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Juvenile Huntington's Disease

Published online by Cambridge University Press:  13 June 2014

Brian O'Shea  and
Jane Falvey
Brian O'Shea
Affiliation:
Newcastle Hospital, Greystones, Co. Wicklow
Jane Falvey
Affiliation:
East Gate Unit, Middlewood Hospital, Sheffield, Yorkshire S61TP
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Abstract

A significant minority of cases of Huntington's disease commence before the age of 20 years and these juvenile cases are more likely to demonstrate paternal transmission and a clinical picture dominated by rigidity. Genetic research and positron emission tomography may make early diagnosis easier but predictive testing in childhood is replete with serious problems. There is no specific treatment for this fatal disorder. Juvenile Huntington's disease has a similar pathology and biochemistry to the adult condition despite a shorter course and certain clinical differences, such as rigidity and convulsions. Racial differences are explored.

Keywords

Juvenile Huntington's diseaserigidityWestphalrace
Type
Review Articles
Information
Irish Journal of Psychological Medicine , Volume 8 , Issue 2 , September 1991 , pp. 149 - 153
Copyright
Copyright © Cambridge University Press 1991

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