Mild cognitive impairment in prediagnosed Huntington disease

K Duff1, J Paulsen, J Mills

  • 1University of Utah, Salt Lake City, USA.

Neurology
|July 9, 2010
PubMed
Abstract

Insights

Nearly 40% of individuals in the pre-Huntington disease (HD) stage show mild cognitive impairment (MCI), a valid prodromal marker. Processing speed impairments were most common in this pre-HD cognitive decline group.

Area of Science:

  • Neuroscience
  • Neurology
  • Genetics

Background:

  • Cognitive decline is a known feature of Huntington disease (HD) and its pre-symptomatic phase (pre-HD).
  • The precise nature and prevalence of cognitive dysfunction before motor symptom onset in HD remain incompletely understood.
  • This study investigates subtle cognitive dysfunction, not meeting dementia criteria, in pre-HD individuals.

Purpose of the Study:

  • To determine the rate of mild cognitive impairment (MCI) in individuals with pre-HD.
  • To characterize the types of cognitive deficits observed in pre-HD.
  • To explore the relationship between proximity to HD diagnosis and MCI prevalence.

Main Methods:

  • Established normative cognitive data from 160 non-gene-expanded controls.
  • Applied MCI criteria (1.5 SD below the mean) to 575 gene-expanded pre-HD participants from the PREDICT-HD study.
  • Stratified pre-HD participants by motor signs and genetic risk for HD.

Main Results:

  • Approximately 40% of pre-HD individuals met criteria for MCI.
  • MCI rates increased as individuals neared clinical HD diagnosis.
  • Nonamnestic MCI was more prevalent than amnestic MCI, and single-domain MCI was more common than multiple-domain MCI.
  • Processing speed deficits were the most frequent impairment within the nonamnestic single-domain MCI subtype.

Conclusions:

  • Mild cognitive impairment (MCI) serves as a valid prodromal marker in the pre-HD period, mirroring findings in Alzheimer disease research.
  • Nearly 40% of individuals exhibit MCI before a formal HD diagnosis.
  • Further research is warranted to establish MCI's utility as a cognitive decline marker in pre-HD.

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