Oral health in a group of patients with Rett syndrome in the regions of Valencia and Murcia (Spain): a case-control study.

Mc Fuertes-Gonzalez,Fj Silvestre

doi:10.4317/medoral.19743

Abstract

Objectives: Rett syndrome (RS) is a rare disease with oral manifestations that have not been described in detail or in a standardized manner in the literature. The present study describes the oral health of the population with RS in two Spanish regions, following the protocol of the World Health Organization for conducting common oral health surveys. Study Design: A prospective, observational case-control study was carried out, involving a group of patients with RS (n1=41) and a mean age of 13.37±3.19 years, and an age- and gender-matched control group without RS (n0=82). The data referred to oral health and habits were recorded by means of a questionnaire and oral examination was used to document caries indicators (prevalence of caries, df(t), df(s), DMF(T), DMF(S) and indices referred to dental loss, morbidity, restoration), the Community Periodontal Index (CPI), and the most characteristic oral manifestations. Results: The most frequent oral habit in the patients with RS was diurnal bruxism, followed by stereotyped tongue movements and oral breathing. The caries scores were lower in the RS population than in the control group, but patients with RS showed greater periodontal alterations and a greater prevalence of drooling, dental wear, high-arched palate and anterior open bite. Conclusions: The population with RS exhibits characteristic and early oral habits and alterations, and periodontal problems that are more notorious than caries disease, so that our efforts should focus on the diagnosis and early correction of the parafunctional habits, promoting restorative treatment, and providing instructions on correct oral hygiene. Key words:Rett syndrome, oral habits, bruxism, caries.

Highlights

Rett syndrome (RS) is a chromosome X-linked neurological development disorder almost exclusively found in females
The poor communication and important mental retardation of these subjects limits the psychological strategies, usually employed in patients with autistic spectrum disorders (ASDs) [16,17,18,19], to controlled physical measures applied in a relaxed and family environment, that was precisely the pattern that we follow to carry out the oral examination in this study
Great similarities in oral hygiene habits are observed with autistic spectrum patients, where assisted manual tooth brushing is the most common practice [20,21] - possibly due to the limit tolerance of the sound and/or vibration of electrical brushes in both types of patients

Summary

Introduction

Rett syndrome (RS) is a chromosome X-linked (mutation of the MECP2 gene located in Xq28) neurological development disorder almost exclusively found in females It is characterized by psychomotor development regression with autistic manifestations, deceleration of cephalic growth, seizures, and stereotyped repetitive movements of the hands [1,2]. Rett syndrome is one of the so-called rare diseases, whose estimated prevalence is four cases per 100,000 inhabitants Application of this rate to the Spanish population indicates that not all cases of RS are adequately recognized and registered. The present study was designed to characterize this rare disease, based on all the documented patients in two Spanish regions, and establishing a standardized registry of their oral manifestations and habits, following the recommendations of the World Health Organization (WHO) for the conduction of oral health surveys

Objectives

Results

Conclusion