1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-4-15 10.15844/pedneurbriefs-4-2-9 Degenerative Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Motor Disorders in Rett Syndrome http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 02 1990 01 08 2016 4 2 15 15 Copyright: © 1990 The Author(s) 1990 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Extrapyramidal involvement in Rett’s syndrome

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.

 Rett Syndrome Gait Ataxia Bradykinetic Disorders

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.

 FitzGerald PM Jankovic J Glaze DG Schultz R Percy AK Extrapyramidal involvement in Rett’s syndrome Neurology 1990 Feb 40 2 293 295 10.1212/WNL.40.2.293 2073236