John M. Stern Noriko Salamon Imaging of Epilepsy A Clinical Atlas 123 Imaging of Epilepsy John M. Stern • Noriko Salamon Imaging of Epilepsy A Clinical Atlas John M. Stern Noriko Salamon Department of Neurology Department of Radiology Geffen School of Medicine, University of Geffen School of Medicine, University of California, Los Angeles California, Los Angeles Los Angeles, CA, USA Los Angeles, CA, USA ISBN 978-3-030-86671-6 ISBN 978-3-030-86672-3 (eBook) https://doi.org/10.1007/978-3-030-86672-3 © Springer Nature Switzerland AG 2022 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, expressed or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Switzerland AG The registered company address is: Gewerbestrasse 11, 6330 Cham, Switzerland “To the patients for all that they have taught us.” “To Jackie, my wife and partner in the adventure of life, with love and appreciation.” John Stern “To Georges Salamon, who opened many doors for me.” Noriko Salamon Preface The clinical evaluation of epilepsy has evolved over the recent decades to increasingly inte- grate brain imaging as a complement to seizure semiology and electroencephalography (EEG). Although diagnosing epilepsy can rely entirely upon the history of unprovoked seizures, deter- mining the specific epilepsy diagnosis, which may be an epilepsy syndrome for generalized epilepsies or a localization for focal epilepsies, depends upon the integration of imaging results and additional diagnostic information. The aim of identifying a specific epilepsy diagnosis is increasingly important because of the relevance to treatment selection, especially as treatment options have increased. In particular, imaging results can substantially impact treatment rec- ommendations for those with medication-resistant epilepsy who are considering non- pharmacologic treatments. To provide best care, the epilepsy clinician’s skills now include a sophisticated knowledge of epilepsy’s imaging findings. The challenge in developing this skill arises from the broad range of findings from abnormalities that are small and subtle to obvious and extensive, and also includes findings that are either incidental or due to epilepsy treatment. Achieving expertise in the interpretation of epilepsy imaging is helped by a knowledge of the collection of findings that are potentially related to epilepsy and the relationships of each to clinical situations. Although numerous types of imaging abnormality may cause epilepsy, the full scope is small within all radiologic abnormalities, and its review is achievable. With this perspective, we created a workshop for the American Epilepsy Society’s annual meeting that reviewed the breadth of imaging findings related to epilepsy and presented the example images within their clinical contexts. Over several years, this workshop simulated a clinical case conference with clinical review alongside radiologic review. Essentially, we used a frame- work built upon a synthesis of clinical history and diagnostic modalities, which is the approach in conventional clinical practice, as an interactive educational program. Transforming the workshop into a book was not initially considered, and we thank the pub- lisher for proposing a reference book version of the workshop. The foundation of this book remains our experience that clinical context is invaluable when interpreting imaging, and this is especially important for epilepsy care with its inherent diagnostic challenges. This book provides a comprehensive review of epilepsy’s imaging findings with the detailed clinical his- tories that make the findings relevant. By understanding patients’ presentations and additional diagnostic testing, this radiologic review’s purpose is to provide an education in imaging find- ings alongside insights into the integration of diagnostic information for epilepsy. The book is divided into parts according to general categories for the imaging findings. Each imaging finding is presented as a collection of images across the relevant imaging modal- ities that relate to one patient, whose history accompanies the images. This detailed presenta- tion of one finding can be supplemented by a review of the whole category to obtain a broader understanding of the finding in the context of related findings. Overall, the case-based organi- zation with clinical history and multiple images is intended to provide a complete visual under- standing of the imaging findings and the corresponding relationship of each finding to the clinical presentation, treatment, and outcome. We are appreciative of our colleagues in the UCLA Seizure Disorder Center, who encom- pass the multiple disciplines needed for epilepsy care, including neurology, radiology, psy- vii viii Preface chology, surgery, psychiatry, nursing, and clinical neurophysiology. We also feel honored by the patients, as they shared their experiences with us and entrusted us with helping them over- come epilepsy. Los Angeles, CA, USA John M. Stern, MD Noriko Salamon, MD, PhD Key Words List Epilepsy Seizure Focal epilepsy Neuroradiology Brain imaging Epilepsy imaging MRI (Magnetic Resonance Imaging) FDG - PET (Fluorodeoxyglucose Positron Emission Tomography) CT (Computerized Tomography) DTI (Diffusion Tensor Imaging) DWI (Diffusion-weighted Imaging) ix Contents Part I Hippocampal Sclerosis 1 Mild, Unilateral Hippocampal Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3 2 Moderate, Unilateral Hippocampal Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 3 Severe, Unilateral Hippocampal Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11 4 Mild, Bilateral Hippocampal Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 15 5 Severe, Bilateral Hippocampal Sclerosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19 6 Hippocampal Sclerosis with Normal MRI and Abnormal PET . . . . . . . . . . . . . . . 23 Part II C erebral Malformations 7 Focal Cortical Dysplasia, Type I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29 8 Focal Cortical Dysplasia, Type IIa . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33 9 Focal Cortical Dysplasia, Type IIb . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 37 10 Focal Cortical Dysplasia, Type IIIb . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41 11 Focal Cortical Dysplasia with the Transmantle Sign . . . . . . . . . . . . . . . . . . . . . . . . 45 12 Focal Cortical Dysplasia with Bottom of the Sulcus Abnormality . . . . . . . . . . . . . 49 13 Focal Cortical Dysplasia with Gray- White Junction Blurring . . . . . . . . . . . . . . . . 53 14 Focal Cortical Dysplasia with Normal MRI and Abnormal PET . . . . . . . . . . . . . 57 15 Focal Cortical Dysplasia of the Temporal Pole . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 61 16 Focal Cortical Dysplasia of the Amygdala . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 65 17 Diffuse Periventricular Heterotopia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 69 18 Multifocal Periventricular Heterotopia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 73 19 Band Heterotopia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 77 20 Heterotopia Within Cerebral White Matter . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 81 21 Polymicrogyria Without Schizencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85 22 Polymicrogyria with Closed-Lip Schizencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . 89 23 Polymicrogyria with Open Lip Schizencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . 93 24 Lissencephaly. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97 25 Hemimegalencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .101 26 Hemimegalencephaly of the Cerebrum. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .107 xi xii Contents 27 Encephalocele . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .111 28 Encephalocele After Surgical Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .115 Part III Trauma 29 Temporal Lobe Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .121 30 Frontal Lobe Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .125 31 Bilateral Cerebral Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .129 32 Multilobar Cerebral Trauma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .133 Part IV I nfection and Inflammation 33 Acute Herpes Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .139 34 Remote Herpes Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .143 35 Acute Neurocysticercosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .147 36 Remote Neurocysticercosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .151 37 GAD65 Autoimmune Limbic Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .155 38 Voltage Gated Potassium Channel Autoimmune Limbic Encephalitis . . . . . . . . .159 39 NMDA Receptor Autoimmune Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .165 40 Hashimoto Encephalopathy also Known as Steroid-Responsive Encephalopathy Associated with Autoimmune Thyroiditis (SREAT) . . . . . . . . . .169 41 Early Stage Rasmussen’s Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .175 42 Late Stage Rasmussen’s Encephalitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .179 Part V Vascular Abnormalities 43 Cavernous Malformation with Acute Hemorrhage . . . . . . . . . . . . . . . . . . . . . . . . .185 44 Cavernous Malformation with Resolving Hemorrhage . . . . . . . . . . . . . . . . . . . . .189 45 Cavernous Malformation with Developmental Venous Anomaly . . . . . . . . . . . . . .193 46 Multiple Cavernous Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .197 47 Arteriovenous Malformation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .203 48 Ischemic Infarction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .207 49 Porencephaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .213 50 Sturge-Weber Syndrome in an Infant . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .217 51 Sturge-Weber Syndrome in a Child . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .223 52 Sturge-Weber Syndrome in an Adult . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .227 Part VI Tumors 53 Astrocytoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .235 54 Oligodendroglioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .239 55 Dysembryoplastic Neuroepithelial Tumor . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .243