doi:10.1093/brain/awx225 BRAIN 2017: 140; 2752–2759 | 2752 DORSAL COLUMN Grey Matter Downloaded from https://academic.oup.com/brain/article-abstract/140/10/2752/4159454 by Lancaster University user on 30 January 2020 Reconnecting with Joseph and Augusta Dejerine: 100 years on Claude J. Bajada, Briony Banks, Matthew A. Lambon Ralph and Lauren L. Cloutman Neuroscience and Aphasia Research Unit (NARU), Division of Neuroscience and Experimental Psychology, School of Biological Sciences, The University of Manchester, UK Correspondence to: Dr Lauren Cloutman Neuroscience and Aphasia Research Unit (NARU) Division of Neuroscience and Experimental Psychology School of Biological Sciences (Zochonis Building) University of Manchester Brunswick Street Manchester, M13 9PL, UK E-mail: [email protected] Correspondence may also be addressed to: Prof. Matthew Lambon Ralph E-mail: [email protected] Introduction The Dejerines: a short history Joseph Dejerine passed away on 28 February 1917 in the Born in Geneva, Joseph Jules Dejerine moved to Paris in midst of a world at war. One hundred years later we 1871 in order to start his medical career. He was a founding celebrate the legacy of this pioneer in neuroscience. In member of the French Neurological Society and proceeded to 1895, Joseph Jules Dejerine published the first volume of become the chair of neurology at ‘La Salpeˆtrie`re’, a position the seminal work, Anatomie des centres nerveux; volume previously held by Jean Martin Charcot. In his 67 years, 2 was published in 1901. In a major section of this tome Dejerine made great contributions to the medical and scien- (vol. 1 pp. 749–80), Joseph Dejerine and his wife and tific community, of which Anatomie des centres nerveux was long-term collaborator, Augusta Dejerine-Klumpke, pro- but one. He became one of the most eminent neurologists of duced a treatise on the white matter pathways of the his era. He was a pioneer in the study of neurological con- brain, composed of anatomical descriptions of meticulous ditions and the structural and functional anatomy the brain. detail and beautiful illustration (drawn by H. Gillet) that His resulting legacy includes many syndromes eponymously reflected a combination of the most advanced methodolo- named after him; arguably the most famous of which is gies of the day and a review of leading neuroscientific the medial medullary syndrome (often referred to as research. We have selected and focused this specific ‘Dejerine syndrome’). Dejerine passed away in 1917 after output (which is provided for the first time as an suffering from Bright’s disease (nephritis) (Schurch and English translation in the Supplementary material) from Dollfus, 1998). the many that the Dejerines published because its ideas As with many great deeds, such as those told in the tales of and findings continue to be of relevance to modern neuro- Greek mythology, Joseph Dejerine’s success could not have science researchers today; especially those with an interest been accomplished without the aid of a great woman. Alas, in connectional anatomy. like Ariadne’s role in helping Theseus to slay the Minotaur, Received May 26, 2017. Revised July 20, 2017. Accepted July 26, 2017 ß The Author (2017). Published by Oxford University Press on behalf of the Guarantors of Brain. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properly cited. Reconnecting with the Dejerines BRAIN 2017: 140; 2752–2759 | 2753 the role of Dejerine’s wife and long-term collaborator, connectional approach posited that (i) regions associated Augusta Dejerine-Klumpke, has also often been over- with specific cognitive functions dynamically interacted with shadowed by that of her husband. Augusta Dejerine- one another to produce a more complex function; and (ii) Klumpke was a great neurologist in her own right who, in resultantly, higher cognitive impairment resulted not only her husband’s words, ‘collaborated assiduously’ with him in from damage to cortical regions but also to the connections the preparation of their masterpiece Anatomie des centres between them. This concept was expanded upon by Dejerine nerveux (Fig. 1). Augusta Marie Dejerine-Klumpke was in his view on pure alexia, which he conceptualized as a born in San Francisco in 1859. She moved to Switzerland disconnection syndrome (Dejerine, 1892). It is worth Downloaded from https://academic.oup.com/brain/article-abstract/140/10/2752/4159454 by Lancaster University user on 30 January 2020 when she was 11 and eventually to Paris. Here she pursued noting, however, that Wernicke disagreed with the her medical studies and became the first female ‘Interne des Dejerines’ view since it required the higher function of read- Hopitaux’ in France. Like her husband, Dejerine-Klumpke ing to be cortically localized to a ‘visual verbal centre’ within made a lasting impression within the field of medicine and the angular gyrus (Catani and ffytche, 2005). was bestowed several honours for her work. Indeed, every With the relatively recent emergence of diffusion MRI and medical student will be familiar with her for a syndrome related tractography techniques, modern neuroscience has that arises from damage to the inferior roots of the brachial been reconnecting with the work of the 19th century masters. plexus; the famous Klumpke’s palsy. She passed away A search of current literature will reveal a rapidly increasing 10 years after her husband, in 1927 (Schurch and Dollfus, interest in the association of specific cognitive functions to the 1998). white matter tracts of the brain. Despite the plethora of The Dejerines lived in a golden age for neurology, functional association studies, there remain many controver- studying and working alongside, but not always in sies surrounding the origin, course, termination and even harmony with, some of the great masters of the time sometimes the very existence of these tracts (Bajada et al., including Charcot, Pierre Marie and Vulpian. They pub- 2015). Understanding the structure of the white matter that lished many works in their career but none as grand as forms the brain’s circuitry remains an important goal for their master work, the two-volume Anatomie des centres researchers who are interested in elucidating the brain’s nerveux. function, as well as clinicians who would like to predict a patient’s outcome when a lesion occurs in one of these fasciculi or to avoid long-term deficits after neurosurgery A golden age for white (Duffau, 2015). With this in mind, and at such a poignant time as the matter neuroanatomy 100th anniversary of the passing of Joseph Dejerine, we highlight the legacy of two pioneers in the subject The 19th century was a time of prolific work in connectional and revive their descriptions of the long association neuroanatomy. It was the era during which most of the white fasciculi of the cerebral cortex. These descriptions were matter fasciculi known today were first dissected and based on an in-depth study of the white matter of the described. It was also the time during which Paul Broca sug- human brain, based on a convergence of methodologies gested that higher cognitive functions could be localized available at the time including gross dissection, histolo- within the cortex, and Carl Wernicke proposed the first gical preparations, animal experimentation and a thor- network approach to functional neuroanatomy. This ough contemporary review of known findings. A full translation (from its original French) of the section regard- ing the Long Association Fasciculi from Anatomie des centres nerveux is made available in the online Supplementary material that accompanies this article. For the remainder of this article we focus upon some of the key themes and findings, highlighting both striking parallels between the original work and modern descrip- tions, as well as 19th century notions that may have important implications for contemporary basic and clinical neuroscience. The long association fasciculi: past and present The Dejerines described five long association fasciculi, all of which are still discussed in the modern literature, namely Figure 1 Joseph Jules Dejerine and his wife Augusta the cingulum, uncinate fasciculus, superior longitudinal/ar- Dejerine-Klumpke. ßWellcome Library London. cuate fasciculus, occipito-frontal fasciculus, and the inferior 2754 | BRAIN 2017: 140; 2752–2759 C. J. Bajada et al. longitudinal fasciculus. While we leave the reader to exam- addition to the major tract body, is an important step ine the full translation for an in-depth description of the in understanding the functional associations of each tracts, we explore three key discussion points. tract. Furthermore, from a clinical neuroscience perspective, this second conceptualization would imply that the exact sequelae of tract damage may vary according to its position (i) The first section explores the concept of a multi-component along each tract. tract; the conglomeration of fibres that underlie different Examples of conceiving the tract as a shared neural functional networks. wiring conduit occur frequently in the Dejerines’ descrip- Downloaded from https://academic.oup.com/brain/article-abstract/140/10/2752/4159454 by Lancaster University user on 30 January 2020 (ii) The second section focuses on regions of white matter tracts tions. For example, they describe the cingulum as an arched outlined by the Dejerines but subsequently lost to the litera- fasciculus on the medial aspect of the brain, yet note that it ture, either in the 19th or 20th century, and some current is composed of smaller bundles of fibres: conceptualizations of these. (iii) The third section highlights the Dejerines’ contributions to ‘Dissections show that the cingulum is not formed of fibres that long-standing debates, some of which are still unresolved in extend the full length of the fasciculus, but of relatively short the contemporary literature.