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Huntington Disease l: Introduction

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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...

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Related Experiment Video

Updated: May 6, 2026

Behavioral Phenotyping of Murine Disease Models with the Integrated Behavioral Station INBEST

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Capturing Behavioral Symptoms in Huntington's Disease Using the Huntington's Disease-Behavioral Questionnaire.

Siena Rigatuso¹, Krisha Bagga¹, Shelby Hughes^1,2

¹Department of Neurosciences, University of California, San Diego, San Diego, California, USA.

Movement Disorders Clinical Practice

|January 20, 2026

Summary

This summary is machine-generated.

The Huntington's Disease Behavioral Questionnaire (HD-BQ) effectively captures behavioral symptoms in Huntington's Disease (HD). Companion reports reveal more severe symptoms than patient self-reports, highlighting anosognosia in HD patients.

Keywords:

Huntington's disease anosognosia behavioral symptoms dual‐informant assessment

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Area of Science:

Neuroscience
Clinical Psychology
Genetics

Background:

The Huntington's Disease Behavioral Questionnaire (HD-BQ) assesses behavioral symptoms across cognitive, psychiatric, and functional domains in Huntington's Disease (HD).
The HD-BQ includes both patient- and companion-reported versions to account for anosognosia, a common deficit in HD.

Purpose of the Study:

To evaluate the effectiveness of the HD-BQ in identifying and quantifying behavioral symptoms experienced by individuals with HD.
To compare patient self-assessments with companion-reported symptoms to understand symptom perception discrepancies.

Main Methods:

The HD-BQ was administered to 71 individuals with manifest HD and their companions, alongside 71 healthy controls (HC).
Statistical analyses, including Mann-Whitney U and Wilcoxon signed-ranks tests, were used to compare HD-BQ scores between groups.

Main Results:

Individuals with HD reported significantly more severe behavioral symptoms compared to healthy controls (p < 0.001).
Companions reported more widespread and severe behavioral symptoms in HD patients than the patients themselves (Z = -3.7, p < 0.001).
Notable discrepancies between patient and companion reports were found in cognitive domains, including difficulties with shifting thoughts, concentration, managing commitments, judgment, and indecisiveness.

Conclusions:

The HD-BQ is a valuable tool for capturing a broad spectrum of behavioral disturbances associated with Huntington's Disease.
Companion reports consistently indicate greater symptom frequency and severity than patient self-reports, underscoring the impact of anosognosia in HD.