Spinal cord gray matter atrophy is associated with functional decline in post-polio syndrome.

OBJECTIVE: To determine if patients with post-polio syndrome (PPS) show spinal cord gray matter (SCGM) atrophy and to assess associations between SCGM atrophy, muscle strength and patient-reported functional decline. METHODS: Twenty patients diagnosed with PPS (March of Dimes criteria) and 20 age- and sex-matched healthy controls (HC) underwent 3T axial 2D-rAMIRA magnetic resonance imaging at the intervertebral disc levels C2/C3-C6/C7, T9/T10 and the lumbar enlargement level (Tmax ) (0.5 × 0.5 mm2 in-plane resolution). SCGM areas were segmented manually by two independent raters. Muscle strength, self-reported fatigue, depression and pain measures were assessed. RESULTS: Post-polio syndrome patients showed significantly and preferentially reduced SCGM areas at C2/C3 (p = 0.048), C3/C4 (p = 0.001), C4/C5 (p < 0.001), C5/C6 (p = 0.004) and Tmax (p = 0.041) compared to HC. SCGM areas were significantly associated with muscle strength in corresponding myotomes even after adjustment for fatigue, pain and depression. SCGM areaTmax together with age and sex explained 68% of ankle dorsiflexion strength variance. No associations were found with age at or time since infection. Patients reporting PPS-related decline in arm function showed significant cervical SCGM atrophy compared to stable patients adjusted for initial disease severity. CONCLUSIONS: Patients with PPS show significant SCGM atrophy that correlates with muscle strength and is associated with PPS-related functional decline. Our findings suggest a secondary neurodegenerative process underlying SCGM atrophy in PPS that is not explained by aging or residua of the initial infection alone. Confirmation by longitudinal studies is needed. The described imaging methodology is promising for developing novel imaging surrogates for SCGM diseases.

Spread of vimentin-immunoreactive cells within the plaque-like lesion in the spinal anterior horn of a patient with post-poliomyelitis syndrome.

A Japanese man in the present study experienced acute weakness in his right leg as a two year old. The strength in his leg gradually recovered and developed, and he could play golf and climb mountains up to around the age of 50. From approximately 55 years of age, he became unable to stand up from a stooped position. Muscle weakness and atrophy spread to his right arm, and an electromyography revealed a neurogenic pattern in his lower and upper extremities. The patient was diagnosed as having post-poliomyelitis syndrome (PPS). Numbness in both the legs and pain in the buttocks occurred after 60 years of age. Computed tomography and magnetic resonance imaging at that time revealed spondylosis and protrusion of an osteophye in lower thoracic vertebrae compressing the second lumbar segment of the spinal cord. He died of malignant lymphoma and acute interstitial pneumonia at 80 years of age. Pathological examination revealed transverse myelopathy at the second lumbar segment of the spinal cord and total necrosis. The anterior horn and the intermediate zone of the third and fourth lumbar segments of the spinal cord on the right side were atrophic and diffusely gliotic. An oval-shaped plaque-like lesion was observed in the right anterior horn at the third and fourth lumbar segments of the spinal cord. Neurons and synaptophysin immunoreactivity had completely disappeared in the plaque-like lesion. A striking spread of vimentin-immunoreactive cells was found corresponding to the lesion, while glial fibrillary acidic protein-immunoreactive astrocytes existed evenly in the anterior horn and intermediate zone on both sides of the third and fourth lumber segments of the spinal cord. Virological examination using the autopsied materials was negative for poliovirus. Neither transactivation response DNA-binding protein of 43 kDa-immunoreactive inclusion nor Bunina body was seen in the spinal cord. The present paper demonstrates new findings of a noteworthy response of the vimentin-immunoreactive cells within the peculiar "plaque-like lesion" in the PPS.

Resultados de um programa de exercício físico combinado na força muscular e capacidade funcional de um indivíduo com síndrome Pós-Poliomielite: relato de caso / Effects of a combined exercise program in muscle strength and functional capacity in a individual with Postpoliomyelitis syndrome: case report

A Poliomielite Anterior Aguda (PAA) é uma patologia causada por um enterovírus, que predominantemente prejudica os neurônios motores inferiores, o que causa a paralisia muscular flácida e assimétrica, principalmente nos membros inferiores e que, tardiamente causa sintomas neuromusculares e declínio funcional denominados como Síndrome Pós-Poliomielite (SPP). Objetivo: Aplicar um programa de exercícios combinado e verificar os resultados na força muscular e capacidade funcional de um indivíduo idoso, com SPP. Métodos: A amostra foi composta por 1 mulher, idade 66 anos, com SPP, hipertensão arterial sistêmica, dislipidemia, hipotireoidismo. A força muscular foi mensurada por meio do teste de 7 ­ 10 repetições máximas (RM). A distância percorrida ao pedalar foi realizada por meio do Teste 12 minutos adaptado e o equilíbrio dinâmico foi avaliado por meio do Timed Up & Go Test. Os exercícios foram realizados em 2 séries de 10 repetições, com intensidade de 60% de 1 RM, por 24 semanas. Resultados: Após 24 semanas, a força muscular de MMII apresentou aumento de 333,3% e a força de MMSS 216,5%. O equilíbrio dinâmico melhorou 34,3%. A distância percorrida ao pedalar aumentou 11,8%. Conclusão: Os dados por nós obtidos sugerem que pessoas com SPP, mesmo que idosas, poderão se beneficiar de um programa de exercícios para aumento da força muscular e melhora da capacidade funcional, porém é importante que outros estudos sejam desenvolvidos para verificar esses resultados em um número maior de pessoas.

Postpoliomyelitis Syndrome is a enterovirus pathology, which predominantly damages the lower muscles, which cause flaccid and asymmetric muscle paralysis, based on lower and late neuromuscular and functional symptoms denominate Postpoliomyelitis Syndrome (PPS). Objective: To apply a combined exercise program and to verify the results on the muscle strength and functional capacity of an elderly individual with PPS. Methods: The sample was one women, 66 years old, with comorbities hypertension, dyslipidemia, hypothyroidism. Muscle strength was measured by 7 - 10 maximal repetitions (MR) test. The distance traveled pedaling was performed by Adapted 12-minute Test and the balance was evaluated by Timed Up & Go Test. The muscle strength training was intensity 60% of a one maximum repetition. Results: After 24 weeks, the muscular strength of lower limbs increased 333.3% and the upper limbs, 216.5%. The balance improved 34.3%. The distance traveled pedaling increased 11.8%. Conclusion: The results suggest that individuals with PPS, even if elderly, may benefit from an exercise program to increase muscle strength and improve functional capacity, but it is important that other studies are developed to verify these results in a larger number of individuals.

Cognitive behavioural therapy for reducing fatigue in post-polio syndrome and in facioscapulohumeral dystrophy: A comparison.

BACKGROUND: Cognitive behavioural therapy does not reduce fatigue in post-polio syndrome, but is effective in facioscapulohumeral dystrophy. This difference in efficacy might be explained by a different role of cognitions in these conditions. OBJECTIVE: To compare fatigue-related cognitions between patients with post-polio syndrome and facio-scapulohumeral dystrophy. SUBJECTS: Patients with post-polio syndrome (n = 21) and facioscapulohumeral dystrophy (n = 24) allocated to a cognitive behavioural therapy intervention in 2 identical trials. METHODS: Assessed cognitions included: sense of control over fatigue; catastrophizing; acceptance; focusing on fatigue; and perceived social support. Group differences in cognitions (independent t-tests or Mann-Whitney U tests) and group differences in the association of cognitions with fatigue (linear regression models) were studied. RESULTS: No differences in cognitions were found between the 2 groups (p > 0.18). Furthermore, there were no cognition-by-group interaction effects, except for "perceived social support", for which a different association with fatigue was found between the 2 groups (p = 0.01). However, univariate models revealed no associations per group. CONCLUSION: Fatigue-related cognitions in severely fatigued patients with post-polio syndrome are not clearly different from that in facioscapulohumeral dystrophy. Thus, the lack of efficacy of cognitive behavioural therapy in post-polio syndrome cannot be attributed to unique cognitive characteristics of this population.

Falls in Korean Polio Survivors: Incidence, Consequences, and Risk Factors.

Falls and fall-related injuries are important issue among polio survivors. The purpose of this study was to determine the incidence of, and consequences and factors associated with falls among Korean polio survivors. A total of 317 polio survivors participated in this study. All participants completed a questionnaire including fall history, symptoms related to post-polio syndrome and other information through a telephone interview. Among them, 80 participants visited our clinic for additional physical measurements and tests. Of the 317 respondents, 68.5% reported at least one fall in the past year. Of the fallers, 42.5% experienced at least one fall during one month. Most falls occurred during ambulation (76.6%), outside (75.2%) and by slipping down (29.7%). Of fallers, 45% reported any injuries caused by falls, and 23.3% reported fractures specifically. Female sex, old age, low bone mineral density, the presence of symptoms related to post-polio syndrome (PPS), poor balance confidence, short physical performance battery and weak muscle strength of knee extensor were not significantly associated with falls. Only leg-length discrepancy using spine-malleolar distance (SMD) was a significant factor associated with falls among Korean polio survivors. Our findings suggest that malalignment between the paralytic and non-paralytic limb length should be addressed in polio survivors for preventing falls.

[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.

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. CONCLUSIONS: MU size and strength declined concomitantly in a homogeneous cohort of patients with PPS and quadriceps dysfunction. 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.

Differential diagnosis between post-poliosyndrome symptoms and temporomandibular disorder – Clinical case

Post-poliomyelitis syndrome (PPS) is characterized by the delayed appearance of new neuromuscular symptoms in patients several years after their acute poliomyelitis paralysis. Clinical features of PPS include fatigue, joint and muscle pain, new muscular weakness and bulbar symptoms. The diagnosis is essentially clinical after excluding other neurological, orthopedic or rheumatologic problems. Temporomandibular disorders (TMD) are usually diagnosed by means of comprehensive review of patient history and clinical examination and the symptoms are pain/ discomfort in the jaw, mainly in the region of the temporomandibular joints (TMJs) and/or masticatory muscles, limitation of mandibular function and/or TMJ sounds. In the same way as PPS, the diagnosis of TMD is challenging. This study reports the case of a patient that presented thesymptoms of both conditions in the stomatognathic system, and discusses how to achieve the differential diagnosis for proper management of the cases.

A fatal neuromuscular disease in an adult patient after poliomyelitis in early childhood: consideration of the pathology of post-polio syndrome.

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.

[Post-polio syndrome. Part I. The "legacy" of forgotten disease, challenges for professionals and polio survivors]. / Zespól post-polio. Czesc I. "Dziedzictwo" zapomnianej choroby, wyzwanie dla lekarzy i pacjentów

The outcome of paralytic polio was believed to be a stable neurological state. Now, it is established that polio has an additional, slowly progressive phase, called post-polio syndrome (PPS) that develops 30-40 years after the acute poliomyelitis in 25-80% of paralytic and about 40% of nonparalytic polio survivors. The clinical symptoms are nonspecific and usually include muscle weakness, fatigue and muscle or joint pain. Some patients suffer from muscular atrophy, respiratory insufficiency, dysphagia, sleep disturbances or cold intolerance. The etiopathogenesis of PPS is unclear and many factors, such as dysfunction of the surviving motor units, aging, defects of neuromuscular transmission, persistence of viral infection and immunological mechanisms, are considered.

[Post-polio syndrome. Part II. Therapeutic management]. / Zespól post-polio. Czesc II. Postepowanie terapeutyczne.

The care of patients with post-polio syndrome ought to be carried out by a multidisciplinary team of specialists, including medical professionals, specialists of rehabilitation, psychologists and social workers. Many therapeutic strategies might be employed to reduce the late effects of polio. Today, the management of post-polio syndrome is based on non-pharmacological intervention, including lifestyle modification, decrease of physical activity, rest periods during the day and an individually tailored training program.

Concomitant CNS pathology in a patient with amyotropic lateral sclerosis following poliomyelitis in childhood.

Post-polio syndrome (PPS) develops in approximately 30% of polio survivors several decades after the acute attack of paralytic poliomyelitis. Some of these patients develop post-poliomyelitis muscular atrophy (PPMA) which is characterized by a slowly progressive muscle weakness. Due to its clinicopathological features, investigators have often studied PPS and PPMA in association with amyotrophic lateral sclerosis (ALS), the underlying hypothesis being an increased risk of developing ALS from a prior acute paralytic poliomyelitis. Various studies, however, have indicated that de novo ALS cases in patients with prior acute paralytic poliomyelitis are rare. Herein, we describe a rare case of a 75-year-old woman who at post-mortem examination presented a combination of a PPS with proven histopathological sporadic ALS features. Furthermore, neuropathology of this case also revealed several other histopathological findings reminiscent of a tauopathy, synucleinopathy and amyloid angiopathy and a large pituitary cyst. To our knowledge, this is the first reported case of PPS with clear pathological hallmarks of sporadic ALS, including ubiquitin-, TDP-43, phosphorylated TDP-43- and p62-positive inclusions, with accompanying features compatible with Alzheimer's and Parkinson's disease.

Poliomielite e síndrome pós-pólio

A cirurgia, agressiva, por vezes abusiva, em pleno inverno paulistano, predispunha à polioencefalite, à perda da consciência! Não bastante, identificavam-se mais fatores desencadeantes: injeções intramusculares, apendicectomias, insolação, exposição ao frio... A carga dos vírus selvagens em circulação na população era fora do comum. Iceberg, o melhor exemplo para estimativa de sua densidade; a ponta, as ocorrências paralíticas; a base, submersa, a imensidade das formas inaparentes.Denominação paralisia infantil? Impropriedade. Adultos ? populares ou famosos ? padeceram da enfermidade.A raiva muda ? forma clínica paralítica da raiva humana ?, figurava entre opções na discussão de casos. Nada simples distinguir enfermidades com sintomas afins.Trata-se agora de rediscutir a estratégia para assegurar de vez a erradicação.A modalidade atual de queixas,retroativas,é a "síndrome pós-pólio".

Síndrome pós-poliomielite / Post-poliomyelitis syndrome

A poliomielite é uma doença erradicada no Brasil, desde os anos 80, mas atualmente estão aparecendo novos sintomas relacionados a essa patologia, a síndrome pós-polio, que é caracterizada por uma nova desordem neurológica. Esses sintomas aparecem 30 a 40 anos após a infecção aguda da poliomielite e, como é uma patologia recente, há poucos estudos e casos diagnosticados. O objetivo deste estudo é descrever o aparecimento do caso dessa síndrome, através do relato de caso de uma paciente que realiza tratamento fisioterapêutico na clínica de Fisioterapia do Hospital Regional de Araranguá, localizado na cidade de Araranguá/SC, em agosto de 2006. Conforme a literatura, os sinais e sintomas que foram relatados pelo estudo são considerados como uma conseqüência tardia da infecção aguda da poliomielite, devido à degeneração dos brotamentos dos axônios atingidos pela poliomielite há 40 anos.

The poliomyelitis disease was eradicated in Brazil in the eighties, but now new symptoms related to this pathology are rising, like the post-polio syndrome, which is characterized by a new neurological disorder. These symptoms rise 30 to 40 years after the acute poliomyelitis infection and, as it is a recent pathology, studies and diagnosed cases are scarce. The objective of this study was to describe the onset of this syndrome, through the case of a patient attended in a Physical Therapy Clinic of a Regional Hospital of Araranguá, located in the city of Araranguá/SC, Brazil, in August 2006. According to literature, signs and symptoms reported in the study are considered as the consequence of acute poliomyelitis infection, due to degeneration of budding axons damaged by the poliomyelitis 40 years ago.

Physiology of the motor cortex in polio survivors.

We hypothesized that the corticospinal system undergoes functional changes in long-term polio survivors. Central motor conduction times (CMCTs) to the four limbs were measured in 24 polio survivors using transcranial magnetic stimulation (TMS). Resting motor thresholds and CMCTs were normal. In 17 subjects whose legs were affected by polio and 13 healthy controls, single- and paired-pulse TMS was used to assess motor cortex excitability while recording from tibialis anterior (TA) muscles at rest and following maximal contraction until fatigue. In polio survivors the slope of the recruitment curve was normal, but maximal motor evoked potentials (MEPs) were larger than in controls. MEPs were depressed after fatiguing exercise. Three patients with central fatigue by twitch interpolation had a trend toward slower recovery. There was no association with symptoms of post-polio syndrome. These changes occurring after polio may allow the motor cortex to activate a greater proportion of the motor neurons innervating affected muscles.

Theodore E. Woodward Award: The devastating backlash of a dread disease: poliomyelitis.

The paper is in three parts. 1) A description of acute anterior poliomyelitis; 2) A summary of the condition of post-polio syndrome; 3) A description of two small epidemics of poliomyelitis juxtaposed and related to one another.