Medida da função motora: versão da escala para o português e estudo de confiabilidade / Motor function measure scale: portuguese version and reliability analysis

CONTEXTUALIZAÇÃO: Instrumentos de avaliação funcional de pacientes com doenças neuromusculares são escassos. A escala Medida da Função Motora (MFM) está disponível no original francês e nas versões inglesa e espanhola. OBJETIVOS: Realizar a versão da escala para o português e identificar a confiabilidade de sua aplicação intra e interexaminador. MATERIAIS E MÉTODOS: Duas traduções da MFM de 2004 foram realizadas em separado, por neurologistas proficientes na língua francesa, resultando em texto consensual, após avaliação dos autores. A escala em português foi aplicada em 58 pacientes de seis a 61 anos, com diagnósticos clínico-laboratoriais de variados tipos de distrofias musculares e miopatias congênitas, documentados em vídeo. O primeiro autor realizou o teste e reteste e outros três fisioterapeutas analisaram os mesmos vídeos para confiabilidade interexaminador. Para análises estatísticas foram utilizados os coeficientes de Kendall, Kappa e Pearson. RESULTADOS: Apresenta-se a escala em seus 32 itens e três dimensões. Os coeficientes de concordância de Kendall para a análise interexaminador e os coeficientes Kappa e de Pearson para o teste e reteste foram estatisticamente significativos (p-valor<0,0001) nos 32 itens da escala e no escore total. CONCLUSÕES: A versão portuguesa da MFM mostrou confiabilidade e mínima variabilidade na sua aplicação, podendo ser utilizada como instrumento de diagnóstico clínico e seguimento das doenças neuromusculares. A alta confiabilidade na aplicação da MFM permitirá incluir pacientes brasileiros em ensaios clínicos internacionais que utilizarão a escala.

BACKGROUND: Functional evaluation instruments for patients with neuromuscular disorders are rare. The Motor Function Measure (MFM) scale is available in the original French and in English and Spanish translations. OBJECTIVE: To make a Portuguese translation of the MFM and to identify its intra and inter-examiner reliability. METHODS: Two translations of the 2004 MFM were produced separately by neurologists who were proficient in French. This procedure resulted in a consensual text after evaluation by the authors. The MFM in Portuguese was applied to 58 patients aged six to 61 years, with clinical and laboratory diagnoses of various types of muscular dystrophy and congenital myopathy that were documented on video. The first author performed the test and retest and another three physical therapists analyzed the same videos to assess the inter-examiner reliability. Statistical analyses were performed using the Kendall, kappa and Pearson coefficients. RESULTS: The scale is presented with its 32 items and three dimensions. The Kendall concordance coefficients for inter-examiner analysis and the kappa and Pearson coefficients for the test-retest comparison were statistically significant (p-value<0.0001) for the 32 items on the scale and for the total score. CONCLUSIONS: The Portuguese version of the MFM showed high reliability and minimal variability when it was applied. It can be used as an instrument for clinical diagnosis and follow-up of neuromuscular disorders. The high reliability in applying the MFM will allow Brazilian patients to be included in international clinical trials that use this scale.

Guillain-Barré syndrome in the elderly: clinical, electrophysiological, therapeutic and outcome features

There are few papers devoted to geriatric Guillain-Barré (GBS) and many related issues remain unanswered. OBJECTIVE: To describe clinical, electrophysiological and therapeutic features in this age. METHOD: Clinico-epidemiological data and therapy of GBS patients older than 60 years were reviewed. Hughes scores were used to quantify neurological deficit and define outcome. RESULTS: Among 18 patients (mean age 64.8 years), 9 had evident prodrome and 80% noticed initially sensory-motor deficit. Demyelinating GBS was found in 8 and axonal in 6 subjects. There was one Miller-Fisher and 3 unclassified cases. Plasmapheresis (PFX) was single therapy in 12 patients and intravenous immunoglobulin (IVIg) in 2. Disability scores just before therapy were similar in both groups, so as short and long term outcome. CONCLUSION: Axonal GBS seems to be more frequent in the elderly and this may have prognostic implications. PFX and IVIg were suitable options, but complications were noticed with PFX. Prospective studies are needed to better understand and manage GBS in the elderly.

Publicações sobre a síndrome de Guillain-Barré (SGB) no idoso são escassas e várias questões sobre o tema estão abertas. OBJETIVO: Descrever aspectos clínico-eletrofisiológicos, terapêuticos e prognóstico no idoso. MÉTODO:Revisamos os prontuários de pacientes acima de 60 anos com SGB. A escala de Hughes foi usada para quantificar os déficits iniciais e finais. RESULTADOS: No total de 18 pacientes (média de idade 64,8 anos), 50% tiveram pródromo e 80% tiveram déficit sensitivo-motor no início. SGB desmielinizante foi encontrada em 8 pacientes, axonal em 6 e uma síndrome de Miller-Fisher. Três casos não puderam ser classificados. Plasmaférese (PFX) foi empregada isoladamente em 12 pacientes e imunoglobulina endovenosa (IVIg) em 2. A disfunção inicial nos dois grupos tratados era semelhante, assim como a evolução a curto e longo prazo. CONCLUSÃO: A forma axonal da SGB parece ser mais freqüente no idoso e isto pode ter implicações prognósticas. PFX e IVIg foram eficazes, mas complicações ocorreram apenas no grupo tratado com PFX. Estudos prospectivos são necessários para um melhor entendimento e manejo da SGB no idoso.

Electrophysiological evaluation in myotonic dystrophy: correlation with CTG length expansion.

In myotonic dystrophy (MD), disease severity has been correlated with expansion of CTG repeats in chromosome 19. The aims of this study were to evaluate efficacy of electromyography in the diagnosis of MD, access the frequency and the characteristics of peripheral involvement in the disease and to verify whether the CTG repeats correlated with the electrophysiological abnormalities. Twenty-five patients and six relatives at risk of carrying the MD gene were examined. Electrical myotonia (EM) was scored. Sensory and motor conduction velocity (CV) were studied in five nerves. Leukocyte DNA analysis was done in 26 subjects. Myopathy and myotonia were found in 27 cases. EM was most frequent in muscles of hand and in tibialis anterior. No significant correlation was found between EM scores and length of CTG expansions. EM scores correlated significantly with the degree of clinical myopathy, expressed by a muscular disability scale. Peripheral neuropathy was found in eight subjects and was not restricted to those who were diabetics.