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肌萎缩侧索硬化症的症状管理

Author
Nestor Galvez-Jimenez, MD, MSc, MHSA, FACP
Section Editors
Jeremy M Shefner, MD, PhD
Ira N Targoff, MD
Deputy Editor
John F Dashe, MD, PhD
Translators
徐丽君, 主任医师，教授

引言

肌萎缩侧索硬化症(amyotrophic lateral sclerosis, ALS)是一种进展性神经变性疾病，可导致肌无力、残疾并最终死亡，中位生存期为3-5年。症状管理是ALS的主要治疗，本文将对其进行总结。有关ALS的其他方面将单独讨论。 (参见“肌萎缩侧索硬化症和其他运动神经元病的临床特征”和“肌萎缩侧索硬化症和其他类型运动神经元病的诊断”和“肌萎缩侧索硬化症的改变病情治疗”)

预后

ALS是一种进展性神经变性疾病。大部分患者在诊断后3-5年内死亡，而生存期更长的病例也并不罕见。大约30%的ALS患者诊断后5年仍存活，10%-20%的患者生存期超过10年。长期存活与症状出现时年龄较小、男性、以肢体症状而非延髓症状起病等有关[1-3]。

告知患者疾病诊断

临床医生告知患者及家属诊断为ALS是一项艰巨的任务。告知时医生应该有足够时间，确保不匆忙，并一定要面对面告知[4,5]。有支持患者的人在场的话可能会有所帮助，欧洲指南指出，如果患者的支持人数超过会议上医务人员的话有可感知到的益处[5]。提醒患者是坏消息、认可并观察患者的反应十分重要。要允许患者提问，要告诉他们ALS的并发症是可治疗的，保证不会放弃患者。如果患者期望的话，可以帮助他们询问其他医生的意见。在短时间内安排一次回访经常会有所帮助，这样便于解决问题，如果家庭成员愿意参加也可加入。

许多患者的诊断延迟，这是非常令人痛苦的。对于这些患者，作出诊断可能是一个好消息，这样可以减少不确定性，并可以开始积极的治疗。讨论诊断的同时也要讨论症状的治疗方法，并建立改变病情的治疗方案(假定不会因费用影响治疗)。 (参见“肌萎缩侧索硬化症的改变病情治疗”，关于‘利鲁唑’一节)

多学科治疗

多学科ALS诊所中参与治疗的人员包括神经科医师、物理治疗师、作业治疗师、语言治疗师、呼吸治疗师、营养师、社会工作者和护理人员[5-8]。因此，专业的诊所能够指导与ALS相关复杂问题的处理，包括呼吸症状、营养、构音障碍、吞咽困难、功能下降以及社会心理问题等。

                        

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Literature review current through: 2017-06 . | This topic last updated: 2017-01-18.
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