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Neuropediatrics 1989; 20(4): 192-195
DOI: 10.1055/s-2008-1071290
Original article

© Georg Thieme Verlag KG Stuttgart · New York

Rett Syndrome: An EEG Study in 52 Girls

Stephanie A. Robb , Ann  Harden , S. G. Boyd
  • The Hospital for Sick Children, Great Ormond Street, London, WC1N 3JH, Great Britain
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Publication History

Publication Date:
19 March 2008 (online)

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Abstract

EEG studies have been carried out on 52 girls with Rett syndrome, the majority of records being taken between two and 7 years of age. Discharges were a common feature, occurring in 43 patients, and did not appear to be related to the onset of seizures. The discharges, consisting of sharp waves or spikes, were characteristically most prominent around the middle third of the head, often occurring asymmetrically and could be infrequent or almost continuous. They were usually enhanced by light sleep and were seen only during sleep in 15 EEGs taken in 13 patients, most of whom were under four years of age.

These EEG features when present may help confirm the diagnosis of Rett syndrome in the appropriate clinical setting and in particular are quite distinct from the usual EEG patterns seen in Angelman (Happy Puppet) syndrome.

Key words

Rett syndrome - Neurodegenerative disorders - EEG features