Dementia due to Huntington's disease

Susan Folstein and Russell L. Margolis

in New Oxford Textbook of Psychiatry

Second edition

Published on behalf of Oxford University Press

Published in print February 2012 | ISBN: 9780199696758
Published online October 2012 | e-ISBN: 9780191743221 | DOI:
Dementia due to Huntington's disease

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Huntington's disease (HD) was first described in 1872 by an American physician living on Long Island, New York. His father and grandfather practised medicine in the same community, so he had access to case notes from several generations of families who lived there. This long period of record keeping allowed him to document a hereditary form of chorea, similar to ‘common (Sydenham's) chorea’, but progressing over many years to death. Its sufferers had a tendency to insanity and suicide. Huntington's brief essay, which also included a clear description of autosomal dominant inheritance, remains one of the classical descriptions of a medical disorder.

Chapter.  4258 words. 

Subjects: Psychiatry ; Neurology

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