RETT SYNDROME AND ASSOCIATED MOVEMENT-DISORDERS

被引：90

作者：

FITZGERALD, PM ^{[1
]}

JANKOVIC, J ^{[1
]}

PERCY, AK ^{[1
]}

机构：

[1] BAYLOR UNIV,DEPT NEUROL,6550 FANNIN 1801,HOUSTON,TX 77030

来源：

MOVEMENT DISORDERS | 1990年 / 5卷 / 03期

关键词：

Bruxism; Chorea; Dystonia; Gait disturbance; Myoclonus; Oculogyric crisis; Parkinsonism; Rett syndrome; Stereotypies;

D O I：

10.1002/mds.870050303

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

Rett syndrome, a progressive neurodegenerative disorder described only in female subjects, is manifested by a wide spectrum of behavioral and motor abnormalities. We studied 32 patients with this disorder, ages 30 months to 28 years old, and characterized their extrapyramidal disturbance. The most common motor abnormalities were stereotyped movements and gait disturbance, seen in all patients. Bruxism, oculogyric crises, parkinsonism, and dystonia were also common, but myoclonus and choreoathetosis were seen only infrequently. The hyperkinetic movement disorders tended to dominate in younger patients, while bradykinetic disorders were more evident in the older patients. This study provides evidence that movement disorders seen in Rett syndrome reflect age‐related neurodegenerative changes in the basal ganglia. Copyright © 1990 Movement Disorder Society

引用

页码：195 / 202

页数：8

共 34 条

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