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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,...
Role of Hippocampus in Memory01:19

Role of Hippocampus in Memory

The hippocampus, a critical brain structure, plays an essential role in memory processing, particularly in the formation and retrieval of memory. This small, seahorse-shaped region is located within the medial temporal lobe, with one hippocampus in each brain hemisphere. Experimental studies involving lesions in the hippocampi of rats have demonstrated significant impairments in tasks such as object recognition and maze navigation, indicating the hippocampus involvement in both recognition and...
Working Memory01:24

Working Memory

Working memory refers to a combination of components, including short-term memory and attention, that allow an individual to hold information temporarily as we perform cognitive tasks. It is an essential cognitive function that enables the execution of complex tasks such as problem-solving, comprehension, and reasoning. Unlike short-term memory, which simply involves the storage of information for a brief period, working memory involves the active manipulation and processing of this information.

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Related Experiment Video

Updated: Jul 3, 2026

Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
09:06

Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease

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Visuospatial working memory in Huntington's disease: behavioural and structural brain correlates.

Marine Lunven1,2, Pamela Van Den Enden3,2, Andrès Gil Salcedo3,2

  • 1Département d'Etudes Cognitives, PSL University, Paris, France marine.lunven@u-pec.fr.

Journal of Neurology, Neurosurgery, and Psychiatry
|July 1, 2026
PubMed
Summary

Huntington

Keywords:
COGNITIVE NEUROPSYCHOLOGYHUNTINGTON'SIMAGE ANALYSISMEMORY

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Area of Science:

  • Neuroscience
  • Cognitive Science
  • Neurology

Background:

  • Visuospatial working memory (VSWM) deficits are early indicators in Huntington's disease (HD), preceding motor symptoms.
  • These deficits likely stem from early disruptions in cortico-striatal circuits.
  • The precise cognitive components and brain correlates of VSWM decline in HD are not well understood.

Purpose of the Study:

  • To characterize the progression of VSWM deficits across different stages of Huntington's disease (HD).
  • To identify the structural brain correlates associated with these VSWM deficits.
  • To investigate the utility of the object-location task (OLT) for detecting cognitive decline in HD.

Main Methods:

  • A prospective international study involving 92 Huntington's disease mutation carriers (HDmc) and 39 healthy controls.
  • Cognitive assessments using the object-location task (OLT) and structural MRI were performed at baseline, with a 1-year follow-up for a subset of participants.
  • MRI analyses focused on striatal (caudate, putamen), hippocampal volumes, and cortical thickness.

Main Results:

  • Premanifest HD showed increased location error and spatial imprecision, correlating with striatal atrophy and parietal/cingulate cortical thinning.
  • Manifest HD individuals exhibited swap errors and hippocampal atrophy, with both error types worsening over time.
  • Principal component analysis revealed distinct striato-cortically mediated location deficits and hippocampally mediated binding errors, supporting a hierarchical VSWM decline model.

Conclusions:

  • Huntington's disease-related VSWM impairment progresses through distinct stages with specific anatomical correlates.
  • The object-location task (OLT) provides sensitive, mechanistic markers for cognitive decline in HD.
  • These markers have potential for early detection and stratification in clinical trials for Huntington's disease.