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Huntington's disease patients display progressive deficits in hippocampal-dependent cognition during a task of

Kate L Harris1, Matthew Armstrong1, Rachel Swain1

  • 1Department of Clinical Neurosciences, John van Geest Centre for Brain Repair, University of Cambridge, Cambridge, UK.

Cortex; a Journal Devoted to the Study of the Nervous System and Behavior
|September 10, 2019
PubMed

Insights

Huntington's disease (HD) patients show significant spatial memory deficits, linked to disease burden, not basal ganglia damage. Premanifest gene carriers performed normally, indicating HD-specific cognitive decline.

Area of Science:

  • Neuroscience
  • Cognitive Science
  • Genetics

Background:

  • Cognitive disturbances are early symptoms of Huntington's disease (HD).
  • Frontal-striatal pathway degeneration is the presumed cause of cognitive decline.
  • Emerging evidence suggests hippocampal dysfunction may also contribute to cognitive impairments in HD.

Purpose of the Study:

  • To investigate the role of hippocampal dysfunction in cognitive deficits in Huntington's disease (HD).
  • To assess spatial memory and perception using a novel task in HD patients and gene carriers.

Main Methods:

  • A spatial memory task was administered to 32 healthy controls, 11 diagnosed HD patients, 16 premanifest HD gene carriers, and 5 basal ganglia lesion patients.
  • The task measured perception and short-term spatial memory using computer-generated visual scenes.

Main Results:

  • HD patients exhibited significant impairments in spatial perception and memory, correlating with disease burden score (DBS).
  • Premanifest HD gene carriers performed comparably to healthy controls.
  • Patients with basal ganglia lesions showed no task-related impairments.

Conclusions:

  • Significant deficits in hippocampal-dependent spatial cognition exist in Huntington's disease (HD).
  • These deficits are not solely attributable to basal ganglia degeneration.
  • Cognitive impairments in HD are related to the disease process and worsen with increased disease burden.
Abstract

Keywords:
CognitionHippocampusHuntington's diseaseNeuropsychologySpatial memory

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