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Related Concept Videos

Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
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One key aspect of implicit...
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Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and microglia. Abnormal...
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Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
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Abnormal explicit but normal implicit sequence learning in premanifest and early Huntington's disease.

Susanne A Schneider1, Leonora Wilkinson, Kailash P Bhatia

  • 1Sobell Department of Motor Neuroscience and Movement Disorders, UCL, Institute of Neurology, Queen Square,London, United Kingdom.

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Summary

Huntington's disease (HD) impairs explicit motor sequence learning, while implicit learning remains intact. Explicit sequence learning deficits may serve as an early cognitive biomarker for HD progression.

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Area of Science:

  • Neuroscience
  • Cognitive Science
  • Genetics

Background:

  • Motor sequence learning involves explicit (intentional) and implicit (incidental) processes.
  • The caudate nucleus and putamen, crucial for motor learning, are affected early in Huntington's disease (HD).
  • Existing research on motor sequence learning deficits in HD presents inconsistent findings.

Purpose of the Study:

  • To investigate implicit and explicit motor sequence learning in individuals with Huntington's disease.
  • To determine if motor sequence learning is impaired in premanifest and early-stage HD.
  • To explore the potential of explicit sequence learning as a biomarker for HD progression.

Main Methods:

  • Assessed implicit and explicit motor sequence learning in 15 individuals with a positive HD genetic test (premanifest and early stage) and 11 matched controls.
  • Utilized sequences of equivalent structure to evaluate both learning types.
  • Compared learning performance between the HD group and the control group.

Main Results:

  • The Huntington's disease group demonstrated normal implicit motor sequence learning.
  • Explicit motor sequence learning was impaired in both premanifest and manifest HD gene carriers.
  • A progressive decline in explicit sequence learning was observed with disease progression.

Conclusions:

  • Implicit motor sequence learning appears preserved in early stages of Huntington's disease.
  • Explicit motor sequence learning is compromised in Huntington's disease, even in premanifest stages.
  • Impaired explicit sequence learning may serve as a valuable cognitive biomarker for monitoring Huntington's disease progression.