Portal de Periódicos da CAPES

Professor Jean Aicardi

2015; Elsevier BV; Volume: 19; Issue: 6 Linguagem: Inglês

10.1016/j.ejpn.2015.08.003

1532-2130

John Stephenson,

Fetal and Pediatric Neurological Disorders

Internationally renowned French Paediatric Neurologist who wrote his textbooks in English. Jean Aicardi was arguably the greatest paediatric neurologist of the modern era. His name will live on in the two separate conditions that he described, Aicardi syndrome and Aicardi-Goutières syndrome, through his several textbooks, all written in English, and through his friends, colleagues and pupils throughout the world. Most of his work was done in Paris but in later years he also held posts in Miami, USA (as Visiting Scientist), and at the Institute of Child Health (as Honorary Professor of Child Neurology) and in Great Ormond Street Hospital (Honorary Consultant Neurologist), London, UK. Jean François Marie Aicardi was born on 8th November 1926 in Rambouillet 50 km from Paris, the seventh of nine children. He writes1Aicardi J. Jean Aicardi: my circuitous path to becoming a French child neurologist and epileptologist.J Child Neurol. 2012; 28: 409-415Crossref Scopus (1) Google Scholar that at school at the Lycée Hoche in Versailles he was “an average student with no burning interest in any particular subject, notably not maths”. However, a vague attraction to biology led him to think of medicine as a career. Despite many difficulties he seems to have become more and more excited by his medical education and finally earned the MD from the University of Paris for a thesis on convulsive disorders in the first year of life. He was inspired to do neurology by Professor Raymond Garcin and to do paediatric neurology by Professor Stéphane Thieffry. After his MD he spent an inspirational year as research fellow at Harvard Medical School and the Children's Hospital Medical Center in Boston. Not only did he get to know people like Cesare Lombroso but most importantly he assimilated North American research methods and their rigour. Thereafter he re-joined Thieffry in what was to be the first paediatric neurology unit in France, and a unit of great productivity over many years, whether at Hôpital des Enfants Malades or Hôpital Saint-Vincent DePaul in Paris. However, it was only when Aicardi joined INSERM (Institut National de la Santé et de la Recgerche Médicale - National Institute of Health and Medical Research) that his career was assured and he was able to write and to collaborate internationally. At INSERM he was Mâitre de Recherche 1969–1991 and Directeur de Recherche 1986–1991. Aicardi's best known papers described his two new syndromes. Aicardi syndrome2Aicardi J. Lefebvre J. Lerique-Koechlin A. A new syndrome: spasms in flexion, callosal agenesis, ocular abnormalities.Electroenceph Clin Neurophysiol. 1965; 19 (Abstract): 609-610Google Scholar, 3Aicardi J. Chevrie J.J. Rousselie F. Spasma-in-flexion syndrome, callosal agenesis, chorioretinal abnormalities.Arch Fr Pediatr. 1969; 26: 1103-1120PubMed Google Scholar consists of infantile spasms, eye defects – mainly chorioretinal lacunae, white “holes” – and agenesis of the corpus callosum and is almost entirely confined to girls (except in Kleinfelters). Aicardi-Goutières syndrome (AGS) – described with Françoise Goutières,4AIcardi J. Goutières F. A progressive familial encephalopathy in infancy with calcifications of the basal ganglia and chronic cerebrospinal fluid lymphocytosis.Ann Neurol. 1984; 15: 49-54Crossref PubMed Scopus (371) Google Scholar with whom he wrote over 60 papers between 1966 and 2007 – is best known as an infantile encephalopathy, but has proved to be of extraordinary interest on a wider scale. The seven genes5Crow Y.J. et al.Characterization of human disease phenotypes associated with mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR, and IFIH1.Am J Med Genet A. 2015; 167A: 296-312Crossref PubMed Scopus (359) Google Scholar (so far) for AGS are involved in pathways that converge on type 1 interferon, a protein involved in innate immunity. Thus it comes about that AGS resembles both congenital viral infections (including HIV) and also auto-immune disorders in particular systemic lupus erythematosus. Another condition to which Aicardi made a major contribution was Rett syndrome6Hagberg B. Aicardi J. Dias K. Ramos O. A progressive syndrome of autism, dementia, ataxia, and loss of purposeful hand use in girls: Rett's syndrome: report of 35 cases.Ann Neurol. 1983; 14: 471-479Crossref PubMed Scopus (1166) Google Scholar also a female-only condition. Such discoveries depended on systematic good record keeping so that undiagnosed patients could be retrieved from lists of clinic attendees. Before anything was published I remember Robert Ouvrier coming up to Jean during an Oxford meeting and saying that he had found a new syndrome: “Is it only in girls?” asked Jean! Aicardi's textbooks have been hugely popular and both Diseases of the nervous system in childhood and Epilepsy in children have run to three editions, so far. He was also the founding Editor and Editor-in-Chief of the journal Epileptic Disorders that includes videos of patients to accompany the written word. His academic honours were numerous. They included UK distinctions such as fellowship of the Royal College of Physicians, honorary fellowship of the Royal College of Paediatrics and Child Health, honorary membership of the British Paediatric Neurology Association (BPNA) and Mac Keith Guest Lecturer to the BPNA in 1982. He received the Cornelia de Lange Medalion from the Dutch Child Neurology Society, the Peter Emil Becker Award of the German Child Neurology Society and the Ramon y Cajal Award of the Iberoamerican Academy of Child Neurology. Worth more than a mention is Aicardi's receipt of the Hower Award of the Child Neurology Society in 1986 – of this he wrote “I would first like to thank the Child Neurology Society for the honor that it has bestowed on me. I am aware of the prestige which is attached to the Hower Award and I deeply value this distinction, especially as I realize that it is the first time the Award has been given to a non-American child neurologist. In addition to honouring me, the Child Neurology Society wanted to demonstrate that neuro-pediatrics has no geographic, political or linguistic borders and that the care of neurologically disabled children requires the efforts of all men and women of good will across lands and oceans.”7Aicardi J. The future of child neurology.J Child Neurol. 1987; 2: 152-159Crossref PubMed Scopus (5) Google Scholar Aicardi was a man without hubris. As an eminent child neurologist from the USA wrote recently, “I was so impressed by how someone of his stature and accomplishment seemed so modest. I have come to believe, largely from this encounter, that truly accomplished people have no real need to tout themselves: a valuable life lesson for all of us.” On a personal note, I was glad to be a friend enough to stay with him in Paris both immediately after the 1973 Kungälv European Study Group on Child Neurology (that I think was a forerunner of the EPNS) – en route to Glasgow (!) and again very recently. Also he and his wife Jeanne stayed with us for several nights in Lenzie (near Glasgow) close to the Kungälv time. He would have come to Islay for the 2005 paroxysmal disorders in infancy meeting but after his tongue operation (doubtless a consequence of heavy smoking of small cigars in earlier years) he felt he could not speak well enough in any language other than French. As it was, he was mentioned in my introduction to that Islay meeting: “As I remember from sitting beside Jean Aicardi in coach journeys to and from Stratford-upon-Avon at the old Mac Keith meetings, much can be exchanged in couple of hours.” Jean married Jeanne early in his career and they lived in a flat in Boulevard Beaumarchais, a flat that she made particularly beautiful. Sadly, Jeanne pre-deceased him. I am deeply sorry that never again will I hold up a card saying “TAXI a BOULEVARD BEAUMARCHAIS”. Professor Jean Aicardi MD (Paris), FRCP (London), HonFRCPCH (UK), paediatric neurologist and epileptologist, was born on 8th November 1926. He died on 3rd August 2015, aged 88.