ADULT ONSET MYOCLONIC HUNTINGTONS-DISEASE

被引：28

作者：

CARELLA, F

SCAIOLI, V

CIANO, C

BINELLI, S

OLIVA, D

GIROTTI, F

机构：

[1] IST NAZL NEUROL C BESTA,PRIMA DIV NEUROL,VIA CELORIA 11,I-20133 MILAN,ITALY

[2] IST NAZL NEUROL C BESTA,SERV NEUROFISIOL,I-20133 MILAN,ITALY

来源：

MOVEMENT DISORDERS | 1993年 / 8卷 / 02期

关键词：

HUNTINGTONS DISEASE; MYOCLONUS;

D O I：

10.1002/mds.870080216

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

A patient with adult onset Huntington's disease (HD) and prominent action myoclonus is described. Neither epileptiform activity nor electroencephalography (EEG) correlates of the movements was found. Unlike the case with most (nonmyoclonic) HD patients, centro-parietal components of somatosensory evoked potentials (SEPs) were well defined and a clear V2 response was found. Treatment with valproic acid greatly reduced myoclonus suggesting that the gamma-aminobutyric acid (GABA) system might be involved in the pathophysiology of myoclonus in HD.

引用

页码：201 / 205

页数：5

共 30 条

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