Yonsei Med J.  1991 Jun;32(2):172-178. 10.3349/ymj.1991.32.2.172.

RETT's syndrome in Korea: report of two cases

Affiliations
  • 1Department of Neurology, Yonsei University College of Medicine, Seoul, Korea.

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.

Keyword

Rett's syndrome; haloperidol

MeSH Terms

Child
Child Development/physiology
Female
Haloperidol/therapeutic use
Human
Korea/epidemiology
Rett Syndrome/drug therapy/*epidemiology/physiopathology
Sex Factors
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