Febrile seizures are the most common seizures in infants and children worldwide, This fact provides strong impetus to study and understand them and their consequences, and consider their treatment. These topics were the focus of the first edition of this book.
The 20 years since the publication of this first edition have witnessed an explosion of new information about febrile seizures, meriting this new edition. Key advances have been made in the genetics and neurobiological underpinnings of febrile seizures and especially the very long fever-related seizures called febrile status epilepticus. The role of neuroinflammatory factors in the emergence of these seizures and their consequences, the demonstration of unique clinical and neuroradiological aspects of febrile status epilepticus, and the prospect of predictive (bio)markers to identify and characterize cognitive and epilepsy outcomes are exciting and important. In this edition, the authors and editors tackle these developments in chapters addressing the questions of parents, physicians, allied health care professionals and basic and translational scientists.
Author(s): Tallie Z. Baram, Shlomo Shinnar, Carl E. Stafstrom
Edition: 2
Publisher: Academic Press
Year: 2022
Language: English
Pages: 368
City: London
Front Cover
Febrile Seizures: New Concepts and Consequences
Copyright
Contents
Contributors
Part I Epidemiology of febrile seizures
Chapter 1 The incidence and prevalence of febrile seizures and febrile status epilepticus
Introduction
What is a febrile seizure?
Historical perspective
Definitions of febrile seizures
National Institutes of Health, International League Against Epilepsy, and American Academy of Pediatrics Definitions
Simple and complex febrile seizures
Febrile status epilepticus
Determining febrile seizure incidence and prevalence
Definitions of epidemiological terms
Study designs to determine incidence and prevalence
Case ascertainment: Was it a febrile seizure?
How common are febrile seizures?
Key studies
Geographical variation in febrile seizures
Seasonal variation in febrile seizures
Febrile seizures are slightly more common in boys
Why do we need epidemiological data?
References
Part II Genetic and acquired syndromes associated with febrile seizures
Chapter 2 The evolving genetic landscape of febrile seizures and GEFS +
Background
Genetic approaches used to study the genetics of GEFS +
Single-gene causes of GEFS + ( Table 1)
Genes with limited evidence for link to FS + /GEFS + ( Table 2)
Common genetic risk variants for FS + /GEFS +
Genetic testing in the clinical setting
Summary and future directions
References
Chapter 3 SCN1A and Dravet syndrome
Introduction
Nav1.1 channel dysfunction in DS
The role of SCN1A variants in the genetics of DS
Sodium channel function and dysfunction in neuronal excitability
Effects of temperature on sodium channels and Dravet-associated SCN1A variants
Circuit-level mechanisms of Dravet syndrome
Axonal dysfunction
Synaptic dysfunction
Cell-specific dysfunction
From cells to circuits
Beyond seizures: Broader aspects of the Dravet syndrome phenotype
Summary
Acknowledgments
References
Chapter 4 Ion channels and febrile seizures: It’s not just SCN1A
Introduction
Ion channel genes with a strong association with FS
Scn1a
Scn2a
Scn1b
Hcn
GABA A receptors
Gabra1
Gabrb3
Gabrg2
Ion channel genes with a weak association with FS
Genes other than ion channel genes that modulate neuronal excitability
Potential pathological mechanisms underlying FS
References
Part III Febrile status epilepticus
Chapter 5 Neuroimmunologic aspects of febrile status epilepticus
Neuroinflammation, FSE, and epilepsy
IL-1R/toll-like receptor signaling
Animal models of FSE and the IL-1ß system
Genetic studies supporting the role of inflammation in FSE
Cytokines associated with febrile seizures and FSE in humans
Cytokines as biomarkers of acute hippocampal injury following FSE and epileptogenesis
References
Chapter 6 Febrile status epilepticus and its consequences: Insights from the “Consequences of Febrile Status Epilepticus i ...
Introduction
The FEBSTAT study
The semiology and duration of FSE
FSE and specific organisms
FSE and cognitive outcomes
FSE and risk for subsequent febrile seizures and a second FSE
FSE and the development of epilepsy: potential predictive markers
Summary
References
Chapter 7 The role of febrile seizures in directing surgical therapies for temporal lobe epilepsy
How has epilepsy surgery contributed to the understanding of the mechanisms of febrile seizures and their links with tempor ...
An introduction to epilepsy surgery
Does a history of febrile seizures have any bearing on surgical outcome?
Surgical treatment for MTS
How should we address the gaps in the literature?
Conclusions
References
Chapter 8 Cognitive outcomes of febrile status epilepticus
Early development and overall functioning
Memory
Language
Visuospatial
Attention, working memory, and executive functioning
Achievement and school outcomes
Summary
References
Part IV The neurobiology of FS and FSE: Experimental approaches
Chapter 9 Why do febrile seizures involve specifically the developing brain?
Introduction
Possible mechanisms of febrile seizure
Genetic factors
Role of cytokines
Direct temperature effects
Developmental factors of increased susceptibility to febrile seizures
Neural circuit development
GABAergic neurotransmission
Glutamatergic neurotransmission
Intrinsic neuronal excitability
Conclusions
References
Chapter 10 Contributions of cytokines to febrile seizures
Introduction
Fever and hyperthermia
What are cytokines?
Animal models of febrile seizures
Evidence for brain cytokine involvement in rodent febrile seizure models
Evidence for brain cytokine involvement in febrile seizures in children
Cellular mechanisms underlying cytokine action in the brain
Conclusions and future directions
References
Chapter 11 Experimental models of febrile seizures and febrile status epilepticus
The need for animal models: They provide causality and mechanisms
Considerations and choices of animal models
Choice of species and “background” genetics
Age
Genetic animal models of febrile seizures and related syndromes
Models of simple FS, recurrent FS, FSE, and epileptogenesis
Modes and models for generating experimental FS and FSE
What have the models taught us? What can they not teach us?
References
Part V The neurobiology of FSE-induced epilepsy and cognitive deficits: Experimental approaches
Chapter 12 Febrile status epilepticus-related epilepsy: Neuroinflammation and epigenetics
Introduction: The complex origins of febrile status epilepticus (FSE)
Does FSE lead to epilepsy or adverse cognitive outcomes?
Neuroinflammation is inherent in the generation of fever and febrile seizures
Neuroinflammation is key to the impact of FSE on brain function and hyperexcitability
Several major inflammatory signaling cascades are implicated in epileptogenesis that may follow FSE
microRNAs: A link between neuroinflammation and epigenetics
Neuroinflammatory processes as therapeutic targets for prevention of FSE-related epileptogenesis
Acknowledgments
References
Further reading
Chapter 13 MicroRNAs and epigenetic processes in FSE-provoked epilepsy
Introduction
Epigenetic regulation of brain development
Prolonged febrile seizures and epigenetics
Basic mechanisms of microRNAs
microRNAs as regulators of the developing brain
MicroRNAs and FS
MicroRNA as biomarkers of epileptogenesis
Targeting microRNA and epigenetic factors to prevent epileptogenesis
Conclusion
Acknowledgments
References
Chapter 14 From prolonged febrile seizures to epilepsy: Potential contribution of HCN channels
HCN channels and epilepsy: Experimental evidence
HCN channels and epilepsy: Clinical data in patients
HCN channels and prolonged febrile seizures: Specifics of the developing brain
References
Chapter 15 Cognitive consequences of experimental febrile status epilepticus
Introduction
Spatial cognition and development
Measuring cognition after eFSE
Hippocampal temporal discoordination is induced by eFSE
Biomarkers and potential therapeutic interventions for impaired cognition after FSE
Hyperthermia model of frequent repetitive FS
Future directions
Acknowledgements
Conflicts of interest
References
Part VI Clinical and translational implications of FSE
Chapter 16 MRI for assessing the impact of febrile status epilepticus and predicting outcomes
Introduction
Acute MRI changes following FSE
Trajectories of MRI changes over time
Relationships between MRI abnormalities and cognition
MRI changes and later development of epilepsy
Significance of these studies for the understanding of FSE and hippocampal injury
Implications for management of FSE
References
Chapter 17 EEG for assessing the impact of febrile status epilepticus and predicting outcomes
Introduction
Prior information regarding the role of EEG in assessing the impact of FSE
Acute EEG findings in FEBSTAT
Additional literature
Can acute EEG findings predict long-term outcomes including the development of mesial temporal lobe epilepsy?
Conclusions
References
Part VII Management of febrile seizures and FSE: Past, present, and future
Chapter 18 Evaluation and practical management: Approach to simple and complex febrile seizures
Introduction
Diagnostic evaluation
History and physical examination
Laboratory investigation
Lumbar puncture
Electroencephalogram and imaging
Acute Treatment
Overview
At home
In hospital
Prophylaxis
Overview
Intermittent therapy
Antipyretics
Benzodiazepines
Barbiturates
Continuous prophylactic therapy
Barbiturates
Valproate
Other antiseizure medications
Immunizations
Supportive family management
Conclusion
References
Chapter 19 What do we tell parents of a child with simple or complex febrile seizures?
Introduction
Information
Questions—What do parents want to know?
Content—What parents should be told
What do parents need to know?
Prevention and treatment
Antipyretics
Intermittent benzodiazepines
Benzodiazepines at time of seizure
First aid for febrile seizures
Manner—Why, where, how, when, and by whom it is told
Learning theories
Conclusions
Available resources
References
Chapter 20 The future of FS, FSE, and their epileptogenic and cognitive outcomes
Epidemiology
The genetic revolution and FS
Epileptogenesis: Neurobiological, neuroimmunological, and epigenetic mechanisms
Predictive markers of epileptogenic and cognitive outcomes after FS and FSE
Management implications: Current and future
References
Index
Back Cover
