Epilepsy in Children and Adolescents

David F. Clarke, MD, Dell Children's Comprehensive Epilepsy Program, Austin, TX, USAAmy L. McGregor, MD, University of Tennessee Health Center and Le Bonheur Children’s Hospital, Memphis, TN, USAPhilip L. Pearl, MD, Children's National Medical Center, Washington, DCYu-Tze Ng, MD, University of Oklahoma College of Medicine, Oklahoma City, OK

• List of contributors xiii Preface xviiSection 1 Epidemiology and classification of childhood epilepsies 1Section editor: Phillip L. Pearl1 Epidemiology and common comorbidities of epilepsy in childhood 3Jay Salpekar, Matthew Byrne, and Georgann Ferrone1.1 Epidemiology 31.2 Incidence and prevalence 41.3 Gender and age 41.4 Classification 51.5 Febrile seizures 61.6 Etiology 61.7 Psychiatric comorbidity 71.8 Psychological and psychosocial stress related to chronic disease 71.9 Psychiatric symptoms related to medication side effects 81.10 Psychiatric comorbidity related to epilepsy pathophysiology 81.11 Attention-deficit/hyperactivity disorder (ADHD) 91.12 Anxiety 101.13 Depression 111.14 Intellectual and developmental disabilities (IDD) 121.15 Conclusion 12References 132 Classification and definition of seizures and epilepsy syndromes in childhood 17Susan E. Combs and Phillip L. Pearl2.1 Introduction 172.2 Purpose and goals of definitions and classification 172.3 Systems of classification and definitions 182.4 Seizures 182.5 Generalized seizures 192.6 Focal seizures 222.7 Syndromes 232.8 Specific age-related epilepsy syndromes 252.9 Future directions 34Acknowledgements 34References 343 Initiating and withdrawing medical management 37David T. Hsieh and Bhagwan Indur Moorjani3.1 Initiating medical management 373.2 The chances of seizure recurrence after the first unprovoked seizure 393.3 Seizure recurrence 423.4 The possible adverse effects of seizure recurrence 423.5 The risks of initiating antiepileptic drug therapy 443.6 The benefits of initiating antiepileptic drug therapy 453.7 How to initiate treatment with antiepileptic drugs 453.8 Special circumstances 483.9 Summary: initiating medical management 483.10 Withdrawing medical management 493.11 The long-term prognosis of childhood-onset epilepsy 503.12 When to consider discontinuing antiepileptic drug therapy 513.13 Risk factors for seizure recurrence after discontinuation 513.14 The risks of discontinuing antiepileptic drug therapy 543.15 The benefits of discontinuing antiepileptic drug therapy 553.16 How to discontinue antiepileptic drugs 553.17 Special circumstances 553.18 Summary: withdrawing medical management 563.19 Disclaimer 57References 574 Common genetic and neurocutaneous disorders in childhood epilepsy 59Dewi Frances T. Depositario-Cabacar, William McClintock, and Tom Reehal4.1 Idiopathic epilepsies 604.2 Symptomatic epilepsies 634.3 Epilepsy in common chromosomal abnormalities 634.4 Epilepsy in metabolic and mitochondrial disorders 654.5 Epilepsy in malformations of cortical development 664.6 Neurocutaneous disorders 674.7 Summary 70References 70Section 2 Diagnostic evaluation of childhood epilepsies 73Section editor: David F. Clarke5 Evaluating the child with seizures 75Kristen Park and Susan Koh5.1 Emergent diagnosis and management 765.2 Subsequent evaluation 795.3 Additional neurodiagnostic evaluation 84References 876 The use of EEG in the diagnosis of childhood epilepsy 90David F. Clarke6.1 Technical aspects of the EEG 916.2 Methods used to increase EEG yield 916.3 When should an EEG be ordered? 926.4 EEG findings in epilepsy and epilepsy syndromes 936.5 Neonatal EEGs 946.6 The EEG in focal epilepsy 966.7 The EEG of generalized epilepsy 996.8 Specific disease-related epilepsy syndromes 1046.9 Conclusion 105References 1057 Imaging of pediatric epilepsy 107Asim F. Choudhri7.1 Introduction 1077.2 Imaging considerations 1077.3 Congenital malformations 1177.4 Neoplasms 1247.5 Acquired/idiopathic abnormalities 126References 1278 Non-epileptic paroxysmal events of childhood 129Sucheta M. Joshi8.1 Introduction 1298.2 Breath-holding spells 1308.3 Parasomnias 1318.4 Benign paroxysmal positional vertigo of childhood 1338.5 Syncope 1348.6 Paroxymal non-epileptic events (PNEs) with a psychiatric or behavioral basis 1348.7 Hyperekplexia 1368.8 Alternating hemiplegia of childhood 1368.9 Movement disorders 1378.10 Sandifer syndrome 1388.11 Conclusion 138References 139Section 3 Principles of treatment 143Section editor: James W. Wheless9 Pharmacology of antiepileptic drugs 145James W. Wheless9.1 Pharmacokinetics 1469.2 Pharmacogenomics 155References 15710 Therapeutic efficacy of antiepileptic drugs 159James W. Wheless10.1 Efficacy-based treatment guidelines 16010.2 Antiepileptic drug selection based on specific pediatric epilepsy syndromes 16410.3 Influence of comorbidities in children with epilepsy 17110.4 Conclusions 172References 17211 Adverse effects of antiepileptic drugs 175James W. Wheless11.1 Introduction 17511.2 Specific drugs 17911.3 At-risk profiles and monitoring 189References 19112 Vagus nerve stimulation therapy and epilepsy surgery 193Kate Van Poppel and James W. Wheless12.1 Vagus nerve stimulation 19512.2 Epilepsy surgery 20312.3 Conclusions 215References 21513 Dietary therapies to treat epilepsy 219James W. Wheless13.1 History 22013.2 Efficacy 22113.3 Mechanism of action 22813.4 Selection of candidates for the diet 23213.5 Initiation and maintenance 23413.6 Complications 23613.7 The ketogenic diet in the twenty-first century 239References 239Resources 240Websites 241Section 4 Generalized seizures and generalized epilepsy syndromes 243Section editor: Amy L. McGregor14 Idiopathic generalized epilepsies 245Amy L. McGregor14.1 Clinical features 24614.2 Natural history 24814.3 Genetics 24814.4 Treatment 24814.5 Classification 24914.6 Myoclonic epilepsy in infancy 24914.7 Childhood absence epilepsy (CAE) 25014.8 Juvenile absence epilepsy (JAE) 25214.9 Juvenile myoclonic epilepsy (JME) 25414.10 Epilepsy with generalized tonic-clonic seizures alone (IGE-GTCs) 25614.11 Epilepsy with myoclonic absence 25714.12 Epilepsy with myoclonic-atonic seizures/Doose syndrome 25814.13 Febrile seizures plus (FS+) 25914.14 Eyelid myoclonia with absences (EMA)/Jeavons syndrome 26014.15 Summary 262References 26415 Cryptogenic and symptomatic generalized epilepsies: epilepsies with encephalopathy 267Karen Keough15.1 Neonatal-onset epilepsies with encephalopathy 26815.2 Infantile-onset epilepsies with encephalopathy 27015.3 Epilepsies with encephalopathy with onset later in infancy 27515.4 Epilepsies with encephalopathy with onset after infancy 27715.5 Continuous spike wave of sleep (CSWS) and Landau–Kleffnersyndrome (LKS) 279References 280Section 5 Partial-onset seizures and localization-related epilepsy syndromes 283Section editor: James W. Wheless16 Idiopathic partial epilepsies 285Freedom F. Perkins Jr16.1 Benign infantile seizures 28616.2 Benign childhood epilepsy with centrotemporal spikes 28716.3 Childhood occipital epilepsy (Panayiotopoulos type) 28916.4 Late-onset childhood occipital epilepsy (Gastaut type) 292References 29417 Cryptogenic and symptomatic partial epilepsies 296Stephen Fulton17.1 Etiology 29617.2 Seizure phenomena 29717.3 Temporal lobe epilepsy 29717.4 Extratemporal epilepsy 30317.5 Occipital lobe epilepsy 30617.6 Parietal lobe epilepsy 30717.7 Hypothalamic hamartoma 30717.8 Other localizing and lateralizing signs 308References 309Section 6 Epilepsies relative to age, etiology, or duration 311Section editor: Yu-Tze Ng18 Neonatal seizures 313Eric V. Hastriter18.1 Significance of neonatal seizures 31318.2 Pathophysiology of neonatal seizures 31418.3 Classification and clinical features of neonatal seizures 31618.4 Electrographic seizures 31718.5 Monitoring and recording 31718.6 Etiology of neonatal seizures 32118.7 Metabolic causes for neonatal seizures 32318.8 Inborn errors of metabolism 32318.9 Treatment 32718.10 Chronic postnatal epilepsy and the need for long-term treatment 32818.11 Potential adverse effects of antiepileptic drugs on the immature CNS 32918.12 Conclusion 329References 33019 Febrile seizures 333Marie Francisca Grill19.1 Introduction 33319.2 Definition 33319.3 Incidence and prevalence 33419.4 Pathophysiology 33419.5 Prognosis 33419.6 Initial evaluation and management 33519.7 Long-term management 33819.8 Management in practice 34119.9 Genetics 34219.10 Parent counseling 34319.11 Conclusion 344References 34420 Status epilepticus in childhood 346Yu-Tze Ng and Rama Maganti20.1 Definition 34620.2 Epidemiology 34920.3 Pathophysiology 34920.4 Etiology 35020.5 Diagnosis and investigations 35120.6 EEG patterns in status epilepticus 35220.7 Treatment 35620.8 Prognosis 359References 359Index 365

“In general, I think this book very sound.  It would be an excellent introduction for neurology residents and a good review for general paediatric neurologists who feel a bit out of touch with current treatments for epilepsy.”  (The Canadian Journal of Neurological Sciences, 1 September 2013)“This is an exceptional read, unlike others in the way it addresses the treatment of children with epilepsy, rather than just the treatment of epilepsy.”  (Doody’s, 2 August 2013)