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http://dx.doi.org/10.5933/JKAPD.2011.38.1.068

RETT SYNDROME : CASE REPORT  

Kong, Eun-Kyung (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital)
Hong, So-Yi (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital)
Mah, Yon-Joo (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital)
Ahn, Byung-Duk (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital)
Jung, Young-Jung (Division of Pediatric Dentistry, Department of Dentistry, Ewha Womans University Mokdong Hospital)
Publication Information
Journal of the korean academy of Pediatric Dentistry / v.38, no.1, 2011 , pp. 68-74 More about this Journal
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.
Keywords
Rett syndrome; Stereotypic hand movement; Bruxism; General anesthesia;
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Times Cited By KSCI : 2  (Citation Analysis)
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1 Ribeiro RA, Romano AR, Birman EG, Mayer MP: Oral manifestations in Rett syndrome: a study of 17 cases. Pediatr Dent, 19:349-352, 1997.
2 Magalhães MH, Kawamura JY, Araujo LC: General and oral characteristics in Rett syndrome. Spec Care Dentist, 22:147-150, 2002.   DOI
3 Friedlander AH, Yagiela JA, Paterno VI, Mahler ME: The pathophysiology, medical management and dental implications of fragile X, Rett, and Prader- Willi syndromes. J Calif Dent Assoc, 31:693-702, 2003.
4 Hagberg B, Aicardi J, Dias K, Ramos O: A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases. Ann Neurol, 14: 471-479, 1983.   DOI
5 Raizis AM, Saleem M, MacKay R, George PM: Spectrum of MECP2 mutations in New Zealand Rett syndrome patients. N Z Med J, 122: 21-28, 2009.
6 Pineda M, Aracil A, Vernet A, et al.: Rett's syndrome in the Spanish population. Rev Neurol, 28:105-109, 1999.
7 Orrico A, Lam C, Galli L, et al.: MECP2 mutation in male patients with non-specific X-linked mental retardation. FEBS Lett, 481:285-288, 2000.   DOI
8 Buccino MA, Weddell JA: Rett syndrome-a rare and often misdiagnosed syndrome: case report. Pediatr Dent, 11:151-157, 1989.   DOI
9 박성진, 이광희, 김대업: Rett syndrome 환자의 제증상에 관한 증례보고. 대한소아치과학회지, 31:131-135, 2004.   과학기술학회마을
10 Hagberg B, Hanefeld F, Percy A, Skjeldal O: An update on clinically applicable diagnostic criteria in Rett syndrome. Comments to Rett Syndrome Clinical Criteria Consensus Panel Satellite to European Paediatric Neurology Society Meeting, Baden Baden, Germany, 11 September 2001. Eur J Paediatr Neurol, 6:293-297, 2002.   DOI
11 Percy AK, Zoghbi HY, Lewis KR, Jankovic J: Rett syndrome: qualitative and quantitative differentiation from autism. J Child Neurol, 3:S65-S67, 1988.   DOI
12 황정환, 이긍호, 최영철: 레트 증후군 환아의 제증상에 관한 증례보고. 대한소아치과학회지, 25:837-842, 1998.   과학기술학회마을
13 Dunn HG: Importance of Rett syndrome in child neurology. Brain Dev, 23:S38-S43, 2001.   DOI
14 Rett A: On a unusual brain atrophy syndrome in hyperammonemia in childhood. Wien Med Wochenschr, 116: 723-726, 1966.
15 Chahrour M, Zoghbi HY: The story of Rett syndrome: from clinic to neurobiology. Neuron, 56: 422-437, 2007.   DOI