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October 1962

Rigid and Akinetic Forms of Huntington's Chorea

Author Affiliations

Department of Neurology, Veterans Administration Hospital, Boston (Dr. Bittenbender); Chief, Department of Neurology, Veterans Administration Hospital, and Associate Professor of Neurology, Boston University School of Medicine (Dr. Quadfasel).

Arch Neurol. 1962;7(4):275-288. doi:10.1001/archneur.1962.04210040027003

William Osler39 considered Huntington's27 original description of chronic chorea a model of cogent observation and concise presentation. The classic paper set forth the characteristic movement disorder and mental changes and implied a dominant heredity which was ultimately proven correct by Entres18 and others. In addition, hypotonia was soon added as a characteristic if not constant finding in this disorder which set it apart from the other heredodegenerative diseases of the basal ganglia.

Davenport,12 in 1916, noted individual and familial variations in the age of onset of the chorea, as well as the delayed onset or complete absence of dementia in some cases. His concept of different "biotypes" was more fully developed by the Continental neurologists to include a variety of hitherto unrelated neurologic and psychiatric phenomena into a broadened concept of "Huntington's disease." One outgrowth of the biotype theory—an enhanced appreciation of the variations of tone

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