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亨廷顿舞蹈症

Huntington's Disease.

作者信息

Higgins Donald S

机构信息

Parkinson's Disease and Movement Disorders Center, Albany Medical College, 215 Washington Avenue Extension, Albany, NY 12205, USA.

出版信息

Curr Treat Options Neurol. 2006 May;8(3):236-44. doi: 10.1007/s11940-006-0014-x.

Abstract

Although available treatments for Huntington's disease (HD) are imperfect, thoughtful application can positively impact quality of life. Dopamine antagonists can provide control of the troublesome hyperkinetic movements. These agents can also diminish the frequency of hallucinations and delusions when symptoms of psychosis occur. Classical neuroleptics have the widest utilization, although atypical antipsychotics are being increasingly used. Suppression of choreiform movements has also been reported with amantadine and tetrabenazine, which is not currently approved in the United States but under investigation. Alteration in mood can be successfully managed with a variety of antidepressant medications. Superior tolerability and value in the management of a variety of behavioral disturbances have lead to extensive use of serotonin reuptake inhibitors. Modest disturbance of mood can sometimes be addressed with anticonvulsant medications. Considered a manifestation of advanced disease, dementia is less commonly addressed therapeutically. However, gathering experience suggests improved cognitive function can occur with cholinesterase inhibitor therapy. Frequently overlooked is the value of rehabilitation services in the management of diverse symptoms. Although the value of a dysphagia evaluation is apparent, the benefit to be derived from physical and occupational therapy involvement cannot be overstated. Current therapeutic trials will undoubtedly provide additional therapies to moderate symptoms, but once the mechanism(s) of selective striatal projection neuron degeneration are delineated, a revolution in the management of HD will occur.

摘要

尽管亨廷顿舞蹈症(HD)的现有治疗方法并不完美,但合理应用可对生活质量产生积极影响。多巴胺拮抗剂可控制恼人的运动亢进。当出现精神病症状时,这些药物还可减少幻觉和妄想的发生频率。经典抗精神病药物应用最为广泛,不过非典型抗精神病药物的使用也日益增多。金刚烷胺和丁苯那嗪也被报道可抑制舞蹈样动作,丁苯那嗪目前在美国尚未获批,但仍在研究中。多种抗抑郁药物可成功控制情绪变化。5-羟色胺再摄取抑制剂因其耐受性良好且在管理多种行为障碍方面具有价值,因而得到广泛应用。轻度情绪障碍有时可用抗惊厥药物治疗。痴呆被视为晚期疾病的一种表现,较少进行治疗。然而,越来越多的经验表明,胆碱酯酶抑制剂治疗可改善认知功能。康复服务在管理各种症状方面的价值常常被忽视。尽管吞咽困难评估的价值显而易见,但物理治疗和职业治疗的益处再怎么强调也不为过。目前的治疗试验无疑将提供更多缓解症状的疗法,但一旦明确选择性纹状体投射神经元变性的机制,HD的治疗将迎来变革。

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