1600JohnF.KennedyBlvd. Ste1800 Philadelphia,PA19103-2899 STROKEINCHILDRENANDYOUNGADULTS ISBN:978-0-7506-7418-8 Copyright#2009bySaunders,animprintofElsevierInc. Allrightsreserved.Nopartofthispublicationmaybereproducedortransmittedinanyformorby anymeans,electronicormechanical,includingphotocopying,recording,oranyinformation storageandretrievalsystem,withoutpermissioninwritingfromthepublisher.Permissionsmaybe soughtdirectlyfromElsevier’sHealthSciencesRightsDepartmentinPhiladelphia,PA,USA:phone: (þ1)2152393804,fax:(þ1)2152393805,e-mail:[email protected] completeyourrequeston-lineviatheElsevierhomepage(http://www.elsevier.com),byselecting “CustomerSupport”andthen“ObtainingPermissions”. Notice Knowledgeandbestpracticeinthisfieldareconstantlychanging.Asnewresearchandexperience broadenourknowledge,changesinpractice,treatment,anddrugtherapymaybecomenecessaryor appropriate.Readersareadvisedtocheckthemostcurrentinformationprovided(i)onprocedures featuredor(ii)bythemanufacturerofeachproducttobeadministered,toverifytherecommended doseorformula,themethodanddurationofadministration,andcontraindications.Itisthe responsibilityofthepractitioner,relyingonhisorherexperienceandknowledgeofthepatient,to makediagnoses,todeterminedosagesandthebesttreatmentforeachindividualpatient,andtotake allappropriatesafetyprecautions.Tothefullestextentofthelaw,neitherthePublishernortheAuthor assumesanyliabilityforanyinjuryand/ordamagetopersonsorpropertyarisingoutoforrelatedto anyuseofthematerialcontainedinthisbook. ThePublisher LibraryofCongressCataloging-in-PublicationData Biller,Jose´. Strokeinchildrenandyoungadults/Jose´ Biller.--2nded. p.;cm. Includesbibliographicalreferencesandindex. ISBN-13:978-0-7506-7418-8 ISBN-10:0-7506-7418-0 1.Cerebrovasculardiseaseinchildren. 2.Cerebrovasculardisease. I.Title. [DNLM: 1.Stroke. 2.Adolescent. 3.Child. 4.Infant. 5.YoungAdult. WL355B597s2009] RJ496.C45S772009 618.92081--dc22 2008054235 AcquisitionsEditor:AdrianneBrigido DevelopmentalEditor:JoanRyan PublishingServicesManager:HemamaliniRajendrababu ProjectManager:JagannathanVaradarajan PrintedinUSA Lastdigitistheprintnumber: 9 8 7 6 5 4 3 2 1 This book is dedicated to the memory of Dr. William DeMyer. Known and loved by many as preeminent neuroanatomist, erudite teacher, tireless advisor, compassionate caregiver, gregarious sportsman, and consummate family man. He will be missed by those whom he touched with any facet of his multidimensional life. CONTRIBUTORS Thomas J. Altstadt, MD Neurological Surgery, Medford Neurological and Spine Center, Medford, Oregon Jose´ Biller, MD, FACP, FAAN, FAHA ProfessorandChairman,DepartmentofNeurology,LoyolaUniversityChicago, Stritch School of Medicine, Maywood, Illinois Rima M. Dafer, MD, MPH Associate Professor, Department of Neurology, Loyola University Chicago, Stritch School of Medicine, Maywood, Illinois William E. DeMyer, MD Professor Emeritus of Child Neurology, Indiana University; Riley Hospital for Children, Indianapolis, Indiana Meredith R. Golomb, MD, MSc Assistant Professor, Department of Neurology, Division of Pediatric Neurology, Indiana University School of Medicine; Riley Hospital for Children, Indianapolis, Indiana Lotfi Hacein-Bey, MD Professor, Departments of Radiology and Neurosurgery and Director, Neuroradiology and Interventional Neuroradiology, Loyola University Medical Center, Chicago, Illinois Betsy B. Love, MD Adjunct Clinical Associate Professor, Department of Neurology, Loyola University Chicago, Stritch School of Medicine, Maywood, Illinois James F. Meschia, MD Professor and Director, Cerebrovascular Division, Department of Neurology, Mayo Clinic, Jacksonville, Florida Thomas C. Origitano, MD, PhD, FACS Professor and Chair, Department of Neurological Surgery, Co-Director, The Center for Cranial Base Surgery, Director, Loyola Neuroscience Service Line, Loyola University Medical Center, Maywood, Illinois Hema Patel, MD Associate Professor, Department of Neurology, Section of Pediatric Neurology, Indiana University School of Medicine; Associate Professor, Department of vii viii Contributors Neurology, Section of Pediatric Neurology, Clarian Health Partners – James Whitcomb Riley Hospital for Children, Indianapolis, Indiana Michael B. Pritz, MD, PhD Professor, Department of Neurological Surgery and Director, Cerebrovascular and Skull Base Surgery, Indiana University School of Medicine; Attending Neurosurgeon, University Hospitals, Indianapolis, Indiana Richard B. Rodgers, MD Assistant Professor, Department of Neurological Surgery, Indiana University School of Medicine, Indianapolis, Indiana Michael J. Schneck, MD Associate Professor of Neurology and Neurosurgery, Department of Neurology, Loyola University Chicago, Stritch School of Medicine, Maywood, Illinois Eugene R. Schnitzler, MD Associate Professor of Neurology and Pediatrics and Chief, Division of Pediatric Neurology, Loyola University Chicago, Stritch School of Medicine; Department of Neurology and Pediatrics, Loyola University Medical Center, Maywood, Illinois Mitesh V. Shah, MD, FACS Associate Professor and Co-Director, Skull Base Surgery, Department of Neurosurgery, Indiana University, Indianapolis, Indiana Deborah K. Sokol, MD, PhD Associate Professor of Clinical Neurology, Section of Pediatrics, Indiana University School of Medicine; Pediatric Neurologist, Riley Hospital for Children, Indianapolis, Indiana Marc G. Weiss, MD Associate Professor, Department of Pediatrics and Director, Division of Neonatology, Loyola University Chicago, Stritch School of Medicine; Medical Director, Neonatal Intensive Care Unit, Ronald McDonald Children’s Hospital of Loyola University Medical Center, Maywood, Illinois FOREWORD Towriteaforewordtoasecondeditionisinmanywaysmucheasierthanfora first. One responds to success rather than predicting it. Fourteen years ago, Professor James Toole pointed out in the “foreword” the need for and the potential importance of this book, Stroke in Children and Young Adults. He concluded with the statement that “Professor Biller and his colleagues have authored a text that will stand the test of time.” Obviously he was correct. The success of the first edition established the need for and importance of the publication and, for 14 years, it stood the test of time. He also predicted that the new generation of clinical neuroscientists specializing in the preven- tion of and therapy for stroke would carry on to new heights of accomplish- ment. Again, he proved to be correct. Since 1994, this new generation has added so much to our understanding of stroke in children and young adults that this new edition is a necessity. ProfessorBillerandhiscolleaguesrespondedtothischallengeandextensively revised and added to the material originally published bringing this document up to date and including information published in the early part of 2008. This has resulted in extensive rewriting of the original 14 chapters and the addition ofthreenewchapters. This isindeedastate-of-the-artpublication. Forexample,most oftherefer- encesarepublishedafter1994.Asonereviewsthegalleys,oneisstruckbyhow much has been added to our knowledge during this time. In addition to many of the original contributors, others have been added and have continued the high quality of work produced in the first edition. The additional three chapters extend and add information to that included in the first edition. In particular, the chapter, Applied Anatomy of the Brain Arteries, by William DeMyer should serve as an invaluable addition for any understanding of vascular supply and clinical syndromes related to the brain arteries and for a reference in the future. It is unlikely that someone not work- ing primarily in stroke would keep all of these details constantly in mind. As this book was in the final editing process, Dr. DeMyer died at the age of 84 years. Although physically incapacitated during his final few months, he continued to work and contribute in many areas of Neurology and completed hisfinal book,TakingtheClinicalHistory:ElicitingSymptoms, EthicalFounda- tions,afewdaysbeforehisdeath.Thededicationofthisbooktohim,expresses the high regard that Biller, his colleagues, and all who know of his many ix x Foreword contributions and his work ethic have for serve as the state-of-the-art source for him.ItisalsoareflectionofProfessorBiller’s understanding of Stroke in Children and good judgment in selecting outstanding Young Adults. contributors for inclusion in this volume. Mark L. Dyken, M.D. Let us hope that the continued rapid Professor Emeritus of Neurology acquisition of knowledge makes it necessary Department of Neurology for a third edition long before 14 years. In Indiana University School of Medicine the meantime, this updated volume will Indianapolis, Indiana PREFACE TO THE FIRST EDITION Cerebrovasculardiseaseinchildrenandyoungadultsrepresentsachallengeto clinicalneurologists.Cerebrovasculardiseasespansallmedicalspecialties,and most clinicians are familiar with the catastrophic consequences of these disorders. Thisbookaddressesthepracticalneedsofhouseofficers,neurologists,neu- rosurgeons, as well as those of specialists in pediatrics, internal medicine, andfamilypracticewhocareforawidevarietyofyoungpatientswithischemic and hemorrhagic cerebrovascular disease. Stroke in Children and Young Adults provides a framework of clinical decision making and management of bothcommonlyandrarelyencounteredcerebrovasculardisordersintheyoung population. Afteranoverviewofstroketypes,riskfactors,prognosis,anddiagnosticstrat- egies in neonates, children, and young adults, the ischemic stroke subtypes are discussed separately to familiarize the reader with relevant issues in atherosclerotic cerebral infarction, non-atherosclerotic cerebral vasculopa- thies, cardiac disorders, and disorders of hemostasis. Additionally, a thorough discourse of miscellaneous topics—migraine and stroke, stroke and preg- nancy, rare genetic disorders associated with stroke, and cerebral venous thrombosis—is included. The final sections contain further insight into the practical and clinical information relative to intracerebral and subarachnoid hemorrhage. We hope our readers find this book useful and that it enhances their ability to optimize care for the young stroke patient. Acknowledgments I owe a special debt to my family for their support during this project. In particular, I wish to express endless gratitude to my wife Ce´lika for her unfail- ing patience and her assistance in organizing and preparing this book for publication. xi PREFACE Cerebrovascular disease in children and young adults accounts for 5% to 10% of all stroke cases and remains one of the top ten causes of childhood death, encompassing a broad range of causes and risk factors. This often represents a diagnostic and therapeutic challenge to clinicians with an average recog- nition time of 35.7 hours for the younger patients. Considerable progress has been made in our understanding of the incidence, etiology, diagnosis, and treatment of stroke in children and young adults. Even with this progress, however, clinicians, parents, patients, and caregivers can sometimes become disappointedorfrustrated because thecause ofthediseasemay remainunde- termined in a considerable percentage of patients and a uniform approach to treatment is often lacking. Cerebrovascular disease occurring in this age cate- gory spans multiple medical specialties. Clinicians caring for young stroke victimsarebecomingincreasinglyfamiliarwiththecatastrophicconsequences of these disorders which include not only a dramatic decline in the quality of life among survivors but potential socioeconomic consequences as well. This edition serves to provide an updated and more expansive resource that will be instrumental to clinical practices focusing on cerebrovascular disease in young people. It continues to address the practical needs of house officers, neurologists,andneurosurgeonsaswellastheneedsofspecialistsinthefields of pediatrics, internal medicine, family practice, emergency medicine, nursing and other allied health professionals who care for a wide variety of young patients with ischemic and hemorrhagic cerebrovascular disease. JustasintheFirstEdition,thebookbeginswithanoverviewofstroketypes, risk factors, prognosis, and diagnostic strategies in neonates, children and young adults. This is followed by a new, highly detailed and thoroughly illu- strated chapter on the applied anatomy of brain arteries, which is presented inordertofamiliarizethereaderwiththerelevantneuroanatomicalcorrelation of symptoms and signs pertaining to important stroke syndromes. Chapters 3 and 4 contain an expanded discussion on the epidemiology, clinical presenta- tion, evaluation, and treatments of stroke during the first 18 years of life and the individualized approach to neonates, children and young adults. The next threechaptersprovide adetaileddiscussiononatheroscleroticcerebralinfarc- tion, non-atherosclerotic vasculopathies, and cardiac disorders and strokes occurring in children and young adults. There are separate and fully updated chapterspertaining to cerebralinfarctionand migraines, aswell as hemostatic disorders presenting as stroke. Since pregnancy-associated stroke remains a major cause of serious morbidity and mortality, a comprehensive review of pregnancyassociatedischemicandhemorrhagic strokesisdiscussedindepen- dently. Similarly, as rare genetic disorders can lead to stroke, and diagnosis of these inherited conditions have important implications for the patient regarding stroke and his family, a concise review of rare genetic disorders that are associated with stroke is contained in Chapter 11. Cerebral venous xiii xiv Preface thrombosis represents less than 1% to 2% of focuses on pediatric central nervous sys- all stroke cases and although patients often tem (CNS) vascular malformations (a com- present later in the course of their disease, mon cause of non-traumatic intracerebral it is more easily diagnosed with the advent hemorrhage in this age group), and the of modern neuroimaging. Chapter 12 covers otheronthevarioustypesofspinalcordvas- theepidemiology,clinicalpresentation,diag- cular malformations in children and young nosis, and management of thrombosis of adults. the cerebral veins and sinuses along with Wehope thereadersofStroke inChildren the various etiologies which contribute to and Young Adults, Second Edition, will find its development. Subsequent chapters con- it to be current and clinically beneficial. In tain further insights into neonatal intracra- addition, we hope that the knowledge about nial hemorrhage (a significant problem in the disorders covered in this book will be neonatal intensive care units), spontaneous utilized to benefit the patients who have intracerebral hemorrhage (which accounts helped us increase our understanding of for about 15% of all strokes), and subarach- stroke within this age group. noid hemorrhage in young adults. Finally, there are two new chapters—one of which Jose Biller, MD
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