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Behavioural problems in Huntington's disease using the Problem Behaviours Assessment
Elisabeth M Kingma1, Erik van Duijn, Reinier Timman
1Department of Psychiatry, Leiden University Medical Centre, 2300 RC Leiden, The Netherlands.
Insights
Huntington's disease (HD) behavioral issues like apathy, depression, and irritability are present even before motor symptoms appear. These problems, assessed reliably by the Problem Behaviours Assessment (PBA), worsen with disease progression.
Area of Science:
- Neuroscience
- Psychiatry
- Genetics
Background:
- Huntington's disease (HD) is a neurodegenerative disorder.
- Behavioral problems are a significant aspect of HD's clinical phenotype.
- Understanding the timing and nature of these problems is crucial for patient care.
Purpose of the Study:
- To investigate the spectrum and progression of behavioral problems in individuals with Huntington's disease (HD).
- To assess the reliability and factor structure of the Problem Behaviours Assessment (PBA) in an HD population.
- To determine if behavioral problems manifest before the onset of motor symptoms.
Main Methods:
- The Dutch version of the Problem Behaviours Assessment (PBA) was administered to 152 HD mutation carriers and 56 non-carriers.
- HD mutation carriers were stratified into pre-symptomatic, early symptomatic, and advanced symptomatic groups based on motor assessments (UHDRS).
- Interrater reliability and factor structure of the PBA were analyzed.
Main Results:
- The PBA demonstrated clinically relevant interrater reliability (0.82 for severity, 0.73 for frequency).
- A three-factor solution emerged: apathy, depression, and irritability.
- HD mutation carriers, including pre-symptomatic individuals, exhibited higher levels of apathy, depression, and irritability compared to non-carriers.
- Apathy increased with disease progression (pre-symptomatic < early < advanced), while depression and irritability did not significantly differ between pre-symptomatic and early symptomatic stages.
Conclusions:
- The Problem Behaviours Assessment (PBA) is a reliable and sensitive tool for evaluating behavioral issues in Huntington's disease (HD).
- Behavioral problems, including apathy, depression, and irritability, are integral to the HD phenotype and emerge prior to motor symptom manifestation.
- Apathy correlates with Huntington's disease severity, underscoring the need for integrated, multidisciplinary care throughout all disease stages.
Objective:
To investigate behavioural problems in Huntington's disease (HD).
Method:
In 152 HD mutation carriers and a control group of 56 noncarriers at initial 50% risk, the Dutch version of the Problem Behaviours Assessment (PBA) was administered. Mutation carriers were divided into three groups according to the motor section of the Unified Huntington's Disease Rating Scale (UHDRS): pre-(motor) symptomatic, early and advanced symptomatic subjects. The factor structure and interrater reliability of the PBA were investigated.
Results:
The clinically relevant interrater reliability of the PBA was 0.82 for severity scores and 0.73 for frequency scores. The PBA showed a three-factor solution: apathy, depression and irritability. Mutation carriers, including presymptomatic subjects, portrayed more apathy, depression and irritability than noncarriers. Early symptomatic subjects had more apathy, but not more depression or irritability, compared to presymptomatic subjects. Advanced symptomatic subjects had more apathy than early symptomatic subjects.
Conclusions:
The PBA is a reliable and sensitive instrument. Behavioural problems occur in all stages of HD and arise before the onset of motor symptoms. Apathy is related to disease severity, whereas depression and irritability are not. The broad clinical phenotype of HD therefore requires adequate service delivery with integrated and multidisciplinary patient care.
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