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.
Keywords
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Article info
Publication history
Accepted:
September 16,
2008
Received in revised form:
September 11,
2008
Received:
June 18,
2008
Identification
Copyright
Published by Elsevier Inc.
