A Family Study of Huntington's Chorea with Unusual Manifestations
Authors: GEORGE A. PERRINE
Journal: Annals of Internal Medicine (1966)

Article1 March 1966A Family Study of Huntington's Chorea with Unusual ManifestationsGEORGE A. PERRINE JR., M.D., RICHARD M. GOODMAN, M.D.GEORGE A. PERRINE JR., M.D.Search for more papers by this author, RICHARD M. GOODMAN, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-64-3-570 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptHuntington's chorea is a relatively rare genetically determined neurological disorder, the incidence of which is about 5 per 100,000 in the United States (1, 2). Classically, it is characterized by: [1] an autosomal dominant mode of inheritance with complete penetrance; [2] onset between the ages of 25 and 45 years; [3] signs and symptoms of choreic movements, hypotonia, intellectual and emotional impairment; and [4] gradual worsening of the disorder. Difficulty establishing the correct diagnosis may be encountered if other neurological signs are present or if some of the typical characteristics are missing. In an early study, Davenport and Muncy (3)...References1. CHANDLERREEDDEJONG JHTERN: Huntington's chorea in Michigan. III. Clinical observations. Neurology (Minneap.) 10: 148, 1960. CrossrefMedlineGoogle Scholar2. PEARSONPETERSONLAZERTEBLOGETTKLEY JSMCJAHEIB: An educational approach to social problems of Huntington's chorea. Proc. Mayo Clin. 30: 349, 1955. MedlineGoogle Scholar3. DAVENPORTMUNCY CBEB: Huntington's chorea in relation to heredity and eugenics. Amer. J. Insan. 73: 195, 1916-17. Google Scholar4. BELL J: Huntington's chorea, in The Treasury of Human Inheritance, vol. IV, Part 1. Cambridge University Press, London, 1934, p. 1. Google Scholar5. BITTENBENDERQUADFASEL JBFA: Rigid and akinetic forms of Huntington's chorea. Arch. Neurol. (Chicago) 7: 275, 1962. CrossrefMedlineGoogle Scholar6. DENNY-BROWN D: The Basal Ganglia and Their Relation to Disorders of Movement. Oxford University Press, Cambridge, 1962, p. 23. Google Scholar7. JERVIS GA: Huntington's chorea in childhood. Arch. Neurol. (Chicago) 9: 244, 1963. CrossrefMedlineGoogle Scholar8. OLTMANFRIEDMAN JES: Comments on Huntington's chorea. Dis. Nerv. Syst. 22: 313, 1961. MedlineGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: GEORGE A. PERRINEJR., M.D.; RICHARD M. GOODMAN, M.D.Affiliations: Columbus, OhioFrom the Departments of Neurology and Medicine, Ohio State University Hospitals, Columbus, Ohio.This study was supported in part by a general research grant, Rotary 320002, Project 327, from the National Institutes of Health, Bethesda, Md.Requests for reprints should be addressed to Richard M. Goodman, M.D., Department of Medicine, Ohio State University Hospitals, 410 W. 10th Ave., Columbus, Ohio 43210. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byJuvenile Huntington diseaseHuntington's disease in monozygotic twins reared apart.Natural HistoryKlinik und Psychopathologie der organischen PsychosenChildhood and adolescent forms of Huntington's disease.Erkrankungen der Basalganglien 1 March 1966Volume 64, Issue 3Page: 570-574KeywordsAutosomal dominant diseasesDystoniaEmotionsHospital medicineNeurologyResearch grantsSigns and symptoms ePublished: 1 December 2008 Issue Published: 1 March 1966 PDF downloadLoading ...