Neurology Image-Based Clinical Review Neurology Image-Based Clinical Review Jonathan Howard, MD Assistant Professor of Neurology and Clerkship Director Clinical Neurological Sciences NYU Langone Medical Center New York, New York Anuradha Singh, MD Clinical Associate Professor Chief of Neurology Director of Clinical Neurophysiology/EEG Laboratory Bellevue Epilepsy Center Bellevue Hospital Center New York, New York NEW YORK Visit our website at www.demosmedical.com ISBN: 9781620701034 e-book ISBN: 9781617052804 Acquisitions Editor: Beth Barry Compositor: Exeter Premedia Services Private Ltd. Copyright © 2017 Springer Publishing Company. Demos Medical Publishing is an imprint of Springer Publishing Company, LLC. Several of the neuropathology images were graciously supplied by Dr. Dimitri Agamanolis. They come from http://neuropathology-web.org and unauthorized reproduction is prohibited. All rights reserved. This book is protected by copyright. No part of it may be reproduced, stored in a retrieval system, or transmitted in any form or by any means, electronic, mechanical, photocopying, recording, or otherwise, without the prior written permission of the publisher. Medicine is an ever-changing science. Research and clinical experience are continually expanding our knowledge, in par- ticular our understanding of proper treatment and drug therapy. The authors, editors, and publisher have made every ef- fort to ensure that all information in this book is in accordance with the state of knowledge at the time of production of the book. Nevertheless, the authors, editors, and publisher are not responsible for errors or omissions or for any consequences from application of the information in this book and make no warranty, expressed or implied, with respect to the contents of the publication. 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Identifi ers: LCCN 2016013927| ISBN 9781620701034 | ISBN 9781617052804 (e-book ISBN) Subjects: | MESH: Diagnostic Techniques, Neurological | Nervous System Diseases—therapy | Neuroimaging | Clinical Medicine—methods | Neurologic Examination | Diagnosis, Differential | Case Reports Classifi cation: LCC RC346 | NLM WL 141 | DDC 616.8/0475—dc23 LC record available at https://lccn.loc.gov/2016013927 Special discounts on bulk quantities of Demos Medical Publishing books are available to corporations, professional associations, pharmaceutical companies, health care organizations, and other qualifying groups. For details, please contact: Special Sales Department Demos Medical Publishing 11 West 42nd Street, 15th Floor, New York, NY 10036 Phone: 800-532-8663 or 212-683-0072; Fax: 212-941-7842 E-mail: [email protected] Printed in the United States of America by Edwards Brothers Malloy. 16 17 18 19 20 / 5 4 3 2 1 We dedicate this book to our patients who have taught us so much through the years and our colleagues who have always been a great support to us. We would like to express our eternal gratitude to our parents and other loved ones for their everlasting love and support. Contents Abbreviations xi 3.17. Posterior Reversible Preface xv Encephalopathy Syndrome 115 Acknowledgments xvii Share Neurology Image-Based Clinical Review 4. Neoplastic Diseases 117 4.1. Glioblastoma 119 1. Ischemia 1 4.2. Juvenile Pilocytic Astrocytoma 126 1.1. Ischemic Stroke 3 4.3. Oligodendroglioma 130 1.2. Brainstem Stroke Syndromes 18 4.4. Primary Central Nervous 1.3. Cerebellar Strokes 22 System Lymphoma 132 1.4. Lacunar Strokes 25 4.5. Dysembryoplastic 1.5. Watershed Strokes 27 Neuroepithelial Tumor 135 1.6. Hypoxic/Ischemic Injury 30 4.6. Ependymoma 137 1.7. Sinus Venous Thrombosis 33 4.7. Subependymoma 141 1.8. Cerebral Autosomal Dominant 4.8. Medulloblastoma 143 Arteriopathy With Subcortical Infarcts 4.9. Ganglioglioma 145 and Leukoencephalopathy 38 4.10. Hemangiopericytoma 147 1.9. Sickle Cell Disease 40 4.11. Pineoblastoma 149 1.10. Spinal Cord Stroke 43 4.12. Germinoma of Pineal Gland 151 1.11. Microvascular Disease 46 4.13. Central Neurocytoma 155 4.14. Hypothalamic Hamartoma 157 2. Hemorrhage 49 4.15. Clivus Chordoma 159 2.1. Hypertensive Hemorrhage 51 4.16. Corpus Callosum Lipoma 162 2.2. Lobar Hemorrhages 57 4.17. Meningioma 164 2.3. Amyloid Beta Related Angiitis 60 4.18. Vestibular Schwannoma 177 2.4. Hemorrhagic Conversion of 4.19. Glomus Jugulare 182 Ischemic Stroke 62 4.20. Esthesioneuroblastoma 184 2.5. Hemorrhagic Tumor 64 4.21. Spinal Epidural Metastases 186 2.6. Spinal Epidural Hematoma 66 4.22. Cerebral Metastases 188 3. Vascular Malformations/Diseases 5. Pituitary Disorders 193 of Blood Vessels 69 5.1. Pituitary Adenoma 195 3.1. Subarachnoid Hemorrhage 71 5.2. Craniopharyngioma 201 3.2. Aneurysms 75 5.3. Rathke’s Cleft Cyst 204 3.3. Dissections 81 5.4. Lymphocytic Hypophysitis 206 3.4. Central Nervous System Vasculitis 84 5.5. Pituitary Apoplexy 208 3.5. Moyamoya Disease 86 3.6. Arteriovenous Malformations 89 6. Cystic Lesions 211 3.7. Capillary Telangiectasia 94 6.1. Arachnoid Cyst 213 3.8. Cerebral Cavernous Malformations 96 6.2. Colloid Cyst of Third Ventricle 216 3.9. Developmental Venous Anomalies 100 6.3. Epidermoid Cyst 219 3.10. Dolichoectasia of 6.4. Ruptured Dermoid Cyst 222 Vertebrobasilar Artery 102 3.11. Fenestration of the Basilar Artery 104 7. Demyelinating Diseases 225 3.12. Persistent Trigeminal Artery 106 7.1. Multiple Sclerosis 227 3.13. Kissing Carotids 108 7.2. Optic Neuritis 238 3.14. Absence of the Internal 7.3. Neuromyelitis Optica 241 Carotid Arteries 110 7.4. Acute Disseminated 3.15. Carotid Paragangliomas 112 Encephalomyelitis 247 3.16. Nonaneurysmal Perimesencephalic 7.5. Susac’s Syndrome 249 Subarachnoid Hemorrhage 114 viii Contents 7.6. Adrenoleukodystrophy 251 11.11. Radiation Necrosis 394 7.7. Adult Polyglucosan Body Disease 253 11.12. Carbon Monoxide 396 7.8. Krabbe’s Disease 255 11.13. Transient Signal Alterations in Splenium 7.9. Metachromatic Leukodystrophy 257 of Corpus Callosum 398 8. Epilepsy 259 12. Pediatrics 401 8.1. Temporal Lobe Epilepsy 261 12.1. Acute Cerebellar Ataxia 403 8.2. Frontal Lobe Epilepsy 270 12.2. Germinal Matrix Hemorrhage 405 8.3. Limbic Encephalitis 273 12.3. Hypoxic-Ischemic 8.4. Status Epilepticus 276 Encephalopathy 409 8.5. Infantile Spasms 280 12.4. Mitochondrial Encephalomyopathy, 8.6. Lennox–Gastaut Syndrome 285 Lactic Acidosis, and Stroke-Like Episodes 8.7. Childhood Absence Epilepsy 290 (MELAS) 414 8.8. Rasmussen’s Encephalitis 292 12.5. Septo-Optic Dysplasia 417 12.6. Aicardi Syndrome 419 9. Infectious Diseases 295 12.7. Joubert’s Syndrome 421 9.1. HIV Dementia Complex 297 12.8. Fabry’s Disease 422 9.2. HIV-Associated Vacuolar 12.9. Pantothenate Kinase-Associated Myelopathy 299 Neurodegeneration 424 9.3. Herpes Simplex Encephalitis 301 12.10. Polymicrogyria 426 9.4. Ramsay Hunt Syndrome 303 12.11. Rhombencephalosynapsis 428 9.5. Progressive Multifocal 12.12. Schizencephaly 430 Leukoencephalopathy 305 12.13. Porencephaly 433 9.6. Tuberculous Meningitis 309 12.14. Colpocephaly 435 9.7. Bacterial Meningitis 313 12.15. Holoprosencephaly 437 9.8. Intracerebral Abscess 315 12.16. Lissencephaly 440 9.9. Spinal Epidural Abscess 318 12.17. Hydranencephaly 442 9.10. Neurocysticercosis 320 12.18. Aqueductal Stenosis 444 9.11. Toxoplasmosis 325 12.19. Neurenteric Cyst 447 9.12. Aspergillosis 329 12.20. Diastematomyelia 449 9.13. Creutzfeldt–Jakob Disease 332 12.21. Chiari I Malformation 451 12.22. Chiari II Malformation 454 10. Neurodegenerative Diseases 335 12.23. Dandy–Walker Syndrome 457 10.1. Alzheimer ’s Disease 337 12.24. Tethered Cord Syndrome 460 10.2. Frontotemporal Dementia 342 12.25. Vein of Galen Malformation 462 10.3. Huntington’s Disease 345 10.4. Parkinson’s Disease 347 13. Neurocutaneous Syndromes 465 10.5. Multiple System Atrophy 352 13.1. Neurofi bromatosis Type 1 467 10.6. Progressive Supranuclear Palsy 356 13.2. Neurofi bromatosis Type 2 471 10.7. Corticobasal Degeneration 358 13.3. Sturge–Weber Syndrome 475 10.8. Amyotrophic Lateral Sclerosis 360 13.4. von Hippel–Lindau Syndrome 479 10.9. Wilson’s Disease 364 13.5. Tuberous Sclerosis 482 10.10. Normal Pressure Hydrocephalus 366 14. Trauma 489 11. Toxic/Metabolic Disorders 371 14.1. Epidural Hematoma 491 11.1. Central Pontine Myelinolysis 373 14.2. Subdural Hematoma 493 11.2. Familial Cerebrovascular 14.3. Hemorrhagic Contusions 495 Ferrocalcinosis 375 14.4. Diffuse Axonal Injury 497 11.3. Hepatic Encephalopathy 377 14.5. Gunshot Wound 499 11.4. Cerebellar Atrophy 379 11.5. Cyclosporin Toxicity 381 15. Miscellaneous 501 11.6. Heroin Leukoencephalopathy 383 15.1. Behcet’s Disease 503 11.7. Marchiafava–Bignami Disease 385 15.2. Neurosarcoidosis 506 11.8. B12 Defi ciency 386 15.3. Langerhans Cell Histiocytosis 511 11.9. Copper Defi ciency Myelopathy 389 15.4. Tolosa–Hunt Syndrome 513 11.10. Wernicke’s Encephalopathy 391 15.5. Orbital Pseudotumor 515 Contents ix 15.6. Orbital Cavernous Venous 15.12. Copper-Beaten Skull 530 Malformation 517 15.13. Tension Pneumocephalus 531 15.7. Dilated Perivascular Spaces 519 15.14. Cervical Spondylotic 15.8. Fibrous Dysplasia of the Skull 521 Myelopathy 533 15.9. Hyperostosis Frontalis 523 15.15. Spinal Disc Herniation 535 15.10. Idiopathic Intracranial 15.16. Ankylosing Spondylitis 538 Hypertension 525 15.17. Brain Herniation Syndromes 540 15.11. Intracranial Hypotension 528 15.18. Brain Death 545 Index 547