CHARACTERISTIC DENTAL ARCHES AND OCCLUSION IN PATIENTS WITH ASPARTYLGLUCOSAMINURIA

Aspartylglucosaminuria (AGU) is a lysosomal storage disorder with prog ressive mental retardation as a presenting manifestation. The disorder is caused by a single nucleotide change in the gene encoding aspartyl glucosaminidase (AGA). This rare disease is relatively common in Finla nd: we were able to examine 81 Finnish AGU-patients for dental and ora l changes. Tooth crown size and crown shape were normal, but dental ma locclusions were common, and prevalences of spacing, large overjet, an terior open bite, and lateral crossbite exceeded Finnish population pr evalences (P<0.0001). Dental arches were already large in childhood, a nd in adult patients, when compared to Finnish population standards, t he lower dental arch was larger in all dimensions (P<0.001). Almost ai l patients had abnormally large tongues, which we assumed to be the re ason for the structural abnormalities observed.