Rett syndrome and associated movement disorders

Rett syndrome and associated movement disorders

FitzGerald P M; Jankovic J; Percy A K

Movement Disorders

1990

Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Movement Disorders/etiology/physiopathology; Rett Syndrome/complications/physiopathology; Stereotyped Behavior; tone and motor problems; Rett syndrome; trajectory; characteristics; stereotypy; gait disturbance

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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