Margiad Evans (1909-1958): A writer's epileptic experiences and their reflections in her work.

Margiad Evans, in the period 1930-1950, an acclaimed English writer, developed convulsive seizures at age 41 and died at age 50 from the tumor that had caused them. In her book "A Ray of Darkness", she describes in profound analytic detail her seizure experiences, especially the isolated auras that had preceded her first convulsion by many years. Their ultimate strangeness echoed a long-standing fascination by the indescribable, which is a recurrent theme in her literary work. Another aspect of her poetry, a focus on the experience of the moment that cannot be retained is likewise reminiscent of the volatility of her aura experiences. Of three texts that are presently being published posthumously, one ("The Nightingale Silenced") is a fragmentary continuation of her epileptic experience. She considered that she still had a lot to describe, contributing the inside of the "outside inside story" of epilepsy, clues on which neurology could work to obtain a deeper understanding. To have a focal motor seizure feels like being invaded by an alien force. An urge to run and heautoscopy are other recorded symptoms. Evans documents the experience of a long-lasting, predominantly nonconvulsive status epilepticus merging into aura continua where her earlier aura experiences appear transformed into a quasipsychotic state. But even in the account of "this appalling, this hellish condition", she is careful to maintain her high literary standards. Together, the two reports on her disease seem to represent the only comprehensive inside case history of epilepsy, a most valuable legacy.

The earliest experimental convulsions by Joseph Priestley in 1766 friendship with Benjamin Franklin.

In 1766, Joseph Priestley (1733-1804) was the first to systematically demonstrate the universal convulsive effect of an electrical discharge applied to the head of all the several species studied. We here republish his overlooked experiments, which often resulted in death, and which ante date the scientific studies of the electrical functions of the brain, the role of "discharges" in seizures, and experimental epilepsy by about a century. Priestley's studies of electricity were influenced by those of Benjamin Franklin (1706-1790), who became a good friend during Franklin's prolonged period in London between 1757 and 1775. Both were elected Fellows of the Royal Society and both were awarded the Copley Medal of that Society. Priestley's experiments are relevant to the history of epilepsy and neuropsychiatry, and to the modern study of sudden unexplained death in epilepsy (SUDEP).

Francisco de Goya and the Seizing Impenitent.

The painting, St. Francis and the Dying Impenitent (1788) by the Spanish Baroque painter, Francisco Goya, is discussed by the author within the context of epilepsy and biographical events in the lives of both the saint and the painter.

A brief history of typical absence seizures - Petit mal revisited.

In this article, we have traced back the history of typical absence seizures, from their initial clinical description to the more recent nosological position. The first description of absence seizures was made by Poupart in 1705 and Tissot in 1770. In 1824, Calmeil introduced the term "absences", and in 1838, Esquirol for the first time used the term petit mal. Reynolds instead used the term "epilepsia mitior" (milder epilepsy) and provided a comprehensive description of absence seizures (1861). In 1854, Delasiauve ranked absences as the seizure type with lower severity and introduced the concept of idiopathic epilepsy. Otto Binswanger (1899) discussed the role of cortex in the pathophysiology of "abortive seizures", whereas William Gowers (1901) emphasized the importance of a detailed clinical history to identify nonmotor seizures or very mild motor phenomena which otherwise may go unnoticed or considered not epileptic. At the beginning of the 20th Century, the term pyknolepsy was introduced, but initially was not universally considered as a type of epilepsy; it was definitely recognized as an epileptic entity only in 1945, based on electroencephalogram (EEG) recordings. Hans Berger, the inventor of the EEG, made also the first EEG recording of an atypical absence (his results were published only in 1933), whereas the characteristic EEG pattern was reported by neurophysiologists of the Harvard Medical School in 1935. The discovery of EEG made it also possible to differentiate absence seizures from so called "psychomotor" seizures occurring in temporal lobe epilepsy. Penfield and Jasper (1938) considered absences as expression of "centrencephalic epilepsy". Typical absences seizures are now classified by the International League Against Epilepsy among generalized nonmotor (absence) seizures.

'Infantile convulsions' in the early nineteenth century. Abnormal brain blood flow and leeches, teething and gums' scarification and food and purgatives: the historical contribution of John Clarke (1760-1815).

In this article, we discuss on the role of the British physician and midwifery practitioner John Clarke (1760-1815) in the characterisation of the various types of seizures and epilepsy and related phenomena ('convulsions') occurring in children. In his unfinished work Commentaries on Some of the Most Important Diseases of Children (1815), Clarke discussed the pathophysiology of convulsions and was the first to describe, 12 years before the French neurologist Louis Francois Bravais (1801-1843) and more than 30 years before the Irish-born physician Robert Bentley Todd (1809-1860), the postictal paresis. He believed that convulsions originated from changes in pressure within the ventricles as a consequence of abnormal blood flow to the cerebral vessels. In keeping with the theories of his time (e.g. Baumes 1789, 1805; Brachet 1824), Clarke believed that teething was a major cause of 'infantile convulsions'. His proposed remedies ranged from scarification of the gums to ammonia, application of leeches, cold water, and purgatives. The use of antispasmodics, quite popular at the time, was instead questioned. In his Practical Observations on the Convulsions of Infants (1826), the London practitioner and midwifery John North (1790-1873) deeply criticised Clarke's view that convulsions arise inevitably as a consequence of organic brain lesions. North inferred that the results of autopsies of children who had died of convulsions revealed no brain damages, and claimed that cerebral irritation could also occur as the effect of distant lesions. Other Clarke's contemporaries (e.g. Jean Baptiste Timothée Baumes-1756-1828) inferred that all convulsions reflected a hereditary diathesis, which rendered children (especially those with softer and limper nervous and muscular tissues!) extremely sensitive to all sorts of provocation that could trigger convulsions, including bad digestion (more pronounced at the time of teething), loud noise, and bright light. Although almost every aspect of Clarke's view on convulsions was subsequently proved wrong, his (and his contemporaries') work provides fascinating insights into the theories and therapies of seizures, which were popular at the dawn of modern neurology.

Epilepsy on the silver screen in the 21st century.

Epilepsy remains an attractive vehicle for filmmakers in the twenty-first century. This review examines the themes of twenty-one films, released between 2000 and 2014, that feature a character with epilepsy or a pivotal scene involving a seizure. Epilepsy continues to be associated with the supernatural in modern cinematic output. Demonic possession and epilepsy now share a similar cinematic lexicon. Unfortunately, the overlap is more than just visual. Supernatural treatments of narratives that claim to be 'based on a true story' of someone with epilepsy continue to blur the lines between medical and spiritual realms. Although there has been a steady progression away from concealment of the condition, epilepsy continues to signal 'otherness' in movie characters and seldom in a good way. All too often, a character has epilepsy to maximize the unease of the audience with them; it is a device that is used to signal 'this character is not like you'. However, amongst the hackneyed negative stereotypes, accurate portrayals of the condition are beginning to emerge, not least due to active collaborations between filmmakers and epilepsy advocacy groups. Far from being worthy, it is heartening that these films are also those that are the most absorbing and thought-provoking of the cinematic output thus far this century. This article is part of a Special Issue entitled "Epilepsy, Art, and Creativity".

Epilepsy and metaphors in literature.

This topic has two different aspects: seizures and epilepsy used as metaphors and seizures described in metaphors. Whereas some metaphors are unique and have high literary value, others can be categorized in prototypical groups. These include sexual metaphors; metaphors of strong emotions, of life crises and breakdown, and also of exultation; religious metaphors; and metaphors of weakness which mostly belong to older literature. Writers with epilepsy, in their literary texts, rarely talk about seizures in metaphors. Authors who do this sometimes seem to use reports that they have received from afflicted persons. The most common metaphors for seizures belong to the realms of dreams and of strong sensory impressions (visual, auditory). More rarely, storm and whirlwind are used as literary metaphors for seizures. This article is part of a Special Issue entitled "Epilepsy, Art, and Creativity".

Representations of epilepsy on the stage: From the Greeks to the 20th century.

Epilepsy is a disorder that has been used by dramatists in various ways over the ages and therefore highlights the views of the disorder as people saw it at the time the plays were written and performed. In the 6th century BC, links between tragedy and epilepsy were developed by Greek playwrights, especially Euripides, in Iphigenia among the Taureans and Heracles where epilepsy and madness associated with extreme violence occur together. Both Heracles and Orestes have episodes after a long period of physical exhaustion and nutritional deprivation. During the Renaissance, Shakespeare wrote plays featuring different neurological disorders, including epilepsy. Epilepsy plays a crucial part in the stories of Julius Caesar and Othello. Julius Caesar is a play about politics, and Caesar's epilepsy is used to illustrate his weakness and vulnerability which stigmatizes him and leads to his assassination. Othello is a play about jealousy, and Othello, an outsider, is stigmatized by his color, his weakness, and his 'seizures' as a form of demonic possession. In modern times, Night Mother portrays the hard life of Jessie, who lives with her mother. Jessie has no friends, her father has abandoned the family, and she has no privacy and is ashamed. Stigma and social pressures lead her to commit suicide. Henry James' novella, The Turn of the Screw, portrays a governess with dream-like states, déjà vu, and loss of temporal awareness who has been sent to the country to look after two small children and ends up killing one. This novella was turned into an opera by Benjamin Britten. Most recently, performance art has been portraying epilepsy as the reality of a personally provoked seizure. Both Allan Sutherland and Rita Marcalo have purposely provoked themselves to have a seizure in front of an audience. They do this to show that seizures are just one disability. Whether this provokes stigma in audiences is unknown. Whether the performance artists understand the potential for status epilepticus has not been discussed. This article is part of a Special Issue entitled "Epilepsy, Art, and Creativity".

[Electroconvulsive therapy and level of evidence: From causality to dose-effect relationship]. / Électroconvulsivothérapie et niveau de preuve : de la causalité à la relation dose-effet.

OBJECTIVES: The first objective of this article is to summarize the history of electroconvulsive therapy (ECT) in psychiatry in order to highlight the transition from clinical level of evidence based on phenomenological descriptions to controlled trial establishing causal relationship. The second objective is to apply the criteria of causation for ECT, to focus on the dose-effect relationship criteria, and thus to analyze the conditions of application of these criteria for ECT. METHODS: A literature review exploring the use of electricity, ECT and electroencephalography (EEG) in psychiatry was conducted. The publications were identified from the Pubmed and GoogleScholar electronic databases. The scientific literature search of international articles was performed in July 2016. RESULTS: In 1784, a Royal commission established in France by King Louis XVI tested Mesmer's claims concerning animal magnetism. By doing that, the commission, including such prominent scientists as the chemist Anton Lavoisier and the scientist and researcher on electricity and therapeutics Benjamin Franklin, played a central role in establishing the criteria needed to assess the level of evidence of electrical therapeutics in psychiatry. Surprisingly, it is possible to identify the classical Bradford Hill criteria of causation in the report of the commission, except the dose-effect relationship criteria. Since then, it has been conducted blinded randomized controlled trials that confirmed the effectiveness of ECT against ECT placebos for the treatment of psychiatric disorders. At present, the dose-effect relationship criteria can be analyzed through an EEG quality assessment of ECT-induced seizures. CONCLUSIONS: EEG quality assessment includes several indices: TSLOW (time to onset of seizure activity ≤5Hz, seconds), peak mid-ictal amplitude (mm), regularity (intensity or morphology of the seizure (0-6)), stereotypy (global seizure patterning, 0-3) and post-ictal suppression (0-3). A manual rating sheet is needed to score theses indices. Such manual rating with example of EEG segments recording is proposed in this article. Additional studies are needed to validate this manual, to better establish the dose-response relationship for the ECT, and thus strengthen the position of the EEG as a central element for clinical good practice for ECT.

William Gowers' interpretation of epileptogenic mechanisms: 1880-1906.

In the 1870s, early in his neurologic career, William Gowers (1845-1911) was exposed to three main schools of thought concerning epileptogenesis, namely, that seizures resulted from (1) excessive neural activity in the medulla oblongata or pons (Marshall Hall, John Russell Reynolds, and Brown-Séquard), (2) excessive local cerebral cortical activity (Hughlings Jackson), or (3) suddenly decreased cerebral activity that released the intrinsic contractibility of skeletal muscle (Radcliffe). By 1881, Gowers had reasoned his way to the idea that epileptogenesis was best accounted for by local cortical overactivity. This overactivity might at times be initiated by local loss of inhibition. The overactivity in turn then might inhibit other parts of the central nervous system to explain loss of consciousness in seizures and postseizure temporary hemiparesis. The possibilities of the idea of inhibition, at first often called "resistance," continued to interest Gowers over the following 25 years. He settled on the synaptic gap as its likely site. However, the inhibition mechanism that he proposed, namely, retraction of dendrites, was rather extraordinary in the light of subsequent knowledge.

Louis François Bravais and Jacksonian epilepsy.

Although it is known that Jacksonian epilepsy was first described by Bravais in 1827, some 40 years before Jackson began his work on the topic, little has been published on what Bravais wrote. Louis François Bravais (1801-1843) came from a French provincial family, which made a number of scientific, mainly botanical, contributions. In his Paris M.D. thesis, based on 25 instances of what he termed "hemiplegic epilepsy," Bravais described a set of unilateral epileptic seizure phenomena, including postseizure hemiparesis, very similar to those Jackson wrote about in 1870. However, Jackson accepted that the initially unilateral convulsive phenomena could spread to involve both sides of the body, whereas Bravais believed that this was incompatible with his entity, unless the generalization was a very rare event in the sufferer. Bravais in his account refused to go beyond descriptions of phenomena, whereas Jackson reasoned from the phenomena to a new concept of epileptogenesis, and also from them deduced that there must be localized representation of function in the cerebral cortex. Although Bravais had the priority, his work went largely unnoticed until Jackson's insights had made their impact on medical thinking.

Historical perspectives and definitions of the postictal state.

The postictal state and its features were recognized by physicians from Babylonian times through to the advent of modern neurology in the late 19th century. Among varied descriptions and definitions lies one of the best known and still used eponyms in medicine, Todd's paralysis. Despite a relative lack of biological insight, many key observations were made in an era mostly devoid of treatments for epilepsy.

Fyodor Dostoevsky and his falling sickness: a critical analysis of seizure semiology.

Fyodor Dostoevsky is a great Russian writer who had epilepsy. As a consequence, there are many references to seizure-related phenomena in his work. His epilepsy syndrome has been a focus of debate. The goal of this article is to delineate his epilepsy syndrome based on a semiological description of seizures, which could be considered one of the most reliable pieces of circumstantial evidence available. It was hypothesized that seizure-related descriptions in his books were based on his own personal experience. The semiology of seizures and related phenomena was compiled from Dostoevsky's own work, his letters to family and friends, and reminiscences of his wife and friend. Those descriptions were analyzed in detail to elicit localizing and lateralizing features of seizures. On the basis of this evidence, it was postulated that Dostoevsky had a partial epilepsy syndrome most probably arising from the dominant temporal lobe.

[Ergotismus convulsivus. the convulsive type of ergotism illustrated by an example in the year 1738]. / Ergotismus convulsivus. Die krampfende Form der Mutterkornvergiftung an einem Beispiel aus dem Jahr 1738.

This paper describes cases of ergotismus convulsivus (the kind of poisoning from ergotized grain marked by convulsions), some of them fatal, in the year 1738. The origins of the formation of the ergotized grain as well as the symptoms of ergotism are presented. Comparisons to other epidemics of ergotism are drawn.

Osler vindicated: glioma of the leg center with Jacksonian epilepsy; removal and cure, with a 50-year follow-up. Historical vignette.

On December 14, 1883, William Osler, then pathologist at the Montreal General Hospital, presented the specimen of a brain with an almond-sized glioma beneath the right motor cortex to the Montreal Medico-Chirurgical Society. The brain specimen was from a young woman who had suffered from intermittent Jacksonian seizures for 14 years and had eventually died in status epilepticus. Aware of the pioneering removal of a tumor from the cortex reported on in 1885 by Bennett and Godlee, Osler wrote of his case, "this was an instance in which operation would have been justifiable and possibly have been the means of saving life." In 1953, a young man with Jacksonian attacks that began in his foot underwent removal of a Grade I glioma from the central fissure. The operation was performed in an awake craniotomy during which cortical mapping was used to define the motor and sensory cortices. Treatment with focal radiation followed, and afterward the patient became seizure-free, stopped taking anticonvulsant medication, and has led an active life over the past 50 years. Reference is made to the experiences of Sherrington, Cushing, and Penfield with cortical stimulation in the awake patient under regional anesthesia as an effective aid to surgery for epileptogenic lesions, tumors, and vascular malformations. Their technique allows for maximal resection with minimal neurological deficits. Over the past 20 years, this approach has been adopted effectively in many neurosurgical centers.

St. Valentine--patron saint of epilepsy: illustrating the semiology of seizures over the course of six centuries.

From the 15th century to the present day, Christian art has portrayed people who suffer from epilepsy as attributes in illustrations of Saint Valentine (SV). The objective of our study was to interpret the works of art from a modern epileptological perspective on the basis of a collection of portrayals of SV in Europe that was as comprehensive as possible. The people depicted as attributes were analyzed with respect to their age, gender, social status, and possible seizure semiology. Three hundred forty-one illustrations of SV from Austria, Belgium, Croatia, Czech Republic, France, Germany, Hungary, Ireland, Italy, Luxembourg, Poland, Scotland, Slovakia, and Switzerland were systematically analyzed. Irrespective of the age of the work of art, among the 143 pictures of people with possible epilepsy characteristics, there were more males than females from various levels of society. As far as could be interpreted, there were 17 infants, 35 children, 7 adolescents, and 84 adults. With respect to possible seizure semiology, infantile spasms (n=10), atonic seizures (n=13), tonic seizures (n=53), absences (n=2), psychogenic seizures (n=4), and postictal or undefinable states (n=61) were differentiated in a subjective assessment. Despite the fact that from a modern perspective, the 15th to 20th centuries in Europe seemed to be dominated by a rather superstitious attitude toward epilepsy, there is striking accuracy in the detail of the semiology in many of the historic portrayals, and a well-founded knowledge of epilepsy is apparent.