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Rett syndrome and associated movement disorders
Author(s) -
FitzGerald Patricia M.,
Jankovic Joseph,
Percy Alan K.
Publication year - 1990
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.870050303
Subject(s) - movement disorders , parkinsonism , rett syndrome , dystonia , extrapyramidal disorder , chorea , myoclonus , gait disturbance , choreoathetosis , basal ganglia , dyskinesia , medicine , psychology , neurological disorder , basal ganglia disease , pediatrics , physical medicine and rehabilitation , central nervous system disease , psychiatry , neuroscience , disease , parkinson's disease , central nervous system , biochemistry , chemistry , gene
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.