Broussolle E, Trocello J-M, Woimant F, Lachaux A, Quinn N
Service de neurologie C, université Claude-Bernard-Lyon I, hospices civils de Lyon, hôpital neurologique Pierre-Wertheimer, 59, boulevard Pinel, 69500 Bron, France; CNRS UMR 5229, centre de neurosciences cognitives, 67, boulevard Pinel, 69675 Bron cedex, France.
Rev Neurol (Paris). 2013 Dec;169(12):927-35. doi: 10.1016/j.neurol.2013.04.006. Epub 2013 Oct 11.
This historical article describes the life and work of the British physician Samuel Alexander Kinnier Wilson (1878-1937), who was one of the world's greatest neurologists of the first half of the 20th century. Early in his career, Wilson spent one year in Paris in 1903 where he learned from Pierre-Marie at Bicêtre Hospital. He subsequently retained uninterrupted links with French neurology. He also visited in Leipzig the German anatomist Paul Flechsig. In 1904, Wilson returned to London, where he worked for the rest of his life at the National Hospital for the Paralysed and Epileptic (later the National Hospital for Nervous Diseases, and today the National Hospital for Neurology and Neurosurgery) in Queen Square, and also at Kings' College Hospital. He wrote on 'the old motor system and the new', on disorders of motility and muscle tone, on the epilepsies, on aphasia, apraxia, tics, and pathologic laughing and crying, and most importantly on Wilson's disease. The other objective of our paper is to commemorate the centenary of Wilson's most important work published in 1912 in Brain, and also in Revue Neurologique, on an illness newly recognized and characterized by him entitled "Progressive lenticular degeneration, a familial nervous disease associated with liver cirrhosis". He analyzed 12 clinical cases, four of whom he followed himself, but also four cases previously published by others and a further two that he considered in retrospect had the same disease as he was describing. The pathological profile combined necrotic damage in the lenticular nuclei of the brain and hepatic cirrhosis. This major original work is summarized and discussed in the present paper. Wilson not only delineated what was later called hepato-lenticular degeneration and Wilson's disease, but also introduced for the first time the terms extrapyramidal syndrome and extrapyramidal system, stressing the role of the basal ganglia in motility. The present historical work emphasizes the special contributions made by Wilson to the study of movement disorders, including akinesia and bradykinesia in Parkinson's disease, and their relation to basal ganglia pathology.
这篇历史文章介绍了英国医生塞缪尔·亚历山大·金尼尔·威尔逊（1878 - 1937）的生平与工作，他是20世纪上半叶世界上最伟大的神经学家之一。在其职业生涯早期，威尔逊于1903年在巴黎待了一年，在比塞特尔医院师从皮埃尔·玛丽学习。随后，他与法国神经学保持着不间断的联系。他还前往莱比锡拜访了德国解剖学家保罗·弗莱施西格。1904年，威尔逊回到伦敦，此后他一生都在女王广场的国立瘫痪和癫痫医院（后来的国立神经疾病医院，如今的国立神经病学与神经外科医院）以及国王学院医院工作。他撰写了关于“旧运动系统与新运动系统”、运动障碍和肌张力障碍、癫痫、失语症、失用症、抽搐以及病理性哭笑等方面的文章，最重要的是关于威尔逊病的文章。我们本文的另一个目的是纪念威尔逊于1912年发表在《大脑》以及《神经学评论》上的最重要著作的百年纪念，该著作论述了一种他新发现并描述其特征的疾病，名为“进行性豆状核变性，一种与肝硬化相关的家族性神经疾病”。他分析了12个临床病例，其中4例是他自己跟踪观察的，还有4例是之前其他人发表的病例，另外2例是他回顾认为与他所描述疾病相同的病例。其病理特征包括脑内豆状核的坏死性损害和肝硬化。本文对这项重要的原创性工作进行了总结和讨论。威尔逊不仅明确了后来被称为肝豆状核变性和威尔逊病的病症，还首次引入了锥体外系综合征和锥体外系这两个术语，强调了基底神经节在运动中的作用。当前的这篇历史研究着重阐述了威尔逊对运动障碍研究的特殊贡献，包括帕金森病中的运动不能和运动迟缓，以及它们与基底神经节病理学的关系。
