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TABLE OF CONTENTS
PART ONE BRAIN DEVELOPMENT
AND CONGENITAL MALFORMATIONS
1 Normal Brain Development and General Clas-
sification of Congenital Malformations, 3
Normal Brain Development, 3
Neurulation, 3
Neural Tube Closure, 5
Formation of Brain Vesicles and Flexures, 5
Disjunction of Cutaneous and Neural Ecto-
derm, 6
Forebrain Formation, 6
Midbrain Formation, 7
Hindbrain Formation, 7
Spine and Spinal Cord Formation, 7
General Classification of Central Nervous Sys-
tem Malformations, 10
2 Disorders of Neural Tube Closure, 15
Chiari Malformations, 15
Chiari I Malformation, 16
Chiari II Malformation, 18
Chiari III Malformation, 24
Cephaloceles, 24
OccipitaI and Parietal Cephaloceles, 25
Sincipital and Sphenopharyngeal Cephalo-
celes, 25
Nasal Cephaloceles, Dermoids, and Gliomas,
25
Atretic Cephaloceles, 25
Corpus Callosum Anomalies, 29
Corpus Callosum Agenesis, 29
Corpus Callosum Lipoma, 34
3 Disorders of Diverticulation and Cleavage, Sul-
cation and Cellular Migration, 37
Diverticulation and Cleavage: Holoprosencepha-
lies and Related Disorders, 37
Alobar Holoprosencephaly, 38
Semilobar Holoprosencephaly, 40
Lobar Holoprosencephaly, 41
Septooptic Dysplasia, 42
Arhinencephaly, 42
and Cellular Migration Disorders, 44
Lissencephaly, 44
Nonlissencephalic Cortical Dysplasias, 46
Heterotopias, 48
Schizencephaly, 52
Unilateral Megalencephaly, 56
4 Posterior Fossa Malformations and Cysts, 59
Dandy-Walker Complex, 59
Dandy-Walker Malformation, 59
Dandy-Walker Variant, 61
Miscellaneous Posterior Fossa Cysts, 63
Mega Cisterna Magna, 63
Posterior Fossa Arachnoid Cyst, 63
Miscellaneous Cerebellar Hypoplasias/Dyspla-
sias, 66
Chiari IV Malformation, 66
Joubert's Syndrome, 66
Rhombencephalosynapsis, 66
Tectocerebellar Dysraphia, 69
Lherrnitte-Duclos Disease, 69
5 Disorders of Histogenesis: Neurocutaneous
Syndromes, 72
Neurofibromatosis, 72
Neurofibromatosis Type 1 (von Reckling-
hausen Disease), 72
Neurofibromatosis Type 2, 84
Segmental Neurofibromatosis, 93
Tuberous Sclerosis, 93
Sturge-Weber Syndrome, 98
Von Hippel-Lindau Disease, 104
Other Neurocutaneous Syndromes, 106
Wyburn-Mason Syndrome, 106
Ataxia-Telangiectasia, 106
Rendu-Osler-Weber Disease, 106
K1ippel-Trenaunay-Weber Syndrome, 107
Meningioangiomatosis, 107
Neurocutaneous Melanosis, 109
Epidermal Nevus Syndrome, 109
Basal Cell Nevus (Gorlin) Syndrome, 110
Miscellaneous Melanocytic Phakomatoses, 110
Cowden Disease, 111
PART TWO CEREBRAL VASCULATURE:
NORMAL ANATOMY AND PATHOLOGY
6 Normal Vascular Anatomy, 117
Arterial Anatomy, 117
Aortic Arch and Great Vessels, 117
Carotid Arteries, 119
Circle of Willis, 126
Cerebral Arteries, 132
Posterior Fossa Arteries, 142
Venous Anatomy, 145
Dural Sinuses, 145
Cerebral Veins, 150
7 Intracranial Hemorrhage, 154
Pathophysiology of Intracranial Hemorrhage,
154
of bleeding, 154
Clot Formation, and Hemorrhage
Evolution, 154
Imaging of Intracranial Hemorrhage, 158
Computed Tomography, 158
Magnetic Resonance Imaging: Factors Influ-
encing Hemorrhage Signal, 160
Nontraumatic Intracranial Hemorrhage, 173
Perinatal Hemorrhage, 173
Hypertension, 174
Hemorrhagic Infarction, 178
Aneurysms and Vascular Malformations, 182
Hemorrhagic Neoplasms and Cysts, 186
Miscellaneous Causes of Intracranial Hemor-
rhage, 192
8 Craniocerebral Trauma, 199
Classification, Etiology, and Frequency of Trau-
matic Brain Injury, 199
Classification of Brain Trauma, 199
Mechanisms of Traumatic Brain Injury, 199
Frequency of Traumatic Lesions, 202
Primary Traumatic Lesions, 202
Skull and Scalp Lesions, 202
Extraaxial Hemorrhage, 204
Intraaxial Lesions, 212
Secondary Effects of Craniocerebral Trauma, 222
Cerebral Herniations, 222
Cerebral Ischemia, Infarction, and Secondary
Hemorrhage, 230
Diffuse Cerebral Edema, 230
Vascular Manifestations and Complications of
Craniocerebral Trauma, 232
Sequelae of Trauma, 240
Encephalomalacia, 240
Pneumocephalus, 241
CSF Leaks and Fistulae, 242
Cranial Nerve Palsies, 242
Diabetes Insipidus, 243
Cephaloceles and Leptomeningeal Cysts, 243
Head Trauma in Children: SpecIal Consider-
ations, 244
CNS ManifestatIons of Nonaccidental Trauma,
244
9 Intracranial Aneurysms, 248
Saccular Aneurysms, 248
Developmental/Degenerative Aneurysms, 248
Imaging of Intracranial Aneurysms, 257
Traumatic Aneurysms, 269
Mycotic Aneurysms, 272
Oncotic Aneurysms, 272
Flow-related Aneurysms, 275
Vasculopathies, Vasculitis, and Drug-related
Aneurysms, 275
Fusiform Aneurysms, 278
Dissecting Aneurysms, 280
10 Intracranial Vascular Malformations, 284
Arteriovenous Malformations, 284
Parenchymal (Pial) AVMs, 285
Dural AVMs and Fistulae, 301
Mixed Pial-Dural AVMs, 306
Capillary Telangiectasias, 309
Cavernous Angiomas, 311
Venous Malformations, 314
Venous Angioma, 314
Vein of Galen Malformations, 320
Venous Varix, 324
11 Stroke, 330
Atherosclerosis, 330
Pathology and Pathogenesis, 330
Imaging of Atheroslcerotic Vascular Disease,
332
Cerebral Ischemia and Infarction, 341
Pathophysiology 341
Imaging of Cerebral Ischemia and Infarction:
Overview, 343
Acute Infarcts, 343
Subacute Infarcts, 349
Chronic Infarcts, 353
Lacunar Infarcts, 355
Hypoxic-ischemic Encephalopathy, 355
Strokes in Specific Vascular Distributions, 360
Strokes in Children, 367
Nonatheromatous Causes of Arterial Narrowing
and Occlusion, 369
Congenital Abnormalities, 369
Acquired Disorders: Vasculitis, Vasculopathy,
and Miscellaneous Lesions, 373
Stroke `Mimics", 383
Venous Occlusions, 385
PART THREE BRAIN TUMORS AND
TUMORLIKE PROCESSES
12 Brain Tumors and Tumorlike Masses: Classifi-
cation and Differential Diagnosis, 401
Classification by Histology, 402
Primary Brain Tumors, 402
Metastatic Tumors, 406
Classification by Age and General Location, 406
Primary Brain Tumors in Children, 406
Adult Primary Brain Tumors, 407
Classification and Differential Diagnosis by
Specific Anatomic Location, 408
Pineal Region Masses, 408
Intraventricular Masses, 422
Cerebellopontine Angle Masses, 437
Foramen Magnum Masses, 450
Sellar/Suprasellar Masses, 461
Skull Base and Cavernous Sinus Masses, 484
Scalp, Cranial Vault, and Meningeal Masses,
511
13 Astrocytomas and Other Glial Neoplasms, 529
Astrocytomas, 529
Astrocytoma (Low-grade astrocytoma), 531
Anaplastic (Malignant) Astrocytoma, 535
Glioblastoma Multiforme, 541
Gliomatosis Cerebri, 550
Gliosarcoma, 550
Pilocytic Astrocytoma, 553
Pleomorphic Xanthoastrocytoma, 558
Subependymal Giant Cell Astrocytoma, 561
Oligodendroglioma, 563
"Pure" Oligodendroglioma, 563
"Mixed" Oligodendroglioma (oligoastrocy-
toma), 566
Ependymoma, 566
Ependymoma, 566
Subependymoma, 570
Choroid Plexus Tumors, 571
Choroid Plexus Papilloma, 572
Choroid Plexus Carcinoma, 574
14 Meningiomas and Other Nonglial Neoplasms,
579
Mixed Glial-Neuronal and Neuronal Tumors, 579
Ganglioglioma and Ganglioneuroma, 580
Gangliocytoma, 581
Lhermitte-Duclos Disease, 581
Dysembryoplastic Neuroepithelial Tumor
(DNET), 582
Central Neurocytoma, 582
Meningeal and Mesenchymal Tumors, 584
Meningioma, 584
Nonmeningotheliomatous Mesenchymal Tu-
mors, 602
Primary Melanocytic Lesions, 602
Hemangiopericytoma, 603
Hemangioblastoma (Capillary Hemangioblas-
toma), 605
Pineal Region Tumors, 607
Germ Cell Tumors, 607
Pineal Cell Tumors, 612
Primitive Neuroectodermal Tumors, 613
Medulloepithelioma, 613
Medulloblastoma, 613
Primary Cerebral Neuroblastoma, 618
Hemopoietic Neoplasms, 620
Primary CNS Lymphoma, 620
Leukemia, 623
15 Miscellaneous Tumors, Cysts, and Metastases,
626
Tumors of Cranial and Spinal Nerves, 626
Schwannoma, 626
Neurofibroma, 630
Malignant Peripheral Nerve Sheath Tumors,
630
Nonneoplastic Tumorlike Lesions, 631
Epidermoid Tumor, 631
Dermoid Tumors, 635
Lipoma, 636
Nonneoplastic Cysts, 639
Arachnoid Cysts, 639
Colloid Cysts, 642
Rathke Cleft Cyst, 645
Neuroglial (neuroepithelial) Cysts, 646
Enterogenous Cysts, 648
Regional Tumors that Involve the CNS by Local
Extension, 649
Pituitary Adenoma, 649
Craniopharyngioma, 654
Metastatic tumors, 657
Skull and Dural Metastases, 657
Leptomeningeal Metastases, 659
Parenchymal Metastases, 660
Limbic Encephalitis, 665
PART FOUR INFECTION, WHITE MATTER
ABNORMALITIES, AND DEGENERATIVE
DISEASES
16 Infections of the Brain and Its Linings, 673
Congenital/Neonatal Infections of the Brain, 673
Cytomegalovirus, 674
Toxoplasmosis, 675
Rubella, 676
Herpes Simplex, 677
HIV Infection, 678
Varicella, 680
Enteroviruses, 680
Meningitis, 680
Acute Pyogenic Meningitis, 680
Acute Lymphocytic Meningitis, 686
Chronic Meningitis, 686
Pyogenic Parenchymal Infections, 688
Cerebritis and Abscess, 688
Complications of Cerebral Abscesses, 691
Encephalitis, 694
Herpes Simplex Encephalitis, 694
HIV Encephalitis and Other CNS Infections in
AIDS, 696
Miscellaneous Viral Encephalitides, 702
Postinfection Encephalitis, 703
Tuberculosis and Fungal Infections, 706
CNS Tuberculosis, 706
Fungal Infections, 707
Parasitic Infections, 709
Neurocysticercosis, 709
Miscellaneous Parasitic Infections, 712
17 Inherited Metabolic, White Matter, and Degen-
erative Diseases of the Brain, 716
Normal Myelination, 717
Birth (Term Infant), 718
Three Postnatal Months, 718
Six Postnatal Months, 718
Eight Postnatal Months, 718
Three Years of Age, 718
Disorders that Primarily Affect White Matter
(Leukodystrophies), 722
Metachromatic Leukodystrophy, 722
Krabbe Disease, 724
Adrenoleukodystrophy (X-linked), 725
Pelizaeus-Merzbacher Disease, 728
Alexander Disease, 730
Canavan Disease, 732
Phenylketonuria and Amino Acid Disorders,
732
Disorders that Primarily Affect Gray Matter, 736
Tay-Sachs Disease and Other Lipidoses, 736
Hurler Syndrome and the Mucopolysacchari-
doses, 737
Mucolipidoses and Fucosidosis, 739
Glycogen Storage Diseases, 739
Disorders that Affect Both Gray and White Mat-
ter, 740
Leigh Disease and Other Mitochondrial En-
cephalopathies, 740
Zellweger Syndrome and Other Proxisomal
Disorders, 743
Basal Ganglia Disorders, 743
Huntington Disease, 743
Hallervorden-Spatz Disease, 745
Fahr Disease, 745
Wilson Disease, 746
18 Acquired Metabolic, White Matter, and Degen-
erative Diseases of the Brain, 748
Normal Aging Brain, 748
White Matter, 748
Sulci, Cisterns, and Ventricles, 752
Brain Iron and the Striatonigral System, 754
Cortical Gray Matter, 755
White Matter Neurodegenerative Disorders, 755
Multiple Sclerosis, 755
Viral and Postviral Diseases, 761
Toxic Demyelination, 761
Trauma, 766
Vascular Disease, 766
Gray Matter Neurodegenerative Disorders, 772
Alzheimer Disease and Other Cortical Demen-
tias, 772
Extrapyramidal Disorders and Subcortical De-
mentias, 774
Parkinson Disease and Related Striatonigral
Degenerations, 776
Miscellaneous Cerebellar and Motor Degener-
ations, 778
PART FIVE SPINE AND SPINAL CORD
19 Normal Anatomy and Congenital Anomalies of
the Spine and Spinal Cord, 785
Normal Anatomy, 785
Lumbosacral Spine, 785
Thoracic Spine, 793
Cervical Spine, 795
Meninges and Spinal Cord, 798
Congenital Malformations of the Spine and Spi-
nal Cord, 799
Open Spinal Dysraphism, 799
Occult Spinal Dysraphism, 802
Anomalies of Abnormal Canalization and Ret-
rogressive Differentiation, 805
Split Notochord Syndromes, 811
Miscellaneous Malformations, 815
20 Nonneoplastic Disorders of the Spine and Spi-
nal Cord, 820
Infection, 820
Spondylitis and Diskitis, 820
Epidural and Subdural Infections, 824
Meningitis, Myelitis, and Cord Abscess, 826
Demyelinating Diseases, 826
Multiple Sclerosis, 826
Acute Transverse Myelopathy, 828
Miscellaneous Myelopathies, 828
Vascular Diseases, 830
Normal Vascular Anatomy, 830
Aneurysms, 832
Vascular Malformations, 832
Infarction, 834
Degenerative Diseases, 836
Normal Aging and Disk Degeneration, 836
Spondylosis, Arthrosis, and Spinal Stenosis,
840
Disk Bulges and Disk Herniation, 849
Normal Postoperative Spine, 856
"Failed Back" Syndromes, 857
Back Pain in Children, 861
Trauma, 867
Mechanisms of Spine Injury,867
Osseous Spine Injury Patterns, 867
loft Tissue Injury, 872
21 Cysts, Tumors, and Tumorlike Lesions of the
spine and Spinal Cord, 876
Extradural Tumors, Cysts, and Tumorlike
Masses, 876
Benign Tumors, 877
Cysts and Other Benign Tumorlike Masses,
884
Malignant Tumors, 887
Intradural Extramedullary Tumors, Cysts, and
Tumorlike Masses, 895
Benign Tumors, 895
Cysts and Other Benign Tumorlike Masses,
900
Malignant Tumors, 902
Intramedullary Tumors, Cysts, and Tumorlike
Masses, 906
Tumors, 906
Cysts and Tumorlike Masses, 915


