Oral conditions in Sjögren-Larsson syndrome

A study was carried out of oral conditions, including caries, gingivitis and periodontitis, malocclusion and enamel hypoplasias, in 34 of the 35 living Swedish patients with Sjögren-Larsson syndrome (SLS). Their residential circumstances (institutional or non-institutional) and medical background variables were recorded. The results were compared with previously published epidemiological data concerning the dental health of fully healthy and also of mentally and physically handicapped individuals. Compared with fully healthy individuals the SLS-patients had higher caries indices, a higher frequency of gingivitis and periodontitis, a higher frequency of enamel hypoplasias, but a similar prevalence of malocclusions. When compared with other mentally retarded individuals, the SLS-patients did not differ significantly with regard to caries frequency and gingival and periodontal status. The frequency of toothlessness in SLS-patients over 40 years of age was high. SLS-patients residing in institutions had a lower average caries prevalence than those who did not reside in institutions. The degree of mental retardation and the occurrence of epilepsy and more severe forms of neuromuscular disorder appeared to influence the extent of gingival and periodontal disease. The frequency of enamel hypoplasias was higher than in patients with cerebral palsy and comparable with the frequency in patients with Down's syndrome.