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 
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


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