INTRODUCTION
The inspiration to write the present volume emanated from the present Editor's love for Sir William Gowers' last book “The Borderland of Epilepsy” published in 1907 wherein he described intermittent disorders, more or less resembling epilepsy but without any pathological substrate: Faints, vagal attacks, vertigo, migraine, and sleep disorders. The Editor, therefore, feels imperative to highlight briefly on the life and legacy of this great man early in the present volume. The stress, of course, would be on his contributions in understanding the nature of epilepsy and its borderlands. Much of the modern practice of clinical neurology in the English speaking world has its root in the practice of British neurology in the late 19th and early 20th century. Of the handful of people who dominated the British neurological scenario in those days, the name of William Gowers stands foremost for his contribution to descriptive and diagnostic neurology. Gowers' legacy in neurology was immense. He was the apogee of the great clinician and was renowned for his uncanny diagnostic accuracy in an era in which ancillary diagnostic tests that have come to dominate modern neurological practice were essentially nonexistent. His diagnostic acumen facilitated pioneering attempts to successfully develop surgical approaches to both the spinal cord and the brain.
William Richard Gowers (Fig. 1.1) was born on March 20, 1845 in the small English village of Hackney, and was the son of a shoemaker and retailer. Despite the apparent modesty of his family's means, he received an excellent education. Following elementary education at a local parochial school, he attended the Christ Church College School until the age of 15 at that time he was apprenticed to a country practitioner, Dr Thomas Simpson in Coggeshall, Essex. His responsibilities included dispensing medication and driving Dr Simpson's carriage. During this period he found time to learn shorthand and to amuse himself with hobbies that included chess, botany, and sketching.2
In 1863, at the age of 18, he left his apprenticeship to begin formal medical training at University College Hospital, London. At University College Hospital, Gowers received extensive exposure to neurology. Several faculty members including Sir J Russell Reynolds, H Charlton Bastian, and Edward J Steve King devoted considerable amount of time to the care and treatment of neurological patients. He received his MRCS in 1867, MB in 1869, and MD with Gold Medal in 1870. Gowers appeared to have been a diligent and accomplished student, winning many medals and other awards. Several of his early textbooks survive, and are filled with marginalia and other annotations. One indication of his success was the fact that his first postgraduate position was as assistant and secretary to Sir William Jenner. Jenner, at that time, was the uncontested dean of the Royal College of Physicians, and the personal physician of Queen Victoria. In 1870, at the age of 35, Gowers successfully applied for the position of Medical Registrar at the new Hospital for the Paralyzed and Epileptic located at No. 24 Queen Square. Bloomsbury.3 "Queen Square", as the hospital quickly became known, had University College Hospital neurologists Reynolds, Charles Édouard Brown-Séquard, Thomas Buzzard, and CB Radcliffe. By 1870, Queen Square had 64 in-patient beds and an out-patient clinic volume in excess of 1,200 visits/year involving over 2,000 different patients.
In 1872, Gowers was promoted to Honorary Assistant Physician at Queen Square and to Assistant Physician and Assistant Professor of Medicine at University College Hospital. It was at this time that the first of Gowers' papers, consisting predominantly of reports of interesting cases, appeared in the medical literature. These early papers, although not earthshaking in significance, reveal something of his peripatetic interests. Among the topics covered were facial paralysis, pseudohypertrophic muscular dystrophy, convulsions, cerebral embolism, post hemiplegic movement disorders, brain tumors, and vertigo. During this period, Gowers also contributed an interesting paper on symptoms of organic brain disease to the inaugural issue of brain.4 In this paper, Gowers notes that abrupt onset of symptoms, such as paralysis, in patients with brain tumors which may have resulted from hemorrhage into the tumor or from infarction secondary to either thrombosis or neoplastic invasion 3of nearby vessels. Taken together, these papers reveal the young Gowers as a keen clinical observer, who was hard at work, honing his clinical skills and meticulously recording the details of every case he encountered.
After a swift start, Gowers' further promotion at the National Hospital was exceptionally slow; it was not until 20 years later that he become full (or senior) physician. He was of course appointed Professor of clinical medicine and staff member at University College Hospital. In 1888, at the age of 43, Gowers took the decision to retire from all commitments at University College Hospital and devoted the rest of his working life to teaching neurology at the National Hospital, Queen Square, London.
For 20 years, Gowers continued to examine and treat patients in a small ill-equipped room. With great thoroughness he recorded in shorthand their symptoms and signs. The enormous body of information and observation that he collected formed the basis of his manual5 and other monographs. He extensively toured Europe and United States where his lectures attracted large number of students and post-graduates. His accomplishments rendered the National Hospital at Queen Square the stature of Mecca of Clinical Neurology worldwide.
Gowers' medical career came to an end with his retirement in 1907 at the age of 62. He died 8 years later. His health had never been good.1 He suffered repeated bouts of appendicitis in an era when this was diagnosed as "perityphlitis" and its surgical treatment was a novelty. In 1894 he had a "breakdown", relieved by one of his rare trips outside England—a long sea voyage to South Africa.1 Later in life he suffered from one of the syndromes he described, ataxic paraplegia. This progressive spinal cord disease had features resembling subacute combined degeneration, in an era before this disease had been described, or its association with B12 deficiency recognized.
We know little of his personal pleasures, and even less of the intimate details of his private life. He apparently had avid interests in botany, art and drawing, and early English archeology. Perhaps surprisingly he appears to have enjoyed and even embraced technology. He loved gadgets of all types and his contemporaries describe him as among the first to install such technological wonder as electricity and the telephone in his house. This enthusiasm for technology was also involved in his professional life. As noted earlier Gowers developed many medical devices, avidly employed the ophthalmoscope and medical electricity in his practice, and frequently illustrated his lectures with photographs. He had an almost obsessional interest in the medical use of shorthand ("phonography") and proselytized endlessly on its behalf.6–8
GOWERS' CONTRIBUTIONS: SOME GLIMPSES
In Gowers' early papers we see the beginnings of what would become a lifelong interest in medical gadgets.1 He developed a safety hypodermic syringe9 with a special stop on the barrel to prevent inadvertent over-delivery of medication. Hawksleys, the medical instruments makers, made the device to Gowers specifications. Gowers would subsequently develop a hemocytometer that was the first to put counting graduations on the slide rather than the eyepiece of the microscope,10 and a hemoglobinometer that determined the hemoglobin content of the blood by colorimetric comparison of the sample with carmine standards.11 Gowers' hemocytometer and hemoglobinometer were in widespread use for several decades. It was during this early period that Gowers mastered the use of the ophthalmoscope. 4His expertise in the use of this instrument drew Gowers to the attention of his senior colleagues. No less august an authority than Hughlings Jackson specifically commented on Gowers’ expertise with this instrument. In 1879, Gowers published his manual Atlas of Medical Ophthalmoscopy.12 Gowers’ “Ophthalmoscopy” became the standard book on the subject and appeared in second (1882),13 third (1890),14 and fourth edition,15 the last two being with the assistance of Marcus Gunn. It is largely due to Gowers’ teaching that the ophthalmoscope became a standard instrument in neurological practice.
In 1878, Gowers wrote a short monograph on Pseudohypertrophic Muscular Paralysis. Given his young age, it is remarkable that he was able to accumulate personal observations on over 20 cases. This monograph followed what would become lifelong practice—clinical observation provided the grist for a series of lectures, which were published in extension by one of the medical journals.16 As further cases were accumulated, the published lectures were, in due course, expanded into a more substantial monograph.
In this case, his "Clinical lecture on pseudohypertrophic muscular paralysis” was delivered to the medical students of University College at National Hospital for the Paralyzed and Epileptic. The lecturers were reprinted nearly verbatim in the Lancet issue of July 1879,16 and published in book form in November of the same year.17 1880 saw the publication of Gowers' Diagnosis of Diseases of the Spinal Cord.18 This was followed 5 years later (1885) by publication of his Lectures on Diagnosis of Diseases of the Brain.19 These volumes would form the starting point for the subsequent publication in 1886/1888 of Gowers' landmark book “A Manual of Diseases of the Nervous System.20 This book has been justifiably referred to as the "Bible of neurology." Gordon Holmes, writing in 1951, called it, "a living classic in clinical medicine”.3 Sir William Osler wrote that the Manual has placed the author, "among the highest living authorities on all matters relating to the nervous system." A Second edition was published in 1891–1893, and third edition of volume I was published with the assistance of James Taylor in 1899.21 Critchley mentioned the existence of a draft version of the third edition of Volume II but it was never been published. The Manual was not intended as an encyclopedic review of previous contributions to neurology, but is rather a personal compilation of Gowers' own experience with neurological disorders. This experience was supplemented when necessary by selected references to the work of others. The book is profusely illustrated with Gowers' own drawings.
It is not possible in a brief space to do justice to the contents of the Manual. Perhaps the best introduction can be found in JD Spillane's Doctrine of the nerves.22 Among the highlights enumerated by Spillane are the sections of the Manual dealing with symptomatology in which Gowers describes his method for evaluating motor power and eliciting reflexes. In the section dealing with "diseases of the nerves" there are wonderful descriptions of many types of individual nerve lesions as well as vivid portrayals of neuropathies due to lead and alcohol. Part 111, Diseases of the Spinal Cord, includes a concise review of the anatomy of the cord as well as a reproduction of Gowers' earlier figure showing the relationship between spinal cord segments and vertebral bodies. There are detailed clinical descriptions of locomotor ataxia, primary lateral sclerosis, ataxic paraplegia, Friedrich's hereditary ataxia, progressive muscular atrophy, poliomyelitis, and syringomyelia. Descriptions of acute ascending paralysis (later described by Guillain, Barré, and Strohl) and muscular dystrophy are included in this section, reflecting then contemporary views of their etiology, but in no way detracting from the clinical acumen reflected in Gowers' description.5
The final section of the Manual, which forms the entirety of Volume II, is devoted to diseases of the brain. Spillane noted that, "It is a tour deforce, which if published today (1981) in paperback, would still provide a sound and readable introduction for students."22
The sections on the symptoms of brain disease and their localization make particularly worthwhile reading. There are remarkable chapters on paralysis agitans, multiple sclerosis, writer's cramp, vertigo, migraine, and lead poisoning. One also finds, avant la lettre descriptions of what would later be called Wilson's disease (“tetanoid chorea"), Schilder's disease ("diffuse sclerosis"), and probable progressive multifocal leukoencephalopathy ("military sclerosis"). Although Gowers remained active in neurology for many years after the publication of his Manual, the luster of many of his subsequent contributions is perhaps unfairly diminished by comparison to his magnum opus. In 1892, he published his Lettsomian Lectures on syphilis,23 and in 1904 a collection of Lectures with the title “Subjective Sensations of Sight and Sound, Abiotrophy, and Other Lectures.”24 This contains several interesting articles including one on myopathies in which Gowers describes distal forms that undoubtedly included cases that would later be classified as myotonic dystrophy and inclusion body myositis. Gowers’ Bowman Lecture, from which the volume took its title, provided fascinating descriptions of migrainous auras and other visual spectra. A lecture on "abiotrophy", delivered at the National Hospital in 1902,25 had a strong influence on ideas concerning the etiology of “degenerative diseases", specially amyotrophic lateral sclerosis (ALS) and hereditary ataxias, which sounds very similar to some of the current thoughts on these diseases.
GOWERS ON EPILEPSY
Gowers collected cases of epilepsy and meticulously recorded clinical observations on the manifestations of this disease. In 1879, Gowers was elected a Fellow of the Royal College of Physicians (FRCP). As the College's youngest recruit in that year he was given the honor of delivering the Gulstonian Lectures.26,27
He chose as his topic epilepsy. He based his lectures, which were widely reported in the major medical journals including Lancet and the Brmsh Medical journal, on his personal experience with 1,450 cases. His lectures were expanded and published in book form (Epilepsy and Other Chronic Convulsive Disorders. Their Causes, Symptoms, and treatment) in 1881,28 and with the assistance of James Collier and Victor Horsley, in a second edition in 1901.29 By the second edition, he was able to report on over 3,000 personal cases, suggesting that he was accumulating approximately 150 new cases a year. This book remains a clinical masterpiece, which has not been surpassed in the quality of its clinical observations even in the modern era.
In the second edition of the book,29 in addition to the introduction, in 12 chapters (with several subtitles) Gowers discussed the various facets of epilepsy including its etiology, symptoms (3 chapters), organic epilepsy, morbid associations (heart disease, chorea, myoclonus multiplex, and migraine), course, pathology, diagnosis, prognosis, and treatment. Hystero-epilepsy would be commented upon separately at the end of this chapter for its special relevance to the present volume. Gowers commented on the sex differences, hereditary factors, birth asphyxia, trauma, febrile illness, and other well-known etiologic factors. The more exciting predisposing factors mentioned include inherited syphilis, worm infestation, sun exposure, prolonged mental anxiety, and masturbation but not tobacco smoking. A masterly discourse in 6three chapters dealt with the clinical symptomatology of various forms of epileptic attacks. Although terminologies have changed, very little has been added to Gowers' meticulous observations in the past over 100 years. Gowers used the term “organic epilepsy” to denote today's concept of symptomatic or secondary epilepsy. Although he made passing remarks about brain tumors, he mostly dealt with cerebrovascular diseases (arterial and venous) at various ages. Classic descriptions of partial, complex partial (minor epilepsy) and partial with secondary generalization (major epilepsy) attacks have been provided.
Gowers' association of chorea with epilepsy actually is interesting but most likely represents coincidence rather than sharing of same pathway, though he hinted at a state of “cerebral instability” in both. He also mentioned of myoclonus multiplex and referred to Unverricht's work (1891) and concluded that such cases “generally presented evidence of more considerable change in the cortex of the brain than is present in ordinary epilepsy.”
Gowers discussed course of epilepsy to take one of three forms. First, by minor attacks (petit mal) which occur alone for months or years before there are severe attacks. Second, by severe fits recurring at short intervals, without any preceding petit mal and third, with a single severe fit and no other fit or sign of epilepsy for months and even years when another attack occurs, after which the intervals gradually become less. He made meticulous observations on the intervals between fits in over 100 cases. He briefly wrote on status epilepticus (etat de mal épileptique of the French) and mentioned of the fatal outcome in many in those days. Gowers discussed the problems of diagnosis of epilepsy under seven headings beautifully summarized in the opening paragraph of Chapter X. These include: (1) The recognition of the occurrence of attacks; (2) The distinction of the attacks from other paroxysmal affections with which they may be confounded; (3) The distinction of hysteroid from epileptic attacks; (4) If there is hysteroid convulsion, we have to ascertain whether it is simple or consecutive to an epileptic seizure.
Gowers’ views on many of these questions would be discussed in a detailed manner later in this chapter as some of these are very relevant to the theme of the present volume. Does seizure beget seizure? This is a hotly discussed topic in modern epileptology. Gowers summarized his views while discussing prognosis in Chapter XI of his book. He made detailed observations on the effect of various factors contributing to overall prognosis and these include sex, age at commencement, duration, inheritance, exciting causes, frequency of attacks, presence of aura, mental state, and character of fits. Much of these still remain valid even today. Although antiepileptic drug therapy was at its infancy in Gowers' time, there is an in depth discussion on treatment in the last chapter of his book. The most efficacious agent was of course bromide which Gowers dealt in details including its mechanism of action and dosage. Various adjuncts to bromides have been mentioned including Digitalis, Strophanthus, Belladonna, cannabis, Stramonium, heroine, and Gelsemium. Illustrative cases have been cited to highlight their usefulness. Other antiepileptic agents mentioned include opium, borax, nitroglycerine, zinc, iron, etc. Gowers did not find chlorate hydrate to be useful in ordinary cases of epilepsy but recommended its use (with bromide) in treatment of status epilepticus. The social aspects of management are also included in a section devoted to education and occupation and on marriage. Gowers opines that marital relation “in moderation” has no influence on the disease but he made rather detailed comments on the risk of the offspring developing the disease. The final section of treatment deals with surgical treatment of epilepsy and was contributed (at least partly) by Victor Horsley. The 7measures discussed include counter-irritation, ligature of vertebral artery, resection of the cervical sympathetic, and of course trephining. The indications have been thoroughly discussed and the author (probably Horsley) opined that “an operation is more often justified by success in the case of organic epilepsy with convulsion of local onset, following a blow, than in cases in which the causal lesion is the result of disease”.
This brings us to understanding the pathology of the disease process which Gowers discussed in an earlier chapter (Chapter X of his book). Gowers seemed surprised to find that the “naked eye appearance of the nerve centers in idiopathic epilepsy is for the most part that of healthy organism” also the microscope not to have “thrown much light on the nature” of the disease process. He doubted whether any great importance is to be ascribed to the “induration of the cornu Annionis pes hippocampi” to which much importance had been given by Meynert and others shortly before Gowers' time. He, therefore, stressed more on the pathological substrates of organic epilepsies, clinical data, and results of experimental studies to unravel the genesis of seizures in idiopathic epilepsy. Although he referred to the works of Brown-Séquard, Kussmaul and Nothingel, in support of a brainstem “conclusive centre”, he felt like Hughlings Jackson that “the process of the fit commences in the cerebral bemispbere”. The only form of seizure which might have a medullary origin, he believed, was those associated with a “pneumogastric” aura. He, however, was not fully convinced and thought such auras might arise from central representation areas of the vagus in the cortex. Gowers tried to distinguish between the seat of the discharge and the seat of disease process in idiopathic epilepsy. After detailed discussion, he concluded that both must lie in the cerebral cortex. However, basing his observation and thoughts of the contemporary anatomical and physiological knowledge, he concluded that” physiological knowledge does not enable us to discern clearly the nature of the process of “discharge.” The precise nature of nerve force and its conduction are still matters of discussion."
WILLIAM GOWERS AND THE BORDERLAND OF EPILEPSY
William Gowers' last book “The Borderland of Epilepsy”30 (Fig. 1.2) was published in 1907 from London, only a single edition was published. The subtitle of the book describes the topics covered: “Faints, Vagal Attacks, Vertigo, Migraine, Sleep symptoms, and their Treatment”.
Gowers mentioned in the Preface that such conditions “seemed to be in the borderland of epilepsy-near it, but not of it. Many were so placed by their features and characters, others because they had given rise to erroneous diagnosis”. Gowers justified the compilation of the features of these conditions in a single book form as “the unfamiliarity, to most persons, or many of the facts and conclusions made it desirable to describe the evidence on which they rest”. And this had been done all through the book with the help of several illustrative cases and their critical analysis.
The present chapter summarizes and comments on Gowers' important observations on the aforementioned conditions and specially their distinction and relationship to epileptic seizures as conceived over 100 years ago by the Father of British Neurology.
Faints and Fainting
Chapter 1 of the book discusses faints and fainting. Gowers used the term “cardiac syncope” to discuss most forms of faints in contrast to the current practice of using the term when syncopes are related to primary cardiac diseases. Gowers mentioned only one form of true cardiac syncope and that is sudden death in patients with aortic regurgitation. He doubted whether true cardiac syncope ever causes absolutely sudden loss of consciousness, except when this is due to a “Fatal arrest of the action of the heart”. He believed, it is seldom, if ever so sudden as to cause “a harmful fall”. Clearly this was before Stokes–Adams attacks and bradyarrhythmias were recognized.
Simple fainting or vasovagal attacks had been discussed in detail with many illustrations. The pathogenesis of loss of consciousness is discussed at length and Gowers commented that “the loss must be immediately due to a state of the nerve elements of the brain produced by the change in the circulation”. He, however, argued against a pure circulatory factor to account for the loss of consciousness putting forward the view that nerve cells derive nutrition from the extracellular fluid rather than directly from the blood in the arteries. As extracellular fluid mass is unlikely to be affected by sudden and transient failure of pumping action of heart, the vascular theory might not be a very appropriate one. He argued in favor of the “mechanical effect of cardiac failure” and thought the loss of pressure by the force of blood pumped to the brain to be of greater importance. Such loss may give a “stunning blow” to the brain altering the function of the nerve elements. He likened this to what occurs spontaneously in epilepsy and in concussion by an actual blow on the skull. He made in depth comments on the pathophysiology and proposed a unifying hypothesis to suggest that syncope, epilepsies and concussions all result from "a discontinuity (?functional) of conduction at the junction of neurons which composed such conducting path”. He mentioned of clinical points differentiating syncopes from minor epilepsy, the foremost of which is facial pallor. This and other peripheral manifestations of syncope were generally thought to be due to direct action on the centre for the vagus but Gowers argued against this. More likely, he thought, the effect is related to cortical areas projecting down to the cardiac centre in the medulla. In support, he mentioned of syncopes resulting from sudden severe pain, sudden outburst of emotion and syncopes precipitated by certain odorous substances. Although Gowers tried to differentiate clinically syncope from minor seizures, he thought however that the state of the nerve elements to be similar. In support, he mentioned of clonic movements during syncopal attacks—a phenomenon now 9well recognized and described as convulsive syncope. More importantly, he cited cases where patients experiencing repeated syncopal attacks ultimately developed frank epileptiform seizures. Gowers believed that the state of the “nerve elements that underlies the loss of consciousness in syncope, may, by repeated induction, acquire a tendency to spontaneous development, which constitutes minor epilepsy”. The “borderland” thus crosses the “borderline”. This is a difficult proposition to examine in the light of current knowledge regarding the pathophysiology of syncopes and epilepsy but the cases cited apparently seem very convincing. Discussing the diagnostic difficulties between faints and minor epilepsy, Gowers referred to the presence of a “dusky tint” coloration of skin (rather than pallor) to be suggestive of seizure. He also mentioned of acute chest pain preceding loss of consciousness as an epileptic aura (and not necessarily to suggest cardiac syncope); startles precipitating seizures (not syncopes; startle epilepsy is well recognized now) and sudden change in postures precipitating seizure and again not necessarily syncopes.
He stressed on the rapidness of the onset phenomenon to diagnose epilepsy than syncope but cautioned in the last sentence of the chapter: “But a single attack of this character does not justify a diagnosis of epilepsy, with all its attendant concern and anxiety”.
Vagal Attacks
Gowers discusses in his inimitable style with help of illustrative cases of vagal and vasovagal attacks in the second chapter of the book. A careful reading would reveal that his concepts of vagal and vasovagal attacks were clearly different from what is understood today. Such attacks had prominent respiratory, cardiac, and gastric components—organs richly innervated by the vagus but the symptomatology lacked any evidence of vagal hyperactivity. No mention has been made about any slowing of pulse though one patient had a “small” pulse. These attacks were prolonged one unlikely the usual syncopal attacks or epileptic ones and were marked by epigastric sensation, breathing problem, and chest pain. There were vasomotor problems such as coldness and moistness of extremities, facial pallor and even in some tingling in limbs and a tetanoid spasm. When vasomotor problems predominated, Gowers called them vasovagal attacks but there was never any loss of consciousness. Gowers referred to such cases being described earlier as “angina-Vasomotoria” and “syndromes médullaires”.
A careful reading of the symptomatology described would suggest that Gowers was probably describing cases with hyperventilation syndrome or panic attacks or even simply some cases with acute anxiety spells. The acral tingling and tetanoid, spasms in some would certainly suggest hyperventilation spells. The breathing in some subjects at times had been “so intense as to amount to orthopnea, and to compel the sufferer, if lying, to sit upright, although there is no corresponding sign of impairment of breathing”. Gowers himself commented on the influence of emotion on such attacks and mentioned of the “frequent submergence of these attacks, beneath the vague conception of hysteria”. These attacks were slow to evolve and prolonged and were very different from epileptic ones and Gowers did not consider them to lie in the borderland. He however, mentioned later in the chapter, cases that had “epileptic” spells while continuing to experience the vasomotor, respiratory, and “pneumogastric” symptoms. These, he thought, to be true “borderland” of epilepsy. Again careful reading of the illustrative case histories would leave some doubt. Features described such as visual phenomenon, epigastric sensation, and feeling 10of unreality, could well be epileptic auras and there had been instances of brief loss of consciousness while vasomotor and cardiorespiratory symptoms had been continuing. However, the attacks seemed too prolonged to merit the diagnosis of seizure disorder. Also no clear description of his subjects having convulsions are available. Both cases cited were females and doubt remains as to the organic nature of the attacks. Gowers however, argued in favor of a concept of extended epilepsy to explain these attacks and supported his contention by the response of some of them to bromides. He conceived “a minor epileptic attack that is extended, its elements protracted with no tendency to be terminated by loss of consciousness, its features would be so different that its nature would not be suspected. Velocity altogether alters the effect of momentum. A bullet fired from a rifle makes a round hole in a pane of glass, which it would smash if thrown against it. Swiftness is an essential element of ordinary epilepsy, but this does not preclude the possibility of deliberation”.
Vertigo
Gowers made a very detailed discourse on vertigo in the book in two chapters. In the first one, he dealt on the pathophysiology of vertigo and its possible physiological relationship to epilepsy. He was very clear in defining vertigo which always should have an element of “turning” and must be distinguished from other forms of giddiness and light-headedness. He mostly discussed aural vertigo produced by labyrinthine disease and the prototype had been Meniere's disease. He made no comments about more common forms such as vestibular neuronitis or benign positional vertigo nor of the central forms of the disease. However, he believed that the sensation of movement results from a mismatch at the central integrative level between afferent sensations coming from various sources. All forms of vertigo, therefore, he conceived, really were a form of cerebral cortical disorder and that is where its closeness to epilepsy lies. He commented at the beginning of the chapter that “the sensation called vertigo is the most frequent subjective evidence of an epileptic seizure.” Just as the central integrative mismatch in labrynthic vertigo, the rotational sensation in epilepsy may be ascribed to an “inequality in the connecting motor discharge in the two hemispheres or to the early discharge beginning in one hemisphere only, before consciousness is lost”. He felt, such inequality to manifest on head and eye deviation in seizure phenomenon. He referred to Hughlings Jackson's work and believed that vertigo would result when such inequality in the functioning of the cortex is too slight to cause actual movement. A more severe form would naturally cause convulsion to occur. In support, he cited the case of a “skilled medical observer” a subject of most severe form of aural vertigo, who occasionally noted at the height of his attacks clonic spasms occurring in the arm and leg on the right side toward which objects seemed to be moving. The present author is not sure how often such phenomenon are observed in clinical practice today, but as would be discussed later in this volume, vestibular epilepsy and nystagmus as a seizure phenomenon are well recognized, though very uncommonly.
Gowers thought that the features that are common to attacks of epilepsy and aural vertigo are suddenness, brevity, loss of consciousness, and loss of sight. Loss of consciousness is generally not considered a feature of peripheral labyrinthine vertigo but Gowers cited cases with brief spells of loss of consciousness specially when the subject had a fall. He commented “the condition in any attack of intense vertigo is one of imperfect perception of the surroundings and of their relation to the sufferer, a condition which itself involves imperfect consciousness”.11
One cannot be absolutely certain that such cases were indeed cases of peripheral origin vertigo and not central and also whether the brief memory loss accompanying was really due to loss of consciousness or simply due to loss of perception related to intense spinning of the surroundings. However, such instances certainly justify discussing vertigo in the borderland of epilepsy. Another aspect of vertigo spells mimicking seizure may be the loss of sight and Gowers cited illustrative cases. He commented “in epilepsy sight may be lost before consciousness and indeed may alone be lost. So in some of these epileptoid forms of pure vertigo”. It is likely that some subject may complain of loss of vision (or blurred) with intense vertigo when they actually experience oscillopsia. However, Gowers cited example of “transverse hemianopia” in a lady with clear labyrinthine disease. The anatomical explanation may be difficult unless one assume a vascular etiology in totality with affection of internal auditory artery and posterior cerebral arteries from diffuse posterior circulation disease.
In the last section of the chapter Gowers referred to “the sense of impulsion” specially in relation to the fall accompanying a sudden severe vertiginous spell which may have close resemblance to an epileptic warning or even fall. In epilepsy however, there may be a feeling of involuntary motion, but not of being “hurled to the ground” as patients with vertigo sometimes describe. Gowers mentioned of cases with labyrinthine disease who felt sudden sensations in the head with a feeling of loss of consciousness, very briefly unaccompanied by any significant vertigo and cautioned against misinterpreting them as epileptic auras. In the second chapter on vertigo, Gowers described more illustrative cases to explain the closeness of aural vertigo to epileptic attacks. He mentioned of “encephalic vertigo”—a curious prolonged cranial sensation in some subjects with aural vertigo (different from the usual subjective vertigo) which may be mistaken for an epileptic aura and the occurrence of sleep attacks, so common in patients with epilepsy.
Later he cites another case who had for a prolonged period a sensation of movement of objects and of herself to the right with nausea and a confused sound in the right ear ultimately culminating in a tonic-clonic seizure. Gowers referred this as “borderline epilepsy” a situation where “epilepsy comes near the dividing line” (from vertigo). Gowers postulated that the patient probably had a “small spot” of damage to the brain near the auditory centre in the first left-temporo-sphenoidal convolution with spread of discharge to the right ear (or left vestibular nucleus?) and the left motor cortex. Lastly, Gowers referred to the occasional occurrence of both aural vertigo and epilepsy in the same subject but believed that the relation is >1 of coincidence. One patient had, in addition to typical attacks of aural vertigo, fairly classic uncinate seizures. Another patient with fairly typical history of epilepsy after years of suffering from labyrinthine vertigo developed epileptic sensory auras at times with loss of consciousness. This, Gowers linked to the development of true epilepsy from recurrent vertigo, a proposition he made in relation to syncopal attacks as well (vide supra). Another lady had attacks of giddiness with tinnitus and nausea for a few years before which she started having typical temporal lobe (complex partial) seizures with lip smacking and loss of consciousness. Occasionally, her attacks of giddiness changed their character and the tinnitus was replaced by sound of ringing bells (a recognized auditory hallucination) and peculiar sensation of taste (gustatory aura) but no loss of consciousness. Gowers opined that the purely peripheral phenomenon, simply by repetition over years, clearly became, purely central in nature—a cortical phenomenon like epilepsy. Gowers also surmised that minimal labyrinthine disease (no hearing loss/tinnitus) could lead to disabling vertigo 12(pseudo-aural vertigo) for years presumably as a result of “central co-operation”. Thus the borderline between labyrinthine vertigo, a peripheral disorder and other central disorders like epilepsy, seems to get less demarcated in many subjects. One may now wonder whether Gowers was referring to recurrent vestibular neuronitis or benign positional vertigo, when he was referring to recurrent vertigo without any hearing impairment and repeating over years.
Migraine and Epilepsy
Perhaps the most fascinating of all the chapters in Gowers' Borderland has been the one (Chapter 5) in which he discussed the relationship between migraine and epilepsy. The opening paragraph summarizes his overall impression: “Some surprise may be felt that migraine is given a place in the borderland of epilepsy, but the position is justified by many relations and among them by the fact that the two maladies are sometime mistaken, and more often their distinction is difficult.” Gowers' views must be appreciated in the light of the fact that these were expressed long before either the vascular or neural theory of migraine became known and not to speak of the concept of neurogenic inflammation and the role of the raphe nuclei and their cortical projections. Of course, the discussion was mostly limited to migraine with aura and what we now know as partial and complex partial seizures.
The first point of similarity, as mentioned by Gowers was alteration in one disease often replaces the other in the same subject. He surmised that if a person, subject to migraine becomes liable to epilepsy, the migraine may cease or become slight and rare, and return if the fits are arrested. He cited examples in support of his contention. Gowers also mentioned of similar premonitory symptoms in migraine and epilepsy though he commented on the difference in the duration of the aura phase in the two conditions. Several forms of migraine with aura, both visual and somatosensory, were described in detail and reference was made to his own Bowman lecture delivered in 1895 on subjective visual sensations. Drawing analogy with the aura and spread of focal epileptic phenomenon he referred to the concept of his peer Hughlings Jackson. Gowers speculated on the pathogenic mechanism of spread of migraine aura as “a peculiar form of activity which seems to spread, like the ripples in a pond” into which a stone has been thrown. One would wonder at this close similarity between this concept and Leão's spreading depression described almost 40 years later. Gowers, of course admitted that the process must involve the “cortical centers of brain, but we know nothing of the precise nature of the process”. Of course, he raised the question of local arterial spasm but did not elaborate. On the other hand, he mentioned that the opinion that it is due to local vasomotor changes is difficult to reconcile with the character of the discharge, uniform in its general character, so orderly in its disorder.
The major points of distinction between migrainous and epileptic auras, according to Gowers include:
- Much briefer duration of epileptic aura—as referred to earlier.
- Occurrence of bilateral sensory disturbances, especially in hands, in migraine, and almost never affection of the feet in migraine—though common in epilepsies.
- Rarity of facial, lingual, and oral affection in migraine.
- Much less severe headache accompanying epileptic attacks than migraine.
Gowers described interesting illustrative cases where migraine auras were mistaken for epilepsy and the vice-versa. Gowers was astute enough to note isolated prodrome of migraine: Acephalgic attacks only with aura and commented on the “great perplexity” these may cause in differentiating from seizure phenomenon. He 13mentioned of guiding points which include most importantly the history of having suffered from paroxysmal headaches in the past with or without such prodromata. Such isolated prodromata rarely lasts more than half an hour, leaving the subject absolutely normal. Gowers however, cautioned not to ignore such focal prodromata in the elderly (degenerative periods of life) where these might have the gathered significance. It is likely that he was referring to transient ischemic attacks and subsequent completed stroke rather than migrainous cerebral infections. According to Gowers “another class of symptoms which may sometime bring migraine near epilepsy, and frequently seem to do so, is disturbance of other brain functions during pain”. Two such symptoms have been given importance—somnolence and delirium, and illustrative cases have been cited. Later he went on to describe cases with loss of consciousness following a migraine attack and conjectured on the development of epilepsy from migraine. The organicity of the loss of consciousness in some such cases may be questioned. The concluding remarks are worth reading bearing in mind that these were written over 100 years ago. Gowers commented “the literature of migraine is large but it contains little that bears on the relations to epilepsy that have been considered here”. He made only a reference to the writing of Edward Liveing without much elaboration; clearly he was influenced by Liveing's hypothesis of “nerve storm” in migraine which is probably the first insight into the pathogenetic aspect linking migraine with epilepsy. Overall, Gowers thought “the traces of a definite relation of migraine to epilepsy are slight” and kept his mind open when he wrote “at present it seems more useful to gather facts than to formulate a hypothesis, and especially facts on the outskirts of the affection, which connect its symptoms with those of other maladies”. He cautioned “the occasional occurrence of prodromal symptoms during the course of the headache may give rise to error, but this should be prevented by a knowledge of the facts.”
Sleep Disorders
In the last chapter of the book, Gowers wrote relatively briefly on some sleep symptoms which he thought may mimic epileptic attacks and hence to be considered to lie in the borderland. He however confirmed that “our ignorance of its (their) nature is still too great to make even speculation profitable.” The conditions he discussed include transitional disturbances, night terrors, somnambulism, and narcolepsy. The first category includes sleep startles which may be simple or more complex with complex sensory symptoms which may look like epileptic attacks and Gowers cited illustrative cases. Such transition may occur from waking to sleep stage as also from sleep to waking stage. Some may have vertiginous element and some vagal components and hence may be labeled as borderland attacks.
Night terrors, according to Gowers, almost always are associated with frightening dreams (night mares really) and though mainly seen in childhood, might persist to adult life up to the age of 30. As both night terrors and somnambulism may occur in patients with epilepsy, Gowers questioned whether these may be manifestations of post-epileptic automatism but opined that the phenomenon were mostly coincidental. Gowers referred to Gelineace who first described narcolepsy in 1880 as attacks of “sudden brief irresistible sleep”.
Gowers described classic cases of narcolepsy, some of whom in addition to such sudden attacks of irresistible sleep had other features distinctly epileptic. Gowers, however, cautioned against mistaking hysteroid attacks from true narcoleptic attacks. Also he stressed on the distinction between narcolepsy and minor epilepsy. 14He, however, stressed on the rarity of the former attacks. In those days, in absence of intensive monitoring, the distinction between minor epileptic attacks with brief spells of “going off to sleep” and psychogenic attacks must have been utterly difficult.
Gowers on Psychogenic Seizures
Gowers discussed psychogenic seizures in a chapter entitled “Hysteroid or Co-ordinated convulsions—Hystero-epilepsy” in his book on Epilepsy rather than in the Borderland; though several references had been made about this condition in the latter. Through several illustrations he described in detail the general characters of these attacks and their differentiation from organic epilepsy. He described hysteroid movements as of “more or less co-ordinated character, such as may be produced by will”. These he referred to as a “purposive aspect”. He mentioned of “laryngeal spasm” which in some cases forms a conspicuous feature of the attack. The fall accompanying such attacks is “often a sliding down rather than a fall” which initiates the coordinated movements usually clonic but at times preceded by a “tonic spasm”. Gowers commented that “consciousness is rather changed” rather than lost: A very vital phenomenon in the history and examination during an attack. Violence is often evident - the more restrain is used, the more is needed. At times “six persons had to sit on him to keep him down”. Gowers referred to a peculiar phenomenon where the noises and actions of animals are strongly imitated. Gowers described in detail the differentiating points in the “conclusion” of hysteroid fits and those of organic epilepsy in British subjects and commented on their differences from French subjects whose cases had been carefully studied at the Salpetriere especially by Charcot and Richer. In the latter race, the initial event resembled more of an epileptic fit with sudden fall, tonic spasm, head deviation and then clonic phase. However, these, were followed by the stage of “grands movements” with opisthotonus, bouncing movements, etc. Gowers commented that the “crucial test of Charcot—compression of the ovaries” was rarely successful in British patients. “An attack can scarcely ever be thus induced, and although it may sometimes be arrested by this means, the effect is not sufficiently constant to possess any diagnostic value. It is certain that in England attacks presenting as an actual combination of true epileptic and hysteroid symptoms are so extremely rare as scarcely to merit consideration. We have therefore no justification for the use of the word “hystero-epilepsy" in the sense it bears in France”. Thus a fairly clear difference in the concept of hysteria and hysterical seizures emerged between the French and British schools. However, interestingly enough, Gowers starts his case illustrations with, description of post-epileptic hysteria defined as “occurrence of hysteroid convulsions as a sequel to true epileptic fits.” Similar instances have been reported by Charcot as well and it appears that the "French connection” could not be easily ignored by the British physicians. Such instances, must be extremely rare in our current practice of neurology. This has been commented upon by the present author in a Commentary on Co-existing Epileptic and Nonepileptic Seizures later in this volume. A similar situation, had been thought to account for hysteroid convulsions during sleep: true sleep seizure followed by a nonepileptic event. Current opinion regarding such nonepileptic seizures occurring during sleep is somewhat different. Gowers went on describing and illustrating in detail “tonic spasm”- quoting Charcot's “crucification” posture, opisthotonus - mentioning and reproducing the drawing of Richer's “arc encircle” and clonic spasm and the co-ordinated movements. Self-inflicted injuries may occur at this stage (“dig the hands” into face and throat) but felt such phenomenon 15to be more common in postepileptic stage. Again, something, we rarely encounter now a days. Clearly, the “borderlines” were less clearly defined in Gowers' time. An unusual form of hysteroid seizure, was noted to have “laryngeal spasm”. Gowers termed these the “chocking fit” and the French writers “hysterical strangulation”. These were thought to be due to an extreme degree of adductor laryngeal spasm, minor degree of which leads to globus hystericus. These could be relieved with hypnosis. However, some even coughed up bloody mucus. It is difficult to conceptualize on the pathogenesis of these attacks. These could have been spells of severe hyperventilation syndrome, hypocalcemic laryngismus stridulus or perhaps deliberate abnormal sound production mimicking stridor. Similarly, Gowers also described pharyngeal spasm and quoted others who noticed severe degrees of the same phenomenon. Gowers mentioned of reported accidental deaths in hysteroid seizures but was generally of the opinion that these were true epileptic ones. He mentioned a number of examples of hysteroid convulsions in young men. Gowers commented on the interictal mental disturbances in between hysteroid attacks. However, as he noted, such disturbances were usually less marked in British subjects as compared to the French. The patients talk in an unnatural manner, and may have distinct hallucinations.
Gowers referred to suicidal tendencies in some of his subjects and postulated a close relation between these hysteroid maniacal states and epileptic mania. He also cited cases which illustrate close connection between the transient maniacal disturbance of hysteria and definite mental derangement: A condition which needs consideration when the maniacal behavior loses its paroxysmal nature and becomes a persistent one. In the concluding section of the chapter Gowers discussed the real “borderland”: Attacks intermediate between hysteria and epilepsy. This is different from instances where a subject manifests both epileptic and hysteroid attacks as discussed earlier. “Coordination of the muscular contractions which constitute the convulsion is the characteristic feature in hysteroid attacks; the absence of co-ordination is the characteristic feature of epileptic fit. But the initial phenomenon of some epileptic fits—such, for instance, as the visual and auditory warnings- show that the discharge in such attacks when slowly developed and in certain situations may be in the form of a co-ordinated nervous process. The same conclusion is suggested by some other initial symptoms such as the act of running.” In one of Gowers' patient thought to have the “intermediate form” both severe (thought epileptic) and slight (thought hysteroid) fits began with hopping round the room on one leg. Such stereotyped motor behavior, are now known to be recognized features of some forms of complex partial seizures. Hence, they are well within the “borderline” and not in the “borderland”. Advent of Video-electroencephalogram (EEG) can clearly (in most situations) demonstrate the two forms of attacks, but in Gowers' time, based purely on clinical grounds, the existence of intermediate forms of attacks was a very reasonable proposition in many patients with unusual seizures. Gowers concluded and justified his contention of intermediate forms “ the morbid action of the nervous system which causes the visible phenomena of attacks may in some cases present such a combination of the processes which underline the hysteroid and epileptic forms of convulsion, that attacks occur in which the characters of the two forms are combined at the same time and not merely associated in consecutive development舦舦舦. the two forms of the diseases are more separated by any fixed and impossible symptomatic boundary”. It must be stressed that these statements were made before electrophysiological characterization of the seizure phenomenon was made available.16
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