Mild cognitive impairment in prediagnosed Huntington disease
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Abstract
Background: Cognitive decline has been reported in Huntington disease (HD), as well as in the period before diagnosis of motor symptoms (i.e., pre-HD). However, the severity, frequency, and characterization of cognitive difficulties have not been well-described. Applying similar cutoffs to those used in mild cognitive impairment (MCI) research, the current study examined the rates of subtle cognitive dysfunction (e.g., dysfunction that does not meet criteria for dementia) in pre-HD.
Methods: Using baseline data from 160 non–gene-expanded comparison participants, normative data were established for cognitive tests of episodic memory, processing speed, executive functioning, and visuospatial perception. Cutoff scores at 1.5 standard deviations below the mean of the comparison group were then applied to 575 gene-expanded pre-HD participants from the observational study, PREDICT-HD, who were stratified by motor signs and genetic risk for HD.
Results: Nearly 40% of pre-HD individuals met criteria for MCI, and individuals closer to HD diagnosis had higher rates of MCI. Nonamnestic MCI was more common than amnestic MCI. Single-domain MCI was more common than multiple-domain MCI. Within the nonamnestic single-domain subtype, impairments in processing speed were most frequent.
Conclusions: Consistent with the Alzheimer disease literature, MCI as a prodromal period is a valid concept in pre-HD, with nearly 40% of individuals showing this level of impairment before diagnosis. Future studies should examine the utility of MCI as a marker of cognitive decline in pre-HD.
Footnotes
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Editorial, page 490
e-Pub ahead of print on July 7, 2010, at www.neurology.org.
Study funding: Supported by the NIH (NINDS 40068 [J.P.], NIMH 01579, and NIA 1K23AG028417 [K.D.]); the Roy J. and Lucille Carver Trust; the Howard Hughes Medical Institute; the Huntington Disease Society of America; and CHDI Foundation, Inc. (formerly High Q Foundation).
Disclosure: Author disclosures are provided at the end of the article.
Received December 30, 2009. Accepted in final form March 29, 2010.
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