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.

中文翻译：

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抗多巴胺能治疗与亨廷顿氏病 (Enroll-HD) 患者的舞蹈病和易激惹减少但认知受损有关。

目标 多巴胺神经传递的改变是亨廷顿病 (HD) 某些临床特征的基础，因此是治疗干预的目标，尤其是舞蹈病和某些行为问题的治疗。然而，这种干预的理由主要基于病例报告和小型开放标签研究，这些药物对 HD 认知的影响仍不清楚。方法 在这项研究中，我们使用 Enroll-HD 观察数据库来评估抗多巴胺能药物在 3 年期间对运动、精神和认知能力下降的影响。我们首先观察了一组服用抗多巴胺能药物的 HD 患者 (n=466) 与未接受治疗的匹配组 (n=466) 的年下降率。使用倾向评分匹配，根据指定的临床变量对这些组进行匹配。接下来，我们研究了另一组 HD 患者，他们在研究的中途服用了此类药物 (n=90)，比较了他们在药物引入前后的变化率，并将其与匹配的对照组进行了比较。结果 我们发现，与未服用此类药物的患者相比，服用抗多巴胺能药物的 HD 患者在舞蹈病和易激惹方面的进展较慢。然而，同一组患者在一系列认知任务中也表现出明显更大的下降速度。结论 总之，我们发现抗多巴胺能治疗与 HD 的舞蹈运动和易激惹的改善有关，但会恶化认知。然而，