Encyclopedia of Clinical Neuropsychology

2018 Edition
| Editors: Jeffrey S. Kreutzer, John DeLuca, Bruce Caplan

Huntington’s Disease

  • Jennifer C. G. LarsonEmail author
  • Madison Niermeyer
  • Yana Suchy
Reference work entry
DOI: https://doi.org/10.1007/978-3-319-57111-9_515


Huntington’s chorea


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

  1. Blumenfeld, H. (2010). Neuroanatomy through clinical cases (2nd ed.). Sutherland: Sinauer Associates.Google Scholar
  2. Lezak, M. D., Howieson, D. B., Bigler, E. D., & Tranel, D. (2012). Neuropsychological assessment (5th ed.). New York: Oxford University Press.Google Scholar
  3. NINDS. (2016). Huntington’s disease: Hope through research. Retrieved April 13, 2016, from http://www.ninds.nih.gov/disorders/huntington/detail_huntington.htm.
  4. Paulsen, J. S. (2011). Cognitive impairment in Huntington disease: Diagnosis and treatment. Current Neurology and Neuroscience Reports, 11(5), 474–483.  https://doi.org/10.1007/s11910-011-0215-xPubMedPubMedCentralCrossRefGoogle Scholar
  5. Shannon, K., & Fraint, A. (2015). Therapeutic advances in Huntington’s disease. Movement Disorders, 30(11), 1539–1546.PubMedCrossRefGoogle Scholar
  6. Walker, F. (2007). Huntington’s disease. Lancet, 369, 218–228.PubMedCrossRefPubMedCentralGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  • Jennifer C. G. Larson
    • 1
    Email author
  • Madison Niermeyer
    • 2
  • Yana Suchy
    • 3
  1. 1.Department of Physical Medicine and RehabilitationUniversity of MichiganAnn ArborUSA
  2. 2.Department of Psychology, Clinical PsychologyThe University of UtahSalt Lake CityUSA
  3. 3.Department of PsychologyThe University of UtahSalt Lake CityUSA