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Preface
I Award Lectures
Autism: A Syndrome of Neurological Dysfunction. Frank Ford Memorial Lecture 1990 1
Physiological and Pathophysiological Roles of Excitatory Amino Acids during Central Nervous System Development. John Prichard Award Lecture 1990 19
II Pediatric Neurogenetics
Molecular Genetics in Child Neurology 31
Fabry Disease: Molecular Heterogeneity and Phenotype-Genotype Correlations 42
Molecular Genetic Analysis of Duchenne/Becker Muscular Dystrophy Families 46
Peripheral Neuropathies in Childhood 60
Rett Syndrome: Update 79
Therapy of Genetically Determined Metabolic Disorders 93
III Embryology and Dysgenesis of the Brain
Disturbances of Neuroblast Migrations after 20 Weeks Gestation in the Human Fetus and Neonate 108
Cerebral Plasticity in Embryological Development 118
Clinical Characteristics of the Prenatal Encephalopathies 132
Magnetic Resonance Imaging: Perspective for Developmental Disorders of Central Nervous System 137
IV Neurocutaneous Syndromes
Overview of the Neurocutaneous Syndromes 144
Genetic Epidemiology of Neurofibromatosis in Italy 154
Complications of Neurofibromatosis Type 1 in a Series of 197 Children 162
Incontinentia Pigmenti: Presentations, Pathology, Pathogenesis and Prognosis 174
Sturge-Weber Syndrome. Recent Developments in Neuroimaging and Surgical Considerations 187
Linkage Heterogeneity in Tuberous Sclerosis. A Collaborative Study
Summary of the Papers on Neurocutaneous Syndromes 203
V Pre- and Perinatal Pathophysiology of Brain Damage
Vascular-Induced Intrauterine Growth Retardation: Animal and Human Models 206
Antepartum Events and Subsequent Handicap 216
Prenatal Pathophysiology of Brain Damage 223
Perinatal Hypoxic-Ischemic Brain Injury: Overview 232
Lost Autoregulation and the Pathogenesis of Intracranial Haemorrhage in Preterm Newborns. An Update 253
Perinatal Hypoxic-Ischemic Brain Injury. New Insights from Experimental Studies 257
VI New Syndromes and Rare Interesting Diseases in Child Neurology
Peroxisomes and Pediatric Neurological Diseases 268
Neurotransmitters and their Disorders in the Developing Brain 285
Inherited Progressive Disorders of the Fetal Brain: A Field in Need of Recognition 299
The Birth and Infancy of a New Disease: The Carbohydrate-Deficient Glycoprotein Syndrome 314
AIDS and the Central Nervous System in Children 320
VII Ethical Problems in Child Neurology
Brain Death Determination in Different Cultures 329
Anencephalic Infants as Organ Sources: Ethical Implications 340
Ethics of Allocating Scarce Medical Resources 346
Ethical Implications of Care in Neurology in Developing Countries 351
The Persistent Vegetative State in Children 357
Summary of the Papers on Ethical Issues in Child Neurology 367
Subject Index 371