Rett syndrome: clinical profile and EEG abnormalities.
We studied 7 children with Rett Syndrome, characterized by behavioral and motor abnormalities which superficially resemble infantile autism, and reviewed the records of 4 adults with the same symptoms. Clinical details are discussed. No abnormalities were found in analyses of amino acids and biogenic amine metabolites. EEG showed a clear pattern of chronological deterioration, with the pattern of marked waking/sleep background contrast appearing consistently only after 5 years of age. There was also a high incidence of spike discharges in the central (Rolandic) region. The clinical pattern of Rett Syndrome should be differentiated from the syndrome of autism.
Ho
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Department of Pediatrics, University of British Columbia, Children's Hospital, Canada.
Wong
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Robertson
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D000293:Adolescent; D001321:Autistic Disorder / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D001523:Mental Disorders / Q000503:physiopathology* / Q000523:psychology; D009422:Nervous System Diseases / Q000503:physiopathology* / Q000523:psychology; D013577:Syndrome