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Beschreibung

Epilepsy in childhood presents a profound challenge

Epilepsy is an unsettling, complex condition. There is no ‘one size fits all’ option. For effective treatment a full understanding of each patient’s situation and clinical history is needed. Recent remarkable improvements in our ability to image brain structures, to define physiological patterns and in treatment options has made the task of care of the child with epilepsy potentially more effective.

 

Epilepsy in Children and Adolescents provides the contemporary, caring guidance you need to diagnose and manage seizures in a young patient. Beginning with an overview of the classification of epilepsy syndromes, the experienced authors cover:

  • Diagnostic evaluation of childhood epilepsies
  • Principles of treatment
  • Generalized seizures and generalized epilepsy syndromes
  • Partial onset seizures and localization-related epilepsy syndromes
  • Epilepsies relative to age, etiology or duration
  • The full range of treatment options: medical, dietary, surgical

Epilepsy in Children and Adolescents takes a practical approach to a common but complex clinical challenge.

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Contents

Cover

Title Page

Copyright

List of contributors

Preface

Section 1: Epidemiology and classification of childhood epilepsies

Chapter 1: Epidemiology and common comorbidities of epilepsy in childhood

1.1 Epidemiology

1.2 Incidence and prevalence

1.3 Gender and age

1.4 Classification

1.5 Febrile seizures

1.6 Etiology

1.7 Psychiatric comorbidity

1.8 Psychological and psychosocial stress related to chronic disease

1.9 Psychiatric symptoms related to medication side effects

1.10 Psychiatric comorbidity related to epilepsy pathophysiology

1.11 Attention-deficit/hyperactivity disorder (ADHD)

1.12 Anxiety

1.13 Depression

1.14 Intellectual and developmental disabilities (IDD)

1.15 Conclusion

References

Chapter 2: Classification and definition of seizures and epilepsy syndromes in childhood

2.1 Introduction

2.2 Purpose and goals of definitions and classification

2.3 Systems of classification and definitions

2.4 Seizures

2.5 Generalized seizures

2.6 Focal seizures

2.7 Syndromes

2.8 Specific age-related epilepsy syndromes

2.9 Future directions

2.10 Acknowledgements

References

Chapter 3: Initiating and withdrawing medical management

3.1 Initiating medical management

3.2 The chances of seizure recurrence after the first unprovoked seizure

3.3 Seizure recurrence

3.4 The possible adverse effects of seizure recurrence

3.5 The risks of initiating antiepileptic drug therapy

3.6 The benefits of initiating antiepileptic drug therapy

3.7 How to initiate treatment with antiepileptic drugs

3.8 Special circumstances

3.9 Summary: initiating medical management

3.10 Withdrawing medical management

3.11 The long-term prognosis of childhood-onset epilepsy

3.12 When to consider discontinuing antiepileptic drug therapy

3.13 Risk factors for seizure recurrence after discontinuation

3.14 The risks of discontinuing antiepileptic drug therapy

3.15 The benefits of discontinuing antiepileptic drug therapy

3.16 How to discontinue antiepileptic drugs

3.17 Special circumstances

3.18 Summary: withdrawing medical management

3.19 Disclaimer

References

Chapter 4: Common genetic and neurocutaneous disorders in childhood epilepsy

4.1 Idiopathic epilepsies

4.2 Symptomatic epilepsies

4.3 Epilepsy in common chromosomal abnormalities

4.4 Epilepsy in metabolic and mitochondrial disorders

4.5 Epilepsy in malformations of cortical development

4.6 Neurocutaneous disorders

4.7 Summary

References

Section 2: Diagnostic evaluation of childhood epilepsies

Chapter 5: Evaluating the child with seizures

5.1 Emergent diagnosis and management

5.2 Subsequent evaluation

5.3 Additional neurodiagnostic evaluation

References

Chapter 6: The use of EEG in the diagnosis of childhood epilepsy

6.1 Technical aspects of the EEG

6.2 Methods used to increase EEG yield

6.3 When should an EEG be ordered?

6.4 EEG findings in epilepsy and epilepsy syndromes

6.5 Neonatal EEGs

6.6 The EEG in focal epilepsy

6.7 The EEG of generalized epilepsy

6.8 Specific disease-related epilepsy syndromes

6.9 Conclusion

References

Chapter 7: Imaging of pediatric epilepsy

7.1 Introduction

7.2 Imaging considerations

7.3 Congenital malformations

7.4 Neoplasms

7.5 Acquired/idiopathic abnormalities

References

Chapter 8: Non-epileptic paroxysmal events of childhood

8.1 Introduction

8.2 Breath-holding spells

8.3 Parasomnias

8.4 Benign paroxysmal positional vertigo of childhood

8.5 Syncope

8.6 Paroxymal non-epileptic events (PNEs) with a psychiatric or behavioral basis

8.7 Hyperekplexia

8.8 Alternating hemiplegia of childhood

8.9 Movement disorders

8.10 Sandifer syndrome

8.11 Conclusion

References

Section 3: Principles of treatment

Chapter 9: Pharmacology of antiepileptic drugs

9.1 Pharmacokinetics

9.2 Pharmacogenomics

References

Chapter 10: Therapeutic efficacy of antiepileptic drugs

10.1 Efficacy-based treatment guidelines

10.2 Antiepileptic drug selection based on specific pediatric epilepsy syndromes

10.3 Influence of comorbidities in children with epilepsy

10.4 Conclusions

References

Chapter 11: Adverse effects of antiepileptic drugs

11.1 Introduction

11.2 Specific drugs

11.3 At-risk profiles and monitoring

References

Chapter 12: Vagus nerve stimulation therapy and epilepsy surgery

12.1 Vagus nerve stimulation

12.2 Epilepsy surgery

12.3 Conclusions

References

Chapter 13: Dietary therapies to treat epilepsy

13.1 History

13.2 Efficacy

13.3 Mechanism of action

13.4 Selection of candidates for the diet

13.5 Initiation and maintenance

13.6 Complications

13.7 The ketogenic diet in the twenty-first century

References

Resources

Websites

Section 4: Generalized seizures and generalized epilepsy syndromes

Chapter 14: Idiopathic generalized epilepsies

14.1 Clinical features

14.2 Natural history

14.3 Genetics

14.4 Treatment

14.5 Classification

14.6 Myoclonic epilepsy in infancy

14.7 Childhood absence epilepsy (CAE)

14.8 Juvenile absence epilepsy (JAE)

14.9 Juvenile myoclonic epilepsy (JME)

14.10 Epilepsy with generalized tonic-clonic seizures alone (IGE-GTCs)

14.11 Epilepsy with myoclonic absence

14.12 Epilepsy with myoclonic-atonic seizures/Doose syndrome

14.13 Febrile seizures plus (FS+)

14.14 Eyelid myoclonia with absences (EMA)/Jeavons syndrome

14.15 Summary

References

Chapter 15: Cryptogenic and symptomatic generalized epilepsies: epilepsies with encephalopathy

15.1 Neonatal-onset epilepsies with encephalopathy

15.2 Infantile-onset epilepsies with encephalopathy

15.3 Epilepsies with encephalopathy with onset later in infancy

15.4 Epilepsies with encephalopathy with onset after infancy

15.5 Continuous spike wave of sleep (CSWS) and Landau–Kleffner syndrome (LKS)

References

Section 5: Partial-onset seizures and localization-related epilepsy syndromes

Chapter 16: Idiopathic partial epilepsies

16.1 Benign infantile seizures

16.2 Benign childhood epilepsy with centrotemporal spikes

16.3 Childhood occipital epilepsy (Panayiotopoulos type)

16.4 Late-onset childhood occipital epilepsy (Gastaut type)

References

Chapter 17: Cryptogenic and symptomatic partial epilepsies

17.1 Etiology

17.2 Seizure phenomena

17.3 Temporal lobe epilepsy

17.4 Extratemporal epilepsy

17.5 Occipital lobe epilepsy

17.6 Parietal lobe epilepsy

17.7 Hypothalamic hamartoma

17.8 Other localizing and lateralizing signs

References

Section 6: Epilepsies relative to age, etiology, or duration

Chapter 18: Neonatal seizures

18.1 Significance of neonatal seizures

18.2 Pathophysiology of neonatal seizures

18.3 Classification and clinical features of neonatal seizures

18.4 Electrographic seizures

18.5 Monitoring and recording

18.6 Etiology of neonatal seizures

18.7 Metabolic causes for neonatal seizures

18.8 Inborn errors of metabolism

18.9 Treatment

18.10 Chronic postnatal epilepsy and the need for long-term treatment

18.11 Potential adverse effects of antiepileptic drugs on the immature CNS

18.12 Conclusion

References

Chapter 19: Febrile seizures

19.1 Introduction

19.2 Definition

19.3 Incidence and prevalence

19.4 Pathophysiology

19.5 Prognosis

19.6 Initial evaluation and management

19.7 Long-term management

19.8 Management in practice

19.9 Genetics

19.10 Parent counseling

19.11 Conclusion

References

Chapter 20: Status epilepticus in childhood

20.1 Definition

20.2 Epidemiology

20.3 Pathophysiology

20.4 Etiology

20.5 Diagnosis and investigations

20.6 EEG patterns in status epilepticus

20.7 Treatment

20.8 Prognosis

References

Index

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Library of Congress Cataloging-in-Publication Data

Epilepsy in children and adolescents / [edited by] James W. Wheless. p.; cm. Includes bibliographical references and index. ISBN 978-0-470-74123-8 (cloth) I. Wheless, James. [DNLM: 1. Epilepsy. 2. Adolescent. 3. Anticonvulsants–therapeutic use. 4. Child. 5. Epilepsy–therapy. WL 385]616.85′300835–dc23

2012024368

A catalogue record for this book is available from the British Library.

Wiley also publishes its books in a variety of electronic formats. Some content that appears in print may not be available in electronic books.

List of contributors

Matthew Byrne, BA Uniformed Services University of the Health Sciences Bethesda, MD USA

Asim F. Choudhri, MD Departments of Radiology and Neurosurgery University of Tennessee Health Science Center Le Bonheur Children's Hospital Memphis, TN USA

Dave Clarke, MBBS, ABPN (Child Neurology and Sleep), ABCN Dell Children's Comprehensive Epilepsy Program Dell Children's Medical Center of Central Texas Austin, TX USA

Susan E. Combs, MD Department of Neurology Children's National Medical Center The George Washington University School of Medicine Washington, DC USA

Dewi Frances T. Depositario-Cabacar, MD Neurology and Pediatrics George Washington University Medical Center Children's National Medical Center Washington, DC USA

Georgann Ferrone, MD Center for Neuroscience and Behavioral Medicine Children's National Medical Center Washington, DC USA

Stephen Fulton, MD Department of Neurology and Pediatrics University of Tennessee Health Science Center LeBonheur Comprehensive Epilepsy Program Le Bonheur Children's Hospital Memphis, TN USA

Marie Francisca Grill, MD Department of Neurology University of California San Francisco San Francisco General Hospital San Francisco, CA USA

Eric V. Hastriter, MD Mayo Clinic Hospital Phoenix, AZ USA

David T. Hsieh, MD San Antonio Military Medical Center Division of Child Neurology Wilford Hall Medical Center Lackland AFB, TX USA

Sucheta M. Joshi, MD, MS Pediatrics and Communicable Diseases Division of Pediatric Neurology University of Michigan Ann Arbor, MI USA

Karen Keough, MD ‘Specially For Children’ Austin, TX USA

Susan Koh, MD Children's Hospital Colorado Aurora, CO USA

Rama Maganti, MD Department of Neurology Barrow Neurological Institute St Joseph's Hospital and Medical Center Phoenix, AZ USA

William McClintock, MD Neurology and Pediatrics George Washington University Medical Center Children's National Medical Center Washington, DC USA

Amy McGregor, MD Department of Pediatrics Division of Child Neurology Le Bonheur Comprehensive Epilepsy Program University of Tennessee Health Science Center Memphis, TN USA

Bhagwan Indur Moorjani, MD, FAAP, FAAN Hope Neurologic Center, La Quinta JFK Memorial Hospital Indio, CA USA

Yu-tze Ng, MD, FRACP University of Oklahoma Health Science Center and The Children's Hospital Pediatric Neurology Oklahoma city, OK USA

Kristen Park, MD Children's Hospital Colorado Aurora, CO USA

Phillip L. Pearl, MD Department of Neurology Children's National Medical Center The George Washington University School of Medicine Washington, DC USA

Freedom F. Perkins Jr, MD Seton Healthcare and Dell Children's Hospital Austin, TX USA

Tom Reehal, BA University of Sheffield School of Medicine Dentistry and Health Sheffield UK

Jay Salpekar, MD Center for Neuroscience and Behavioral Medicine Children's National Medical Center Washington, DC USA

Kate Van Poppel, MD Department of Pediatric Neurology University of Tennessee Health Science Center Le Bonheur Comprehensive Epilepsy Program and Neuroscience Institute Le Bonheur Children's Hospital Memphis, TN USA

James W. Wheless, MD, FAAP, FACP, FAAN Department of Pediatric Neurology University of Tennessee Health Science Center Le Bonheur Comprehensive Epilepsy Program and Neuroscience Institute Le Bonheur Children's Hospital Memphis, TN USA

Preface

Of all the neurological disorders that affect infants, children, and adolescents, epilepsy is a profound challenge for the patients, caregivers, and physicians and demands expertise to evaluate and treat. As with every illness, gathering a clinical history is an important first step in helping define the problem. However, remarkable improvements in our ability to image brain structures, define physiological patterns, and select medications has made the task of caring for the child with epilepsy more effective than in past years. I envision this book to be a resource for all physicians and other professionals taking care of children with seizures or epilepsy. The goal was for each chapter to be succint, so a physician confronted with a child who has seizures would have an efficient resource for answering questions and designing treatment. I thank the authors for their focus and persistence. I am ever mindful of the patients and their families who bear the challenge of epilepsy with courage. I have learned from them and am keenly aware of our responsibility to do the very best for their care.

James W. WhelessMemphis, TN, USAJune, 2012

Section 1

Epidemiology and classification of childhood epilepsies

Phillip L. Pearl

1

Epidemiology and common comorbidities of epilepsy in childhood

Jay Salpekar1, Matthew Byrne2 and Georgann Ferrone1

1Center for Neuroscience and Behavioral Medicine, Children's National Medical Center, Washington, DC, USA

2Uniformed Services University of the Health Sciences, Bethesda, MD, USA

Epilepsy is a common illness in childhood, and the epidemiology has been well described. However, epilepsy is also complex and controversial in terms of optimal methods for diagnosis and treatment. Classification schemes for seizures have been refined over the years and improved treatment options have allowed better outcomes for children with epilepsy. Understanding of comorbidity, particularly psychiatric comorbidity, has also improved over recent years, yet in many cases it is difficult to resolve whether psychiatric illness is coincidental or associated with the underlying seizure disorder. This chapter addresses the incidence and prevalence of childhood epilepsy and strategies for identifying and managing common psychiatric comorbidities.

1.1 Epidemiology

An epileptic seizure is defined as the clinical manifestation of abnormal or excessive discharge of neurons in the brain [1]. Epilepsy is defined as recurrent seizures, specifically two or more seizures separated by 24 hours but within 18 months of one another [1,2]. This common consensus is based on observations that children who experience one seizure have an approximately 50% chance of recurrence within 2 years [3,4]. It is important to note that febrile seizures are not included in most epidemiological studies of epilepsy.

Population-based studies concerning seizures and epilepsy have been done in numerous communities around the world. Although many international studies of prevalence are based on small communities, the results can be extrapolated to reflect wider regions of the world. In the United States, there are approximately 2.3 million people diagnosed with epilepsy, which reflects an incidence of approximately 1% of the population [5]. The pediatric population, however, has a higher prevalence of epilepsy; 4–10% of children will experience a seizure before the age of 16. Thus, a working knowledge of epilepsy is very important for primary and specialty clinicians in pediatrics, as well as for pediatric neurologists [6].

Terminology review
Incidence: The rate at which new cases of disease occur in a population during a given period of time.
Prevalence: The proportion of a population who have a disease during a given time period.

1.2 Incidence and prevalence

In the general population, the incidence of epilepsy is reported at between 40 and 70 cases per 100 000 [7]. The incidence of childhood epilepsy has been reported to be 82.2 per 100 000 children, markedly higher than that of the overall population [8]. A meta-analysis of over 40 epidemiological studies found that the highest incidence of epilepsy occurs in childhood and in the geriatric population. Interestingly, the incidence of epilepsy has been decreasing over the past 50 years. This decrease in incidence could be explained by more stringent and/or universally followed diagnostic criteria or perhaps from a decrease in exposure to epilepsy risk factors [8].

The overall number of children affected by epilepsy, or the prevalence of the disease, is higher than the incidence because of the chronic nature of epilepsy. A significant variation in prevalence is found in international epidemiology studies [9-12]. In the United States, epilepsy prevalence averages 3.83 per 1000 children, while in northern Tanzania, it is 7.39 per 1000 [13,14]. This discrepancy may result from a variety of factors including possible misclassification of a single seizure as epilepsy. Environmental factors, access to healthcare, and different methods of reporting may also account for some of the variability. The prevalence of epilepsy in varying regions across the world is described in Table 1.1.

Table 1.1 International epidemiology studies.

1.3 Gender and age

Studies have consistently found that males are diagnosed with epilepsy more often than females [18]. While the difference between the genders is slight, this trend holds true for most populations [13]. Although there are exceptions to this trend, they are rarely statistically significant in children [10,11]. Analysis of prevalence among children of varying ages found that epilepsy was most common in children under the age of 5, with a gradual decline in occurrence in older age groups [15]. Figure 1.1 demonstrates the peak of prevalence at a young age and a gradual decrease in children as they age.

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