Objective Huntington disease (HD) is an autosomal dominant neurodegenerative disease involving motor disturbances, cognitive decline and psychiatric symptoms. Psychotic symptoms occur in a significant proportion of patients. We sought to characterise the clinical outcomes of this group of patients.
Methods Data were drawn from the Cooperative Huntington Observational Research Trial, a prospective, multi-centre observational study. 1082 patients with HD were recruited. Measures of cognition, function, behavioural disturbance and motor function were completed annually over 5 years.
Results Overall, 190 patients (17.6%) displayed psychotic symptoms. These patients demonstrated worse cognition, function and behavioural disturbances than patients without psychosis over time. Patients with psychosis also demonstrated lower levels of chorea than patients without psychosis, despite adjusting for concurrent antipsychotic and tetrabenazine use.
Conclusions Psychosis in HD is associated with poorer outcomes in cognition, function and behavioural symptoms. Patients with psychotic symptoms may also have less chorea. Altogether, the findings suggest patients with psychosis have a distinct clinical course.
- Huntington disease
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Presented at An earlier version of this work was presented at the Neuropsychiatric Syndromes symposium of the International Society to Advance Alzheimer's Research and Treatment (ISTAART) Professional Interest Area day at the Alzheimer’s Association International Conference (AAIC) on 13 July 2019 in Los Angeles, USA.
Contributors MHC helped to conceptualise the paper, analysed the data, interpreted the data and drafted the manuscript. AT-P helped to conceptualise the paper, provided statistical advice, interpreted the data and revised the manuscript for intellectual content. CTL contributed to acquisition of the data, helped to conceptualise the paper, provided statistical advice, interpreted the data and revised the manuscript for intellectual content.
Funding MHC was partially supported by the Dementia Centre for Research Collaboration, which is funded by the National Health and Medical Research Council (NHMRC) in Australia. AT-P was partially supported by NHMRC program grant 633003 to the Screening & Test Evaluation Program (STEP). CTL was supported by a NHMRC Dementia Research Development Fellowship (APP1107657). The CHDI Foundation, a non-for-profit organisation dedicated to finding treatments for people with Huntington disease, funded the COHORT study. The CHDI Foundation had no role in the analyses, data interpretation or writing of this paper.
Competing interests None declared.
Patient consent for publication Not required.
Ethics approval Ethics approval was obtained from institutional ethics committees associated with individual testing centres (National Institute of Health clinical trials registry number: NCT00313495).
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement Data are available upon reasonable request. Data may be obtained from a third party and are not publicly available.
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