Musical and poetic creativity and epilepsy

Associations between epilepsy and musical or poetic composition have received little attention. We reviewed the literature on links between poetic and musical skills and epilepsy, limiting this to the Western canon. While several composers were said to have had epilepsy, John Hughes concluded that none of the major classical composers thought to have had epilepsy actually had it. The only composer with epilepsy that we could find was the contemporary composer, Hikari Oe, who has autism and developed epilepsy at age 15 years. In his childhood years, his mother found that he had an ability to identify bird sound and keys of songs and began teaching him piano. Hikari is able to compose in his head when his seizures are not severe, but when his seizures worsen, his creativity is lost. Music critics have commented on the simplicity of his musical composition and its monotonous sound.Our failure to find evidence of musical composers with epilepsy finds parallels with poetry where there are virtually no established poets with epilepsy. Those with seizures include Lord George Byron in the setting of terminal illness, Algernon Swinburne who had alcohol-related seizures, Charles Lloyd who had seizures and psychosis, Edward Lear who had childhood onset seizures, and Vachel Lindsay. The possibility that Emily Dickinson had epilepsy is also discussed.It has not been possible to identify great talents with epilepsy who excel in poetic or musical composition. There are few published poets with epilepsy and no great composers. Why is this? Similarities between music and poetry include meter, tone, stress, rhythm, and form, and much poetry is sung with music. It is likely that great musical and poetic compositions demand a greater degree of concentration and memory than is possible in epilepsy, resulting in problems retaining a musical and mathematical structure over time. The lack of association between recognizable neuropsychiatric disorders and these skills is a gateway to understanding facets of the relationship between the brain and creativity.This article is part of a Special Issue entitled “Epilepsy, Art, and Creativity”.     

Dostoevsky's epilepsy: a new approach to retrospective diagnosis

There has been considerable debate about Fyodor Dostoevsky's epilepsy. Was his epilepsy generalized or focal? Was the dramatic ecstatic experience an epileptic phenomenon or a literary invention? We compared probable epileptically related behavioral manifestations in The Idiot with experiences of current patients, studied with a modern interdisciplinary approach involving neurosurgery, neurology, and neuropsychiatry. Patients were studied by all disciplines starting with their initial evaluation and trial of antiseizure medication, during hospitalization for long-term monitoring for epilepsy at scalp and depth levels with electrical brain stimulation, during intraoperative interviews, and in long-term postoperative follow-up. Behavioral manifestations, clearly shown to be phenomena of the epilepsy in our patients, were the template for defining the epileptic nature of the behavioral symptomatology described by Dostoevsky in his literary character, Prince Myshkin. We conclude that Dostoevsky had temporolimbic epilepsy and that the ecstatic experience is an epileptic phenomenon.     

The relationship between epilepsy and religiosity illustrated by the story of the visionary mystic Wise-Knut

The story of Wise-Knut is remarkable. He was born in a poor mountain district in Norway in 1792 and lived for 84 years. He had severe and untreated epilepsy with apparent ictal, postictal, and interictal religious symptoms. He heard voices and had religious delusions; a spiritual awakening after a seizure cluster was a turning point in his life. Contemporary biographers have narrated his major life events in detail, but without a precise separation between ictal and postictal spiritual symptoms. Religious and supernatural significance was attributed to his experiences; he himself believed that his extraordinary abilities were a gift from God: “The prophets have had it like myself.” His story corroborates the impression that epilepsy may have had a considerable role in the history of religions.However, apart from anecdotes on visionary and healing abilities, his biographies contain nothing that is miraculous or incredible. He falls into the line of various mystics and religious figures of the past that are currently thought to have had epilepsy. Apparently, the advancing understanding of epilepsy and its complications have influenced the dynamic balance between faith, superstition, and rationalism.     

A parent's perspective on dietary treatments for epilepsy

Four families gave their accounts of the how Dietary Therapies had impacted on their lives and that of their children who suffered with intractable epilepsy. All with very different stories, experiences and outcomes. Niamh was diagnosed with migrating partial epilepsy of infancy with an underlying metabolic problem was fed via her jejunostomy and the parents overcame every obstacle in their path to have as much time with their daughter as possible. Niamh's family now work with the Matthew's Friends organization to promote dietary awareness and make these treatments available for all those who need them. Carson, who with infantile spasms, was able to access the diet as her first line treatment without any medication being taken. Carson is the inspiration behind the Carson Harris Foundation and her family promote dietary awareness in USA. Matthew, with Dravet Syndrome, was refused for the diet for years, suffered brain damage and was put on a whole host of unsuitable medications before finally managing to get the diet. The diet proved his saving but what could have been the outcome if the family had got the diet when it was first asked for? Matthew is the inspiration behind the Matthew's Friends - Dietary Treatments for Epilepsy organization. Charlie, suffered with intractable epilepsy, was put on a whole host of medications that did not work and underwent brain surgery before finally getting to the diet that cured him of his epilepsy. He is the inspiration behind The Charlie Foundation in the USA and his father, Jim Abrahams made the film 'First Do No Harm' which told the true story of a child who was cured of his epilepsy using the Ketogenic Diet, just like Charlie and thousands upon thousands of other children around the world. The Charlie Foundation and Matthew's Friends work side by side in the promotion, education and funding of these treatments and have asked other family organizations to work with them so that families of the future will not have to struggle to gain the information that they so desperately need. Families have the right to make an informed choice and these organizations work together in order to be able to provide this.     

Current controversies in the relationships between autism and epilepsy

The controversies that have arisen in endeavoring to establish the nature of the relationships between autism and epilepsy might be summarized in a few simple questions, most of which do not yet have clear, complete answers. Does epilepsy cause autism? Does autism cause epilepsy? Are there underlying brain mechanisms that predispose to both conditions? What is the role of genetics in this regard? What is the importance of prenatal, perinatal, and postnatal environmental factors? Do any of the proposed relationships between autism and epilepsy provide insight into useful management or treatment? Is the prognosis of either autism or epilepsy different when the other condition is also present? What is the role of additional comorbidities, such as intellectual impairment or attention deficit hyperactivity disorder, in the relationship between the two conditions and in influencing treatment choices? From the evidence currently available, it would appear that epilepsy can rarely be the cause of autistic features but is not the cause of autism in most cases. There is currently no credible mechanism for suggesting that autism might cause epilepsy. There is strong evidence for an underlying predisposition for both conditions, particularly arising from genetic investigations. However, many issues remain unresolved. Considering the amount of research that has been published in this area, it is surprising that so few definitive answers have been established. The papers in this issue’s special section provide additional insights into the relationships between autism and epilepsy; while they do not provide answers to all the questions, they represent considerable progress in this area and, at the very least, give some strong indication of what research might, in the future, provide such answers.     

Ethical issues related to epilepsy care in the developing world

There are three major issues of ethical concern related to epilepsy care in the developing world. First, is it ethical for a developing country to channel its limited resources from direct epilepsy care to research? The main considerations in addressing this question are the particular research questions to be addressed and whether such research will bring direct benefits to the local community. Second, in a country with limited resources, when does ignoring the high treatment gap become an ethical issue? This question is of particular concern when the community has enough resources to afford treatment for its poor, yet is not providing such care because of gross wastage and misallocation of the national resources. Third, do countries with plentiful resources have an ethical responsibility to help relieve the high epilepsy treatment gap of poor countries? Indeed, we believe that reasonable health care is a basic human right, and that human rights transcend national boundaries. Although health care is usually the responsibility of the nation‐state, many modern states in the developing world are arbitrary creations of colonization. There is often a long process from the establishment of a political‐legal state to a mature functional nation. During the long process of nation building, help from neighboring countries is often required.     

Ictal asystole—Late manifestation of partial epilepsy and importance of cardiac pacemaker

Ictal asystole (IA) is a life-threatening complication of epilepsy and is a potential mechanism of sudden unexplained death in epilepsy (SUDEP). This entity has been proven by multiple case reports and small case series. The management of the patients with IA is still in early phase of discussion. We report a patient with medically intractable cryptogenic partial epilepsy for 27 years who presented with new onset drop attacks. During the epilepsy monitoring unit stay he was found to have a left fronto-temporal partial onset seizures which triggered brady-arrhythmia followed by asystole for 20 s. A cardiac pacemaker was implanted and the patient was followed for 2 years. He continued to have simple and complex partial seizures but did not have drop attacks anymore. He still occasionally feels the activation of his pacemaker during simple partial phase of his seizures but the characteristic loss of muscle tone never happened again which made him highly satisfied. Our case demonstrates that IA can even happen decades after the onset of epilepsy. Cardiac pacemaker should be considered in all patients with IA as it prevents ictal falls and possibly SUDEP.     

Todd, Faraday, and the electrical basis of epilepsy

PURPOSE: To consider the origins of our understanding of the electrical basis of epilepsy in the light of the Lumleian lectures to the Royal College of Physicians in London for 1849, "On the pathology and treatment of convulsive diseases," by Robert Bentley Todd (1809-1860). METHODS: I have reviewed Todd's neglected Lumleian lectures and his observations and concepts of the electrical basis of epilepsy in relation to the influence of Michael Faraday (1791-1867), his contemporary in London, and in relation to later nineteenth century writings on the subject by Jackson, Ferrier, and Hitzig, all of whom overlooked Todd's lectures. RESULTS: Todd was a clinical scientist as well as Professor of Physiology and Morbid Anatomy, with a special interest in the nervous system, at King's College, where he came into contact with Michael Faraday, the greatest electrical scientist of all time, at the nearby Royal Institution. On the basis of his own clinical and experimental studies and his cutting-edge knowledge of neuroanatomy, neurophysiology, neuropathology, and electrical science, Todd brilliantly developed his concepts of the electrical basis of brain activity and of epilepsy in particular. With his microscope, he perceived each nerve vesicle and its related fibres (neurone in later terminology) as distinct entities for the generation of nervous polarity (force) and its transmission in the white nerve fibres throughout the nervous system by unknown molecular mechanisms. In epilepsy, an increase in electrical tension, especially in the grey matter of the hemispheres, led to periodic, sudden explosive discharges, based on Faraday's concept of disruptive discharges. CONCLUSIONS: Todd was the United Kingdom's first outstanding neurologist and neuroscientist before these disciplines existed. Influenced by Faraday, he proposed and confirmed the electrical basis of nervous discharges in epilepsy more than 20 years ahead of Jackson, Ferrier, and Hitzig, who did not refer to his priority, although Ferrier also worked at King's College, and Jackson also gave his own famous Lumleian lectures on the same subject in 1890. Todd deserves the credit for laying the foundations of our modern understanding of epilepsy.     

Christiane vs. Goethe's final illness

This paper deals with the diseases of Christiane, Goethe's wife, during the period of their marriage (1806-1816). Recurrent stomach complaints, due perhaps to gastro-duodenitis or an ulcer, were, it is suggested, of psychosomatic origin. They might have resulted from an intrinsic conflict between Christiane's unsatisfied desire for nearness and Goethe's absolute insistence on periods of distance and seclusion for the sake of his creative work. In contrast to this, Christiane's final illness was organic in nature. Contemporary records clearly indicate that she was suffering, not from suspected cerebrovascular accidents, but from the late form of symptomatic epilepsy with single grand mal seizures documented in 1815. In May/June 1816 these gave way to a series of grand mal seizures continuing into a malignant grand mal state, from which she never recovered. Pathogenetic aspects of the disease, allowing for the hypothesis of a latent infantile cerebral paresis together with the proven alcohol consumption are discussed and, in particular, the reasons why the correct diagnosis has been missed by posterity, even though it was clearly pointed out by M?bius as long ago as 1903. It is suggested that persistent irrational and unobjective prejudices relating to epilepsy, to Christiane's personality and last but not least to M?bius' pathographic approach have, up to the present, led to a dismissal of the true diagnosis of Christiane v. Goethe's final illness.     

The idiosyncratic aspects of the epilepsy of Fyodor Dostoevsky

The goal of this article is to review the idiosyncratic aspects of the epilepsy of Fyodor Dostoevsky, one of the greatest writers of all time. The onset of his seizures is controversial, with some evidence pointing to his childhood and other reports that would place the onset in his teens or his twenties. His life in prison in Siberia and then in the Russian army is reviewed. His lifestyle included many factors that exacerbated his epilepsy, especially stress and sleep deprivation. His compulsion for gambling played an important role in producing great stress in his life, as he tried to reverse his poverty in the casinos. The most idiosyncratic aspect of his epilepsy was his so-called ecstatic aura. The etiology of his seizures was probably inherited as revealed by the seizures of his father and the status epilepticus and death of his young son. This great writer died from lung hemorrhages in 1891. Discussed in this review is that he did not likely have an aura of ecstasy; only a few such possible cases can be found in the world literature. For those few cases, evidence from electrical self-stimulation studies in animals and humans, investigating "pleasure centers," can be found to involve the limbic system, especially the septal nucleus. Data from the human amygdala provide evidence why almost all auras are, in fact, unpleasant and not pleasant. A review of recent data on the risks to offspring of epileptic fathers confirms that the etiology of Dostoevsky's epilepsy was probably inherited and that he probably had an idiopathic generalized epilepsy with minor involvement of the temporal lobe. A relationship is seen between his severe obsession with gambling and his epilepsy. Finally, Fyodor Dostoevsky is an excellent example of the "temporal lobe personality."     

ILAE Genetic Literacy Series: Postmortem Genetic Testing in Sudden Unexpected Death in Epilepsy

A 24-year-old man with non-lesional bitemporal lobe epilepsy since age 16 years was found dead in bed around midday. He was last seen the previous night when he was witnessed to have a tonic–clonic seizure. Before his death, he was experiencing weekly focal impaired awareness seizures and up to two focal-to-bilateral tonic–clonic seizures each year. He had trialed several antiseizure medications and was on levetiracetam 1500 mg/day, lamotrigine 400 mg/day, and clobazam 10 mg/day at the time of death. Other than epilepsy, his medical history was unremarkable. Of note, he had an older brother with a history of febrile seizures and a paternal first cousin with epilepsy. No cause of death was identified following a comprehensive postmortem investigation. The coroner classified the death as “sudden unexpected death in epilepsy” (SUDEP), and it would qualify as “definite SUDEP” using the current definitions.¹ This left the family with many questions unanswered; in particular, they wish to know what caused the death and whether it could happen to other family members. Could postmortem genetic testing identify a cause of death, provide closure to the family, and facilitate cascade genetic testing of first-degree family members who may be at risk of sudden death? While grieving family members struggle with uncertainty about the cause of death, we as clinicians also face similar uncertainties about genetic contributions to SUDEP, especially when the literature is sparse, and the utility of genetic testing is still being worked out. We aim to shed some light on this topic, highlighting areas where data is emerging but also areas where uncertainty remains, keeping our case in mind as we examine this clinically important area.     

Espace et temps des corps chez Samuel Beckett

What are we subject of? This major question works the work of Beckett in such a stubborn way that the reader, witnessing a vital fight of which he does not perceive the challenge, is embarrassed. If the desire to rise the subject is the cause, how to localize it? Mindful not to deflect Beckett from his own personal approach, we will examine him through his reading of Proust. Whereas Proust purports to save love, Beckett saves the body. From this perspective, we will read Watt, Molloy and The Unnamable, focusing closely on the bodies and the way the objects, which will direct them in the space that they will define, are chosen. Becket does not deduce that these objects are assumed by a subject, or even that these objects imply a subject; he supposes a body which assumes a topological subject. Thus, by tracking the objects provided by the body, Beckett gets the subject's saying. In doing so, one wonders if the subject of the science with Proust is not replaced by the body of the science with Beckett. Thus, Proust did not affect Beckett; his analysis has determined his ethical stance as shown. Yet, in writing, Beckett keeps on a subject still possible.     

An open hypothesis: is epilepsy learned, and can it be unlearned?

Plasticity is central to the ability of a neural system to learn and also to its ability to develop spontaneous seizures. What is the connection between the two? Learning itself is known to be a destabilizing process at the algorithmic level. We have investigated necessary constraints on a spontaneously active Hebbian learning system and find that the ability to learn appears to confer an intrinsic vulnerability to epileptogenesis on that system. We hypothesize that epilepsy arises as an abnormal learned response of such a system to certain repeated provocations. This response is a network-level effect. If epilepsy really is a learned response, then it should be possible to reverse it, that is, to unlearn epilepsy. Unlearning epilepsy may then provide a new approach to its treatment.     

Issues related to the pharmacological management of patients with brain tumours and epilepsy

The patient affected by epilepsy related to brain tumours presents certain features linked to the summation of his cancer-related problems and his epilepsy-related problems. Furthermore, epilepsy in brain tumour patients is often refractory to pharmacological treatments and can complicate the therapeutic management of these patients due to the increased incidence of pharmacological interactions and adverse effects. Analysis of the data in the literature suggests that it is opportune, when planning antiepileptic therapy in these cases, to choose the new-generation drugs, as these show a lower incidence of pharmacological interactions with the therapies used in brain tumour patients (chemotherapies, radiotherapy and support therapies), have fewer adverse effects, and have less impact on neuropsychological functions, all factors that strongly influence the patient's quality of life. Of the new antiepileptic drugs, the following seem to be promising in the treatment of cancer-related epilepsy: oxcarbazepine, topiramate and levetiracetam (the latter as an add-on therapy). The pharmacokinetic features of these drugs, their effectiveness in controlling seizures, and the reduced incidence of adverse effects make them useful in this particular group of patients.     

Could Valerian Have Been the First Anticonvulsant?

PURPOSE: To assess the available evidence for the belief that valerian, highly recommended in the past for treating epilepsy, possessed real anticonvulsant effectiveness. METHODS: Review of available literature. RESULTS: In 1592, Fabio Colonna, in his botanical classic Phytobasanos, reported that taking powdered valerian root cured his own epilepsy. Subsequent reports of valerian's anticonvulsant effectiveness appeared. By the late 18th and early 19th centuries, it was often regarded as the best available treatment for the disorder. Valerian preparations yield isovaleric acid, a substance analogous to valproic acid and likely to possess anticonvulsant properties, as isovaleramide does. In favorable circumstances, high valerian doses can be calculated to have sometimes provided potentially effective amounts of anticonvulsant substance for epilepsy patients. CONCLUSIONS: Valerian probably did possess the potential for an anticonvulsant effect, but the uncertain chemical composition and content of valerian preparations, and their odor and taste, made it unlikely that they could ever prove satisfactory in widespread use.     

Putnam,Merritt, and the Discovery of Dilantin®

In the first part of this essay, the “common wisdom” about Putnam and Merritt's contributions to the treatment of epilepsy was summarized (Rowland, 1982). Based on the history that has been presented here, how true are these “wisdoms”? Putnam and Merritt did devise “a simple and reliable method to test drugs of anticonvulsant effect” and they did show “that anticonvulsant effects in cats accurately predicted effects in humans,” but others before them had done these same things. Dilantin, contrary to common wisdom, was not the first anticonvulsant drug to be tested in animals before it was given to human subjects; at least a year before, Cobb and his co-workers had done the same thing using vital dyes. However, Dilantin did represent the first time an anticonvulsant tested in animals was subsequently studied in a large series of patients. Nor were Putnam and Merritt the first to show that “anticonvulsant and sedative effects of drugs could be separated.” Potassium borotartrate, ketogenic diet, ketone bodies, and vital dyes were anticonvulsive without necessarily being sedative. However, Putnam and Merritt were probably the first to make so explicit a statement to this effect. It may well have been this particular statement—and the fact that it was so well heard by other researchers—that represented their greatest achievement. In Kuhn's theory of scientific revolutions, the great step forward may not be so much the accumulation of evidence that the existing paradigm is not a feasible one, but rather the use of this evidence to form a new model or paradigm which is then accepted by normal science in such a fashion that the results prove to be productive. This, it would seem, is what Putnam and Merritt did. From it came their own major discovery, Dilantin, which, in Rowland's words, remains “a mainstay of treatment” for epilepsy up to the present time, and which “opened the way to the development of other anticonvulsant drugs.”     

Photoparoxysmal EEG response and genetic dissection of juvenile myoclonic epilepsy

Heritable EEG traits are often associated with epilepsy, and photoparoxysmal EEG response (PPR) is the most notable example of this observation in JME. Such EEG traits may be a subclinical expression of the defective mechanism that leads to epilepsy. Therefore, these traits can be used to map epilepsy genes by dissecting the complex epilepsy phenotype in endophenotypic sections that on their own have a presumed monogenic cause. Two characteristics make PPR particularly interesting as a useful endophenotype for epilepsy gene mapping. First, it shows an increased comorbidity with some but not all forms of epilepsy. Second, its mode of inheritance is compatible with a monogenic cause, which promises relative straightforward gene identification through positional cloning. Here, we summarize the current state of affairs.This article is part of a supplemental special issue entitled Juvenile Myoclonic Epilepsy: What is it Really?     

Dietary treatments: the road from Phoenix to Edinburgh

The ketogenic diet is now an established treatment in children for drug resistant epilepsy. Although in use for almost 100 years, it has taken time for us to determine an evidence base to verify its use in these children. However, increasing research both from a clinical and basic science perspective as well as a wide clinical interest has now enabled its use throughout the world. This aside, there remains lack of resources to guarantee its availability to all those who may be suitable. It is also now quite clear that it may have benefit in wider areas of neurology beyond epilepsy, specifically in metabolic disorders where glucose cannot be used as a primary energy source.