This volume in the High-Yield Pathology Series, with access to ExpertConsult.com, provides a pragmatic review of clinical and pathological features of a range of nervous system diseases. Our intent in producing this volume is not to replace several other outstanding comprehensive textbooks in the field but rather to provide quick reference via bulleted summaries of key facts that are supported by multiple representative photographs on topics of interest in the major areas of neuropathology. We have attempted to provide rapid access to a range of nervous system disorders for trainees and practitioners.A\Establishing the correct neuropathologic diagnosis routinely requires knowledge of clinical and imaging findings that allow optimal interpretation of gross, his-tological, and genetic studies. Hence, pertinent clinical and radiological features for each entity covered herein precede a summary of the pertinent pathological findings with corresponding representative photographs. It is hoped that this book will find use for trainees of all experience levels in pathology, neurology, neuro-surgery, neuroradiology, neuro-oncology, and related fields. It should also be a handy practical reference for practicing pathologists, including neuropathologists requiring quick access to the field. Anthony T. Yachnis, MD; Marie L. Rivera-Zengotita, MD

I.BASIC REACTIONS 1

A.Cerebral Edema, 2

B.Hydrocephalus, 4

C.Herniations, 6

II.DEVELOPMENTAL DISORDERS 9

A.Malformations, 10

      Neural Tube Defects, 10

      Holoprosencephaly, 12

      Posterior Fossa: Chiari Malformations, 15

      Cerebellar Vermis Malformations, 17

      Lhermitte-Duclos Disease, 19

      Neuronal Migration Defects, 21

B.Acquired Developmental Defects, 23

      Germinal Matrix Hemorrhage, 23

      Periventricular Leukomalacia, 25

      Gray Matter Lesions, 27

      PorencephaFies, 29

III.CEREBROVASCULAR DISORDERS, 31

A.Cerebral Ischemia and Hypertensive Changes, 32

      Ischemic Cerebrovascular Disease, 32

      Hypertensive Cerebrovascular Disease: Ischemic Changes, 35

      Hypertensive Cerebrovascular Disease: Spontaneous Intracerebral Hemorrhage, 37

      Hypertensive Cerebrovascular Disease: Posterior Reversible Encephalopathy Syndrome (PRES), 39

B.INTRACRANIAL ANEURYSMS, 40

      Saccular ("Berry") Aneurysms, 40

      Fusiform Aneurysms, 42

      Infective ("Mycotic") Aneurysms, 43

C.Vascular Malformations, 45

      Arteriovenous Malformations (AVM), 45

      Cavernous Angiomas (CA), 48

      Capillary Telangiectasis, 49

      Venous Angioma, 50

D.Vasculitis, 51

      Giant Cell Arteritis, 51

      Polyarteritis Nodosa (PAN), 53

      Primary CNS Angiitis, 54

E.Inherited Cerebrovascular Diseases, 55

      Cerebral Amyloid Angiopathy, 55

      Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL), 58

      Moyamoya Syndrome, 60

IV.TRAUMA, 61

A.Closed versus Open (Penetrating) Head Trauma, 62

      Contusion/Laceration (Including Coup and Contrecoup Lesions), 62

      Diffuse Axonal Injury (Diffuse Traumatic Brain Injury), 65

B.Traumatic Intracranial Hemorrhage, 67

      Epidural Hematoma, 67

      Subdural Hematoma, 68

V.BRAIN TUMORS, 71

A.Diffuse Gliomas: AStrocytic, 72

      Diffuse Astrocytoma, 72

      Anaplastic Astrocytoma, 75

      Glioblastoma, 77

      Glioblastoma Variants, 80

B.Diffuse Gliomas: Oligodendroglial, 84

      Oligodendroglioma, 84

      Anaplastic Oligodendroglioma, 87

      Mixed Glioma (Oligoastrocytoma, Anaplastic Oligoastrocytoma), 89

      Gliomatosis Cerebri, 90

C.Other Astrocytic Tumors, 91

      Pleomorphic Xanthoastrocytoma (PXA), 91

      Pilocytic Astrocytoma, 93

      Subependymal Giant Cell Astrocytoma, 96

D.Ependymomas and SUBEPENDYMOMA, 99

      Ependymoma, 99

      Anaplastic Ependymoma, 102

      Myxopapillary Ependymoma, 103

      Subependymoma, 105

E.Tumors with Ependymal-LIKE Features, 107

      Angiocentric Glioma, 107

      Chordoid Glioma, 109

      Astroblastoma, 111

F.Choroid Plexus Tumors, 112

      Choroid Plexus Papilioma, 112

      Choroid Plexus Carcinoma, 114

G.NEuronal And GLioneuronal Tumors, 115

      Ganglion Cell Tumors, 115

      Desmoplastic Infantile Astrocytoma/Ganglioglioma, 117

      Central Neurocytoma, 119

      Dysembryoplastic Neuroepithelial Tumor (DNET), 121

      Papillary Glioneuronal Tumor, 123

      Rosette-Forming Glioneuronal Tumor of the Fourth Ventricle, 125

H.Embryonal (Primitive) Neuroepithelial Tumors, 127

      Medulloblastoma, 127

      Central Nervous System-Primitive Neuroectodermal Tumor (CNS-PNET), 132

      Atypical Teratoid/Rhabdoid Tumors (AT/RT), 134

I.MeningioMas (Tumors of The MEninges), 116

      Meningiomas: Overview and General Characteristics, 136

      Meningioma: WHO Grade I Variants, 139

      Atypical Meningioma, 142

      Malignant [Anaplastic) Meningiomas, 144

      Hemangiopericytoma: Solitary Fibrous Tumor, 146

J.Nerve Sheath Tumors, 149

      Schwannoma, 149

      Neurofibroma, 152

      Perineurioma, 154

      Malignant Peripheral Nerve Sheath Tumor (MPNST), 156

K.Primary CNS Lymphoma (PCNSL), 159

L.Pineal Parenchymal Tumors, 162

      Pineocytoma, 162

      Pineal Parenchymal Tumor of Intermediate Differentiation, 164

      Pineoblastoma, 166

      Papillary Tumor of the Pineal Region, 167

M.Germ Cell Tumors of the Central Nervous System, 168

      Germinoma, 168

      Other Germ Cell Tumors, 170

N.Hemangioblastoma, 172

O.Sellar and Suprasellar Tumors, 175

      Pituitary Adenomas, 175

      Prtuicytoma, 179

      Craniopharyngioma, 181

      Rathke Cleft Cyst, 183

      Langerhans Cell Histiocytosis, 184

P.Primary Melanocytic Tumors of the Central Nervous System, 186

Q.Central Nervous System Cysts, 188

R.Metastatic Brain Tumors, 191

S.Tumors of the Skull Base, 194

      Chordoma of Skull Base, 194

      Chondrosarcoma of Skull Base, 197

VI.INFECTIOUS DISEASES, 199

A.Bacterial Infections, 200

      Acute Purulent Meningitis, 200

      Cerebral Bacterial Abscess, 202

      Nacardia/Actinomyces Species, 204

      Mycobacterial Infection, 206

      Neurosyphilis, 208

B.Mycoses, 210

      Cerebral Cryptococcosis, 210

      Aspergillosis, 212

      Mucormycosis, 214

      Candidiasis, 215

C.Parasitic Infections, 216

      Cerebral Toxoplasmosis, 216

      Cerebral Cysticercosis, 218

      Primary Amoebic Meningoencephalitis, 220

      Granulomatous Amoebic Encephalitis, 222

      Cerebral Malaria, 224

D.Viral Infections, 225

      General Viral Effects on the Central Nervous System, 225

      Herpes Simplex Encephalitis, 227

      Cytomegalovirus Encephalitis, 229

      Rabies Encephalitis, 230

      Arboviruses, 231

      Progressive Multifocal Leukoencephalopathy, 232

E.Neuropathology of AIDS, 235

      Primary Central Nervous System HIV Infection, 235

      HIV-Associated Vacuolar Myelopathy (HAM), 236

      Opportunistic CNS Infections in AIDS, 237

      CNS Immune Reconstitution Inflammatory Syndrome (Neuro-IRIS), 238

F.PrIon Diseases, 239

      Prion-Related Diseases (Overview), 239

      Creutzfeldt-Jakob Disease, 240

      Other Prion-Related Diseases, 242

VII.NEURODEGENERATIVE DISORDERS, 243

A.ALZHEIMER'S DISEASE, 244

B.FrontoteMporal LoBar Degenerations and Related Tauopathies, 247

      Corticobasal Degeneration (Rebeiz Disease), 247

      Progressive Supranuclear Palsy (PSP: Steele-Richardson-Olszewski Syndrome), 250

      Pick Disease, 252

      Frontotemporal Lobar Degeneration: Frontotemporal Dementia and Parkinsonism Linked to Chromosome 17 (FTDP-17), 255

C.Frontotemporal Lobar Degenerations with Ubiouitin-InclUsions (FTLD-U and FTLD-MND) (TDP-43), 256

D.Parkinsons Disease and Related Alpha-SynucleInopathIes, 258

      Parkinson's Disease, 258

      Dementia with Lewy Bodies, 261

      Multiple System Atrophy (MSA), 263

E.Amyotrophic Lateral Sclerosis (ALS), 265

F.Triple-Repeat Inherited Neurodegenerations, 267

      Huntington Disease, 267

      Autosomal Recessive Spinocerebellar Degeneration (Friedreich's Ataxia), 269

      Autosomal Dominant Spinocerebellar Ataxias (SCA), 270

      Fragile X Tremor/Ataxia Syndrome (FXTAS), 271

G.NeUroaxonal Dystrophies, 273

      Neurodegeneration with Brain Iron Accumulation Type 1 (NBIA 1), 273

      Other Inherited Neuroaxonal Dystrophies, 275

H.Vascular Dementia and Binswanger Disease, 276

VIII.DEMYELINATING DISEASES, 279

A.Multiple Sclerosis, 280

      Multiple Sclerosis, 280

      Neuromyelitis Optica Spectrum Disorders, 283

B.Acute Disseminated Encephalomyelitis, 285

C.Acute Hemorrhagic Leuicoencephalitis (Hurst Disease), 287

D.tumefactive demyelinating lesions (TDL), 289

IX.TOXIC, NUTRITIONAL, METABOLIC DISEASES, 291

A.Toxic Injury, 292

      Toxic Leukoencephalopathy, 292

      Carbon Monoxide, 294

      Ethanol-Related Injury (Including Superior Vermis Atrophy and Central Pontine Myelinolysis), 296

      Hepatic Encephalopathy, 298

B.Nutritional Diseases, 299

      Wernicke-Korsakoff Disease, 299

      Subacute Combined Degeneration, 300

C.Metabolic Diseases, 302

      Neuronal Storage Diseases, 302

      Leukodystrophies, 303

      Neuronal Ceroid Lipofuscinosis, 305

      Wilson Disease (Hepatolenticular Degeneration),307

      Alexander Disease, 309

X.NEUROMUSCULAR DISORDERS, 311

A.Peripheral Nerve Disease, 312

      Basic Reactions in Peripheral Nerve Disorders, 312

      Immune-Mediated Inflammatory Neuropathies, 315

      Charcot-Marie-Tooth Disease and Related Congenital Neuropathies, 317

B.Skeletal Muscle Diseases, 319

      Essential Features: Myopathic versus Neurogenic Changes, 319

      Dermatomyositis, 322

      Polymyositis, 324

      Inclusion Body Myopathy and Myositis, 325

      Dystrophinopathies (Duchenne and Becker Muscular Dystrophies), 327

      Myotonic Dystrophies, 329

      Periodic Paralyses, 331

      Limb-Girdle Muscular Dystrophies, 333

      Nemaline Myopathy, 336

      Central Core Disease, 338

      Pompe Disease, 339

      McArdle Disease, 341

      Mitochondrial Myopathies, 343

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