Tuberøs sclerose

Oversigtsartikel Dato: 14.02.2008

Sygdomsbilledet tuberøs sclerose har en odontologisk interesse, idet der forekommer forandringer i tænder og i mundslimhinde samt i ansigtet hos personer med tuberøs sclerose. Findes disse forandringer kan de være med til at sikre diagnosen og dermed føre til generel behandling samt genetisk vejledning. Tuberøs sclerose er en neurologisk sygdom der griber ind på mange organer og væv blandt andet hårdvævene. I denne oversigt omtales klassifikation, diagnostik, genetik og behandling af tuberøs sclerose.

Tuberous sclerosis (TS) is a heterogeneous genetic disease characterized by a potential for hamartomatous growths in multiple organs – skin, oral mucosa, viscera and changes in skeleton and teeth. TS has a broad range of expression. Mild cases can be difficult to detect, while the classic triade of mental retardation, seizures, and angiofibromas represents the severe end of the spectrum. Investigations have demonstrated pits in the enamel in deciduous and in permanent teeth. The observation of pits in the enamel specially in the deciduous teeth may be useful for the early diagnosis of TS. Awareness of the condition TS is important to dental treatment and for the medical evaluation and control of risk factors to sedation and to general anesthesia.