Title Details

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

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

“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)