Jean Aicardi Research Articles

Foreword.

Welcome to the 42nd Annual Conference of the BPNA. Congratulations to the Sheffield team for putting together an exciting programme combining a rich array of keynote speakers, scientific papers and posters, personal practice sessions and interest group meetings. There is something for all of our members and more. On a personal note, I would like to make this year's meeting an opportunity to celebrate the life and work of Jean Aicardi who sadly died in 2015 at the age of 88. He was not only one of the most respected and influential of figures in paediatric neurology but also a long time friend and member of our association. He wrote many influential papers and three definitive textbooks. I am particularly pleased this year to welcome our MacKeith lecturer, Professor Yanick Crow who will be describing the remarkable advances in understanding that have grown out of the unravelling of the disease that Jean Aicardi and Francoise Goutières first described in 1984. I am pleased also to welcome as a guest, one of Jean Aicardi's former pupils and collaborators, Professor Alexis Arzimanoglou from Lyon. This celebration of our French connection reminds us of the great and mutual benefits of collaboration with our international colleagues. I would particularly like to welcome our younger participants, medical students, trainees and others for whom I wish the meeting will provide further inspiration and stimulus to pursue a career in the exciting and challenging field of paediatric neurology.

Professor Jean Aicardi

Professor Jean Aicardi

A new perspective for Epileptic Disorders

Since its launch in late 1999 under the direction of Jean Aicardi, founding Editor-in-Chief, Epileptic Disorders has served the medical and scientific community in an expanding discipline of epileptology in which new diagnostic techniques and approaches to management are continually being developed. A significant amount of new information in all areas of epileptology has accumulated at a brisk pace, leading to a steady increase in the number of articles submitted to the journal. As intended from the outset, the descriptive detailed clinical symptomatology and phenomenology of epileptic seizures and syndromes, in relation to diagnosis and management, published in Epileptic Disorders has served to complement other journals in the field. Epileptic Disorders continues to provide audiovisual material

Jean Aicardi: My Circuitous Path to Becoming a French Child Neurologist and Epileptologist

Jean Aicardi: My Circuitous Path to Becoming a French Child Neurologist and Epileptologist

DISEASES OF THE NERVOUS SYSTEM IN CHILDHOOD, 3RD EDITION

edited by Jean Aicardi, 912 pp., Mac Keith Press, 2009, $335 Reviewing a major textbook is a daunting task. Reading a 950-page tome cover-to-cover is obviously out of the question, while reviewing by sampling at random is inadequate. The solution was to use the book for several months, as it was meant to be used: as a reference book. Fortunately, the book arrived as I was preparing to write test questions and posttest review discussions for the 2010 RITE examination. As each and every question requires multiple references, this …

Aicardi syndrome in a 47, XXY male neonate with lissencephaly and holoprosencephaly

Aicardi syndrome (AS) is a rare neuro-ophthalmic disorder first described by Jean Aicardi in 1965 with a characteristic triad of corpus callosal agenesis (CCA), chorioretinal lacunae (CRL), and infantile spasms (IS). All known cases of AS have been sporadic and a responsible gene has not been identified. With 5 exceptional males, potential X-linked dominant genetic mutation characterizes AS occurring almost exclusively in girls. Most of male AS cases were still debatable in diagnosis either for their 46 XY karyotype or too atypical presentations to fit the formerly stricter diagnostic criteria. We report a 47, XXY male neonate presenting some undisputable, but otherwise some regarded as atypical features in AS. We compare his distinctively clinical pictures with previously reported male cases and find CRL is less pathognomonic and lissencephaly appears frequently among male AS. Because of insufficient genetic and biochemical markers for definite diagnosis at this moment, we suggest the experience of a relatively rare male case would help to shed light on the underlying genetic pathogenesis of AS.

Aicardi's Epilepsy in Children, Third Edition. 2004. By Alexis Arzimanoglou, Renzo Guerrini, Jean Aicardi. Published by Lippincott Williams & Wilkins. 487 pages. C$175 approx.

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Book Reviews: Movement Disorders in Children, by Emilio Fernandez-Alvarez, Jean Aicardi. Published in 2001 by MacKeith Press, London, 272 pages, $75.00

Book Reviews: Movement Disorders in Children, by Emilio Fernandez-Alvarez, Jean Aicardi. Published in 2001 by MacKeith Press, London, 272 pages, $75.00

EPILEPSY AND MOVEMENT DISORDERS. 2002. Edited by Renzo Guerrini, Jean Aicardi Frederick Andermann and Mark Hallet. Published by Cambridge University Press. 557 pages. C$193.40 approx.

EPILEPSY AND MOVEMENT DISORDERS. 2002. Edited by Renzo Guerrini, Jean Aicardi Frederick Andermann and Mark Hallet. Published by Cambridge University Press. 557 pages. C$193.40 approx.

Book Reviews

Book Reviewed in this articleMovement Disorders in Childre By Emilio Fernandez‐Alvarez and Jean Aicardi London, Mac Keith Press (International Review of Child Neurology Series)Child Neurology (6th edn) Edited by John H Menkes and Harvey B Stewart Philadelphia PA, Lippincott Williams and Wilkins.Limbic seizures in children Edited by G Avanzini, A Beaumanoir and L Mira London, John Libbey.Keeping Healthy‘Down Below’By Sheila Hollins, Jackie Downer, and Beth Webb London, Gaskell and St George's Hospital Medical School.Looking After My Breasts By Sheila Hollins, Jackie Downer, Wendy Perez, and Beth Webb London, Gaskell and St George's Hospital Medical School.

DISEASES OF THE NERVOUS SYSTEM IN CHILDHOOD.

By Jean Aicardi. 1998. Cambridge: Cambridge University Press.Price £150. ISBN 1-898-68316-6. The practice of the neurology of childhood is a particularly fascinating and demanding occupation. Maturational and developmental changes in the anatomy, physiology and biochemistry of all parts of the nervous system influence the possible historical details and physical findings. The recognition of the early manifestations of disorders more readily diagnosed when fully developed is especially challenging. It is essential to have a comprehensive text with which to tackle the problems of children whose neurological signs are static, but very debilitating; those with deteriorating conditions; and those, such as the ones with epilepsy, for whom treatment can lead to improvement, if not always cure. Jean Aicardi's encyclopaedic knowledge of the disorders which might cause neurological problems in children was recognized long before he produced the first edition of his definitive text. This second edition has been thoroughly updated. Although written almost exclusively by Aicardi, there are chapters on metabolic diseases and cerebral palsy to which Ogier and Bax have contributed. The section on developmental and neuropsychiatric disorders is by Christopher Gillberg. The layout and chapter …

Book Reviews

Book reviewed in this article: Paediatric Epilepsy Syndromes and Their Surgical Treatment. Current Problems in Epilepsy: 11. I. Tuxhorn, H. Holthausen, and H. Boenigk, eds. Diagnosis and Management of Neonatal Seizures. Eli M. Mizrahi and Peter Kellaway. Jean Aicardi: Diseases of the Nervous System to Childhood, 2nd Ed. Clinics in Development Medicine Series. Challenge Epilepsy‐New Antiepileptic Drugs. Herman Stefan, Günter Krämer, and Bruno Mamoli, eds. Quality of Life and Pharmacoeconomics, An Introduction. Joyce A. Cramer and Bert Spilker.

Alternating hemiplegia of childhood (the international review of child neurology) Edited by Frederick Andermann, MD, FRCP, Jean Aicardi, MD, FRCP, and Frederico Vigeuano, MD New York, Raven press, 1995 240 pp, $85.00

Alternating hemiplegia of childhood (the international review of child neurology) Edited by Frederick Andermann, MD, FRCP, Jean Aicardi, MD, FRCP, and Frederico Vigeuano, MD New York, Raven press, 1995 240 pp, $85.00

Book Review: Epilepsy in Children, 2nd ed (The International Review of Child Neurology), by Jean Aicardi. Published in 1994 by Raven Press, New York, 571 pages, $95.00

Book Review: Epilepsy in Children, 2nd ed (The International Review of Child Neurology), by Jean Aicardi. Published in 1994 by Raven Press, New York, 571 pages, $95.00

Alternating Hemiplegia of Childhood (International Review of Child Neurology Series)

edited by FREDERICK ANDERMANN, JEAN AICARDI, and FEDERICO VIGEVANO, 240 pp, ill., New York, Raven Press, 1995. $85.00 This is a monograph published as part of the International Review of Child Neurology series. Its 28 brief chapters are the result of presentations on the topic made at a meeting of …

Book Review

Book review are in this article: Epilepsy in Children, 2nd edition. Jean Aicardi, ed.

Book Review: Diseases of the Nervous System in Childhood, by Jean Aicardi. Published in 1992 by Mac Keith Press, London, 1408 pages, $155.00

Book Review: Diseases of the Nervous System in Childhood, by Jean Aicardi. Published in 1992 by Mac Keith Press, London, 1408 pages, $155.00

Book Review Diseases of the Nervous System in Childhood (Clinics in Developmental Medicine. No. 115/118.) by Jean Aicardi, with three contributors. 1408 pp., illustrated. London, MacKeith Press, 1992. $155. (Distributed in the U.S. by Cambridge University Press, New York.) 0-521-41273-0

Book Review Diseases of the Nervous System in Childhood (Clinics in Developmental Medicine. No. 115/118.) by Jean Aicardi, with three contributors. 1408 pp., illustrated. London, MacKeith Press, 1992. $155. (Distributed in the U.S. by Cambridge University Press, New York.) 0-521-41273-0

Book Review: Epilepsy in Children, by Jean Aicardi. Published in 1986 by Raven Press, New York, 425 pages, $67.50

Book Review: Epilepsy in Children, by Jean Aicardi. Published in 1986 by Raven Press, New York, 425 pages, $67.50

Epilepsy in Children

As its three predecessors have been, this fourth volume of the International Review of Child Neurology series (official publications of the International Child Neurology Association) is a classic. This book, by Jean Aicardi, MD, a singleauthor publication with over 1500 references, is a masterPiece. As he notes in the preface, many pediatric specialists are only imperfectly aware of recent developments in epileptology. To rectify this situation, Dr Aicardi stresses clinical aspects of seizures and epileptic syndromes, emphasizing information that will help pediatricians to diagnose, investigate, and treat epilepsy in children. The book's four major parts include general notions about seizures; the major types of seizures and epileptic syndromes; epileptic manifestations of infancy and childhood; and general aspects of diagnosis, prognosis, and treatment. While only slightly over 300 pages in length, it is almost encyclopedic in its coverage of specific seizure types (and may tell the practicing clinician more than he