Acute Pediatric Neurology
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1 Acute Pediatric Neurology
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3 Thomas Sejersen Ching H. Wang Editors Acute Pediatric Neurology
4 Editors Thomas Sejersen Department of Pediatric Neurology Astrid Lindgrens Barnsjukhus Karolinska University Hospital Stockholm Sweden Pediatric Neurology unit Department of Women s and Children s Health Karolinska Institutet Stockholm Sweden Ching H. Wang Department of Pediatric Neurology Driscoll Children s Hospital Corpus Christi TX USA ISBN ISBN (ebook) DOI / Springer London Heidelberg New York Dordrecht Springer-Verlag London 2014 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. Exempted from this legal reservation are brief excerpts in connection with reviews or scholarly analysis or material supplied specifically for the purpose of being entered and executed on a computer system, for exclusive use by the purchaser of the work. Duplication of this publication or parts thereof is permitted only under the provisions of the Copyright Law of the Publisher's location, in its current version, and permission for use must always be obtained from Springer. Permissions for use may be obtained through RightsLink at the Copyright Clearance Center. Violations are liable to prosecution under the respective Copyright Law. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. While the advice and information in this book are believed to be true and accurate at the date of publication, neither the authors nor the editors nor the publisher can accept any legal responsibility for any errors or omissions that may be made. The publisher makes no warranty, express or implied, with respect to the material contained herein. Printed on acid-free paper Springer is part of Springer Science+Business Media (
5 We would like to dedicate this book to all our patients and their families. They gave us the opportunities to acquire our knowledge and experience that cumulated to the production of this book.
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7 Preface A substantial proportion of admissions to pediatric emergency departments, urgent care clinics, hospital wards, and pediatric intensive care units have pediatric neurological causes (20 30 %), or are accompanied by neurological issues. This illustrates that neurological diseases account for a large volume of pediatric emergencies, and many severe and critical pediatric cases. There is thus a great need for care providers to have access to updated literature aiding in diagnosis and management of emergencies related to pediatric neurology. However, we could not find such a book in the present day publications. Although there are very good books in acute pediatrics, they do not cover the topics of acute pediatric neurology in sufficient details. Considering the importance of pediatric neurology both in volume and in severity of pediatric emergencies, it was therefore a surprise for us to find the lack of an updated book dedicated to this field. This issue became more pressing to both of us when we encountered frequent requests for neurological consultations from general pediatricians and pediatric intensivists. These health providers frequently expressed an urgent need for a reference book that covers comprehensive topics of pediatric neurological issues that can be used as a rapid guide for the care of children in acute settings. Having previously had a very pleasant and productive collaboration in developing care guidelines for neuromuscular diseases, we (Thomas Sejersen and Ching Wang) decided to team up again to edit this book on acute pediatric neurology. It is our hope that we can to some extent fill this gap with the present multi-authored book. This book is intended to be a practical guide, covering major pediatric neurological diseases of relevance in the acute settings. The first part of the book, Chaps. 1, 2, 3, 4, 5, 6, 7, covers major acute neurological symptoms, and differential diagnoses to consider. These should help clinicians to recognize various neurological conditions presented, perform essential neurological examination, and formulate some differential diagnoses in preparation for further diagnostic workup. The second part of the book, Chaps. 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, is intended to give practical advice on managements including diagnostic assessments and therapeutic interventions on the common and important acute pediatric neurological vii
8 viii Preface conditions. Although each chapter will have much of its own individual characteristic, they will all have three key elements: (1) description of presenting symptoms, (2) recommended assessments, and (3) recommended interventions. Most chapters have at least one European and one North American author. With this we hope to cover some of the differences that may exist in diagnosis and management in Europe and North America. We hope that this book will reach a wide readership including pediatricians, pediatric neurologists, neurologists, pediatric intensivists, and neonatologists. Should this book be well received, we hope that possible mistakes will be corrected in a second edition to follow. This will also allow us to keep updating and adding valuable new information on managements in acute pediatric neurology. We want to use this opportunity to thank all the authors who contributed to the chapters in this book. They generously shared their precious experience in their field of expertise and worked with us patiently through the several revisions of the manuscripts. We also want to thank the developmental editors Barbara Lopez-Lucio and Joanna Bolesworth who helped compiling the chapters and keeping track on the production timelines, and the entire production team from Springer who produced this book in a pleasant and readable format. Lastly, we want to thank Drs. Darryl De Vivo and Lieven Lagae for their mentoring and encouragement and critical reading of the chapters. Stockholm, Sweden TX, USA Thomas Sejersen Ching H. Wang
9 Foreword I In times of nonstop and everywhere availability of web- knowledge, it seems strange to advocate a classic textbook on pediatric neurology and especially on acute problems in clinical pediatric neurology. Most of our fellows (those who actually see the patients in the emergency ward) know very well where to find the adequate dosage for treating an ongoing seizure, but sometimes lack the background scientific knowledge about long-lasting seizures and status epilepticus. Our classical teaching is very good in describing the most common neurological diseases, but not too much emphasis is put on the acute aspects of these diseases. In this respect, it is indeed time for a good and reliable textbook on acute aspects in pediatric neurology. Looking at my (full) bookcase, I find only one book on neurologic emergencies in infancy and childhood (eds. J.M. Pellock, E.C.Myer), published back in 1993! It is one of the few textbooks I have actually read from page 1 to page 408 (I admit) and I still consult it in late night situations when I get nervous about an acute neurological problem in a child on the ward. But now, I get even more nervous realizing that I continue to turn to a 20-year-old textbook The book you are holding in your hands, Acute Pediatric Neurology, therefore certainly fills a gap and a need. The book deals with almost all acute neurological problems in childhood and is written by experts in the field. What I like best in most chapters is the theoretical and pathophysiological background of these acute neurological situations. This is what we need to fully understand our treatment paradigms. It remains the only way to prevent the art of medicine of becoming a web-based cookbook business. In addition, lots of flowcharts and tables give advice on what diagnostic tests to run and which treatments are the most adequate. The editors and authors have to be congratulated with this textbook! I am sure it will be used in many teaching opportunities and in everyday clinical situations, but my advice is to simply start reading it now! It will give you a good and reassuring feeling, either because you will continue to fool yourself with I already know this or because (as in my case), you will silently admit a lot of things have changed in the last 20 years and you badly needed this magnificent update. Leuven, Belgium Lieven Lagae ix
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11 Foreword II It is hard to imagine anything more frightening to a parent than an acutely ill child with new-onset neurological symptoms. The presenting symptoms might include sudden loss of consciousness or memory, confusion or disorientation, fever and neck stiffness, acute-onset headache with early morning vomiting, a new-onset seizure or limb paralysis, acute loss of vision, dizziness, or vertigo. Furthermore, it is hard to imagine anything more challenging to the physician than evaluating this acutely ill child, noting the key presenting signs, formulating a differential diagnosis, and initiating an appropriate management plan. This textbook edited by Professors Thomas Sejersen and Ching Wang successfully prepares the clinician for the challenges presented by these common acute pediatric neurological disorders. It is required reading for any physician or nurse practitioner who may be responsible for the evaluation and the management of an acutely ill child with such a neurological complaint. These children may be seen in the doctor s office, in a walk-in clinic, or in the emergency room. As a result, the family physician, general practitioner, pediatrician, emergency medicine physician, and pediatric house staff need to be prepared to make the initial decisions when evaluating these acutely ill children; and, when immediately available, the child neurologist should be consulted and directly involved in formulating the diagnosis, evaluation, and treatment plan. The textbook is organized in an orderly and logical fashion with the first part addressing the acute symptomatic presentation, and the second part focusing on the urgent management of these many different conditions. Some of the disorders are acutely disabling, but transient, whereas others are life-threatening, such as bacterial meningitis and intracranial hypertension. Professors Sejersen and Wang share their considerable professional talent and expertise as senior child neurologists. Both editors are internationally recognized for their expertise in pediatric neuromuscular diseases. Each also has broad experience in the field of child neurology. To complement their collective clinical skills, the editors have assembled an international group of experts from eight different countries as authors of the 19 chapters. The end result is a modern, up-to-date description of the presentation and the xi
12 xii Foreword II management of the most common acute neurological disorders presenting in childhood. I suspect that you will enjoy reading their lucid, informative chapters as much as I have. New York, USA Darryl C. De Vivo, MD
13 Contents Part I Acute Neurological Symptoms 1 Unconsciousness, Coma, and Death by Neurological Criteria Tommy Stödberg, Claes G. Frostell, and Björn A. Larsson 2 Seizures James J. Riviello Jr. and Rod C. Scott 3 Acute Headache Jens Böhmer and Alyssa A. LeBel 4 Acute Disturbance of Motor Function Thomas Sejersen and Ching H. Wang 5 Acute Disturbance of Vision Kristina K.Teär Fahnehjelm and Douglas R. Fredrick 6 Dizziness and Vertigo Daune L. MacGregor and Maja Steinlin 7 Acute Changes of Behavior and Memory Per-Anders Rydelius Part II Management of Acute Neurological Conditions 8 Ischemic and Hypoxic Insults: Near Drowning, Asphyxia, Carbon Monoxide Poisoning Fenella J. Kirkham and Rebecca N. Ichord 9 Neurometabolic Crisis Linda J. De Meirleir xiii
14 xiv Contents 10 Approach to Childhood-Onset Muscle Cramps, Exercise Intolerance, and Recurrent Myoglobinuria Ingrid Tein 11 Management of Seizures and Status Epilepticus Suresh Pujar, James J. Riviello Jr., and Rod C. Scott 12 Management of Increased Intracranial Pressure Bengt Gustavsson 13 Management of Migraine and Other Headaches Jens Böhmer and Alyssa A. LeBel 14 Central Nervous System Infections (Bacteria and Parasites) Charles R.J.C. Newton 15 Management of Acute Neuromuscular Disorders Ching H. Wang and Thomas Sejersen 16 Stroke and Cerebrovascular Diseases Pinki A. Munot, Gabrielle A. deveber, and Vijeya Ganesan 17 Viral Infections and Autoimmune and Demyelinating Conditions of the Central Nervous System Marc Tardieu, Ariane G. Soldatos, and Mark P. Gorman 18 Spasticity, Dystonia, and Other Movement Disorders: A Comprehensive Treatment Guide A. Sebastian Schroeder, Steffen Berweck, Edward R. Dabrowski, and Florian Heinen 19 Acute Pain Stefan Lundeberg and Alyssa A. LeBel Index
15 Contributors Steffen Berweck, MD, PD Departments of Neuropaediatrics and Neurological Rehabilitation, Epilepsy Centre for Children and Adolescents, Day Clinic for Neuropaediatrics, Schoen Klinik Vogtareuth, Vogtareuth, Germany Jens Böhmer, MD Department of Pediatrics, Queen Silvia Children s Hospital, Sahlgrenska University, Gothenburg, Sweden Edward R. Dabrowski, MD Pediatric Physical Medicine and Rehabilitation, Children s Hospital of Michigan, Wayne State University, Detroit, MI, USA Department of Pediatrics, Children s Hospital of Michigan, Wayne State University, Detroit, MI, USA Linda J. De Meirleir, MD, PhD Division of Pediatric Neurology, UZ Brussels, Brussels, Belgium Gabrielle A. deveber, MD, MHSc Division of Neurology, The Hospital for Sick Children, Toronto, ON, Canada Kristina K. Teär Fahnehjelm, MD, PhD Department of Paediatric Ophthalmology and Strabismus, St. Erik Eye Hospital, Institute of Clinical Neuroscience, Karolinska Institutet, Stockholm, Sweden Douglas R. Fredrick, MD Department of Ophthalmology, Byers Eye Institute, Stanford University, Palo Alto, CA, USA Claes G. Frostell, MD, PhD Department of Anesthesia and Intensive Care, Karolinska Institutet at Danderyd Hospital, Stockholm, Sweden Vijeya Ganesan, MBChB, MD Neurosciences Unit, Institute of Child Health, UCL, London, UK Mark P. Gorman, MD Pediatric Neuro-Immunology, Department of Neurology, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA Bengt Gustavsson, MD Department of Neurosurgery, Clinical Neuroscience, Karolinska University Hospital, Stockholm, Sweden xv
16 xvi Contributors Florian Heinen, MD Department of Pediatric Neurology and Developmental Medicine, Social Pediatrics, Hauner Children s Hospital, University of Munich, Munich, Bavaria, Germany Rebecca N. Ichord, MD Division of Neurology and Pediatrics, The Children s Hospital of Philadelphia, Philadelphia, PA, USA Department of Neurology and Pediatrics, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA Fenella J. Kirkham, MB, B Chir, MD, FRCPCH Department of Neurosciences, UCL Institute of Child Health, University College London, London, UK Department of Child Health, University Hospital Southampton NHS Foundation Trust, Southampton, UK Björn A. Larsson, MD, PhD Paediatric Intensive Care Unit, Astrid Lingren Children s Hospital, Karolinska University Hospital, Stockholm, Sweden Alyssa A. LeBel, MD Pediatric Headache Program, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA Chronic Pain Clinic, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA Pain Medicine, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA Department of Neurology, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA Department of Anesthesia, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA Stefan Lundeberg, MD, PhD Pain Treatment Service, Department of Pediatric Anesthesia and Intensive Care, Astrid Lindgren Children s Hospital, Karolinska University Hospital, Stockholm, Sweden Daune L. MacGregor, MD, DCH, PBDM, FRCP(C) Department of Pediatrics, The Hospital for Sick Children, Toronto, ON, Canada Pinki A. Munot, MD, MRCPCH, MBBS Neurosciences Unit, Great Ormond Street Hospital NHS Trust, London, UK Charles R.J.C. Newton, MBChB, MRCP, MD, FRCPCH Department of Psychiatry, University of Oxford, Oxford, UK Suresh Pujar, MBBS, MD, MRCPCH Department of Paediatric Neurosciences, UCL Institute of Child Health, London, UK James J. Riviello, Jr., MD Division of Pediatric Neurology, Department of Neurology, New York University School of Medicine, New York, NY, USA
17 Contributors xvii NYU Comprehensive Epilepsy Center, NYU Langone Medical Center, New York, NY, USA Per-Anders Rydelius, MD, PhD Department of Women s and Children s Health, Astrid Lingren s Children s Hospital, Karolinska Institutet, Stockholm, Sweden A. Sebastian Schroeder, MD Department of Pediatric Neurology and Developmental Medicine, Social Pediatrics, Hauner Children s Hospital, University of Munich, Munich, Bavaria, Germany Rod C. Scott, MD, PhD Department of Neurosciences, UCL Institute of Child Health, London, UK Department of Neurology, Dartmouth Medical School, Hanover, NH, USA Thomas Sejersen, MD, PhD Department of Pediatric Neurology, Astrid Lindgrens Barnsjukhus, Karolinska University Hospital, Stockholm, Sweden Pediatric Neurology unit, Department of Women s and Children s Health, Karolinska Institutet, Stockholm, Sweden Ariane G. Soldatos, MD Department of Neurology, Children s Hospital Boston, Harvard Medical School, Boston, MA, USA Maja Steinlin, MD Department of Paediatrics, Inselspital, Bern University Hospital, Bern, Switzerland Tommy Stödberg, MD Neuropediatric Unit, Astrid Lindgren Children s Hospital, Karolinska University Hospital, Stockholm, Sweden Marc Tardieu, MD, PhD Department of Neurosciences, Hôpitaux universitaires Paris Sud, Université Paris Sud, Paris, France Ingrid Tein, BSc, MD, FRCP (C) Department of Neurology, Neurometabolic Clinic and Research Laboratory, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada Genetic and Genome Biology Program, The Research Institute, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada Department of Pediatrics, Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada Ching H. Wang, MD, PhD College of Medicine, Texas A&M University Health Science Center, Bryan, TX, USA Department of Pediatric Neurology, Driscoll Children s Hospital, Corpus Christi, TX, USA
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