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History of Neurology: Seminal Citations
February 2001

Huntington Disease

Author Affiliations

From Eli Lilly and Company, Indianapolis, Ind.

 

CHRISTOPHER G.GOETZMD

Arch Neurol. 2001;58(2):308-310. doi:10.1001/archneur.58.2.308

Huntington disease (HD) is a genetically transmitted, gradually progressive disorder characterized by involuntary movements and dementia, with an average duration of 17 years between onset of symptoms and death. Whereas current research focuses primarily on molecular biology and novel treatments, these efforts are based on initial observations by clinicians who were able to discern the constellations of symptoms linked to the disorder.

In obedience to your kind request, I improve my first leisure since my return home in giving you, in as lucid and satisfactory a manner as possible, an account of a singular affection somewhat common in the southeastern portion of this State [New York], and known among the common people as "the magrums." Whence the name originated I do not know. . . . The disease is markedly hereditary, and is most common among the lower classes, though cases of it are not infrequent among those who by industry and temperance, have raised themselves to a respectable rank in society. . . . This derangement of muscular action is by no means uniform; in some it exists to a greater, in others to a less extent, but in all cases it gradually induces a state of more or less perfect dementia.—Charles Waters,1 1841

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