Oral parafunction and bruxism in Rett syndrome and associated factors: An observational study.

OBJECTIVES: To explore patterns of parafunction, and bruxism, and its relationships with genotype and snoring in individuals with Rett syndrome (RTT). METHODS: Retrospective observational data of those with confirmed MECP2 mutations in the InterRett database (n = 216) were used to investigate experience of parafunctional habits, and bruxism and their relationships with genotype and snoring using multivariable linear regression. RESULTS: The prevalence of parafunction was 98.2%. Bruxism was reported (66.2%) with the patterns mostly both diurnal and nocturnal (44.1%) and exclusively diurnal (42.7%). Compared to individuals with C-terminal deletion, individuals with p.Arg106Trp mutations were less likely to have bruxism reported (aOR = 0.15; 95% CI 0.02-0.98, p = 0.05) and those with p.Arg168* mutation were more likely to have frequent bruxism than none or occasional bruxism reported (aROR 3.4; 95% CI 1.1-10.7 p = 0.04). The relative odds of having nocturnal bruxism constantly, compared to none/occasionally, were higher among those 'always' snoring (aROR 6.24; 95% CI 2.1-18.2, p = 0.001) than those with no snoring. CONCLUSIONS: There appeared to be genotypic association with bruxism in p.Arg168* and p.Arg106Trp mutations and association between nocturnal bruxism and frequent snoring in an international sample of individuals with RTT. Clinical significance of the high prevalence of bruxism should be highlighted in relation to difficulty communicating pain and increased dental treatment need in RTT.

Enablers and barriers in dental attendance in Rett syndrome: an international observational study.

AIMS: Intellectual and developmental disabilities are heterogeneous in aetiology and presentation, and one cannot make assumptions about the oral health barriers of those with Rett syndrome (RTT) based on findings from generic studies. This study investigated caregivers' perceptions regarding access to dental care for those with (RTT), and associations of dental treatments received by those with RTT with their caregivers' perceived value of oral health and perception of their own as well as their daughter's dental anxiety. METHODS AND RESULTS: Retrospective observational data of a subset of individuals with confirmed MECP2 mutations in the InterRett database (n = 216) were used to explore caregiver-related factors and their relationships with longitudinal data on dental service utilisation, using negative binomial regression. The main reported barriers to dental care access for individuals with RTT were primarily dentist-related in nature, regardless of dental service history. Those with reported dental nonattendance were of older age. Increasing levels of caregiver-reported dental fear were associated with less frequent dental check-ups or for any appointments for affected individuals. CONCLUSIONS: Dentist-related barriers and caregiver-reported anxiety may both adversely affect dental attendance for those with RTT. Future research should explore caregivers' beliefs and oral health literacy.

Oral health education and promotion in special needs children: Systematic review and meta-analysis.

OBJECTIVE: To review the effectiveness of oral health education and oral health promotion interventions for children and adolescents with intellectual and developmental disabilities (IDD), in ensuring optimal gingival health, caries experience and oral health-related quality of life, compared to no interventions or alternative interventions. METHODS: A systematic review was conducted to identify published studies from four databases (Medline, PsycINFO, CINAHL and ERIC). Randomised or quasi-randomised controlled trials were included. Two independent reviewers performed risk of bias and qualitative analysis. Meta-analysis was performed as appropriate. RESULTS: Eight treatment comparisons were identified. There was low certainty evidence that fluoride interventions provided long-term reductions in caries in those with IDD; and there was some evidence that chlorhexidine albeit with low certainty provided short-term and long-term reductions in plaque and gingivitis. There was moderate certainty evidence for short-term reductions in dental plaque from the use of modified toothbrushes, but not compelling evidence for powered toothbrushes. CONCLUSIONS: Most studies provided a low quality of evidence, and so any adaptations made to oral health practices of individuals with IDD need to consider their individual needs. PROSPERO registration: CRD42019145784.