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

Life-Span Research on the Prediction of Psychopathology By L. Erlenmeyer-Kimling; Nancy E. Miller Lawrence Erlbaum Associates, 1986
Librarian's tip: Chap. 20 "Prediction in the Onset and Detection of Huntington's Disease"
An Illustrated Pocketbook of Parkinson's Disease and Related Disorders By G. David Perkin Parthenon, 2003
Librarian's tip: "Huntington's Disease" begins on p. 62
The Neuropsychology of Degenerative Brain Diseases By Robert G. Knight Lawrence Erlbaum Associates, 1992
Librarian's tip: Chap. 7 "Huntington's Disease"
Neuropsychology of Eye Movements By Cris W. Johnston; Francis J. Pirozzolo Lawrence Erlbaum Associates, 1988
Librarian's tip: "Huntington's Disease" begins on p. 155
The Shared Experience of Illness: Stories of Patients, Families, and Their Therapists By Susan H. McDaniel; Jeri Hepworth; William J. Doherty Basic Books, 1997
Librarian's tip: Chap. 18 "'Do You Need to Know?': Genetic Testing for Huntington's Disease"
Case Studies: The Price of Silence By Fletcher, John C.; Wertz, Dorothy; Lynch, Abbyann; Czeizel, Andrew; Salzano, Francisco M.; Berg, Kare The Hastings Center Report, Vol. 20, No. 3, May-June 1990
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