Age of death and duration in Huntington's disease in Tasmania

The records of all Huntington's disease affected individuals born in Tasmania were examined. An age of birth exclusion criterion (only those born before 1928 were accepted) removed some bias in the determination of duration of illness and age of death. The mean age of death was at least 62.9 years. This is the oldest mean age of death reported for any geographical region. The sex of the affected individual and the sex of the transmitting parent did not significantly influence the mean age of death. The children of affected women lived to a greater age than theri mothers and the children of affected men died at a younger age than their fathers, but statistical significance was not achieved. The duration of the disease was 14.8 years.