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Rett syndrome and associated movement disorders

✍ Scribed by Patricia M. FitzGerald; Dr. Joseph Jankovic; Alan K. Percy

Publisher
John Wiley and Sons
Year
1990
Tongue
English
Weight
894 KB
Volume
5
Category
Article
ISSN
0885-3185

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✦ Synopsis

Abstract

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.

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