ELECTROMYOGRAPHY AND NERVE-CONDUCTION STUDIES IN FRIEDREICHS ATAXIA AND AUTOSOMAL RECESSIVE SPASTIC ATAXIA OF CHARLEVOIX-SAGUENAY (ARSACS)

被引:40
作者
BOUCHARD, JP
BARBEAU, A
BOUCHARD, R
BOUCHARD, RW
机构
[1] CLIN RES INST MONTREAL, 110 PINE AVE W, MONTREAL H2W 1R7, QUEBEC, CANADA
[2] HOP ENFANT JESUS, QUEBEC CITY, QUEBEC, CANADA
关键词
D O I
10.1017/S0317167100119614
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Twenty four ataxie patients were investigated with electromyography and nerve conduction studies. They were divided in two groups according to the area they came from, the evolution of the disease, and the clinical signs. Group I patients from the Rimouski area displayed all the clinical and electrophysiological signs of Friedreich's ataxia. Group II comprised patients who presented with a new syndrome known as the autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Although the clinical evolution was better in the latter, there were more electromyographic signs of denervation and the motor conduction velocities were slower. Both groups showed identical and important abnormalities in sensory nerve conduction. The results of electrophysiological studies in spastic ataxia have not been reported to our knowledge. They underline the place of spastic ataxia as distinct f rom Friedreich's ataxia, spastic paraplegia, and the known familial neuropathies. © 1979, Canadian Neurological Sciences Federation. All rights reserved.
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页码:185 / 189
页数:5
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