NEUROMYOTONIA IN HEREDITARY MOTOR NEUROPATHY

被引:50
作者
HAHN, AF
PARKES, AW
BOLTON, CF
STEWART, SA
机构
[1] UNIV WESTERN ONTARIO,DEPT CLIN NEUROL SCI,LONDON N6A 3K7,ONTARIO,CANADA
[2] ST JOSEPHS HOSP,LONDON N6A 4V2,ONTARIO,CANADA
关键词
D O I
10.1136/jnnp.54.3.230
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Two siblings with a distal motor neuropathy experienced cramping and difficulty in relaxing their muscles after voluntary contraction. Electromyographic recordings at rest revealed repetitive high voltage spontaneous electrical discharges that were accentuated after voluntary contraction and during ischaemia. Regional neuromuscular blockage with curare indicated hyperexcitability of peripheral nerve fibres and nerve block suggested that the ectopic activity originated in proximal segments of the nerve. Symptoms were improved with diphenylhydantoin, carbamazepine and tocainide.
引用
收藏
页码:230 / 235
页数:6
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