Rett syndrome and associated movement disorders

Title

Rett syndrome and associated movement disorders

Creator

FitzGerald P M; Jankovic J; Percy A K

Publisher

Movement Disorders

Date

1990

Subject

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

Description

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.

Rights

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Citation

FitzGerald P M; Jankovic J; Percy A K, “Rett syndrome and associated movement disorders,” Pediatric Palliative Care Library, accessed May 23, 2024, https://pedpalascnetlibrary.omeka.net/items/show/16652.