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

J Neurol Neurosurg Psychiatry. 1999 Jan;66(1):52-6. doi: 10.1136/jnnp.66.1.52.

Abstract

Objectives: 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.

Methods: 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.

Results: 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.

Conclusions: Duration of disease is influenced by the age at symptom onset with juvenile and late onset patients having the shortest duration.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Age Distribution
  • Age of Onset
  • Aged
  • Disease Progression
  • Female
  • Humans
  • Huntington Disease / diagnosis*
  • Huntington Disease / genetics
  • Huntington Disease / mortality
  • Male
  • Middle Aged
  • Point Mutation / genetics
  • Retrospective Studies
  • Sex Distribution
  • Surveys and Questionnaires
  • Survival Rate
  • Time Factors