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Related Experiment Video

Updated: Sep 9, 2025

Whole-brain Segmentation and Change-point Analysis of Anatomical Brain MRI—Application in Premanifest Huntington's Disease
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How Early Does Anosognosia Begin in Huntington's Disease?

Krisha Bagga1, Allen Xu1, Andrew Hall1

  • 1Neurosciences, UC San Diego, La Jolla, CA, USA.

Movement Disorders Clinical Practice
|September 1, 2025
PubMed
Summary

Anosognosia, or lack of disease awareness, is present early in Huntington's disease (HD). This finding suggests caution when using self-reported data in HD clinical research.

Keywords:
CAP scoreHD‐ISS stagingHuntington's diseaseanosognosiacomposite UHDRStotal functional capacity

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Area of Science:

  • Neuroscience
  • Neurology
  • Clinical Research

Background:

  • Huntington's disease (HD) frequently involves anosognosia, a deficit in self-awareness of impairments.
  • The precise onset of anosognosia during the progression of HD is not well-understood.

Purpose of the Study:

  • To determine when anosognosia emerges in individuals with Huntington's disease.
  • To assess the presence and absence of anosognosia across different stages of HD.

Main Methods:

  • The Anosognosia Scale was administered to 60 Huntington's disease gene carriers and their study partners.
  • Participants were stratified using the HD Integrated Staging System (HD-ISS), Total Functional Capacity (TFC), CAG-Age-Product (CAP), and composite Unified Huntington's Disease Rating Scale (cUHDRS).
  • Anosognosia was identified by a score difference of ≥6 points between patient and informant reports.

Main Results:

  • Anosognosia was observed in 18% of participants in HD-ISS stage 0/1.
  • 22% of individuals with a TFC score of 13 exhibited anosognosia.
  • 37% of participants eligible for clinical trials (CAP score 400-500) and 23% with a cUHDRS ≥14 showed signs of anosognosia.

Conclusions:

  • Anosognosia is an early-occurring symptom in Huntington's disease.
  • The early onset of anosognosia indicates a need to limit reliance on self-report measures in HD clinical studies.