Yonsei Medical Journal  1991;32(2):172-178

doi:10.3349/ymj.1991.32.2.172

RETT's syndrome in Korea: report of two cases.

Young Chul CHOI 1 ; Byung In LEE ; Kyoon HUH ; Gyung Whan KIM

Affiliations

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Keywords

Rett's syndrome; haloperidol

Country

Republic of Korea

Language

English

Abstract

Rett's syndrome(RS) is a progressive neurodegenerative disorder characterized by exclusive occurrence in females, autistic behavior, dementia, gait ataxia, loss of purposeful use of the hands with stereotypic hand movement, and seizures. Initially RS was considered to be very rare; however, recent reports suggest that the prevalence is considerably higher and occurrence is world-wide. Because the pathophysiological process remains unknown, the diagnosis of RS is based mainly on its characteristic clinical features and course. We experienced two cases of RS which, to our knowledge, are the first reported in Korea. It is quite possible that many patients with RS not yet being diagnosed in Korea.