An unusually low prevalence of Huntington's disease in Iceland.

• Published in: European Neurology
• Main Authors: Sveinsson, Olafur, Halldórsson, Sigurður, Olafsson, Elias
• Other Authors: Department of Neurology, Landspitali University Hospital, Reykjavik, Iceland
• Format: Article in Journal/Newspaper
• Language: English
• Published: Karger 2013
• Subjects: Adult; Female; History; 16th Century; Humans; Huntington Disease; Iceland; Incidence; Male; Middle Aged; Prevalence; Young Adult; Huntington; Norway
• Online Access: http://hdl.handle.net/2336/301913; https://doi.org/10.1159/000337680

To access publisher's full text version of this article. Please click on the hyperlink in Additional Links field. Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder characterized by involuntary movements and psychiatric disturbances, found worldwide, with a variable prevalence. The purpose of this study was to determine the history of HD in Iceland and determine the prevalence and incidence of HD. Clinical information was obtained from general, neurologic, and psychiatric hospitals, practicing neurologists, general practitioners, and family members of affected individuals. Twenty-seven individuals were identified with typical symptoms of HD from the 1850s to 2007. All but one sporadic case are descendants of a husband and wife living in the early and mid-19th century. The point prevalence of HD in Iceland is 1.0 per 100,000 individuals. The prevalence of HD in Iceland is markedly lower than in the neighboring countries (Norway and the British Isles), where Icelanders originate from Iceland's University research fund