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Published on: March 11, 2020
Neuropsychiatry of Huntington's disease
1Department of Psychiatry, Division of Neurobiology, Johns Hopkins University School of Medicine, Baltimore, MD 21287, USA. arosenb3@jhmi.edu
Insights
Huntington's disease (HD) presents with diverse psychiatric symptoms, including depression and executive dysfunction. Many of these neuropsychiatric conditions show positive responses to treatment, offering hope for improved patient care.
Area of Science:
- Neuroscience
- Psychiatry
- Genetics
Background:
- Psychiatric manifestations are a core feature of Huntington's disease (HD).
- These symptoms can mimic idiopathic disorders or be unique to HD, stemming from subcortical neuropathology.
- Common presentations include major depression and a distinct executive dysfunction syndrome.
Purpose of the Study:
- To review the spectrum of psychiatric manifestations in Huntington's disease.
- To discuss the potential neuropathological underpinnings of these symptoms.
- To explore treatment responsiveness and implications for broader psychiatric research.
Main Methods:
- Review of existing literature on psychiatric symptoms in Huntington's disease.
- Analysis of case series, anecdotal reports, and clinical experience regarding treatment.
- Synthesis of findings to understand the neuropsychiatry of HD.
Main Results:
- Psychiatric symptoms in HD range from idiopathic-like disorders to unique syndromes like executive dysfunction.
- Subcortical neuropathologic changes are the presumed cause of most psychiatric issues.
- Evidence suggests many HD psychiatric syndromes respond well to treatment, despite limited controlled studies.
Conclusions:
- Neuropsychiatric symptoms are a significant aspect of Huntington's disease.
- Further research into HD neuropsychiatry may illuminate mechanisms of psychiatric conditions in the general population.
- Prompt recognition and treatment of psychiatric symptoms can improve outcomes for HD patients.
Abstract:
Psychiatric manifestations are an integral part of Huntington's disease. They may be divided into those syndromes which resemble idiopathic disorders, but for which HD patients may be particularly at risk, those constellations which are peculiar to HD and related conditions, such as the executive dysfunction syndrome, and those symptoms that can truly be regarded as nonspecific, such as delirium. Most of these problems are believed to arise from subcortical neuropathologic changes. Major depression is a common psychiatric diagnosis, but the executive dysfunction syndrome, a difficult-to-define condition characterized by often simultaneous apathy and disinhibition, may be even more widespread. There are no large controlled studies of psychiatric treatments in HD, but case series, anecdotal reports, and clinical experience indicate that many of these syndromes respond readily to treatment. Further study of the neuropsychiatry of HD may help to reveal the underpinnings of psychiatric conditions found in the general population.
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