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Dementia due to Huntington's disease 

Dementia due to Huntington's disease
Chapter:
Dementia due to Huntington's disease
Author(s):

Susan Folstein

and Russell L. Margolis

DOI:
10.1093/med/9780199696758.003.0047
Page of

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date: 08 March 2021

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.

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