Journal of the Korean Pediatric Society 1994;37(5):718-725.
Published online May 15, 1994.
A Case of Rett Syndrome Observed with Video-EEG Monitoring
Hyun Mi Kim, Young Ah Lee, TaecSung Ko, Hyung Nam Moon, Chang Yee Hong
Department of Pediatrics, College of Medicine University of Ulsan, Seoul, Korea
비디오-뇌파 감시기로 관찰한 Rett 증후군 1례
김현미, 이영아, 고태성, 문형남, 홍창의
울산대학교 의과대학 소아과
Abstract
Rett syndrome is progressive neurodegenerative disorder in female patients, characterized by autistic behavior, mental retardation, loss of purposeful hand skills, sterotypic hand movement, breathing dysfunction, severely impaired language, ataxia, and seizure. The diagnosis of Rett syndrome is based on its characteristic clinical manifestation and course. The electroencephalographic(EEG) findings of Rett syndrome are nonspecific, but a progressive deteriorationin the EEF, characterized by a slowing of background activity and spike sharp wave discharges, may be observed. We experienced one case of Rett syndrome in a 5 year old girl having mental retardation, loss of purposeful hand skills, stereotypic hand movements (clapping, washing, hand-on-mouth), breathing dysfunction (hyperventilation/apnea). Her EEG findings on Video-EEG monitoring are excessive slowing waves during awake state and frequent spike discharges from left or centrotemporal area during sleeping. We report a case of Rett syndrome with brief review of related literatures.
Key Words: Rett syndrome, Video-EEG monitoring


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