Mitsumoto Hiroshi, Rabkin Judith G
Eleanor and Lou Gehrig ALS/MDA Center, College of Physicians and Surgeons, Columbia University, Neurological Institute, New York, NY 10032, USA.
JAMA. 2007 Jul 11;298(2):207-16. doi: 10.1001/jama.298.2.207.
Amyotrophic lateral sclerosis (ALS) is a devastating terminal neurodegenerative disease with a highly predictable clinical course such that palliative care should begin at or soon after diagnosis. The outcome is certain in most cases. The only medication approved for treatment in the United States, riluzole, extends life by about 2 months. Virtually all skeletal muscles eventually are affected. Multiple problems require a multidisciplinary approach including aggressive symptomatic management, rehabilitation to maintain motor function, nutritional and respiratory support, augmentative communication devices, and psychological support for both patients and families because family members so often play a central role in management and care. Social, bioethical, and financial issues as well as advance directives should be addressed long before enteral feeding or assistive ventilatory support might be considered. Goals of care should be assessed on an ongoing basis. Presenting the unusual case of a patient with ALS who is also a prominent neurologist specializing in ALS, we enumerate issues in management and palliative care applicable to ALS but also to other fatal, progressive neurologic diseases such as Huntington's chorea and late-stage Parkinson disease.
肌萎缩侧索硬化症（ALS）是一种毁灭性的晚期神经退行性疾病，其临床病程具有高度可预测性，因此姑息治疗应在诊断时或诊断后不久开始。在大多数情况下，结局是确定的。美国唯一批准用于治疗的药物利鲁唑可将生命延长约2个月。几乎所有骨骼肌最终都会受到影响。多种问题需要多学科方法，包括积极的症状管理、维持运动功能的康复治疗、营养和呼吸支持、辅助沟通设备，以及为患者和家属提供心理支持，因为家庭成员在管理和护理中往往起着核心作用。在考虑肠内喂养或辅助通气支持之前很久，就应该解决社会、生物伦理和财务问题以及预先指示。应持续评估护理目标。通过介绍一位患有ALS的患者的特殊病例，该患者也是一位专门研究ALS的著名神经科医生，我们列举了适用于ALS以及其他致命的、进行性神经疾病（如亨廷顿舞蹈症和晚期帕金森病）的管理和姑息治疗问题。
