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Fetal and Neonatal Neurology and Neurosurgery E-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