Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2009 Jan 15;276(1-2):159-62.
doi: 10.1016/j.jns.2008.09.029. Epub 2008 Oct 31.

Late onset Huntington Disease: clinical and genetic characteristics of 34 cases

Hillary Lipe  1 , Thomas Bird
Affiliations

Late onset Huntington Disease: clinical and genetic characteristics of 34 cases

Hillary Lipe et al. J Neurol Sci. .

Abstract

We performed a retrospective observational study of thirty-four persons with late onset of Huntington Disease (HD) (onset range 60-79 years). CAG trinucleotide expansion size ranged from 38-44 repeats. Even at this late age a significant negative correlation (r=-0.421, p<0.05) was found between the length of repeat and age of onset. Important characteristics of these older subjects were: (1)Most (68%) were the first in the family to have a diagnosis of HD, (2) Motor problems were the initial symptoms at onset, (3) Disability increased and varied from mild to severe (4) Disease duration was somewhat shorter (12 years) than that reported for mid-life onset, (5) Death was often related to diseases of old age, such as cancer and cerebrovascular disease, (6) Serious falls were a major risk and (7) Global dementia may be associated with coincident Alzheimer disease. Recognizing these characteristics will help physicians and other health care providers better identify and follow the late onset presentation of this disease.

PubMed Disclaimer

Figures

Figure Legend 1
Figure Legend 1
Longitudinal UHDRS Motor scores in nine late onset subjects. Individual subjects indicated by colored lines are the same in Figures 1 and 2.
Figure Legend 2
Figure Legend 2
Longitudinal UHDRS Functional Capacity scores in nine late onset subjects. Individual subjects indicated by colored lines are the same in Figures 1 and 2.
Figure Legend 3
Figure Legend 3
Age at Onset vs. CAG expansion size scatterplot with linear regression line for 34 subjects.
See this image and copyright information in PMC

References

    1. Kremer B. Clinical neurology of Huntington's disease. In: Bates G, Harper P, Jones L, editors. Huntington's Disease, Third Edition Oxford Monographs on Medical Genetics. New York: Oxford University Press; 2002. p. 30.
    1. Huntington Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable in Huntington disease chromosomes. Cell. 1993;72:971–983. - PubMed
    1. Roos R, Hermans J, Vegter-van der Vlis M, van Ommen G, Bruyn G. Duration of illness in Huntington's disease is not related to age at onset. J Neurol Neurosurg Psychiatry. 1993;56:98–100. - PMC - PubMed
    1. Foroud T, Gray J, Ivashina J, Conneally M. Differences in duration of Huntington's disease based on age at onset. J Neurol Neurosurg Psychiatry. 1999;66:52–56. - PMC - PubMed
    1. Mahant N, McCusker EA, Byth K, Graham S The Huntington Study Group. Huntington's Disease: Clinical correlates of disability and progression. Neurol. 2003;61:1085–1092. - PubMed

Publication types