Epilepsy in Children and Adolescents

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

p>List of contributors xiii Preface xvii Section 1 Epidemiology and classification of childhood epilepsies 1 Section editor: Phillip L. Pearl 1 Epidemiology and common comorbidities of epilepsy in childhood 3 Jay Salpekar, Matthew Byrne, and Georgann Ferrone 1.1 Epidemiology 3 1.2 Incidence and prevalence 4 1.3 Gender and age 4 1.4 Classification 5 1.5 Febrile seizures 6 1.6 Etiology 6 1.7 Psychiatric comorbidity 7 1.8 Psychological and psychosocial stress related to chronic disease 7 1.9 Psychiatric symptoms related to medication side effects 8 1.10 Psychiatric comorbidity related to epilepsy pathophysiology 8 1.11 Attention-deficit/hyperactivity disorder (ADHD) 9 1.12 Anxiety 10 1.13 Depression 11 1.14 Intellectual and developmental disabilities (IDD) 12 1.15 Conclusion 12 References 13 2 Classification and definition of seizures and epilepsy syndromes in childhood 17 Susan E. Combs and Phillip L. Pearl 2.1 Introduction 17 2.2 Purpose and goals of definitions and classification 17 2.3 Systems of classification and definitions 18 2.4 Seizures 18 2.5 Generalized seizures 19 2.6 Focal seizures 22 2.7 Syndromes 23 2.8 Specific age-related epilepsy syndromes 25 2.9 Future directions 34 Acknowledgements 34 References 34 3 Initiating and withdrawing medical management 37 David T. Hsieh and Bhagwan Indur Moorjani 3.1 Initiating medical management 37 3.2 The chances of seizure recurrence after the first unprovoked seizure 39 3.3 Seizure recurrence 42 3.4 The possible adverse effects of seizure recurrence 42 3.5 The risks of initiating antiepileptic drug therapy 44 3.6 The benefits of initiating antiepileptic drug therapy 45 3.7 How to initiate treatment with antiepileptic drugs 45 3.8 Special circumstances 48 3.9 Summary: initiating medical management 48 3.10 Withdrawing medical management 49 3.11 The long-term prognosis of childhood-onset epilepsy 50 3.12 When to consider discontinuing antiepileptic drug therapy 51 3.13 Risk factors for seizure recurrence after discontinuation 51 3.14 The risks of discontinuing antiepileptic drug therapy 54 3.15 The benefits of discontinuing antiepileptic drug therapy 55 3.16 How to discontinue antiepileptic drugs 55 3.17 Special circumstances 55 3.18 Summary: withdrawing medical management 56 3.19 Disclaimer 57 References 57 4 Common genetic and neurocutaneous disorders in childhood epilepsy 59 Dewi Frances T. Depositario-Cabacar, William McClintock, and Tom Reehal 4.1 Idiopathic epilepsies 60 4.2 Symptomatic epilepsies 63 4.3 Epilepsy in common chromosomal abnormalities 63 4.4 Epilepsy in metabolic and mitochondrial disorders 65 4.5 Epilepsy in malformations of cortical development 66 4.6 Neurocutaneous disorders 67 4.7 Summary 70 References 70 Section 2 Diagnostic evaluation of childhood epilepsies 73 Section editor: David F. Clarke 5 Evaluating the child with seizures 75 Kristen Park and Susan Koh 5.1 Emergent diagnosis and management 76 5.2 Subsequent evaluation 79 5.3 Additional neurodiagnostic evaluation 84 References 87 6 The use of EEG in the diagnosis of childhood epilepsy 90 David F. Clarke 6.1 Technical aspects of the EEG 91 6.2 Methods used to increase EEG yield 91 6.3 When should an EEG be ordered? 92 6.4 EEG findings in epilepsy and epilepsy syndromes 93 6.5 Neonatal EEGs 94 6.6 The EEG in focal epilepsy 96 6.7 The EEG of generalized epilepsy 99 6.8 Specific disease-related epilepsy syndromes 104 6.9 Conclusion 105 References 105 7 Imaging of pediatric epilepsy 107 Asim F. Choudhri 7.1 Introduction 107 7.2 Imaging considerations 107 7.3 Congenital malformations 117 7.4 Neoplasms 124 7.5 Acquired/idiopathic abnormalities 126 References 127 8 Non-epileptic paroxysmal events of childhood 129 Sucheta M. Joshi 8.1 Introduction 129 8.2 Breath-holding spells 130 8.3 Parasomnias 131 8.4 Benign paroxysmal positional vertigo of childhood 133 8.5 Syncope 134 8.6 Paroxymal non-epileptic events (PNEs) with a psychiatric or behavioral basis 134 8.7 Hyperekplexia 136 8.8 Alternating hemiplegia of childhood 136 8.9 Movement disorders 137 8.10 Sandifer syndrome 138 8.11 Conclusion 138 References 139 Section 3 Principles of treatment 143 Section editor: James W. Wheless 9 Pharmacology of antiepileptic drugs 145 James W. Wheless 9.1 Pharmacokinetics 146 9.2 Pharmacogenomics 155 References 157 10 Therapeutic efficacy of antiepileptic drugs 159 James W. Wheless 10.1 Efficacy-based treatment guidelines 160 10.2 Antiepileptic drug selection based on specific pediatric epilepsy syndromes 164 10.3 Influence of comorbidities in children with epilepsy 171 10.4 Conclusions 172 References 172 11 Adverse effects of antiepileptic drugs 175 James W. Wheless 11.1 Introduction 175 11.2 Specific drugs 179 11.3 At-risk profiles and monitoring 189 References 191 12 Vagus nerve stimulation therapy and epilepsy surgery 193 Kate Van Poppel and James W. Wheless 12.1 Vagus nerve stimulation 195 12.2 Epilepsy surgery 203 12.3 Conclusions 215 References 215 13 Dietary therapies to treat epilepsy 219 James W. Wheless 13.1 History 220 13.2 Efficacy 221 13.3 Mechanism of action 228 13.4 Selection of candidates for the diet 232 13.5 Initiation and maintenance 234 13.6 Complications 236 13.7 The ketogenic diet in the twenty-first century 239 References 239 Resources 240 Websites 241 Section 4 Generalized seizures and generalized epilepsy syndromes 243 Section editor: Amy L. McGregor 14 Idiopathic generalized epilepsies 245 Amy L. McGregor 14.1 Clinical features 246 14.2 Natural history 248 14.3 Genetics 248 14.4 Treatment 248 14.5 Classification 249 14.6 Myoclonic epilepsy in infancy 249 14.7 Childhood absence epilepsy (CAE) 250 14.8 Juvenile absence epilepsy (JAE) 252 14.9 Juvenile myoclonic epilepsy (JME) 254 14.10 Epilepsy with generalized tonic-clonic seizures alone (IGE-GTCs) 256 14.11 Epilepsy with myoclonic absence 257 14.12 Epilepsy with myoclonic-atonic seizures/Doose syndrome 258 14.13 Febrile seizures plus (FS+) 259 14.14 Eyelid myoclonia with absences (EMA)/Jeavons syndrome 260 14.15 Summary 262 References 264 15 Cryptogenic and symptomatic generalized epilepsies: epilepsies with encephalopathy 267 Karen Keough 15.1 Neonatal-onset epilepsies with encephalopathy 268 15.2 Infantile-onset epilepsies with encephalopathy 270 15.3 Epilepsies with encephalopathy with onset later in infancy 275 15.4 Epilepsies with encephalopathy with onset after infancy 277 15.5 Continuous spike wave of sleep (CSWS) and Landau Kleffner syndrome (LKS) 279 References 280 Section 5 Partial-onset seizures and localization-related epilepsy syndromes 283 Section editor: James W. Wheless 16 Idiopathic partial epilepsies 285 Freedom F. Perkins Jr 16.1 Benign infantile seizures 286 16.2 Benign childhood epilepsy with centrotemporal spikes 287 16.3 Childhood occipital epilepsy (Panayiotopoulos type) 289 16.4 Late-onset childhood occipital epilepsy (Gastaut type) 292 References 294 17 Cryptogenic and symptomatic partial epilepsies 296 Stephen Fulton 17.1 Etiology 296 17.2 Seizure phenomena 297 17.3 Temporal lobe epilepsy 297 17.4 Extratemporal epilepsy 303 17.5 Occipital lobe epilepsy 306 17.6 Parietal lobe epilepsy 307 17.7 Hypothalamic hamartoma 307 17.8 Other localizing and lateralizing signs 308 References 309 Section 6 Epilepsies relative to age, etiology, or duration 311 Section editor: Yu-Tze Ng 18 Neonatal seizures 313 Eric V. Hastriter 18.1 Significance of neonatal seizures 313 18.2 Pathophysiology of neonatal seizures 314 18.3 Classification and clinical features of neonatal seizures 316 18.4 Electrographic seizures 317 18.5 Monitoring and recording 317 18.6 Etiology of neonatal seizures 321 18.7 Metabolic causes for neonatal seizures 323 18.8 Inborn errors of metabolism 323 18.9 Treatment 327 18.10 Chronic postnatal epilepsy and the need for long-term treatment 328 18.11 Potential adverse effects of antiepileptic drugs on the immature CNS 329 18.12 Conclusion 329 References 330 19 Febrile seizures 333 Marie Francisca Grill 19.1 Introduction 333 19.2 Definition 333 19.3 Incidence and prevalence 334 19.4 Pathophysiology 334 19.5 Prognosis 334 19.6 Initial evaluation and management 335 19.7 Long-term management 338 19.8 Management in practice 341 19.9 Genetics 342 19.10 Parent counseling 343 19.11 Conclusion 344 References 344 20 Status epilepticus in childhood 346 Yu-Tze Ng and Rama Maganti 20.1 Definition 346 20.2 Epidemiology 349 20.3 Pathophysiology 349 20.4 Etiology 350 20.5 Diagnosis and investigations 351 20.6 EEG patterns in status epilepticus 352 20.7 Treatment 356 20.8 Prognosis 359 References 359 Index 365