Seizures and Epilepsy

This second edition of Seizures and Epilepsy, written almost a quarter of a century after the ground-breaking first edition, is more than an update: it is a complete revision due to tremendous advances in the field. Our understanding of the fundamental neuronal mechanisms underlying epileptic phenomena, as well as current diagnosis and treatment, have been heavily influenced over the past several decades by seminal neuroscientific developments, particularly the introduction of molecular neurobiology, genetics, and modern neuroimaging. According to the World Health Organization, epilepsy accounts for 1% of the global burden of disease, equivalent to breast cancer in women and lung cancer in men. Among primary disorders of the brain, it is equivalent to depression, dementia, and substance abuse. Singly authored by Jerome Engel, Jr, this must-read from 1989 reasserts itself as a modern classic comprehensive textbook covering a broad range of both basic and clinical epileptology. Reviews The new edition of Seizures and Epilepsy updates an outstanding volume, initially published in 1989. Dr. Engel has been able to reduct the numerous current advances in epileptology, (i.e. genetics, imaging, molecular biology, electroencephalography, treatments) since the original publication, and make them all readily available to all practitioners interested in epilepsy. This easy to read volume is a must." -- Solomon L. Mosh, MD, President, International League Against Epilepsy, Charles Frost Chair In Neurosurgery and Neurology, Professor of Neurology, Neuroscience & Pediatrics, Vice-Chair, Dept. of Neurology, Director, Pediatric Neurology & Clinical Neurophysiology, Einstein College of Medicine, Bronx, NY "Professor Engel has been one of the most influential world figures in epilepsy over the last 40 years. The 2nd edition of this book has thus been eagerly awaited, and it is indeed a magnificent achievement. It is a compendium of knowledge and wisdom that will surely become a standard reference for all clinicians working in the field." --Simon Shorvon FRCP. Professor in Clinical Neurology, UCL Institute of Neurology and the National Hospital for Neurology and Neurosurgery, Queen Square London, and Co-Editor in Chief of Epilepsia About the Author Jerome Engel, Jr., MD, PhD, is Director of the Seizure Disorder Center, and The Jonathan Sinay Distinguished Professor of Neurology, Neurobiology, and Psychiatry and Biobehavioral Sciences at UCLA. He is past president of the American Clinical Neurophysiology Society, the American Epilepsy Society, and the International League Against Epilepsy, and is past co-chair of the Global Campaign against Epilepsy. His bibliography lists over 1,000 publications and over 30 books, and he is principal investigator on three research grants from the National Institute of Neurological Disorders and Stroke. He has received numerous awards and honors, including a Fulbright Scholarship, a Guggenheim Fellowship, and a Javits Award. Table of Contents PART I: INTRODUCTION ; CHAPTER 1 ; TERMINOLOGY AND CLASSIFICATIONS ; TERMINOLOGY ; EPILEPTIC SEIZURES ; DEFINITION ; DISCUSSION ; EPILEPSY DISORDERS ; DEFINITION ; DISCUSSION ; EPILEPTIC AND EPILEPSY ; DEFINITION ; DISCUSSION ; ICTAL, POSTICTAL, AND INTERICTAL ; DEFINITIONS ; DISCUSSION ; EPILEPTOGENESIS AND EPILEPTOGENICITY ; DEFINITION ; DISCUSSION ; EPILEPTIC SPIKE FOCUS, EPILEPTIC LESION, AND EPILEPTOGENIC ZONE OR REGION ; DEFINITION ; DISCUSSION ; CONTROL, CURE, AND DRUG RESISTANCE ; DEFINITION ; DISCUSSION ; ANTIEPILEPTOGENESIS, PREVENTION, AND DISEASE MODIFICATION ; DEFINITION ; DISCUSSION ; NONEPILEPTIC SEIZURES ; DEFINITION ; DISCUSSION ; CLASSIFICATIONS ; OLDER CLASSIFICATIONS OF EPILEPTIC SEIZURE ; INTERNATIONAL CLASSIFICATION OF EPILEPTIC SEIZURES ; OTHER SEIZURE CLASSIFICATIONS ; OLDER CLASSIFICATIONS OF THE EPILEPSIES ; THE 1970 INTERNATIONAL CLASSIFICATION ; THE WHO CLASSIFICATIONS ; THE 1985 AND 1989 INTERNATIONAL CLASSIFICATIONS ; RECENT EFFORTS TO REVISE THE INTERNATIONAL CLASSIFICATIONS ; PRACTICAL CONSIDERATIONS ; SUMMARY AND CONCLUSIONS ; CHAPTER 2 ; PERSPECTIVES ; EPILEPSY THEN-HISTORICAL PERSPECTIVE ; MEDICAL HISTORY ; ANCIENT AND MEDIEVAL PERIODS ; THE MODERN ERA ; EPILEPSY AND RELIGION ; EPILEPSY AND GENIUS ; EPILEPSY NOW-EPIDEMIOLOGICAL PERSPECTIVE ; DEFINITIONS OF INCIDENCE AND PREVALENCE ; METHODOLOGICAL CONSIDERATIONS ; ESTIMATIONS OF INCIDENCE AND PREVALENCE ; GLOBAL BURDEN OF DISEASE ; SUMMARY AND CONCLUSIONS ; PART II: PATHOPHYSIOLOGY ; CHAPTER 3 ; MECHANISMS OF NEURONAL EXCITATION AND SYNCHRONIZATION ; THE NEURON ; THE EXCITABLE MEMBRANE AND ITS MICROENVIRONMENT ; VOLTAGE GATED CHANNELS ; MEMBRANE PHYSIOLOGY ; INTRACELLULAR PROCESSES ; STRUCTURE-FUNCTION RELATIONSHIP OF NEURONAL ELEMENTS ; INTERNEURONAL CONNECTIONS ; CHEMICAL SYNAPSES ; ELECTROTONIC SYNAPSES ; NONSYNAPTIC COMMUNICATION ; GLIAL INFLUENCES ; NEURONAL NETWORKS ; INTRINSIC ORGANIZATION OF AMMON'S HORN ; INTRINSIC ORGANIZATION OF THE NEOCORTEX ; SUBCORTICAL AND INTERHEMISPHERIC CONNECTIONS ; PHYLOGENY AND ONTOGENY ; SPECIES DIFFERENCES ; CORTICAL DEVELOPMENT ; MATURATIONAL EFFECTS ON EXCITABILITY ; SYSTEMIC INFLUENCES ; NEURONAL BASIS OF EEG ACTIVITY ; METHODOLOGICAL DEVELOPMENTS ; SUMMARY AND CONCLUSIONS ; CHAPTER 4 ; BASIC MECHANISMS OF SEIZURES AND EPILEPSY ; EXPERIMENTAL MODELS OF SEIZURES AND EPILEPSY ; ACUTE MODELS ; IN VIVO MODELS OF FOCAL SEIZURES ; IN VIVO MODELS OF GENERALIZED SEIZURES ; IN VITRO STUDIES OF ACUTE SEIZURES ; CHRONIC MODELS ; IN VIVO MODELS OF EPILEPSY WITH FOCAL SEIZURES ; LESION AND INSULT MODELS ; MODELS IN THE DEVELOPING BRAIN ; SECONDARY EPILEPTOGENESIS ; IN VIVO MODELS OF EPILEPSY WITH GENERALIZED SEIZURES ; IN VITRO STUDIES OF CHRONIC EPILEPSY ; STUDIES OF HUMAN EPILEPSY ; ELECTROPHYSIOLOGICAL INVESTIGATIONS ; INVESTIGATIONS USING BRAIN IMAGING TECHNIQUES ; MICROANATOMICAL INVESTIGATIONS ; BIOCHEMICAL AND MOLECULAR INVESTIGATIONS ; MOLECULAR GENETIC INVESTIGATIONS ; GENE DISCOVERY ; ACQUIRED GENETIC DISTURBANCES ; POSSIBLE MECHANISMS OF HUMAN EPILEPTIC PHENOMENA ; EPILEPTOGENESIS ; INTERICTAL STATE ; ICTOGENESIS ; ICTUS ; ICTAL TERMINATION ; POSTICTAL PERIOD ; POSSIBLE CONSEQUENCES OF EPILEPTIC SEIZURES ; SUMMARY AND CONCLUSIONS ; CHAPTER 5 ; CAUSES OF HUMAN EPILEPSY ; NONSPECIFIC PREDISPOSING FACTORS ; GENETIC FACTORS ; ENVIRONMENTAL FACTORS ; DYNAMIC ASPECTS OF THRESHOLD ; SPECIFIC EPILEPTOGENIC DISTURBANCES ; GENETIC CAUSES OF EPILEPSY DISORDERS ; GENETIC EPILEPSIES ; GENETIC DISORDERS ASSOCIATED WITH EPILEPSY ; ACQUIRED CAUSES OF EPILEPSY DISORDERS ; ANOXIA AND TRAUMA ; CEREBROVASCULAR DISORDERS ; BRAIN TUMORS ; INFECTIONS DISEASES ; INFLAMMATION AND IMMUNE-MEDIATED DISORDERS ; MALFORMATIONS OF CORTICAL DEVELOPMENT ; TOXIC AND METABOLIC DISTURBANCES ; HIPPOCAMPAL SCLEROSIS ; PRECIPITATING FACTORS ; NONSPECIFIC PRECIPITATING FACTORS ; SPECIFIC PRECIPITATING FACTORS ; SUMMARY AND CONCLUSIONS ; PART III: PHENOMENOLOGY ; CHAPTER 6 ; EPILEPTIC SEIZURES ; NEUROBIOLOGICAL CONSIDERATIONS FOR THE DIAGNOSIS OF EPILEPTIC ; SEIZURE TYPES ; ANATOMIC SUBSTRATES ; PATHOPHYSIOLOGICAL MECHANISMS ; PHARMACOLOGICAL CONSIDERATIONS ; TYPES OF EPILEPTIC SEIZURES ; DEFINITIONS ; FOCAL SEIZURES ; NEOCORTICAL SEIZURES ; MECHANISMS ; PHENOMENOLOGY ; MOTOR SIGNS ; SENSORY SYMPTOMS ; EEG ; CLINICAL CONSIDERATIONS ; LIMBIC SEIZURES ; MECHANISMS ; PHENOMENOLOGY ; LIMBIC SENSORY SYMPTOMS ; AUTONOMIC SYMPTOMS AND SIGNS ; PSYCHIC SYMPTOMS ; AUTOMATISMS ; EEG ; CLINICAL CONSIDERATIONS ; FOCAL SEIZURES EVOLVING INTO SECONDARILY GENERALIZED SEIZURES ; MECHANISMS ; PHENOMENOLOGY ; CLINICAL CONSIDERATIONS ; GENERALIZED SEIZURES ; TONIC-CLONIC SEIZURES ; MECHANISMS ; PHENOMENOLOGY ; EEG ; CLINICAL CONSIDERATIONS ; TYPICAL ABSENCE SEIZURES ; MECHANISMS ; PHENOMENOLOGY ; EEG ; CLINICAL CONSIDERATIONS ; ATYPICAL ABSENCE SEIZURES ; MECHANISMS ; PHENOMENOLOGY ; EEG ; CLINICAL CONSIDERATIONS ; MYOCLONIC ABSENCES ; EYELID MYOCLONIA ; MYOCLONIC SEIZURES ; MECHANISMS ; PHENOMENOLOGY ; EEG ; CLINICAL CONSIDERATIONS ; MYOCLONIC ATONIC SEIZURES ; MYOCLONIC TONIC SEIZURES ; CLONIC SEIZURES ; MECHANISMS ; PHENOMENOLOGY ; EEG ; CLINICAL CONSIDERATIONS ; TONIC SEIZURES ; MECHANISMS ; PHENOMENOLOGY ; EEG ; CLINICAL CONSIDERATIONS ; ATONIC SEIZURES ; MECHANISMS ; PHENOMENOLOGY ; EEG ; CLINICAL CONSIDERATIONS ; UNCLASSIFIED SEIZURES ; EPILEPTIC SPASMS ; REFLEX SEIZURES ; NEONATAL SEIZURES ; MYOCLONUS ; SUMMARY AND CONCLUSIONS ; CHAPTER 7 ; EPILEPSY SYNDROMES ; NEONATAL PERIOD ; BENIGN FAMILIAL NEONATAL EPILEPSY (BFNE) ; EARLY MYOCLONIC EPILEPSY (EME) ; OHTAHARA SYNDROME (OS) ; INFANCY ; EPILEPSY OF INFANCY WITH MIGRATING FOCAL SEIZURES (MPSI) ; WEST SYNDROME (WS) ; CLINICAL DESCRIPTION ; EPIDEMIOLOGY ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; MYOCLONIC EPILEPSY IN INFANCY (MEI) ; BENIGN INFANTILE EPILEPSY (BIE) AND BENIGN FAMILIAL INFANTILE EPILEPSY (BFIE) ; DRAVET SYNDROME (DS) ; MYOCLONIC ENCEPHALOPATHY IN NON-PROGRESSIVE DISORDERS ; CHILDHOOD ; FEBRILE SEIZURES PLUS (FS+) ; PANAYIOTOPOULOS SYNDROME (PS) ; EPILEPSY WITH MYOCLONIC-ATONIC SEIZURES (EMAS) ; BENIGN EPILEPSY WITH CENTROTEMPORAL SPIKES (BECTS) ; CLINICAL DESCRIPTION ; EPIDEMIOLOGY ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; AUTOSOMAL-DOMINANT NOCTURNAL FRONTAL LOBE EPILEPSY (ADNFLE) ; LATE CHILDHOOD OCCIPITAL EPILEPSY (GASTAUT TYPE) ; EPILEPSY WITH MYOCLONIC ABSENCES (EMA) ; LENNOX-GASTAUT SYNDROME (LGS) ; CLINICAL DESCRIPTION ; EPIDEMIOLOGY ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; EPILEPTIC ENCEPHALOPATHY WITH CONTINUOUS SPIKE AND WAVE DURING SLEEP (CSWS) ; LANDAU-KLEFFNER SYNDROME (LKS) ; CHILDHOOD ABSENCE EPILEPSY (CAE) ; CLINICAL DESCRIPTION ; EPIDEMIOLOGY ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; ADOLESCENCE-ADULT ; JUVENILE ABSENCE EPILEPSY (JAE) ; JUVENILE MYOCLONIC EPILEPSY (JME) ; CLINICAL DESCRIPTION ; EPIDEMIOLOGY ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; EPILEPSY WITH GENERALIZED TONIC-CLONIC SEIZURES ALONE ; PROGRESSIVE MYOCLONUS EPILEPSIES (PME) ; AUTOSOMAL-DOMINANT EPILEPSY WITH AUDITORY FEATURES (ADEAF) ; OTHER FAMILIAL TEMPORAL LOBE EPILEPSIES ; LESS SPECIFIC AGE RELATIONSHIP ; FAMILIAL FOCAL EPILEPSY WITH VARIABLE FOCI ; REFLEX EPILEPSIES ; DISTINCTIVE CONSTELLATIONS ; MESIAL TEMPORAL LOBE EPILEPSY WITH HIPPOCAMPAL SCLEROSIS (MTLE WITH HS) ; CLINICAL DESCRIPTION ; EPIDEMIOLOGY ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; RASMUSSEN SYNDROME (RS) ; GELASTIC SEIZURES WITH HYPOTHALAMIC HAMARTOMA ; HEMICONVULSION-HEMIPLEGIA EPILEPSY (HHE) ; CONDITIONS WITH EPILEPTIC SEIZURES THAT ARE TRADITIONALLY NOT DIAGNOSED AS A FORM OF EPILEPSY PER SE ; BENIGN NEONATAL SEIZURES (BNS) ; FEBRILE SEIZURES (FS) ; CLINICAL DESCRIPTION ; EPIDEMIOLOGY ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; REACTIVE SEIZURES ; NEONATAL SEIZURES DUE TO STRUCTURAL AND METABOLIC DISORDERS ; CLINICAL DESCRIPTION ; EPIDEMIOLOGY ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; MYOCLONIC SYNDROMES ; SUMMARY AND CONCLUSIONS ; CHAPTER 8 ; EPILEPTOGENESIS ; THE DEVELOPMENT OF EPILEPSY ; PHENOMENOLOGY ; CHARACTER OF THE LESION ; LOCATION OF THE LESION ; GENETIC FACTORS ; PRACTICAL CONSIDERATIONS ; PROPHYLAXIS FOR EARLY SEIZURES ; PROPHYLAXIS FOR CHRONIC EPILEPSY ; THE PROGRESSIVE NATURE OF EPILEPSY ; SECONDARY EPILEPTOGENESIS ; CLINICAL EVIDENCE FOR PROGRESSION ; PRACTICAL CONSIDERATIONS ; EPILEPTIC SEIZURES AND BRAIN DEVELOPMENT ; EPILEPTIC SUSCEPTIBILITY OF THE IMMATURE BRAIN ; EVOLUTION OF EPILEPTIC SYNDROMES ; MIGRATION OF THE INTERICTAL EEG SPIKE ; PRACTICAL CONSIDERATIONS ; SUMMARY AND CONCLUSIONS ; CHAPTER 9 ; PERIICTAL PHENOMENA ; ICTAL INITIATION ; MECHANISMS ; CLINICAL CONSIDERATIONS ; REFLEX SEIZURES ; GENETIC REFLEX EPILEPSIES ; REFLEX EPILEPSIES DUE TO STRUCTURAL LESIONS ; SIMPLE VS. COMPLEX REFLEX EPILEPSIES ; REFLEX MYOCLONUS ; ABORTIVE STIMULI ; SLEEP-WAKE CYCLES AND CHRONOBIOLOGY ; METABOLIC AND TOXIC INFLUENCES ; PSYCHOLOGICAL STRESS ; POSTICTAL SYMPTOMS ; MECHANISMS ; CLINICAL CONSIDERATIONS ; SPECIFIC POSTICTAL CEREBRAL DYSFUNCTION ; NONSPECIFIC POSTICTAL CEREBRAL DYSFUNCTION ; NONCEREBRAL POSTICTAL DYSFUNCTION ; MORTALITY ; COMORBIDITY ; SUDDEN UNEXPECTED DEATH ; DROWNING ; SUMMARY AND CONCLUSIONS ; CHAPTER 10 ; STATUS EPILEPTICUS ; GENERALIZED CONVULSIVE STATUS EPILEPTICUS ; CLINICAL DESCRIPTION ; TONIC-CLONIC STATUS EPILEPTICUS ; OTHER FORMS OF GENERALIZED CONVULSIVE STATUS EPILEPTICUS ; INCIDENCE ; ETIOLOGY ; TREATMENT ; LORAZEPAM ; DIAZEPAM ; FOSPHENYTOIN ; PHENOBARBITAL ; PARALDEHYDE ; OTHER DRUGS ; GENERAL ANESTHESIA ; EEG MONITORING, VENTILATION, AND FOLLOW-UP ; PROGNOSIS ; ABSENCE STATUS EPILEPTICUS ; CLINICAL DESCRIPTION ; INCIDENCE ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; EPILEPSIA PARTIALIS CONTINUA ; CLINICAL DESCRIPTION ; INCIDENCE ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; FOCAL DYSCOGNITIVE (COMPLEX PARTIAL) STATUS EPILEPTICUS ; CLINICAL DESCRIPTION ; INCIDENCE ; ETIOLOGY ; DIFFERENTIAL DIAGNOSIS ; TREATMENT ; PROGNOSIS ; SUBTLE STATUS EPILEPTICUS ; FEBRILE STATUS EPILEPTICUS ; NEONATAL STATUS EPILEPTICUS ; EPILEPTIC BRAIN DAMAGE ; SUMMARY AND CONCLUSIONS ; CHAPTER 11 ; CHRONIC BEHAVIORAL DISTURBANCES ; EPIDEMIOLOGY OF P...