Hull, England, United Kingdom
|Fig 1. Jules and Augusta Dejerine. Via Wikimedia. CC BY 4.0.|
In the last quarter of the nineteenth century, medicine in Paris flourished.1 Under the charismatic Charcot, it matched or excelled the contemporary advances in Germany and Britain. In the footsteps of Cruveilhier, Gratiolet, and Vicq d’Azyr came Charcot, Vulpian, Pierre Marie, Babinski, Gilles de la Tourette, and Sigmund Freud, who each made significant and lasting contributions. Vulpian and Charcot were interns at La Pitié in the late 1840s. They started together as chiefs at La Salpêtrière on January 1, 1862, making joint rounds, conducting research, and collaborating on publications. Although it was the histrionic genius Charcot who found celebrity, several years before him Vulpian was appointed full professor at the Académie Nationale de Médecine and the Académie des Sciences and became dean of the Paris Faculty of Medicine. Although their eminent protégés often quarreled, their own friendship was lifelong.
Joseph Jules Dejerine
Born in Geneva, Dejerine volunteered to work in a Geneva hospital during the Franco-Prussian War. He studied medicine in Paris and was Vulpian’s most distinguished student.2 He then became head of clinic at Hôpital Bicêtre (1879–1886), professor of neurology and chief consultant at Hôpital Bicêtre (1887–1894), and professor of neurology at La Salpêtrière (1895–1911). Finally, he was appointed professor of diseases of the nervous system at La Salpêtrière in 1911.3,4
Not only did he contribute to the developing field of clinico-pathological studies of the nervous system, but stimulated by Paul Dubois of Bern, whom he would visit on holidays, he acquired an interest in “functional disorders” and psychotherapy.5
Some Dejerine* syndromes
Dejerine instructed students: “It is rare you will be able to use subtle logic; it is your heart that carries you along … In man, emotion is almost everything and reason very little.”5
His name is associated with several signs and syndromes—a great source of material for those entranced by eponyms.5 One of his best known is Le syndrome thalamique (Dejerine-Roussy), characterized by severe scalding or stabbing pain with hyperesthesia and choreoathetosis in hemiplegic limbs.6 Other syndromes include facio-scapulo-humeral dystrophy (Landouzy-Dejerine) and hypertrophic interstitial polyneuritis (Dejerine-Sottas),7 the long title encapsulating the essential features—now named hereditary motor and sensory neuropathy type III. The syndrome of olivo-ponto-cerebellar atrophy is named after Dejerine and André Thomas. Dejerine also wrote on aphasia and described alexia without agraphia (pure alexia), the result of lesions of the supramarginal and angular gyri, and alexia with agraphia. Sometimes known as Dejerine’s sign it is the precipitation of spinal nerve root pain by sneezing and coughing.5 His book with André Thomas, Traité des maladies de la moëlle épinière, was published in 1902 and his Séméiologie des affections du système nerveux in 1914.
Dejerine married Augusta Klumpke (1859 – 1927), an American-born, highly accomplished neuroanatomist (Fig 1).8
The Dejerine marriage comprised two greatly talented researchers who collaborated and inspired each other, comparable only to the likes of the Curies and the Vogts.2,5 In 1885, Augusta observed that patients with a lower brachial plexus injury had weakness of the hand and forearm with miosis. She reported these in her thesis Des polynévrites en général etdes paralysies et atrophies saturnines en particulier. Etude clinique et anatomo-pathologique. 1885). She ascribed this to a lesion of the C8 and T1 nerve roots and contrasted it with C5-C6 root injuries of Erb’s palsy.
Thus, she provided another eponym, Klumpke’s palsy, for posterity.5,9 For this work, she won the Godard Prize of the medical academy in 1886. Jules owed much to Augusta, who had studied medicine in Paris and whose skills in science and determination enabled her—despite “fierce opposition”—to become the first woman to receive the title of “interne des hôpitaux.”5 Together, they published the seminal treatise Anatomie des Centres Nerveuxi (1895).
Pierre Marie vs. Jules Dejerine
An intriguing episode was when Pierre Marie and Jules Dejerine became rivals. Pierre Marie was a disciple of Charcot’s school at Salpêtrière Hospital;10 Dejerine, a devotee of Vulpian, had his own neurology school. A paper titled Du rôle joué par les lésions des racines postérieures dans la sclérose médullaire des ataxiques by Pierre Marie was the subject of Dejerine’s dispute; a demand was issued for a retraction on threat of a duel to the death.11 Only the intercession of their seconders appeased them and prevented the duel. A further dispute about aphasia was ventilated in Paris in 1908. They had several scientific confrontations, not least the fierce competition for Charcot’s chair at Salpêtrière after his death. Ironically, neither was appointed at that time; after an interim period under Brissaud, Fulgence Raymond was chosen as Charcot’s successor.
Dejerine was elected to the Academy of Medicine in 1908. Nine years later, on February 26, 1917, he died of renal failure. His wife Augusta continued his practice and research. The centenary of his birth was celebrated at the Fourth International Neurological Congress in Paris in 1949, when Dejerine’s pupil, André Thomas, gave a discourse on his life and work.5
*According to Mme le Dr Sorrel-Dejerine, Dejerine’s daughter, the name is not spelled Déjerine or Déjérine.5
- Clarac F, Barbara J. -G, Broussolle E, Poirier J. Figures and institutions of the neurological sciences in Paris from 1800 to 1950. Revue neurologique 2012;168: 2-14.
- Haymaker W and Schiller F, Eds. The Founders of Neurology. Eds. 2nd edn. Springfield. Charles C Thomas 1970. 426-30.
- Micklewright J.L., King T.Z. (2011) Dejerine, Joseph Jules (1849–1917). In: Kreutzer J.S., DeLuca J., Caplan B. (eds) Encyclopedia of Clinical Neuropsychology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-79948-3_614.
- Gauckler E. Le professeur J. Dejerine 1849-1917. Paris: Masson et Cie 1922.
- Pearce JMS. Dejerine-Sottas Disease (Progressive Hypertrophic Polyneuropathy). Eur Neurol 2006;55:115-117. doi: 10.1159/000092790.
- Pearce JMS. Dejerine and the thalamic syndrome. J Neurol Neurosurg Psychiatry 1988;51:676. See also Pearce JMS. Dejerine and the thalamic syndrome. In: Fragments of Neurological History. London Imperial College Press. 2003. 217-9.
- Dejerine JJ Sottas J. Sur la névrite interstitielle hypertrophique et progressive de l’enfance; affection souvent familiale et à debut infantile caractérisée par une atrophie musculaire des extrémities avec troubles marqués de la sensibilité et ataxie des mouvements et relevant d’une névrite interstitielle hypertrophique a marche ascendante avec lésions médullaires consecutives. Comp. Rend. Soc. Biol. Paris. 1893; 45:63-96.
- Bogousslavsky J. The Klumpke Family – Memories by Doctor Déjerine, Born Augusta Klumpke. Eur Neurol 2005;53:113-20.
- Dejerine-Klumpke A. Contribution à l’étude des paralysies radiculaires du plexus brachial. Paralysies radiculaires totales. Paralysies radiculaires inférieures. De la participation des filets sympathiques oculo-pupillaires dans ces paralysies. Revue de médecine 1885, 5: 591-616, 739-90.
- Pearce JMS. A note on Pierre Marie (1853-1940). Journal of Neurology Neurosurgery and Psychiatry 2004;75:1583.
- Teive H, Ferreira M, G, Camargo C, H, F, Munhoz R, P, Walusinski O: The Duels of Pierre Marie and Jules Dejerine. Eur Neurol 2020;83:345-350.
JMS PEARCE is a retired neurologist and author with a particular interest in the history of science and medicine.