Cognitive impairment in Huntington disease: diagnosis and treatment.

Publisher: Current Science Inc Country of Publication: United States NLM ID: 100931790 Publication Model: Print Cited Medium: Internet ISSN: 1534-6293 (Electronic) Linking ISSN: 15284042 NLM ISO Abbreviation: Curr Neurol Neurosci Rep Subsets: MEDLINE

Original Publication: Philadelphia, PA : Current Science Inc., c2001-

Cognition Disorders*/diagnosis ; Cognition Disorders*/etiology ; Cognition Disorders*/therapy; Huntington Disease/*complications; Attention Deficit Disorder with Hyperactivity/etiology ; Awareness ; Communication ; Disability Evaluation ; Emotions ; Executive Function ; Humans ; Memory ; Neuropsychological Tests ; Smell

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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R01 NS040068 United States NS NINDS NIH HHS; R01 NS054893 United States NS NINDS NIH HHS; R01 NS040068-11 United States NS NINDS NIH HHS

Date Created: 20110824 Date Completed: 20120110 Latest Revision: 20231106

20240513

PMC3628771

10.1007/s11910-011-0215-x

21861097