Mild cognitive impairment in prediagnosed Huntington disease

K Duff; J Paulsen; J Mills; L J Beglinger; D J Moser; M M Smith; D Langbehn; J Stout; S Queller; D L Harrington

doi:10.1212/WNL.0b013e3181eccfa2

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Mild cognitive impairment in prediagnosed Huntington disease

Journal article

Open access

Peer reviewed

Mild cognitive impairment in prediagnosed Huntington disease

K Duff, J Paulsen, J Mills, L J Beglinger, D J Moser, M M Smith, D Langbehn, J Stout, S Queller and D L Harrington

Neurology, Vol.75(6), pp.500-507

08/10/2010

DOI: 10.1212/WNL.0b013e3181eccfa2

PMCID: PMC2918475

PMID: 20610833

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Abstract

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. 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. 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. 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.

Predictive Value of Tests

Prospective Studies

Humans

Middle Aged

Risk Factors

Huntington Disease - psychology

Male

Cognition Disorders - genetics

Huntington Disease - complications

Cognition Disorders - diagnosis

Cognition Disorders - complications

Huntington Disease - genetics

Adult

Female

Huntington Disease - diagnosis

Cognition Disorders - psychology

Details

Title: Subtitle: Mild cognitive impairment in prediagnosed Huntington disease
Creators: K Duff - University of Utah, Salt Lake City, USA
J Paulsen
J Mills
L J Beglinger
D J Moser
M M Smith
D Langbehn
J Stout
S Queller
D L Harrington
Resource Type: Journal article
Publication Details: Neurology, Vol.75(6), pp.500-507
DOI: 10.1212/WNL.0b013e3181eccfa2
PMID: 20610833
PMCID: PMC2918475
NLM abbreviation: Neurology
ISSN: 0028-3878
eISSN: 1526-632X
Publisher: United States
Grant note: UL1 RR024979 / NCRR NIH HHS NS 40068 / NINDS NIH HHS Howard Hughes Medical Institute 1K23AG028417 / NIA NIH HHS MH 01579 / NIMH NIH HHS
Language: English
Date published: 08/10/2010
Academic Unit: Psychiatry; Psychological and Brain Sciences; Iowa Neuroscience Institute; Medicine Administration
Record Identifier: 9984020624602771

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