Journal : Revue photographique des hôpitaux de Paris ; vol. 1.
Paris : Adrien Delahaye, 1869.
Description : [1 l.] pl., 41-45 p. ; ill.: 1 photo. ; 22 cm.
Photograph : mounted albumen, composite of 5 images.
Photographer : Duchenne.
Subject : Primary Myopathy — Pseudohypertrophic Muscular Dystrophy ; Duchenne-Griesinger Disease.
Cited :
He described the initial pseudohypertrophies
in detail, but did not interpret them, as Erb did. The most definite thing which Duchenne
described was the bulbar or Glosso-labial-lingual form of muscular paralysis (1860), which
is known by his name, as also the pseudohypertophic form of muscular paralysis (1868).
Although the latter is simply one of the many forms of muscular dystrophy now recognized,
it was Duchenne's careful work in the hopital wards which first opened up the whole subject.
— Garrison.1
Notes :
Les cinq figures photographiques qui forment la planche IX, représentent le thorax et la face de sujets atteints d'atrophie musculaire progressive de l'enfance. — L'atrophie musculaire progressive, lorsqu'elle fut décrite pour la premiére fois, était considérée comme une maladie exclusive à l'àge adulte. — (p. 41).
Nous croyons ètre entrés dans des détails suffisants pour bien faire connaitre l'affection curieuse décrite sous le nom d'atrophie musculaire progressive de l'enfance, dont le diagnostic peut se lire pour ainsi dire sur la face du malade. — (p. 45).
We believe we have gone into sufficient detail for a good identification of this curious affection, described by name as progressive muscular atrophy of infancy and whose diagnosis can be read, so to speak, on the face of the patient.
This paper was supplemental to a series of articles 2 Duchenne published in 1868 on the juvenile form of hereditary muscular dystrophy known by his name alongside the name of Wilhelm Griesinger (1817-1868) who wrote an independent report in 1865 (GM 4738).3 In those articles Duchenne used the terms paralysie musculaire pseudohypertrophique and paralysie myosclerosique to classify the disease as arising within the neuromuscular anatomy and proving unrelated to childhood myopathies that originate from bulbar disorders. His first clinical observation was made in 1858 with the examination of a patient referred by his friend Sauveur Henri Victor Bouvier (1799-1877). This led to published observations of two cases in the 1861 second edition of his De l'Electrisation localisée,4 one of whom he photographed for an atlas 5 complementaire to the book. Duchenne was also the photographer of the four subjects depicted here which include a mother (Fig. 1) and her two sons (Fig. 2, Fig.s 3-4) who both exhibit the facial paresis and eversion of the lower lip that so often typifies an early stage pseudohypertrophic form of dystrophy. Confusion over the pathogenesis of myofacial paresis appears in numerous reports of the time, especially those that cite or attack Duchenne, but he found a champion in Charcot who incorporated this paper into a lecture titled, Révision nosographique des amyotrophies,6 in which he wryly noted that "Duchenne's disease" must be rare for it was hardly mentioned in the standard literature! Charcot also helped Duchenne prepare the histological evidence for his third edition of De l'Electrisation localisée published in 1872. Without a doubt, had there been a photographic atlas for that edition, these two boys and their mother would have been included.
Duchenne was not the first to study the progressive muscular dystrophies, but his copious writings defined its terms and greatly advanced the science. He also innovated the use of faradization techniques for this research and introduced the emporte-pièce, a clever miniature harpoon he used to pluck tissue samples from his living subjects.
1 Garrison, Fielding H. (1914), An Introduction to
the history of medicine, Philadelphia: W. B. Saunders Co.; p. 573.
2 Duchenne, G. B. (1868), Recherches sur la paralysie musculaire
pseudohypertrophique ou paralysie myo-sclérosique, Paris: P. Asselin;
"Archives Générales de Médécine," 6. s., xi. (5, 179, 305, 421, 552).
3 Griesinger, Wilhelm (1817-1868), Über Muskelhypertrophie,
Leipzig: Otto Wigand; "Archiv der Heilkunde," 6: 1-13.
4 Duchenne, Guillaume-Benjamin (1861), De l'Electrisation localisée et de son application
à la physiologie, à la pathologie et à la thérapeutique, Paris: J. B. Baillière et fils, 1861 (2nd edition).
5 Duchenne, Guillaume-Benjamin (1862), Album de photographies
pathologiques complementaire du livre intitulé De l'electrisation localisée, Paris:
J. B. Baillière et fils; Plates 1 & 2.
6 Charcot, Jean Martin (1872-1887), Leçons sur les Maladies du Système nerveux
faites à la Salpêtrière. Recueillies et publiées par Bourneville, Paris: A. Delahaye & E. Lecrosnier; page 192.
