Bonduelle M
Rev Neurol (Paris). 1990;146(2):97-106.
A description of progressive muscular atrophy, the first item in neuro-muscular nosography, figures in the memoir published by F.A. Aran in 1850. There, all the essential features of the disease can be found: its usual onset at the distal end of the upper limbs, its slowly progressive worsening, with muscular atrophy sparing certain muscles or muscular fascicles, its peculiar "claw hand", its muscular "fasciculations" and cramps, with untouched sensitivity. After praising Aran's "beautiful description", G.B. Duchenne de Boulogne subsequently persisted in claiming paternity, untiringly referring to a memoir on "muscular atrophy with fatty transformation" said to have been submitted to the Académie des Sciences in 1849. There is no trace of this memoir, and while it is true that the "localized electrisation" technique was applied by Duchenne to all the patients in Aran's memoir, and that he was the sole author of two of his observations, it is Aran who must be credited with the clinical description, the synthetic presentation and the appellation of "progressive muscular atrophy". Initially, this term covered a number of disparate facts which were later identified and put in their proper nosological place, even though this dismemberment left standing what Charcot called "Duchenne-Aran disease" before the Aran-Duchenne denomination prevailed. This denomination is now customary, and rightly so.
进行性肌萎缩的描述是神经肌肉疾病分类中的第一项，出现在F.A. 阿让于1850年发表的论文中。在该论文中，可以找到该病的所有基本特征：通常起病于上肢远端，病情缓慢进展恶化，肌肉萎缩不累及某些肌肉或肌束，其特有的“爪形手”，肌肉“肌束震颤”和痉挛，而感觉不受影响。在赞扬了阿让的“精彩描述”之后，G.B. 迪谢纳·德·布洛涅随后坚持宣称这是他的功劳，不厌其烦地提及一篇关于“脂肪变性的肌肉萎缩”的论文，据说该论文于1849年提交给了科学院。但这篇论文毫无踪迹可寻，虽然迪谢纳确实将“局部电刺激”技术应用于阿让论文中的所有患者，并且他是其中两项观察结果的唯一作者，但临床描述、综合呈现以及“进行性肌萎缩”这一称谓应归功于阿让。最初，这个术语涵盖了一些不同的情况，后来这些情况被识别并归入适当的疾病分类位置，尽管在阿让 - 迪谢纳这一命名流行之前，这种拆分使得夏科所称的“迪谢纳 - 阿让病”依然存在。现在这个命名是惯例，且当之无愧。
