Bassetti, Claudio

Jagella, E.

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J Neurol (2006) 253 : 823–824DOI 10.1007/s00415-006-0905-5 PIONEERS IN NEUROLOGYClaudio L. BassettiE. Caroline JagellaJoseph Jules Dejerine (1849–1917)JON 1905Dejerine was the third occupant ofthe world’s first chair of neurologyat ‘La Salpêtrière’, after Jean MartinCharcot and Fulgence Raymond. Heis well known as a strenuous ‘local-izationist’ of higher brain functionsbut should be remembered mainlyfor his innumerable contributionsto clinical neurology as well as for his monumental anatomical andanatomo-pathological studies.Dejerine was born in Switzer-land near Geneva as a son of a Savo-yard carriage-proprietor of modestincome. In 1871 he arrived in Paristo study medicine. In 1875 he wasappointed ‘Médecin des hôpitaux’ atthe ‘Hôpital de la Pitié’, where hepublished his first papers on pe-ripheral neuropathies, with EdméFélix Alfred Vulpian (1826–1887). In1879, after having completed a doc-toral thesis on acute ascendingparalysis, he became ‘Chef de clin-ique’ at the ‘Hôpital Bicètre’, wherein 1886 he was nominated ‘pro-fesseur agrégé’. In 1887 he joinedthe Salpêtrière. In the followingyears he became professor of his-tory of medicine and surgery(1900), internal medicine (1907),and finally neurological diseases(1910). Dejerine died of the conse-quences of Bright’s disease in 1917,while ‘La Salpêtrière’ had beentransformed into an army hospitalduring World War I.Dejerine was in 1899 among the17 founding members of the FrenchNeurological Society, of whom 13were pupils of Charcot. He receivedmany honours including the title of‘Chevalier’ and later ‘Officier’ of theLegion of Honor; in 1914 he re-ceived the Moxon gold medal of theRoyal College of Physicians ofLondon, after Hughlings Jackson,Richard Gowers and David Ferrier.Dejerine was an imposing figurewho was remembered by his pupilsas affectionate and loyal. In 1888 hemarried Auguste Marie Klumpke(1859–1927), an American-bornphysician, with whom he shared hispassion and work for anatomicaland pathological studies. The cou-ple had one daughter, who also be-came a physician. The Dejerine’swere described as very hard work-ers, they had a rather simple style ofliving and were fond of nature andsports. Robert Bing was amongthose who wrote an obituary [1]while Gauckler published the onlybiography to date [2].As a pupil and soon also co-worker of both Charcot andVulpian, Dejerine combined theanatomo-clinical approach of theformer with the experimental-clini-cal method of the latter. The influ-ence and the relationship withVulpian were the strongest andmost enduring in his career.Dejerine’s work focused onanatomical and anatomo-patholog-ical studies, which were conductedmainly with his wife and which ledReceived: 21 February 2005Accepted: 16 March 2005Prof. C. L. Bassetti () · E. C. JagellaNeurology DepartmentUniversity HospitalZürich-SwitzerlandE-Mail: claudio.bassetti@usz.chJoseph Jules Dejerine (1849–1917)823_824_Bassetti_JON_1905  22.06.2006  8:12 Uhr  Seite 823824to the publication of the ‘Anatomiedes centres nerveux’(1895–1901 [3],and the ‘Sémiologie des affectionsdu système nerveux’ (1914 [4]). Es-sential for these studies were thediscoveries of Golgi’s staining, Gud-den’s microtome, Marchi’s serialsections,and the use of the principleof secondary degeneration for thelocalisation of fibre tracts. Thesetechniques enabled Dejerine to de-scribe radicular myotomes and der-matomes, the somatotopy and con-nections of the pyramidal tracts, theaberrant corticobulbar bundles (ex-plaining for example central facialpalsy in medullary lesions), the as-cending sensory tracts, and the con-nections between the thalamus andthe cerebral hemispheres.Parallel to his laboratory work,Dejerine made important clinico-pathological correlations: scapulo-humeral atrophy (with Landouzy1885 [5]), hypertrophic progressiveinterstitial neuritis (with Sottas1893 [6]), olivo-ponto-cerebellaratrophy (with André-Thomas 1902and 1912 [7]), peripheral and cen-tral ataxia (with Egger 1903), thethalamic syndrome (with Roussy1906 [8]), sensory parietal syn-dromes (pseudoradicular withChiray 1904; cortical with Crouzon1914; pseudothalamic with Mouzon1915), and medial medullary syn-drome (1914). Dejerine also de-scribed intermittent vascular clau-dication of the spinal cord (1906),the phenomenon that aphasics canby a show of fingers identify thenumber of syllables in a word (De-jerine-Lichtheim phenomenon), thefirst callosal disconnection syn-drome (alexia without agraphia,1892), and tactile agnosia.Some of these studies wererevolutionary. The description ofscapulo-humeral muscular atrophyintroduced a new concept of muscu-lar atrophy in the absence of neu-ropathy. In his work on the thala-mus, Dejerine contradicted Charcotby demonstrating the existence ofsensory deficits from a brain lesionwithout peripheral sensory lesionsor motor deficits.In the second part of his life,Dejerine devoted an increasingamount of energy to the study ofhigher brain functions. He was cer-tainly influenced by Charcot’s vi-sion on aphasia and amnesia.Dejerine was convinced thereshould be distinct types of aphasicdisturbances according to well de-fined structural lesions, partly com-bined with other deficits of higherbrain functions [9, 10]. In 1908Pierre Marie challenged Broca’s fa-mous brain-studies and at the sametime Dejerine’s position as leadingaphasiologist of his time by suggest-ing the existence of only a singletype of aphasia (Wernicke type)while dismissing motor aphasia asmere anarthric phenomenon. Thisdebate lacked a clear winner but wasfundamental because it questionedthe relevance of anatomical findingsin understanding higher brain func-tions.In the last decade of his life De-jerine developed an interest in psy-chiatry and psychotherapy, partlystimulated by his friendship withhis later biographer Gauckler andwith Paul Dubois (1848–1918), oneof the founders of the Swiss Neuro-logical Society. He claimed that thesuccess of psychiatric treatment de-pends mainly on the personality ofthe therapist, thus stressing theemotional rather than the rationalelement. This empirical positionreflected his own empathic behav-iour towards both his patients andpupils.Dejerine’s investigations fit per-fectly in the context of the neurolog-ical research of his time and reflectthe main problems and issues of this‘age d’or’ of Neurology. Dejerine ac-cepted scientific arguments onlywhen based on morphologic evi-dence. Nevertheless, as exemplifiedby his contributions on the thalamicsyndrome, he was also able to ex-tend the localization principle to amore general (and functional) vi-sion of the nervous system. In this,Dejerine proved himself capable oflinking the main themes of neuro-logical research in the 19th and 20thcenturies.References1. Bing R (1918) Jules Dejerine(1849–1917). Schweiz Arch Neurol Psy-chiatr 2:314–3152. Gauckler E (1922) Le Professeur Dejer-ine, 1849–1917. Paris3. Dejerine J, Dejerine-Klumpke A(1895–1901) Anatomie des centresnerveux. Paris: Rueff and Cie4. Dejerine J (1914) Sémiologie des affec-tions du système nerveux. Paris: Mas-son5. Landouzy L, Dejerine J (1885) De lamyopathie atrophique progressive.Myopathie sans neuropathie, débutantd’ordinaire dans l’enfance, par la face.Rev Méd (Paris) 5:81–117, 253–3666. Dejerine J, Sottas J (1893) Sur lanévrite interstitielle hypertrophique etprogressive de l’enfance. Comptes Ren-dus de la Société de Biologie 45:63–967. Dejerine J, Thomas A (1902) Traité desmaladies de la moelle épinière. Paris:Baillière, J. B.8. Dejerine J, Roussy G (1906) Le syn-drome thalamique. Rev Neurol (Paris)12:521–5329. Dejerine J (1906) L’aphasie sensorielle:sa localisation et sa physiologiepathologique. Presse Médicale 55:437–43910. Dejerine J (1906) L’aphasie motrice:sa localisation et sa physiologiepathologique. Presse Médicale 57:453–457823_824_Bassetti_JON_1905  22.06.2006  8:12 Uhr  Seite 824

Joseph Jules Dejerine (1849-1917)

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Joseph Jules Dejerine (1849-1917)

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