Saft Carsten, Burgunder Jean-Marc, Dose Matthias, Jung Hans Heinrich, Katzenschlager Regina, Priller Josef, Nguyen Huu Phuc, Reetz Kathrin, Reilmann Ralf, Seppi Klaus, Landwehrmeyer Georg Bernhard
Department of Neurology, Huntington-Zentrum NRW, St. Josef-Hospital, Ruhr-Universität Bochum, Bochum, Germany.
Department of Neurology, Schweizerisches Huntington-Zentrum, Bern University, Bern, Switzerland.
Neurol Res Pract. 2023 Nov 16;5(1):61. doi: 10.1186/s42466-023-00285-1.
INTRODUCTION: Ameliorating symptoms and signs of Huntington's disease (HD) is essential to care but can be challenging and hard to achieve. The pharmacological treatment of motor signs (e.g. chorea) may favorably or unfavorably impact other facets of the disease phenotype (such as mood and cognition). Similarly, pharmacotherapy for behavioral issues may modify the motor phenotype. Sometimes synergistic effects can be achieved. In patients undergoing pragmatic polypharmacological therapy, emerging complaints may stem from the employed medications' side effects, a possibility that needs to be considered. It is recommended to clearly and precisely delineate the targeted signs and symptoms (e.g., chorea, myoclonus, bradykinesia, Parkinsonism, or dystonia). Evidence from randomized controlled trials (RCTs) is limited. Therefore, the guidelines prepared for the German Neurological Society (DGN) for German-speaking countries intentionally extend beyond evidence from RCTs and aim to synthesize evidence from RCTs and recommendations of experienced clinicians. RECOMMENDATIONS: First-line treatment for chorea is critically discussed, and a preference in prescription practice for using tiapride instead of tetrabenazine is noted. In severe chorea, combining two antidopaminergic drugs with a postsynaptic (e.g., tiapride) and presynaptic mode of action (e.g., tetrabenazine) is discussed as a potentially helpful strategy. Sedative side effects of both classes of compounds can be used to improve sleep if the highest dosage of the day is given at night. Risperidone, in some cases, may ameliorate irritability but also chorea and sleep disorders. Olanzapine can be helpful in the treatment of weight loss and chorea, and quetiapine as a mood stabilizer with an antidepressant effect. CONCLUSIONS: Since most HD patients simultaneously suffer from distinct motor signs and distinct psychiatric/behavioral symptoms, treatment should be individually adapted.
引言:改善亨廷顿舞蹈病(HD)的症状和体征对治疗至关重要,但具有挑战性且难以实现。运动体征(如舞蹈症)的药物治疗可能对疾病表型的其他方面(如情绪和认知)产生有利或不利影响。同样,行为问题的药物治疗可能会改变运动表型。有时可实现协同效应。在接受实用多药联合治疗的患者中,新出现的不适可能源于所用药物的副作用,这是一个需要考虑的可能性。建议清晰准确地描述目标体征和症状(如舞蹈症、肌阵挛、运动迟缓、帕金森症或肌张力障碍)。随机对照试验(RCT)的证据有限。因此,为德语国家的德国神经学会(DGN)制定的指南有意超越RCT的证据,旨在综合RCT的证据和经验丰富的临床医生的建议。 建议:对舞蹈症的一线治疗进行了批判性讨论,并指出在处方实践中更倾向于使用硫必利而非丁苯那嗪。在严重舞蹈症中,讨论了将两种具有不同作用方式的抗多巴胺能药物(一种为突触后作用方式,如硫必利;另一种为突触前作用方式,如丁苯那嗪)联合使用作为一种可能有用的策略。如果在夜间给予两类化合物中的最高剂量,其镇静副作用可用于改善睡眠。在某些情况下,利培酮可能改善易怒情绪,还可改善舞蹈症和睡眠障碍。奥氮平有助于治疗体重减轻和舞蹈症,喹硫平可作为具有抗抑郁作用的情绪稳定剂。 结论:由于大多数HD患者同时患有不同的运动体征和不同的精神/行为症状,治疗应个体化调整。
Neurol Res Pract. 2023-11-16
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