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

Title order Author order Journal order Date order
Category: Immune Response

Title: Factors Associated with the Severity of COVID-19 Outcomes in People with Neuromuscular Diseases: Data from the International Neuromuscular COVID-19 Registry
Author: Chiara Pizzamiglio (1,2), Robert D. S. Pitceathly (1,2), Michael P. Lunn (1), Stefen Brady (3), Fabiola De Marchi (4), Lucia Galan (5), Jeannine M. Heckmann (6), Alejandro Horga (5), Maria J. Molnar (7), Acary S. B. Oliveira (8), Wladimir B. V. R. Pinto (8), Guido Primiano (9,10), Ernestina Santos (11), Benedikt Schoser (12), Serenella Servidei (9,10), Paulo V. Sgobbi Souza (8), Venugopalan Vishnu (13), Michael G. Hanna (1,2), Mazen M. Dimachkie (14), Pedro M. Machado (1), The Neuromuscular Diseases and COVID-19 Study Group
Affiliation: For The Neuromuscular Diseases and COVID-19 Study Group. Individual affiliations are not listed.
Journal: European Journal of Neurology
Citation: Pizzamiglio, C., Pitceathly, R.D.S., Lunn, M.P., Brady, S., De Marchi, F., Galan, L., Heckmann, J.M., Horga, A., Molnar, M.J., Oliveira, A.S.B., Pinto, W.B.V.R., Primiano, G., Santos, E., Schoser, B., Servidei, S., Sgobbi Souza, P.V., Vishnu, V., Hanna, M.G., Dimachkie, M.M., Machado, P.M. and (2022), Factors Associated with the Severity of COVID-19 Outcomes in People with Neuromuscular Diseases: Data from the International Neuromuscular COVID-19 Registry. Eur J Neurol. Accepted Author Manuscript. https://doi.org/10.1111/ene.15613
Publication Year and Month: 2022 10

Abstract: BACKGROUND
To determine factors associated with the severity of COVID-19 outcomes in people with neuromuscular diseases (NMDs).

METHODS
NMD cases of any age and confirmed/presumptive COVID-19, submitted to the International Neuromuscular COVID-19 Registry up to 31/December/2021, were included. A mutually exclusive ordinal COVID-19 severity scale was defined: (1) no hospitalisation; (2) hospitalisation without oxygenation; (3) hospitalisation with ventilation/oxygenation; (4) death. Multivariable ordinal logistic regression analyses were used to estimate odds ratios for severe outcome, adjusting for age, sex, race/ethnicity, NMD, comorbidities, baseline functional status (modified Rankin scale [mRS]), use of immunosuppressive/immunomodulatory medication, and pandemic calendar period.

RESULTS
Of 315 patients from 13 countries (mean age 50.3 [±17.7] years, 154 [48.9%] female), 175 (55.5%) were not hospitalised, 27 (8.6%) were hospitalised without supplemental oxygen, 91 (28.9%) were hospitalised with ventilation/supplemental oxygen, and 22 (7%) died. Higher odds of severe COVID-19 outcomes were observed for: age≥50 years (50-64 years: OR=2.4, 95%CI 1.33-4.31; >64 years: OR=4.16, 95%CI 2.12-8.15; both vs. <50 years), non-White race/ethnicity (OR=1.81, 95%CI 1.07-3.06; vs. White), mRS moderately severe/severe disability (OR=3.02, 95%CI 1.6-5.69; vs. no/slight/moderate disability), history of respiratory dysfunction (OR=3.16, 95%CI 1.79-5.58), obesity (OR=2.24, 95%CI 1.18-4.25), ≥3 comorbidities (OR=3.2, 95%CI 1.76-5.83; vs. ≤2; if comorbidity count used instead of specific comorbidities), glucocorticoid treatment (OR=2.33, 95%CI 1.14-4.78), and Guillain-Barré syndrome (OR=3.1, 95%CI 1.35-7.13; vs. mitochondrial disease).

Conclusions: CONCLUSIONS
Among people with NMDs, there is differential risk of COVID-19 outcomes according to demographic and clinical characteristics. These findings could be used to develop tailored management strategies and evidence-based recommendations for NMD patients.

Outcome of Research: More research required

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

Comments (if any): SUMMARY
• 315 cases of COVID in database across 13 countries mostly from Britain

• Those with PPS = 3 (1%); hence PPS was grouped with “other” in the analysis/results

• Those with anterior horn cell (AHC) conditions = 24 (7.6%); this includes those with PPS

• 44% of those diagnosed with COVID with a NMD required hospitalisation and ¾ of those hospitalised required ventilation or supplemental oxygen

• 7% died from severe COVID; this is obviously a higher than population fatality rate

• More severe cases of COVID were associated with:
o Older; being 50-65 and >65; when compared to those <50y.o.
o Non-white ethnicity
o A diagnosis of GBS
o Moderate to severe and severe disability; on modified Rankin Scale
(mRS); primarily those unable to walk independently
o History or respiratory dysfunction (NMD and non-NMD diseases,
obesity)
o Obesity
o Glucocorticoid prescription active at the time of COVID
o > three comorbidities

• GBS had worst outcomes. For associations listed above, even when GBS cohort was removed from the analysis these risk factors for severe COVID remained.

• Long term sequalae of COVID was observed in 30% of this group

• Seems to be worse outcome/COVID in those with autoimmune disease (as a comorbidity)

• The burden of COVID is higher being increased disability

• A higher risk is present for those with higher disability at baseline

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


Category: Immune Response

Title: Intrathecal immune response in patients with the post-polio syndrome
Author: Sharief MK, Hentges R, Ciardi M
Affiliation: Department of Clinical Neurochemistry, National Hospital for Neurology and Neurosurgery, London, United Kingdom
Journal: The New England Journal of Medicine
Citation: N Engl J Med. 1991 Sep 12;325(11):749-55
Publication Year and Month: 1991 09

Abstract: 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).

Conclusions: An intrathecal immune response against poliovirus is present in many patients with the post-polio syndrome. In some of these patients the recrudescence of muscle weakness may be caused by persistent or recurrent infection of neural cells with the poliovirus.

Outcome of Research:

Availability of Paper:

Comments (if any): See also comments in:
N Engl J Med. 1992 Feb 27;326(9):641; author reply 642
N Engl J Med. 1992 Feb 27;326(9):640-1; author reply 642
N Engl J Med. 1992 Feb 27;326(9):641; author reply 642

Link to Paper (if available):


Category: Immune Response

Title: Leukocyte myeloperoxidase and pathogenesis of the post-polio syndrome
Author: Movitz C, Bergström T, Borg K, Hellstrand K, Lycke E, Lycke J
Affiliation: Department of Infectious Diseases, University of Gothenburg, Gothenburg; Department of Public Health Services, Division of Rehabilitation Medicine, Danderyds University Hospital, Karolinska Institute, Stockholm; Department of Neuroscience and Physiology, Sahlgrenska University Hospital, Gothenburg, Sweden - [email protected]
Journal: Scandinavian Journal of Infectious Disease
Citation: Scand J Infect Dis. 2010 Dec;42(11-12):958-60. doi: 10.3109/00365548.2010.524663
Publication Year and Month: 2010 12

Abstract: Letter to the Editor - does not have an abstract.

Conclusions:

Outcome of Research: Not applicable.

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

Comments (if any):

Link to Paper (if available): View first of three pages here


Category: Immune Response

Title: Normal serum levels of immune complexes in postpolio patients
Author: Melin E (1), Sohrabian A (2), Rönnelid J (2), Borg K (1)
Affiliation: (1) Division of Rehabilitation Medicine, Department of Clinical Sciences, Karolinska Institutet, Danderyd University Hospital, Stockholm, Sweden; (2) Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden
Journal: Results in Immunology
Citation: Results in Immunology. 2014; 4: 54–57. doi: 10.1016/j.rinim.2014.06.001
Publication Year and Month: 2014 06

Abstract: 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.

Conclusions: There was no increase in circulating immune complex or in tumor necrosis factor-inducing effects of circulating immune complex between postpolio patients and healthy controls, indicating that the postpolio syndrome is not due to an autoimmune reaction.

Outcome of Research: More research required.

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

Comments (if any):

Link to Paper (if available): Click here to view full text or to download


There are currently 4 papers in this category.

Category: Immune Response

Title: Factors Associated with the Severity of COVID-19 Outcomes in People with Neuromuscular Diseases: Data from the International Neuromuscular COVID-19 Registry
Author: Chiara Pizzamiglio (1,2), Robert D. S. Pitceathly (1,2), Michael P. Lunn (1), Stefen Brady (3), Fabiola De Marchi (4), Lucia Galan (5), Jeannine M. Heckmann (6), Alejandro Horga (5), Maria J. Molnar (7), Acary S. B. Oliveira (8), Wladimir B. V. R. Pinto (8), Guido Primiano (9,10), Ernestina Santos (11), Benedikt Schoser (12), Serenella Servidei (9,10), Paulo V. Sgobbi Souza (8), Venugopalan Vishnu (13), Michael G. Hanna (1,2), Mazen M. Dimachkie (14), Pedro M. Machado (1), The Neuromuscular Diseases and COVID-19 Study Group
Affiliation: For The Neuromuscular Diseases and COVID-19 Study Group. Individual affiliations are not listed.
Journal: European Journal of Neurology
Citation: Pizzamiglio, C., Pitceathly, R.D.S., Lunn, M.P., Brady, S., De Marchi, F., Galan, L., Heckmann, J.M., Horga, A., Molnar, M.J., Oliveira, A.S.B., Pinto, W.B.V.R., Primiano, G., Santos, E., Schoser, B., Servidei, S., Sgobbi Souza, P.V., Vishnu, V., Hanna, M.G., Dimachkie, M.M., Machado, P.M. and (2022), Factors Associated with the Severity of COVID-19 Outcomes in People with Neuromuscular Diseases: Data from the International Neuromuscular COVID-19 Registry. Eur J Neurol. Accepted Author Manuscript. https://doi.org/10.1111/ene.15613
Publication Year and Month: 2022 10

Abstract: BACKGROUND
To determine factors associated with the severity of COVID-19 outcomes in people with neuromuscular diseases (NMDs).

METHODS
NMD cases of any age and confirmed/presumptive COVID-19, submitted to the International Neuromuscular COVID-19 Registry up to 31/December/2021, were included. A mutually exclusive ordinal COVID-19 severity scale was defined: (1) no hospitalisation; (2) hospitalisation without oxygenation; (3) hospitalisation with ventilation/oxygenation; (4) death. Multivariable ordinal logistic regression analyses were used to estimate odds ratios for severe outcome, adjusting for age, sex, race/ethnicity, NMD, comorbidities, baseline functional status (modified Rankin scale [mRS]), use of immunosuppressive/immunomodulatory medication, and pandemic calendar period.

RESULTS
Of 315 patients from 13 countries (mean age 50.3 [±17.7] years, 154 [48.9%] female), 175 (55.5%) were not hospitalised, 27 (8.6%) were hospitalised without supplemental oxygen, 91 (28.9%) were hospitalised with ventilation/supplemental oxygen, and 22 (7%) died. Higher odds of severe COVID-19 outcomes were observed for: age≥50 years (50-64 years: OR=2.4, 95%CI 1.33-4.31; >64 years: OR=4.16, 95%CI 2.12-8.15; both vs. <50 years), non-White race/ethnicity (OR=1.81, 95%CI 1.07-3.06; vs. White), mRS moderately severe/severe disability (OR=3.02, 95%CI 1.6-5.69; vs. no/slight/moderate disability), history of respiratory dysfunction (OR=3.16, 95%CI 1.79-5.58), obesity (OR=2.24, 95%CI 1.18-4.25), ≥3 comorbidities (OR=3.2, 95%CI 1.76-5.83; vs. ≤2; if comorbidity count used instead of specific comorbidities), glucocorticoid treatment (OR=2.33, 95%CI 1.14-4.78), and Guillain-Barré syndrome (OR=3.1, 95%CI 1.35-7.13; vs. mitochondrial disease).

Conclusions: CONCLUSIONS
Among people with NMDs, there is differential risk of COVID-19 outcomes according to demographic and clinical characteristics. These findings could be used to develop tailored management strategies and evidence-based recommendations for NMD patients.

Outcome of Research: More research required

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

Comments (if any): SUMMARY
• 315 cases of COVID in database across 13 countries mostly from Britain

• Those with PPS = 3 (1%); hence PPS was grouped with “other” in the analysis/results

• Those with anterior horn cell (AHC) conditions = 24 (7.6%); this includes those with PPS

• 44% of those diagnosed with COVID with a NMD required hospitalisation and ¾ of those hospitalised required ventilation or supplemental oxygen

• 7% died from severe COVID; this is obviously a higher than population fatality rate

• More severe cases of COVID were associated with:
o Older; being 50-65 and >65; when compared to those <50y.o.
o Non-white ethnicity
o A diagnosis of GBS
o Moderate to severe and severe disability; on modified Rankin Scale
(mRS); primarily those unable to walk independently
o History or respiratory dysfunction (NMD and non-NMD diseases,
obesity)
o Obesity
o Glucocorticoid prescription active at the time of COVID
o > three comorbidities

• GBS had worst outcomes. For associations listed above, even when GBS cohort was removed from the analysis these risk factors for severe COVID remained.

• Long term sequalae of COVID was observed in 30% of this group

• Seems to be worse outcome/COVID in those with autoimmune disease (as a comorbidity)

• The burden of COVID is higher being increased disability

• A higher risk is present for those with higher disability at baseline

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


Category: Immune Response

Title: Normal serum levels of immune complexes in postpolio patients
Author: Melin E (1), Sohrabian A (2), Rönnelid J (2), Borg K (1)
Affiliation: (1) Division of Rehabilitation Medicine, Department of Clinical Sciences, Karolinska Institutet, Danderyd University Hospital, Stockholm, Sweden; (2) Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden
Journal: Results in Immunology
Citation: Results in Immunology. 2014; 4: 54–57. doi: 10.1016/j.rinim.2014.06.001
Publication Year and Month: 2014 06

Abstract: 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.

Conclusions: There was no increase in circulating immune complex or in tumor necrosis factor-inducing effects of circulating immune complex between postpolio patients and healthy controls, indicating that the postpolio syndrome is not due to an autoimmune reaction.

Outcome of Research: More research required.

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

Comments (if any):

Link to Paper (if available): Click here to view full text or to download


Category: Immune Response

Title: Leukocyte myeloperoxidase and pathogenesis of the post-polio syndrome
Author: Movitz C, Bergström T, Borg K, Hellstrand K, Lycke E, Lycke J
Affiliation: Department of Infectious Diseases, University of Gothenburg, Gothenburg; Department of Public Health Services, Division of Rehabilitation Medicine, Danderyds University Hospital, Karolinska Institute, Stockholm; Department of Neuroscience and Physiology, Sahlgrenska University Hospital, Gothenburg, Sweden - [email protected]
Journal: Scandinavian Journal of Infectious Disease
Citation: Scand J Infect Dis. 2010 Dec;42(11-12):958-60. doi: 10.3109/00365548.2010.524663
Publication Year and Month: 2010 12

Abstract: Letter to the Editor - does not have an abstract.

Conclusions:

Outcome of Research: Not applicable.

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

Comments (if any):

Link to Paper (if available): View first of three pages here


Category: Immune Response

Title: Intrathecal immune response in patients with the post-polio syndrome
Author: Sharief MK, Hentges R, Ciardi M
Affiliation: Department of Clinical Neurochemistry, National Hospital for Neurology and Neurosurgery, London, United Kingdom
Journal: The New England Journal of Medicine
Citation: N Engl J Med. 1991 Sep 12;325(11):749-55
Publication Year and Month: 1991 09

Abstract: 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).

Conclusions: An intrathecal immune response against poliovirus is present in many patients with the post-polio syndrome. In some of these patients the recrudescence of muscle weakness may be caused by persistent or recurrent infection of neural cells with the poliovirus.

Outcome of Research:

Availability of Paper:

Comments (if any): See also comments in:
N Engl J Med. 1992 Feb 27;326(9):641; author reply 642
N Engl J Med. 1992 Feb 27;326(9):640-1; author reply 642
N Engl J Med. 1992 Feb 27;326(9):641; author reply 642

Link to Paper (if available):


There are currently 4 papers in this category.

Category: Immune Response

Title: Factors Associated with the Severity of COVID-19 Outcomes in People with Neuromuscular Diseases: Data from the International Neuromuscular COVID-19 Registry
Author: Chiara Pizzamiglio (1,2), Robert D. S. Pitceathly (1,2), Michael P. Lunn (1), Stefen Brady (3), Fabiola De Marchi (4), Lucia Galan (5), Jeannine M. Heckmann (6), Alejandro Horga (5), Maria J. Molnar (7), Acary S. B. Oliveira (8), Wladimir B. V. R. Pinto (8), Guido Primiano (9,10), Ernestina Santos (11), Benedikt Schoser (12), Serenella Servidei (9,10), Paulo V. Sgobbi Souza (8), Venugopalan Vishnu (13), Michael G. Hanna (1,2), Mazen M. Dimachkie (14), Pedro M. Machado (1), The Neuromuscular Diseases and COVID-19 Study Group
Affiliation: For The Neuromuscular Diseases and COVID-19 Study Group. Individual affiliations are not listed.
Journal: European Journal of Neurology
Citation: Pizzamiglio, C., Pitceathly, R.D.S., Lunn, M.P., Brady, S., De Marchi, F., Galan, L., Heckmann, J.M., Horga, A., Molnar, M.J., Oliveira, A.S.B., Pinto, W.B.V.R., Primiano, G., Santos, E., Schoser, B., Servidei, S., Sgobbi Souza, P.V., Vishnu, V., Hanna, M.G., Dimachkie, M.M., Machado, P.M. and (2022), Factors Associated with the Severity of COVID-19 Outcomes in People with Neuromuscular Diseases: Data from the International Neuromuscular COVID-19 Registry. Eur J Neurol. Accepted Author Manuscript. https://doi.org/10.1111/ene.15613
Publication Year and Month: 2022 10

Abstract: BACKGROUND
To determine factors associated with the severity of COVID-19 outcomes in people with neuromuscular diseases (NMDs).

METHODS
NMD cases of any age and confirmed/presumptive COVID-19, submitted to the International Neuromuscular COVID-19 Registry up to 31/December/2021, were included. A mutually exclusive ordinal COVID-19 severity scale was defined: (1) no hospitalisation; (2) hospitalisation without oxygenation; (3) hospitalisation with ventilation/oxygenation; (4) death. Multivariable ordinal logistic regression analyses were used to estimate odds ratios for severe outcome, adjusting for age, sex, race/ethnicity, NMD, comorbidities, baseline functional status (modified Rankin scale [mRS]), use of immunosuppressive/immunomodulatory medication, and pandemic calendar period.

RESULTS
Of 315 patients from 13 countries (mean age 50.3 [±17.7] years, 154 [48.9%] female), 175 (55.5%) were not hospitalised, 27 (8.6%) were hospitalised without supplemental oxygen, 91 (28.9%) were hospitalised with ventilation/supplemental oxygen, and 22 (7%) died. Higher odds of severe COVID-19 outcomes were observed for: age≥50 years (50-64 years: OR=2.4, 95%CI 1.33-4.31; >64 years: OR=4.16, 95%CI 2.12-8.15; both vs. <50 years), non-White race/ethnicity (OR=1.81, 95%CI 1.07-3.06; vs. White), mRS moderately severe/severe disability (OR=3.02, 95%CI 1.6-5.69; vs. no/slight/moderate disability), history of respiratory dysfunction (OR=3.16, 95%CI 1.79-5.58), obesity (OR=2.24, 95%CI 1.18-4.25), ≥3 comorbidities (OR=3.2, 95%CI 1.76-5.83; vs. ≤2; if comorbidity count used instead of specific comorbidities), glucocorticoid treatment (OR=2.33, 95%CI 1.14-4.78), and Guillain-Barré syndrome (OR=3.1, 95%CI 1.35-7.13; vs. mitochondrial disease).

Conclusions: CONCLUSIONS
Among people with NMDs, there is differential risk of COVID-19 outcomes according to demographic and clinical characteristics. These findings could be used to develop tailored management strategies and evidence-based recommendations for NMD patients.

Outcome of Research: More research required

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

Comments (if any): SUMMARY
• 315 cases of COVID in database across 13 countries mostly from Britain

• Those with PPS = 3 (1%); hence PPS was grouped with “other” in the analysis/results

• Those with anterior horn cell (AHC) conditions = 24 (7.6%); this includes those with PPS

• 44% of those diagnosed with COVID with a NMD required hospitalisation and ¾ of those hospitalised required ventilation or supplemental oxygen

• 7% died from severe COVID; this is obviously a higher than population fatality rate

• More severe cases of COVID were associated with:
o Older; being 50-65 and >65; when compared to those <50y.o.
o Non-white ethnicity
o A diagnosis of GBS
o Moderate to severe and severe disability; on modified Rankin Scale
(mRS); primarily those unable to walk independently
o History or respiratory dysfunction (NMD and non-NMD diseases,
obesity)
o Obesity
o Glucocorticoid prescription active at the time of COVID
o > three comorbidities

• GBS had worst outcomes. For associations listed above, even when GBS cohort was removed from the analysis these risk factors for severe COVID remained.

• Long term sequalae of COVID was observed in 30% of this group

• Seems to be worse outcome/COVID in those with autoimmune disease (as a comorbidity)

• The burden of COVID is higher being increased disability

• A higher risk is present for those with higher disability at baseline

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


Category: Immune Response

Title: Normal serum levels of immune complexes in postpolio patients
Author: Melin E (1), Sohrabian A (2), Rönnelid J (2), Borg K (1)
Affiliation: (1) Division of Rehabilitation Medicine, Department of Clinical Sciences, Karolinska Institutet, Danderyd University Hospital, Stockholm, Sweden; (2) Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden
Journal: Results in Immunology
Citation: Results in Immunology. 2014; 4: 54–57. doi: 10.1016/j.rinim.2014.06.001
Publication Year and Month: 2014 06

Abstract: 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.

Conclusions: There was no increase in circulating immune complex or in tumor necrosis factor-inducing effects of circulating immune complex between postpolio patients and healthy controls, indicating that the postpolio syndrome is not due to an autoimmune reaction.

Outcome of Research: More research required.

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

Comments (if any):

Link to Paper (if available): Click here to view full text or to download


Category: Immune Response

Title: Leukocyte myeloperoxidase and pathogenesis of the post-polio syndrome
Author: Movitz C, Bergström T, Borg K, Hellstrand K, Lycke E, Lycke J
Affiliation: Department of Infectious Diseases, University of Gothenburg, Gothenburg; Department of Public Health Services, Division of Rehabilitation Medicine, Danderyds University Hospital, Karolinska Institute, Stockholm; Department of Neuroscience and Physiology, Sahlgrenska University Hospital, Gothenburg, Sweden - [email protected]
Journal: Scandinavian Journal of Infectious Disease
Citation: Scand J Infect Dis. 2010 Dec;42(11-12):958-60. doi: 10.3109/00365548.2010.524663
Publication Year and Month: 2010 12

Abstract: Letter to the Editor - does not have an abstract.

Conclusions:

Outcome of Research: Not applicable.

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

Comments (if any):

Link to Paper (if available): View first of three pages here


Category: Immune Response

Title: Intrathecal immune response in patients with the post-polio syndrome
Author: Sharief MK, Hentges R, Ciardi M
Affiliation: Department of Clinical Neurochemistry, National Hospital for Neurology and Neurosurgery, London, United Kingdom
Journal: The New England Journal of Medicine
Citation: N Engl J Med. 1991 Sep 12;325(11):749-55
Publication Year and Month: 1991 09

Abstract: 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).

Conclusions: An intrathecal immune response against poliovirus is present in many patients with the post-polio syndrome. In some of these patients the recrudescence of muscle weakness may be caused by persistent or recurrent infection of neural cells with the poliovirus.

Outcome of Research:

Availability of Paper:

Comments (if any): See also comments in:
N Engl J Med. 1992 Feb 27;326(9):641; author reply 642
N Engl J Med. 1992 Feb 27;326(9):640-1; author reply 642
N Engl J Med. 1992 Feb 27;326(9):641; author reply 642

Link to Paper (if available):


There are currently 4 papers in this category.

Category: Immune Response

Title: Factors Associated with the Severity of COVID-19 Outcomes in People with Neuromuscular Diseases: Data from the International Neuromuscular COVID-19 Registry
Author: Chiara Pizzamiglio (1,2), Robert D. S. Pitceathly (1,2), Michael P. Lunn (1), Stefen Brady (3), Fabiola De Marchi (4), Lucia Galan (5), Jeannine M. Heckmann (6), Alejandro Horga (5), Maria J. Molnar (7), Acary S. B. Oliveira (8), Wladimir B. V. R. Pinto (8), Guido Primiano (9,10), Ernestina Santos (11), Benedikt Schoser (12), Serenella Servidei (9,10), Paulo V. Sgobbi Souza (8), Venugopalan Vishnu (13), Michael G. Hanna (1,2), Mazen M. Dimachkie (14), Pedro M. Machado (1), The Neuromuscular Diseases and COVID-19 Study Group
Affiliation: For The Neuromuscular Diseases and COVID-19 Study Group. Individual affiliations are not listed.
Journal: European Journal of Neurology
Citation: Pizzamiglio, C., Pitceathly, R.D.S., Lunn, M.P., Brady, S., De Marchi, F., Galan, L., Heckmann, J.M., Horga, A., Molnar, M.J., Oliveira, A.S.B., Pinto, W.B.V.R., Primiano, G., Santos, E., Schoser, B., Servidei, S., Sgobbi Souza, P.V., Vishnu, V., Hanna, M.G., Dimachkie, M.M., Machado, P.M. and (2022), Factors Associated with the Severity of COVID-19 Outcomes in People with Neuromuscular Diseases: Data from the International Neuromuscular COVID-19 Registry. Eur J Neurol. Accepted Author Manuscript. https://doi.org/10.1111/ene.15613
Publication Year and Month: 2022 10

Abstract: BACKGROUND
To determine factors associated with the severity of COVID-19 outcomes in people with neuromuscular diseases (NMDs).

METHODS
NMD cases of any age and confirmed/presumptive COVID-19, submitted to the International Neuromuscular COVID-19 Registry up to 31/December/2021, were included. A mutually exclusive ordinal COVID-19 severity scale was defined: (1) no hospitalisation; (2) hospitalisation without oxygenation; (3) hospitalisation with ventilation/oxygenation; (4) death. Multivariable ordinal logistic regression analyses were used to estimate odds ratios for severe outcome, adjusting for age, sex, race/ethnicity, NMD, comorbidities, baseline functional status (modified Rankin scale [mRS]), use of immunosuppressive/immunomodulatory medication, and pandemic calendar period.

RESULTS
Of 315 patients from 13 countries (mean age 50.3 [±17.7] years, 154 [48.9%] female), 175 (55.5%) were not hospitalised, 27 (8.6%) were hospitalised without supplemental oxygen, 91 (28.9%) were hospitalised with ventilation/supplemental oxygen, and 22 (7%) died. Higher odds of severe COVID-19 outcomes were observed for: age≥50 years (50-64 years: OR=2.4, 95%CI 1.33-4.31; >64 years: OR=4.16, 95%CI 2.12-8.15; both vs. <50 years), non-White race/ethnicity (OR=1.81, 95%CI 1.07-3.06; vs. White), mRS moderately severe/severe disability (OR=3.02, 95%CI 1.6-5.69; vs. no/slight/moderate disability), history of respiratory dysfunction (OR=3.16, 95%CI 1.79-5.58), obesity (OR=2.24, 95%CI 1.18-4.25), ≥3 comorbidities (OR=3.2, 95%CI 1.76-5.83; vs. ≤2; if comorbidity count used instead of specific comorbidities), glucocorticoid treatment (OR=2.33, 95%CI 1.14-4.78), and Guillain-Barré syndrome (OR=3.1, 95%CI 1.35-7.13; vs. mitochondrial disease).

Conclusions: CONCLUSIONS
Among people with NMDs, there is differential risk of COVID-19 outcomes according to demographic and clinical characteristics. These findings could be used to develop tailored management strategies and evidence-based recommendations for NMD patients.

Outcome of Research: More research required

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

Comments (if any): SUMMARY
• 315 cases of COVID in database across 13 countries mostly from Britain

• Those with PPS = 3 (1%); hence PPS was grouped with “other” in the analysis/results

• Those with anterior horn cell (AHC) conditions = 24 (7.6%); this includes those with PPS

• 44% of those diagnosed with COVID with a NMD required hospitalisation and ¾ of those hospitalised required ventilation or supplemental oxygen

• 7% died from severe COVID; this is obviously a higher than population fatality rate

• More severe cases of COVID were associated with:
o Older; being 50-65 and >65; when compared to those <50y.o.
o Non-white ethnicity
o A diagnosis of GBS
o Moderate to severe and severe disability; on modified Rankin Scale
(mRS); primarily those unable to walk independently
o History or respiratory dysfunction (NMD and non-NMD diseases,
obesity)
o Obesity
o Glucocorticoid prescription active at the time of COVID
o > three comorbidities

• GBS had worst outcomes. For associations listed above, even when GBS cohort was removed from the analysis these risk factors for severe COVID remained.

• Long term sequalae of COVID was observed in 30% of this group

• Seems to be worse outcome/COVID in those with autoimmune disease (as a comorbidity)

• The burden of COVID is higher being increased disability

• A higher risk is present for those with higher disability at baseline

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


Category: Immune Response

Title: Normal serum levels of immune complexes in postpolio patients
Author: Melin E (1), Sohrabian A (2), Rönnelid J (2), Borg K (1)
Affiliation: (1) Division of Rehabilitation Medicine, Department of Clinical Sciences, Karolinska Institutet, Danderyd University Hospital, Stockholm, Sweden; (2) Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden
Journal: Results in Immunology
Citation: Results in Immunology. 2014; 4: 54–57. doi: 10.1016/j.rinim.2014.06.001
Publication Year and Month: 2014 06

Abstract: 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.

Conclusions: There was no increase in circulating immune complex or in tumor necrosis factor-inducing effects of circulating immune complex between postpolio patients and healthy controls, indicating that the postpolio syndrome is not due to an autoimmune reaction.

Outcome of Research: More research required.

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

Comments (if any):

Link to Paper (if available): Click here to view full text or to download


Category: Immune Response

Title: Leukocyte myeloperoxidase and pathogenesis of the post-polio syndrome
Author: Movitz C, Bergström T, Borg K, Hellstrand K, Lycke E, Lycke J
Affiliation: Department of Infectious Diseases, University of Gothenburg, Gothenburg; Department of Public Health Services, Division of Rehabilitation Medicine, Danderyds University Hospital, Karolinska Institute, Stockholm; Department of Neuroscience and Physiology, Sahlgrenska University Hospital, Gothenburg, Sweden - [email protected]
Journal: Scandinavian Journal of Infectious Disease
Citation: Scand J Infect Dis. 2010 Dec;42(11-12):958-60. doi: 10.3109/00365548.2010.524663
Publication Year and Month: 2010 12

Abstract: Letter to the Editor - does not have an abstract.

Conclusions:

Outcome of Research: Not applicable.

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

Comments (if any):

Link to Paper (if available): View first of three pages here


Category: Immune Response

Title: Intrathecal immune response in patients with the post-polio syndrome
Author: Sharief MK, Hentges R, Ciardi M
Affiliation: Department of Clinical Neurochemistry, National Hospital for Neurology and Neurosurgery, London, United Kingdom
Journal: The New England Journal of Medicine
Citation: N Engl J Med. 1991 Sep 12;325(11):749-55
Publication Year and Month: 1991 09

Abstract: 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).

Conclusions: An intrathecal immune response against poliovirus is present in many patients with the post-polio syndrome. In some of these patients the recrudescence of muscle weakness may be caused by persistent or recurrent infection of neural cells with the poliovirus.

Outcome of Research:

Availability of Paper:

Comments (if any): See also comments in:
N Engl J Med. 1992 Feb 27;326(9):641; author reply 642
N Engl J Med. 1992 Feb 27;326(9):640-1; author reply 642
N Engl J Med. 1992 Feb 27;326(9):641; author reply 642

Link to Paper (if available):


There are currently 4 papers in this category.

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