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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,...
Behavior Modification01:21

Behavior Modification

Behavioral approaches have often been criticized for ignoring mental processes and focusing solely on observable behavior. However, these approaches provide an optimistic perspective for individuals seeking to change their behaviors. Rather than concentrating on intrinsic personality traits, behavioral approaches suggest that even longstanding habits can be modified by changing the reward contingencies that maintain them.
A real-world application of operant conditioning principles is applied...

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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.

General Hospital Psychiatry
|February 23, 2008
PubMed
Summary
This summary is machine-generated.

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.

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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.