Long-term monitoring of the mortality trend of Huntington?s disease in Austria

Recent increasing incidence and prevalence rates of Huntington's disease (HD), a fatal neurodegenerative disorder, prompted us to investigate the epidemiological dynamic of HD in Austria during the period 1970--2001. Our study demonstrated a stable HD mortality rate throughout Austria of 0.125 per 100,000 individuals during the investigated period. The median age at death from HD was 56.5 years for both sexes and remained stable during the entire period observed, indicating no prolonged survival. An above average mortality rate from HD was observed in the north-eastern parts of Austria. Our data reflect the epidemiology of HD prior to and after the availability of genetic testing and provide a solid baseline for future investigations on the epidemiology of HD.