Editor
Ambar Chakravarty
Honorary Professor and Emeritus Consultant in Neurology Vivekananda Institute of Medical Science Kolkata, West Bengal, India Past President Indian Academy of Neurology (2005–2006)
Foreword
Professor David Bates
Headquarters
Jaypee Brothers Medical Publishers (P) Ltd
4838/24, Ansari Road, Daryaganj
New Delhi 110 002, India
Phone: +91-11-43574357
Fax: +91-11-43574314
Email: jaypee@jaypeebrothers.com
Overseas Office
J.P. Medical Ltd
83 Victoria Street, London
SW1H 0HW (UK)
Phone: +44 20 3170 8910
Fax: +44 (0)20 3008 6180
Email: info@jpmedpub.com
Website: www.jaypeebrothers.com
Website: www.jaypeedigital.com
© 2020, Jaypee Brothers Medical Publishers
The views and opinions expressed in this book are solely those of the original contributor(s)/author(s) and do not necessarily represent those of editor(s) of the book.
All rights reserved. No part of this publication may be reproduced, stored or transmitted in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, without the prior permission in writing of the publishers.
All brand names and product names used in this book are trade names, service marks, trademarks or registered trademarks of their respective owners. The publisher is not associated with any product or vendor mentioned in this book.
Medical knowledge and practice change constantly. This book is designed to provide accurate, authoritative information about the subject matter in question. However, readers are advised to check the most current information available on procedures included and check information from the manufacturer of each product to be administered, to verify the recommended dose, formula, method and duration of administration, adverse effects and contraindications. It is the responsibility of the practitioner to take all appropriate safety precautions. Neither the publisher nor the author(s)/editor(s) assume any liability for any injury and/or damage to persons or property arising from or related to use of material in this book.
This book is sold on the understanding that the publisher is not engaged in providing professional medical services. If such advice or services are required, the services of a competent medical professional should be sought.
Every effort has been made where necessary to contact holders of copyright to obtain permission to reproduce copyright material. If any have been inadvertently overlooked, the publisher will be pleased to make the necessary arrangements at the first opportunity. The CD/DVD-ROM (if any) provided in the sealed envelope with this book is complimentary and free of cost. Not meant for sale.
Inquiries for bulk sales may be solicited at: jaypee@jaypeebrothers.com
Mimics of Epileptic Seizures / Ambar Chakravarty
First Edition: 2020
9789390020966
Printed at
fm_5Dedicated to
fm_7Contributors
- Editor
- Ambar Chakravarty MD FRCPC FRCP FRCPE FIAN
- Honorary Professor and Emeritus Consultant in Neurology
- Vivekananda Institute of Medical Science
- Kolkata, West Bengal, India
- Contributing Authors
- Ajitesh Roy MD DM
- Assistant Professor
- Department of Endocrinology
- Vivekananda Institute of Medical Science
- Kolkata, West Bengal, India
- Ambar Chakravarty MD FRCPC FRCP FRCPE FIAN
- Honorary Professor and Emeritus Consultant in Neurology
- Vivekananda Institute of Medical Science
- Kolkata, West Bengal, India
- Amit Halder MD DM
- Consultant
- Department of Neurology, Fortis Hospital
- Kolkata, West Bengal, India
- Angshuman Mukherjee MD DM
- Professor and Head
- Department of Neurology
- Vivekananda Institute of Medical Science
- Kolkata, West Bengal, India
- Arparajita Chatterjee MD DM
- Consultant
- Department of Neurology
- National Institute of Mental Health and Neurosciences
- Bengaluru, Karnataka, India
- Asha Kishore MD DM FAMS
- Professor
- Department of Neurology
- Comprehensive Care Center for Movement Disorders, Sree Chitra Tirunal Institute for Medical Sciences and Technology
- Thiruvananthapuram, Kerala, India
- Ashalatha Radhakrishnan MD DM FRCP FANA
- Professor
- Department of Neurology
- R Madhavan Nayar Center for Comprehensive Epilepsy Care
- Sree Chitra Tirunal Institute for Medical Sciences and Technology
- Thiruvananthapuram, Kerala, India
- Debasis Roy MD DNB DM
- Professor
- Department of Neurology
- Vivekananda Institute of Medical Science
- Kolkata, West Bengal, India
- Deb K Pal PhD FRCP
- Professor of Pediatric Epileptology
- Department of Basic and Clinical Neurosciences Institute of Psychiatry,
- Psychology and Neuroscience King's College,
- Gagandeep Singh DM FAMS FRCP
- Honorary Associate Professor
- Department of Clinical and Experimental Epilepsy
- NIHR University College London Hospitals
- Biomedical Research Centre,
- UCL Queen Square Institute of Neurology,
- London WC1N 3BG, & Chalfont Centre for Epilepsy,
- Chalfont St Peter SL9 0RJ,
- United Kingdom
- Professor and Head
- Department of Neurology
- Dayanand Medical College
- Ludhiana, Punjab, India
- Neelu Desai MD
- Consultant
- Department of Pediatric Neurology
- PD Hinduja National Hospital
- Lokmanya Tilak Municipal Medical College and Hospital
- Mumbai, Maharashtra, India
- Nitika Mahajan MD DM
- Assistant Professor
- Department of Neurology
- Dayanand Medical College
- Ludhiana, Punjab, India
- P Suresh Babu MD DM
- Consultant
- Department of Neurology
- Krishna Institute of Medical Sciences
- Secunderabad, Hyderabad, India
- Roopa Rajan MD DM
- Assistant Professor
- Department of Neurology
- All India Institute of Medical Sciences
- New Delhi, India
- Sanjib Sinha MD DM
- Professor
- Department of Neurology
- National Institute of Mental Health and Neurosciences
- Bengaluru, Karnataka, India
- Subhas Kaul MD DM FIAN
- Professor
- Department of Neurology
- Krishna Institute of Medical Sciences
- Secunderabad, Hyderabad, India
- Sudhansu Chokroverty MD FRCP FACP
- Professor
- Department of Neurology
- Clinical Neurophysiology and Sleep Medicine
- Hackensack Meridian Health-JFK Medical Center, JFK Neuroscience Institute
- Edison, New Jersey, USA
- Sudip Chatterjee MD MNAMS FRCP FACP
- Professor
- Department of Endocrinology
- Vivekananda Institute of Medical Science
- Kolkata, West Bengal, India
- Sushanth Bhat MD
- Consultant
- Department of Neurology
- Hackensack Meridian Health-JFK Medical Center, JFK Neuroscience Institute
- Edison, New Jersey, USA
- Vrajesh Udani MD
- Senior Consultant, Department of Pediatric
- Neurology, PD Hinduja National Hospital
- BJ Wadia Hospital for Children
- Mumbai, Maharashtra, India
There can be few conditions which cause greater concern to the man in the street or to the physician than the “Falling Sickness” or the Epilepsies. The drama of a sudden collapse with an atonic or tonic-clonic seizure, the subtlety of an absence or minor seizure and the bizarre behavior of someone with psychogenic epilepsy demonstrate a plethora of presentations which is complimented by a multitude of underlying pathologies and differential diagnoses. But if the recognition and diagnosis of the varied epilepsy syndromes is problematic it is compounded by the need to remember that not all collapses are seizures and not all seizures are epileptic; the importance of making a diagnosis of one of the mimics of epileptic seizures cannot be overstated, lives can be ruined, therapies will fail and avoidable deaths may occur when epilepsy is diagnosed incorrectly.
In this multi-author book, edited by and with commentaries from Professor Ambar Chakravarty, tribute is paid to Sir William Gowers whose book “The Borderland of Epilepsy” described these difficulties over a century ago. In the 21st century, the physician is aided by modern electrophysiology, imaging with CT and MRI and functional imaging with fMRI and PET scans, genetic testing, sophisticated cardiological, and endocrinological tests, and especially by the ability to observe a “typical” attack on video or smart phone. But the essence of making any diagnosis remains clinical and is based on obtaining a careful history, abetted by a witness history since this is the one occasion where the patient cannot tell you the diagnosis but the witness may, and in this respect experience is vital, whether learned practically over a lifetime in medicine or from the recorded histories of cases and advice from senior physicians. This volume provides such insights from physicians working in India, USA, and UK in the fields of neurology, endocrinology, physiology, internal medicine, and pediatrics all of whom describe examples of their experience demonstrating occasions when epilepsy was shown to be due to another pathology or where a bizarre attack turned out to be epileptic. The importance of achieving an accurate diagnosis in developing a regimen of therapy is self-evident.
Although the methods of diagnosis, understanding of causation, and potentials for therapy of the episodic disorders resembling the epilepsies have changed markedly since Gowers’ day, Chakravarty and his colleagues remind us of the challenges still faced by every physician in dealing with a patient who collapses and this 21st century version of the “Borderlands of Epilepsy” will be useful to all fm_10physicians and particularly to those embarking on a career in Clinical Neurology. The physician who learns from the experiences and opinions of the contributors to “Mimics of Epileptic Seizures” and digests the comments and commentaries appended to the chapters will gain confidence in asking the relevant questions, formulating a differential diagnosis, ordering the appropriate tests, and providing the best advice and therapy for the benefit of the patient.
Professor David Bates
University of Newcastle upon Tyne
United Kingdom
fm_11Preface
Epilepsy poses as a multifaceted problem to any individual with this disorder as well as to his/her treating physician. These several facets of the disease include clinical, electrophysiological, neuroradiological, biochemical, genetic, neuropathological, psychological, social, economic, legal, and many others, which become involved in proper management of persons with this disorder. The problem can be viewed as an inverted pyramid which in essence rests on or revolves round a single issue—did the person concerned really have or have not had an epileptic seizure? Occurrence of two or more such attacks (unprovoked) is needed to make a confirmed diagnosis of epilepsy in any given case. The entire workup and all management related issues rest on the apical point of this upside down pyramid; and this is the correct diagnosis of having had a definite epileptic seizure. Failing this, the entire workup of the subject concerned, would fall down like a house of cards. In practice, the most common cause of failure to control a person's seizures adequately in spite of proper and adequate antiepileptic drug therapy happens to be a wrong diagnosis which occurs in 20–30% of cases admitted to any epilepsy monitoring unit for control of medically refractory seizures.
There are two aspects of making a correct diagnosis of an epileptic seizure. The first is to make a “positive” diagnosis of seizure, which is essentially a thorough, demanding, and time consuming clinical exercise and later supported by appropriate tests specially electrophysiology and imaging. The second can be labeled, for literally sake, a “exclusion” approach whereby the physician tries to exclude all other seizure look-alikes, again through a thorough clinical exercise, supported by appropriate investigations, electrophysiological or otherwise. Such seizure look-alikes have been termed as “mimics” or “imitators” of epileptic seizures. The International League Against Epilepsy (ILAE) prefers the latter term and in 2017 had published a long list of such imitators, which has been reproduced (with permission) at the end of Chapter 2 as an Appendix. Of these multitude of conditions listed there in, which one or ones to choose for exclusion in a given person experiencing a “spell” is entirely a clinical decision which would vary from case to case.
William Gowers was the first person to write a comprehensive account of almost all these mimics or imitators of epileptic seizures in his exhaustive monograph titled “The Borderland of Epilepsy–Faints, Vagal attacks, Vertigo, Migraine, Sleep symptoms, and their treatment”, published in 1907. Gowers chose to call all these look-alikes of epileptic seizures as “Borderland” as some of these tend to so closely fm_12resemble an epileptic seizure that it becomes difficult to find a clear cut “border” in between the two conditions and some may really rest in the “no man's land” zone. Use of such terms like “mimics” or “imitators”, Gowers probably felt would not have been sufficient/explicit enough to express their closeness to each other (or their overlapping nature with epilepsy). With passage of time, we have now realized that some of these conditions indeed have pathophysiologic (e.g., migraine with aura and occipital seizures) and/or genetic (e.g., paroxysmal nocturnal dyskinesia and autosomal dominant frontal lobe epilepsy; familial hemiplegic migraine, episodic ataxia, and epilepsy) overlapping zones with epilepsy.
An interesting question keeps coming to my mind for a long time. Who can be called an “astute” clinical neurologist? One may come up with various suggestions. Many years back, during my early years of training, I realized that one who, purely on clinical grounds, through thorough history taking, and clinical examination, can distinguish between an epileptic and a nonepileptic event, with the least number of mistakes, can be designated as an “astute” clinical neurologist. That was long before VEEG or mobile phone videos came into practice. My interest in the Borderland conditions really stem from the very early development of this particular notion, mentioned above. Honestly speaking I always strive hard to attain this goal! And who, other than Sir William Richard Gowers, can remain as my idol in this venture? I treasure possessing a copy of his book (a “pre-owned” copy) for over 40 years now.
In 2003, on behalf of the Association of Neuroscientists of Eastern India (in my capacity as the Founder Editor of the Journal of the Association and later as its President), I published the first Indian monograph to address the issue of differentiating the look-alike conditions from epileptic seizures with a “near-plagiarized” title “Borderlands of Epilepsy” (“Borderlands” in place of Gowers’ “Borderland”), the circulation of which had not been up to my satisfaction due to financial constraints and other technical reasons. In the intervening years, two similar publications had been made in the International field namely an exhaustive text by Kaplan and Fisher (2005) and another, a very concise but extremely useful book by Panayiotopoulos (2012). These inspired me to write, edit, and publish an expanded and updated version of my earlier publication through a reputed publishing house. New chapters have been added (bearing in mind the changing practice of neurology worldwide and the recommendations of ILAE), new contributing authors have been inducted and perhaps most importantly, illustrative case studies have been added. I realized the value and utility of publishing books which include case studies from the feedbacks I received from various corners following the recent publications of two of my books namely “Case Studies in Uncommon Headache Disorders (2018)” and “Vertigo and Dizziness: A Case Based Study (2019)”. In addition, I decided to include one or more commentaries (mostly written by me) to follow most chapters, focusing either on some practical issues or some debatable issues or issues not adequately covered in the main chapter. The book thus includes 22 chapters, 23 commentaries, and several case studies with comments from the editor. On the flip side, I must mention that certain fm_13issues concerned with the diagnosis of epileptic seizures, had been mentioned on more than one occasions in various chapters and commentaries. Examples include the diagnosis of visual auras, sleep parasomnias, and avoidance of over-interpreting EEG records a few others. I have allowed such repetitions to stay, as I felt that going over such important issues on more than one occasions would only help young neurologists and trainees to remember them well throughout their practicing years.
Having been promised, a generous educational grant from Astera Division of Intas Pharmaceuticals through the good offices of Dr Michael D'Souza, Vice President, had been most helpful. The company has promised free distribution of the book to all members of the Indian Academy of Neurology and to all trainees in neurology in various institutions in the country. My sincere gratitude goes to Dr Michael D'Souza of Astera Division and Intas Pharmaceuticals for the same. Additionally, the book should be available directly from the publishers and also through Amazon.
I would also like to thank profusely the contributing authors, some of whom are world renowned authorities, for kindly writing for this volume in the midst of their numerous commitments.
My sincere thanks go to Professor David Bates of Newcastle upon Tyne, United Kingdom, for being kind enough to write the foreword to this volume and to my former teacher Professor John Pearce of Hull, United Kingdom, for his very thoughtful criticisms of some of my writings featured in the book.
I am indebted to the publisher, Mr Jitendar P Vij of Jaypee Brothers Medical Publishers (P), Ltd, New Delhi, for his cooperation to bring out the book in time. I am also thankful to his associates particularly to, Mr MS Mani, Mr Shashi Kumar Shamboo, Mr Sabyasachi Hazra, Dr Richa Saxena, Ms Kamakshi Khanna and the team.
Lastly I wish and pray that the book becomes really useful to all those for whom it had been written.
Ambar Chakravarty