Journal of the Japanese Society for Disability and Oral Health
Online ISSN : 2188-9708
Print ISSN : 0913-1663
ISSN-L : 0913-1663
 
Oral Characteristics in Rett Syndrome
Kanako TSUTSUMIJumpei MURAKAMIChiaki FUJISHIROYukiko NAKAMURAYosuke HIROSETomoyuki ONISHIMasako OKAShigehisa AKIYAMAIchijiro MORISAKI
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2017 Volume 38 Issue 1 Pages 69-73

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Abstract

Rett syndrome is a neurodevelopmental disorder appearing mainly in females, and causes neurological symptoms age-dependently. From late infancy to childhood, both mental and motor functions regress, followed by subsequent development of mental retardation, stereotypical hand movements, breathing abnormalities, and seizures. Bruxism has been mentioned frequently in previous reports, however, there is little information on oral findings. We present and discuss the general and oral characteristics of 12 female patients. The 12 patients with Rett syndrome were examined by dentists.

Results:1. Both the number and morphology of deciduous and permanent teeth seemed normal. 2. Dental occlusion was normal in the majority (5/12), followed by deep bite (3/12), edge-to-edge bite (2/12), anterior cross bite (1/12) or open bite (1/12). One of the patients with normal occlusion and with deep bite had been treated for anterior cross bite. 3. The most common oral finding was bruxism (66.7%) with attrition (50.0%). 4.75% of patients of Rett syndrome could walk, but none of them was able to speak. Involuntary and repeated hand-washing movement was found in 83.3%.

Conclusions:Patients with Rett syndrome seem to have no specific number or shape of tooth abnormalities, but there is a high incidence of bruxism. It is suggested that malalignment is caused by their hand movement and oral habits. All the patients need to be helped in eating and brushing because of their hand dysfunction, and early professional care is essential to maintain their oral health and QOL.

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© 2017 The Japanese Society for Disability and Oral Health
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