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Dejerines': an Historical Review and Homage to Two Pioneers in the Field

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Dejerines': an Historical Review and Homage to Two Pioneers in the Field Spinal Cord (1998) 36, 78 ± 86 1998 International Medical Society of Paraplegia All rights reserved 1362 ± 4393/98 $12.00 The `Dejerines': an historical review and homage to two pioneers in the ®eld of neurology and their contribution to the understanding of spinal cord pathology B Schurch1 and P Dollfus2 1Swiss Paraplegic Centre, University Hospital Balgrist, Zurich, Forchstrasse 340, 8008 Zurich, Switzerland; 272 rue des CarrieÁres, 68100 Mulhouse, France Our purpose, in this number of Spinal Cord devoted to the French speaking Society of Paraplegia (AFIGAP), is to render homage to two very distinguished doctors, who by their work at the end of the XIXth and the beginning of our century contributed greatly to our knowledge of the nervous system and in particular the spinal cord (SC). This was at the time a ®eld of considerable interest in France and abroad. Professor Jules Dejerine was from 1911 ± 1917 the holder of the Chair for Nervous System Diseases created for Charcot. Dejerine and his American born wife, Augusta Klumpke, and had very limited means for investigation compared to actual technological advances. They relied mainly on their superb clinical observations and neuropathological examinations. Dejerine was also a pioneer in the growing ®eld of neuroanatomy. In 1895 he published a treatise on the anatomy of the nervous system, which is still considered worlwide to be a masterpiece. Augusta Dejerine-Klumpke, the ®rst woman Intern in Paris Hospitals, was not only a ®ne clinician, neuroanatomist and pathologist, but also contributed greatly to her husband's work. Amongst other things she is known for the `Klumpke palsy'. She was also a pioneer in France, during the First World War and subsequent following years, in the treatment and rehabilitation (medical and vocational) of the large number of soldiers aicted by wounds of the nervous system and especially of the SC. During the same period, many authors contributed to SC pathology, but only a few to the treatment and rehabilitation of these patients. This was brought to our attention, in the sixties, by Professor Pierre Houssa, pioneer in Belgium in the ®eld of comprehensive care of those who have SC lesions. Augusta Dejerine-Klumpke also contributed to our present knowledge of heterotopic ossi®cation following a SC injury, including its pathogenesis. Most of their clinical and pathological ®ndings and discussions are recorded in Dejerine's famous monograph which was published in 1914: La seÂmeÂiologie des Aections du SysteÁme Nerveux (The Semiology of the Diseases of the Nervous System). Keywords: Dejerine; Dejerine-Klumpke; spinal cord; semiology; neuroanatomy; historical review Introduction The history of Medicine oers amongst other conditions than on medical causes for such lesions. privileges, that of being able to appreciate and look Joseph Jules Dejerine (1849 ± 1917) (he was usually into the life and the work of people who have made known as Jules), was a person who marked his epoch signi®cant contributions concerning the Science and the not only as neurologist, neuroanatomist and patholo- Art of Medicine. The history of spinal cord pathology, gist but also as a straightforward very hard working traumatic and non traumatic, has been available at and loyal person. His reputation and that of his least in the French language since the time of Ollivier American born wife Augusta Dejerine-Klumpke d'Anger's book `Le traite de la moelle e pinieÁ re et de ses (Figure 1) were acknowledged and respected not only maladies' published in 1827.1 More recently, the reader in France but also wordwide. The quality of their can refer in the English language to the writings of Sir achievements in all the domain of the rising speciality Ludwig Guttmann2 and of Ohry and Ohry-Kossoy,3 of neurology was recognised in several European who focussed perhaps more on traumatic spinal cord countries, mainly in Germany and Great Britain, but also in North and South America where French culture Requests for reprints: Mr G Maillard, President, IRME (Institute was particularly appreciated. Towards the latter part of pour la Recherche sur la Moelle EpinieÁ re) 45 rue Vineuse, 75116 the XIXth century, thanks to Forel, His, Golgi, and Paris, France Ramon y Cajal, the theory of the neurone as an The Dejerines: Physiology and pathology of the nervous systems B Schurch and P Dollfus 79 Table 1 Dejerine hand re¯ex or phenomenon: clonic contracontractions of the hand (wrist) on tapping the dorsum of the hand or the volar side of the forearm near the wrist; it occurs in normal persons but is exaggerated in pyramidal tract lesions. Dejerine-Lichtheim sign: in subcortical aphasia. Dejerine cortical sensory syndrome: impairment in the ability to make ®ne sensory distinctions. Dejerine-Roussy syndrome: or thalamic apoplexy, sometimes referred to as thalamic hyperpathia. Dejerine-Sottas syndrome: hypertrophic interstitial neuritis in childhood, which includes the consecutive sclerosis of the Figure 1 Portrait of the Professor J Dejerine and his wife A posterior columns. Dejerine-Klumpke. DFC PhototeÁque; Copyright AP-HP; Paris. Klumpke-Dejerine syndrome: lower plexus lesion including a Claude Bernard-Horner syndrome when the sympathetic ramo of T1 are involved. independent entity was de®nitely proven. The turn of the XIXth and the beginning of the XXth century was trophic lateral sclerosis), combined and primitive considered as `. the time when `dynamic' and sclerosis, transverse myelitis, poliomyelitis, haemato- `topographic' viewpoints in neurology were in con¯ict, myelia (post-traumatic and non traumatic), syringo- with the real issues becoming more and more myelia, spinal cord ataxia, hereditary progressive clouded'.4 Dejerine was certainly one of the pioneers hypertrophic interstitial neuritis in childhood, as well in making things clear.5±9 This period was marked by as other clinical and organic aspects of disorders of the considerable progresses in medical sciences, especially spinal cord and its nerve roots (Table 1).12 He also within the ®elds of neuroanatomy, neuropathology, described in detail the clinical signs of intermittent clinical investigations and neurophysiology, applied claudication due to vascular pathology of the spinal and experimental. In France, people such as Duchenne cord,12 and gave a precise description of the de Boulogne, Brown-Sequard, Claude Bernard, Vul- somatotopy and the connections of the ventral pian, Charcot, Pierre Marie, and others, in their time, corticospinal tract as well as of the lateral and were certainly among the many outstanding ®gure- anterior spinothalamic tracts (the `faisceau en crois- heads of the French School of Neurology.10,11 The sant' de Dejerine: the `crescent like' fasciculus) referred Dejerines, have not only been unanimously placed at to as the `anterolateral system', (ALS) (Figure 2).12,13 the same level but their pioneering work in the ®elds of Through his anatomical and pathological studies of correlative neuroanatomy has reinforced, even today, the upper cervical cord and medulla oblongata, he our clinical knowledge especially of the semiology of contributed to the knowledge of the somatotopy of the the nervous system.12 The latter term has unfortunately trigeminal cranial nerve, its descending tracts and nowadays become rather obsolete. nucleus, with its particular `onion skin pattern' sensory Jules Dejerine's achievements are not only known in topography. This is particularly interesting in the the ®elds of neurology, but also in the new science of clinical features of a patient with ascending cervical psychiatry developed by Charcot where `he was a man syringomyelia or syringobulbia.12,14 With Andre who could listen to the suered, understand and was Thomas (1867 ± 1963), as one of his closest collabora- patient.6 Nevertheless, according to his contempor- tors,9 he contributed among other studies to the aries, patience was not always his usual quality. His anatomical study of the pyramidal tract, which was books, and publications, had an international renown also one of Babinski's clinical interests.15,16 Together inallofthe®eldsofneuroanatomy5and neuropathol- with Andre Thomas, he also published in 1902 a work ogy. They were beautifully illustrated and the of reference: `Le Traite des Maladies de la Moelle correlation between anatomy and pathology with an EpineÁre' (A Treatise on Spinal Cord Diseases).17 Other amazing precision, provided an explanation of the books and articles were also published in the English functional and organic courses of certain diseases of language on the same subject, which were cited by the central nervous system, such as aphasia, aster- these authors, but appear to have been forgotten eognosis, apraxia, secondary encephalic degeneration, nowadays.18,19 In the German language, it is also as well as disturbances of the peripheral nervous important to mention Kocher's works.20 Andre system. Regarding the spinal cord, he contributed Thomas was also well known, even as a young doctor greatly to the study of: tabes dorsalis, Friedreich's at the time, by his studies on the autonomic nervous disease, multiple sclerosis, Charcot's disease (amyo- system, especially in relation to spinal cord lesions and The Dejerines: Physiology and pathology of the nervous systems B Schurch and P Dollfus 80 many of their neurovegetative aspects. This aspect was de la Pitie '. Vulpian was not only a great physician but also of great interest for Dejerine.12 Two publications also a renowned anatomist and physiologist.6,10,11,24
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