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Case-Based Brain Imaging
Second Edition

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Case-Based Brain Imaging
Second Edition

A. John Tsiouris, MD

Associate Professor of Clinical Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital

New York, New York

Pina C. Sanelli, MD, MPH

Associate Professor of Radiology and Public Health
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Joseph P. Comunale, MD

Associate Professor of Clinical Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Thieme
New York • Stuttgart

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Library of Congress Cataloging-in-Publication Data
Case-based brain imaging / edited by A. John Tsiouris, Pina C. Sanelli, Joseph P. Comunale. — 2nd ed.
p. ; cm.

Rev. ed. of: Teaching atlas of brain imaging / Nancy J. Fischbein, William P. Dillon, A. James Barkovich.
2000.
Includes bibliographical references and index.
ISBN 978-1-60406-953-2
I. Tsiouris, A. John. II. Sanelli, Pina C. III. Comunale, Joseph P. IV. Fischbein, Nancy J. Teaching atlas
of brain imaging.
[DNLM: 1. Brain Diseases—diagnosis—Atlases. 2. Brain Diseases—diagnosis—Case Reports.
3. Diagnostic Imaging—Atlases. 4. Diagnostic Imaging—Case Reports. WL 17]
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To our patients, who are an infinite source of challenging cases that motivate us to continuously
improve our knowledge and skills.

I dedicate this book to my father, Dr. John A. Tsiouris, for the many sacrifices he made throughout his life
so my brother and I could succeed.
Apostolos John Tsiouris, MD

I dedicate this book to my loving and supportive husband, George, and to our three children, Isabella,
Sophia, and Nicholas, who are truly our pride and joy.
Pina C. Sanelli, MD, MPH

I dedicate this book to my parents, for their unconditional support and encouragement, and to my
colleagues, residents, and fellows, who continue to motivate me to be the best I can be.
Joseph P. Comunale, MD

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Contents
Foreword by Robert D. Zimmerman, MD, FACR . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xi
Preface. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .xiii
Acknowledgments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiv
Contributors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xv
Abbreviations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xvi
Section I. Neoplasms
IA. Supratentorial
Case 1 Low-grade Astrocytoma (WHO Grade II) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3
Case 2 Anaplastic Astrocytoma (WHO Grade III) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7
Case 3 Glioblastoma Multiforme (WHO Grade IV) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12
Case 4 Oligodendroglioma (WHO Grade II or III) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16
Case 5 Central Neurocytoma (WHO Grade II) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21
Case 6 Ganglioglioma (WHO Grade I–III) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27
Case 7 Gliomatosis Cerebri (WHO Grade IV) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
Case 8 Metastatic Breast Cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 36
Case 9 Dural Metastasis from Stage IV Breast Cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 42
Case 10 Lymphomatous Meningitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
Case 11 Primary CNS Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 51
Case 12 Dysembryoplastic Neuroepithelial Tumor (WHO Grade I) . . . . . . . . . . . . . . . . . . . 57
Case 13 Ependymoblastoma (WHO Grade IV) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60
Case 14 Pineocytoma (WHO Grade I) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65
Case 15 Pineoblastoma (WHO Grade IV) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 68
Case 16 Pineal Region Germinoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72
Case 17 Pituitary Microadenoma (WHO Grade I) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77
Case 18 Pituitary Macroadenoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 82
Case 19 Rathke Cleft Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87
Case 20 Craniopharyngioma (WHO Grade I) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90
Case 21 Meningioma (WHO Grade I) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94
Case 22 Subependymoma of Fourth Ventricle (WHO Grade I) . . . . . . . . . . . . . . . . . . . . . . . 99
Case 23 Choroid Plexus Papilloma (WHO Grade I) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 103
Case 24 Arachnoid Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 107
Case 25 Dermoid Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 111
Case 26 Mature Pineal Teratoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 115
Case 27 Colloid Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 119
Case 28 Neurenteric Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123
Case 29 Lipoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127
Case 30 Psammomatoid Ossifying Fibroma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 130

vii

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viii

CONTENTS

IB. Infratentorial
Case 31 Juvenile Pilocytic Astrocytoma (WHO Grade I) . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137
Case 32 Tectal Glioma (WHO Grade I or II) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 141
Case 33 Brainstem Glioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145
Case 34 Medulloblastoma (WHO Grade IV) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151
Case 35 Ependymoma (WHO Grade II or III) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155
Case 36 Vestibular Schwannoma (WHO Grade I) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161
Case 37 Epidermoid Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
Section II. Inflammatory Diseases
IIA. Infectious
Case 38 Herpes Simplex Virus Type I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 173
Case 39 Bacterial Meningitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 177
Case 40 Acute Cerebellitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 184
Case 41 Brain Abscess . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 188
Case 42 Subdural Empyema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194
Case 43 Neurocysticercosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 199
Case 44 Tuberculosis Meningitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 203
Case 45 Fungal (Aspergillosis) Abscess . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 207
Case 46 HIV Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 211
Case 47 Progressive Multifocal Leukoencephalopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 215
Case 48 CNS Toxoplasmosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 220
Case 49 Cryptococcal Meningitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 224
IIB. Non-Infectious
Case 50 Systemic Lupus Erythematosus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 231
Case 51 Langerhans Cell Histiocytosis. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 237
Case 52 Mesial Temporal Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 242
Case 53 Neurosarcoidosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 246
Case 54 Lymphocytic Hypophysitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 251
Case 55 Intracranial Hypotension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 255
Section III. Cerebrovascular Diseases
Case 56 Aneurysmal Subarachnoid Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 261
Case 57 Giant Aneurysm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 267
Case 58 Mycotic Aneurysm. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 272
Case 59 Perimesencephalic Nonaneurysmal Subarachnoid Hemorrhage . . . . . . . . . . . . . 277
Case 60 Middle Cerebral Artery Embolus and Acute Infarction . . . . . . . . . . . . . . . . . . . . . 281
Case 61 Watershed Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 287
Case 62 Basilar Artery Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 292
Case 63 Arterial Dissection . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 297
Case 64 Hypertensive Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 301
Case 65 Global Anoxic Brain Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 306
Case 66 Cavernous Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 312
Case 67 Arteriovenous Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 316
Case 68 Developmental Venous Anomaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 322
Case 69 Carotid Cavernous Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 326
Case 70 Dural Arteriovenous Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 331
Case 71 Primary Angiitis of the CNS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 336

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CONTENTS

Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case

72
73
74
75
76
77
78
79
80
81
82

Case 83
Case 84
Section IV.
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case
Case

ix

Fibromuscular Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 342
Periventricular Leukomalacia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 347
Neonatal Hypoxic-Ischemic Encephalopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 352
Moyamoya Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 358
Vein of Galen Aneurysmal Malformation. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 364
Sickle Cell Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371
Transverse Venous Sinus Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 377
Superficial Siderosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 382
Vasospasm . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 387
Primary Cerebral Amyloid Angiopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 391
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarctions
and Leukoencephalopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 396
Isolated Cortical Vein Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 401
Ataxia-Telangiectasia. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 405

Neurodegenerative/White Matter Diseases/Metabolic
85 Multiple Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 411
86 Tumefactive Multiple Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 416
87 Acute Disseminated Encephalomyelitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 421
88 Osmotic Demyelination Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 425
89 Reversible Postictal Cerebral Edema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 428
90 Carbon Monoxide Poisoning . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 431
91 Metachromatic Leukodystrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 434
92 X-Linked Adrenoleukodystrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 437
93 Krabbe Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 440
94 Pelizaeus-Merzbacher Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 443
95 Metronidazole-induced Encephalopathy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 446
96 Amyotrophic Lateral Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 449
97 Creutzfeldt-Jakob Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 453
98 Pantothenate Kinase-associated Neurodegeneration . . . . . . . . . . . . . . . . . . . . . . 456
99 Multiple System Atrophy–Cerebellar Type . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 459
100 Alzheimer Dementia Complex . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 462
101 Multi-Infarct Dementia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 466
102 Wernicke Encephalopathy. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 469
103 Parry-Romberg Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 473

Section V. Trauma
Case 104 Traumatic Subarachnoid Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 479
Case 105 Epidural Hematoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 484
Case 106 Subdural Hematoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 488
Case 107 Diffuse Axonal Injury (DAI) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 492
Case 108 Traumatic Parenchymal Hemorrhagic Contusion . . . . . . . . . . . . . . . . . . . . . . . . . 497
Case 109 Nonaccidental Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 501
Case 110 Subfalcine and Uncal Herniation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 505
Case 111 Leptomeningeal Cyst Associated with a Skull Fracture . . . . . . . . . . . . . . . . . . . . 510
Section VI. Congenital/Developmental Malformations and Syndromes
VIA. Supratentorial
Case 112 Agenesis of the Corpus Callosum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 517
Case 113 Alobar Holoprosencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 522

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x

CONTENTS

Case
Case
Case
Case
Case
Case
Case
Case

114
115
116
117
118
119
120
121

Hydranencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 527
Septo-Optic Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 530
Frontoparietal Encephalomeningocele . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 533
Hamartoma of the Tuber Cinereum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 538
Benign Enlargement of the Subarachnoid Spaces of Infancy . . . . . . . . . . . . . . . 541
Porencephalic Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 544
Sturge-Weber Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 548
Neurocutaneous Melanosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 553

VIB. Infratentorial
Case 122 Chiari I Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 559
Case 123 Chiari II Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 564
Case 124 Chiari III Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 569
Case 125 Dandy-Walker Spectrum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 571
Case 126 Dysplastic Cerebellar Gangliocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 576
Case 127 Rhombencephalosynapsis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 580
VIC. Malformations of Cortical Development
Case 128 Hemimegalencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 587
Case 129 Subependymal Nodular Heterotopia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 591
Case 130 Band Heterotopia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 595
Case 131 Classic (Type I) Lissencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 600
Case 132 Polymicrogyria . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 605
Case 133 Schizencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 609
Case 134 Focal Cortical Dysplasia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 613
VID. Phakomatoses
Case 135 Neurofibromatosis Type I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 619
Case 136 Neurofibromatosis Type II . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 625
Case 137 Tuberous Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 629
Case 138 Von Hippel-Lindau Disease (Hemangioblastoma) . . . . . . . . . . . . . . . . . . . . . . . . . 634
Section VII. Cranial Nerves
Case 139 Olfactory Neuroblastoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 641
Case 140 Optic Neuritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 644
Case 141 Optic Nerve Glioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 646
Case 142 Optic Nerve Sheath Meningioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 649
Case 143 Pseudotumor of the Cavernous Sinus (Tolosa-Hunt Syndrome) . . . . . . . . . . . . 651
Case 144 Vascular Compression . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 654
Case 145 Trigeminal Nerve Schwannoma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 656
Case 146 Cavernous Sinus Thrombosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 658
Case 147 Bell’s Palsy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 661
Case 148 Hemangioma of the Facial Nerve Canal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 663
Case 149 Perineural Spread of Parotid Adenoid Cystic Carcinoma . . . . . . . . . . . . . . . . . . 665
Case 150 Meningioma of Jugular Foramen . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 667
Case 151 Lateral Medullary Acute Infarction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 670
Case 152 Glomus Jugulare Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 673

Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 675

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Foreword

The second edition of the popular Teaching Atlas of Brain Imaging by Drs. Fischbein, Dillon and
Barkovich has finally been produced after a hiatus of 12 years, now renamed Case-Based Brain
Imaging. The wait was clearly worth it!
The editors of this second edition, Drs. Tsiouris, Comunale, and Sanelli, are all my colleagues at
NewYork-Presbyterian, Weill Cornell Medical College. They are outstanding clinicians and teachers
who have used their combined experience and expertise to carefully choose 152 first-rate CT and
MRI cases that illustrate the key imaging features of the full spectrum of brain disease in an easy-toaccess format. The result is a book that is both comprehensive and concise. Each chapter starts with
an unknown case. In many of the chapters, additional companion images and cases are provided to
enhance the reader’s knowledge of the topic and demonstrate variations of the profiled disease. The
key imaging, pathologic, and pathophysiologic findings for each disease are clearly outlined for each
case. I especially appreciate the Pearls and Pitfalls sections at the end of each case that summarize
wise tips for the reader.
Since the first edition, there have been major advances in the CT and MR imaging techniques
utilized in neuroradiology. Numerous cases in this text include imaging techniques such as CT angiography, MR angiography, CT and MR perfusion, and MR spectroscopy that are now commonly used
in practice for the diagnosis and surveillance of CNS disease. The all-new images are spectacular,
having been obtained on state-of-the-art CT and high field MR scanners. The discussions are clear
and concise, and the references have all been updated and the cases presented in a clean and uncluttered layout.
This book is meant to provide trainees and practicing radiologists, neurologists, and neurosurgeons with an opportunity to learn quickly about entities they encounter in their daily clinical practice, and it succeeds in this mission admirably. If you see it in practice, it is included in this book.
It also includes numerous rare zebra cases that can cause diagnostic dilemmas. Lastly, this excellent
text provides the reader with the opportunity to test their skills in the interpretation of unknown
cases. For me this is the guilty pleasure of this book. Let’s face it, radiologists love visual puzzles.
The enduring popularity of case-of-the-day presentations, unknown case sessions, and film panels
at our national meetings speaks to this love. This book offers each of us the opportunity to test our
knowledge on representative cases and, in the process, gain significant information about a variety
of entities.
I believe this book will be enormously useful to the reader interested in the imaging of CNS
diseases. I congratulate my colleagues on their innumerable hours of work on the production of this
outstanding case-based review. I hope you enjoy reading it as much as I did.
Robert D. Zimmerman, MD, FACR
Professor of Radiology and Vice Chair for Education
Weill Cornell Medical College
New York, New York

xi

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Preface

Since the first edition of the Teaching Atlas of Brain Imaging by Drs. Fischbein, Dillon, and Barkovich
published over 10 years ago, imaging of the brain has undergone tremendous changes with marked
improvements in both spatial and contrast resolution. New computed tomography (CT) scanners
are equipped with more detectors to provide faster acquisition of images with improved resolution
and decreased movement artifacts. These advantages have not only further developed anatomic and
functional imaging techniques but have also allowed the translation of CT angiography and perfusion
in clinical practice. CT angiography has all but replaced catheter digital subtraction angiography as
the initial imaging modality for the diagnosis of most cerebrovascular diseases. Magnetic resonance
(MR) has also drastically improved, with an appreciable increase in image resolution related to newer
software, faster gradients, and receiver coil improvements as well as higher magnet field imaging.
New MR techniques such as perfusion, spectroscopy, and diffusion tensor imaging have matured and
are now being implemented in the diagnosis and preoperative planning in the clinical setting.
The first edition was a very well-organized and immensely useful teaching atlas and review for
the radiology resident and neuroradiology fellow. It contained clear examples of the most commonly
encountered pathologies in brain imaging as well as outstanding examples of the “zebras” in neuroradiology. As was the case with the first edition, we believe that the updated images and information in this book will be immensely beneficial to all radiology residents, practicing radiologists, and
neuroradiologists. Neurologists and neurosurgeons in training and practice will also benefit from
this text, because imaging has become an indispensable part of their practice for the diagnosis and
treatment of CNS diseases.
This book is composed of seven sections covering the entire spectrum of brain diseases. The
sections are organized by pathology and cover brain neoplasms; infectious and inflammatory diseases; cerebrovascular diseases; neurodegenerative, white matter, and metabolic diseases; traumatic
brain injury; congenital, developmental malformations and syndromes; and the cranial nerves. In the
preparation of this second edition, we maintained the format of the first edition, but included all new
updated images and references. The outline format with key bulleted facts allows for a quick review
of the presented pathologies. Novel and emerging imaging techniques are included when pertinent
to aiding in diagnosis. As in the prior edition, each case includes important pearls and pitfalls for
diagnosis of the pathologies presented.
It is our sincere hope and expectation that this book will be a valuable tool for the diagnosis of brain diseases for the resident, fellow, and attending physician in radiology, neurology, and
neurosurgery.

xiii

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Acknowledgments

The three editors would like to wholeheartedly thank all the contributing authors, who were predominately composed of an industrious group of our residents and fellows at the NewYork-Presbyterian
Hospital–Weill Cornell Medical College. Without all their hard work and dedication, this book would
not have been remotely possible. In particular, Drs. Andrew Schweitzer, Janice Jeon, Sahil Sood, Ajay
Gupta, and Rachel Gold contributed a large number of cases.

xiv

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Contributors

Matthew Amans, MD, MSc
Department of Radiology and Biomedical Imaging
University of California, San Francisco
San Francisco, California

Jan Christopher Mazura, MD
Department of Radiology and Biomedical Imaging
University of California, San Francisco
San Francisco, California

J. Levi Chazen, MD
Department of Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Sarah Sarvis Milla, MD
Department of Radiology
NYU Langone Medical Center
New York, New York
Daniel G. Rosenbaum, MD
Department of Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Peiweng Chen, MD
Department of Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Pina C. Sanelli, MD, MPH
Associate Professor of Radiology and Public Health
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Joseph P. Comunale, MD
Associate Professor of Clinical Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Andrew Damien Schweitzer, MD
Department of Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Rachel Gold, DO
North Shore University Hospital
New York, New York
Edward D. Greenberg, MD
Interventional Neuroradiologist
Fairfax Radiological Consultants, PC
Inova Fairfax Hospital
Fairfax, Virginia

Jennifer Shih, MD
Department of Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Ajay Gupta, MD
Department of Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Sahil Sood, MD
Neuroradiologist
Valley Radiology Medical Associates, Inc.
Los Gatos, California

Jana Ivanidze, MD, PhD
Department of Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

A. John Tsiouris, MD
Associate Professor of Clinical Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

Janice Y. Jeon, MD
Department of Radiology
Weill Cornell Medical College
NewYork-Presbyterian Hospital
New York, New York

xv


Abbreviations

ADC: apparent diffusion coefficient
CECT: contrast-enhanced CT
DSA: digital subtraction angiography/angiogram
DTI: diffusion tensor imaging
DWI: diffusion-weighted imaging
EPI: echo-planar imaging
GRE: gradient recall echo
NCCT: noncontrast CT
MRS: magnetic resonance spectroscopy
PWI: perfusion-weighted imaging
SPGR: spoiled gradient recall echo
SWI: susceptibility-weighted imaging
T1W: T1-weighted
T1W FLAIR: T1-weighted fluid-attenuated inversion recovery
T2W: T2-weighted
T2W FLAIR: T2-weighted fluid-attenuated inversion recovery
TOF: Time-of-flight

xvi

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Section I

Neoplasms
A. Supratentorial

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Case 1
Clinical Presentation
A 25-year-old man presents with a generalized tonic-clonic seizure; he reports an increasing number of headaches over the past 6 months.

Radiologic Findings

A

B
Fig. 1.1 (A, B) Axial T2W fluid-attenuated inversion recovery
(FLAIR) and T1W postcontrast images demonstrate a well-circumscribed nonenhancing homogeneously T1 hypointense and
T2 hyperintense mass within the medial left frontal lobe. There is
involvement of both the white matter and overlying cortex with
local mass effect and effacement of adjacent sulci. There is no
surrounding vasogenic edema. A serpentine flow void within the
lesion is most consistent with a vessel. Incidental note is made of
a nonspecific T2 hyperintense white matter focus posterior to the
mass that is most likely unrelated. (C) Coronal T1W postcontrast
spoiled gradient recalled echo (SPGR) image also demonstrates
no significant enhancement within this mass. There is mass effect upon the corpus callosum and the left lateral ventricle.

C

Diagnosis
“Low-grade” astrocytoma (WHO grade II)

Differential Diagnosis


GradeIIIanaplasticastrocytoma(typicallymoreinfiltrative,morelikelytobeassociatedwithsurroundingedema,cysticchange,hemorrhage,orcalcifications,butgenerallyindistinguishableonimaging)

3

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4

CASE-BASED BRAIN IMAGING



Cerebral infarction (usually wedge-shaped, restricted diffusion in acute stage, chronic infarction
will have volume loss)
Oligodendroglioma(oftencalcifiedandcorticallybased)
Cerebritis(usuallypatchyenhancement,acutesymptomonset,fever)
Ganglioglioma(oftencontainscystsorcalcification,hasapredilectionforthetemporallobe,mural
nodule)
Statusepilepticus(transientvasogenic/cytotoxicedema,restricteddiffusion,gyriformpattern)






Discussion
Background
Astrocytomas are primary brain tumors of astrocytic origin. Most primary brain tumors in adults arise
supratentorially; ,50% of these are gliomas and ,90% of gliomas are astrocytomas. Ten to 15% of astrocytomas are “low grade.” These lesions are most commonly located in the frontal or temporal lobes.
They most often occur between the ages of 20 and 45 and have a slight male predilection.

Clinical Findings
Low-gradeastrocytomasusuallypresentinyoungadults,andthesymptomsvarywithtumorlocation.
Headache,seizures,andfocalneurologicdeficitsarecommon.

Pathology
Arisefromdifferentiatedastrocytesorastrocyticprecursors.AstrocytomasaredividedintogradesIto
IVaccordingtotheWorldHealthOrganization(WHO)classification:





GradeI:juvenilepilocyticastrocytoma,benign
GradeII:fibrillary/diffuseastrocytoma
GradeIII:anaplasticastrocytoma
GradeIV:glioblastomamultiforme

Grades II–IV constitute the malignant gliomas, with increasing malignancy corresponding to higher
grades.Gradingisusefultoassessprognosisandguidemanagement.Themostmalignantpathological
tissue submitted for evaluation determines the ultimate tumor grade. Many tumors are also mixed
with oligodendrocytic components (Fig. 1.2A-F).

Gross




Diffuselyinfiltratingmasswithlossofgray-whitedistinction
Localmildmasseffect
Rarelycysticchangeorcalcifications

Microscopic





Well-differentiatedneoplasticastrocytes;fibrillary,gemistocytic,andprotoplasmicsubtypes
Moderatelyincreasedcellularity
Generalabsenceofmitoticactivity
Nomicrovascularinvasionorproliferation

Imaging Findings
Despiteoftenappearingwellcircumscribedonimaging,thetumormarginsareinfiltrativeandneoplastic cells are invariably found within areas of normal magnetic resonance (MR) signal. Tumor

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I NEOPLASMS–SUPRATENTORIAL

A

5

B

D

C

E
Fig. 1.2 Grade II oligoastrocytoma with mild interval growth at 3 years. (A–C) Axial T2W FLAIR, T2W,
and GRE images demonstrate a homogeneous wellcircumscribed T2 hyperintense lesion that involves
cortex and subcortical white matter within the posterior right frontal lobe. There is no surrounding vasogenic edema and minimal mass effect upon adjacent
sulci. Lack of internal susceptibility effect on the GRE

Tsiouris_CH01.indd 5

F
confirms absence of internal calcifications or hemorrhage. This lesion is hypointense and nonenhancing on the
(D) sagittal T1W postcontrast image. After 3 years, the
(E, F) sagittal postcontrast T1W and axial T2W FLAIR
images reveal stable MR signal characteristics, but mild
interval growth. Note that the tumor extends to involve
more of the right precentral gyrus cortex and has slightly more mass effect in F (arrow).

11/8/12 10:59 PM


6

CASE-BASED BRAIN IMAGING

predominatelyinvolveswhitematterbutoftenextendstocortexordeepgraymatterstructures.These
lesions may grow slowly over time (Fig. 1.2A-F).

Computed Tomography



Ill-definedlowdensitymassonnoncontrastscan
Little or no contrast enhancement, avid enhancement should raise suspicion for higher grade
neoplasm or infectious etiology

Magnetic Resonance






Masslesionwithlow-signalintensityonT1Wimage,andhigh-signalintensityonT2Wimage
Generallylackcontrastenhancement
Littleornosurroundingedema
Veryrarelycysticdegenerationand/orhemorrhage
Absenceofrestricteddiffusion

Treatment



Initial:surgicalexcision,ifaccessible,oftenfollowedbyradiationtherapy
Recurrentdisease:oftenofhighergrade(“de-differentiation”)
– Re-resection
– Stereotacticradiosurgery
– Chemotherapy

Prognosis



The major cause of mortality from a low-grade astrocytoma is differentiation into a high-grade
astrocytoma,whichhasameantimeofoccurrenceof4to5years.
Youngerage,grosstotalresection,andlongcourseofpreoperativesymptomsareassociatedwith
longer survival. Most series report median survivals of 6 to 10 years.

PEARLS




Considerthisdiagnosisforwell-circumscribedintra-axialT2hyperintenselesions
Closelyfollowwithmagneticresonanceimaging(MRI)forsignsofde-differentiationintohigher
grade(newenhancement,hemorrhage,ornecrosis)

PITFALLS





Alow-gradeastrocytomamaybemistakenforinfarction,soconsiderafollow-upscan.Acuteinfarctionsevolveoverashortperiodoftime,whereaslow-gradetumorsremainstable.Alternatively,
diffusion-weightedimagingisusefultodifferentiateacuteinfarctionfromtumor.
Enhancementcharacteristicsareunreliableforgrading:tissuecorrelationisrequired.

Suggested Readings
RicciPE,DunganDH.Imagingoflow-andintermediate-gradegliomas.SeminRadiatOncol2001;11(2):103–112Review
Scarabino T, Giannatempo GM, Nemore F, Popolizio T, Stranieri A. Supratentorial low-grade gliomas. Neuroradiology.
JNeurosurgSci2005;49(3):73–76Review
Walker DG, Kaye AH. Diagnosis and management of astrocytomas, oligodendrogliomas and mixed gliomas: a review.
AustralasRadiol2001;45(4):472–482Review

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Case 2
Clinical Presentation
A 23-year-old man presents with new onset seizure.

Radiologic Findings

A

B

Fig. 2.1 The (A) axial T2W image demonstrates a
well-demarcated intra-axial mass that is hyperintense compared with brain parenchyma, centered in
the left lateral temporal lobe. No surrounding edema
is noted. (B, C) Axial and coronal T1W postcontrast
images demonstrate that the mass is hypointense
to brain parenchyma with no appreciable internal
enhancement. Note the mild mass effect associated
with this lesion, with subtle partial effacement of the
left sylvian fissure as compared with the right side.

C

Diagnosis
Anaplastic astrocytoma (WHO grade III)

Differential Diagnosis


Grade II astrocytoma (uncommonly surrounded by edema, but generally indistinguishable on
imaging)

7

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8

CASE-BASED BRAIN IMAGING



Glioblastoma multiforme (GBM) (typically enhances, with regions of necrosis, hemorrhage, and
moreextensiveadjacentT2signalabnormality)
Oligodendroglioma(oftenhasinternalcalcification)
Ganglioglioma(oftencontainscystsorcalcification,hasapredilectionforthetemporallobe)




Discussion
Background
Anastrocytomaisaprimarybraintumorofastrocyticcellorigin.Mostprimarybraintumorsinadults
arise supratentorially: 50% of these are gliomas, and 90% of gliomas are astrocytomas. Anaplasticastrocytomas(WHOgradeIII)compriseaboutone-thirdofallgliomasandhaveimagingfindings
inbetweenlow-gradediffuseastrocytomas(WHOgradeII)andGBM(WHOgradeIV).Theselesions
aremostcommonlylocatedinthehemisphericwhitematter,especiallyinthefrontalandtemporal
lobes.Althoughtheselesionsmayappearwelldemarcatedonimaging,theyalmostalwaysinfiltratethe
adjacentnormalappearingbrain.

Clinical Findings
Anaplasticastrocytomasusuallypresentinyoungtomiddleagedadults,andthesymptomsvarywith
tumorlocation.Headache,seizures,andfocalneurologicdeficitsarecommon.

Pathology
AstrocytomasaredividedintogradesItoIVaccordingtotheWHOclassification:





GradeI:juvenilepilocyticastrocytoma,benign
GradeII:fibrillary/diffuseastrocytoma
GradeIII:anaplasticastrocytoma
GradeIV:glioblastomamultiforme

Grades II–IV constitute the malignant gliomas, with increasing malignancy corresponding to higher
grades.Gradingisusefultoassessprognosisandguidemanagement.Themostmalignantpathological
tissuesubmittedforpathologicevaluationdeterminestheultimatetumorgrade.

Imaging Findings
Typicalimagingfindingsofanaplasticastrocytomas(WHOgradeIII)includethefollowing.

Computed Tomography



Lowdensitymassonnoncontrastscan
Littleornocontrastenhancement

Magnetic Resonance



Tsiouris_CH02.indd 8

Masslesionwithlow-signalintensityonT1Wimage,andhomogenouslyhigh-signalintensityon
T2W image
Generallylackscontrastenhancement;patchy,focal,ornodularenhancementarelesscommonbut
can occur (Figs. 2.1C and 2.2A,B)

11/8/12 11:09 PM


I NEOPLASMS–SUPRATENTORIAL






9

Littleornosurroundingedema
Cysticdegenerationandcalcificationarerare
DynamicsusceptibilityT2*MRperfusionmapsshowanincreasedregionalcerebralbloodvolume
(rCBV)relativetolow-gradeastrocytomas
MR spectroscopy demonstrates elevated choline/creatine (Cho/Cr) and choline/N-acetylaspartate
(Cho/NAA)ratioswithdecreasedabsoluteNAA

Treatment



Initial:surgicalexcision,ifaccessible,oftenfollowedbyradiationtherapy
Recurrentdisease:oftenofahighergradehistologically(“de-differentiation”intoGBM)
– Re-resection
– Stereotacticradiosurgery
– Chemotherapy

A

C

Tsiouris_CH02.indd 9

B

Fig. 2.2 Anaplastic astrocytoma. (A) Axial T2W image
demonstrates a well-circumscribed hyperintense lesion
centered in the left temporal lobe with internal regions of
cystic change (arrow). Note the relatively well-circumscribed
margins of the mass with minimal adjacent T2 signal abnormality. (B, C) Axial and coronal T1W postcontrast images
demonstrate patchy regions of enhancement (arrows), a
less common imaging finding associated with these tumors.

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10

CASE-BASED BRAIN IMAGING

A

B

C

D

E

Tsiouris_CH02.indd 10

Fig. 2.3 (A, B) Anaplastic astrocytoma within the
right temporal lobe and insula. (C) A multivoxel threedimensional MR spectroscopy was performed of the
tumor to assess for tumor grade and biopsy planning.
(D, E) Overlay Cho/NAA and Cho/Cr color ratio maps were
generated to assess for the optimal biopsy site, with the
central region demonstrating the highest Cho/NAA and
Cho/Cr ratios targeted intraoperatively (arrows).

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I NEOPLASMS–SUPRATENTORIAL

11

Prognosis



Majorcauseofmortalityfromananaplasticastrocytomaisde-differentiationintoaGBM,occurring
in 2 years in most patients
Mediansurvivalfromdiagnosisis2to3years

Suggested Readings
ProvenzaleJM,WangGR,BrennerT,PetrellaJR,SorensenAG.Comparisonofpermeabilityinhigh-gradeandlow-grade
braintumorsusingdynamicsusceptibilitycontrastMRimaging.AJRAmJRoentgenol2002;178(3):711–716
ThurnherMM.WorldHealthOrganizationclassificationoftumoursofthecentralnervoussystem.CancerImaging2009;9
SpecNoA:S1–3
YoungRJ,KnoppEA.BrainMRI:tumorevaluation.JMagnResonImaging2006;24(4):709–724Review

Tsiouris_CH02.indd 11

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