F058 Anosognosia in HD: how early does it begin?

<h3>Background</h3> Individuals with Huntington’s disease (HD) commonly experience anosognosia, a lack of awareness of deficits. However, exactly when anosognosia begins in the course of HD remains unclear. <h3>Objective</h3> To characterize the onset of anosognosia in HD by examining its presence at various stages of disease. <h3>Methods</h3> The Anosognosia Scale (AS) consists of 8 items on which individuals rate their abilities relative to peers, with responses ranging from very impaired to excellent. The AS was given to 53 gene carriers stratified by CAG-Age- Product (CAP) and composite UHDRS (cUHDRS) score, in addition to their caregivers. Differences between caregiver and patient scores of ≥6 points were considered as evidence of anosognosia. <h3>Results</h3> 18% of individuals with CAP scores &lt;400 (mean DCL=2.64); 38% of individuals with CAP scores 400–500 (mean DCL=3.31); and 31% of individuals with CAP scores &gt;500 (mean DCL=4) showed anosognosia. Similarly, 21% (mean DCL=2.74); 26% (mean DCL=3.89); and 46% (mean DCL=4.0), respectively, of individuals stratified by cUHDRS score, showed anosognosia. AS score differences were greatest with regard to cognitive symptoms in the earliest stages of HD, but most pronounced with regard to motor/functional symptoms as the disease progressed. <h3>Conclusions</h3> Our findings suggest that anosognosia appears early in the course of HD, even prior to manifest disease onset. The lack of awareness of deficits, both cognitive and motor/functional, should dissuade against the use of self-report instruments as outcome measures in HD clinical research. Further studies will be needed to confirm and extend these findings.

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Published
2024-09-01
DOI
https://doi.org/10.1136/jnnp-2024-ehdn.176
Primary Topic
Spatial Neglect and Hemispheric Dysfunction
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article
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article

F058 Anosognosia in HD: how early does it begin?

Jody Corey‐Bloom, Paul E. Gilbert, Andrew Hall, Krisha Bagga et al.
Spatial Neglect and Hemispheric Dysfunction
article

F058 Anosognosia in HD: how early does it begin?

Jody Corey‐Bloom, Paul E. Gilbert, Andrew Hall, Krisha Bagga, Sean Patel, Nick Stovall, Sofia Gamboa, Allen Xu
article en

Abstract

<h3>Background</h3> Individuals with Huntington’s disease (HD) commonly experience anosognosia, a lack of awareness of deficits. However, exactly when anosognosia begins in the course of HD remains unclear. <h3>Objective</h3> To characterize the onset of anosognosia in HD by examining its presence at various stages of disease. <h3>Methods</h3> The Anosognosia Scale (AS) consists of 8 items on which individuals rate their abilities relative to peers, with responses ranging from very impaired to excellent. The AS was given to 53 gene carriers stratified by CAG-Age- Product (CAP) and composite UHDRS (cUHDRS) score, in addition to their caregivers. Differences between caregiver and patient scores of ≥6 points were considered as evidence of anosognosia. <h3>Results</h3> 18% of individuals with CAP scores &lt;400 (mean DCL=2.64); 38% of individuals with CAP scores 400–500 (mean DCL=3.31); and 31% of individuals with CAP scores &gt;500 (mean DCL=4) showed anosognosia. Similarly, 21% (mean DCL=2.74); 26% (mean DCL=3.89); and 46% (mean DCL=4.0), respectively, of individuals stratified by cUHDRS score, showed anosognosia. AS score differences were greatest with regard to cognitive symptoms in the earliest stages of HD, but most pronounced with regard to motor/functional symptoms as the disease progressed. <h3>Conclusions</h3> Our findings suggest that anosognosia appears early in the course of HD, even prior to manifest disease onset. The lack of awareness of deficits, both cognitive and motor/functional, should dissuade against the use of self-report instruments as outcome measures in HD clinical research. Further studies will be needed to confirm and extend these findings.

San Diego State University (US), University of California San Diego (US)
Openalex Percentile: Top 85%
Spatial Neglect and Hemispheric Dysfunction
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F058 Anosognosia in HD: how early does it begin? — Jody Corey‐Bloom, Paul E. Gilbert, et al. · (2024) | TGRS Research Map | TGRS