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<p>Huntington disease (HD), a devastating autosomal-dominant neurodegenerative disease resulting from an expanded CAG trinucleotide repeat, is clinically characterized by a triad of symptoms including involuntary motions, cognitive decline, and psychiatric disturbances. Psychotic, often schizophrenia-like symptoms, previously underestimated, have become an increasing challenge and have had a profound impact on quality of life and disease burden. Psychotic manifestations vary and may precede motor and cognitive changes, but can occur during the course of HD. In HD families, schizophrenia-like syndromes may affect most HD-members and polygenic risk scores for psychiatric disorders suggest a common genetic liability. Prevalence estimates of psychosis in HD vary widely (3%-11%). Like schizophrenia, it is associated with apathy, suicidality and social cognitive dysfunction, and frequently with worse motor, cognitive, and behavioral performance. Psychosis in HD is associated with a lower number of CAG repeats and younger age at clinical diagnosis, suggesting a unique phenotype in the HD spectrum. The neurobiology of psychosis in HD is not well understood, but shows some genetic and pathogenetic overlap with schizophrenia. Frontostriatal network dysfunctions, abnormal dopamine/glutamatergic signaling, dysregulation of calcium homeostasis in striatal and cortical neurons, and shared patterns of glial transcriptional dysregulation link these two otherwise unrelated disorders. The development of psychosis in HD is due to an interaction between genetic risk, neuronal changes, and environmental psychosocial stresses. Clinically, this implies a treatment of HD psychosis that is similar to that in schizophrenia, including antipsychotic drugs (e.g. Olanzapine) &amp; electroconvulsive therapy (ECT) for refractory psychiatric symptoms in HD, except for an awareness of the antipsychotic effects on HD motor symptoms.</p>

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Psychosis in Huntington disease: a short update

  • 期刊论文

  • 发表日期：

    2026年4月2日

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  • Kurt A. Jellinger

摘要

Huntington disease (HD), a devastating autosomal-dominant neurodegenerative disease resulting from an expanded CAG trinucleotide repeat, is clinically characterized by a triad of symptoms including involuntary motions, cognitive decline, and psychiatric disturbances. Psychotic, often schizophrenia-like symptoms, previously underestimated, have become an increasing challenge and have had a profound impact on quality of life and disease burden. Psychotic manifestations vary and may precede motor and cognitive changes, but can occur during the course of HD. In HD families, schizophrenia-like syndromes may affect most HD-members and polygenic risk scores for psychiatric disorders suggest a common genetic liability. Prevalence estimates of psychosis in HD vary widely (3%-11%). Like schizophrenia, it is associated with apathy, suicidality and social cognitive dysfunction, and frequently with worse motor, cognitive, and behavioral performance. Psychosis in HD is associated with a lower number of CAG repeats and younger age at clinical diagnosis, suggesting a unique phenotype in the HD spectrum. The neurobiology of psychosis in HD is not well understood, but shows some genetic and pathogenetic overlap with schizophrenia. Frontostriatal network dysfunctions, abnormal dopamine/glutamatergic signaling, dysregulation of calcium homeostasis in striatal and cortical neurons, and shared patterns of glial transcriptional dysregulation link these two otherwise unrelated disorders. The development of psychosis in HD is due to an interaction between genetic risk, neuronal changes, and environmental psychosocial stresses. Clinically, this implies a treatment of HD psychosis that is similar to that in schizophrenia, including antipsychotic drugs (e.g. Olanzapine) & electroconvulsive therapy (ECT) for refractory psychiatric symptoms in HD, except for an awareness of the antipsychotic effects on HD motor symptoms.