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The large Huntington's disease family of Tasmania


Pridmore, S, The large Huntington's disease family of Tasmania, The Medical journal of Australia, 153, (10) pp. 593-595. ISSN 0025-729X (1990) [Refereed Article]

Copyright Statement

Copyright 1990 Australian Medical Publishing Company (AMPCo)

Official URL:

DOI: doi:10.5694/j.1326-5377.1990.tb126270.x


This article presents data on the large Huntington's disease (HD) family of Tasmania. Nine generations have been traced, starting with the father of the woman who brought the disease to the State. From that woman, six lines have living affected descendants. There are 765 living descendants who are "at risk" of HD. The numbers of affected males and females are equal. Affected members of the family have a mean age of onset of HD of 48.6 years and a mean age of death of 61.8 years. Affected members are at least as fertile as members of the general population. Late-onset disease is associated with significantly greater fertility (in men more so than women) compared with that of unaffected siblings of the same sex. Men with late-onset disease had a significantly older mean age of reproduction than that of their unaffected male siblings, and they had significantly more offspring than the number calculated for the general population of Tasmania. Unaffected siblings produced fewer offspring than in the general population.

Item Details

Item Type:Refereed Article
Research Division:Biomedical and Clinical Sciences
Research Group:Neurosciences
Research Field:Neurosciences not elsewhere classified
Objective Division:Health
Objective Group:Public health (excl. specific population health)
Objective Field:Mental health
UTAS Author:Pridmore, S (Professor Saxby Pridmore)
ID Code:115726
Year Published:1990
Web of Science® Times Cited:10
Deposited By:Medicine
Deposited On:2017-04-06
Last Modified:2017-05-12

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