Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Late-onset variant of Huntington's chorea.

Late-onset variant of Huntington's chorea. We identified a large Georgia kinship in which a hereditary autosomal dominant chorea appeared at an average age of 65 years, much later than usual for Huntington's chorea. Progression was slow. Dementia was not an obvious initial feature. Family members denied that affected persons became demented, and those affected cognitively intact with bedside testing. However, deficits of memory were apparent on formal psychologic testing. In the propositus' generation, five of 12 siblings were affected during their 60s. Of 23 persons in a younger generation, aged 30 to 60 years, none was yet affected. A number of variants of Huntington's chorea have been proposed. In elderly patients without obvious dementia, psychologic testing may be of diagnostic importance, revealing characteristic memory deficits. When the disorder consistently occurs at an advanced age and progresses slowly, the implications for the family may be less grave than with Huntington's chorea of earlier onset. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Southern medical journal Pubmed

Late-onset variant of Huntington's chorea.

Southern medical journal , Volume 76 (10): -1195 – Nov 23, 1983

Late-onset variant of Huntington's chorea.


Abstract

We identified a large Georgia kinship in which a hereditary autosomal dominant chorea appeared at an average age of 65 years, much later than usual for Huntington's chorea. Progression was slow. Dementia was not an obvious initial feature. Family members denied that affected persons became demented, and those affected cognitively intact with bedside testing. However, deficits of memory were apparent on formal psychologic testing. In the propositus' generation, five of 12 siblings were affected during their 60s. Of 23 persons in a younger generation, aged 30 to 60 years, none was yet affected. A number of variants of Huntington's chorea have been proposed. In elderly patients without obvious dementia, psychologic testing may be of diagnostic importance, revealing characteristic memory deficits. When the disorder consistently occurs at an advanced age and progresses slowly, the implications for the family may be less grave than with Huntington's chorea of earlier onset.

Loading next page...
 
/lp/pubmed/late-onset-variant-of-huntington-s-chorea-y1ZfiST9lA

References

References for this paper are not available at this time. We will be adding them shortly, thank you for your patience.

ISSN
0038-4348
DOI
10.1097/00007611-198310000-00018
pmid
6226105

Abstract

We identified a large Georgia kinship in which a hereditary autosomal dominant chorea appeared at an average age of 65 years, much later than usual for Huntington's chorea. Progression was slow. Dementia was not an obvious initial feature. Family members denied that affected persons became demented, and those affected cognitively intact with bedside testing. However, deficits of memory were apparent on formal psychologic testing. In the propositus' generation, five of 12 siblings were affected during their 60s. Of 23 persons in a younger generation, aged 30 to 60 years, none was yet affected. A number of variants of Huntington's chorea have been proposed. In elderly patients without obvious dementia, psychologic testing may be of diagnostic importance, revealing characteristic memory deficits. When the disorder consistently occurs at an advanced age and progresses slowly, the implications for the family may be less grave than with Huntington's chorea of earlier onset.

Journal

Southern medical journalPubmed

Published: Nov 23, 1983

There are no references for this article.