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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
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
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
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
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
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