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57 - Huntington's disease

from Part X - Other neurodegenerative diseases

Published online by Cambridge University Press:  04 August 2010

M. Flint Beal ,
Anthony E. Lang  and
Albert C. Ludolph
By
Christoph M. Kosinski  and
Bernhard Landwehrmeyer
M. Flint Beal
Affiliation:
Cornell University, New York
Anthony E. Lang
Affiliation:
University of Toronto
Albert C. Ludolph
Affiliation:
Universität Ulm, Germany
Christoph M. Kosinski
Affiliation:
Department of Neurology, University Hospital Aachen, Germany
Bernhard Landwehrmeyer
Affiliation:
Department of Neurology, University of Ulm, Germany
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Summary

Introduction

The description by George Huntington in 1872 of the disease that has subsequently borne his name is remarkable for its clarity and comprehensiveness (Huntington, 1872). It was not the first description of the disorder (see, for instance, Charles Oscar Waters, 1842, and Johan Christian Lund, 1860) (Waters, 1842; Lund, 1860), but it stands out as the first full delineation of the condition as a specific disease entity, quite separate from other forms of chorea. Huntington's paper was given at Middleport, Ohio, and published later in the Philadelphia journal, The Medical and Surgical Reporter. One can do no better than quote it here:

The hereditary chorea, as I shall call it, is confined to certain and fortunately a few families, and has been transmitted to them, an heirloom from generations away back in the dim past. It is spoken of by those in whose veins the seeds of the disease are known to exist, with a kind of horror, and not at all alluded to except through dire necessity, when it is mentioned as ‘that disorder’. It is attended generally by all the symptoms of common chorea, only in an aggravated degree hardly ever manifesting itself until adult or middle life, and then coming on gradually but surely, increasing by degrees, and often occupying years in its development, until the hapless sufferer is but a quivering wreck of his former self. (…) There are three marked peculiarities in this disease: 1. Its hereditary nature. 2. A tendency to insanity and suicide.[…]

Type
Chapter
Information
Neurodegenerative Diseases
Neurobiology, Pathogenesis and Therapeutics
 , pp. 847 - 860
Publisher: Cambridge University Press
Print publication year: 2005

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