome reported significantly higher levels of fatigue and reduced quality of life in terms of physical mobility, pain and energy when compared with patients without postpolio-syndrome and control group. It was not reported a statistically significant difference in social and emotional functioning and sleep quality between postpolio-syndrome, non-postpolio-syndrome and control groups. Also it was not found any statistically significant difference in Beck Depression Scale scores among the groups.
Quality of Life
Surviving polio in a post-polio world
Excitement mounts as the global health and international development communities anticipate a polio-free world. Despite substantial political and logistical hurdles, only 223 cases of wild poliovirus in three countries were reported in 2012. Down 99% from the estimated 350,000 annual cases in 125 countries in 1988—this decline signals the imminent global eradication of polio.

However, elimination of new polio cases should not also signal an end to worldwide engagement with polio. As many as 20 million continue to live with the disabling consequences of the disease. In developed countries where polio immunization became universal after dissemination of the polio vaccine in the 1950s, almost all individuals who have had polio are now above age 50. But in many developing countries where polio vaccination campaigns reached large segments of the population only after 1988, millions disabled by polio are still children or young adults. Demographically, this group is also different. After three decades of immunization efforts, those children unvaccinated in the late 1980s were more likely to be from poorer rural and slum communities and to be girls—groups not only harder to reach than more affluent members of the population but also individuals who, if they contract polio, are less likely to have access to medical and rehabilitation programs or education, job training, employment and social support services.

The commitment to eradicate polio should not be considered complete while those living with the disabling sequelae of polio continue to live in poor health, poverty and social isolation. This paper reviews what is currently known about disabled survivors of polio and highlights areas of need in public health research, policy and programming. Based on a literature review, discussion and field observations, we identify continuing challenges posed by polio and argue that the attention, funding and commitment now being directed towards eradication be shifted to provide for the rehabilitative, medical, educational and social needs of those for whom the disabling sequelae of polio will remain a daily challenge for decades to come.
Quality of Life
Polio survivors' perceptions of the meaning of quality of life and strategies used to promote participation in everyday activities
INTRODUCTION: The term ‘post-polio syndrome’ (PPS) is used to describe new and late manifestations of poliomyelitis that occur later in life. Research in this area has focused upon health status rather than its effect on quality of life.

AIM: To gain an in-depth understanding of the meaning of quality of life for polio survivors and to determine the type of strategies that are used by people with PPS and the support that they consider as important to facilitate participation in everyday life activities that have an impact on their quality of life.

METHOD: Six focus groups were conducted with 51 participants from two regions in England. Data were audio-taped and analysed using thematic analysis.

RESULTS: Our research found that polio survivors used terms used to describe quality of life which could be associated with that of happiness. Our research has identified resolvable factors that influence quality of life namely inaccessible environments, attitudes of health-care professionals and societal attitudes. Polio survivors have tried alternative therapies, chiefly acupuncture and massage, and found them to be effective in enhancing their quality of life.
Quality of Life
Factors associated with reduced quality of life in polio survivors in Korea
The purpose of this study is to assess health-related quality of life in polio survivors (PS) compared with that in the general population in Korea. Polio survivors (n = 120) from outpatient clinics at two hospitals, healthy controls (HC, n = 121) and members of the general population with activity limitations (AL, n = 121) recruited through a proportional-allocation, systematic sampling strategy from the Fourth Korean National Health and Nutrition Examination Survey were surveyed with self-rated health-related quality of life (Euro QoL five-dimensions). The proportion of participants who reported problems in mobility, usual activity, and symptoms of anxiety/depression were higher in the PS group compared with the HC and AL groups. There was no significant difference in the self-care dimension across the groups. Polio-specific questionnaire, pain, depression, fatigue, Modified Barthel Index (K-MBI) and Short Physical Performance Battery (SPPB) were assessed in the PS group. Those with post-poliomyelitis syndrome had greater problems in mobility, usual activity, and depression/anxiety. Polio survivors, especially those with more pain and fatigue symptoms, and those who did not have access to medical services had poorer health-related quality of life. These findings afford useful information for potential intervention improving quality of life in polio survivors.
Quality of Life
The influence of post-polio syndrome on independence and life satisfaction
PURPOSE: The aim of the study was to find out the influence of the new symptoms on life satisfaction and independent living and the most frequent disabilities in patients with post-polio syndrome that are affecting the satisfaction and independence.

METHOD: A questionnaire was sent to all the post-polio survivors (207) who visited the Rehabilitation Institute in Ljubljana at least once in the last ten years. We got 100 answers, which were analysed by SPSS (statistical package for social sciences).

RESULTS: Sixty nine reported that they had new symptoms that may be classified as post-polio syndrome.
Quality of Life
Survey of the Late Effects of Polio in Lothian
The Edinburgh Branch of the British Polio Fellowship (BPF) expressed the concern of members that the medical and related professions often appeared unfamiliar with the late consequences of polio and that services were not meeting their needs. In an attempt to determine the number of people affected and the nature of their experience, this postal survey was done in 1998.

A set of questionnaires were sent to 221 people, in Edinburgh and the Lothians, who had been identified as suffering polio in the past from those known to the BPF and hospital records. There were 125 replies which constituted the study population: 60% were female; the median age was 59 years; and the median time since original diagnosis was 51 years.

Common symptoms were: cold intolerance (70%); fatigue (66%); increased weakness in previously affected muscles (67%); new weakness in unaffected muscles (60%); muscle pain (64%); joint pain (61%); and joint stiffness (64%). These symptoms were often of recent onset. Other complaints included sleep disturbance (63%); exertional breathlessness (56%); headaches (33%); and swallowing difficulty (18%).

In terms of disability: 78% had difficulty with stairs; 72% with walking and 70% with bending. This was reflected in over a third reporting difficulty getting outdoors without help. Motor problems affecting upper limbs were present in over half the subjects. Daily activities were restricted because of severe pain in 38%. Over half (55%) the study population described difficulty living alone. The major factors restricting the lives of the subjects were physical problems, lack of energy and pain.

During the preceding 5 years, contact with health services were: hospital clinic 62%; physiotherapy 46%; occupational therapy 29%; speech therapy 2%; orthotics 40%; and wheelchair service 42%. The majority described satisfaction with these services but further study is warranted.

A conservative estimate is that there are about 300 people in Edinburgh and the Lothians with the range of difficulties demonstrated in this study and we suggest that special provision is made for them.
Renal Complications
Renal failure in a patient with postpolio syndrome and a normal creatinine level
Patients with renal failure who are taking trimethoprim have an increased risk of developing hyperkalemia, which can cause muscle weakness. In patients with postpolio syndrome, a normal creatinine level could be abnormally high, renal failure is possible because of lack of creatinine production, and the muscle weakness from resultant hyperkalemia could be more severe because of their underlying condition. This abnormally high creatinine level has been termed from this point relative renal failure. The objective of the study was to review a case in which relative renal failure and hyperkalemia caused muscle weakness that manifested as shortness of breath and confusion with electrocardiographic changes. A dehydrated patient with relative renal failure and postpolio syndrome had taken trimethoprim-sulfamethoxazole that caused symptomatic hyperkalemia. The patient presented with muscle weakness, shortness of breath, and confusion, with her postpolio syndrome compounding the situation and likely making the muscle weakness more severe. A patient on trimethoprim with renal failure is at an increased risk of developing hyperkalemia. Patients with postpolio syndrome could have severe muscle weakness from the hyperkalemia and could have renal failure even with a normal creatinine level. This case report will remind treating physicians to evaluate such patients for hyperkalemia if they present with muscle weakness, especially if the patient has renal failure and is on trimethoprim.
Respiratory Complications and Management
Poliomyelitis: late respiratory complications and management
One hundred forty-six respiratory polios have been reviewed for complications and current respiratory aids. One hundred thirty-eight of these people required respirator assistance at the onset of their poliomyelitis infection. Fifteen patients have kyphoscoliosis. Seventy-five percent of the total patients require some assisted ventilation. Fifty-two percent have tracheostomies. Most of these people have been followed at Rancho Los Amigos Medical Center for many years. The literature regarding late respiratory complications of polio is reviewed.
Respiratory Complications and Management
Lung function in post-poliomyelitis syndrome: a cross-sectional study
OBJECTIVE: To compare lung function between patients with post-poliomyelitis syndrome and those with sequelae of paralytic poliomyelitis (without any signs or symptoms of post-poliomyelitis syndrome), as well as between patients with post-poliomyelitis syndrome and healthy controls.

METHODS: Twenty-nine male participants were assigned to one of three groups: control; poliomyelitis (comprising patients who had had paralytic poliomyelitis but had not developed post-poliomyelitis syndrome); and post-poliomyelitis syndrome. Volunteers underwent lung function measurements (spirometry and respiratory muscle strength assessment).

RESULTS: The results of the spirometric assessment revealed no significant differences among the groups except for an approximately 27% lower mean maximal voluntary ventilation in the post-poliomyelitis syndrome group when compared with the control group (p = 0.0127). Nevertheless, the maximal voluntary ventilation values for the post-poliomyelitis group were compared with those for the Brazilian population and were found to be normal. No significant differences were observed in respiratory muscle strength among the groups.
Respiratory Complications and Management
Sleep-disordered breathing in neuromuscular disease
Sleep-disordered breathing in neuromuscular diseases is due to an exaggerated reduction in lung volumes during supine sleep, a compromised physiologic adaptation to sleep, and specific features of the diseases that may promote upper airway collapse or heart failure. The normal decrease in the rib cage contribution to the tidal volume during phasic REM sleep becomes a critical vulnerability, resulting in saw-tooth oxygen desaturation possibly representing the earliest manifestation of respiratory muscle weakness. Hypoventilation can occur in REM sleep and progress into non-REM sleep, with continuous desaturation and hypercarbia. Specific characteristics of neuromuscular disorders, such as pharyngeal neuropathy or weakness, macroglossia, bulbar manifestations, or low lung volumes, predispose patients to the development of obstructive events. Central sleep-disordered breathing can occur with associated cardiomyopathy (e.g., dystrophies) or from instability in the control of breathing due to diaphragm weakness. Mitigating factors such as recruitment of accessory respiratory muscles, reduction in REM sleep, and loss of normal REM atonia in some individuals may partially protect against sleep-disordered breathing. Noninvasive ventilation, a standard-of-care management option for sleep-disordered breathing, can itself trigger specific sleep-disordered breathing events including air leaks, patient-ventilator asynchrony, central sleep apnea, and glottic closure. These events increase arousals, reduce adherence, and impair sleep architecture. Polysomnography plays an important role in addressing pitfalls in the diagnosis of sleep-disordered breathing in neuromuscular diseases, identifying sleep-disordered breathing triggered by noninvasive ventilation, and optimizing noninvasive ventilation settings.
Respiratory Complications and Management
Feasibility of lung volume recruitment in early neuromuscular weakness: a comparison between amyotrophic lateral sclerosis, myotonic dystrophy, and postpolio syndrome
BACKGROUND: Lung volume recruitment (LVR) is a cough assistance technique used in persons with neuromuscular disorders (NMDs), most typically in those requiring noninvasive ventilation (NIV). Whether it may be useful in persons with NMDs who have milder respiratory impairment is unknown.

OBJECTIVE: To assess the feasibility, impact on quality of life (QOL), and preliminary physiological effects of daily LVR in different categories of persons with NMDs who have an early stage of respiratory impairment.

DESIGN: Feasibility study.

SETTING: Academic tertiary care center.

PARTICIPANTS: Outpatients diagnosed with amyotrophic lateral sclerosis (n = 8), postpolio syndrome (n = 10), and myotonic dystrophy (n = 6) who had restrictive respiratory defects but were not yet using NIV.

METHODS: Participants were asked to perform LVR up to 4 times daily and log their LVR use in a diary. Physiological measurements and questionnaires were completed at baseline and after 3 months.

MAIN OUTCOME MEASUREMENTS: Compliance with LVR use was assessed, along with QOL and willingness to continue the treatment. Physiological measurements included forced vital capacity (FVC), lung insufflation capacity (LIC), and the LIC minus FVC difference.

RESULTS: Of the 24 recruited subjects, 7 with amyotrophic lateral sclerosis, 7 with postpolio syndrome, and 5 with myotonic dystrophy completed the study (n = 19). At baseline, mean values for FVC and spontaneous peak cough flow were 59.9% predicted and 373.1 L/min, respectively. For subjects completing the study, 74% were willing to continue long-term LVR use, and QOL scores were not adversely affected by LVR in any NMD subgroup. The LIC-FVC difference increased from baseline to follow-up by a mean of 0.243 L (P = .006) in all subjects (n = 19), suggesting a possible improvement in respiratory system mechanics.
Respiratory Complications and Management
1246: Missed chronic respiratory failure in a post-polio syndrome patient
This is a brief Case Report which does not have an abstract. This is an extract:

INTRODUCTION: Prior to the introduction of the polio vaccine, paralytic poliomyelitis was a major cause of morbidity and death. Twenty-five to fifty percent of the survivors are known to develop post-polio syndrome. Symptoms include fatigue, insidious respiratory failure, obstructive sleep apnea, bulbar neuropathy, central ventilatory abnormalities, hemi-diaphragmatic paralysis and progressive functional decline with new onset weakness, among others. We present a case of post-polio syndrome presenting with hypercapnic respiratory failure.
Respiratory Complications and Management
Pulmonary dysfunction and its management in post-polio patients
Respiratory dysfunction is extremely common and entails considerable risk of morbidity and mortality for individuals with past poliomyelitis. Although it is usually primarily due to respiratory muscle weakness, post-poliomyelitis individuals also have a high incidence of scoliosis, obesity, sleep disordered breathing, and bulbar muscle dysfunction. Although these factors can result in chronic alveolar hypoventilation (CAH) and frequent pulmonary complications and hospitalizations, CAH is usually not recognized until acute respiratory failure complicates an otherwise benign upper respiratory tract infection. The use of non-invasive inspiratory and expiratory muscle aids, however, can decrease the risk of acute respiratory failure, hospitalizations for respiratory complications, and need to resort to tracheal intubation. Timely introduction of non-invasive intermittent positive pressure ventilation (IPPV), manually assisted coughing, and mechanical insufflation-exsufflation (MI-E) and non-invasive blood gas monitoring which can most often be performed in the home setting, are the principle interventions for avoiding complications and maintaining optimal quality of life.
Respiratory Complications and Management
Pulmonary Dysfunction and Sleep Disordered Breathing as Post-Polio Sequelae: Evaluation and Management
Post-polio sequelae can include sleep disordered breathing and chronic alveolar hypoventilation (CAH). Both conditions develop insidiously and can render the post-polio survivor susceptible to cardiopulmonary morbidity and mortality when not treated in a timely and appropriate manner. These conditions can be diagnosed by a combination of spirometry, noninvasive blood gas monitoring, and ambulatory polysomnography. Sleep disordered breathing is most frequently managed by nasal continuous positive airway pressure, while tracheostomy ventilation is the most common treatment for ventilatory failure. We report the more effective and comfortable techniques recently made available for managing sleep disordered breathing and the use of noninvasive treatment options for CAH, respiratory failure, and impaired airway clearance mechanisms. One hundred forty-three cases are reviewed.
Respiratory Complications and Management
Airway Secretion Clearance by Mechanical Exsufflation for Post-Poliomyelitis Ventilator-Assisted Individuals
Pulmonary complications from impaired airway secretion clearance mechanisms are major causes of morbidity and mortality for post-poliomyelitis individuals. The purpose of this study was to review the long-term use of manually assisted coughing and mechanical insufflation-exsufflation (MI-E) by post-poliomyelitis ventilator-assisted individuals (PVAIs) and to compare the peak cough expiratory flows (PCEF) created during unassisted and assisted coughing. Twenty-four PVAIs who have used noninvasive methods of ventilatory support for an average of 27 years, relied on methods of manually assisted coughing and/or MI-E without complications during intercurrent respiratory tract infections (RTIs). Nine of the 24 individuals were studied for PCEF. They had a mean forced vital capacity (FVC) of 0.54 ± 0.47L and a mean maximum insufflation capacity achieved by air stacking of ventilator insufflations and glossopharyngeal breathing of 1.7L. The PCEF were as follows: unassisted, 1.78 ± 1.16L/sec; following a maximum assisted insufflation, 3.75 ± 0.73L/sec; with manual assistance by abdominal compression following a maximum assisted insufflation, 4.64 ± 1.42L/sec; and with MI-E, 6.97 ± 0.89L/sec. We conclude that manually assisted coughing and MI-E are effective and safe methods of airway secretion clearance for PVAIs with impaired expiratory muscle function who would otherwise be managed by endotracheal suctioning. Severely decreased maximum insufflation capacity but not vital capacity indicate need for a tracheostomy.
Restless Legs Syndrome
Restless legs syndrome and post polio syndrome: a case−control study
BACKGROUND AND PURPOSE: The aim was to investigate the prevalence of restless legs syndrome (RLS), fatigue and daytime sleepiness in a large cohort of patients affected by post polio syndrome (PPS) and their impact on patient health-related quality of life (HRQoL) compared with healthy subjects.

METHODS: PPS patients were evaluated by means of the Stanford Sleepiness Scale and the Fatigue Severity Scale (FSS). The Short Form Health Survey (SF-36) questionnaire was utilized to assess HRQoL in PPS. RLS was diagnosed when standard criteria were met. Age and sex matched healthy controls were recruited amongst spouses or friends of PPS subjects.

RESULTS: A total of 66 PPS patients and 80 healthy controls were enrolled in the study. A significantly higher prevalence of RLS (P < 0.0005; odds ratio 21.5; 95% confidence interval 8.17–57) was found in PPS patients (PPS/RLS+ 63.6%) than in healthy controls (7.5%). The FSS score was higher in PPS/RLS+ than in PPS/RLS− patients (P = 0.03). A significant decrease of SF-36 scores, including the physical function (P = 0.001), physical role (P = 0.0001) and bodily pain (P = 0.03) domains, was found in PPS/RLS+ versus PPS/RLS− patients. Finally, it was found that PPS/RLS+ showed a significant correlation between International Restless Legs Scale score and FSS (P < 0.0001), as well as between International Restless Legs Scale score and most of the SF-36 items (physical role P = 0.0018, general health P = 0.0009, vitality P = 0.0022, social functioning P = 0.002, role emotional P = 0.0019, and mental health P = 0.0003).
Restless Legs Syndrome
Restless legs syndrome in post-polio syndrome: a series of 10 patients with demographic, clinical and laboratorial findings
BACKGROUND: Few studies have described the occurrence of restless legs syndrome in post-polio syndrome.

METHODS: We studied 10 consecutive patients with post-polio syndrome and symptoms of restless legs syndrome. We look at demographic, clinical and laboratorial data.

RESULTS: A remarkable finding was the concomitant onset of symptoms of both diseases, suggesting a possible underlying mechanism. Severity of restless legs symptoms was moderate to very severe.
Restless Legs Syndrome
Sleep disorders frequency in post-polio syndrome patients caused by periodic limb movements
Post-polio syndrome (PPS) in individuals with polio longer than 15 years is characterized by weakness and/or muscle fatigue, deficit of deglutition and breath and periodic limb movements (PLM) during sleep. We undertook a review of 99 patients with PPS, and assessed the frequency of PLM through polysomnographic recordings at our sleep disorders unit. The total number of PLM, total time of sleep (TTS), efficiency of sleep (EfS), awaking index (AI) and apnea-hypopnea index (AHI) were analyzed. Sixteen patients presented PLM in excess of 5 for the entire night. When comparing these with the group without PLM, a correlation was found (p=0.001). Significant difference was found for the correlation of the parameters: IAH, ID, TTS and EfS when compared the two groups. There is a close relationship between PPS and PLM.
Restless Legs Syndrome
Restless legs syndrome in patients with sequelae of poliomyelitis
BACKGROUND: No studies have examined the association between RLS and the sequelae of poliomyelitis (PM). We studied the frequency and severity of RLS in a group of consecutive patients with the sequelae of poliomyelitis (PM) and the effect of treatment with dopaminergic drugs.

METHODS: A diagnosis of RLS was made according to the criteria of the International RLS Study Group, and severity was assessed by the RLS rating scale. Information on sex, age, age at onset, site affected by PM, disease duration of PM, and history of post-polio syndrome (pPS) was obtained in a cohort of 52 PM patients.

RESULT: The mean age was 55.9 ± 6.5 years; 39 patients had post-polio syndrome (75%). RLS was diagnosed in 21 (40.4%) patients. Sixteen of the 21 patients (76.2%) with RLS had pPS, which was similar to the non-RLS group (74.2% patients with pPS). RLS symptoms were very severe in 5 patients, severe in 13, moderate in 2 and mild in 1. Nineteen of the 21 patients with RLS had symptoms predominantly in the more affected lower limb (90% of patients). Sixteen patients received dopaminergic agonist treatment with a significant reduction in their scores on the RLS severity scale from 28.3 ± 4.7 to 6.9 ± 7.3 (p < 0.001).

DISCUSSION: RLS occurs frequently in patients with PM, both in those with and without pPS, and responds well to treatment with dopaminergic drugs.
Restless Legs Syndrome, Sleep Analysis
Sleep disorders frequency in post-polio syndrome patients caused by periodic limb movements
Post-polio syndrome (PPS) in individuals with polio longer than 15 years is characterized by weakness and/or muscle fatigue, deficit of deglutition and breath and periodic limb movements (PLM) during sleep. We undertook a review of 99 patients with PPS, and assessed the frequency of PLM through polysomnographic recordings at our sleep disorders unit. The total number of PLM, total time of sleep (TTS), efficiency of sleep (EfS), awaking index (AI) and apnea-hypopnea index (AHI) were analyzed. Sixteen patients presented PLM in excess of 5 for the entire night. When comparing these with the group without PLM, a correlation was found (p=0.001). Significant difference was found for the correlation of the parameters: IAH, ID, TTS and EfS when compared the two groups. There is a close relationship between PPS and PLM.
Transcranial direct current stimulation (tDCS) for sleep disturbances and fatigue in patients with post-polio syndrome
PURPOSE: Post-polio syndrome develops about 20-40 years after acute paralytic poliomyelitis, and manifests with progressively deteriorating muscle strength and endurance. Here, we assessed whether transcranial direct current stimulation (tDCS) improves sleep and fatigue symptoms in patients with post-polio syndrome.

METHODS: We enrolled 32 patients with a diagnosis of post-polio syndrome. tDCS (1.5 mA, 15 min) was delivered by a direct current stimulator connected to three electrodes: two anodal electrodes on the scalp over the right and left pre-motor cortex and the other above the left shoulder (cathode). 16 patients received anodal tDCS and the remainder sham tDCS. We evaluated changes induced by tDCS (daily for five days a week, for three weeks) on clinical scales (Short Form Health Survey [SF-36], Piper Fatigue Scale [PFS], Fatigue Severity Scale [FSS], 101-Point Numerical Rating [PNR-101], Hamilton Rating Scale for Depression [HRSD], Pittsburgh Sleep Quality Index [PSQI]) at baseline (T0) and three weeks later (T1).

RESULTS: At T1 SF-36 sub-items physical functioning, role physical, vitality, social functioning and role emotional improved significantly more in patients who received tDCS (p < 0.01) than in sham-treated patients. Also, PSQI scores improved more in treated patients (p < 0.05, two-way ANOVA with "stimulation" and "time" as factors: p < 0.01). tDCS-induced benefits were more pronounced in patients who were younger at primary infection (p < 0.05).
Sleep Analysis
Analysis of sleep characteristics in post-polio syndrome patients
The main post-polio syndrome (PPS) symptoms are new weakness, new atrophy, fatigue, pain and sleep disturbances. Polysomnography is the gold standard for sleep analysis.

OBJECTIVE: To analyze sleep patterns in PPS patients.

METHOD: Sixty patients (mean age 46.8+/-11.3 years) at the Federal University of São Paulo (UNIFESP/EPM) complaining of sleep disturbances were evaluated by means of polysomnography, performed at the Sleep Institute.

RESULTS: Sleep efficiency was lower due to high sleep latency and arousal index. The apnea and hypopnea index (AHI) and the periodic limb movements (PLM) index were higher. Sleep architecture was also impaired. There were no abnormalities of oxygen saturation, carbon dioxide levels, respiratory rate or heart rate.
Speech Pathology
Incidence and nature of dysphagia in polio survivors
Questionnaires pertaining to swallowing function were mailed to 220 members of postpolio support groups in Connecticut. Of the 109 responses, 80 individuals reported having no difficulty with swallowing, while 29 reported having either intermittent or consistent swallowing problems. Twenty-one of the 29 were seen for videofluoroscopic swallowing studies and pulmonary function testing. The swallowing studies showed that 43% of these individuals had difficulty with bolus control, 19% with delayed swallow response, and 81% with decreased pharyngeal transit. Although none of these individuals were observed to aspirate, two were judged to be at significant risk. Incidence of dysphagia within the group of polio survivors was estimated to be approximately 18%. Seventeen of the 20 postpolio subjects with dysphagia also demonstrated decreased breathing capacity. Although moderately to severely depressed values in the pulmonary function measures accompanied moderate dysphagia in certain postpolio individuals, reduced values in these same measures were also present in individuals with minimal swallowing dysfunction. Therefore, although impaired breathing may complicate swallowing dysfunction and vice versa, it does not appear that one can be predicated from the other. Management of dysphagia in postpolio individuals is discussed.
Speech Pathology
Evaluation, treatment, and follow-up results of post polio patients with dysphagia
Twenty consecutive patients were evaluated for reports of dysphagia from post-polio clinics. Only half the patients reported a history of swallowing problems at the time of their acute poliomyelitis. Each patient received a videofluorographic evaluation of the oral and pharyngeal phases of swallowing, and then was provided with recommendations to improve swallowing skills. A follow-up questionnaire was sent to all patients. The respondents had an average interval of 12 months since the initial evaluation. Of the 18 patients responding to the questionnaire, 14 (77%) reported regular use of the swallowing suggestions. Comparison of pre-evaluation results to followup of the 18 respondents yielded a statistically significant decline in the frequency of choking (p = 0.0156) and food sticking in the throat (p = 0.0195). We conclude that a dysphagia program can result in significant improvement of the swallowing symptoms reported with the post-polio population.
Speech Pathology
Myasthenia gravis masquerading as post-poliomyelitis syndrome
A 79-year-old man with previous bulbar poliomyelitis developed dysphagia and was diagnosed as having post-polio syndrome. Over 2 years, his swallowing deteriorated and he suffered an aspiration pneumonia. Only after the subsequent development of fatigue and facial weakness was myasthenia gravis diagnosed. Diagnostic criteria for post-polio syndrome include the exclusion of all other neurological conditions such as myasthenia gravis. Moreover, in any instance where a patient develops new symptoms, it is advisable to reconsider the original diagnosis.
Speech Pathology
Otolaryngology manifestation of postpolio syndrome
Twenty-one patients with postpolio syndrome were surveyed to determine otolaryngologic symptoms. An alteration in voice, dysphagia, and fatigue were the most common symptoms reported. Prevailing etiologic theories are presented, and treatment recommendations are offered.
Speech Pathology
Dysphagia and dysphonia among persons with post-polio syndrome – a challenge in neurorehabilitation
OBJECTIVE: To study the occurrence of dysphagia and dysphonia in persons with post-polio syndrome admitted into the centre for neurological rehabilitation in Finland.

MATERIALS AND METHODS:  Fifty-one persons with post-polio syndrome who were rehabilitated at Käpylä Rehabilitation Centre, Helsinki, Finland, in 2003–2004 were interviewed on problems with swallowing and voice production. Pulmonary function testing and grip strength measurement were performed. A clinical assessment of oral motor and laryngeal functions was carried out for those who reported daily problems with voice production or swallowing.

RESULTS: Fifteen persons (29.4%) reported daily problems with swallowing or voice production. In the clinical assessment, the most commonly observed deficits in swallowing included decreased pharyngeal transit (n = 13) and the food catching in the throat (n = 4). The disturbance of co-ordination of breathing and voice production was seen in 12 persons. There were no significant differences in any of the potential predictors between the groups.
Speech Pathology
Dysphonia as the initial presenting symptom in postpolio syndrome: a case report
Post-polio syndrome is a slowly progressive condition that affects polio survivor’s years after their initial infection with polio virus. Individuals with post-polio syndrome suffer from a variety of symptoms that negatively impact their independence and overall happiness, including daily general fatigue (48- 93%), pain (72-91%), respiratory issues (11- 41%), depression (13.45%), and sleep disorders (13-48%). Here, we present a patient with post-polio syndrome who presents with an unusual symptom manifestation of hoarseness.
Postpolio syndrome and anesthesia
The development of polio vaccines 50 years ago essentially halted childhood polio epidemics in the industrialized world. During the past quarter century, a constellation of delayed neuromuscular symptoms, called postpolio syndrome, became recognized among the aging polio survivors. The prevalence of postpolio syndrome in the US population is estimated to be in the hundreds of thousands. The most common symptoms are fatigue, pain, and new onset weakness thought to be related to delayed deterioration of motor neuron function. When a patient with postpolio syndrome presents for surgery, special precautions are warranted, because these patients may have respiratory impairment, sleep apnea, swallowing difficulties, and cold intolerance. This article first reviews clinical features and some pathoetiologic theories of postpolio syndrome and then focuses on anesthetic considerations including the use of common anesthetics, neuromuscular blockade, regional anesthesia, and general anesthetic management strategies.
Anesthetic implications of postpolio syndrome: new concerns for an old disease
Poliomyelitis was pandemic in the United States and much of the world in the first half of the 20th century. The uses of polio vaccines have essentially eradicated the disease in the United States today. But poliovirus infection survivors who had experienced a paralytic attack can see a return of some symptoms, which is a syndrome called postpolio syndrome (PPS). The anesthetist must preoperatively assess reported amounts of patient physical activity and patient age, which can indicate the amount of muscle degeneration that may have already occurred. Patients with PPS demonstrate altered respiratory function, cold intolerance, a risk for aspiration, and experience chronic pain in muscles and joints. Patients with PPS display an increased sensitivity to some anesthetic agents such as long-acting narcotics and potent inhaled anesthetic gases with a high blood-gas partition coefficient, along with report of increased fatigue, weakness, and somnolence after anesthesia. Anesthesia care must center on the preservation of muscle function postoperatively. The anesthetist should consider the use of short-acting anesthetic agents, increased doses of analgesics, the use of warming devices, and careful attention to patient positioning. Prolonged postoperative care and hospital admission after surgery are possible.
Anaesthetists need to be wary of postpolio syndrome
This is a brief Safety and Quality news item which does not have an abstract. This is an extract:

Postpolio syndrome (PPS) occurs in a significant proportion of polio survivors years after recovering from the original illness. Anaesthetists need to be aware of the syndrome as polio survivors are relatively numerous (40,000 cases of paralytic polio in Australia from the 1930s to the 1960s), they are of an age where they will be increasingly presenting for elective and emergency procedures and the syndrome has important. Postpolio syndrome patients present a number of potential problems for the anaesthetist. However for those who are aware depressant effects of analgesics of the syndrome, careful assessment and planning should minimise the risk of perioperative complications and provide optimal patient outcomes.
Vocational Implications
Occupations that people with late effects of polio perceive difficult to perform
The aims of this study were to describe which occupations that people with late effects of polio perceive difficult to perform, which occupational area the occupations were related to and their level of complexity. The aims were also to describe their own perception of the importance, performance and satisfaction with these occupations. Sixty-two participants (mean age 61 years) were assessed with the Canadian Occupational Performance Measure. A total of 431 occupations were reported (43% self-care, 32% productivity and 25% leisure). Two subcategories, household management (27 %) and functional mobility (23 %), represented half of all the reported occupations. Ratings for prioritized occupations (N = 300) were high for importance and generally low for performance and satisfaction. A wide variety of occupations were reported, related to both occupational areas and different levels of complexity within an occupational area. The results underscore the importance of using assessment tools that can capture both the variety and complexity of occupations. By obtaining more detailed information about occupations that people with late effects of polio perceive difficult to perform, this will enable occupational therapists to offer targeted interventions that can facilitate engagement in meaningful and purposeful occupations. A larger and more heterogeneous sample may enable the results to be generalized to more people with late effects of polio. Future studies should focus on methods that can facilitate engagement in meaningful and purposeful occupations for people with late effects of polio.
Vocational Implications
Vocational implications of post-polio syndrome
Of more than 1,000,000 survivors of poliomyelitis living in the United States, about 80% experience symptoms associated with post-polio syndrome (PPS). New weakness, fatigue, and pain are the most common symptoms that may appear years after acute polio. PPS is reported to be the most prevalent progressive neuromuscular disease in North America. The physical symptoms of PPS can be severe enough to decrease an individual's quality of life, significantly alter work function, and impose lifestyle changes. The psychological implications of battling once again with a disease that had stabilized decades ago can be devastating. Rehabilitation physicians and counselors knowledgeable of post-polio syndrome and its possible impact on employment can have a positive influence on persons with PPS.
Vocational Implications
Overcoming barriers to work participation for patients with postpoliomyelitis syndrome
PURPOSE: This study aimed to explore the perceived work ability of patients with postpoliomyelitis syndrome (PPS), to identify barriers and factors that are impeding or conducive, respectively, to work participation, and to identify possible interventions.

METHODS: Qualitative cross-sectional interview study with 17 subjects with PPS, 12 women and 5 men, mean age 49 years (SD: 11). Semi-structured interviews were held with the subjects in their homes, with the aid of a topic list. The COREQ criteria list for qualitative research was used as guideline in design and analysis.

RESULTS: Thirteen out of 17 subjects rated their work ability six or higher on a scale from 0 to 10. Most subjects worked in an administrative, educational or managerial function. Five subjects stopped working, four worked between 0 and 20 h/week and eight worked between 20 and 40 h/week. Factors conducive to working were physical adaptations in the workplace, accessibility of the workplace and high decision latitude. Barriers to full work ability in PPS patients were high physical job demands, low social support and the symptoms of PPS, especially fatigue and pain.
Vocational Implications
Workplace disability management in postpolio syndrome
Postpolio 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. These new problems may lead to loss of employment as well as new deficits in instrumental activities in daily living (cleaning, washing, shopping, transportation, etc.), walking, climbing stairs, and personal assistance. We presented three cases of PPS with working disabilities in Japan, and stated the issues confronted with. Particularly at the workplace, PPS individuals need special supports from both rehabilitation medicine and occupational health services, including improved nutrition, achieving ideal body weight, regular and sensible exercise, frequent checkups, and modifying working conditions.

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