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Dejerine: The concept of peripheral neuropathy - 28/01/17

Doi : 10.1016/j.neurol.2016.12.018 
Jean-Michel Vallat 1, , Stéphane Mathis 2
1 Service de neurologie, centre de référence des neuropathies rares, CHU de Limoges, 2, avenue Martin-Luther-King, 87000 Limoges, France 
2 Service de neurologie, unité nerf-Muscle, groupe hospitalier Pellegrin, CHU de Bordeaux, place Amélie-Raba-Léon, 33000 Bordeaux, France 

Corresponding author.

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Résumé

As noted by Michel Fardeau in 2010: “les travaux de Dejerine dans le domaine neuromusculaire ne représentent qu’une petite partie d’une œuvre impressionnante par son ampleur et par sa qualité puisqu’elle a résisté à l’œuvre du temps. Il n’y a pratiquement aucun chapitre de la clinique ou de l’anatomie du système nerveux central et périphérique auquel Dejerine n’ait apporté sa contribution” (“The studies of Dejerine on the neuromuscular system only make up a small part of an impressive body of work which has stood the test of time. He has in fact contributed to just about all clinical and anatomical aspects of the central and peripheral nervous systems”).

Dejerine became interested in the peripheral nervous system rather early in his career. In 1875, it stemmed from work in the Laboratory of Comparative Pathology directed by Prof. Vulpian. One of his first presentations was a study of the influence of electric currents on the appearance of secondary trophic disorders in a peripheral nerve lesion. In the same year, he started to investigate the pathophysiological processes involved in Wallerian degeneration and made a more thorough study of the process of distal degeneration of the peripheral motor nerve following the dissection of its cellular body (’trophic center’). From autopsies of patients with a previous limb amputation, he confirmed the homolateral medullary atrophy in the missing limb, and also showed a distal deterioration of the nerves of the missing limb (at the stump and never backing up to the spinal cord). Dejerine and Vulpian were thus convinced that this medullary atrophy was simply functional, a consequence of the distal suppression of the nerve trunks and not an extension of an “irritating” process of the nerves and muscles towards the spinal cord, contrary to the descriptions of other authors at the time (such as Friedreich on muscular atrophy of the Aran-Duchenne type).

Following on from the work of Octave Landry, he contributed descriptions of neuropathies of acute course, which later led to the entity of the Guillain-Barré-Strohl syndrome. Thus, in 1876, Dejerine and Goetz described the case of a patient presenting distal paraesthesias of all four limbs, sharp pains in the lower extremities, followed by a rapidly progressive quadriplegia over six days (along with fever and an urinary retention), leading to the death of the patient. On autopsy, the spinal cord was intact (both macroscopically and microscopically) throughout its length; a single anterior cervical root was studied, revealing a rather marked atrophy and a multiplication of “intertubular connective tissue” nodes. The authors proposed the diagnosis of “acute ascending paralysis”. The autopsies of other patients identified infiltrates of inflammatory cells in the nerve roots (1878) confirmed in 1969 by Asbury and colleagues. Comparing neuropathies of acute course with those of chronic course, Dejerine noted the difficulty in determining etiologies of neuropathies of chronic course. This is still true today.

Thereafter, Dejerine and his collaborators (1883) helped show that impairments of peripheral nerves were responsible for “locomotor ataxias” which were thought at the time to be tabetic “sclerosis of the posterior columns”. With the thorough study of the various peripheral neuropathies, especially based on autopsy findings, Dejerine can be viewed as a pioneer in showing, for example, that nerve damage in “neuritis” decreased towards the limb roots (later identified as a length-dependant retrograde degeneration (of the axons) described in English as a “length-dependantdying back” process); he identified and defined what he called “hypertrophic and progressive interstitial neuritis of childhood”, now called Dejerine-Sottas syndrome.

In addition, Dejerine (in collaboration with distinguished workers including his wife Augusta Dejerine-Klumpke, Sottas, André-Thomas, and numerous others) described cases of peripheral nervous system involvement that helped define known entities such as compression paralysis, toxic and infectious peripheral neuritis, several observations of “hypertrophic interstitial neuritis”, a case of “rheumatic facial palsy” (or “a frigore” facial palsy), etc. His publications and presentations are collected chronologically and summarized in the interesting “Epreuves de Titres and Travaux” published in 1895 and 1901.

Our presentation will recap and discuss the characteristics of what is now called Dejerine-Sottas syndrome (hypertrophic interstitial neuritis), and its links with Charcot-Marie-Tooth (CMT) disease, which strangely was refuted by Dejerine himself, and of its significance and relevance to the current classification of hereditary neuropathies in the molecular biological age. In addition, the electron microscopic studies of Gruner (1960) and Garcin and colleagues (1966) demonstrated that the homogeneous nervous hypertrophy of this condition is more related to a swirling Schwann cell proliferation, referred to as “onion bulbs” around the remaining axons, rather than a simple and marked increase in interstitial connective tissue.

The opus of Dejerine in determining, defining, and identifying peripheral nerve diseases along with the force of his clinical and neuropathologic descriptions illustrates the importance of clinical and pathological correlations. They have been subsequently confirmed and better defined using recent light and electron microscopic techniques.

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 Meeting abstract Jules Dejerine: Bilingual publication (English version).


© 2016  Publié par Elsevier Masson SAS.
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Vol 173 - N° S1

P. S17-S18 - février 2017 Retour au numéro
Article précédent Article précédent
  • Dejerine : le concept de neuropathie périphérique
  • Jean-Michel Vallat, Stéphane Mathis
| Article suivant Article suivant
  • Jules Dejerine, André-Thomas et la pathologie du cervelet
  • Emmanuel Broussolle

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