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Cognitive and functional decline in Huntington's disease: Dementia criteria revisited

2010; Wiley; Volume: 25; Issue: 9 Linguagem: Inglês

10.1002/mds.22953

1531-8257

Guerry M. Peavy, Mark W. Jacobson, Jody Goldstein, Joanne M. Hamilton, Amy Kane, Anthony Gamst, Stephanie Lessig, J. C. Lee, Jody Corey‐Bloom,

Neurology and Historical Studies

Abstract The importance of designating criteria for diagnosing dementia lies in its implications for clinical treatment, research, caregiving, and decision‐making. Dementia diagnosis in Huntington's disease (HD) is often based on criteria developed for Alzheimer's disease requiring memory loss. However, it is likely that other cognitive deficits contribute to functional impairment in HD before memory declines. The goal is to identify cognitive deficits that contribute to functional impairment to support dementia criteria that reflect HD neuropathology. Eighty‐four HD mutation‐positive subjects completed neuropsychological tests and the Unified Huntington's Disease Rating Scale Functional Independence Scale (FIS). Functional impairment was defined as 80 or below on the FIS. Speed of processing, initiation, and attention measures accounted for 70.0% of the variance in FIS ratings (linear regression) and correctly classified 91.7% of subjects as functionally impaired or intact (logistic regression). Measures of memory, motor impairment except dysarthria, neuroleptic use, and depressed mood did not improve prediction. A definition of HD dementia that includes cognitive impairment in at least two areas of cognition but does not require a memory deficit, in the context of impaired functional abilities and a deteriorating course, more accurately reflects HD neuropathology and could lead to improved research methods and patient care. © 2010 Movement Disorder Society