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

Abstract

OBJECTIVES
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.
MATERIALS AND METHODS
Clinical information was obtained from general, neurologic, and psychiatric hospitals, practicing neurologists, general practitioners, and family members of affected individuals.
RESULTS
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.
CONCLUSIONS
The prevalence of HD in Iceland is markedly lower than in the neighboring countries (Norway and the British Isles), where Icelanders originate from.

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  • Publisher Full Text
  • Authors

    Sveinsson O, Halldórsson S, Olafsson E

    Institution

    Department of Neurology, Landspitali University Hospital, Reykjavik, Sweden.

    Source

    European neurology 68:1 2012 pg 48-51

    MeSH

    Adult
    Female
    History, 16th Century
    Humans
    Huntington Disease
    Iceland
    Incidence
    Male
    Middle Aged
    Prevalence
    Young Adult

    Pub Type(s)

    Historical Article
    Journal Article
    Research Support, Non-U.S. Gov't

    Language

    eng

    PubMed ID

    22722209