Dharmadasa Thanuja, Matamala José M, Kiernan Matthew C
Brain and Mind Centre, Sydney Medical School, University of Sydney, Sydney, New South Wales, Australia.
Curr Opin Neurol. 2016 Oct;29(5):581-91. doi: 10.1097/WCO.0000000000000369.
Although there is no cure for motor neurone disease (MND), the advent of multidisciplinary care and neuroprotective agents has improved treatment interventions and enhanced quality of life for MND patients and their carers.
Evidence-based multidisciplinary care, respiratory management and disease-modifying therapy have improved the outcomes of patients diagnosed with MND. Supportive approaches to nutritional maintenance and optimization of symptomatic treatments, including management of communication and neuropsychiatric issues, improve the quality of life for MND patients.
Recent progress in the understanding of the clinical, pathophysiological and genetic heterogeneity of MND has improved the approach of clinicians to treatment. Notwithstanding improvement to care and quality of life, survival benefit has become evident with the advent of a multidisciplinary care framework, early treatment with riluzole and noninvasive ventilation. Weight maintenance remains critical, with weight loss associated with more rapid disease progression. The end-of-life phase is poorly defined and treatment is challenging, but effective symptom control through palliative care is achievable and essential. Encouragingly, current progress of clinical trials continues to close the gap towards the successful development of curative treatment in MND.
尽管运动神经元病（MND）无法治愈，但多学科护理和神经保护药物的出现改善了治疗干预措施，提高了MND患者及其护理人员的生活质量。
基于证据的多学科护理、呼吸管理和疾病修饰疗法改善了MND诊断患者的预后。营养维持的支持性方法和对症治疗的优化，包括沟通和神经精神问题的管理，提高了MND患者的生活质量。
对MND临床、病理生理和基因异质性认识的最新进展改善了临床医生的治疗方法。尽管护理和生活质量有所改善，但随着多学科护理框架的出现、利鲁唑的早期治疗和无创通气，生存获益已变得明显。体重维持仍然至关重要，体重减轻与疾病进展更快相关。临终阶段定义不明确且治疗具有挑战性，但通过姑息治疗实现有效的症状控制是可行且必不可少的。令人鼓舞的是，目前临床试验的进展继续缩小与成功开发MND治愈性治疗之间的差距。
