Amanda Madalena de Azevedo Fernandes, Dryele Cavalcanti Santa Cruz, Isabella Brito Gil Barros, Nicole Nunes Viana, Yara Siqueira Nascimento Melo, Mônica Soares de Albuquerque

Universidade Federal de Pernambuco, Recife, Pernambuco, Brasil.

American Journal of Geographical Research and Reviews

Introduction: Rett syndrome (RS) consists in a neurological severe disorder caused by a mutation in the X chromosome, mainly on the MECP2 gene, affecting the neuropsychomotor development. It is known too that the disease manifests, with higher indices in the female gender.
Objective: This work is designed to present, through a review of the literature, the general aspects about the Rett syndrome and their principal implications in the dentistry.
Methodology: In the present study was conducted a review of the literature with the clearance of information on the following database: Scielo, Periódicos CAPES and Google Acadêmico. There were selected eleven articles, six of these articles were review of literature and the others five were cases report.
Results: Besides the genetic linkage of the syndrome, there are factors which aggravate demonstrations on individual’s overall health, mainly lack of adequate dental care’s monitoring.
Conclusion: It is very important the frequency of dentistry ambulatory and clinical care to the patient with the syndrome, with the objective of promoting an oral health and prevent grievances to the general health, providing a better quality of life to this patients.

Keywords: rett syndrome, rare diseases, neuropsychomotor.

Free Full-text PDF

How to cite this article:
Amanda Madalena de Azevedo Fernandes, Dryele Cavalcanti Santa Cruz, Isabella Brito Gil Barros, Nicole Nunes Viana, Yara Siqueira Nascimento Melo, Mônica Soares de Albuquerque. RETT SYNDROME AND ITS IMPLICATIONS FOR DENTAL TREATAMENTS . International Journal of Dental Research and Reviews, 2019, 2:27.


1. Hagberg В., Aicardi J., Dias K., and Ramos O., 1983. 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.
2. Archer HL, Whatley SD, Evans JC, Ravine D, Huppke P, Kerr A, Bunyan D, Kerr B, Sweeney E, Davies SJ, Reardon W, Horn J, Macdermot KD, Smith
R A, Magee A, Donaldson A, Crowy, Hermon G, Miedzybrodzka Z, Cooper DN, Lazaoul, Butler R, Sampson J, Pilz DT, Laccone F, Clarke AJ. 2006. Gross rearrangements of the MECP2 gene are found in both classical and atypical Rett syndrome patients. J. Med. Genet. 43(5):451-456.
3. Calfa G, Percy AK, Pozzo L. 2011. Experimental models of Rett syndrome based on MECP2 dysfunction. Exp. Biol. Med. (Maywood). 236(1):3-19.
4. RSDCWG (RETT SYNDROME DIAGNOSTIC CRITERIA WORK GROUP). 1988. Diagnostic criteria for Rett syndrome. Ann. Neurol. 23(4):425-428.
5. Neul JL, Kaufmann WE, G Glase DD, Christodoulou J, Clarke AJ, Bahi-Buisson N, et al. Rett Syndrome: revised diagnóstic criteria and nomenclature. Ann Neurol. 2010;68(6):944-50.
6. Sigafoos J, Kagohara D, Meer L van der, Green VA, O’Reilly MF, Lancioni GE, et al. Communication assessment for individuals with Rett Syndrome: a systematic review. Res Autism Spectr Disord. 2011;5(2):692- 700.
7. Lozano MPF, Ferreras AP, Gomariz MJB, Bogdanovitch AP. Rasgos conductales y cognitivos de los Síndrome Rett, Cri-duchat, X-frágil y Williams. Liberabit. 2010;16(1):39- 50.)
8. Silva Natalia et al. Sindrome de rett: uma revisão de literatura, J Health Sci Inst. 2016;34(1):53-7
9. Lotan M et al. functional skills and physical fitness in children with Rett Syndrome. J Intellect Disabil Res. 2004;48(Pt8):730-5
10. Fernandes, Carlos et al. SINDROME DE RETT, Escola Superior de Educação Almeida Garrett, Lisboa, 2011
11. Ribeiro RA, Romano AR, Birman EG, Mayer MP. Oral manifestations in Rett syndrome: a study of 17 cases. Pediatr Dent. 1997;19(5):349-52.
12. Magalhães MHCG, Kawamura JY, Araújo LCA. General and oral characteristics in Rett syndrome. Spec Care Dentist. 2002 Jul-Aug;22(4):147-50.
13. Barbosa RC, Giacheti CM. Síndrome de Rett: Considerações Gerais, Gênese Etiológica e Tratamento. RECCS: R. Cent. Ci. Saúde, Fortaleza. 2001 Dez;14:36-43.
14. Fuertes-González MC, Javier Silvestre F, Almerich-Silla JM. Oral findings in Rett syndrome: A systematic review of the dental literature. Med Oral Patol Oral Cir Bucal. 2011 Jan;1;16 (1):37-41. doi:10.4317/medoral.16.e37.
15. Fuertes-González MC, Javier Silvestre F. 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 Nov 1;19 (6):598-04.
16. Nho JS, Shin DS, Moon JY, Yi JW, Kang JM, Lee BJ, Kim DO, Chung JY. Anesthetic management of an adult patient with Rett syndrome and limited mouth opening-A case report. Korean J Anesthesiol. 2011 Nov;61(5):428-430. Report kjae.2011.61.5.428.
17. Motil KJ, Caeg E, Barrish JO, Geerts S, Lane JB, Percy AK, et al. Gastrointestinal and nutritional problems occur frequently throughout life in girls and women with Rett Syndrome. J Pediatr Gastroenterol Nutr. 2012;55(3):292-8.
18. Portolan Camila et al. , odontologia e pacientes especiais: conhecer, orientar e prevenir REVISTA SAÚDE INTEGRADA, v. 10, n. 20 (2017) Edição Especial – ISSN 2447-7079
19. Kaufmann W.E., Tierney E., Rohde C.A., Suarez-Pedraza M.C., Clarke M.A., Salorio C.F. Social impairments in Rett syndrome: Characteristics and relationship with clinical severity. Journal of Intellectual Disability Research. 2012;56:233–247.

Terms of Use/Privacy Policy/ Disclaimer/ Other Policies:
You agree that by using our site, you have read, understood, and agreed to be bound by all of our terms of use/privacy policy/ disclaimer/ other policies (click here for details).

This work and its PDF file(s) are licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.