Journal
CURRENT NEUROLOGY AND NEUROSCIENCE REPORTS
Volume 11, Issue 5, Pages 474-483Publisher
SPRINGER
DOI: 10.1007/s11910-011-0215-x
Keywords
Mild cognitive impairment; Dementia; Cognitive impairment; Diagnosis; Huntington disease; Prodrome of HD
Categories
Funding
- National Institutes for Health, National Institute of Neurological Disorders and Stroke [R01 NS040068-11]
- CHDI Foundation, Inc.
- National Institute of Neurological Disorders & Stroke, Cognitive and Functional Brain Changes in Preclinical HD [R01 NS054893]
- Schlesinger Associates
- National Institutes of Health
- CHDI Foundation
- Huntington's Disease Society of America
- Huntington's Study Group
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Cognition has been well characterized in the various stages of Huntington disease (HD) as well as in the prodrome before the motor diagnosis is given. Although the clinical diagnosis of HD relies on the manifestation of motor abnormalities, the associated impairments have been growing in prominence for several reasons. First, research to understand the most debilitating aspects of HD has suggested that cognitive and behavioral changes place the greatest burden on families, are most highly associated with functional decline, and can be predictive of institutionalization. Second, cognitive impairments are evident at least 15 years prior to the time at which motor diagnosis is given. Finally, cognitive decline is associated with biological markers such as brain atrophy, circulating levels of brain-derived neurotrophic factors, and insulin-like growth factor 1. Efforts are now underway to develop valid and reliable measures of cognition in the prodrome as well as in all stages of HD so that clinical trials can be conducted using cognitive outcomes.
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