Jules-Joseph Dejerine

Jules Déjerine (1849 – 1917) 200

Jules-Joseph Déjerine (1849 – 1917) was a Swiss-French neurologist.

Eminent neurologist of the French ‘classical neurology’ era along with Brissaud, Marie and Babinski. Dejérine researched the anatomy of the nervous system and studied aphasia, encephalitis, poliomyelitis, syringomyelia and neuritis.

Provided one of the first modern descriptions of agraphia in 1891 and defined the first two forms of alexia 1891/2. Author of two important neurology tomes ‘Anatomie des centres nerveux‘ (1895, 1901) with his wife; and ‘Séméiologie des affections du système nerveux‘ (1914)

Later in his career Dejerine became interested in psychology and psychotherapy, a proponent of the view that the personality of the psychotherapist was crucial in any interaction with the patient. ”In man, emotion is almost everything and reason very little”


Biography

  • Born 3 August 1849 in Plain-Palais, Geneva, Switzerland
  • 1868-1870 Académie de Genève developed a keen interest in biology as well as comparative anatomy
  • 1870 – Volunteered at a Geneva hospital during the Franco-Prussian War
  • 1871 – Baccalauréat et inscriptions à la Faculté de Médecine de Paris
  • 1879 – Doctorat de médecine. These: Recherches sur les lésions du système nerveux dans la paralysie ascending aiguë (with Le prix Godard de l’Académie de Médecine)
  • 1880 – Head of the clinic at the Hospice de la Charité
  • 1886 – Le Prix Montyon remis par l’Académie des sciences
  • 1887-1894 Head of the clinic at the Bicêtre
  • 1888 – Married American neurologist Augusta Klumpke (1859 – 1927) with Louis Landouzy (1845 – 1917) as witness…
  • 1895-1900 Chef du Clinique at La Salpêtrière; professor in general pathology and therapeutics
  • 1907 – Chair of medical pathology, la Salpètrière
  • 1908 – Elected to l’Académie de médecine
  • 1910 – Professor of the clinic of diseases of the nervous system, la Salpètrière until his death in 1917 (replaced by Pierre Marie (1853 – 1940))
  • 1913 – Chevalier de la Légion d’honneur
  • Died 26 February 1917 in Paris, France

Medical Eponyms

Landouzy-Dejerine syndrome (1884) [aka *dystrophie de Landouzy-Déjérine; facioscapulohumeral muscular dystrophy] Condition first recorded by Duchenne, in nine patients and published in 1872. Dejerine with Louis Landouzy (1845 – 1917) described an autosomal dominant hereditary from of progressive muscular dystrophy with atrophic changes in the muscles of the face and scapulohumeral group. Commences in childhood with weakness of the muscles of the face and shoulder girdle. The condition is slowly progressive and the musculature of other regions is eventually involved.


Dejerine-Sottas Disease (1893) Dejerine and Jules Sottas (1866–1943) presented autopsy studies of a brother and sister with progressive atrophy of the muscles of the extremities. They described a form of heredity peripheral neuropathy leading to amyotrophy secondary to a mutation of one of the many genes responsible for the formation of myelin. Now known as ‘hereditary motor and sensory neuropathy type III’; ‘Type 3 Charcot-Marie-Tooth disease‘; or hypertrophic interstitial neuritis of infancy.

The title of the paper says it all really: “On hypertrophic and progressive interstitial neuritis in childhood; often familial and early childhood disease characterized by muscular atrophy of the extremities with marked disturbances of sensitivity and ataxia of movements and arising from hypertrophic interstitial neuritis with ascending course with consequent spinal cord lesions”

Dejerine-Sottas disease 1893 67-68
Dejerine-Sottas disease 1893 67-68
Original
English

Atrophie musculaire excessive des jambes et des cuisses, diminuant un peu et remontant vers la racine des membres. Les deux pieds présentent les deformations suivantes (fig 1) Aux membres supérieurs, l’atrophie musculaire est très marquée dans les muscles des mains, un peu moins dans ceux des avant-bras. Mains simiennes. Atrophie des thénars et des interosseux sans griffe cubitale (fig 2)

Dejerine, Sottas 1893: 67-68

Excessive muscular atrophy of the legs and thighs, decreasing a little and rising towards the roots of the limbs. Both feet present the following deformations (fig 1) In the upper limbs, muscular atrophy is very marked in the muscles of the hands, a little less in those of the forearms. Simian hands. Atrophy of the thenars and interossei without ulnar claw (fig 2)

Dejerine, Sottas 1893: 67-68

Dejerine-Thomas syndrome (1900) [*syndrome de Déjérine-Thomas; olivopontocerebellar atrophy or cerebellar ataxia]


Dejerine sign (1902) The precipitation of acute nerve root pain, and aggravation of radiculitis symptoms due to coughing, sneezing and defecation efforts. The precipitation of radicular pain by sudden changes in intraspinal pressure in the context of cervical radiculopathy


Dejerine-Roussy syndrome (1906) [*syndrome de Déjérine-Roussy, thalamic syndrome, capsulothalamic syndrome] Dejerine, with Gustave Roussy (1874-1948) described thalamic pain following lesions of the ventroposterior thalamic nuclei or the deep white matter of the parietal lobe following contralateral hemiparesis, hemiataxia and choreoathetosis. Characterized by severe scalding, shooting or stabbing pain on the opposite side of the body.

Dejerine and Roussy provided the earliest formal accounts of “le syndrome thalamique” as a syndrome clinically characterised:

  1. A slight hemiplegia, usually without contracture and rapidly regressive.
  2. A persistent superficial hemianaesthesia ofan organic character, which can in some cases be replaced by cutaneous hyperaesthesia but is always accompanied by marked and persistent disturbances of deep sensation.
  3. Mild hemiataxia and more or less complete astereognosis.
  4. Severe, persistent, paroxysmal and often intolerable pains on the hemiplegic side, not yielding to any analgesic treatment.
  5. Choreoathetoid movements in the ‘limbs on the paralyzed side’
  6. In 1923

In 1923 Charles Foix (1882-1927) and Masson demonstrated that the most common cause was “Le syndrome de l’artère cérébrale postérieure


Neurological neologisms

agraphia (1891) Provided one of the first modern descriptions of agraphia: Sur un cas de cécité verbal avec agraphie, suivie d’autopsie

alexia [Gr, lack of + lexicon] (1891) Inability to comprehend the meaning of written or printed language symbols. Dejerine described the two major types (though a thrid and fourth have since been defined). Dejerine determined that the left angular gyrus was the centre for the interpretation of the visual images which constitute written language.

  1. alexia with agraphia; described in 1891 as difficulty reading and naming, especially of letters but also of words (literal and verbal alexia); severe impairment of spontaneous writing and writing to dictation; and an inability to comprehend spelled words and to spell aloud.
  2. alexia without agraphia: described in 1892 as an inability to read letters, words, or musical notation, in association with a right hemianopia and difficulties with color naming.


Controversies

Name: Déjèrine; Déjerine; Déjérine; Dejerine and Jules-Joseph; Joseph-Jules; Jules [According to Dejerine’s daughter, Mme. le Dr. Sorrel-Dejerine, the name is written Dejerine, without acute accents, not Déjerine or Déjérine.


Major Publications


References

Biography

Eponyms


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eponym

the person behind the name

Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM with a passion for rugby; medical history; medical education; and informatics. Asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | vocortex |

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