Mitsumoto Hiroshi, Howe Wesley J
Eleanor and Lou Gehrig MDA/ALS Research Center, Neurological Institute, Columbia University, USA.
Rinsho Shinkeigaku. 2006 Nov;46(11):828-31.
For the past decade or so, the research in ALS has dramatically changed, coinciding with the discovery of SOD1 mutations, the generation of SOD1 transgenic rodents, and the introduction of riluzole. The ALS practice guidelines have been published in a number of countries, whereas the National Database has shown the actual reality of patient practice in ALS. Multidisciplinary ALS Clinics, consisting of a team of health care professionals, provide highly specialized quality care for patients and families, and have become the standard of care in many countries. A number of mostly retrospective studies report that non-invasive positive pressure ventilation prolongs survival and improves the quality of life and other functions. Enteral feeding certainly improves quality of life, but it is still uncertain if it prolongs survival. Although end of life issues in ALS care have been difficult more and more ALS clinicians are paying serious attention to it. Clinical trials are imperative to find effective medications for ALS, and many different pharmacological agents, based on a number of different hypotheses in ALS, are being actively tested or are about to be tested in the near future. Undoubtedly, we are in the midst of incredible progress in ALS.
在过去十年左右的时间里，随着超氧化物歧化酶1（SOD1）突变的发现、SOD1转基因啮齿动物的培育以及利鲁唑的引入，肌萎缩侧索硬化症（ALS）的研究发生了巨大变化。许多国家都发布了ALS诊疗指南，而国家数据库展示了ALS患者实际的诊疗情况。由一组医疗保健专业人员组成的多学科ALS诊所为患者及其家属提供高度专业化的优质护理，并已成为许多国家的护理标准。一些大多为回顾性的研究报告称，无创正压通气可延长生存期，改善生活质量及其他功能。肠内营养肯定能改善生活质量，但它是否能延长生存期仍不确定。尽管ALS护理中的临终问题一直很棘手，但越来越多的ALS临床医生正在认真关注这一问题。开展临床试验以找到治疗ALS的有效药物势在必行，基于ALS的多种不同假设，许多不同的药物正在积极进行试验或即将在不久的将来进行试验。毫无疑问，我们正处于ALS令人难以置信的进展之中。
