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J Neurol Neurosurg Psychiatry 1999;66:52-56 ( January )

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

Tatiana Foroud, Jacqueline Gray, Julia Ivashina, P Michael Conneally

Department of Medical and Molecular Genetics, Indiana University School of Medicine, Indianapolis, IN, USA

Correspondence to: Department of Medical and Molecular Genetics, Indiana University School of Medicine, Medical Research and Library Building, IB 155, 975 W Walnut Street, Indianapolis, IN 46202, USA. Telephone 001 317 278 1291; fax 001 317 274 2387; email foroud{at}medgen.iupui.edu

Received 11 June 1998; Accepted 3 August 1998

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.

Keywords: Huntington's disease; duration; age at onset


© 1999 by Journal of Neurology, Neurosurgery, and Psychiatry



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