Spinal cord histopathology in long‐term survivors of poliomyelitis

Kaminski, Henry J.; Tresser, Nancy; Hogan, Robert; Martin, Eric

doi:10.1002/mus.880181020

Cited by 19 publications

(6 citation statements)

References 6 publications

(1 reference statement)

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The level of TNF‐ α in the CSF from patients with previous polio and stable neuromuscular function, have not been investigated. Inflammatory changes in the spinal cord and meningeal infiltrates have been reported in patients with previous polio in a small post‐mortem study, but these patients were not examined for PPS [18]. We cannot establish whether the increased TNF‐ α is a result of a chronic inflammation because of previous polio, or a result of a new dysimmune regulation in the spinal cord where surviving motor neurons are under metabolic stress and supply larger motor units than normal.…”

Section: Discussionmentioning

confidence: 97%

Post‐polio syndrome patients treated with intravenous immunoglobulin: a double‐blinded randomized controlled pilot study

Farbu

Rekand

Vik-Mo

et al. 2007

Euro J of Neurology

View full text Add to dashboard Cite

Post-polio syndrome (PPS) is characterized by new muscle weakness, atrophy, fatigue and pain developing several years after the acute polio. Some studies suggest an ongoing inflammation in the spinal cord in these patients. From this perspective, intravenous immunoglobulin (IvIg) could be a therapeutic option. We performed a double-blinded randomized controlled pilot study with 20 patients to investigate the possible clinical effects of IvIg in PPS. Twenty patients were randomized to either IvIg 2 g/kg body weight or placebo. Primary endpoints were changes in pain, fatigue and muscle strength 3 months after treatment. Surrogate endpoints were changes in cerebrospinal fluid (CSF) cytokine levels. Secondary endpoints were pain, fatigue and isometric muscle strength after 6 months. Patients receiving IvIg reported a significant improvement in pain during the first 3 months, but no change was noted for subjective fatigue and muscle strength. CSF levels of tumour necrosis factor-alpha (TNF-alpha) were increased compared with patients with non-inflammatory neurological disorders. In conclusion, in this small pilot study no effect was seen with IvIg treatment on muscle strength and fatigue, however IvIg treated PPS patients reported significantly less pain 3 months after treatment. TNF-alpha was increased in the CSF from PPS patients. The results are promising, but not conclusive because of the low number of patients studied.

show abstract

Section: Discussionmentioning

confidence: 97%

Post‐polio syndrome patients treated with intravenous immunoglobulin: a double‐blinded randomized controlled pilot study

Farbu

Rekand

Vik-Mo

et al. 2007

Euro J of Neurology

View full text Add to dashboard Cite

show abstract

“…It is well known that PPS can appear in a large proportion of patients in whom old poliomyelitis has stabilized 4–6 . However, autopsy studies of post‐poliomyelitis and PPS have been rare; 15,17–20 in such polio survivors, with or without PPS, degeneration of the corticospinal tracts is not a feature, 17,18 and infiltration of inflammatory cells into the spinal cord is a feature in some cases, 18–20 but not all 15,17…”

Section: Discussionmentioning

confidence: 99%

A fatal neuromuscular disease in an adult patient after poliomyelitis in early childhood: Consideration of the pathology of post‐polio syndrome

et al. 2012

View full text Add to dashboard Cite

Post-polio syndrome (PPS) characterized by new neuromuscular problems can appear many years after acute poliomyelitis in polio survivors. We report a 77-year-old man with antecedent poliomyelitis who newly developed neuromuscular disease with a clinical course of 27 years, the final 10 years of which were characterized by apparent progression, thus raising doubt as to the clinical diagnosis of amyotrophic lateral sclerosis (ALS) following PPS. Pathologically, plaque-like, old poliomyelitis lesions were found almost exclusively in the lumbosacral cord, showing complete neuronal loss and glial scars in the anterior horns. Although less severe, neuronal loss and gliosis were also evident outside the old lesions, including the intermediate zone. Moreover, symmetrical degeneration of the corticospinal tracts, as evidenced by CD68 immunostaining, was a feature of the white matter of the lower spinal cord. In the motor cortex, loss of Betz cells was also confirmed. Synaptophysin immunostaining of the lumbosacral cord also revealed decreased expression outside the old lesions, excluding the posterior horn. Interestingly, decreased expression of synaptophysin was also evident in the cervical anterior horns, where no old lesions were observed. No Bunina bodies, TDP-43 inclusions, or Golgi fragmentation were found. Neurogenic atrophy was evident in the iliopsoas and scalenus muscles, and inclusion body myositis-like changes were also observed in these muscles and the tongue. Was it possible to have diagnosed this patient as having ALS? We consider that the features in this case may have represented the pathology of long-standing and/or fatal PPS itself, and not ALS.

show abstract

“…Immunological mechanisms have been proposed as contributing factors to PPS. Evidence for active inflammation has been found in the spinal cord of poliomyelitis patients who died years later of other causes 66, 100. Such findings could relate to a persistent poliovirus infection, an immune‐mediated (autoimmune) syndrome, or a response to degenerating neurons 64.…”

Section: Pathophysiologymentioning

confidence: 99%

Post-poliomyelitis syndrome

2004

View full text Add to dashboard Cite

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.

show abstract

Spinal cord histopathology in long‐term survivors of poliomyelitis

Cited by 19 publications

References 6 publications

Post‐polio syndrome patients treated with intravenous immunoglobulin: a double‐blinded randomized controlled pilot study

Post‐polio syndrome patients treated with intravenous immunoglobulin: a double‐blinded randomized controlled pilot study

A fatal neuromuscular disease in an adult patient after poliomyelitis in early childhood: Consideration of the pathology of post‐polio syndrome

Post-poliomyelitis syndrome

Contact Info

Product

Resources

About