Dental and Medical Problems
2018, vol. 55, nr 4, October-December, p. 441–445
Publication type: review article
Oral findings in Rett syndrome: An update and review of the literature
Zmiany w jamie ustnej w zespole Retta – przegląd współczesnego piśmiennictwa
1 Department of Surgery and Translational Medicine, University of Milano-Bicocca, Milan, Italy
Rett syndrome is a progressive pediatric neurodevelopmental disorder, predominantly affecting females, characterized by a seemingly normal prenatal and perinatal period, followed by neurodevelopmental stagnation, and then rapid regression.
The purpose of this study was to provide an update of the literature on the oral aspects of Rett syndrome and their possible treatment in patients suffering from this pathology. After an electronic and manual search in MEDLINE (PubMed) and the Cochrane Library, 12 articles were found, for a total of 142 patients affected by Rett syndrome. A high prevalence of bruxism, anterior open bite, ogival palate, sucking habits, and difficulties in maintaining oral hygiene was noted. There were also oral findings related to the pharmacological treatment, which included xerostomia, glossitis, erythema multiforme, gingival hyperplasia, dysphagia, and lingual paralysis. It is important for the dentist to know what problems related to the oral cavity can be encountered in a patient diagnosed with Rett syndrome and what preventive measures can be applied.
autism, bruxism, Rett syndrome, oral manifestations
autyzm, bruksizm, zespół Retta, objawy w jamie ustnej
- Chattopadhyay S, Arora R. The ironies of human mind: A case of Rett syndrome. Ethiop J Health Sci. 2014;24:171–174.
- Cianfaglione R, Clarke A, Kerr M, Hastings RP, Oliver C, Felce D. Ageing in Rett syndrome. J Intellect Disabil Res. 2016;60:182–190.
- Ribeiro RA, Romano AR, Birman EG, Mayer MP. Oral manifestations in Rett syndrome: A study of 17 cases. Pediatr Dent. 1997;19:349–352.
- Janas A, Osica P. Dental issues in Rett syndrome. Dev Period Med. 2015;19:478–481.
- Hagberg B, Hanefeld F, Percy A, Skjeldal O. An update on clinically appreciable 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. 2002;6:293–297.
- Amir RE, Van den Veyver IB, Wan M, Tran CQ, Francke U, Zoghbi HY. Rett syndrome is caused by mutations in X-linked MECP2, encoding methyl-CpG-binding protein. Nat Genet. 1999;23:185–188.
- Bienvenu T, Chelly J. Molecular genetics of Rett syndrome: When DNA methylation goes unrecognized. Nat Rev Genet. 2006;7:415–426.
- Matsuishi T, Yamashita Y, Takahashi T, Nagamitsu S. Rett syndrome: The state of clinical and basic research, and future perspectives. Brain Dev. 2011;33:627–631.
- Di Bona MC. Hospital dentistry for a child with Rett’s syndrome. Spec Care Dent. 1985;5:62–63.
- Pagnacco A, Ferrara M, Vangelisti R, Randon C. Rett syndrome: A “new” and rare disease. A clinical case [in Italian]. Dent Cadmos. 1988;56:93–95.
- Coleman M, Brubaker J, Hunter K, Smith G. Rett syndrome: A survey of North American patients. J Ment Defic Res. 1988;32:117–124.
- Buccino MA, Weddell JA. Rett syndrome – a rare and often misdiagnosed syndrome: Case report. Pediatr Dent. 1989;11:151–157.
- Peak J, Eveson JW, Scully C. Oral manifestation of Rett’s syndrome. Br Dent J. 1992;172:248–249.
- Magalhães MH, Kawamura JY, Araújo LC. General and oral characteristics in Rett syndrome. Spec Care Dent. 2002;22:147–150.
- Alpoz AR, Ergul N, Oncag O. Bruxism in Rett syndrome: A case report. J Clin Pediatr Dent. 1999;23:161–163.
- Bathla M, Chandna S, Bathla JC. Rett’s syndrome: Diagnostic and therapeutic dilemma. Ger J Psych. 2010;13:157–160.
- Fuertes-González MC, Silvestre FJ. Oral health in a group of patients with Rett syndrome in the regions of Valencia and Murcia (Spain): A case-control study. Med Oral Patol Oral Cir Bucal. 2014;19:e598–e604.
- Fuertes-González MC, Silvestre FJ, Almerich-Silla JM. Oral findings in Rett syndrome: A systematic review of the dental literature. Med Oral Patol Oral Cir Bucal. 2011;16:e37–e41.
- 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. 2003;31:693–702.