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.

Effects of early spasticity treatment on children with hemiplegic cerebral palsy: a preliminary study.

OBJECTIVE: To compare motor and functional performance of two groups of children with hemiplegic cerebral palsy (HCP). Only the study group (SG) received early treatment of spasticity with botulinum neurotoxin type A (BXT-A). METHODS: Gross Motor Function Measure (GMFM), functional performance (Pediatric Evaluation of Disability Inventory - PEDI), range of movement, gait pattern (Physician Rating Scale - PRS) and the speed of hand movements were considered. RESULTS: The SG, composed of 11 HCP (45.64±6.3 months), was assessed in relation to the comparison group, composed of 13 HCP (45.92±6.4 months). SG showed higher scores in four of the five GMFM dimensions, which included scores that were statistically significant for dimension B, and higher scores in five of the six areas evaluated in the PEDI. Active wrist extension, the speed of hand movements and PRS score were higher in the SG. CONCLUSION: Children who received early BXT-A treatment for spasticity showed higher scores in motor and functional performance.

Effects of early spasticity treatment on children with hemiplegic cerebral palsy: a preliminary study / Efeitos do tratamento precoce da espasticidade em criancas com paralisia cerebral hemiplegica: um estudo preliminar

Objective To compare motor and functional performance of two groups of children with hemiplegic cerebral palsy (HCP). Only the study group (SG) received early treatment of spasticity with botulinum neurotoxin type A (BXT-A). Methods Gross Motor Function Measure (GMFM), functional performance (Pediatric Evaluation of Disability Inventory - PEDI), range of movement, gait pattern (Physician Rating Scale - PRS) and the speed of hand movements were considered. Results The SG, composed of 11 HCP (45.64±6.3 months), was assessed in relation to the comparison group, composed of 13 HCP (45.92±6.4 months). SG showed higher scores in four of the five GMFM dimensions, which included scores that were statistically significant for dimension B, and higher scores in five of the six areas evaluated in the PEDI. Active wrist extension, the speed of hand movements and PRS score were higher in the SG. Conclusion Children who received early BXT-A treatment for spasticity showed higher scores in motor and functional performance. .

Objetivo Comparar a performance motora e funcional de dois grupos de crianças com paralisia cerebral hemiplégica (PCH). Apenas o grupo de estudo (GE) recebeu tratamento precoce da espasticidade com toxina botulínica do tipo A (BXT-A). Métodos Foram considerados a Função Motora Grossa (Gross Motor Function Measure - GMFM), performance funcional (Pediatric Evaluation of Disability Inventory - PEDI), amplitude de movimento, padrão da marcha (Physician Rating Scale - PRS) e a velocidade de movimento das mãos. Resultados O GE, composto de 11 PCH (45,64±6,3 meses), foi analisado em relação ao grupo de comparação, composto por 13 PCH (45,92±6,4 meses). O GE mostrou maiores escores em quatro das cinco dimensões da GMFM, sendo a diferença estatisticamente significativa na dimensão B, e melhores escores em cinco das seis áreas avaliadas na PEDI. A extensão ativa do punho, a velocidade de movimento das mãos e o escore na PRS foram maiores no GE. Conclusão As crianças que receberam tratamento precoce da espasticidade com BXT-A mostraram melhores escores motores e funcionais. .