Mitsumoto H, Hanson M R, Chad D A
Department of Neurology, Cleveland Clinic Foundation, OH 44106.
Arch Neurol. 1988 Feb;45(2):189-202. doi: 10.1001/archneur.1988.00520260077025.
We reviewed the current status of pathogenesis and therapeutic trials in amyotrophic lateral sclerosis (ALS). Clinical studies have identified several rare but definable causes for apparent ALS. Certain clinical features previously considered unlikely to occur in ALS are found on careful examination. Epidemiologic surveillance and recent studies of neurotoxic plant seeds used in Guam have shed light on the pathogenesis of endemic ALS. Extensive analyses of biochemical, metabolic, immunologic, viral, and toxic factors have provided provocative results requiring further studies. Reflecting on some of these hypotheses, therapeutic trials have been performed more vigorously than ever. Amyotrophic lateral sclerosis is now investigated at the molecular genetic level. Human autopsy and experimental animal studies have expanded our understanding of basic mechanisms involving motoneuronal degeneration. In the future, we must continue a relentless search for the pathogenesis of ALS, prospective clinical studies to define the limits of ALS, and well-designed, controlled therapeutic trials.
我们回顾了肌萎缩侧索硬化症（ALS）发病机制及治疗试验的现状。临床研究已确定了一些看似为ALS的罕见但可明确的病因。经仔细检查发现，某些以前认为不太可能在ALS中出现的临床特征确实存在。流行病学监测以及最近对关岛使用的神经毒性植物种子的研究，为地方性ALS的发病机制提供了线索。对生化、代谢、免疫、病毒和毒性因素的广泛分析得出了一些引人深思的结果，有待进一步研究。鉴于这些假说中的一些，治疗试验比以往任何时候都开展得更加积极。现在，肌萎缩侧索硬化症正在分子遗传学层面进行研究。人体尸检和实验动物研究拓展了我们对涉及运动神经元变性的基本机制的理解。未来，我们必须继续不懈地探寻ALS的发病机制，开展前瞻性临床研究以界定ALS的范围，并进行精心设计、对照的治疗试验。
