THE SACRED DISEASE
THE SACRED DISEASE………… this phrase the sacred disease achieved immortality in the written sense with that utilization by Hippocrates. There have been descriptions of epileptics and epilepsy of one type or another for many years. There is a very famous description in the New Testament in Mark that I think you will recall the man that came to Christ and said “I have brought to thee my son which hath a dumb spirit”. The concept of epilepsy being something from without, the term epilepsy is being something that seizes a man from without himself, a Greek term has had a connotation and has been probably been encouraged by some people, perhaps charlatans and other people who had something to gain from it by being possessed by a spirit, possessed by a god, hence, I think the use of the term “the Sacred Disease”.
Shakespeare made popular another term “the falling illness” in his description of Julius Caesar. There were many people who are said to have been epileptics. The evidence for this is more secure in certain instances than others. Hercules is thought by some to be an epileptic killing his wife and children in a fit of epileptic rage. Caesar, Alexander the Great, perhaps, Caligulia apparently definitely was. Petrarch in his elder years, Charles V of Spain, Peter the Great, Dickens, which is something I didn’t realize, Moliere, both Byron and his mother were epileptics and you all know about Dostoevski. Alfred Nobel was an epileptic and this is something I hadn’t realized. He has left his name to us in many areas, both explosive and peaceful. The written description as I indicated for written discussion of epilepsy really the first one is that chapter of those writing ascribed to Hippocrates.
This is one of the few illnesses that is not in the Edwin’s the V papyrus or any of the early Egyptian papyri. There is reference to epilepsy in the, I hate to say the Hammurbi code, concerning the laws dealing with epilepsy both in marriage and their testimony in court but there are no writings that I am aware of that could turn up antedating the Hippocratic writings dealing with epilepsy as a disease so that the chapter as far as I am aware is the earliest written record discussing it as an illness. An interesting thing is that many of the concepts proposed there were unfortunately lost and not brought back into prominence until around the 1600’s and really were not universally accepted, some of the basic principles until right around the turn of the nineteenth century.
Hippocrates, as you know, his writings are ascribed to around the turn of the fifth century B.C. One of the things that is in the book is the very clear assignment locust for the pathology for epilepsy in the brain and also Hippocrates in his writings recognized the contra lateral representation of neuro function in the brain. Also trephination for epilepsy is mentioned in there. Trephination for traumatic epilepsy is mentioned in the Hippocratic writings. Now Hippocrates, interestingly enough, attributed epilepsy to blocking of the air entering the veins to the head and this really is probably not the least in some patients and not too inaccurate in that hypoxic states or interference with cerebral circulation do tend to bring on convulsions. Granted his anatomy and his pathophysiology was a little bit unique, but never the less, the concept of inadequate oxygenation of the brain is undoubtedly a valid reason for some types of seizure disorders.
The next individual who made any lasting mark in discussion of epilepsy is a man by the name of Pelops in the second century A.D. His little mark in this saga was the introduction of the term aura which means a breeze. Pelops was the preceptor of Galen who was his instructor, his teacher, and he obtained this term from a patient. The patient described an aura as being like a cold breeze coming up one leg and ascending through his body and he seized upon this term and termed this initial phase of the seizure as an aura. One of the peculiar things about this was that then for the next four hundred years the only thing that was called an aura was if the patient had a cold breeze ascending up his leg. All the others, as we now accept as auras, were not called auras and this happens to be a very rare kind of aura so their weren’t many seizure patients that had auras during the next four hundred years. Now Galen, a student of Pelops, really didn’t make any contribution to the study of epilepsy. He kind of back slid from Hippocrates and earlier people. He felt that seizures were due to an obstruction of the ventricles by viscid fluids or viscid humor and really didn’t have anything constructive in the terms of therapy although he did talk about voiding a lot of the excessive types of treatment that were used. The violent purgatives, blood lettings, there were a lot of medications that were used that he disavowed, but he really didn’t make any contribution other than saying do not harm. I think that was about his main contribution.
Most of the sixteenth century physicians appear to regard the various internal organs the starting point of the seizures. Again having lost the concept of seizures starting in the brain, we had bilious convulsions, we had splenic seizures. This unfortunately still stayed with us to a certain extent right up until modern day.
There was a man by the name of Lahey that you may have heard about who published an article for Colectomy for the treatment of seizures. Now granted that he was thinking of something a little different, he was trying to remove, but still in theory attacking the cause of the convulsion. Removing a septic focus if you will, but never the less concepted the real cause of the seizure was somewhere else in the body. There was a number of things that were going on at this time during the sixteenth and seventeenth century. There were physicians who were using ligatures, cautery and amputation in patients who had an aura. They had numbness, tingling or some form of sensory aura beginning in the hand or foot. There were physicians quite willing to try and treat the seizures by cauterizing, putting a ligature around either temporary or permanent or amputating that portion of the body to try and treat the seizures. Again this was not the very happy time for an epileptic.
Now at the end of the sixteenth century was the first to clearly reaffirm in writing that all epileptic seizures were of central origin, by central I mean originated in the brain. This was not widely accepted. There was another physician about that same time by the name of Geratis who reported relief of seizures by removing a depressed bone spicule in the brain in an eighteen year old boy who had sustained a depressed skull fracture six years earlier. Pare and Geratis were aware of each others work and feelings but this did cure an epileptic by removal of this bone spicule. Although at this time These were still little islands of illumination in a rather large sea of ignorance right up through this time. There are still ligatures, cautery, and amputation being abdicated by many. Pare and Val Salva at the later end of this period again reaffirmed the contra lateral representation of the brain and the arm that had nothing in spite of Pare extensive war experience had nothing really constructive to say about epilepsy, its management and the concepts leading into it.
Brown-Sequard who really was active just at the beginning of the modern era and who was the teacher of, or preceptor, of both Gowers and Jackson standing really just at the threshold still believe that the cause of the seizures were peripheral producing a reflex increasing excitability of the brain. He agreed that the convulsions started in the brain but were produced in a reflex fashion by some peripheral etiology.
That brings us just kind of stepping over the threshold to the people who really annunciated the real modern teachings.
Jackson and Gowers, two of the giants working with them Ferrier and Horseley also. All of these gentlemen in the same area and in the same hospitals, working together in the same hospitals largely at Queen’s Square. There was in one of the references a description of how these four men interacted with one another. It was kind of interesting, Jackson was termed the neurological philosopher. Gowers was the man who laid the foundation of basic clinical neurology upon which the work of Ferrier and Horseley were the experimental investigators who were raising the pillars and Jackson was a neurological philosopher who directed the fine -----buccathis? For the perfectual strength of the School of British Neurology at the time and they were all very active in dealing with epilepsy. Epilepsy was a major interest for all of them.
Jackson, of the group, probably made the most interesting original observations. He came from an interesting background. I think it can keep us all a little bit humble. He was the son of a Yorkshire farmer and again an interesting phrase. He went through the customary years of indifferent schooling that people were subjected to at that time. He apprenticed as a medical student at the York School of Medicine and St. Bartholomew’s Hospital. He qualified as a physician at the age of twenty but after qualifying, came very near to leaving medicine. He had a major interest in philosophy. This is a curious thing for him because he was not a tremendously literate man. He did not speak easily. Words did not come easily to him. He did not write easily. Most of his writings are rather tedious and very repetitious going over and over the same ground with footnotes on footnotes qualifying, expanding and modifying statements that he had made on the main body of his writings. He was, however, I guess fortunately for all of us, persuaded by medicine, and worked with Dr. Laycock at St. Bartholomew’s and Dr. Laycock, I believe, went on to Edinburgh and became professor of Medicine there. He also worked at Hutchinson and Brown – Sequard I don’t know if he overcame that. After cleaning house I guess things looked up and Jackson spent the rest of his life working at Queen’s Square and working also at Morefield’s Eye Hospital. This was another area where he was very active and shared his interest with Gowers. He is responsible for the principles of corticolocalization and this was one of his main contributions. Through observation of patients and particularly through observation of seizure patients, he had a very personal interest in this, his wife had focal epilepsy, he arrived at a crude cortical map through the clinical progression of the seizure discharge in focal epilepsy. This was in advance of any experimental work. He produced this purely from his clinical observations he very happily had this confirmed by Ferrier and Horseley a few years later working in the same group and with the interdiscussion they were able to reproduce convulsions by electrical stimulation to the animal brain and to carry out cortical mapping in the animal and to confirm his thesis concerning discreet cortical localization of motor and sensory functions to the brain. Pretty much all else evolved from this in terms of this concept of focal cortical function is really essential for any concept of surgical therapy of epilepsy. This is why we touch on him. In the Neuro-surgical field, Jackson is probably put on about as high a pedestal as any of the modern neuro scientists.
Now Gowers played a major role in making sure that the findings and teachings and beliefs of this group at Queen’s Square got national and international recognition. Gowers was a teacher par excellence. He was someone whose words came easily. He had the social graces that Jackson did not. Jackson was always described as a rather stern, austere, not unfriendly, but rather unapproachable sort of person. In Jackson’s writings, he always discussed the illness. He never discussed the patient.
Gowers was quite a different type of individual, he was considerably warmer and he really is responsible for the acceptance and spreading of the gospel of much of the work done by the group at Queen’s Square. His textbook in Neurology became the bible of Neurology for many years. He also did that manual and Atlas of Medical Ophtalmoscopy. He was quite a talented painter. He regularly had paintings hung at the Royal Academy of Arts. He also was a bit of a nut particularly in his later years. Gowers had a real thing about shorthand. The three pictures I had was a photograph of Jackson and Gowers and another couple of gentlemen but he looks very distinguished, very neatly attired. Jackson, at least in his reproductions, his beard is a little bit -------------- and his eyebrows were a little bit fine and he looked rather formal. Gowers looks extremely polished sauve with a neatly trimmed, though fairly full beard and looked like a gentlemen of the drawing room yet there is apparently a very valid antidote to this gentlemen in his later years. Going through Southampton row he asked his coachman to stop leaping out, leaping out, going over grabbing a young man that he didn’t know by the arm and asking him “young man do you know shorthand”. The young man looking rather astounded at this man with the flowing beard, he said “you are a fool ----------. He was a little bit of a nut as well, but these eccentricities are kind of nice.
The medical people in spite of the fact that now it was known that many seizures had a focus and could be treated surgically. There was still a problem with surgical approach to this and that is a spinal fluid as an excellent cultural medium and this made it much more difficult to approach the central nervous system then any other part of the body. So the next big step was the introduction of the Bromides by Seguinne for uterine epilepsy in 1857 and two years later Samuel Wilks introduced bromides in a general for the therapy of epilepsy. This was the first medication that really worked. It was a quite a time on until something else came along the barbiturates introduced in 1912 but really didn’t become widely used as a therapy for epilepsy until around 1922 and then of course the giant step the introduction of diphenylhydantoin in 1938. Tracy Putnam and Houston Merritt did all the hard work but Tracy Putnam was the general who said “I think you ought to do this go do it” and Merritt went and did it and really was a screening of a large number of possibly anticonvulsive medication.