The motor and behavioral findings in 32 patients with Rett syndrome aged 21 months to 30 years, are reported from the Departments of Neurology and Pediatrics, Baylor College of Medicine, Houston, TX. Hand stereotypies and gait abnormalities were present in all patients. Clapping, wringing, and clenching were the most common, followed by washing, patting, and rubbing movements. Gait ataxia was present in 31%, a broad based gait in 13%, and inability to walk in 28%. Bruxism was the next most common involuntary movement (97%) and occurred only when awake. Drooling occurred in 75%. Other motor disturbances included ocular deviations (63%), parkinsonian rigidity (44%), bradykinesia (41%), dystonia (59%), sometimes focal and sometimes associated with scoliosis (50%). Myoclonus, choreoathetosis and intention tremor also occurred. Hyperkinetic disorders were prominent in younger patients and bradykinetic disorders occurred more frequently in older patients. [1]
COMMENT. The number of cases and the plethora of published reports on Rett syndrome add credence to the viral infectious cause postulated by Hagberg (see Ped Neur Briefs 1989; 3:44). A toxic environmental cause might also be considered and pursued further.