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Antidopaminergic treatment is associated with reduced chorea and irritability but impaired cognition in Huntington's disease (Enroll-HD).

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Kuan, Wei-Li 
Mason, Sarah L 
Barker, Roger A 


OBJECTIVES: Alterations in dopamine neurotransmission underlie some of the clinical features of Huntington's disease (HD) and as such are a target for therapeutic intervention, especially for the treatment of chorea and some behavioural problems. However, justification for such an intervention is mainly based on case reports and small open label studies and the effects these drugs have on cognition in HD remain unclear. METHODS: In this study, we used the Enroll-HD observational database to assess the effects of antidopaminergic medication on motor, psychiatric and cognitive decline, over a 3-year period. We first looked at the annual rate of decline of a group of HD patients taking antidopaminergic medication (n=466) compared with an untreated matched group (n=466). The groups were matched on specified clinical variables using propensity score matching. Next, we studied a separate group of HD patients who were prescribed such medications part way through the study (n=90) and compared their rate of change before and after the drugs were introduced and compared this to a matched control group. RESULTS: We found that HD patients taking antidopaminergic medication had a slower progression in chorea and irritability compared with those not taking such medications. However, this same group of patients also displayed significantly greater rate of decline in a range of cognitive tasks. CONCLUSION: In conclusion we found that antidopaminergic treatment is associated with improvements in the choreic movements and irritability of HD but worsens cognition. However, further research is required to prospectively investigate this and whether these are causally linked, ideally in a double-blind placebo-controlled trial.



Adult, Aged, Chorea, Cognition, Cognition Disorders, Databases, Factual, Dopamine Antagonists, Female, Humans, Huntington Disease, Irritable Mood, Male, Middle Aged

Journal Title

J Neurol Neurosurg Psychiatry

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Cambridge University Hospitals NHS Foundation Trust (CUH) (146281)
Department of Health (via National Institute for Health Research (NIHR)) (NF-SI-0616-10011)
Wellcome Trust (203151/Z/16/Z)
Medical Research Council (MR/S005528/1)
Medical Research Council (MC_PC_12009)
Medical Research Council (MC_PC_17230)
The authors would like to thank the patients and their families who participate in this study. The Enroll-HD is a CHDI Foundation Project and therefore we would like to thank the CHDI Foundation for granting us access to the Enroll-HD database. This research has been funded/supported by the National Institute for Health Research Rare Diseases Translational Research Collaboration (NIHR RD-TRC). The views expressed are those of the author(s) and not necessarily those of the NHS, the NIHR or the Department of Health. We would also like to thank the NIHR Cambridge Biomedical Research Centre, Dementia and Neurodegeneration Theme (146281). RAB is an NIHR Senior Investigator (NF-SI-0616-10011) and is supported by the WT/MRC Stem Cell Institute (203151/Z/16/Z).