Front cover

Pin page

Front Matter

Series page

Neurology: Neonatology Questions and Controversies

Copyright page

Contributors

Series Foreword

Preface

Table of Contents

Chapter 1 Introduction

Chapter 2 Cerebral Circulation and Hypotension in the Premature Infant: Diagnosis and Treatment

Definition of Hypotension

Pathogenesis and Diagnosis of Pathologic Cerebral Blood Flow

Doppler Ultrasound

Impedance Electrical Cardiometry

Near-Infrared Spectroscopy

Amplitude-Integrated EEG (Cerebral Function Monitoring)

Summary

Treatment Strategies

Systemic Hypotension

Treatment of Hypotension Associated with PDA

Treatment of Hypotension Associated with Other Causes

Impact of Provision of Intensive Care on Systemic and Cerebral Hemodynamics

Summary and Recommendations

Diagnosis of Hypotension

Treatment of Hypotension

References

Chapter 3 Intraventricular Hemorrhage and White Matter Injury in the Preterm Infant

Periventricular-Intraventricular Hemorrhage

Background

Neuropathology: Relevance to Clinical Findings

Pathogenesis

Periventricular White Matter Injury Associated with IVH

Clinical Features

Complications

Prevention

Perinatal Strategies

Route of Delivery

Postnatal Strategies

Postnatal Factors Associated with an Increased Risk

Postnatal Administration of Medications to Reduce Severe IVH

White Matter Injury in the Absence of Hemorrhage

Periventricular Leukomalacia

Pathogenesis

Vascular Factors

Intrinsic Vulnerability of the Differentiating Oligodendrocyte

Free Radical Injury

Excitotoxic Injury (Glutamate)

Cytokines

Maternal Fetal Infection and/or Inflammation and White Matter Injury

Clinical Factors Associated with PVL

Prevention

Outcome

Intraventricular Hemorrhage

PVL

Gaps in Knowledge

Conclusions

References

Chapter 4 Posthemorrhagic Hydrocephalus Management Strategies

Question 1: What Measurements of Ventricular Size Are Used in Diagnosis of PHVD?

Question 2: How Can Ventricular Dilation Driven by Cerebrospinal Fluid Under Pressure Be Distinguished from Ventricular Dilation Due to Loss of Periventricular White Matter?

Question 3: How Is Excessive Head Enlargement Defined?

Question 4: How Is Raised Intracranial Pressure Recognized?

Question 5: What Is Infant A’s Prognosis?

Question 6: What Is the Mechanism of PHVD?

Question 7: How Can PHVD Injure White Matter?

Raised Intracranial Pressure, Parenchymal Compression, and Ischemia

Free Radical–Mediated Injury

Proinflammatory Cytokines

Loss of White Matter and Gray Matter

Question 8: What Interventions Have Been Used in PHVD, and Is There Any Evidence That They Improve Outcome?

Ventriculoperitoneal Shunt Surgery

Objectives in Treating PHVD

Repeated Lumbar Punctures or Ventricular Taps

Drug Treatment to Reduce CSF Production

Intraventricular Fibrinolytic Therapy

External Ventricular Drain

Tapping via an Ommaya Reservoir

Third Ventriculostomy

Choroid Plexus Coagulation

Drainage, Irrigation and Fibrinolytic Therapy

Conclusions

Gaps in Knowledge

References

Chapter 5 The Use of Hypothermia to Provide Neuroprotection for Neonatal Hypoxic-Ischemic Brain Injury

Experimental Evidence for the Use of Therapeutic Hypothermia

Animal Work

Clinical Trials

Implementation of a New Therapy

Temperature Control Before and After Therapeutic Hypothermia

Assessment of Encephalopathy

Is an Amplitude-Integrated Electroencephalography Necessary?

Cooling on Transport

Selective Use of Therapeutic Hypothermia

Future of Therapeutic Hypothermia

References

Chapter 6 General Supportive Management of the Term Infant with Neonatal Encephalopathy Following Intrapartum Hypoxia-Ischemia

Delivery Room Management

Early Identification of Infants at Highest Risk for Development of Hypoxic-Ischemic Brain Injury

Supportive Care

Ventilation

Maintenance of Adequate Perfusion

Fluid Status

Control of Blood Glucose Concentration

Temperature

Seizures

Prophylactic Phenobarbital

Potential Neuroprotective Strategies Aimed at Ameliorating Secondary Brain Injury

Oxygen Free Radical Inhibitors and Scavengers

Excitatory Amino Acid Antagonists

Potential Role of Magnesium

Xenon

Erythropoietin

Other Therapies

Gaps in Knowledge

References

Chapter 7 Perinatal Stroke

Case Histories

Definitions

Etiology

Etiology and Risk Factors for Fetal Stroke

Etiology and Risk Factors for Neonatal Stroke

Pathology

Fetal Stroke

Neonatal Stroke

Incidence

Gender Effect in the Incidence of Stroke

Clinical Manifestations

Seizures

Neonatal Encephalopathy

Spastic Hemiplegic Cerebral Palsy

Diagnosis

Role of Ultrasound

Role of Magnetic Resonance Imaging

Role of Placental Pathology

Treatment

Prognosis

Fetal Stroke

Mortality and Long-Term Outcome

Indicators of Long-Term Prognosis

Neonatal Stroke

Mortality

Recurrence

Long-Term Prognosis

Indicators of Long-Term Prognosis

Conclusions

Acknowledgment

References

Chapter 8 Diagnosis and Treatment of Neonatal Seizures

Recognition of Neonatal Seizures

Clinical Seizure Criteria

Subtle Seizure Activity

Clonic Seizures

Multifocal (Fragmentary) Clonic Seizures

Tonic Seizures

Myoclonic Seizures

Nonepileptic Behaviors of Neonates

Tremulousness or Jitteriness with EEG Correlates

Neonatal Myoclonus without EEG Seizures

Neonatal Dystonia without EEG Seizures

Electrographic Seizure Criteria

Ictal EEG Patterns: A More Reliable Marker for Surface-Recorded Seizure Onset, Duration, and Severity

Seizure Duration and Topography

Subcortical Seizures versus Nonictal Functional Decortication

Brainstem Release Phenomena

Electroclinical Dissociation Suggesting Subcortical Seizures

Variation in the Incidence of Neonatal Seizures Based on Clinical versus EEG Criteria

Seizures in the Clinical Context of Maternal-Fetal-Placental-Neonatal Disease: Developing a Diagnostic Algorithm

Clinical Findings

Etiology

Diagnostic Considerations

Principles of Therapy

Emergency Anticonvulsant Drug Treatment

Efficacy of Treatment

Discontinuation of Drug Use

Novel Drug Approaches

Single or Synergistic Treatments for General Neonatal Disease States

Treatments That Alter Neurotransmission

Selective Alteration of a Specific Receptor

Summary

Gaps in Knowledge

References

Chapter 9 Glucose and Perinatal Brain Injury:

Glucose Metabolism in the Fetus and Newborn

Preterm Infants

Intrauterine Growth Restriction

Cerebral Metabolism of Glucose

Alternative Substrates to Glucose

Glucose Transporters

Definitions

Controversy and Question

Symptomatic Versus Asymptomatic Hypoglycemia

Duration of Hypoglycemia

Causes of Hypoglycemia

Incidence

Pathophysiology of Hypoglycemia

Cerebral Blood Flow, Glucose Utilization, and Cerebral Energy Metabolism

Cerebral Biochemical Alterations During Hypoglycemia

Hypoglycemia and Brain Damage

Neuroimaging Abnormalities

Controversy and Question

Hypoglycemia and Hypoxia-Ischemia, Seizures

Outcome

Treatment

Conclusions

References

Chapter 10 Hyperbilirubinemia and the Risk for Brain Injury

Case History

Pathogenesis

Approaches: Strategies for Diagnosis and Treatment

Acute Bilirubin Encephalopathy (Acute Kernicterus)

Diagnosis of Chronic Bilirubin Encephalopathy (Chronic Kernicterus)

Recommendations for Treatment

Gaps in Knowledge

The New Frontier—Bilirubin-Induced Neurologic Disorders in Preterm Infants: Part of a Kernicterus Spectrum Disorder

Acknowledgments

References

Chapter 11 Neonatal Meningitis:

Question 1: What Risk Factors Predispose this Infant to Early-Onset Bacterial Meningitis?

Question 2: Do Infants with Meningitis Have Positive Blood Culture Results?

Question 3: What is the Optimal Evaluation for Possible Late-Onset Sepsis in Preterm Infants in the NICU?

Question 4: What is the Empiric Antimicrobial Choice for Possible Late-Onset Sepsis in the NICU?

Question 5: What is the Treatment of Meningitis in Neonates, and in Particular that due to Gram-Negative Bacilli?

Question 6: Should Other Therapies Be Considered?

Question 7: What is the Duration of Treatment for Meningitis in Neonates?

Question 8: When Should Neuroimaging Be Considered, and What Type of Examination is Recommended?

Question 9: Should Other Adjunctive Therapies Be Provided to an Infant with Meningitis?

Question 10: What If the Infant’s CSF Is Abnormal but Routine Bacterial Cultures of CSF and Blood Are Sterile?

Question 11: What is the Outcome of Meningitis in Neonates?

Conclusions

References

Chapter 12 Neonatal Herpes Simplex Virus and Congenital Cytomegalovirus Infections

Question 1: When Does Infection Occur?

Neonatal HSV Disease

Congenital CMV Infection

Question 2: What Are the Risk Factors for Neonatal Infection?

Neonatal HSV Infection

Congenital CMV Infection

Question 3: What Are the Clinical Manifestations of Neonatal Infection and Disease?

Neonatal HSV Disease

Congenital CMV Infection

Question 4: What Are the Treatments and Outcomes for HSV and CMV Brain Infections in Neonates?

Neonatal HSV Disease

Congenital CMV Infection

Question 5: Do All Babies with HSV and CMV Infections Have to Be Treated?

Neonatal HSV Disease

Congenital CMV Infection

Question 6: What Is the Appropriate Diagnostic Approach to a Baby in Whom HSV or CMV Infection is Suspected?

Neonatal HSV Disease

Congenital CMV Infection

Question 7. How Should You Monitor the Response to Treatment?

Neonatal HSV Disease

Congenital CMV Infection

Question 8. What Are the Biggest Gaps in Our Current Understanding of the Natural History, Diagnosis, and Management of these Infections?

Neonatal HSV Disease

Congenital CMV Infection

Conclusions

References

Chapter 13 Pain and Stress

Pain Circuits

Development of Pain Circuits

Developmental Fine-Tuning of Pain Circuits

Influence of Neurotrophins

Influence of Excitatory and Inhibitory Synaptic Connections

Influence of Descending Pathways

Neonatal Pain Reflex Versus Pain Experience

Long-Term Consequences of Early Pain Exposure

Evidence from Rodent Models

Evidence in Humans

Stress

The Stress Axis

The Stress Response

Development of the Stress Axis

Corticotropin-Releasing Hormone

Arginine Vasopressin

Adrenocorticotropic Hormone

Cortisol

Allostasis

Long-Term Consequences of Early Stress Exposure

Effect of Early Stress on Stress Reactivity

Experimental Observations

Human Observations

Effect of Early Stress on Cognition

Effect of Early Stress on Affect and Behavior

Conclusions

References

Chapter 14 Neonatal Hypotonia and Neuromuscular Disorders

Definition of Hypotonia

Physical Examination and Assessment of a Hypotonic Child

Traction Response

Vertical Suspension

Horizontal Suspension

Differential Anatomic Diagnosis of Hypotonia

Common Neuromuscular Disorders Manifesting Principally with Hypotonia

Anterior Horn Cell and Peripheral Nerve Disorders

Spinal Muscular Atrophies

SMA Type I, or Werdnig-Hoffmann Disease

SMA Types II and III

Congenital Neuropathies

Congenital Hypomyelinating and Axonal Neuropathies

Disturbances of Neuromuscular Transmission

Transient Neonatal Myasthenia Gravis

Acquired Autoimmune Myasthenia Gravis

Congenital Myasthenic Syndromes

Infantile Botulism

Magnesium Intoxication

Muscle Disorders

Congenital Muscular Dystrophies

Congenital Muscular Dystrophies Without Structural CNS Anomalies

Merosin-Deficient Classic Congenital Muscular Dystrophy (MDC1A).

Merosin-Positive Classic Congenital Muscular Dystrophy.

Congenital Muscular Dystrophies with Structural CNS Anomalies and/or Mental Retardation

Fukuyama Muscular Dystrophy.

Walker-Warburg Syndrome.

Muscle-Eye-Brain Disease (Santavuori Congenital Muscular Dystrophy).

Congenital Myotonic Dystrophy

Infantile Facioscapulohumeral Muscular Dystrophy

Congenital Myopathies

Nemaline Myopathy

Central Core Disease

Centronuclear/Myotubular Myopathy

Congenital Fiber Type Disproportion

Minicore Myopathy

Other Congenital Myopathies

Metabolic Myopathies

Acid Maltase Deficiency (Glycogen Storage Disease II)

Infantile Acid Maltase Deficiency (Pompe’s Disease).

Mitochondrial Myopathies

Cytochrome c Oxidase Deficiency

Fatal Infantile Myopathy.

Benign Infantile Myopathy.

Fatty Acid Oxidation Defects

Nonlysosomal Glycogenoses

Phosphorylase Deficiency

Phosphofructokinase Deficiency

Approach to Hypotonia

References

Chapter 15 Amplitude-Integrated EEG and Its Potential Role in Augmenting Management Within the NICU

Amplitude-Integrated EEG

Assessment of aEEG Background Pattern

Comparison with Standard EEG

Background Pattern

Prognostic Value of aEEG in HIE: Noncooled Situation

Prognostic Value of aEEG in HIE: Cooled Situation

aEEG and Seizures

Seizure Detection

Should Subclinical Seizures Be Treated?

aEEG in Preterm Infants

Pitfalls and Artifacts

Seizure-Like Artifacts

aEEG in Other Clinical Conditions

Gaps in Knowledge

Conclusion

References

The Term Newborn

Cranial Ultrasonography for the Evaluation of HIE

MR Techniques in the Evaluation of Perinatal Asphyxia or HIE

Conventional MRI Sequences and Features in HIE

Selective Neuronal Necrosis after Perinatal Asphyxia

Parasagittal Cerebral Injury

Multicystic Encephalomalacia

Diffusion-Weighted Imaging Sequences and Features in HIE

Magnetic Resonance Spectroscopy in HIE

MRI in the Settings of Therapeutic Neonatal Hypothermia

MRI During Hypothermia

MRI Performed after Hypothermia

Focal and Multifocal Ischemic Brain Necrosis without Asphyxia

MR Techniques in the Evaluation of Focal Ischemic Infarction in the Term Newborn

Traumatic Brain Lesions of the Posterior Fossa

MR Techniques in the Evaluation of Traumatic Brain Injury in the Term Newborn

The Preterm Infant

MR Techniques in the Evaluation of the Preterm Infant

Cranial Ultrasonography in the Preterm Infant

Conventional MRI in the Preterm Infant

DWI in the Preterm Infant

MRS in the Preterm Infant

Advanced Quantitative MRI with Image Analysis Tools

Conclusions

Acknowledgement

References

Chapter 17 Long-Term Follow-Up of Very Low-Birth-Weight Infants

Outcome: Cerebral Palsy and Other Neurologic or Sensory Sequelae

The 18- to 24-Month-Old Child

Cerebral Palsy

Visual Assessment

Hearing Assessment

Early Developmental Assessments

Bayley Scales of Infant Development II

Important Lessons for Diagnosis and Intervention

The 3- to 6-Year-Old Child

Important Lessons for Diagnosis and Intervention

The School Age Child

Important Lessons for Diagnosis and Intervention

The Adolescent and Young Adult

Important Lessons for Diagnosis and Intervention

Gaps in Knowledge

Conclusions

References

Index

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D

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H

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K

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M

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O

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U

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X

Y

Z