Epigraph Vol. 25 Issue 4, Fall 2023

A conversation about The Idea of Epilepsy: Dr. Phillip Pearl and Dr. Simon Shorvon

Edited and produced by Nancy Volkers, ILAE communications officer

Cite this article: Pearl PL. A conversation about The Idea of Epilepsy: Dr. Phillip Pearl and Dr. Simon Shorvon. Epigraph 2023; 25(4): 108-115.

Published in 2023, Dr. Simon Shorvon’s The Idea of Epilepsy covers the history of epilepsy from multiple perspectives over the past 160 years. It ends with a tantalizing question: Does epilepsy actually exist? Dr. Phillip Pearl interviews Dr. Shorvon about the book and what it has to offer clinicians, researchers, and non-scientists alike.

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Podcast Transcript

[00:00:00] Dr. Phillip Pearl: My name is Dr. Phillip Pearl, and I'm the Director of Epilepsy and Clinical Neurophysiology at Boston Children's Hospital and the William G. Lennox Chair here in the Department of Neurology at Boston Children's Hospital and Harvard Medical School in Boston. But across from the pond from me is Professor Simon Shorvon from University College London. And well, I'm going to actually ask Dr. Shorvon to introduce himself more formally, but I just want to say this podcast is with reference to a history of epilepsy that Dr. Shorvon wrote that is just magnificent. It's detailed, it's comprehensive, but it is no boring history tome. It is fascinating. And so I'm giving you my—no spoiler alert intended, but I am recommending this book to every student of epilepsy. In fact, it was the gift I gave last year to my fellows from the epilepsy fellowship here who graduated and will be the gift in the years to come.

The year of publication is 2023, so you can't get any more up to date, but this book must have taken a long time to prepare. So, Professor Shorvon, Simon, could you go ahead and introduce yourself and then introduce the task of what it took to put this together.

[00:01:27] Dr. Simon Shorvon: Well, Phillip, that's, yeah, you're very kind, I would suggest overly kind, about my book. But I'm Simon Shorvon. I've been a neurologist specializing in epilepsy since the late 1970s. I was a consultant neurologist at the National Hospital, Queen's Square, I still am, and I was a professor of neurology at University College London, the Institute of Neurology, and I was Chair of Neurology there.

And I've been involved in the ILAE too, on the Executive Committee for 16 years, and also as editor of Epilepsia with, with Phil Schwartz. We were co-editors together.

So this book is partly a lockdown phenomenon. You know, nothing to do in lockdown, so, what should I do? Maybe try and write this book, but actually it's a book I've been wanting to write for years, and it's taken longer than the COVID epidemic, I must say. It's probably taken four years to write. And writing it has been quite fascinating experience for me, I must say, and I've learned a lot.

The book is, it's a narrative. It's got two real purposes. Firstly, it's to give a narrative history of epilepsy in the modern era, and I chose the modern era…I was originally going to say the 20th century, so I was going to start at 1900, but I soon realized that I really should start with Jackson in 1860. And that's really where [Oswei] Temkin left off. So his magnificent history of epilepsy, which is an unbeatable book, ends with Jackson, really. There is a short bit about the post Jackson era, but the book basically ends with Jackson. The last chapter he calls “The end of epilepsy”, which is something I also take up. So I start in 1860 and I go right through to the present day. And I divide the narrative into five chapters of different periods. That's the first point. But the second point is to try and understand why the history took the course it did. And to do that, the first realization is that the history is very different to people who are doctors. It's very different to patients. It's very different to scientists. It's very different to society.

And so perspective was very important. And I think the reason it's taken a rather erratic course over the years is that these different influences have all become more or less important over time. I think the 20th century could be considered the century where science becomes predominant.

But it's a great mistake to think that science is objective. It's wholly dependent on social and political and economic factors. And so I've tried to incorporate all of these into the book. And the most difficult bit was the patient perspective. Because up until about 1980 or 1990 even, patients didn't write, nobody wrote, very few people anyway, wrote an autobiographical book about their experiences of their own epilepsy. And the reason I think is really quite simple, because the condition was concealed because of the social stigma, mainly, and the hostility of society. So if you had epilepsy, you just kept it quiet. And it's quite fascinating, but I searched and searched and searched and could not find autobiographical writings.

There was one exception in the 1950s, this remarkable woman called Margiad Evans, who I do go on about in the book. She conversed with Lennox, and Lennox was very impressed by her, and he wrote about her in his book. And she has written remarkable descriptions of epilepsy [A Ray of Darkness and The Nightingale Silenced]. Very poetic, very insightful. Not epilepsy in a kind of medical sense, but what it's like to have epilepsy.

And then at the end of the century, 1980s, 1990s, there's been an explosion of books about epilepsy and what it's like to have epilepsy. And I think that demonstrates a change in society for the good, obviously. And I go into some of these books, but getting the patient's feelings about epilepsy has been very difficult, and I've had to sort of rely on literature and film, as well as a few very recent autobiographies.

So, the book is mainly medical, and I have tried to bring in these other aspects. I'm not sure where I succeeded, but that's what I've tried to do anyway.

[00:07:00] Dr. Phillip Pearl: Well, with reference to the patient's perspective, there are interspersed very nice reproductions of paintings by a David Cobley dated in 2021, and they seem to me to depict the patient's perception or self-perception, their own experience. Who is David Cobley? Who's the artist?

[00:07:21] Dr. Simon Shorvon: So David Cobley doesn't have epilepsy, by the way, but when I was the medical director of Chalfont Epilepsy Center, we opened an MRI unit there, and at the time, we raised money to do this, I thought it'd be nice to have some paintings around. So we commissioned David Cobley to do these paintings of an epilepsy center and the paintings are still in the center, particularly in the MRI unit.

And he became very interested in epilepsy. I became a bit of a friend of David and knew him over the years. And so when I wrote this book, I asked him whether he would paint some, whether he would paint some, well I asked him to draw some illustrations, which I could use in the book, and he remarkably, he decided he would paint them.

They're very insightful. He's got into the head of people with epilepsy, and he's kept away from getting into the head of doctors, which I think is a very good thing. So I think they add a kind of nuance to the book, I must say. I'm glad you noticed these paintings. Because not many people have commented on them. I think they're rather wonderful.

[00:08:38] Dr. Phillip Pearl: Yeah, that was one part of the book when I finished was still a mystery to me, the background of the illustrator, so thank you so much. I mean, you do address epilepsy from these different perspectives, from the perspectives of medicine, science, society, and the patient and the caregiver, and I thought it was remarkable how you went from that early autobiography you mentioned to Kurt Eichenwald's from the New York Times, his and others. And you also covered epilepsy in film, maybe even television. I mean, is that something that you've paid attention to all these years?

[00:09:11] Dr. Simon Shorvon: Yeah. So, well, I'm quite interested in film. Yeah. But I had a predecessor, Sallie Baxendale, particularly, I don't know if you know her, a psychologist. She's made special study of films. So it was easy to piggyback on the back of Sallie and look at the films she was talking about. One or two other people have kind of majored in films. Film is very much culturally determined and, you know, what you are allowed to get onto the screen is what society thinks is okay.

And you know, in the 1920s, you had all these eugenic films, which were horrifying, absolutely horrifying. I talk particularly about the film The Black Stork. And then when I was, you know, a kid we used to watch on television, Dr. Kildare. I don't know if anybody's ever heard of him now, but he was a sort of Brylcreem handsome doctor, and he did a couple of episodes about epilepsy. you know, how, how terrible it was. And you know, he would cure people of epilepsy and then they could get married and things like that. So I think film is a very interesting way, quite an easy way of identifying cultural trends in epilepsy.

[00:10:34] Dr. Phillip Pearl: Could you talk a little bit about, I think it's a wonderful literary tool, how you chose the metaphor of a voyage of a ship to base the currents of the book?

[00:10:46] Dr. Simon Shorvon: Yeah. So, yeah, I quite like metaphors. I know one or two people have said they find them very tiresome. But anyway, I didn't. So I thought the metaphor of the ship was a good one, because, you know, if you're an ocean liner, you go from point A to point B in a straight line. And the history of epilepsy is not at all in a straight line. It's more like a little ship buffeted around by different currents. The currents of science, the currents of the legislation and society, the currents of medicine, and each of them come along and the ship is sent along into some kind of cul de sac up some fjord somewhere, which goes nowhere, and then it comes back and then it staggers on, and it's sort of becomes encrusted with barnacles which weigh it down.

And that's how I sort of see the history of epilepsy, as a ship which is making it slowly towards its final destination, not that we know what that is. But it's an erratic journey. And the history of medicine in epilepsy is full of you know, mistakes and really quite harmful treatments and things like that. And so, it sort of reminds me of a voyage of a ship which is a bit lost on the high seas.

[00:12:15] Dr. Phillip Pearl: Now, you have some very colorful stories. and personalities in this book, and I want to ask you about a few of them. I'm going to ask you about the ones that hit closest to home for me. Maybe it represents my American roots and my own education and where I am right now.

Of course, you covered such important figures as Gowers and John Hughlings Jackson and John Russell Reynolds, but I would like to kind of skip right to William Lennox. Yeah. Strikes me as the person you covered the most in this book and who I think you described as the most significant figure in epilepsy and I'd say the first half, I guess, of the 20th century. Could you just give a summation of your own perceptions about Lennox?

[00:13:04] Dr. Simon Shorvon: Yeah. Well, I think Lennox was a quite remarkable person, I must say. And he was remarkable for a number of reasons. He was an excellent scientist. He was one of the first epilepsy scientists, he did this physiological work with Cobb, which really was excellent and they published a whole series of papers, sort of 17 or 18 of them, I think, each on a different topic, exploring sort of physiological aspects of epilepsy.

So that's one thing. He then also was an incredible lobbyist for epilepsy. And he was one of the first people to realize that you link up with the patient organization and between you, you're a very powerful lobby, something we've learned much later on. Fifity years later, it again began to dawn.

And so Lennox went to lobby Congress. He lobbied all sorts of people. He set up a kind of interest in legislation in epilepsy. And he was very important in getting the book by Barrow published, which was about epilepsy in the law. He then also kept the ILAE going during the war. The ILAE was about to collapse. Lennox did everything he could to keep it going. He virtually single-handedly kept Epilepsia going, I suspect, probably, at some extent, at his own cost. He managed to get all the American epileptologists, all the American neurologists interested in epilepsy, it seems to me. He seems to be a figure who drew people together.

And then he wrote this incredible book; the two-volume book, Epilepsy, is a remarkable achievement, in my opinion. And it's written in this wonderfully kind of folksy style, which I think is so kind of attractive, I must say. Anyway, he, as my book is not written like that, but he's full of amusing kind of words and phrases.

There's a sort of dark side to Lennox too, of course. Firstly he absolutely was a patient's advocate, and he said, for instance, that concealing epilepsy was the greatest… I've forgotten the word he used, greatest cancer or something, you know, the worst thing about it, but then, you know, his own daughter had epilepsy and he never, he kept it absolutely secret. So this was a sort of hypocritical stance to take, really. And he had epilepsy in the family, particularly petit mal epilepsy, of course, he was extremely important in identifying petit mal. He was one of the major figures in EEG, of course, which was just coming out. He had a knack of choosing the right technology and developing it.

He set up the Boston Epilepsy service. And of course there was then the question of eugenics. Now he, like almost all the neurologists of the time, were eugenicists. And he said some unfortunate things and wrote some unfortunate things from our perspective. But, you know, it's a huge mistake to judge historical time by the standards of contemporary life. Things have changed completely. And I don't, you know, I don't hold... Lennox's eugenics against him.

I think he was one of the great, well, the greatest figure in epilepsy, certainly in the mid-century, last century, and probably the whole of the century, really.

[00:16:58] Dr. Phillip Pearl: Well, I appreciate your historical perspective, which is of considerable valence. I want to move on to two figures. who, for me, were mentors from a distance, meaning I did not train directly under them, but at a national level, were very inspirational to me. And those were Kiffin Penry and Fritz Dreifuss. Could you say a word about each of those?

[00:17:20] Dr. Simon Shorvon: Well I spent three months with Dreifuss in Charlottesville when I was training. And he was a remarkable guy, I must say, and I was immensely honored to learn from him. He took a very broad view of epilepsy. He was always interested in the patient, not just the disease, I've always thought that's the best thing about epilepsy, you know, is the biography of the patient is really, really interesting how the disease affects the patient as he goes through life. And I learned, really learned that from Dreifuss, I think.

And he did some excellent things in Virginia. He was president of the ILAE. He had a wonderful way of speaking, I must say, with very amusing bon mots. And he made big contributions to EEG, and petit mal and partial epilepsy. So, I think he was a great man.

And then you come to Penry, who I also met and did like very much, and he was a extraordinary kind of dynamo. He did so many different things. He was really probably single-handedly responsible for raising epilepsy from the sort of dungeon of medicine, in the NIH, for instance, right up to the top of neurology.

He created these enormous data sheets, and then he was involved in the five-volume report on epilepsy, and he set up this monthly magazine, which used to come out. Kiffin had quite serious diabetes which ended his life early, really. But he was a wonderful teacher and, you know, the Kiffin Penry fellowships I think carry on to this day. And he was very, very innovative. And he too made huge contributions to the ILAE. In fact, the ILAE in those days was run from America and it was an international organization and Penry and Dreifuss were very keen on bringing in the international people, but it was, you know, the leadership was almost entirely American, really.

And the American kind of way of epilepsy dominated and still, I mean, made an indelible mark. My book has been criticized for being too Anglocentric or at least Anglophone centric, being more about the American and the British contribution. I tried very hard not to do that, actually, and I spent a long time talking, for instance, about Gasteau, who was the other great figure, I think, in 20th century epilepsy, and made enormous contributions.

But you know, I think it had to be said that it was Europe and America who were driving all the research and developments in epilepsy, right up until the last couple of decades of the last century. Prior to that, it was almost entirely European and American. And now it's all over the world, and that's partly due to the ILAE, which I think has made a big contribution, but you know, there are centers now in Australia, in China, in Africa, in Russia, you know, there are places all over the place, we're making major contributions to epilepsy. But it really wasn't the case until towards the end of the 20th century.

[00:21:20] Dr. Phillip Pearl: Well, I have to say, you make these comments in the book, and you do take a very global view, and you admit some of the emphases that you just described. And I just have to tell the reader, for example, when you finished your description of Dr. Dreifuss, one of my heroes, and you said he died at the height of his powers - it was show stopping for me. I mean, one line after another was fantastic. I could go on and on.

But I want to move to the title of the book because it's actually partly how I became drawn to it. I always thought of epilepsy as kind of an intangible concept, an idea, and the fact that that was the title of your book just tantalized me. And then you mentioned in the beginning that you're going to, at the end, talk about maybe we shouldn't even be using this term, which carries the reader, certainly carried me, all the way to the last word of the final page.

So can you talk about the title and your message at the end about how we should even be questioning the term?

[00:22:24] Dr. Simon Shorvon: Sure. Yeah you know, like you, I'm quite, I'm fascinated by the idea of disease, and actually Temkin goes into this a lot too.

Epileptic seizures are the same as they were 1,000 years ago, 10,000 years ago, probably, Hippocrates described them, they haven't changed at all. Nothing's changed about seizures. But the idea about epilepsy is that it is so bound up with culture and with advances in medicine and science and fashion to some extent, that the concept of epilepsy, what it means to people, its wider connotations, change enormously. And so, you know, if you say, if you asked somebody in 1900 what epilepsy was, you'd get a completely different answer than if you ask somebody in 2020 what epilepsy is. In fact, I don't think a doctor in 1900 would recognize what you were talking about. And ditto, I don't think a doctor in 2020 would recognize what the doctor in 1900 was really going on about.

I always thought there were four things which sort of underpin this, at least the medical things. One is, is it inherited? Two is, is it a mental disorder? Three is, is it just seizures? Or is it seizures plus all these other things, like comorbidities, we call them now? In the past, people talked about epileptic personalities. Is it just seizures, or is it a much broader thing? Is it the barnacles on the ship, which is staggering around on the high seas?

You know, I think these factors have fluctuated enormously over the, over the 20th century. I mean, heredity, for instance, in 1900, it was considered to be an inherited disease. There was no question. This led really, was one of the major reasons for eugenics. And then after the Second World War, nobody talked about their, the inheritance of epilepsy. Virtually nobody. Actually Lennox did, but few other people. And then, very recently, we're suddenly going back to the idea that it's largely an inherited disease and people go on about the missing inheritance and so on.

I'm a bit critical about modern genetics. I don't know whether you picked that up in the book, but I think we're heading, probably heading in the wrong direction there. So that's one thing.

And then, ditto, the pendulum swung on whether it was a mental disorder. The pendulum swung violently about whether it's just seizures. And I think the invention of EEG, for instance, changed the focus completely on epilepsy being a disease, which is a cerebral dysrhythmia— that was Lennox's term, which I think is a fascinating term. But he saw the squiggles on the EEG and he thought it was a dysrhythmia. And that's how it was conceived for most of the latter half of the last century. So the concept varies enormously. And now, so in the end I bring up this question of whether we should use the term, and the kind of basis is that it's a term which has got all these barnacles attached to it, it's a term which carries a lot of stigma, although thankfully much less nowadays than it used to.

And it isn't a disease in a medical sense. You know, it has so many different forms, so many different causes, so many different physiologies. it doesn't make sense to call it a disease. It's the same as calling anemia a disease. If it's anything, it's a kind of broad neurological syndrome or something, but it's not a disease in the strictly medical sense.

So if it isn't a disease, why do we call it a disease? And why do we use the term? And the reason I think is that you do need a sort of shorthand term for describing what it's like to have epilepsy, what's the propensity to have seizures and the consequences of that, social, medical, scientific, degenerative, what all these consequences are.

You may need some term, but, in using the term, you bring up all the barnacles too, and if we really wanted, you know, there was a move to change, stop calling people epileptics, but call them people with epilepsy. I think actually that missed the point, it would have been better to call them people who have epileptic seizures or people with this disease or that disease or the other disease.

I think using the term epilepsy is probably one of the reasons why stigma persists. I mean, you know, in psychiatry, nobody calls people lunatics anymore because lunatics has a pejorative meaning, or imbeciles or retarded people, all these sort of terms where, you know, words do matter. And it's interesting to see that the Korean Epilepsy Society changed the name of epilepsy, the word in Korean, and the same thing happened in China, I think. And in other countries, people have altered the terms because terms carry with them all sorts of hidden and deep prejudices and meanings. And I think the term epilepsy does too.

I don't know that there's a real answer to this, but if we didn't use the term “epilepsy,” but say talked about Dravet syndrome or talked about head injury with seizures or something, perhaps we would improve the quality of life for people who have epilepsy.

And I don't think this is just playing with words. I think words really matter. I think medicine, by stopping using the term “epilepsy” and being more specific, talking about, you know, focal seizures or something, that might be a way of improving the social attitudes to epilepsy.

That's the basis of my argument, but I don't think it's going to go anywhere, I must say.

[00:29:20] Dr. Phillip Pearl: There's a lot to think about there. Yeah. I'd like to follow up on a statement you made about how you think maybe modern genetics may be going the wrong direction in some way. Could you explain what you meant by that?

[00:29:32] Dr. Simon Shorvon: Yeah, well, now I'm going out on a limb, I know that. But you know, there's been a tendency for people to jump on this genetic bandwagon and call everything genetics. There are, of course, genetic epilepsies, particularly in pediatrics. In adult neurology, the impact of genetics have been far less than in pediatrics, where of course, it's tremendously important.

But I think, you know, there have been all these gene-searching enterprises with thousands of patients entering into huge databases. And you find that this gene has a very minute effect, association with epilepsy. And you know, there are obviously monogenic diseases. The inborn errors, a lot of dysplasias, most of the infantile encephalopathies. These are often genetic. Some are inherited, but others are de novo mutations. But then the searching for these genes which have minor effects, I think is a mistake to call that genetic. You know, they're just variations of humanity. Height is a good example. You know, it's influenced by 200 genes or something, but it's also influenced by putting hormones in milk or by having a loving childhood.

You know these environmental things and developmental things I think are equally important. And if we focus too much on genetics, we're going to not focus on these other things. And it's very difficult, for instance, to find any papers about the influence of environment, for instance, on developing epilepsy.

So, I think that's the framework in which genetics handicaps us and means we don't look elsewhere. And then another point, I think, which has really gone off the rails is all this stuff about personalized medicine because, you know, epilepsy is a very complex phenomenon. Brain activity is a very complex problem. It's very unlikely to be due in most cases to either a single gene affecting one biochemical pathway, because all sorts of other biochemical pathways then interact, blood-brain barrier you know, transport. So I think the idea, which was very predominant 20 years ago in Britain, we had a visit from the head of GSK [Glaxo Smith Kline] once, who congregated all the English neurologists together and said that we need to give them the DNA of all our patients because personalized medicine in five years’ time will be the thing and we'll have identified medicines which work for individual patients by their genomic footprint or fingerprint.

And that was in 2000, and now we're at 2023, and we're not five years, we're 25 years away, it still hasn't even remotely happened. And I think, well, that's another reason I rather feel genetics has gone down the wrong routes. I think we should spend more time thinking about development and thinking about environment, I must say.

Anyway, that's a personal view, which I express at the end of the book, but it's just, it's just a personal view.

[00:33:04] Dr. Phillip Pearl: Well, yeah, there is a lot of exuberance about a lot of new technologies that we're jumping into with really very little knowledge, data, evidence, even an idea of whether it's worth all this.

So, I think, I think I'm going to wind up by quoting for everyone, just one of many wonderful quotes that are so illustrative. In your introduction, you quote, Blaise Pascal, who himself was a French prodigy in math, physics, invention, philosophy, a writer. He lived in the 1600s, and you pointed out that he himself died in status epilepticus, which I had no idea.

And then he said, “The last thing we discover in writing a book is what to put in first.” And I mean, that was just such a beautiful quote. So now I just want to ask you, did that resonate with you after you finished the book? Did you then decide what you should have put in first, or did you decide something you should have changed?

[00:34:09] Dr. Simon Shorvon: Yeah, no, absolutely. I did not have a plan for this book other than I thought I'd do a history. But as I was doing it, the ideas sort of evolved, and literally the last thing that was written was the introduction, which is obviously the wrong way around. If you're a proper scientist, you would have your hypothesis and you go out and test it. When you're writing a history, I don't think you can do that.

I think you start modifying what you think as you're going along. And so the beginning is at the end or the end is at the beginning.

[00:34:57] Dr. Phillip Pearl: Well, I have to congratulate you on not only your career, you've written other books and great literature, but I think that completing this book is a great capstone to a career, and I think completing it is just marvelous. As you can tell, I'm pretty enthusiastic about it and for all of our fellows, resident students, really all physicians and non-physicians alike can get so much out of it.

And it's been my privilege to talk to you about it. So thank you very much.

[00:35:32] Dr. Simon Shorvon: Well, thank you Phil, for being so kind and so generous in your thoughts. I really appreciate that.