Differences in duration of Huntington's disease based on age at onset.

Abstract

Data from a sample of 2494 patients affected with Huntington's disease (HD), collected as part of the National Research Roster for Huntington Disease Patients and Families, were examined to determine if there was a relation between age at onset and duration of illness. Sufficient data for inclusion in analysis was available from 2068 patients, of whom 828 were deceased and 1240 were living. The median duration of disease was 21.4 years with a range of 1.2 to 40.8 years. Patients were categorised into one of four groups based on their age at onset. Significant differences in duration based on the age at onset were found (p<0.025), with juvenile and late onset patients with HD having shorter duration of illness compared with those with an onset between 20-49 years. Duration of disease is influenced by the age at symptom onset with juvenile and late onset patients having the shortest duration.