Objective: To investigate the clinical and cognitive correlates of psychotic symptoms in patients with Huntington’s disease (HD).

Background: Psychiatric manifestations are common in HD, but their relationship with objective markers of disease severity remains incompletely understood. While affective symptoms are frequently reported, psychosis may represent a distinct neuropsychiatric phenotype associated with greater clinical impairment.

Method: This cross-sectional observational study included 55 patients with genetically confirmed HD followed at a specialized movement disorders center in Brazil. Participants underwent standardized clinical and neuropsychiatric assessment using the Unified Huntington’s Disease Rating Scale (UHDRS), Montreal Cognitive Assessment (MoCA), Frontal Assessment Battery (FAB), semantic verbal fluency tasks, and structured psychiatric evaluation with the Mini International Neuropsychiatric Interview (MINI). Clinical characteristics were compared between patients with and without psychotic symptoms.

Results: Psychotic symptoms were identified in 23.6% of patients. Patients with psychosis demonstrated significantly worse cognitive performance, including lower MoCA scores (median 11 vs. 17; p=0.007), reduced semantic verbal fluency (animals p=0.030; food p=0.033), and poorer executive performance on the FAB (p=0.029). Psychosis was also associated with greater motor severity, reduced functional independence, and longer disease duration, suggesting a broader pattern of clinical impairment.

Conclusion: Psychosis in HD is associated with worse cognitive performance and greater global disease severity. These findings suggest that psychotic symptoms may represent a marker of more advanced neurodegeneration and highlight the importance of systematic neuropsychiatric assessment in the clinical monitoring of HD.

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MARTINEZ-HORTA, S.; PEREZ-PEREZ, J.; VAN DUIJN, E. et al. Neuropsychiatric symptoms are very common in premanifest and early stage Huntington’s disease. Parkinsonism & Related Disorders, v. 25, p. 58–64, 2016. DOI: 10.1016/j.parkreldis.2016.02.008;

MCALLISTER, B.; GUSELLA, J. F.; LANDWEHRMEYER, G. B. et al. Timing and impact of psychiatric, cognitive, and motor abnormalities in Huntington’s disease. Neurology, v. 96, n. 19, p. e2395–e2406, 2021. DOI: 10.1212/WNL.0000000000011893;

ELLIS, N.; TEE, A.; MCALLISTER, B. et al. Genetic risk underlying psychiatric and cognitive symptoms in Huntington’s disease. Biological Psychiatry, v. 87, n. 9, p. 857–865, 2020. DOI: 10.1016/j.biopsych.2019.12.010;

BATES, G. P.; DORSEY, R.; GUSELLA, J. F. et al. Huntington disease. Nature Reviews Disease Primers, v. 1, p. 15005, 2015.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/psychotic-symptoms-and-cognitive-impairment-in-huntingtons-disease/