BOOK
Fetal and Neonatal Neurology and Neurosurgery E-Book
Malcolm I. Levene | Frank A. Chervenak
(2014)
Additional Information
Book Details
Abstract
This book provides a definitive reference work on the developing brain, from conception through the first year of life. Its purpose is to provide the range of specialists involved in the management of the fetus and the neonate with the latest information on the developmental neurology and pathology of the developing central nervous system, so that they can provide prompt and informed treatment of neurological disability – the most feared complication of pregnancy and the early months of the life of the newborn child.
- A cross-specialty book: brings together the work of specialists on the immature brain from the fields of obstetrics, paediatrics and neurosurgery.
- An international book: edited and written by an international team of experts.
- Highly illustrated: includes over 800 images and line drawings that capture the latest investigative techniques.
- Focused on treatment: key points boxes pick out the most important information for the busy specialist.
- Evidence-based information: extensively referenced with the latest and most important papers and articles.
- Includes the latest ultrasound techniques – to reflect the massive improvements since the last edition.
- Medico-legal issues highlighted – in recognition of the role of specialists as expert witnesses.
- Covers the latest MR techniques: fast sequences for fetal brain imaging, DWI, tractography.
Table of Contents
Section Title | Page | Action | Price |
---|---|---|---|
Front Cover | Cover | ||
Fetal and Neonatal Neurology and Neurosurgery | iii | ||
Copyright Page | iv | ||
Contents | v | ||
Preface to the fourth edition | viii | ||
Preface to the first edition | ix | ||
Acknowledgments | x | ||
List of contributors | xi | ||
SECTION I: STRUCTURAL DEVELOPMENT OF THE CNS | 1 | ||
CHAPTER 1. The molecular basis of brain development | 1 | ||
INDUCTION AND PATTERNING OF THE FETAL HUMAN BRAIN | 1 | ||
ORGANIZATION OF THE BRAIN | 9 | ||
SUMMARY | 10 | ||
REFERENCES | 11 | ||
CHAPTER 2. Early embryonic development of the brain | 12 | ||
INTRODUCTION | 12 | ||
ORGANOGENESIS | 12 | ||
FUTURE PROSPECTS | 19 | ||
REFERENCES | 19 | ||
CHAPTER 3. Development of consciousness: fetal, neonatal and maternal interactions | 21 | ||
NEURONS: THE ATOMS OF CONSCIOUSNESS | 21 | ||
THE LOCALIZATION OF CONSCIOUSNESS | 21 | ||
DEVELOPMENTAL ANATOMY OF CONSCIOUSNESS | 21 | ||
THE NEUROCHEMISTRY OF CONSCIOUSNESS | 23 | ||
METHODS TO STUDY CONSCIOUSNESS IN THE FETUS AND THE INFANT | 23 | ||
COMPONENTS OF CONSCIOUSNESS | 24 | ||
INTEGRATION OF THE COMPONENTS | 26 | ||
MATERNAL INTERACTION | 26 | ||
MINIMAL CONSCIOUSNESS | 26 | ||
ETHICAL CONSIDERATIONS | 27 | ||
REFERENCES | 28 | ||
CHAPTER 4. Ultrasound assessment of normal fetal brain development | 29 | ||
INTRODUCTION | 29 | ||
FIRST TRIMESTER, <14 WEEKS | 30 | ||
SECOND TRIMESTER | 36 | ||
THIRD TRIMESTER | 40 | ||
VASCULATURE OF THE BRAIN | 41 | ||
CONCLUSION | 42 | ||
REFERENCES | 42 | ||
CHAPTER 5. Magnetic resonance imaging of the fetal central nervous system | 45 | ||
INTRODUCTION | 45 | ||
THE SEQUENCES | 45 | ||
SAFETY | 45 | ||
THE TECHNIQUE | 46 | ||
CLINICAL APPLICATIONS | 47 | ||
NORMAL BRAIN DEVELOPMENT | 48 | ||
POSTERIOR FOSSA | 51 | ||
FETAL MR MEASUREMENTS | 52 | ||
MR SPECTROSCOPY | 53 | ||
DIFFUSION-WEIGHTED IMAGING (DWI) | 53 | ||
PATHOLOGIES | 53 | ||
CONCLUSION | 65 | ||
REFERENCES | 65 | ||
CHAPTER 6. Imaging of the neonatal brain | 68 | ||
INTRODUCTION | 68 | ||
IMAGING MODALITIES | 68 | ||
NORMAL ANATOMY AND BRAIN DEVELOPMENT | 72 | ||
RADIOGRAPHIC PATHOLOGY | 81 | ||
WHITE MATTER DISEASE | 86 | ||
REFERENCES | 98 | ||
SECTION II: FUNCTIONAL ASSESSMENT OF CNS DEVELOPMENT | 103 | ||
CHAPTER 7. Functional assessment of the fetal CNS | 103 | ||
INTRODUCTION | 103 | ||
FETAL BODY MOVEMENTS | 103 | ||
FETAL HEART RATE | 104 | ||
FETAL BREATHING MOVEMENTS AND HICCUPS | 106 | ||
FETAL EYE MOVEMENTS | 107 | ||
FETAL MOUTH MOVEMENTS | 107 | ||
FETAL BEHAVIORAL STATES | 107 | ||
TWINS | 108 | ||
FETAL NEUROLOGY | 109 | ||
SUMMARY: CLINICAL RELEVANCE OF FETAL BEHAVIOR | 109 | ||
REFERENCES | 109 | ||
CHAPTER 8. The development of senses | 111 | ||
INTRODUCTION | 111 | ||
SENSITIVITY AND EXPERIENCE OF PAIN | 112 | ||
HEARING | 115 | ||
OLFACTION AND TASTE | 120 | ||
VISION | 122 | ||
CONCLUSIONS | 124 | ||
ACKNOWLEDGEMENTS | 125 | ||
REFERENCES | 125 | ||
CHAPTER 9. Clinical assessment of the infant nervous system | 128 | ||
INTRODUCTION | 128 | ||
VARIOUS ORIGINS, VARIOUS INSTRUMENTS | 128 | ||
EARLY CNS FUNCTIONS: CONCEPTUAL FRAMEWORK | 130 | ||
METHODOLOGICAL REPERTOIRE FOR A BASIC NEUROLOGICAL ASSESSMENT | 131 | ||
ADAPTATION OF THE ASSESSMENT TO THE PRETERM PERIOD | 142 | ||
APPLICATION TO THE TERM PERIOD (FULL-TERM OR 40 WEEKS CORRECTED) | 143 | ||
CONTRIBUTION OF CLINICAL FINDINGS TO IDENTIFICATION OF THE MOST COMMON DISORDERS | 144 | ||
APPLICATION TO THE RECOVERY PERIOD | 145 | ||
CONCLUDING COMMENTS | 147 | ||
REFERENCES | 147 | ||
APPENDIX 1. Amiel-Tison neurological assessment at term | 149 | ||
INSTRUCTIONS | 149 | ||
CHAPTER 10. Perinatal cerebral circulation | 155 | ||
BASIC PHYSIOLOGY AND HISTORIC STEPS IN THE DETECTION OF FETAL CEREBRAL BLOOD FLOW | 155 | ||
ANATOMY OF BRAIN CIRCULATION | 156 | ||
FETAL CEREBRAL BLOOD FLOW DISTRIBUTION UNDER PHYSIOLOGIC CONDITIONS | 160 | ||
BRAIN BLOOD FLOW DISTRIBUTION UNDER PATHOLOGIC CONDITIONS | 162 | ||
CHANGES FROM PRENATAL TO POSNATAL LIFE | 165 | ||
CEREBRAL CIRCULATION AND OUTCOME | 166 | ||
CONCLUSIONS | 167 | ||
ACKNOWLEDGMENTS | 168 | ||
REFERENCES | 168 | ||
CHAPTER 11. Cerebral blood flow and energy metabolism in the developing brain | 171 | ||
INTRODUCTION | 171 | ||
CLINICAL RESEARCH METHODS | 171 | ||
THE KETY–SCHMIDT METHOD | 171 | ||
133Xe CLEARANCE | 172 | ||
SINGLE PHOTON EMISSION COMPUTED TOMOGRAPHY (SPECT) | 173 | ||
STABLE XENON-ENHANCED COMPUTED TOMOGRAPHY | 173 | ||
POSITRON EMISSION TOMOGRAPHY | 173 | ||
NEAR-INFRARED SPECTROPHOTOMETRY | 173 | ||
MAGNETIC RESONANCE SPECTROSCOPY | 174 | ||
DOPPLER ULTRASOUND | 176 | ||
OTHER TECHNIQUES USED IN NEWBORN INFANTS | 176 | ||
A NOTE ON RADIATION AND RISK OF CANCER | 177 | ||
THE PHYSIOLOGICAL REGULATION OF CBF | 177 | ||
COUPLING OF CBF TO CEREBRAL ENERGY METABOLISM | 178 | ||
PERFUSION PRESSURE-FLOW AUTOREGULATION | 178 | ||
CARBON DIOXIDE–CBF REACTIVITY | 179 | ||
NEUROGENIC REGULATION OF CBF | 179 | ||
CBF, CEREBRAL OXYGEN DELIVERY AND CMR IN NORMAL NEONATES | 180 | ||
PATHOPHYSIOLOGY | 181 | ||
MEASUREMENT OF CBF AND CMR IN CLINICAL PRACTICE | 188 | ||
MANAGING CBF | 188 | ||
REFERENCES | 188 | ||
CHAPTER 12. EEG and evoked potentials in the neonatal period | 192 | ||
INTRODUCTION | 192 | ||
METHODOLOGY AND MATURATION OF THE EEG | 192 | ||
EEG-MONITORING | 200 | ||
METHODOLOGY AND MATURATION OF EVOKED POTENTIALS | 204 | ||
CLINICAL APPLICATIONS OF BAEPS AND MAXIMUM LENGTH SEQUENCE BAEPS | 208 | ||
FULL-TERM INFANTS | 208 | ||
PRETERM INFANTS | 209 | ||
CLINICAL APPLICATIONS OF VISUAL EVOKED POTENTIALS | 210 | ||
CLINICAL APPLICATIONS OF SOMATOSENSORY EVOKED POTENTIALS | 212 | ||
CONCLUSIONS | 215 | ||
REFERENCES | 215 | ||
SECTION III: ANOMALIES | 222 | ||
CHAPTER 13. Congenital structural defects of the brain | 222 | ||
INTRODUCTION | 222 | ||
NEURAL TUBE DEFECTS | 224 | ||
SPINA BIFIDA OCCULTA | 224 | ||
MENINGOCELE | 225 | ||
MYELOMENINGOCELE | 226 | ||
ARNOLD–CHIARI MALFORMATION | 231 | ||
ENCEPHALOCELE | 233 | ||
ANENCEPHALY | 235 | ||
HOLOPROSENCEPHALY | 237 | ||
NEURONAL MIGRATION DISORDERS | 238 | ||
DISORDERS OF NEURONAL PROLIFERATION | 239 | ||
DISORDERS OF NEURONAL MIGRATION | 242 | ||
NEURONAL HETEROTOPIAS | 246 | ||
AGENESIS OF THE CORPUS CALLOSUM | 248 | ||
AGENESIS | 250 | ||
AGENESIS OF THE VERMIS | 251 | ||
CEREBELLAR HYPERPLASIA | 253 | ||
MACRO CISTERNA MAGNA | 253 | ||
DANDY–WALKER SYNDROME | 253 | ||
INTRACRANIAL ARACHNOID CYSTS | 255 | ||
HYDROCEPHALUS | 256 | ||
HYDRANENCEPHALY | 257 | ||
POLYMICROGYRIA | 257 | ||
REFERENCES | 259 | ||
CHAPTER 14. Genetics of neurodevelopmental anomalies | 266 | ||
INTRODUCTION | 266 | ||
NEURAL TUBE DEFECTS (NTDs) | 267 | ||
HYDROCEPHALUS | 268 | ||
HYDRANENCEPHALY AND PORENCEPHALY | 270 | ||
AGENESIS OF THE CORPUS CALLOSUM (ACC) | 271 | ||
MICROCEPHALY | 272 | ||
HOLOPROSENCEPHALY (HPE) | 272 | ||
LISSENCEPHALY (AGYRIA-PACHYGYRIASUBCORTICAL BAND HETEROTOPIA SPECTRUM) | 275 | ||
CLASSIC (TYPE I) LISSENCEPHALY | 275 | ||
COBBLESTONE (TYPE II) LISSENCEPHALY | 277 | ||
POLYMICROGYRIA (PMG) | 280 | ||
SCHIZENCEPHALY | 281 | ||
PERIVENTRICULAR NODULAR HETEROTOPIA (PNH) | 282 | ||
CEREBELLAR HYPOPLASIA | 284 | ||
DANDY–WALKER MALFORMATION (DWM) | 286 | ||
REFERENCES | 287 | ||
CHAPTER 15. Antenatal assessment of CNS anomalies, including neural tube defects | 291 | ||
INTRODUCTION | 291 | ||
NURAL TUBE DEFECTS | 291 | ||
HYDROCEPHALUS AND VENTRICULOMEGALY | 299 | ||
PROCENCEPHALIC DEVELOPMENTAL DISORDER | 304 | ||
POSTERIOR FOSSA ANOMALY | 310 | ||
DANDY–WALKER MALFORMATION, DANDY–WALKER VARIANT, MEGACISTERNA MAGNA | 313 | ||
NEURONAL PROLIFERATION DISORDER | 314 | ||
NEURONAL MIGRATION DISORDERS | 317 | ||
OTHER CONGENITAL ANOMALIES | 319 | ||
ACQUIRED BRAIN ABNORMALITIES IN UTERO | 331 | ||
NORMAL VARIANTS | 334 | ||
CONCLUSIONS | 335 | ||
REFERENCES | 337 | ||
CHAPTER 16. Transvaginal fetal neuroscan | 339 | ||
INTRODUCTION | 339 | ||
EQUIPMENT AND TECHNIQUE | 339 | ||
SCANNING PLANES | 340 | ||
ANATOMIC EVALUATION USING 2-D TRANSVAGINAL FETAL NEUROSCAN | 343 | ||
THE 3-D TRANSVAGINAL NEUROSCAN | 345 | ||
SELECTED FETAL NEUROPATHOLOGY USING TRANSVAGINAL TRANSFONTANELLE SCANNING | 351 | ||
REFERENCES | 363 | ||
CHAPTER 17. Epidemiology and prevention of neural tube defects | 366 | ||
DEFINITIONS | 366 | ||
EMBRYOLOGY | 366 | ||
ETIOLOGY | 366 | ||
EPIDEMIOLOGY | 368 | ||
REASONS FOR EPIDEMIOLOGICAL STUDY | 368 | ||
PREVALENCE OF NTDs | 369 | ||
THE IMPACT OF PRENATAL SCREENING AND DIAGNOSIS ON THE PREVALENCE OF NTDs | 369 | ||
FOLIC ACID SUPPLEMENTATION AND DIETARY FORTIFICATION | 370 | ||
ETIOLOGY OF NTDs | 372 | ||
REFERENCES | 373 | ||
SECTION IV: HEMORRHAGIC AND ISCHEMIC LESIONS | 375 | ||
CHAPTER 18. Fetal brain injury and multiple pregnancies | 375 | ||
INTRODUCTION | 375 | ||
EPIDEMIOLOGY | 375 | ||
REFERENCES | 383 | ||
CHAPTER 19. Infection, inflammation and brain injury | 385 | ||
INTRODUCTION | 385 | ||
INFLAMMATION AS A MECHANISM OF DISEASE | 385 | ||
THE FETAL INFLAMMATORY RESPONSE SYNDROME | 385 | ||
WHY DOES THE FETUS MOUNT AN INFLAMMATORY RESPONSE? | 386 | ||
CONTRIBUTION OF FETAL INFECTION/ INFLAMMATION TO WHITE MATTER DAMAGE AND CEREBRAL PALSY | 386 | ||
PROPOSED MECHANISMS OF WHITE MATTER DAMAGE DURING INFECTION/INFLAMMATION | 387 | ||
PROPOSED PROPHYLACTIC INTERVENTIONS TO PREVENT OR REDUCE THE RISK OF BRAIN DAMAGE | 391 | ||
ACKNOWLEDGMENT | 391 | ||
REFERENCES | 392 | ||
CHAPTER 20. Neonatal intracranial hemorrhage | 395 | ||
INTRODUCTION | 395 | ||
GERMINAL MATRIX HEMORRHAGE AND INTRAVENTRICULAR HEMORRHAGE | 395 | ||
INTRAPARENCHYMAL HEMORRHAGE | 411 | ||
SUBDURAL HEMORRHAGE | 413 | ||
SUBARACHNOID HEMORRHAGE | 417 | ||
CHOROID PLEXUS HEMORRHAGE | 418 | ||
INTRACEREBELLAR HEMORRHAGE (ICBH) | 419 | ||
THALAMIC HEMORRHAGE | 420 | ||
PARENCHYMAL HEMORRHAGE | 420 | ||
OTHER CRANIAL HEMORRHAGES | 423 | ||
SINOVENOUS THROMBOSIS | 424 | ||
REFERENCES | 424 | ||
CHAPTER 21. Cerebral ischemic lesions | 431 | ||
INTRODUCTION | 431 | ||
PERIVENTRICULAR WHITE MATTER INJURY | 431 | ||
PERINATAL STROKE | 455 | ||
REFERENCES | 464 | ||
SECTION V: PERINATAL ASPHYXIA | 472 | ||
CHAPTER 22. Pathophysiology of asphyxia | 472 | ||
INTRODUCTION | 472 | ||
SYSTEMIC AND CARDIOVASCULAR ADAPTATION TO ASPHYXIA | 472 | ||
PATHOGENESIS OF CELL DEATH | 479 | ||
PATHOPHYSIOLOGIC CORRELATES OF THE PHASES OF NEURAL INJURY | 481 | ||
PATHOPHYSIOLOGIC DETERMINANTS OF ASPHYXIAL INJURY | 486 | ||
ACKNOWLEDGMENTS | 489 | ||
REFERENCES | 489 | ||
CHAPTER 23. Antenatal prediction of asphyxia | 491 | ||
INTRODUCTION | 491 | ||
DEFINITION OF PERINATAL ASPHYXIA | 491 | ||
PATHOPHYSIOLOGY OF PERINATAL ASPHYXIA | 492 | ||
FETAL RESPONSE TO ASPHYXIA | 493 | ||
MARKERS FOR PERINATAL ASPHYXIA | 494 | ||
NEUROIMAGING AND PERINATAL HYPOXIC–ISCHEMIC BRAIN INJURY | 496 | ||
ANTEPARTUM ASPHYXIA | 497 | ||
ANTEPARTUM FETAL TESTING | 497 | ||
DOPPLER VELOCIMETRY OF THE UMBILICAL VESSELS | 502 | ||
SUMMARY | 503 | ||
REFERENCES | 503 | ||
CHAPTER 24. Intrapartum monitoring for asphyxia | 506 | ||
INTRODUCTION | 506 | ||
FETAL HEART RATE AUSCULTATION | 506 | ||
‘FETAL DISTRESS’ AND ‘FETAL ASPHYXIA’ | 506 | ||
CEREBRAL PALSY | 507 | ||
HISTORY OF FHR PATTERNS AND FETAL CONDITION | 509 | ||
ELECTRONIC FETAL MONITORING VS. TRADITIONAL AUSCULTATION | 509 | ||
RANDOMIZED CONTROLLED TRIALS OF EFM VS. AUSCULTATION | 509 | ||
POTENTIAL BENEFITS OF ELECTRONIC FETAL MONITORING | 513 | ||
POTENTIAL RISKS OF ELECTRONIC FETAL MONITORING | 514 | ||
OTHER METHODS OF FETAL MONITORING | 514 | ||
DEFINITIONS OF FHR PATTERNS | 517 | ||
DECELERATION | 519 | ||
BASELINE FETAL HEART RATE | 521 | ||
DECELERATIONS | 522 | ||
REFERENCES | 524 | ||
CHAPTER 25 Prediction of asphyxia with fetal gas analysis | 528 | ||
INTRODUCTION | 528 | ||
DEFINITIONS | 528 | ||
NORMAL OXYGEN DELIVERY TO THE FETUS | 529 | ||
IMPAIRED OXYGEN DELIVERY TO THE FETUS | 531 | ||
ASPHYXIA | 532 | ||
CONCLUSIONS | 538 | ||
REFERENCES | 539 | ||
CHAPTER 26. The asphyxiated newborn infant | 542 | ||
INTRODUCTION | 542 | ||
DEFINITION | 542 | ||
INCIDENCE | 546 | ||
PATHOLOGY | 546 | ||
CLINICAL FEATURES | 552 | ||
INVESTIGATIONS | 554 | ||
MANAGEMENT | 562 | ||
OUTCOME | 570 | ||
NEONATAL BRAIN DEATH | 578 | ||
REFERENCES | 580 | ||
CHAPTER 27. Neuroprotection of the fetal and neonatal brain | 587 | ||
PATHOPHYSIOLOGY OF PERINATAL BRAIN DAMAGE | 587 | ||
POTENTIAL TARGETS FOR NEUROPROTECTION | 589 | ||
ETHICAL ISSUES | 592 | ||
CONCLUSIONS | 592 | ||
ACKNOWLEDGMENTS | 592 | ||
REFERENCES | 593 | ||
CHAPTER 28. Medico-legal issues: the United Kingdom perspective | 595 | ||
THE SOCIAL CLIMATE | 595 | ||
THE LEGAL CLIMATE | 595 | ||
THE NHS REDRESS SCHEME | 596 | ||
LITIGATION AND RISK MANAGEMENT | 596 | ||
EXPERT EVIDENCE | 597 | ||
ESTABLISHING LIABILITY | 597 | ||
REFERENCES | 601 | ||
CHAPTER 29. Malpractice issues in perinatal medicine: the United States perspective | 603 | ||
INTRODUCTION | 603 | ||
HISTORIC PERSPECTIVE OF MALPRACTICE IN THE UNITED STATES | 603 | ||
MALPRACTICE LAW | 603 | ||
INFORMED CONSENT | 608 | ||
COMPLICATION OR ERROR? | 609 | ||
PURSUING THE CASE | 610 | ||
MALPRACTICE MYTHOLOGY — THE FAILURE OF MEDICO-LEGAL EDUCATION | 610 | ||
PEER REVIEW | 612 | ||
THE ROLE OF THE PHYSICIAN | 614 | ||
COMMON AREAS OF LITIGATION DURING LABOR | 615 | ||
FETAL CARDIOTOCOGRAPHY (CTG) | 616 | ||
THE LEGAL CLIMATE | 616 | ||
UNDERWRITER DATA CLAIMS PAYMENTS | 617 | ||
JURY AWARDS AND SETTLEMENTS | 618 | ||
PREVALENCE OF MEDICAL MALPRACTICE | 618 | ||
MEDICAL RECORDS | 620 | ||
LEGAL VULNERABILITY | 621 | ||
TORT REFORM MEASURES | 621 | ||
ALTERNATIVE SYSTEM REFORMS | 623 | ||
MEDIATION | 624 | ||
ARBITRATION | 624 | ||
SPECIALIZED MEDICAL MALPRACTICE COURTS | 624 | ||
PRETRIAL SCREENING PANELS | 625 | ||
ENTERPRISE LIABILITY | 625 | ||
TORT REFORM AND FINANCES — LITIGATION AND RISK MANAGEMENT | 626 | ||
SOLVING THE MALPRACTICE PROBLEM | 627 | ||
NOTE 1 | 628 | ||
NOTE 2 | 628 | ||
NOTE 3 | 628 | ||
NOTE 4 | 628 | ||
REFERENCES | 629 | ||
SECTION VI: INFECTION OF THE CNS | 630 | ||
CHAPTER 30. Toxoplasmosis | 630 | ||
MODES OF CONTAMINATION | 630 | ||
IMMUNOLOGY | 630 | ||
EPIDEMIOLOGY | 632 | ||
PREVENTION | 632 | ||
CONGENITAL TOXOPLASMOSIS | 633 | ||
TREATMENT | 635 | ||
REFERENCES | 638 | ||
CHAPTER 31. Congenital viral infections and the central nervous system | 640 | ||
INTRODUCTION | 640 | ||
REFERENCES | 653 | ||
CHAPTER 32. Bacterial and fungal infections | 657 | ||
INTRODUCTION | 657 | ||
EPIDEMIOLOGY | 657 | ||
PATHOGENESIS | 660 | ||
CLINICAL FEATURES | 662 | ||
MANAGEMENT | 665 | ||
COMPLICATIONS | 668 | ||
SHUNT INFECTIONS | 671 | ||
FUNGAL CNS INFECTIONS | 673 | ||
NEONATAL TETANUS | 674 | ||
CONGENITAL NEUROSYPHILIS | 675 | ||
REFERENCES | 676 | ||
SECTION VII: METABOLIC DISORDERS | 678 | ||
CHAPTER 33. Inborn errors of metabolism presenting with encephalopathy | 678 | ||
INTRODUCTION | 678 | ||
METABOLIC ENCEPHALOPATHIES IN THE NEWBORN: MAIN CHARACTERISTICS | 678 | ||
DIAGNOSTIC APPROACH TO NEONATAL ENCEPHALOPATHIES DEPENDING ON CLINICAL MANIFESTATIONS | 679 | ||
IEM GIVING RISE TO NEUROLOGIC DETERIORATION DUE TO INTOXICATION | 679 | ||
IEM GIVING RISE TO SEVERE NEONATAL SEIZURES | 684 | ||
IEM GIVING RISE TO SEVERE NEONATAL HYPOTONIA | 686 | ||
IEM GIVING RISE TO RIGID-AKINETIC SYNDROME | 692 | ||
OTHER DISORDERS | 695 | ||
DIAGNOSTIC STEPS IN A NEWBORN WITH ENCEPHALOPATHY SUSPECTED OF METABOLIC ORIGIN | 695 | ||
REFERENCES | 696 | ||
SECTION VIII: SEIZURE DISORDERS | 698 | ||
CHAPTER 34. Seizure disorders of the neonate | 698 | ||
INTRODUCTION | 698 | ||
EPIDEMIOLOGY | 698 | ||
PATHOPHYSIOLOGY | 699 | ||
CLASSIFICATION, CLINICAL AND EEG FEATURES | 700 | ||
ETIOLOGY | 704 | ||
TREATMENT | 707 | ||
PROGNOSIS | 708 | ||
REFERENCES | 708 | ||
CHAPTER 35. Hypoglycemia and brain injury — when neonatal metabolic adaptation fails | 711 | ||
INTRODUCTION | 711 | ||
FETAL METABOLISM AND METABOLIC CHANGES AT BIRTH | 712 | ||
‘TRANSITIONAL HYPOGLYCEMIA’ AND PROTECTIVE RESPONSES | 712 | ||
WHEN NEONATAL METABOLIC ADAPTATION FAILS — THE PATHOLOGICAL SEQUELAE OF HYPOGLYCEMIA | 713 | ||
APPLICATION TO CLINICAL PRACTICE | 718 | ||
SUMMARY | 718 | ||
REFERENCES | 719 | ||
CHAPTER 36. Kernicterus | 720 | ||
INTRODUCTION: KERNICTERUS THEN AND NOW | 720 | ||
BILIRUBIN CHEMISTRY, METABOLISM AND NEWBORN JAUNDICE | 722 | ||
BILIRUBIN AND KERNICTERUS | 726 | ||
CLINICAL ASPECTS OF KERNICTERUS | 730 | ||
CLINICAL MANIFESTATIONS OF BILIRUBIN TOXICITY | 731 | ||
EVALUATION OF THE JAUNDICED NEWBORN | 733 | ||
TREATMENT OF NEWBORN JAUNDICE | 735 | ||
SUMMARY | 736 | ||
REFERENCES | 736 | ||
SECTION IX: THE SPECIAL SENSES | 743 | ||
CHAPTER 37. Disorders of vision | 743 | ||
INTRODUCTION | 743 | ||
EYE MOVEMENTS | 743 | ||
VISION | 752 | ||
DISORDERS OF THE ANTERIOR VISUAL PATHWAY | 758 | ||
DISORDERS OF THE POSTERIOR VISUAL PATHWAY | 760 | ||
REFERENCES | 764 | ||
CHAPTER 38. Hearing disorders | 769 | ||
INTRODUCTION | 769 | ||
DEVELOPMENT OF THE EAR | 769 | ||
MATERNAL DIABETES AND DEAFNESS | 770 | ||
MATERNAL INFECTIONS AND DEAFNESS | 770 | ||
OTHER INFECTIONS CAUSING HEARING LOSS | 770 | ||
DRUGS DURING PREGNANCY AND DEAFNESS | 770 | ||
GENETIC CONDITIONS AFFECTING HEARING | 770 | ||
SYNDROMIC DEAFNESS INCLUDE THE FOLLOWING CONDITIONS | 771 | ||
PERINATAL CONDITIONS AFFECTING BABIES’ HEARING | 772 | ||
CONDITIONS IN THE NEONATE AND INFANT AFFECTING HEARING | 773 | ||
ASSESSING HEARING IN THE NEWBORN AND INFANT, AND FOLLOW-UP | 774 | ||
SPECIFIC CONDITIONS | 774 | ||
SUMMARY | 775 | ||
REFERENCES | 776 | ||
SECTION X: DISORDERS OF THE NERVE AND MUSCLE | 778 | ||
CHAPTER 39. Disorders of the spinal cord, cranial and peripheral nerves | 778 | ||
INTRODUCTION | 778 | ||
CRANIAL NERVES | 778 | ||
THE SPINAL CORD | 781 | ||
CAUDAL REGRESSION SYNDROME (CRS) | 781 | ||
BRACHIAL PLEXUS PALSY | 785 | ||
PERIPHERAL NERVE PALSIES | 788 | ||
REFERENCES | 790 | ||
CHAPTER 40. Neuromuscular disorders | 792 | ||
CLINICAL PRESENTATION | 792 | ||
INVESTIGATIONS | 793 | ||
MANAGEMENT | 794 | ||
THE MUSCULAR DYSTROPHIES | 794 | ||
THE CONGENITAL MYOPATHIES | 799 | ||
MYASTHENIA GRAVIS | 801 | ||
OTHER NEUROMUSCULAR DISORDERS | 803 | ||
NON-NEUROMUSCULAR DISORDERS | 806 | ||
CONCLUSION | 808 | ||
REFERENCES | 808 | ||
SECTION XI: HYDROCEPHALUS AND NEUROSURGERY | 810 | ||
CHAPTER 41. Fetal neurosurgical interventions | 810 | ||
INTRODUCTION | 810 | ||
TREATMENT OF FETAL HYDROCEPHALUS | 810 | ||
TREATMENT OF MYELOMENINGOCELE AND THE CHIARI II MALFORMATION | 814 | ||
REFERENCES | 818 | ||
CHAPTER 42. Neonatal hydrocephalus — clinical assessment and non-surgical treatment | 819 | ||
DEFINITION | 819 | ||
CSF CIRCULATION AND ABSORPTION | 819 | ||
ETIOLOGY | 819 | ||
HYDROCEPHALUS PRODUCES NEUROPATHOLOGIC CHANGES | 823 | ||
DIAGNOSIS | 823 | ||
CSF PRESSURE MEASUREMENT | 826 | ||
PROGNOSIS | 827 | ||
NON-SURGICAL TREATMENT OF HYDROCEPHALUS | 828 | ||
ACETAZOLAMIDE | 829 | ||
ISOSORBIDE | 831 | ||
GLYCEROL | 831 | ||
REFERENCES | 832 | ||
CHAPTER 43. Neurosurgical management of hydrocephalus | 834 | ||
INTRODUCTION | 834 | ||
PATHOLOGY | 834 | ||
CLINICAL FEATURES | 836 | ||
SURGICAL TREATMENT OF HYDROCEPHALUS | 837 | ||
COMPLICATIONS OF SHUNTS | 842 | ||
MANAGEMENT OF SHUNT COMPLICATIONS | 843 | ||
SUMMARY | 845 | ||
REFERENCES | 845 | ||
CHAPTER 44. Surgical management of neural tube defects | 847 | ||
INTRODUCTION | 847 | ||
ENCEPHALOCELES | 848 | ||
ANENCEPHALY | 848 | ||
SPINA BIFIDA | 849 | ||
MYELOMENINGOCELE | 849 | ||
MENINGOCELE | 851 | ||
OCCULT SPINAL DYSRAPHISM | 852 | ||
REFERENCES | 854 | ||
CHAPTER 45. Congenital defects, vascular malformations and other lesions | 856 | ||
INTRODUCTION | 856 | ||
GROUP 1. CEREBROCRANIAL DYSPLASIA (BRAIN AND SKULL DEFECTS) | 856 | ||
GROUP 2. CEREBROFACIAL DYSPLASIA (BRAIN AND FACE DEFECTS) | 857 | ||
GROUP 3. CRANIOFACIAL DYSPLASIA (SKULL AND FACIAL DEFECTS) | 857 | ||
CRANIOFACIAL DYSPLASIA WITH CLEFTING | 861 | ||
GROUP 4. CRANIOFACIAL DYSPLASIA OF OTHER ORIGIN | 861 | ||
OTHER NEUROSURGICAL LESIONS IN THE NEONATAL PERIOD | 864 | ||
REFERENCES | 865 | ||
SECTION XII: EPIDEMIOLOGY OF NEUROLOGIC DISABILITY | 867 | ||
CHAPTER 46. The epidemiology of the cerebral palsies | 867 | ||
DEFINITION: A REAL CHALLENGE FOR EPIDEMIOLOGISTS | 867 | ||
DIFFERENTIAL DESCRIPTIONS: IMPORTANT FOR BOTH EPIDEMIOLOGISTS AND SERVICE PROVIDERS | 868 | ||
HETEROGENEITY AND MULTIFACTORIALITY OF CAUSE: THE IMPORTANCE OF CAUSAL PATHWAY THINKING | 869 | ||
THE FREQUENCY OF CEREBRAL PALSY: THE NEED FOR CAREFUL INTERPRETATION | 869 | ||
TRENDS IN FREQUENCY OVER TIME | 870 | ||
RISK FACTORS FOR CEREBRAL PALSY | 870 | ||
CONCLUSION | 873 | ||
ACKNOWLEDGMENTS | 874 | ||
REFERENCES | 874 | ||
CHAPTER 47. The epidemiology of intellectual disabilities | 876 | ||
INTRODUCTION | 876 | ||
DEFINITION AND CLASSIFICATION | 878 | ||
PREVALENCE | 879 | ||
ETIOLOGY AND RISK FACTORS | 882 | ||
TRANSITION INTO ADULTHOOD | 891 | ||
CONCLUSION | 893 | ||
REFERENCES | 893 | ||
SECTION XIII: ETHICAL DILEMMAS | 898 | ||
CHAPTER 48. Issues for the obstetrician | 898 | ||
INTRODUCTION | 898 | ||
OBSTETRICS ETHICS | 898 | ||
THE CONCEPT OF THE FETUS AS A PATIENT | 899 | ||
CONCLUSION | 904 | ||
REFERENCES | 904 | ||
CHAPTER 49. Issues for the neonatologist | 905 | ||
INTRODUCTION | 905 | ||
THE PRINCIPLE OF THE SANCTITY OF LIFE | 905 | ||
QUALITY OF LIFE | 905 | ||
ACTS OF COMMISSION AND OMISSION | 905 | ||
DIAGNOSIS OF NEUROLOGIC AND NEUROSURGICAL PROBLEMS IMMEDIATELY AFTER DELIVERY | 906 | ||
DIAGNOSIS OF NEUROLOGIC AND NEUROSURGICAL PROBLEMS AFTER PROLONGED INTENSIVE CARE | 907 | ||
CONCLUSION | 908 | ||
REFERENCES | 908 | ||
Index | 909 | ||
Color Plate | 923 |