Neuropsychiatric aspects of Huntington's disease

TLDR

Neuropsychiatric symptoms are common in Huntington’s disease, often presenting early, yet research on them is variable and limited. The study aims to better understand these symptoms to improve disease management, prognosis, and quality of life for patients and caregivers. The authors evaluated 52 Huntington’s disease patients and their caregivers with standardized cognitive, psychiatric, motor, and neuropsychiatric inventory measures, and explored links to frontal‑striatal circuitry. Nearly all patients (98%) exhibited neuropsychiatric symptoms—primarily dysphoria, agitation, irritability, apathy, and anxiety—ranging from mild to severe and independent of cognitive or motor deficits, highlighting the need for dimensional assessment in Huntington’s disease.

Abstract

<h3>OBJECTIVE</h3> Neuropsychiatric symptoms are common in Huntington9s disease and have been considered its presenting manifestation. Research characterising these symptoms in Huntington9s disease is variable, however, encumbered by limitations within and across studies. Gaining a better understanding of neuropsychiatric symptoms is essential, as these symptoms have implications for disease management, prognosis, and quality of life for patients and caregivers. <h3>METHOD</h3> Fifty two patients with Huntington9s disease were administered standardised measures of cognition, psychiatric symptoms, and motor abnormalities. Patient caregivers were administered the neuropsychiatric inventory. <h3>RESULTS</h3> Ninety eight per cent of the patients exhibited neuropsychiatric symptoms, the most prevalent being dysphoria, agitation, irritability, apathy, and anxiety. Symptoms ranged from mild to severe and were unrelated to dementia and chorea. <h3>CONCLUSIONS</h3> Neuropsychiatric symptoms are prevalent in Huntington9s disease and are relatively independent of cognitive and motor aspects of the disease. Hypothesised links between neuropsychiatric symptoms of Huntington9s disease and frontal-striatal circuitry were explored. Findings indicate that dimensional measures of neuropsychiatric symptoms are essential to capture the full range of pathology in Huntington9s disease and are vital to include in a comprehensive assessment of the disease.