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Acute Encephalopathy and Encephalitis in Infancy and Its Related Disorders

Acute Encephalopathy and Encephalitis in Infancy and Its Related Disorders

Hideo Yamanouchi | Solomon L. Moshé | Akihisa Okumura | Helen Cross | Aristea S Galanopoulou | Russell Dale | Philip Pearl | Masashi Mizuguchi | Federico Vigevano

(2017)

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Book Details

Abstract

Stay current with recent progress in the field of acute encephalopathy and encephalitis in infants with this practical resource by Drs. Hideo Yamanouchi, Solomon L. Moshé, and Akihisa Okumura. This practical resource covers key information relevant to physicians, surgeons, and nurses who often must take prompt action in the everyday clinical care of patients with these disorders.

  • Features a wealth of information for all health care professionals who encounter these complex conditions.
  • Covers diagnostic strategy, subtypes of acute encephalopathy, and management of acute encephalopathy and encephalitis.
  • Consolidates today’s available information and guidance on acute encephalopathy and encephalitis in infancy, in addition to related disorders, into one convenient resource.

Table of Contents

Section Title Page Action Price
Front Cover Cover
Acute Encephalopathy and Encephalitis in Infancy and Its Related Disorders i
Acute Encephalopathy and Encephalitis in Infancy and Its Related Disorders iii
Copyright iv
List of Contributors v
Preface ix
Contents xi
I INTRODUCTION 1
1 - Overview and Definitions 1
DEFINITION 1
CATEGORIES 1
INITIAL STEPS LEADING TO THE FINAL DIAGNOSIS 3
REFERENCES 4
2 - Classification and Epidemiology of Acute Encephalopathy 5
CLASSIFICATION OF ACUTE ENCEPHALOPATHY 5
Classic Reye Syndrome 5
Acute Necrotizing Encephalopathy 7
Acute Encephalopathy With Biphasic Seizures and Late Reduced Diffusion 7
Mild Encephalitis/Encephalopathy With a Reversible Splenial Lesion 8
EPIDEMIOLOGY OF ACUTE ENCEPHALOPATHY 8
REFERENCES 9
II PATHOPHYSIOLOGY 11
3 - Translational Studies of Infantile Epileptic Encephalopathies 11
INTRODUCTION 11
SEIZURE CLASSIFICATION 11
DESIGNING ANIMAL MODELS 11
MECHANISMS OF SEIZURES IN INFANCY 12
ANIMAL MODELS 14
KINDLING 14
STATUS EPILEPTICUS 15
LIPOPOLYSACCHARIDE-ASSOCIATED SEIZURES IN DEVELOPING RATS 16
Febrile Seizures 16
Effects of Lipopolysaccharide-Induced Inflammation on SE 17
LIPOPOLYSACCHARIDE AND INFANTILE SPASMS 17
TREATMENT OF SEIZURES AND STATUS EPILEPTICUS IN THE DEVELOPING BRAIN 17
EFFECTS OF ANTISEIZURE DRUGS ON THE DEVELOPING BRAIN 18
CONCLUSION 18
REFERENCES 19
4 - Ictogenic and Epileptogenic Mechanisms of Neuroinflammation: Insights From Animal Models 23
INTRODUCTION 23
INFLAMMATORY PATHWAYS INVOLVED IN ICTOGENESIS AND EPILEPTOGENESIS 23
MECHANISTIC INSIGHTS 26
CONSEQUENCES OF NEUROINFLAMMATION ON IMMATURE BRAIN EXCITABILITY 26
CONCLUSIONS 27
ACKNOWLEDGMENTS 28
REFERENCES 28
5 - Neuroinflammation in the Pathogenesis of Early Life Epileptic Encephalopathies 33
TYPES AND ETIOLOGIES OF EARLY LIFE EPILEPTIC ENCEPHALOPATHIES 33
ROLE OF INFLAMMATION IN EPILEPTIC ENCEPHALOPATHIES 33
Infantile Spasms/West Syndrome 35
Lennox-Gastaut Syndrome 37
Landau-Kleffner Syndrome/Continuous Spike Waves in Sleep 38
Dravet Syndrome 38
CONCLUSIONS AND FUTURE PERSPECTIVES 39
LIST OF ABBREVIATIONS 39
ACKNOWLEDGMENTS 40
REFERENCES 40
6 - Genetic Background of Encephalopathy 45
INTRODUCTION 45
IMMUNOLOGIC GENETIC BACKGROUND 45
NEURONAL NETWORK GENETIC BACKGROUND 46
METABOLIC GENETIC BACKGROUND 49
CONCLUSIONS 50
ACKNOWLEDGMENTS 51
REFERENCES 51
III DIAGNOSTIC STRATEGY 53
7 - Neuroimaging on Pediatric Encephalopathy in Japan 53
INTRODUCTION 53
ACUTE ENCEPHALOPATHY WITH BIPHASIC SEIZURES AND LATE REDUCED DIFFUSION 53
Clinical Features of Acute Encephalopathy With Biphasic Seizures and Late Reduced Diffusion (Fig. 7.1) 53
Radiologic Features of Acute Encephalopathy With Biphasic Seizures and Late Reduced Diffusion (Box 7.1) 53
CLINICALLY MILD ENCEPHALITIS/ENCEPHALOPATHY WITH A REVERSIBLE SPLENIAL LESION 57
Clinical Features of Clinically Mild Encephalitis/Encephalopathy With a Reversible Splenial Lesion 57
Radiologic Features of Clinically Mild Encephalitis/Encephalopathy With a Reversible Splenial Lesion (Box 7.1) 57
Hypothesis for the Pathogenesis of Clinically Mild Encephalitis/Encephalopathy With a Reversible Splenial Lesion 58
RADIOLOGIC FEATURES OF ACUTE NECROTIZING ENCEPHALOPATHY (BOX 7.1) 60
ABBREVIATIONS 60
REFERENCES 62
8 - Electroencephalography in Children With Acute Encephalitis/Encephalopathy 63
INTRODUCTION 63
CONVENTIONAL ELECTROENCEPHALOGRAPHY IN CHILDREN WITH ACUTE ENCEPHALOPATHY 63
Generalized Slowing 63
Unilateral or Focal Slowing 63
Low Voltage 64
Abnormalities in Fast Activities 65
Increased fast activities 65
Decreased fast activities 65
Periodic Discharges 65
Paroxysmal Discharges 65
Episodic Transients 66
Ictal Changes 66
CONTINUOUS ELECTROENCEPHALOGRAPHY MONITORING IN CHILDREN 67
QUANTITATIVE ELECTROENCEPHALOGRAPHY IN CHILDREN 68
FUTURE DIRECTIONS 69
REFERENCES 69
9 - Electroencephalographic Approach for Early Diagnosis of Acute Encephalopathy and Encephalitis 71
BACKGROUND 71
ELECTROENCEPHALOGRAPHY FINDINGS OF PEDIATRIC ACUTE ENCEPHALITIS AND ENCEPHALOPATHY 71
General Electroencephalography Findings During Acute Phase of Pediatric Acute Encephalitis 71
General Electroencephalography Findings During Acute Phase of Pediatric Acute Encephalopathy 72
ELECTROENCEPHALOGRAPHY FINDINGS DURING ACUTE PHASE IN FEBRILE COMATOSE CHILDREN 72
CHILDREN Case 72
Discussion of Case Presentation 72
SUMMARY 75
REFERENCES 75
10 - Biomarkers for the Diagnosis and Evaluation in Acute Encephalopathy 77
INTRODUCTION 77
ACUTE ENCEPHALOPATHY DUE TO CYTOKINE STORM 77
ACUTE ENCEPHALOPATHY DUE TO EXCITOTOXICITY 77
REFERENCES 78
11 - Differential Diagnosis 81
INTRODUCTION 81
DEFINITIONS 81
DIAGNOSTIC TOOLS 81
Clinical Features 82
Seizures and Electroencephalogram Features 83
Laboratory Features 83
Radiologic Features 83
DIAGNOSTIC ALGORITHM 84
REFERENCES 85
IV SUBTYPES OF ACUTE ENCEPHALOPATHY 87
12 - Acute Necrotizing Encephalopathy 87
NOSOLOGY 87
EPIDEMIOLOGY 87
CLINICAL AND LABORATORY FINDINGS 89
RADIOLOGIC AND PATHOLOGIC FINDINGS 90
PATHOGENESIS 90
GENETIC BACKGROUND 91
DIAGNOSIS 91
TREATMENT 91
REFERENCES 91
13 - Acute Encephalopathy With Febrile Convulsive Status Epilepticus (AEFCSE) 93
INTRODUCTION 93
ACUTE ENCEPHALOPATHY WITH FEBRILE CONVULSIVE STATUS EPILEPTICUS 93
Theory 93
Examination of Cases Hospitalized at the Osaka City General Hospital, Japan12 94
Pathogenesis of Acute Encephalopathy With Febrile Convulsive Status Epilepticus 96
DIAGNOSTIC CRITERIA OF ACUTE ENCEPHALOPATHY WITH FEBRILE CONVULSIVE STATUS EPILEPTICUS (ACUTE ENCEPHALOPATHY WITH BIPHASIC SEIZURES AND LATE REDUCED DIFFUSION)* 96
DISCUSSION 97
Preventing Acute Encephalopathy With Febrile Convulsive Status Epilepticus 97
Treatment of Acute Encephalopathy With Febrile Convulsive Status Epilepticus 97
CONCLUSION 97
REFERENCES 97
14 - Acute Mitochondrial Encephalopathy 99
MITOCHONDRIAL DISEASES AND ENCEPHALOPATHY 99
LEIGH SYNDROME/ENCEPHALOPATHY 99
Case Presentation 100
MITOCHONDRIAL MYOPATHY, ENCEPHALOPATHY, LACTIC ACIDOSIS, AND STROKELIKE EPISODES 102
Case Presentation 102
THERAPY FOR MITOCHONDRIAL ENCEPHALOPATHY 103
CONCLUSION 104
REFERENCES 104
15 - Genetic-Metabolic Disorders Presenting as Acute, but Reversible, Severe Epilepsies 105
INTRODUCTION 105
VITAMIN-RESPONSIVE EPILEPSIES 105
Vitamin B6–Related Disorders 105
Biotinidase Deficiency 107
Cobalamin Deficiency 107
TRANSPORTOPATHIES 109
Glucose Transporter 1 Deficiency 109
Cerebral Folate Deficiency 109
Biotin-Thiamine–Responsive Basal Ganglia Disease 110
AMINO AND ORGANIC ACIDOPATHIES 110
Serine Biosynthesis Disorder 110
Creatine Synthesis Disorder 110
Molybdenum Cofactor Deficiency 110
NEUROTRANSMITTER DEFECTS 111
DISORDERS OF GLUCOSE HOMEOSTASIS 112
CONCLUSION 114
REFERENCES 114
16 - Acute Encephalopathy in Infants With Sulfite Oxidase Deficiency and Molybdenum Cofactor Deficiency 117
INTRODUCTION 117
NEUROPATHOGENESIS 117
CLINICAL MANIFESTATIONS 118
NEUROIMAGING FEATURES 119
LABORATORY FINDINGS 119
TREATMENT 120
CONCLUSION 121
DECLARATION OF CONFLICTING INTERESTS 121
REFERENCES 121
V SUBTYPES OF ACUTE ENCEPHALITIS 123
17 - Autoimmune Encephalitis: Overview of Clinical Recognition, Autoantibody Diagnostic Markers, and Treatment of Autoimmune Encephalitis 123
BACKGROUND: BRAIN INFLAMMATION AND ENCEPHALITIS 123
CLINICAL SYNDROMES 123
CELL SURFACE AUTOANTIBODIES 124
ANTI–N-METHYL-D-ASPARTATE RECEPTOR ENCEPHALITIS 124
ANTI–MYELIN OLIGODENDROCYTE GLYCOPROTEIN ANTIBODY–ASSOCIATED DEMYELINATION 126
ANTIGLYCINE RECEPTOR ANTIBODY–ASSOCIATED ENCEPHALITIS 127
ANTI–Γ-AMINOBUTYRIC ACID-A RECEPTOR ANTIBODY–ASSOCIATED ENCEPHALITIS 127
BASAL GANGLIA ENCEPHALITIS 127
ANTI–GLUTAMIC ACID DECARBOXYLASE ANTIBODY–ASSOCIATED ENCEPHALITIS 127
OTHER RARE SYNDROMES 128
RELAPSING COURSE 128
CLINICAL CRITERIA 128
SERONEGATIVE SUSPECTED AUTOIMMUNE ENCEPHALITIS 128
THERAPEUTICS 129
FUTURE PRIORITIES 130
18 - Acute Disseminated Encephalomyelitis 133
INTRODUCTION 133
DEFINITIONS 133
EPIDEMIOLOGY 134
PATHOPHYSIOLOGY 134
CLINICAL FEATURES 135
NEUROIMAGING 135
CEREBROSPINAL FLUID ABNORMALITIES 137
ANTI–MYELIN OLIGODENDROCYTE PROTEIN ANTIBODIES 137
DIFFERENTIAL DIAGNOSIS 138
TREATMENT 138
OUTCOME 139
RELAPSES 139
CONCLUSION 139
REFERENCES 140
19 - Epidemiology of Acute Disseminated Encephalomyelitis 143
INTRODUCTION 143
DEFINITION OF ACUTE DISSEMINATED ENCEPHALOMYELITIS 143
International Pediatric Multiple Sclerosis Study Group Definition 143
Brighton Collaboration Case Definition 143
Subtypes of Acute Disseminated Encephalomyelitis by Antecedent Events 144
EPIDEMIOLOGY OF ACUTE DISSEMINATED ENCEPHALOMYELITIS 144
Incidence of Pediatric Acute Disseminated Encephalomyelitis 144
Incidence of Adult Acute Disseminated Encephalomyelitis 145
Ethnicity 145
Sex 145
Age 145
Infection 146
Vaccination 146
Geography 146
Genetics 147
Other Factors 147
VACCINATION AND ACUTE DISSEMINATED ENCEPHALOMYELITIS 147
LIMITATIONS IN ACUTE DISSEMINATED ENCEPHALOMYELITIS EPIDEMIOLOGIC STUDIES 147
Epidemiologic Survey of Pediatric Diseases in Japan 148
REFERENCES 148
20 - Autoimmune-Mediated Encephalitis With Antibodies to NMDA-Type GluRs: Early Clinical Diagnosis 151
INTRODUCTION 151
PATIENTS AND METHODS 152
Definition of Clinical Stages 152
Inclusion and Exclusion Criteria of Nonherpetic Acute Limbic Encephalitis Patients 152
Healthy Controls and Disease Controls of Infection for Evaluation of Blood Data in the Preceding Stage 152
Disease Controls and Disease Controls of Aseptic Meningitis for Evaluation of Cerebrospinal Fluid Data in the Preceding Stage 152
Study for the Prodromal Stage 153
Study for the Preceding Stage 153
Statistics 153
RESULTS 153
History of Nonherpetic Acute Limbic Encephalitis Patients 153
Preceding Symptoms of Nonherpetic Acute Limbic Encephalitis Patients 154
Blood Data in the Preceding Stage of Nonherpetic Acute Limbic Encephalitis Patients 154
Cerebrospinal Fluid Data in the Preceding Stage of Nonherpetic Acute Limbic Encephalitis Patients 155
DISCUSSION 155
CONCLUSIONS 156
ACKNOWLEDGMENTS 156
REFERENCES 156
21 - Clinical Features of HHV-6B Encephalitis: Difference Between Primary Infection and Reactivation 157
VIROLOGIC INFORMATION OF HUMAN HERPESVIRUS 6B 157
NEUROVIRULENCE OF HUMAN HERPESVIRUS 6B 158
HUMAN HERPESVIRUS 6B ENCEPHALITIS AT THE TIME OF PRIMARY INFECTION 158
POSTTRANSPLANT HUMAN HERPESVIRUS 6B ENCEPHALITIS 159
CONCLUSION 159
REFERENCES 160
22 - Bacterial, Fungal, and Parasitic Encephalitis 163
INTRODUCTION 163
BACTERIAL INFECTIONS 163
Scrub Typhus 163
Clinical features 163
Diagnosis 164
Treatment 164
Future directions 164
Shigella 164
VI ENCEPHALOPATHY / ENCEPHALITIS WITH REFRACTORY EPILEPTIC STATUS: RIMA NABBOUT 175
23 - Febrile Infection–Related Epilepsy Syndrome 175
INTRODUCTION 175
PATHOGENESIS OF FEBRILE INFECTION–RELATED EPILEPSY SYNDROME 175
CLINICAL PRESENTATION 176
INSTRUMENTAL AND LABORATORY INVESTIGATION 176
DIAGNOSIS AND DIFFERENTIAL DIAGNOSES 177
TREATMENT (SEE TABLE 23.1) 177
PROGNOSIS 177
REFERENCES 179
24 - Acute Encephalitis With Refractory, Repetitive Partial Seizures 181
INTRODUCTION 181
HISTORY 181
CLINICAL CHARACTERISTICS 181
EXAMINATION FINDINGS 182
Examination of Cerebrospinal Fluid 182
Electroencephalographic Examination 182
Neuroimaging Diagnosis 182
ARE ACUTE ENCEPHALITIS WITH REFRACTORY, REPETITIVE PARTIAL SEIZURES AND FEBRILE INFECTION–RELATED REFRACTORY EPILEPSY SYNDROME THE SAME DISEASE? 183
ETIOLOGY 183
Epilepsy Hypothesis 183
Inflammation Hypothesis 183
TREATMENT 184
REFERENCES 184
25 - Children With Encephalitis/Encephalopathy-Related Status Epilepticus and Epilepsy—A Global View of Postencephalitic Epilepsy 187
INTRODUCTION 187
ACUTE SYMPTOMATIC SEIZURES IN THE ACUTE STAGE OF ENCEPHALITIS 187
HOW TO APPROACH A PATIENT WITH FEBRILE STATUS EPILEPTICUS—ROLE OF INFECTIOUS PATHOGENS AND ANTINEURONAL ANTIBODIES IN CHILDREN W... 188
Detection of the Underlying Etiology 188
Coexistence of Infectious Pathogens and Antineuronal Antibodies in a Patient With Encephalitis and Status Epilepticus 188
RISK FACTORS FOR DEVELOPING POSTENCEPHALITIC EPILEPSY 190
MULTIMODAL MANAGEMENT OF POSTENCEPHALITIC EPILEPSY 191
Antiepileptic Drugs for Acute Seizures and Chronic Seizures of Postencephalitic Epilepsy 191
Immunotherapy for Seizures With Encephalitis 191
OUTCOMES OF POSTENCEPHALITIC EPILEPSY 191
ACKNOWLEDGMENTS 192
REFERENCES 192
VII MANAGEMENT OF ACUTE ENCEPHALOPATHYAND ENCEPHALITIS 195
26 - Intensive Care Management of Acute Encephalopathy and Encephalitis 195
EVALUATION AND DIAGNOSIS 195
Cerebrospinal Fluid Analysis 195
Neuroimaging 197
Electroencephalography 198
IMMEDIATE STABILIZATION 198
Airway and Breathing 198
Hemodynamic Stabilization 199
Addressing Correctable Factors 199
SPECIFIC THERAPY 200
Antimicrobial Therapy 200
Other Treatable Conditions 200
ADJUNCTIVE THERAPY AND SUPPORTIVE CARE 200
Corticosteroids 200
Nursing Care 200
Positioning of comatose patients 203
Eye care 203
Sedation, Analgesia, and Paralysis 203
Fluid, Electrolytes, and Nutrition 203
Seizures and Status Epilepticus 204
Raised Intracranial Pressure 204
Intracranial pressure monitoring 204
General measures 206
Hyperosmolar therapy 206
Hyperventilation 206
Barbiturate coma 206
Cerebrospinal fluid drainage 206
Decompressive craniectomy 207
Mechanical Ventilation 207
Isolation Precautions 207
Family Support 207
OUTCOME 207
REFERENCES 208
27 - Strategy for the Treatment of Intractable Epilepsy Secondary to Acute Encephalopathy and Encephalitis 211
HAVE WE USED THE CORRECT DRUG? 211
WHAT DO WE MEAN BY DRUG RESISTANCE? 212
SHOULD ALTERNATIVE THERAPY BE CONSIDERED? 212
TREATING THE UNDERLYING CAUSE 212
TREATMENTS ON THE HORIZON 213
ARE SEIZURES THE ONLY CONCERN? 213
REFERENCES 213
28 - The Pros and Cons of Therapeutic Hypothermia for Acute Encephalitis and Refractory Status Epilepticus 215
INTRODUCTION OF ACUTE ENCEPHALITIS AND REFRACTORY STATUS EPILEPTICUS 215
MULTIPLE MECHANISMS OF TARGETED TEMPERATURE MANAGEMENT 215
CLINICAL APPLICATIONS OF TARGETED TEMPERATURE MANAGEMENT FOR ACUTE ENCEPHALITIS AND REFRACTORY STATUS EPILEPTICUS 215
Targeted Temperature Management for Acute Encephalitis 215
Targeted Temperature Management for Refractory Status Epilepticus 216
PROS AND CONS 216
Pros 216
Cons 217
CONCLUSION 218
ACKNOWLEDGMENT 218
REFERENCES 218
29 - Therapeutic Hypothermia as Potential Intervention on Acute Encephalopathy 219
INTRODUCTION 219
HISTORY AND EXPERIMENTAL EVIDENCE 219
GENERAL PRACTICE FOR BRAIN HYPOTHERMIC THERAPY 219
CONCLUSION 220
REFERENCES 221
30 - Neurosurgical Interventions for Encephalitis-Related Seizures and Epilepsy 223
INTRODUCTION 223
NEUROSURGICAL INTERVENTIONS FOR ACUTE ENCEPHALITIS AND STATUS EPILEPTICUS 223
NEUROSURGICAL INTERVENTIONS FOR POSTENCEPHALITIC EPILEPSY 224
RASMUSSEN ENCEPHALITIS 225
Pathological and Clinical Background Targeted by Current Medical Treatments 225
Neurosurgical Interventions for Rasmussen Encephalitis 225
CONCLUSIONS AND FUTURE DIRECTIONS 226
ACKNOWLEDGMENTS 226
REFERENCES 226
31 - Rehabilitation After Acute Encephalopathy in Childhood 229
OVERVIEW 229
ACUTE ENCEPHALOPATHY WITH BIPHASIC SEIZURES AND LATE REDUCED DIFFUSION 229
HEMORRHAGIC SHOCK AND ENCEPHALOPATHY 230
HYPOXIC ISCHEMIC ENCEPHALOPATHY 231
SUMMARY 232
REFERENCES 232
Index 233
A 233
B 235
C 235
D 236
E 237
F 237
G 238
H 238
I 239
J 240
K 240
L 240
M 240
N 241
O 241
P 242
Q 243
R 243
S 243
T 243
U 244
V 244
W 244
X 244
Z 244