CORTICAL MYOCLONUS IN HUNTINGTONS-DISEASE

被引：40

作者：

THOMPSON, PD

BHATIA, KP

BROWN, P

DAVIS, MB

PIRES, M

QUINN, NP

LUTHERT, P

HONOVAR, M

OBRIEN, MD

MARSDEN, CD

HARDING, AE

机构：

[1] UNIV LONDON, INST NEUROL, DEPT CLIN NEUROL, LONDON WC1N 3BG, ENGLAND

[2] INST NEUROL, MRC, HUMAN MOVEMENT & BALANCE UNIT, LONDON WC1N 3BG, ENGLAND

[3] INST NEUROL, DEPT NEUROPATHOL, LONDON WC1N 3BG, ENGLAND

[4] INST PSYCHIAT, DEPT NEUROPATHOL, LONDON SE5 8AF, ENGLAND

[5] GUYS HOSP, DEPT NEUROL, LONDON SE1 9RT, ENGLAND

来源：

MOVEMENT DISORDERS | 1994年 / 9卷 / 06期

关键词：

CORTICAL MYOCLONUS; HUNTINGTONS DISEASE;

D O I：

10.1002/mds.870090609

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

We describe three patients with Huntington's disease, from two families, in whom myoclonus was the predominant clinical feature. The diagnosis was confirmed at autopsy in two cases and by DNA analysis in all three. These patients all presented before the age of 30 years and were the offspring of affected fathers. Neurophysiological studies documented generalised and multifocal action myoclonus of cortical origin that was strikingly stimulus sensitive, without enlargement of the cortical somatosensory evoked potential. The myoclonus improved with piracetam therapy in one patient and a combination of sodium valproate and clonazepam in the other two. Cortical reflex myoclonus is a rare but disabling component of the complex movement disorder of Huntington's disease, which may lead to substantial diagnostic difficulties.

引用

页码：633 / 641

页数：9

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