Manual of Epilepsy: Medical Management and Social Aspects HV Srinivas
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1MANUAL OF EPILEPSY Medical Management and Social Aspects
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3MANUAL OF EPILEPSY Medical Management and Social Aspects
HV Srinivas MD (Gen) MD (Neurology) Consultant Neurologist Agadi Hospital and Sagar Hospital, Bengaluru Visiting Consultant and Postgraduate Teacher for DNB Neurology Department of Neurology Narayana Institute of Neurosciences Bengaluru, Karnataka, India Foreword PV Rai
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Manual of Epilepsy: Medical Management and Social Aspects
First Edition: 2016
9789352501311
Printed at
5Dedicated to
My students for their abiding interest to learn6
7Foreword
Although epilepsy has been considered as an illness of the brain by Hippocrates 400 years before Christ, there are still myths and misconceptions surrounding this brain disorder, not only in developing countries such as India but also even in industrially advanced Western countries. However, medical science has made great advances in the field of neurology, particularly over the past 150 years, proving without doubt that the brain is the seat of epileptic discharges. Two English Neurologists, William A Gowers (1845–1915) and John Hughlings Jackson (1835–1911) were the earliest to make major contributions for the scientific evaluation of the clinical phenomenon of a seizure correlating to the cerebral pathophysiology. For the last one hundred years, epilepsy became a domain of extensive research at different levels—clinical, neurophysiological and pharmacological. Fundamental research with biological studies contributed a good deal towards understanding the basic mechanisms leading to cerebral seizures, even though several aspects of a single unprovoked seizure (a symptom) leading to a treatable condition, epilepsy or epilepsy-syndrome, are still poorly understood.
However, today, there is an extensive research worldwide in neurosciences and epilepsy takes a good share in these studies as this is a common neurological condition and a well-treatable one. As a clinical discipline, epileptology has become an important subspecialty of neurology. The neurology resident today does not talk in the old terminology of ‘Grand mal’ or ‘Petit mal’ but in a differentiated seizure classification as presented over the years by the International League against epilepsy. This has enabled him/her to make a differential diagnosis of the seizure phenomenology and of the epilepsy from the point of view of its possible nosology. A neurophysician, therefore, is in a position not only to find the proper antiepileptic drug but also to assess the probable long-term prognosis related to the medical and social aspects of the patient.
The misconceptions about epilepsy over the earlier centuries as ‘incurable’ and ‘mysterious’ were partly because of lack of understanding of this brain dysfunction and also because there were no proper drugs for treatment. The patients were placed in mental hospitals along with serious psychiatric patients and sometimes treated with heavy sedative drugs such as bromide. The side effects of such drugs were sometimes more damaging than the actual health problem itself. A breakthrough 8came with the introduction of phenobarbitone in 1912, which is used even today, however, in lesser cases because of other better tolerable drugs introduced in the later years. With the present-day drug regimen, over 70% of people with epilepsy remain free of seizures and lead normal lives. Surgery helps another smaller percentage of patients. The intractable epilepsies are caused mostly by different forms of early brain damage and some kinds of severe childhood encephalopathies, which are still beyond the scope of proper medical treatment.
The book Manual of Epilepsy: Medical Management and Social Aspects by HV Srinivas is a welcome addition in the field of neurology/epileptology. The specialty of the book is its emphasis on clinical management with salient features for the diagnosis and differentiation of epileptic seizures and epilepsies. He encourages the readers to go into intensive history-taking and clinical evaluation to make sure that the seizure is epileptic. This, however, is only the first step for HV Srinivas. An epileptic seizure does not always mean that it needs to be treated. Further differentiation as provoked seizure/unprovoked isolated seizure must be considered. Only two or more unprovoked seizures lead to a diagnosis of epilepsy. He gives extensive case reports to document the differential diagnosis. His recommendation for using the four older drugs, viz. phenobarbitone, phenytoin, carbamazepine and sodium valproate is in line with the international studies. The newer drugs are needed when there is intolerance to these drugs or for ‘therapy refractory epilepsies’.
He is eminently suited to write such a book because of his almost 40 years of experience as a neurologist/epileptologist, his experience as a long-standing postgraduate teacher and as a socially oriented consultant. He has held offices as Chair, South East Asia Regional Executive Committee, IBE; President, Indian Epilepsy Association, Central Office; President, Indian Academy of Neurology; President, Karnataka Neuroscience Academy; President, Indian Epilepsy Association Bengaluru Chapter.
He has delivered several lectures to medical professions and general public and is a very much sought after epilepsy specialist at all levels.
PV Rai
Formerly Associate Medical Director
Swiss Epilepsy Center Zurich, Switzerland
9Preface
Epilepsy is a common neurological disorder affecting almost 1% of the population, which means in India there are about 90–100 lakhs of persons with epilepsy. We no longer use the term ‘epileptic’; instead we say “person with epilepsy (PWE)”. There are about 1000 plus neurologists in the country who are concentrated mainly in cities. It is impossible for a neurologist to see every patient with epilepsy. More importantly, there is absolutely no need for a neurologist to be involved with every case. With the available antiepileptic drugs, today, epilepsy is eminently treatable in 75–80% of patients. Further, this amount of success can be achieved by non-neurologists such as family physicians, primary care physicians, pediatricians and other medical professionals across the board.
I have been teaching neurology to postgraduate students in medicine for the last 38 years and have also addressed several medical meetings. I realized that with some guidance and information, epilepsy can be managed well by non-neurologists also.
With this intention, I decided to write this book which basically is a guide to the proper diagnosis and adequate management of patients with epilepsy. Even today, in spite of all the technological advances, the diagnosis of epilepsy is based entirely on the history provided by the patient and the onlooker who has witnessed the seizure. In other words, the diagnosis is highly cost-effective and can be done even at the primary healthcare center level! With reference to management, cost-effective primary or first-line antiepileptic drugs, available even in small towns, are sufficient for the effective treatment of 70–75% of patients with epilepsy.
The book is divided into two chapters: Chapter 1—Medical aspects of epilepsy, which deals with diagnosis and management and Chapter 2—Social aspects of epilepsy, which deals with education, employment, marriage, etc.
10The social aspect is usually forgotten by medical professionals and hence, the important message is not conveyed to the patients and the caregivers. In this section, I have compiled several questions, which have been asked by my patients, with their answers. I also found that many-a-time doctors give very vague answers and have no time to discuss the topic. I hope the book will help to dispel some of the myths even among the medical fraternity!
For further information, the readers are advised to refer to books on epilepsy.
HV Srinivas
11Acknowledgments
My wife Dr Pushpa Srinivas and my sister-in-law Lalita Sudarshan were prodding me to write this book since several years. At last, I made up my mind to put my experience into a book format hoping that it will benefit primary care physicians. I thank them for their persistence.
Of course, the idea of writing this book is a by-product of my continued interaction with postgraduate students in medicine and primary care physicians during the last 35 years. But for the interest of the student population, there would not have been any idea of writing this book!
My sincere thanks to Dr PV Rai for writing the Foreword and also for his guidance and useful comments in writing the book. My sincere thanks to Mr Sreeram Rama Chandran (Architect, Designer and Artist), who has drawn a number of sketches which is incorporated in this book.
I thank Meena Chandramohan for copyediting the book.
Finally, I acknowledge the sincere hard work put up by my secretary Theresa Pinto for typing several drafts ungrudgingly before it took the final shape.121314
15Introduction
Today, epilepsy can be well controlled in 75–80% of cases by any primary care physician provided, I repeat provided, they follow a few basic principles in the diagnosis and management. This book is an attempt to address this situation. The role of a neurologist is to sort out the cases difficult to diagnose and management of a small but significant number of 20–25% of people with epilepsy who have “intractable seizures” or “difficult to control seizures”.
In earlier years, data about epilepsy was collected from institutions where a majority of patients with long standing epilepsy approached for management. This generated a skewed data, with the conclusion that epilepsy is ‘chronic’ in the majority of patients requiring “long- term treatment,” if not “for lifetime.” Subsequently, community- based data where the information was collected at the doorstep of an individual showed that epilepsy is not all that bad! In fact, it has a spontaneous remission of 10–15%. Subsequent analysis showed that 75–80% of seizures can be well controlled and is chronic in only 20–25%.
The management of epilepsy in India is multidimensional involving medical, social, financial and societal attitudes. This results in a large number of patients with epilepsy not receiving antiepileptic drugs—the term used is “treatment gap”. The treatment gap is further increased by nonavailability of antiepileptic drugs (AEDs) on a continuous basis, poor drug compliance in view of prolonged treatment and the alternative therapy promising a “quicker cure”. While these are the problems on the patients’ side, there are contributions from the medical side such as inadequate and improper diagnosis, wrong choice of drugs, improper dosage, all because of lack of adequate knowledge about epilepsy. The treatment gap in India varies from 22% in urban population to 90% in rural population.
Epilepsy is primarily a clinical diagnosis from history given by the patient and the observer. It is very rare for the treating physician to witness a seizure. Fortunately, with the advances in technology, home video recording on mobiles, which is now easily available to all, sections of society has made a great contribution in the diagnosis of epilepsy. No amount of description of seizure can match watching a video clip of the said attack. This is especially useful to differentiate a genuine seizure from nonepileptic attack disorder (NEAD) as often NEAD is prolonged giving enough time to record it on the video.
16The diagnosis of epilepsy does not require any investigations to confirm or reject. The other interesting aspect is that in a great majority of people with epilepsy (PWE), there is no need for investigations such as EEG, CT, MRI, which are reserved and mandatory for specific group of people with epilepsy, which will be elaborated subsequently. Every patient with history of cough does not require X-ray of chest so, also every case of epilepsy does not require EEG, CT, MRI, etc. The primary drugs or the first-line drugs, viz. phenobarbitone, phenytoin, carbamazepine and sodium valproate are the sheet anchor treatment in 70–80% of people with epilepsy and, happily, these drugs are freely available in rural and urban places. In short, epilepsy can be easily diagnosed without hitech investigations and can be managed with the drugs available.
Aggressive marketing has pushed in several newer AED which no doubt have less side effects but are considered to be only equally effective as the older AED, which makes the older AED preferred drugs even today. In addition, the higher cost of newer AED favors the older AED. It is important to realize that the AED is to be taken for a minimum of 2–4 years from the last seizure and hence, the cost factor has to be considered both by treating physician and patient. A great majority of patients are obviously treated by physicians and internists and this book will help them to identify and manage epilepsy correctly.
Though the diagnosis of epilepsy can be straightforward and simple enough, it can also be one of the greatest clinical challenges. Almost 30% of the cases are underdiagnosed, e.g. complex partial seizures (CPS) and juvenile myoclonic epilepsy (JME); another 20–25% are overdiagnosed as generalized tonic-clonic seizures (GTCS), and world over 15–20% of people on antiepileptic drug therapy with a diagnosis of epilepsy, in fact, do not have epilepsy! The golden clinical rule is that when in doubt, it is better to wait for a definitive diagnosis than start antiepileptic drug therapy in order to avoid its consequent side effects and more importantly the social impact once the person is labeled as an epileptic. ‘Do not know’ is a better diagnosis than wrong diagnosis.
With this background, this write-up was conceived to appraise the medical community and to give succor to people with epilepsy.
17Abbreviations AED
Antiepileptic Drug
BRE
Benign Rolandic Epilepsy
CBZ
Carbamazepine
CLB
Clobazam
CLN
Clonazepam
CPS
Complex Partial Seizure
CT
Computerized Tomography
DBS
Deep Brain Stimulation
DZP
Diazepam
EEG
Electroencephalography
ESL
Eslicarbazepine
ESM
Ethosuximide
GBP
Gabapentin
GTCS
Generalized Tonic-clonic Seizure
JME
Juvenile Myoclonic Epilepsy
LCM
Lacosamide
LEV
Levetiracetam
LTG
Lamotrigine
LZP
Lorazepam
MJ
Myoclonic Jerk
MRI
Magnetic Resonance Imaging
NEAD
Nonepileptic Attack Disorder
OXC
Oxcarbazepine
PB
Phenobarbitone
PGN
Pregabalin
PHT
Phenytoin
PWE
Person with Epilepsy
RUF
Rufinamide
SPS
Simple Partial Seizure
TGA
Transient Global Amnesia
TGB
Tiagabine
TIA
Transient Ischemic Attacks
TPA
Topiramate
VGB
Vigabatrin
VNS
Vagus Nerve Stimulation
VPA
Valproic Acid
ZON
Zonisamide