McCusker Elizabeth A, Loy Clement T
Huntington Disease Service, Westmead Hospital, Westmead, Australia.
Huntington Disease Service, Westmead Hospital, Westmead, Australia; School of Public Health, University of Sydney, Sydney, Australia.
Handb Clin Neurol. 2017;144:141-150. doi: 10.1016/B978-0-12-801893-4.00012-2.
The motor and movement disorders of Huntington disease (HD) are managed in the context of the other disease features. Chorea and dystonia are the most common HD-associated movement disorders, and they can be assessed on research rating scales. However other motor manifestations have a significant impact. In particular, dysphagia influences choice and tolerance of treatment for the movement disorder, as will comorbidities, patient awareness, and distress related to the motor feature or movement. Treatment for other disease features may aggravate the motor disorder, e.g., increased swallowing difficulty associated with antipsychotic agents. Basic principles in deciding to institute a treatment are outlined as well as treatment of specific motor manifestations and movements. There is a paucity of evidence to support the treatments available for the motor disorder, with only one agent with class 1 evidence, tetrabenazine, for chorea. There are, however, treatments informed by expert opinion which reflect the management of a wider HD phenotype than that represented in clinical trials. Some treatments are based on evidence from use in other conditions. Medical management is usually undertaken later in the disease with concurrent nonmedical interventions after multidisciplinary assessments. Medication review with HD progression is essential.
亨廷顿舞蹈症（HD）的运动和运动障碍是在其他疾病特征的背景下进行管理的。舞蹈症和肌张力障碍是与HD相关的最常见运动障碍，可通过研究评分量表进行评估。然而，其他运动表现也有重大影响。特别是吞咽困难会影响运动障碍治疗的选择和耐受性，合并症、患者意识以及与运动特征或运动相关的痛苦也会产生影响。其他疾病特征的治疗可能会加重运动障碍，例如，抗精神病药物会增加吞咽困难。本文概述了决定进行治疗的基本原则以及特定运动表现和运动的治疗方法。支持现有运动障碍治疗方法的证据很少，只有一种具有1类证据的药物——丁苯那嗪可用于治疗舞蹈症。然而，有一些基于专家意见的治疗方法，这些方法反映了比临床试验中所代表的更广泛的HD表型的管理。一些治疗方法是基于在其他病症中的使用证据。疾病后期通常在多学科评估后同时进行非药物干预的情况下进行药物治疗。随着HD病情进展进行药物审查至关重要。
