Neurodegenerative Disorders: George Huntington's Description of Hereditary Chorea

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Back to table of contents Previous article Next article Classic ArticlesFull AccessNeurodegenerative DisordersGeorge Huntington's Description of Hereditary ChoreaThomas C. Neylan, M.D., Section EditorThomas C. NeylanSearch for more papers by this author, M.D., Section EditorPublished Online:1 Feb 2003https://doi.org/10.1176/jnp.15.1.108AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail I have drawn your attention to this form of chorea gentlemen, not that I considered it of any great practical importance to you, but merely as a medical curiosity, and as such it may have some interest. —George Huntington, 1872At the age of 22, the year following his graduation from medical school at Columbia, George Huntington (1850–1916) made his one contribution to medical research, publishing his report on a hereditary form of chorea in The Medical and Surgical Reporter in the April 13, 1872, issue.1 Huntington came from several generations of physicians and as a boy accompanied his father and grandfather on medical rounds in East Hampton, New York.2 Huntington wrote up his observations and incorporated observations made by his father and grandfather, who offered a crucial longitudinal perspective. Although several descriptions of the disorder predated Huntington's report,3 none were as clear as his.The paper that follows was originally read to the Meigs and Mason Academy of Medicine at Middleport, Ohio, on February 15, 1872. The first part of Huntington's brief report consists of a broad review of choreiform disorders. The discussion on therapeutics provides a glimpse of the 19th-century practice of medicine, which includes techniques such as bleeding, applied electricity, purgatives, and tonics. In the last seven paragraphs, Huntington describes the novel disorder that ultimately bears his eponym. In his writing, the essential features of this disorder were ”1. Its hereditary nature. 2. A tendency to insanity and suicide. 3. Its manifesting itself as a grave disease only in adult life.” Huntington noted that the disorder was confined to “a few families, and has been transmitted to them, an heirloom from generations away back in the dim past.” He also noted that in unaffected offspring, “the thread is broken and the grandchildren and great-grandchildren of the original shakers may rest assured that they are free from the disease.”Huntington's disease is of tremendous interest to neuropsychiatry because it has a known cause and manifests in changes in behavior, cognition, and affect. It is known to be caused by a mutation resulting in trinucleotide CAG repeats (polyglutamine) on the Huntington protein encoded on the short arm of chromosome 4.4 Neuropsychiatric studies of Huntington's disease may lead to breakthroughs in understanding the neuropathological correlates of psychiatric disorders. (For a review, see Lauterbach et al.5)References1 Huntington G: On chorea. Med Surg Rep 1872; 26:317-321Google Scholar2 Durbach N, Hayden MR: George Huntington: the man behind the eponym. J Med Genet 1993; 30:406-409Crossref, Medline, Google Scholar3 Siemers E: Huntington disease. Arch Neurol 2001; 58:308-310Crossref, Medline, Google Scholar4 Ross CA: Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders. Neuron 2002; 35:819-822Crossref, Medline, Google Scholar5 Lauterbach EC, Cummings JL, Duffy J, et al: Neuropsychiatric correlates and treatment of lenticulostriatal diseases: a review of the literature and overview of research opportunities in Huntington's, Wilson's, and Fahr's diseases. A report of the ANPA Committee on Research. American Neuropsychiatric Association. J Neuropsychiatry Clin Neurosci 1998; 10:249-266Link, Google Scholar FiguresReferencesCited byDetailsCited ByGlucose Metabolic Dysfunction in Neurodegenerative Diseases—New Mechanistic Insights and the Potential of Hypoxia as a Prospective Therapy Targeting Metabolic Reprogramming31 May 2021 | International Journal of Molecular Sciences, Vol. 22, No. 11The psychopharmacology of Huntington diseaseRevista de la Facultad de Medicina, Vol. 65, No. 2Gene, Vol. 556, No. 2, Vol. 82Huntington’s Disease Volume 15Issue 1 February 2003Pages 108-108 Metrics PDF download History Published online 1 February 2003 Published in print 1 February 2003