Table of Contents Introduction: Normal Development and Basic Reactions 1 1. Gross and Microscopic Development of the Central Nervous System .... 2 Timing of Early Embryonal Landmarks 2. — Mass Growth of the Brain 3. — Cerebral Gyri 4. — Lamination of Cerebral Cortex 4. — Superficial Granular Layer 5. — Cells of Cajal-Retzius 5. — Ammon's Horn 5. — Periventricular Germinal Layer 6. — Volume of White Matter 7. — Myelination Gliosis and Development of Astrocytes 7. — Myelination 8. — Regional Timing of Myelination 8. — Basal Ganglia 9. — Mineralization of Cerebral Tissue 9. — Ventricular System 10. — Brain Stem Nuclei 12. — Melanization of Nuclei 12. — Cerebellum 12. — Spinal Cord 16. — References 17. 2. Some Features of Basic Reactions Characteristic for Immature Nervous Tissue 21 Reaction of Immature Nervous Tissue to Necrosis 21. — Age-Dependent Variation in Anoxic Tissue Damage 21. — Anoxic Neuronal Necrosis 21. — Myelination Gliosis 22. — Reactive Gliosis 22. — Fibrillary Gliosis 23. — Differentiation of Glia in the Germinal Layer 23. — Metabolic Astrocytosis 23. — Macrophage Responses 24. — References 24. First Part: Acquired Lesions in Newborns and Infants 27 3. Porencephaly, Hydranencephaly, Multicystic Encephalopathy 28 Porencephaly 28. — Hydranencephaly 31. — Basket Brains 34. — Hydranen­ cephaly or Porencephaly Related to Fetal Infections 35. — Hydranencephaly with Proliferative Vasculopathy 35. — Cavitated Cerebral Lesions in Twins 35. — Multicystic Encephalopathy 36. — Global Hemispheric Necrosis in Infants 38. — Pathogenetic Considerations 39. — References 40. 4. Hemorrhages in Asphyxiated Premature Infants 44 Subependymal and Intraventricular Hemorrhages 45. — Choroid Plexus Hemorrhages 47. — Subarachnoid Hemorrhages 49. — Subpial Hemorrhages 49. — Hemorrhages into the Falx 50. — Cerebellar Hemorrhages 50. — Hemorrhages at Other Sites 51. — Residual Lesions 52. — Posthemorrhagic Hydrocephalus 53. — Other Residua 54. — Pathogenetic Considerations 54. — References 55. http://d-nb.info/881184314 VIII Table of Contents 5. Lesions of Traumatic Birth and Other Causes of Hemorrhage Intradural Hemorrhages 59. — Lacerations of Tentorium or Falx 59. — Bone Injuries 60. — Epidural and Subdural Hemorrhages 61. — Embolism of Cere­ bral Tissue 61. — Injuries of the Spinal Cord 61. — Intracerebral Hematomas 62. — Hemorrhagic Disease of the Newborn 62. — Erythrocyte Disorders 64. — Herniation of Cerebral Tissue from Increased Intracranial Pressure 64. — References 66. 6. Perinatal Lesions of White Matter Periventricular Infarcts (Periventricular Leukomalacia) 69. — Gross and Mi­ croscopic Features of Periventricular Infarcts 70. — Hemorrhages into Infarcts 72. — Residual Lesions of Periventricular Infarcts 72. — Differential Diagno­ sis of Periventricular Cavitated Lesions 74. — Pathogenetic Considerations 74. — Subcortical Infarcts 74. — Lesions in Areas of Primary Myelination 75. — Perinatal Telencephalic Leukoencephalopathy 75. — Cerebral White Matter Hypoplasia 75. — Diffuse Fatty Change ofWhite Matter 75. — References 78. 7. Perinatal Lesions of Gray Matter Ulegyria of Cerebral Cortex - Definition 82. — Acute and Subacute Lesions of Ulegyria 84. — Residual Lesions of Ulegyria 84. — Regional Distribution of Ulegyria 85. — Associated Cerebellar Lesions 86. — Status Marmoratus of Basal Ganglia - Definition 86. — Early Lesions of Status Marmoratus 87. — Late Lesions of Status Marmoratus 87. — Association of Status Marmoratus with Other Lesions 89. — Nature of the Lesions in Status Marmoratus 89. — Symmetrical Thalamic Sclerosis in Infants 89. — Other Lesions of Basal Ganglia 89. — Lesions Causing Specific Sensory Defects 90. — Pontosubicular Neuron Necrosis - Definition 90. — Lesions of Pontosubicular Neuron Necro­ sis 91. — Pathogenetic Considerations on Perinatal Asphyxial Cerebral Damage 92. — References 94. 8. Postnatal Lesions of Gray Matter Cardiac Arrest Encephalopathy 98. — Spinal Cord Necrosis 99. — Retrolental Fibroplasia 99. — Hypoglycemic Lesions 100. — Sudden Infant Death Syn­ drome 101. — Hemorrhagic Shock Encephalopathy 101. — Postconvulsive Brain Damage - General Aspects 101. — Postconvulsive-postanoxic Diffuse Cerebral Cortical Sclerosis - Definition of Lesions 102. — Postconvulsive Cerebral Damage - Clinical Course and Types of Lesions 102. — Hemiatrophy 103. — Cerebellar Lesions 105. — Patterns of Transneuronal Degeneration- Crossed Cerebellar Atrophy 106. — Aplasia Versus Atrophy of the Cerebellar Granular Layer 107. — Superficial Siderosis 108. — Familial Degeneration of the Pallidonigral System 110. — Infantile Bilateral Striatal Necrosis 110. — Congenital Pick Cell Encephalopathy 111. — References 111. Table of Contents IX 9. Kernicterus (Bilirubin Encephalopathy) 115 Metabolism of Bilirubin 115. — Etiologies of Kernicterus 116. — Gross and Microscopic Features of Kernicterus 117. — Posticteric Encephalopathy 118. — Kernicterus, Status Dysmyelinisatus and Lesions of Perinatal Anoxia 120. — Pathogenetic Considerations 120. — References 122. 10. Arterial Diseases in Infancy 125 Occlusion of Stems of Major Cerebral Arteries in Newborns 125. — Smaller, Randomly Disposed Infarcts in Newborns 127. — Arterial Calcification in In­ fants 128. — Disseminated Vascular Disease in the Congenital Rubella Syn­ drome 129. — Secondary Changes in Atrophic Arteries near Residual Perina­ tal Lesions 130. — Vasculo-occlusive Processes in Childhood 130. — Moya- moya Disease 131. — Vasculo-occlusive Disease in Homocystinuria 132. — Fibromuscular Dysplasia 132. — Dissecting Aneurysms of Cerebral Arteries 133. — Congenital Aneurysms of Cerebral Arteries 133. — References 134. 11. Thrombosis of Intracranial Sinus and Veins 138 Phlebothrombosis Complicating General Diseases 138. — Gross and Micro­ scopic Features of Phlebothrombosis 139. — Residual Lesions of Phlebo­ thrombosis 141. — Pathogenetic Considerations 143. — Thrombophlebitis from Local Propagation of Infections 144. — References 145. 12. Cerebral Lesions in Congenital Cardiac Disease 147 Abscesses 147. — Phlebothrombosis 148. — Infarcts 148. — Aneurysms 149. — Other Lesions 149. — Subendocardial Fibroelastosis 150. — References 150. 13. Craniocerebral Trauma in Infancy 152 Cerebral Lesions from Blunt Trauma in Early Infancy 152. — Subdural Hema­ tomas 153. — Widening Skull Fracture of Childhood 153. — References 155. 14. Infections of the Fetus 156 Rubella Embryopathy 156. — Generalized Cytomegalic Inclusion Disease 158. — Other Viral Infections 160. — Toxoplasmosis 161. — Connatal Syphilis 164. — HTLV-III Virus (AIDS) 165. — Experimental Malformations Pro­ duced by Fetal Infections and Their Bearing on Human Disease 165. — Ref­ erences 166. 15. Purulent Leptomeningitis in Newborns and Infants 169 Neonatal Leptomeningitis 169. — Gross and Microscopic Features of Neonatal Leptomeningitis 170. — Postmeningitic Encephalopathy 173. — X Table of Contents Pathogenetic Considerations 174. — Neonatal Listeriosis 174. — Intrauterine Meningitis 175. — Leptomeningitis in Infants 175. — Subdural Effusions in Meningitis 176. — Postmeningitic Lesions in Infants 176. — Abscesses and Empyemas 177. — References 177. 16. Meningoencephalitic Processes in the Peri- and Postnatal Periods, Including CNS Involvement by some Hematologic Diseases 180 Candida 180. — Mucor 182. — Aspergillus 182. — Cryptococcus 182. — Coc- cidioides 182. — Helminths 182. — Herpes Simplex 183. — Coxsackie Virus 184. — Poliomyelitis 185. — Aseptic Meningitis due to Enteroviruses 186. — Arthropod-borne Viruses 186. — Unclassified Chronic Encephalitis 186. — Tuberculosis 186. — HTLV-III Virus (AIDS) 187. — Hypo- and Agamma­ globulinemia 187. — Chronic Granulomatous Disease of Childhood 187. — Chediak-Higashi Disease 190. — Familial Lymphohistiocytosis (Erythro- phagocytic or Hemophagocytic Lymphohistiocytosis or Reticulosis) 192. — References 194. 17. Subdural Hematomas, Hygromas and Effusions 198 Structure and Reactions of the Subdural Space 198. — Subdural Hemorrhage in Infancy 200. — Supratentorial Hematomas 200. — Infratentorial Hematomas 201. — Pathology of Chronic Subdural Hematomas 201. — Sub­ dural Hygromas 203. — Chronic Subdural Hematomas and Hydrocephalus 203. — Subdural Hemorrhage upon Dehydration 204. — Chronic Subdural Hematomas Coexisting with Atrophic Hemispheric Lesions 204. — Benign Subdural Effusions 205. — Subdural Effusions in Leptomeningitis 205. — Traumatic Subdural Effusions 205. — Subdural Hemorrhages in General Diseases 206. — Congenital Subdural Cysts 206. — Pathogenetic Consider­ ations 206. — References 207. 18. Meningeal Cysts 209 Arachnoid and Glioependymal Cysts - General Features and Classification 209. — Structure of Arachnoid Cysts 209. — Structure of Glioependymal Cysts 210. — Topographic Survey: Cysts of the Cerebral Hemispheres 211. — Intracerebral Cysts 213. — Basal Midline Cysts 213. — Supracollicular and Paramesencephalic Cysts (Cysts of Cisterna Ambiens) 214. — Retrocerebellar and Basal Infratentorial Cysts 214. — Spinal Cysts 215. — Syndromes with Arachnoid or Glioependymal Cysts 216. — Choroid Plexus Cysts 216. — Dural Cysts 216. — References 217. 19. Hydrocephalus - Basic Concepts and General Pathology 220 Circulation of CSF in Small, Primitive Brains 220. — Circulation of CSF in Large Brains 221. — CSF and Extracellular Tissue Compartment 222. — Pathophysiology of Disturbed CSF Circulation 222. — Compensatory Mecha­ nisms of CSF Resorption 223. — Changes in Production of CSF 223. — Effects of CSF Composition 223. — Effects of Pressure Pulses 224. — General Pathol­