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Eur Neurol. 2012;68(1):48-51. doi: 10.1159/000337680. Epub 2012 Jun 20.

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

Author information

1
Department of Neurology, Landspitali University Hospital, Reykjavik, Sweden.

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.

PMID:
22722209
DOI:
10.1159/000337680
[Indexed for MEDLINE]

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