title: Rett syndrome: EEG presentation.
abstract: Rett syndrome, a degenerative neurological disorder of girls, has a classical presentation and typical EEG findings. The electroencephalograms (EEGs) of 7 girls whose records have been followed from the onset of symptoms to the age of 5 or more are presented. These findings are tabulated with the Clinical Staging System of Hagberg and Witt-Engerström (1986). The records show a progressive deterioration in background rhythms in waking and sleep. The abnormalities of the background activity may only become evident at 4-5 years of age or during stage 2--the Rapid Destructive Stage. The marked contrast between waking and sleep background may not occur until stage 3--the Pseudostationary Stage. In essence EEG changes appear to lag behind clinical symptomatology by 1-3 years. An unexpected, but frequent, abnormality was central spikes seen in 5 of 7 girls. They appeared to be age related and could be evoked by tactile stimulation in 2 patients. We hypothesize that the prominent 'hand washing' mannerism may be self-stimulating and related to the appearance of central spike discharges.
authors: Department of Diagnostic Neurophysiology, British Columbia's Children's Hospital, Vancouver, Canada.
authors: R
authors: R
authors: Robertson
authors: L
authors: L
authors: Langill
authors: P K
authors: PK
authors: Wong
authors: H H
authors: HH
authors: Ho
mesh_terms: D001072:Apraxias / Q000503:physiopathology; D001259:Ataxia / Q000503:physiopathology; D001921:Brain / Q000503:physiopathology*; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000503:physiopathology; D004569:Electroencephalography; D005260:Female; D006257:Head / Q000473:pathology; D006801:Humans; D007223:Infant; D009422:Nervous System Diseases / Q000503:physiopathology*; D012890:Sleep; D013577:Syndrome