RETT SYNDROME : CASE REPORT

Kong, Eun Kyung; Hong, So Yi; Ma, Yon Joo; Ahn, Byung Duk; Jung, Young Jung

Journal of the Korean Academy of Pediatric Dentistry 2011;38(1):68-74.
Published online February 28, 2011.

RETT SYNDROME : CASE REPORT

Eun Kyung Kong, So Yi Hong, Yon Joo Ma, Byung Duk Ahn, Young Jung Jung

Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital

Rett syndrome 환자의 증례보고

공은경, 홍소이, 마연주, 안병덕, 정영정

이화여자대학교 목동병원 소아치과

Correspondence: Young Jung Jung, Tel: 02-2650-2660, Email: tetra0@hanmail.net

Received: 15 October 2010 • Accepted: 18 December 2010

Abstract

Rett syndrome is a X-linked genetic neurological disorder characterized by developmental regression, particularly in relation to expressive language and use of the hands, together with profound mental retardation, that almost exclusively affects females. Oral manifestations of the disease are commonly associated with the clinical conditions such as convulsion activity, difficulties for oral hygiene behavior, walking problems and oral/digitalmanual habits. Bruxism is the most frequently observed oral habit in patients with Rett syndrome. Two cases with the Rett syndrome were reported. Both patients had the typical manifestations like stereotypic hand movement, bruxism and digit-hand sucking. Caries control for the patients was perfomed under general anesthesia.

Key Words: Rett syndrome, Stereotypic hand movement, Bruxism, General anesthesia