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Neurology: Neonatology Questions and Controversies Series E-Book

Neurology: Neonatology Questions and Controversies Series E-Book

Jeffrey M Perlman

(2012)

Additional Information

Book Details

Abstract

Neurology, a volume in Dr. Polin’s Neonatology: Questions and Controversies Series, offers expert authority on the toughest neurological challenges you face in your practice. This medical reference book will help you provide better evidence-based care and improve patient outcomes with research on the latest advances.

  • Reconsider how you handle difficult practice issues with coverage that addresses these topics head on and offers opinions from the leading experts in the field, supported by evidence whenever possible.
  • Find information quickly and easily with a consistent chapter organization.
  • Get the most authoritative advice available from world-class neonatologists who have the inside track on new trends and developments in neonatal care.

Table of Contents

Section Title Page Action Price
Front Cover cover
Pin page fm2
Half-titile page i
Series page ii
Neurology: Neonatology Questions and Controversies iii
Copyright Page iv
Contributors v
Series Foreword ix
Preface xi
Table Of Contents xiii
1 Introduction 1
2 Cerebral Circulation and Hypotension in the Premature Infant: Diagnosis and Treatment 3
Definition of Hypotension 4
Pathogenesis and Diagnosis of Pathologic Cerebral Blood Flow 8
Doppler Ultrasound 11
Impedance Electrical Cardiometry 12
Near-Infrared Spectroscopy 12
Amplitude-Integrated EEG (Cerebral Function Monitoring) 13
Summary 14
Treatment Strategies 14
Systemic Hypotension 15
Treatment of Hypotension Associated with PDA 16
Treatment of Hypotension Associated with Other Causes 17
Impact of Provision of Intensive Care on Systemic and Cerebral Hemodynamics 18
Summary and Recommendations 19
Diagnosis of Hypotension 19
Treatment of Hypotension 20
References 22
3 Intraventricular Hemorrhage and White Matter Injury in the Preterm Infant 27
Periventricular-Intraventricular Hemorrhage 28
Background 28
Neuropathology: Relevance to Clinical Findings 28
Pathogenesis 28
Periventricular White Matter Injury Associated with IVH 30
Clinical Features 30
Complications 31
Prevention 31
Perinatal Strategies 31
Route of Delivery 31
Postnatal Strategies 32
Postnatal Factors Associated with an Increased Risk 33
Postnatal Administration of Medications to Reduce Severe IVH 33
White Matter Injury in the Absence of Hemorrhage 33
Periventricular Leukomalacia 34
Pathogenesis 35
Vascular Factors 35
Intrinsic Vulnerability of the Differentiating Oligodendrocyte 35
Free Radical Injury 35
Excitotoxic Injury (Glutamate) 36
Cytokines 36
Maternal Fetal Infection and/or Inflammation and White Matter Injury 37
Clinical Factors Associated with PVL 37
Prevention 37
Outcome 38
Intraventricular Hemorrhage 38
PVL 38
Gaps in Knowledge 39
Conclusions 40
References 40
4 Posthemorrhagic Hydrocephalus Management Strategies 47
Question 1: What Measurements of Ventricular Size Are Used in Diagnosis of PHVD? 49
Question 2: How Can Ventricular Dilation Driven by Cerebrospinal Fluid Under Pressure Be Distinguished from Ventricular Dilation Due to Loss of Periventricular White Matter? 50
Question 3: How Is Excessive Head Enlargement Defined? 50
Question 4: How Is Raised Intracranial Pressure Recognized? 50
Question 5: What Is Infant A’s Prognosis? 52
Question 6: What Is the Mechanism of PHVD? 53
Question 7: How Can PHVD Injure White Matter? 53
Raised Intracranial Pressure, Parenchymal Compression, and Ischemia 54
Free Radical–Mediated Injury 55
Proinflammatory Cytokines 55
Loss of White Matter and Gray Matter 55
Question 8: What Interventions Have Been Used in PHVD, and Is There Any Evidence That They Improve Outcome? 55
Ventriculoperitoneal Shunt Surgery 55
Objectives in Treating PHVD 56
Repeated Lumbar Punctures or Ventricular Taps 56
Drug Treatment to Reduce CSF Production 56
Intraventricular Fibrinolytic Therapy 56
External Ventricular Drain 57
Tapping via an Ommaya Reservoir 57
Third Ventriculostomy 58
Choroid Plexus Coagulation 58
Drainage, Irrigation and Fibrinolytic Therapy 58
Conclusions 59
Gaps in Knowledge 60
References 60
5 The Use of Hypothermia to Provide Neuroprotection for Neonatal Hypoxic-Ischemic Brain Injury 63
Experimental Evidence for the Use of Therapeutic Hypothermia 65
Animal Work 65
Clinical Trials 65
Implementation of a New Therapy 67
Temperature Control Before and After Therapeutic Hypothermia 68
Assessment of Encephalopathy 69
Is an Amplitude-Integrated Electroencephalography Necessary? 69
Cooling on Transport 71
Selective Use of Therapeutic Hypothermia 72
Future of Therapeutic Hypothermia 73
References 73
6 General Supportive Management of the Term Infant with Neonatal Encephalopathy Following Intrapartum Hypoxia-Ischemia 77
Delivery Room Management 79
Early Identification of Infants at Highest Risk for Development of Hypoxic-Ischemic Brain Injury 80
Supportive Care 80
Ventilation 81
Maintenance of Adequate Perfusion 81
Fluid Status 82
Control of Blood Glucose Concentration 82
Temperature 82
Seizures 83
Prophylactic Phenobarbital 83
Potential Neuroprotective Strategies Aimed at Ameliorating Secondary Brain Injury 84
Oxygen Free Radical Inhibitors and Scavengers 84
Excitatory Amino Acid Antagonists 85
Potential Role of Magnesium 85
Xenon 85
Erythropoietin 86
Other Therapies 87
Gaps in Knowledge 87
References 87
7 Perinatal Stroke 91
Case Histories 92
Definitions 94
Etiology 96
Etiology and Risk Factors for Fetal Stroke 96
Etiology and Risk Factors for Neonatal Stroke 97
Pathology 99
Fetal Stroke 99
Neonatal Stroke 99
Incidence 99
Gender Effect in the Incidence of Stroke 100
Clinical Manifestations 100
Seizures 100
Neonatal Encephalopathy 101
Spastic Hemiplegic Cerebral Palsy 101
Diagnosis 101
Role of Ultrasound 101
Role of Magnetic Resonance Imaging 102
Role of Placental Pathology 102
Treatment 102
Prognosis 102
Fetal Stroke 102
Mortality and Long-Term Outcome 102
Indicators of Long-Term Prognosis 103
Neonatal Stroke 103
Mortality 103
Recurrence 103
Long-Term Prognosis 103
Indicators of Long-Term Prognosis 104
Conclusions 104
Acknowledgment 105
References 105
8 Diagnosis and Treatment of Neonatal Seizures 109
Recognition of Neonatal Seizures 110
Clinical Seizure Criteria 111
Subtle Seizure Activity 111
Clonic Seizures 113
Multifocal (Fragmentary) Clonic Seizures 116
Tonic Seizures 116
Myoclonic Seizures 118
Nonepileptic Behaviors of Neonates 118
Tremulousness or Jitteriness with EEG Correlates 120
Neonatal Myoclonus without EEG Seizures 120
Neonatal Dystonia without EEG Seizures 120
Electrographic Seizure Criteria 122
Ictal EEG Patterns: A More Reliable Marker for Surface-Recorded Seizure Onset, Duration, and Severity 124
Seizure Duration and Topography 124
Subcortical Seizures versus Nonictal Functional Decortication 124
Brainstem Release Phenomena 125
Electroclinical Dissociation Suggesting Subcortical Seizures 125
Variation in the Incidence of Neonatal Seizures Based on Clinical versus EEG Criteria 127
Seizures in the Clinical Context of Maternal-Fetal-Placental-Neonatal Disease: Developing a Diagnostic Algorithm 128
Clinical Findings 129
Etiology 129
Diagnostic Considerations 130
Principles of Therapy 130
Emergency Anticonvulsant Drug Treatment 131
Efficacy of Treatment 133
Discontinuation of Drug Use 134
Novel Drug Approaches 134
Single or Synergistic Treatments for General Neonatal Disease States 134
Treatments That Alter Neurotransmission 135
Selective Alteration of a Specific Receptor 136
Summary 136
Gaps in Knowledge 137
References 137
9 Glucose and Perinatal Brain Injury: 143
Glucose Metabolism in the Fetus and Newborn 144
Preterm Infants 144
Intrauterine Growth Restriction 145
Cerebral Metabolism of Glucose 145
Alternative Substrates to Glucose 146
Glucose Transporters 146
Definitions 147
Controversy and Question 148
Symptomatic Versus Asymptomatic Hypoglycemia 148
Duration of Hypoglycemia 149
Causes of Hypoglycemia 150
Incidence 150
Pathophysiology of Hypoglycemia 151
Cerebral Blood Flow, Glucose Utilization, and Cerebral Energy Metabolism 151
Cerebral Biochemical Alterations During Hypoglycemia 151
Hypoglycemia and Brain Damage 152
Neuroimaging Abnormalities 153
Controversy and Question 155
Hypoglycemia and Hypoxia-Ischemia, Seizures 155
Outcome 157
Treatment 157
Conclusions 158
References 159
10 Hyperbilirubinemia and the Risk for Brain Injury 163
Case History 163
Pathogenesis 165
Approaches: Strategies for Diagnosis and Treatment 167
Acute Bilirubin Encephalopathy (Acute Kernicterus) 168
Diagnosis of Chronic Bilirubin Encephalopathy (Chronic Kernicterus) 170
Recommendations for Treatment 172
Gaps in Knowledge 174
The New Frontier—Bilirubin-Induced Neurologic Disorders in Preterm Infants: Part of a Kernicterus Spectrum Disorder 176
Acknowledgments 177
References 178
11 Neonatal Meningitis: 181
Question 1: What Risk Factors Predispose this Infant to Early-Onset Bacterial Meningitis? 182
Question 2: Do Infants with Meningitis Have Positive Blood Culture Results? 183
Question 3: What is the Optimal Evaluation for Possible Late-Onset Sepsis in Preterm Infants in the NICU? 185
Question 4: What is the Empiric Antimicrobial Choice for Possible Late-Onset Sepsis in the NICU? 186
Question 5: What is the Treatment of Meningitis in Neonates, and in Particular that due to Gram-Negative Bacilli? 187
Question 6: Should Other Therapies Be Considered? 191
Question 7: What is the Duration of Treatment for Meningitis in Neonates? 193
Question 8: When Should Neuroimaging Be Considered, and What Type of Examination is Recommended? 193
Question 9: Should Other Adjunctive Therapies Be Provided to an Infant with Meningitis? 194
Question 10: What If the Infant’s CSF Is Abnormal but Routine Bacterial Cultures of CSF and Blood Are Sterile? 196
Question 11: What is the Outcome of Meningitis in Neonates? 196
Conclusions 197
References 197
12 Neonatal Herpes Simplex Virus and Congenital Cytomegalovirus Infections 203
Question 1: When Does Infection Occur? 204
Neonatal HSV Disease 204
Congenital CMV Infection 204
Question 2: What Are the Risk Factors for Neonatal Infection? 204
Neonatal HSV Infection 204
Congenital CMV Infection 205
Question 3: What Are the Clinical Manifestations of Neonatal Infection and Disease? 206
Neonatal HSV Disease 206
Congenital CMV Infection 206
Question 4: What Are the Treatments and Outcomes for HSV and CMV Brain Infections in Neonates? 208
Neonatal HSV Disease 208
Congenital CMV Infection 211
Question 5: Do All Babies with HSV and CMV Infections Have to Be Treated? 212
13 Pain and Stress 219
Pain Circuits 220
Development of Pain Circuits 221
Developmental Fine-Tuning of Pain Circuits 221
Influence of Neurotrophins 221
Influence of Excitatory and Inhibitory Synaptic Connections 223
Influence of Descending Pathways 224
Neonatal Pain Reflex Versus Pain Experience 224
Long-Term Consequences of Early Pain Exposure 225
Evidence from Rodent Models 226
Evidence in Humans 226
Stress 227
The Stress Axis 227
The Stress Response 227
Development of the Stress Axis 227
Corticotropin-Releasing Hormone 228
Arginine Vasopressin 228
Adrenocorticotropic Hormone 229
Cortisol 229
Allostasis 230
Long-Term Consequences of Early Stress Exposure 230
Effect of Early Stress on Stress Reactivity 230
Experimental Observations 230
Human Observations 230
Effect of Early Stress on Cognition 231
Effect of Early Stress on Affect and Behavior 231
Conclusions 232
References 232
Sources for Table 13-3 236
14 Neonatal Hypotonia and Neuromuscular Disorders 237
Definition of Hypotonia 237
Physical Examination and Assessment of a Hypotonic Child 237
Traction Response 238
Vertical Suspension 238
Horizontal Suspension 239
Differential Anatomic Diagnosis of Hypotonia 239
Common Neuromuscular Disorders Manifesting Principally with Hypotonia 241
Anterior Horn Cell and Peripheral Nerve Disorders 241
Spinal Muscular Atrophies 241
SMA Type I, or Werdnig-Hoffmann Disease 242
SMA Types II and III 242
Congenital Neuropathies 243
Congenital Hypomyelinating and Axonal Neuropathies 243
Disturbances of Neuromuscular Transmission 243
Transient Neonatal Myasthenia Gravis 243
Acquired Autoimmune Myasthenia Gravis 244
Congenital Myasthenic Syndromes 244
Infantile Botulism 245
Magnesium Intoxication 245
Muscle Disorders 245
Congenital Muscular Dystrophies 245
Congenital Muscular Dystrophies Without Structural CNS Anomalies 246
Merosin-Deficient Classic Congenital Muscular Dystrophy (MDC1A). 246
Merosin-Positive Classic Congenital Muscular Dystrophy. 248
Congenital Muscular Dystrophies with Structural CNS Anomalies and/or Mental Retardation 248
Fukuyama Muscular Dystrophy. 248
Walker-Warburg Syndrome. 249
Muscle-Eye-Brain Disease (Santavuori Congenital Muscular Dystrophy). 249
Congenital Myotonic Dystrophy 249
Infantile Facioscapulohumeral Muscular Dystrophy 250
Congenital Myopathies 251
Nemaline Myopathy 251
Central Core Disease 254
Centronuclear/Myotubular Myopathy 254
Congenital Fiber Type Disproportion 254
Minicore Myopathy 255
Other Congenital Myopathies 255
Metabolic Myopathies 255
Acid Maltase Deficiency (Glycogen Storage Disease II) 255
Infantile Acid Maltase Deficiency (Pompe’s Disease). 255
Mitochondrial Myopathies 256
Cytochrome c Oxidase Deficiency 256
Fatal Infantile Myopathy. 256
Benign Infantile Myopathy. 256
Fatty Acid Oxidation Defects 256
Nonlysosomal Glycogenoses 257
Phosphorylase Deficiency 257
Phosphofructokinase Deficiency 257
Approach to Hypotonia 257
References 258
15 Amplitude-Integrated EEG and Its Potential Role in Augmenting Management Within the NICU 263
Amplitude-Integrated EEG 263
Assessment of aEEG Background Pattern 264
Comparison with Standard EEG 266
Background Pattern 266
Prognostic Value of aEEG in HIE: Noncooled Situation 266
Prognostic Value of aEEG in HIE: Cooled Situation 267
aEEG and Seizures 268
Seizure Detection 268
Should Subclinical Seizures Be Treated? 271
aEEG in Preterm Infants 272
Pitfalls and Artifacts 274
Seizure-Like Artifacts 275
aEEG in Other Clinical Conditions 276
Gaps in Knowledge 281
Conclusion 281
References 281
16 Magnetic Resonance Imaging’s Role in the Care of the Infant at Risk for Brain Injury 285
The Term Newborn 285
Cranial Ultrasonography for the Evaluation of HIE 294
MR Techniques in the Evaluation of Perinatal Asphyxia or HIE 295
Conventional MRI Sequences and Features in HIE 295
Selective Neuronal Necrosis after Perinatal Asphyxia 295
Parasagittal Cerebral Injury 296
Multicystic Encephalomalacia 296
Diffusion-Weighted Imaging Sequences and Features in HIE 296
Magnetic Resonance Spectroscopy in HIE 297
MRI in the Settings of Therapeutic Neonatal Hypothermia 300
MRI During Hypothermia 301
MRI Performed after Hypothermia 301
Focal and Multifocal Ischemic Brain Necrosis without Asphyxia 302
MR Techniques in the Evaluation of Focal Ischemic Infarction in the Term Newborn 305
Traumatic Brain Lesions of the Posterior Fossa 305
MR Techniques in the Evaluation of Traumatic Brain Injury in the Term Newborn 307
The Preterm Infant 307
MR Techniques in the Evaluation of the Preterm Infant 307
Cranial Ultrasonography in the Preterm Infant 313
Conventional MRI in the Preterm Infant 316
DWI in the Preterm Infant 316
MRS in the Preterm Infant 319
Advanced Quantitative MRI with Image Analysis Tools 319
Conclusions 320
Acknowledgement 320
References 320
17 Long-Term Follow-Up of Very Low-Birth-Weight Infants 325
Outcome: Cerebral Palsy and Other Neurologic or Sensory Sequelae 325
The 18- to 24-Month-Old Child 326
Cerebral Palsy 326
Visual Assessment 327
Hearing Assessment 328
Early Developmental Assessments 328
Bayley Scales of Infant Development II 328
Important Lessons for Diagnosis and Intervention 331
The 3- to 6-Year-Old Child 331
Important Lessons for Diagnosis and Intervention 333
The School Age Child 334
Index 341
A 341
B 341
C 342
D 343
E 343
F 343
G 344
H 344
I 345
J 346
K 346
L 346
M 346
N 347
O 347
P 347
R 348
S 348
T 349
U 350
V 350
W 350
X 350
Y 350
Z 350