Discrepâncias no crescimento dos dimídios em crianças com paralisia cerebral hemiplégica: associação com a função, atividades e participação social

Objective: Evaluate side-to-side discrepancies in children with hemiplegic cerebral palsy (HCP), and investigate associations of these discrepancies with patients’ age at initiation of physical therapy, motor and cognitive function, and degree of activities and social participation. Method: We obtained eight side-to-side measurements from 24 HCP children with mean age 49.3±5.2 months. Results: Early initiation of physical therapy was associated with lower discrepancy in hand length (p=0.037). Lower foot length discrepancy was associated with lower requirement for caregiver assistance in activities related to mobility. Increased side-to-side discrepancy was associated with reduced wrist extension and increased spasticity. Discrepancy played a larger role in children with hemineglect and in those with right involvement. Conclusion: Increased discrepancy in HCP children was associated with reduced degree of activity/social participation. These results suggest an association between functional use of the extremities and limb growth.Objetivo: Avaliar a discrepância entre o crescimento dos lados do corpo em crianças com paralisia cerebral hemiplégica (PCH), e investigar sua associação com a idade de início do tratamento de fisioterapia, função motora e cognitiva, grau de atividades e participação social. Método: Comparamos oito medidas obtidas de 24 crianças com PCH e com média de idade de 49,3±5,2 meses. Resultados: O início precoce da fisioterapia se relacionou à menor discrepância no comprimento da mão (p=0,037). A menor discrepância no comprimento do pé se relacionou à menor necessidade de ajuda do cuidador em atividades de mobilidade. A maior discrepância esteve relacionada à menor extensão de punho e à maior espasticidade. A discrepância foi mais importante em crianças com heminegligência e com envolvimento à direita. Conclusão: Crianças com PCH com maior discrepância apresentaram menor atividade/participação social. Os resultados sugerem associação entre o uso funcional da mão e o crescimento das extremidades.CuritibaPRUniversidade Federal do Paraná Hospital de Clínicas Centro de NeuropediatriaSão PauloSPUniversidade Federal de São Paulo (UNIFESP)Universidade Federal do Paraná Departamento de PediatriaUniversidade Federal do Paraná Departamento de Clínica MédicaHospital Albert EinsteinUNIFESPSciEL

Neuroradiological Findings in the Spinocerebellar Ataxias

Background: The spinocerebellar ataxias (SCAs) are a group of autosomal dominant degenerative diseases characterized by cerebellar ataxia. Classified according to gene discovery, specific features of the SCAs – clinical, laboratorial, and neuroradiological (NR) – can facilitate establishing the diagnosis. The purpose of this study was to review the particular NR abnormalities in the main SCAs. Methods: We conducted a literature search on this topic. Results: The main NR characteristics of brain imaging (magnetic resonance imaging or computerized tomography) in SCAs were: (1) pure cerebellar atrophy; (2) cerebellar atrophy with other findings (e.g., pontine, olivopontocerebellar, spinal, cortical, or subcortical atrophy; “hot cross bun sign”, and demyelinating lesions); (3) selective cerebellar atrophy; (4) no cerebellar atrophy. Discussion: The main NR abnormalities in the commonest SCAs, are not pathognomonic of any specific genotype, but can be helpful in limiting the diagnostic options. We are progressing to a better understanding of the SCAs, not only genetically, but also pathologically; NR is helpful in the challenge of diagnosing the specific genotype of SCA