[Post-polio Syndrome in the Perioperative Phase]. / Das Post-Polio-Syndrom in der perioperativen Phase.

Due to the numerous poliomyelitis epidemics that have continued over the last decades and the post-polio syndrome (PPS) that occurs 10 - 30 years after poliomyelitis infection, the prevalence of PPS is also expected to increase in Europe. At the same time, due to the musculoskeletal disorders associated with the underlying disease, PPS patients often require surgery for which special anaesthetic requirements must be taken into account. In this analysis we summarise the current evidence and recommendations.

Postpolio syndrome and the late effects of poliomyelitis. Part 1. pathogenesis, biomechanical considerations, diagnosis, and investigations.

Postpolio syndrome (PPS) is characterized by new muscle weakness and/or muscle fatigability that occurs many years after the initial poliomyelitis illness. Many theories exist regarding the pathogenesis of PPS, which remains incompletely understood. In contrast, the late effects of poliomyelitis are often a consequence of biomechanical alterations that occur as a result of polio-related surgeries, musculoskeletal deformities, or weakness. Osteoporosis and fractures of the polio-involved limbs are common. A comprehensive clinical evaluation with appropriate investigations is essential to fulfilling the established PPS diagnostic criteria. PPS is a diagnosis of exclusion in which a key clinical feature required for the diagnosis is new muscle weakness and/or muscle fatigability that is persistent for at least 1 year. Electromyographic and muscle biopsy findings including evidence of ongoing denervation cannot reliably distinguish between patients with or without PPS. Muscle Nerve 58:751-759, 2018.

Ultrasound-Guided Carpal Tunnel Release Using Dynamic Expansion of the Transverse Safe Zone in a Patient With Postpolio Syndrome: A Case Report.

The prevalence of carpal tunnel syndrome (CTS) in patients with postpolio syndrome occurs at a rate of 22%. Irrespective of those with CTS, 74% of postpolio patients weight bear through their arms for ambulation or transfers. As open carpal tunnel release is performed along the weight-bearing region of the wrist, their functional independence may be altered while recovering. This case demonstrates that ultrasound-guided carpal tunnel release was successfully performed in a patient with postpolio syndrome allowing him to immediately weight bear through his hands after the procedure so he could recover at home. LEVEL OF EVIDENCE: V.

Relationship of depression and medications on incidence of falls among people with late effects of polio.

The purpose of this study was to determine if falls in polio survivors, with or without post-polio syndrome (PPS), are related to number of medications taken, use of anti-depressant or psychoactive medications, or self-report of depression. A survey was sent to 300 members of a regional polio support group, asking them to document their fall history, medications used, and the presence of depression. Depression was measured by self-report and with the Geriatric Depression Scale, short form (GDS-15). One hundred and seventy-two usable surveys were returned with 146 of those completing the medication list. Sixty-two percent reported at least one fall in the past year. The multiple logistic regression was significant (p = 0.023), and it indicated depression to be a significant predictor (p = 0.012) of falls in polio survivors with and without PPS. The number of total medications or anti-depressant or psychoactive medications used was not related to fall incidence. Routine screening and treatment for depression may be one aspect of fall prevention which can be implemented through primary care.

Outcomes of total hip replacement in limbs affected by poliomyelitis.

INTRODUCTION: The outcomes of total hip replacement in patients suffering from residual poliomyelitis are poorly covered in the literature. In this retrospective study we posed the question of whether total hip replacement performed for degenerative hip diseases in limbs with residual poliomyelitis could determine satisfactory mid-term clinical and radiographic results, with a reasonable complication rate. METHODS: A retrospective study was carried out to assess the results of 14 total hip replacements performed on 14 patients with residual poliomyelitis on the involved limb from June 1999 to September 2011. Average age at the time of surgery was 51 years (range 26-66 years). Mean duration of follow-up was 92 months (range 52-156 months). Surgery was performed through a direct lateral approach on all hips. All but one were cementless implants. RESULTS: 2 implants failed, 1 due to traumatic acetabular fracture 6 days after surgery, and 1 due to aseptic cup loosening 13 years after surgery. Surgery was uneventful in all patients except 1 (7%), who experienced a transient sensory sciatic nerve palsy. At the latest follow up Harris Hip Score was 83.3 (range 72-91) with a marked improvement when compared to preoperative score (average 52, range 32-78). No dislocations had occurred. CONCLUSIONS: Total hip replacement can be considered a feasible option for hip osteoarthritis in patients with limbs affected by residual poliomyelitis. Longer follow-up studies are needed to assess the effectiveness of unconstrained total hip replacement in polio patients.

Anesthesia considerations for monitoring TCMEPs in adults diagnosed with poliomyelitis as children: a case report.

UNLABELLED: The use of transcranial motor evoked potentials (TCMEPs) to detect and hopefully prevent injury to the brain, spinal cord, and peripheral nerves intraoperatively has increased greatly in recent years. It is well established that in addition to certain anesthetic agents, patient factors such as advanced age, obesity, diabetes, hypertension, and a collection of neurological and neuromuscular diseases and disorders can greatly reduce or completely eliminate the ability to monitor TCMEPs effectively. One such disease, poliomyelitis (polio), is a highly contagious viral disease that has been mostly forgotten since its near-eradication through vaccination. Over the past three decades there has been increasing recognition of late onset neurological deterioration in individuals who were afflicted by, and apparently recovered from, paralytic poliomyelitis much earlier in life. This condition is known as post-poliomyelitis syndrome (PPS). Patients that appear to have fully recovered from polio, and those with PPS, may require special anesthetic considerations to facilitate effective TCMEP monitoring. CASE REPORT: We report the rapid loss of only lower extremity TCMEPs bilaterally during a C6-C7, C7-T1 ACDF in a 67-year-old female to treat left-sided C7-C8 radiculopathy and C6-T1 foraminal stenosis. The general anesthetic maintenance regimen of 0.3 MAC sevoflurane and 100 microg/kg/min propofol was paused, and a wake-up test was initiated. Full upper and lower extremity motor function was observed. A thorough review of the patient's medical history revealed the potential risk factor of full recovery from poliomyelitis as a child. The sevoflurane was removed from the anesthetic regimen, and the lower extremity TCMEPs returned and were present for the remainder of the surgery.

Síndrome pós pólio: um desafio para educação física adaptada / Post-poliomyelite syndrome: a challenge for adapted physical education

A Síndrome pós-poliomielite é definida como uma nova desordem neurológica, desenvolvendo nova fraqueza muscular e fadiga muscular anormal em indivíduos que tiveram poliomielite aguda. Objetivo: apresentar conhecimentos básicos sobre a Síndrome pós-poliomielite, esclarecendo as peculiaridades deste quadro clínico e realizar uma reflexão acerca da adequação da atividade física para as pessoas acometidas por esta doença. Além de refletirmos sobre esta questão, neste trabalho também sumarizamos as condutas para o treinamento e prescrição de exercícios usadas nestes últimos 10 anos. Métodos: trata-se de um ensaio teórico, exploratório. Conclusões: a questão relevante que se apresenta para a atividade física adaptada é a carga ideal de treinamento físico a ser prescrita para as pessoas que tiveram poliomielite, uma vez que o supertreinamento é um fator de risco para o desenvolvimento Síndrome pós-poliomielite...

Post-poliomyelitis syndrome is defined as a new neurological disorder, with the development of new muscular weakness and abnormal muscular fatigue in people that had acute poliomyelitis. Objectives: the aim was to present the basic post-poliomyelitis syndrome knowledge, clarifying the peculiarities of this clinical condition, and make a reflection about the physical activity adequacy for whom had this disease. Besides reflect about this, we summarized the approach for physical training and exercise prescription proposed on those last 10 years. Methods: this is atheoretical exploratory study. Conclusions: the big issue for the adapted physical education is about the ideal amount overload that should be prescribed in physical training, considering that overtraining is a risk factor for Post-poliomyelitis syndrome...

Frequency and clinical manifestations of post-poliomyelitis syndrome in a brazilian tertiary care center / Frequência e manifestações clínicas da síndrome pós-poliomielite em um centro terciário brasileiro

OBJECTIVE: To determine the frequency and clinical manifestations of patients with post-poliomyelitis syndrome (PPS) in a Brazilian division of neuromuscular disorders. METHODS: A total of 167 patients with prior history of paralytic poliomyelitis was investigated for PPS, based on international diagnostic criteria. Other variables analyzed were: gender, race, age at poliomyelitis infection, age at PPS onset, and PPS symptoms. RESULTS: One hundred and twenty-nine patients presented PPS, corresponding to 77.2% of the studied population. 62.8% were women and 37.2% were men. Mean age of patients with PPS at onset of PPS symptoms was 39.9±9.69 years. Their main clinical manifestations were: new weakness in the previously affected limbs (69%) and in the apparently not affected limbs (31%); joint pain (79.8%); fatigue (77.5%); muscle pain (76%); and cold intolerance (69.8%). CONCLUSIONS: Most patients of our sample presented PPS. In Brazil, PPS frequency and clinical features are quite similar to those of other countries.

OBJETIVO: Determinar a frequência e as manifestações clínicas de pacientes com síndrome pós-poliomielite (SPP) em um setor terciário de doenças neuromusculares brasileiro. MÉTODOS: Um total de 167 pacientes com história prévia de poliomielite paralítica foi estudado para diagnóstico de SPP, de acordo com critérios diagnósticos internacionais. Além da SPP, as variáveis analisadas foram: gênero, raça, idade à época da poliomielite aguda e idade no início dos sintomas da SPP. RESULTADOS: Cento e vinte e nove pacientes apresentaram SPP, correspondendo a 77,2% da população estudada. Mulheres constituíram 62,8% dos pacientes e os homens, 37,2%. A média de idade dos pacientes com SPP à época do início dos sintomas foi de 39,9±9,69 anos. Suas principais manifestações clínicas foram: manifestações novas de fraqueza em membros previamente afetados (69%) e em membros aparentemente não afetados (31%); dores articulares (79,8%); fadiga (77,5%); dor muscular (76%) e intolerância ao frio (69,8%). CONCLUSÕES: A maioria dos pacientes da presente casuística apresentou SPP. No Brasil, a frequência e as características clínicas da SPP são similares às observadas em outros países.

Perfil clínico e funcional de pacientes com Síndrome Pós-Poliomielite: uma análise de 18 casos / Functional and clinical profile of patients with Post-Polio Syndrome: an analysis of 18 cases

INTRODUÇAO: A Síndrome Pós-Pólio (SPP) é uma entidade neurológica caracterizada por um novo episódio de fraqueza muscular e/ou fadiga anormal em indivíduos que apresentaram a Poliomielite Anterior Aguda (PAA) anos antes. Acredita-se que a prevalência possa representar até 70% dos vitimados pela PAA. OBJETIVOS: A proposta do presente estudo foi analisar os dados de 18 pacientes com diagnóstico de SPP, identificar possíveis fatores preditivos na gênese da síndrome e estabelecer um perfil clínico e funcional desses pacientes. MÉTODOS: Coleta de dados e análise estatística de 18 pacientes com diagnóstico de SPP atendidos no Serviço de Neurologia do Hospital Universitário Antônio Pedro (HUAP) e na Associação Nacional de Deficientes Físicos (ANDEF). RESULTADOS: O perfil clínico e funcional desses pacientes é heterogêneo em relação às complicações mais frequentes e o nível de independência funcional. CONCLUSÃO: As manifestações clínicas e prejuízos funcionais apresentados pelos pacientes com SPP são fundamentais para construção de um arcabouço teórico-conceitual, visando a solução de problemas na prática clínica.

INTRODUCTION: The Post-Polio Syndrome (PPS) is a neurological entity characterized by a new episode of muscular weakness and/or abnormal fatigue in individuals that were attained years before by Acute Anterior Poliomyelitis (AAP). It is estimated that the prevalence can reach up to 70% of the patients with AAP. OBJECTIVES: The proposal of the present study was to analyze the data of 18 patients with PPS diagnosis, to identify possible predictive factors related to the genesis of the syndrome, and to establish a clinical and functional profile of these subjects. METHODS: Collection of data and statistical analysis of 18 patients with PPS was undertaken in the Service of Neurology of the University Hospital Antonio Pedro (HUAP) and in the Associação Nacional de Deficientes Físicos (ANDEF). RESULTS: The clinical and functional profile of the patients is heterogeneous in relation to the most frequent complications, as well as to the level of functional independence. CONCLUSION: The clinical manifestations and functional damages presented by the patients with PPS are essential for a theoretical-conceptual frame, aiming for a solution of problems in the clinical practice.

Myotonic dystrophy mimicking postpolio syndrome in a polio survivor.

We describe a 38-yr-old polio survivor with newly developed weakness from myotonic dystrophy. He suffered muscle atrophy and weakness in his legs as a result of poliomyelitis at the age of 3 yrs. After a stable interval of about 30 yrs, he felt new weakness and fatigue in his legs. Electromyography revealed generalized myotonic discharges, early recruitment, and findings of chronic denervation in his left leg. Genetic testing was consistent with myotonic dystrophy type 1. A biopsy from the right gastrocnemius revealed findings of both myotonic dystrophy and chronic denervation. This case report shows the importance of considering other uncommon conditions in the differential diagnoses of postpolio syndrome.

Síndrome pós-poliomielite (SPP): orientações para profissionais de saúde

As doenças neuromusculares representam um grupo de afecções que comprometem a unidade motora, ou seja, o corpo celular do neurônio motor inferior, o seu prolongamento, a junção neuromuscular ou o tecido muscular. Dentre as doenças neuromusculares, encontram-se as lesões neuronais motoras, que são condições nas quais há alterações morfológicas ou bioquímicas que ocorrem no corpo do neurônio. A lesão neuronal motora caracteriza-se por envolvimento do corpo celular do neurônio motor inferior (NMI). As principais doenças são: poliomielite anterior aguda (Pólio), atrofia muscular espinhal progressiva (AMEP) e doença do neurônio motor (DNM). A síndrome pós-poliomielite(SPP)é um transtorno neurológico, denro do capítulo dos efeitos tardios da poliomielite, caracterizados por nova fraqueza muscular e/ou fadiga muscular anormal em indivíduos que tiveram poliomielite aguda, muitos anos antes. A SPP encontra-se na categoria das doenças do nerônio motor (neuronopatia motora) em virtude dos quadros clínico e histológico estarem intimamente relacionados com disfunção dos neurônios motores inferiores.

Post-poliomyelitis syndrome: case report

The post-polio syndrome (PPS) is an entity characterized for an episode of muscular weakness and/or abnormal muscular fatigue in individuals that had presented acute polio years before. We report the case of PPS in a patient, 40 years, that thirty-five years after had had paralytic poliomyelitis, developed new symptoms of fatigue, muscular atrophy, dyspnea, difficulties in deambulation and muscular and joint pain. The electromyographic findings revealed injuried neurons of the anterior horn of the marrow and reinnervation after muscular tests.

A síndrome pós-polio (SPP) é entidade caracterizada por um episódio de fraqueza muscular e/ou fadiga muscular anormal em indivíduos que apresentaram poliomielite aguda anos antes. Relatamos o caso de SPP em um paciente, 40 anos, que trinta e cinco anos após haver tido poliomielite paralítica, desenvolveu novos sintomas de fadiga, atrofia muscular, dispnéia, dificuldades na deambulação e dores articulares e musculares. Os achados eletromiográficos revelaram acometimento dos neurônios da ponta anterior da medula e reinervação após a realização de testes musculares.

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.

Prevalence and risk factors of post-polio syndrome in a cohort of polio survivors.

OBJECTIVE: To investigate frequency and associated factors of post polio syndrome (PPS) in an Italian cohort of people with prior poliomyelitis. METHODS: We screened subjects admitted for poliomyelitis at the paediatric hospital of the University of Palermo during the time frame 1945-1960. Patients who developed PPS were identified through a structured questionnaire and a neurologic examination. PPS diagnosis was made according to specified diagnostic criteria. Frequency of PPS was calculated in the selected cohort of polio survivors. The association with the investigated risk factors (sex, age at onset of polio, extension and severity of polio, education, associated diseases, cigarette smoking, trauma, polio vaccination) was analysed by the calculation of the odds ratio. RESULTS: Forty-eight participants met the adopted diagnostic criteria for PPS, giving a prevalence of 31.0%. The prevalence rate was significantly higher in women than in men (p=0.02). Logistic regression analyses revealed a significant inverse association with onset of poliomyelitis at over 12 months of age (OR 0.33; CI 0.14-0.79) a higher degree of education (OR 0.20; CI 0.07-0.79), and a significant association with the presence of other diseases (OR 9.86; CI 3.69-26.34). CONCLUSIONS: In our survey one-third of patients with prior poliomyelitis had PPS. Higher age at onset of poliomyelitis is inversely associated with PPS. The association with other diseases may indicate that a chronic physical stress, particularly in already weak motor units, can contribute to the development of signs and symptoms of PPS. Our results also suggest the impact of socio-economic conditions on the risk of PPS.

Role of fatigue in limiting physical activities in humans with neuromuscular diseases.

New methods of examining both central and peripheral fatigue are now available. A broader understanding of the mechanisms of fatigue in healthy human subjects has begun to emerge. The mechanisms of fatigue in patients with various neuromuscular diseases are even more complex than in healthy persons. Examples of both central and peripheral fatigue in various neuromuscular diseases and other disorders are presented, including metabolic myopathy, chronic fatigue syndrome, postpolio syndrome, and amyotrophic lateral sclerosis.

[Belated diagnosis of medullar compression in a case of post-polio syndrome]. / Découverte tardive dune compression médullaire à loccasion de la prise en charge dun syndrome post-polio.

The physiatrist observes about his practice individuals with sequela of old poliomyelitics. A part of them have unusual fatigue and muscular pains and weakness. The hypothesis of an evolution of neuro-biological mechanism suggested by few authors isn't, actually, demonstrated. More probably, the modifications of lesional and, functional changes with disability observed are the consequence of elderly effects and decreasing of physical activites. We report a case of spinal cord compression by intramedullar tumor, associated with a post-polio syndrome.