Huntington's Disease

Huntington's disease, hereditary, acute disturbance of the central nervous system usually beginning in middle age and characterized by involuntary muscular movements and progressive intellectual deterioration; formerly called Huntington's chorea. The disease is sometimes confused with chorea or St. Vitus's dance, which is not hereditary. A faulty gene produces a defective protein attacks neurons in the basal ganglia, clusters of nerve tissue deep within the brain that govern coordination.

The onset is insidious and inexorably progressive; no treatment is known. Psychiatric disturbances range from personality changes involving apathy and irritability to bipolar or schizophreniform illness. Motor manifestations include flicking movements of the extremities, a lilting gait, and motor impersistence (inability to sustain a motor act such as tongue protrusion).

In 1993 the gene responsible for the disease was located; within that gene a small segment of code is, for some reason, copied over and over. Genetic counseling is extremely important, since 50% of the offspring of an affected parent inherit the gene, which inevitably leads to the disease.

The Columbia Encyclopedia, 6th ed. Copyright© 2018, The Columbia University Press.

Huntington's Disease: Selected full-text books and articles

Delving into HUNTINGTON'S DISEASE By Goolkasian, Virginia USA TODAY, Vol. 130, No. 2676, September 2001
Huntington's Disease By Lechich, Anthony J Care Management Journals, Vol. 9, No. 2, July 1, 2008
PEER-REVIEWED PERIODICAL
Peer-reviewed publications on Questia are publications containing articles which were subject to evaluation for accuracy and substance by professional peers of the article's author(s).
Huntington's Disease: A Caring Approach to the End of Life By Klager, Joseph; Duckett, Ayana; Sandler, Susan; Moskowitz, Carol Care Management Journals, Vol. 9, No. 2, July 1, 2008
PEER-REVIEWED PERIODICAL
Peer-reviewed publications on Questia are publications containing articles which were subject to evaluation for accuracy and substance by professional peers of the article's author(s).
Encyclopedia of Family Health By David B. Jacoby; Robert M. Youngson Marshall Cavendish, vol.7, 2005 (3rd edition)
Clinical Neuropsychology By Kenneth M. Heilman; Edward Valenstein Oxford University Press, 2003
The Neuropsychology of Degenerative Brain Diseases By Robert G. Knight Lawrence Erlbaum Associates, 1992
Health Watch: How Huntington's Disease Devasted My Family The News Letter (Belfast, Northern Ireland), October 16, 2002
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