Dopa-responsive dystonia: Clinical and family study in Taiwanese

被引：4

作者：

Chen, RS ^{[1
]}

Huang, CC ^{[1
]}

Lu, CS ^{[1
]}

机构：

[1] CHANG GUNG MEM HOSP & MED COLL,DEPT NEUROL,TAIPEI,TAIWAN

来源：

CLINICAL NEUROLOGY AND NEUROSURGERY | 1996年 / 98卷 / 01期

关键词：

dopa-responsive dystonia; Segawa disease; diurnal fluctuation; Parkinsonism; L-dopa; family survey;

D O I：

10.1016/0303-8467(95)00081-X

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

We report the clinical features of four female patients with dopa-responsive dystonia (DRD), and the survery of the family members. The patients were 2 sisters and 2 sporadic patients from 3 families. Their age of onset ranged between 5 and 13 years. The clinical manifestation was characterized by limb dystonia which was relieved by L-dopa treatment. Diurnal fluctuation disappeared 15 years later in one patient. There was a wide spectrum of Parkinsonian features and variability of dystonia. Response of L-dopa was still excellent 20 years later. In survey of the family members, there was neither bradykinesia, rigidity, tremor nor dystonia.

引用

页码：43 / 46

页数：4

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