Goetz C G
Department of Neurological Sciences, Rush University/Rush Presbyterian St. Luke's Medical Center, Chicago, Illinois 60612, USA.
Muscle Nerve. 2000 Mar;23(3):336-43. doi: 10.1002/(sici)1097-4598(200003)23:3<336::aid-mus4>3.0.co;2-l.
Amyotrophic lateral sclerosis is historically an important entity because its manifestations involve distinct signs that can be correlated with gray and white matter lesions at specific sites within the central nervous system. Working at the end of the nineteenth century, the celebrated neurologist, Jean-Martin Charcot, used this disorder as a prototypic example of the power of his research method, termed "méthode anatomoclinique." Using clinical cases and autopsy material, he showed how anatomical lesions in the nervous system could be accurately determined by the presence of carefully analyzed clinical signs. Charcot's work on amyotrophic lateral sclerosis brought together neurological entities formerly considered as disparate disorders, primary amyotrophy and primary lateral sclerosis. In addition, these studies contributed to the understanding of spinal cord and brain stem anatomy and the organization of the normal nervous system. Because of Charcot's fundamental contributions, the eponym "Charcot's disease" has been used internationally in association with amyotrophic lateral sclerosis.
从历史角度来看，肌萎缩侧索硬化症是一个重要的病症，因为其临床表现涉及一些独特的体征，这些体征可与中枢神经系统特定部位的灰质和白质病变相关联。19世纪末，著名神经学家让 - 马丁·夏科运用这种病症作为他所谓“解剖临床法”研究方法强大之处的典型例子。通过临床病例和尸检材料，他展示了如何通过仔细分析临床体征的存在来准确确定神经系统中的解剖病变。夏科关于肌萎缩侧索硬化症的研究将以前被视为不同病症的原发性肌萎缩和原发性侧索硬化这两种神经病症归为一类。此外，这些研究有助于人们对脊髓和脑干解剖结构以及正常神经系统组织的理解。由于夏科的基础性贡献，“夏科病”这一名称在国际上一直与肌萎缩侧索硬化症相关联使用。
