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Huntington’s disease (HD) is a fatal, autosomal dominant, neurodegenerative disease characterized by movement disorder (generally a choreiform movement disorder, marked by involuntary continuous fluid or jerky “dance-like” movements), psychiatric disturbance, and cognitive decline.
Historical Background
Observations of dance-like movements (i.e., chorea) have been documented since the mid-fourteenth century. In 1500, it was suggested that chorea evolves from the central nervous system; in 1686, post-infectious chorea was described; and in 1832, an inherited form of chorea was identified (Walker, 2007). In 1872, George Huntington (1850–1916) published his seminal paper titled On Chorea, in which he provided a detailed description of the inherited form of chorea that manifests most often in adults and is marked by both psychiatric and cognitive impairment. Using the reports of his father and grandfather, both family practitioners, Huntington was...
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References and Readings
Blumenfeld, H. (2002). Neuroanatomy through clinical cases. Sutherland, MA: Sinauer Associates.
Lezak, M., Howieson, D., & Loring, D. (2004). Neuropsychological assessment. New York: Oxford.
Montoya, A., Price, B., Menear, M., & Lepage, M. (2006). Brain imaging and cognitive dysfunctions in Huntington’s disease. Journal of Psychiatry & Neuroscience, 31(1), 21–29.
NINDS. (2008). Huntington’s disease: Hope through research. Retrieved April 15, 2008, from http://www.ninds.nih.gov/disorders/huntington/detail_huntington.htm
Walker, F. (2007). Huntington’s disease. Lancet, 369, 218–228.
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Larson, J.C.G., Suchy, Y. (2011). Huntington’s Disease. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-79948-3_515
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