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Nonmotor Symptoms in Huntington Disease
1UFMG, Belo Horizonte, Brazil.
Insights
Huntington disease (HD) involves motor, cognitive, and behavioral changes. This chapter details nonmotor symptoms like depression and cognitive decline in HD patients and gene carriers, reviewing current management strategies.
Area of Science:
- Neuroscience
- Genetics
- Clinical Neurology
Background:
- Huntington disease (HD) is a neurodegenerative disorder.
- It is defined by motor, cognitive, and behavioral symptoms.
- Nonmotor symptoms are increasingly recognized as significant aspects of HD.
Purpose of the Study:
- To describe the frequency and clinical features of behavioral and cognitive dysfunction in Huntington disease.
- To review the management strategies for these nonmotor symptoms.
- To highlight the presence of these changes in premanifest gene carriers.
Main Methods:
- Literature review and expert consensus.
- Analysis of clinical features and frequency of nonmotor symptoms.
- Evaluation of current therapeutic options.
Main Results:
- Common behavioral abnormalities include depression, apathy, irritability, and compulsions.
- All patients with Huntington disease experience cognitive decline.
- Nonmotor symptoms are detectable even in premanifest Huntington disease gene carriers.
Conclusions:
- Behavioral and cognitive dysfunction are core features of Huntington disease.
- Management relies on expert agreement due to limited empirical data.
- Early identification of nonmotor symptoms in gene carriers is crucial for future interventions.
Abstract:
Huntington disease (HD) is characterized by the triad of motor abnormalities, cognitive dysfunction, and behavioral changes. The aim of this chapter is to describe the frequency, clinical features, and management of behavioral and cognitive dysfunction in HD. Depression, suicidal ideation, apathy, irritability, aggressiveness, obsessions, and compulsions are the most common behavioral abnormalities in HD. All HD patients develop cognitive decline. Recent data suggest that these nonmotor changes are found in premanifest HD gene carriers. Finally, we review the therapeutic options available for behavioral and cognitive abnormalities in HD. The management of these conditions is based on experts' agreement since there is a lack of empirical data.
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