Synonyms
Huntington’s chorea
Definition
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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Larson, J.C.G., Niermeyer, M., Suchy, Y. (2018). Huntington’s Disease. In: Kreutzer, J.S., DeLuca, J., Caplan, B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, Cham. https://doi.org/10.1007/978-3-319-57111-9_515
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DOI: https://doi.org/10.1007/978-3-319-57111-9_515
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