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The Interictal Behavior Syndrome of Temporal Lobe Epilepsy
Abstract
• A distinct syndrome of interictal behavior changes occurs in many patients with temporal lobe epilepsy. These changes include alterations in sexual behavior, religiosity, and a tendency toward extensive, and in some cases compulsive, writing and drawing. The concomitants of abnormal limbic activity therefore include behavior alterations as well as manifest seizures. The demonstration of interictal spike activity in temporal structures provides a pathophysiologic basis for this syndrome. The constellation of behavioral changes may be of great diagnostic value. In addition, it provides an example of a human behavioral syndrome associated with dysfunction at specific anatomic loci. The behavior syndrome of temporal lobe epilepsy may prove to be a useful model in studies on the neural substrates for behavior.
... Initially the PBDs of PWE were not attributed to underlying epileptic pathology but rather to factors such as antiepileptic drugs and psychosocial comorbidity. However, some clinicians suggested intrinsic epileptogenic biological factors contributing to a more complex correlation between PBDs and epilepsy (8,9). During the early 21 st century, the concept of the "bidirectional association" between epilepsy and psychiatric comorbidity arose, based on observations of higher incidence of PBDs in PWE. ...
... However, some personality traits might emerge as part of more complex PBDs in PWE as a result of ictal activity. For instance, people with TLE can exhibit a specific interictal behavioral syndrome the "Gastaut-Geschwind syndrome" (9), which consists of alterations in sexual behavior, irritability, increased religiosity, hypergraphia and circumstantiality. This syndrome has been attributed to limbic system dysfunction in individuals with TLE. ...
Introduction
People with epilepsy (PWE) have been hypothesized to have higher prevalence of personality disorders and cognitive disorders. The objective of this study was to investigate the controversial notion of “epileptic personality,” a series of supposedly specific personality traits of people with epilepsy (PWE).
Methods
For this purpose, 29 individuals with Mesial Temporal lobe Epilepsy (MTLE) and 23 with Juvenile myoclonic epilepsy (JME) as confirmed by electroencephalography (EEG), MRI scans and clinical examination, underwent a thorough neuropsychological and personality assessment. The resulting neuropsychological profiles were statistically analyzed considering possible personality disorders, character traits, cognitive and linguistic deviations from 20 healthy controls (HC).
Results
Our findings suggest accumulative cognitive and linguistic deficits in individuals with epilepsy compared to controls. It is possible that these might be misinterpreted as personality disorders. Specifically, personality traits (p = 0.049) and verbal fluency (p = 0.013), were significantly different between PWEs and controls. Also, the type of epilepsy and lateralization seem to affect executive function (p = 0.049) and pragmatology scores (p < 0.001), exhibiting differences in subgroup analysis.
Discussion
Different theories are considered as plausible pathophysiological explanations for the aforementioned differences. This research might serve as a basis to further investigate the cognitive aspects of epilepsy and possible pharmacological interventions, which are currently lacking.
... These observations reinforced the role of temporal lobes in mood, cognition, and behavior. In the 1970s, Norman Geschwind's studies validated the early findings linking deepened emotions to temporal lobes, also including hypergraphic, philosophical interests, and religiosity traits in the spectrum of TLE-related interictal behaviors 18 . In the 1990s, Blumer et al. 16 proposed objective measures of GGS by adapting the Bear-Fedio Inventory (BFI) 19 into the Neurobehavior Inventory (NBI) 16 . ...
The interface between epilepsy and religiosity has been a long-standing matter of debate. Epilepsy has affected several religious leaders throughout history. Hyperreligiosity may be observed in patients with temporal lobe epilepsy as a component of the so-called Gastaut-Geschwind syndrome which involves other behavioral and personality traits such as hyposexuality, viscosity, philosophical concerns, sense of personal destiny, hypergraphy, emotionality, and irritability. Saint Paul, the Apostle, probably had temporal lobe epilepsy. He was a genius man of intellectual excellence and refined culture, whose life and writings exerted a decisive influence on Western history. The current paper investigates the elements of Gastaut-Geschwind syndrome in Saint Paul’s life and Epistles and discusses the potential influence of these traits on Pauline theology.
Keywords: Epilepsy; Personality; Behavior
... With respect to religion-related seizure activity, the TPM cycling account of RSE phenomena may throw new light on clinical observations that arguably launched the field of religion and brain studies back in the 1970s (Dewhurst and Beard, 1970;Waxman and Geschwind, 1975;Geschwind, 1979;Bear and Fedio, 1977). These reports described a small group of patients with a particular form of temporal lobe epilepsy (TLE) who seemed to ascribe extraordinary meaning and significance to their interictal experiences. ...
We review and synthesize recent religion and brain studies and find that at a broad network neuroscience level, religious/spiritual experiences (RSEs) appear to depend crucially upon interactions between the default mode network (DMN), the frontoparietal network (FPN), and the salience network (SN). We see this general result as broadly consistent with Menon’s et al. “Triple Network or Tripartite Model” (TPM) of neuropsychiatric function/dysfunction. A TPM cycling model is here offered to account for details of neural bases of an array of RSE phenomena including ecstatic seizures, neuroimaging of religious participants, psychedelically induced mystical states and perceptions of supernatural agents. To adequately account for SA perceptions, however, recent evidence suggests that REM sleep and dreaming mechanisms likely play a role. Future research should examine neurodevelopmental mechanisms of acquired SA perceptions as well as societal-level effects such as brain mediated religious beliefs of in-group cohesion and out-group hostility.
... Chronic TLE causes mild interictal personality changes with a slowly but progressive cumulative effect. 10 This links to early evidence 11,12 pointing to the relationship involving TLE, specific interictal personality traits, and psychopathological symptoms known as Geschwind syndrome. 13 Waxman and Geschwind 11 focused on specific interictal behavioral phenomena, including hypergraphia, hyperreligiosity, and hyposexuality, circumstantiality, cyclothymia, intensified mental life, and odd social behavior. ...
Background The role of temporal lobe epilepsy (TLE) in determining personality traits and neurobehavioral symptoms, collectively known as the interictal behavioral syndrome (also known as Geschwind syndrome or “Gastaut-Geschwind syndrome”), as well as the syndrome's association with the particular artistic expression of many epileptic litterateurs are well known in neurology and psychiatry. A deepening of emotionality along with a serious, highly ethical, and spiritual behavior have been described as positive personality changes among patients with chronic mesial-TLE.
Objectives Our narrative-based clinical hypothesis aims at contributing to the ongoing debate on the association between TLE and artistic expression, as well as the latter's supposed implication for epileptology in general and the neuropsychology of epilepsy in particular.
Methods Through an analysis of the biography, language, and literary work of Greek novelist Demosthenes Voutyras, we hypothesize that his mystical and dark writing style could be attributed to medial temporal interictal dynamics.
Conclusions We suggest that the psycholiterary profile of Voutyras is consistent with the idiosyncratic characteristics of the temporal lobe personality, while a non-dominant temporal lobe contribution has been proposed.
... This field is fraught but interesting. Bear and Fedio, 2 influenced by Waxman and Geschwind, 3 argued that a long history of subepileptic discharges 'kindled' a seizure focus and led to a fairly stereotyped set of personality traits. As far as I know, kindling has not been demonstrated convincingly in humans. ...
Morning report is an important clinical learning activity in many neurological institutions. A long experience of these meetings allows identification of several components to enhance its success. Meetings are best if brief (one or two cases) and held regularly, preferably daily and early in the working day, with full in-person team engagement. A senior clinician should lead the meeting and commit to a single interpretation, without fear of being wrong. Although the environment is relaxed (refreshments typically provided), it is a working meeting and with the essential focus on the patient rather than the learners. The rich learning experience is greatly enhanced by a subsequent confidential email summary and interpretation of the case(s) sent to all participants.
... The right posterior temporal lobe and the temporal/parietal area are the most common sites for out-of-body experiences. As a result, it appears that NDEs share characteristics with other scenarios and can be triggered by pharmaceutical drugs, epileptic discharges, or direct brain stimulation (Blanke and Thut 2007;Waxman 1975). Spiritual models stand in opposition to biological answers. ...
There has been a continuous discussion of religious experience since William James,
culminating in a rich and varied literature on the epistemology of religious experience in the late
twentieth century. There has also been a burgeoning literature on near-death experiences (NDEs),
largely on neurology and physiology and sometimes as possible counterevidence to naturalism. One
important subject is largely missing, and that is a discussion of NDEs as religious experiences, and
what light that might shed on their epistemic status. This paper is an attempt to fill that gap. In part
one, we will delineate the topic of NDEs and what medical science has to say about them. In part
two, we will lay out a general view of the epistemology of religious experience. In part three, we will
apply that understanding of religious experience to NDEs and draw what lessons we may
... G eschwind Syndrome (GS) is a controversial clinical diagnosis defined as a cluster of inter-ictal behavioral manifestations as hypergraphia, hyperreligiosity, hyposexuality, mental rigidity, verbal and non-verbal viscosity (1). Behavioral manifestations of this syndrome are traditionally thought to be stemmed from temporal lobe epileptic seizures (TLE) via hyper-reactivity in the limbic networks (2). ...
... The controversial relationship between epilepsy and personality characteristics has been recognized for centuries, with important studies emphasizing this association [1][2][3][4]. In contrast, the findings and evidence of some studies for the existence of an epileptic personality are inconsistent [5][6][7][8]. ...
Purpose: The purpose of this study was to investigate personality characteristics and clinical parameters in two well-defined epilepsies: mesial temporal lobe epilepsy related to hippocampal sclerosis (MTLE/HS) and juvenile myoclonic epilepsy (JME) through NEO Revised Personality Inventory (NEO-PI-R) and Neurobehavior Inventory (NBI) standardized instruments.
Methods: One hundred patients undergoing corticoamygdalohippocampectomy (CAH), 100 patients with JME, and 100 control subjects answered the personalitymeasures. Clinical parameters such as psychiatric symptoms, seizure frequency, duration of epilepsy, and side of the lesion in MTLE/HS group were investigated. Statistical analysis consisted of the mean and standard deviation (SD) of each variable. Student's t-test or Fisher exact test were used according to the variable studied.
Results: The three groups were within the average range of NEO-PI-R and NBI, although ‘tendencies’ and differences were demonstrated. The MTLE/HS and control subjects had a similar profile: low scores in Neuroticism and high in Conscientiousness (r = −0.330; p b 0.001/r = −0.567; p b 0.001, respectively) in opposition to what occurred in JME, low in Conscientiousness and high in Neuroticism (r = −0.509; p = 0.005). The NBI sense of personal destiny’ trait was higher (3.15; p =0.003) in MTLE/HS than in JME and controls. The JME 'law and order' scores were lower than in other groups (p=0.024). A tendency towards specific NBI traits differentiates MTLE/HS (Factor 3) from JME (Factor 1) groups. Psychiatric symptoms and seizure frequency were correlated with worse scores in NBI and, especially, in Neuroticism domain of NEO-PI-R.
Conclusion: Specific personality features were linked to each epileptic disease. These findings highlight the importance of considering unique features linked to epilepsy conditions in daily clinical observation to develop support programmes.
... The kindling is extensively used as an animal experimental model in attempts to interpret the phenomenon of interictal emotionality and effects of epileptiform activity on emotional behavior in humans. These aspects frequently fit the main goal of the respective studies [7] [8], but the reverse statement is incorrect. It is interesting that interictal emotional disorders related to temporal epilepsy per se can be significantly modified under the action of anticonvulsants. ...
Оцінювання психічного здоров'я людини є, мабуть найважливішою та найскладнішою складовою визначення здоров'я індивіда та популяції. Проаналізовано концептуальну можливість кількісного оцінювання психічного здоров'я та визначення критерію психічного здоров'я індивіда та популяції. Висловлено думку, що на даний період розвитку медицини методика інтегрального оцінювання психічного здоров'я відсутня. Відповідно необхідна додаткова робота для вивчення факторів, пов'язаних із психічним здоров'ям і процесами прийняття рішень стосовно політики оцінювання та укріплення психічного здоров'я. Головне - створення інтегрального кількісного критерія оцінювання психічного здоров'я людини. Існує потреба у достовірних і надійних показниках стану психічного здоров'я індивіда та популяції, а також в обґрунтуванні показників для відстеження валідності та справедливості даних. Рівень електронних досліджень в області психічного здоров'я постійно зростає. Це пов'язане з надзвичайно швидким зростанням впровадження інформаційних технологій, що включають Інтернет речей, робототехніку, штучний інтелект, який застосовується для надання психіатричних послуг.
Epilepsy has a fascinating history. To the medical historian Oswei Temkin it was 'the paradigm of the suffering of both body and soul in disease'. It is justifiably considered a window on brain function. And yet its story is more than simply a medical narrative, but one influenced also by scientific, societal and personal themes. Written for a medical and non-medical readership, this book describes the major developments in epilepsy between 1860–2020, a turbulent era in which science dominated as an explanatory model, medical theories and practices steered an erratic course, and societal attitudes and approaches to epilepsy fluctuated dramatically. In the middle of this maelstrom was the person with epilepsy at the mercy of social attitudes and legislation, and at times harmed as well as helped by medicine and science. So entangled is the history that intriguingly, as an entity, epilepsy may now be thought not even to exist.
Stereotypic neural networks are repeatedly activated in drug-refractory epilepsies (DRE), reinforcing the expression of certain psycho-affective traits. Geschwind syndrome (GS) can serve as a model for such phenomena among patients with temporal lobe DRE. We describe stereo-electroencephalogram (SEEG) exploration in a 34-year-old male with DRE and GS, and his treatment by SEEG-radiofrequency (SEEG-RF) ablation. We hypothesized that this approach could reveal the underlying epileptic network and map eloquent faculties adjacent to SEEG-RF targets, which can be further used to disintegrate the epileptic network. The patient underwent a multi-modal pre-surgical evaluation consisting of video EEG (VEEG), EEG source localization, 18-fluorodexyglucose-PET/MRI, neuropsychological and psychiatric assessments. Pre-surgical multi-modal analyses suggested a T4-centered seizure onset zone. SEEG further localized the SOZ within the right amygdalo-hippocampal region and temporal neocortex, with the right parieto-temporal region as the propagation zone. SEEG-RF ablation under awake conditions and continuous EEG monitoring confirmed the abolishment of epileptic activity. Follow-up at 20 months showed seizure suppression (Engel 1A/ILEA 1) and a significantly improved and stable psycho-affective state. To the best of our knowledge this is the first description of the intracranial biomarkers of GS and its further treatment through SEEG-RF ablation within the scope of DRE.
Postmaterial spiritual psychology posits that consciousness can contribute to the unfolding of material events and that the human brain can detect broad, non-material communications. In this regard, this emerging field of postmaterial psychology marks a stark departure from psychology's traditional quantum measurements and tenets. The Oxford Handbook of Psychology and Spirituality codifies the leading empirical evidence in the support and application of postmaterial psychological science. Sections in this volume include: personality and social psychology factors and implications; spiritual development and culture; spiritual dialogue, prayer, and intention in Western mental health; Eastern traditions and psychology; physical health and spirituality; positive psychology; and scientific advances and applications related to spiritual psychology. With articles from leading scholars in psychology, medicine, physics, and biology, this text is an interdisciplinary reference for a rapidly emerging approach to contemporary science.
Objective:
This study aims to assess the prevalence of Interictal Dysphoric Disorder (IDD) in drug-resistant epilepsy (DRE) and to describe its clinical and psychopathological profile, including personality, as well as its impact on quality of life (QOL).
Method:
A retrospective cross-sectional study from an Epilepsy Unit from January 2007 to December 2017. All patients were diagnosed with DRE. Patients underwent a battery of tests (HADS, SCL-90R, PDQ-4+, QOLIE-31) and a psychiatrist assessed the presence of Axis-I disorders and IDD. Statistical procedures were carried out using R-4.0.1 software.
Results:
A total of 282 patients were included. A statistically significant association was found between IDD and mood and anxiety disorders (p < 0.001 and p < 0.05 respectively), and between IDD and higher scores in all HADS and SCL-90-R items compared to subjects without IDD (p < 0.001). A statistically significant association was also found between IDD and obsessive-compulsive, borderline and depressive personality disorder (p < 0.05). Scores in all QOLIE-31 items except for 'medication effects' were significantly lower in subjects with IDD compared with subjects without IDD (p < 0.001).
Conclusions:
In DRE, IDD subjects show differences in the psychopathological profile and QOL scores compared to subjects without a diagnosis of IDD. An early diagnosis of IDD could facilitate prompt interventions which might positively impact QOL.
For a long time, people suffering from epilepsy have been feared, suspected, and misunderstood, socially stigmatized and viewed as outcasts. On the other hand, epilepsy was attributed to many remarkable personalities, among whom are Julius Caesar, Alexander the Great, Socrates, Aristotle, Joan of Arc, Leonardo da Vinci, Michelangelo, Ivan the Terrible, Newton, Peter the Great, Napoleon, Walter Scott, Paganini, Byron, Van Gogh, Dostoevsky, Leo Tolstoy, and many others. The article is aimed to confirm on historical facts or debunk the myths about the essence of epilepsy in some of these individuals.
Objective
In spite of anecdotal reports describing an association between chronic epilepsy and interictal aggressiveness, and of a few studies suggesting that such an association is common in temporal lobe epilepsy, this concept has not been generally accepted by epileptologists. In the course of studies of the long‐term consequences of limbic status epilepticus (SE) in juvenile rats, we noticed that experimental animals, unlike littermate controls, could not be housed together because of severe fighting. We now report a study of interictal aggression in those rats.
Methods
Long‐term behavioral consequences of lithium/pilocarpine SE were studied 3 months after SE had been induced with lithium and pilocarpine in male Wistar rats at age 28 days. Chronic spontaneous seizures developed in 100% of animals. We tested rats for territorial aggression under the resident‐intruder paradigm. We measured the number of episodes of dominance (mounting and pinning), and agonistic behavior (attacks, boxing, and biting).
Results
Untreated lithium/pilocarpine SE induced a large increase in aggressive behavior, which involved all aspects of aggression in the resident‐intruder paradigm when tested 3 months after SE. The experimental rats were dominant toward the controls, as residents or as intruders, and showed episodes of biting and boxing rarely displayed by controls. They also displayed increased aggressiveness compared with controls when tested against each other.
Significance
This robust model offers an opportunity to better understand the complex relationship between seizures, epilepsy, and aggression, and the role of age, SE vs. recurrent spontaneous seizures, and focal neuronal injury in the long‐term behavioral effects of SE.
Background
While language is frequently explored in stroke and focal lesions, complex language disorders in paroxysmal conditions remain relatively underexplored. Individuals with temporal lobe epilepsy (TLE) often have an impairment of conversational language manifesting as verbosity and attributable to disruption of cognitive-linguistic networks. The micro- and macrolinguistic underpinnings of this disturbance, and the role of epilepsy and cognitive variables, are yet to be explored.
Methods and Procedures
We examined the elicited language output of 16 individuals with TLE and 14 healthy controls under separate monologic discourse tasks: a structured and constrained context, elicited by description of the ‘Cookie Theft’ picture, and an unstructured, unconstrained context, elicited by description of a ‘Typical Day’. We hypothesised that language output in the unstructured context would be characterised by verbosity to a greater extent than language elicited in a structured context.
Outcomes and Results
Following transcription and coding, detailed multi-level discourse analysis suggested that a constrained context gives rise to microlinguistic disturbances in individuals with TLE, reducing fluency, with more pauses and fillers. Under an unconstrained context, as anticipated, classical aspects of verbosity emerge in those with TLE, manifesting as longer speaking time, a longer duration of pauses, and a higher proportion of repetitive or redundant statements. Macrolinguistic elements such as coherence and informativeness are widely impacted, particularly disturbing language formulation. Correlations suggest that microlinguistic disturbances are closely linked with the immediate impact of seizures on cognitive-linguistic function, while macrolinguistic disturbances are more broadly impacted by disorder severity and word retrieval deficits.
Conclusions
These findings suggest that cognitive-linguistic disturbances in TLE produce vulnerability to different psycholinguistic impairments as a function of differing linguistic challenges imposed by constrained and unconstrained discourse contexts. Where constrained, output fluency is primarily affected, while in an unconstrained context disturbed discourse planning and organisation declares itself. We conclude that these patterns reflect a dynamic linguistic system taking shape under specific contextual conditions.
Epilepsy has a fascinating history. To the medical historian Oswei Temkin it was 'the paradigm of the suffering of both body and soul in disease'. It is justifiably considered a window on brain function. And yet its story is more than simply a medical narrative, but one influenced also by scientific, societal and personal themes. Written for a medical and non-medical readership, this book describes the major developments in epilepsy between 1860–2020, a turbulent era in which science dominated as an explanatory model, medical theories and practices steered an erratic course, and societal attitudes and approaches to epilepsy fluctuated dramatically. In the middle of this maelstrom was the person with epilepsy at the mercy of social attitudes and legislation, and at times harmed as well as helped by medicine and science. So entangled is the history that intriguingly, as an entity, epilepsy may now be thought not even to exist.
This article aims to provide a historical overview of the relationship between epilepsy and religiosity. Although the link between epilepsy and religiosity has been observed since ancient times, empirical research has not supported the direct relationship between epilepsy and religiosity in any unequivocal way. The rich reference to the historical relationship between epilepsy and religiosity often served as a kind of evidence in itself, even though observations of epileptic religiosity were far less detailed as modern scholars referred to them. A detailed analysis of the connection between epilepsy and religiosity only emerged in the 20th century. By introducing a symbolic level of interpretation, psychoanalysis significantly contributed to the psychological theory of the epileptic personality in general and epileptic religiosity in particular. The older concept of the epileptic personality (and religiosity) mixed with psychoanalytic interpretation was preserved in the Szondi Test. Based on the present historical analysis, the author concludes that the connection between epilepsy and increased religiosity was overstated in the 20th century.
The Cognitive Neuroscience of Religious Experience, now updated and expanded in a new edition, updates key topics covered in the first edition including: decentering and self-transformation, supernatural agent cognitions, mystical states, religious language, ritualization, and religious group agency. It expands upon the first edition to include major findings on brain and religious experience over the past decade, focusing on methodology, future thinking, and psychedelics. It provides an up-to-date review of brain-based accounts of religious experiences, and systematically examines the rationale for utilizing neuroscience approaches to religion. While it is primarily intended for religious studies scholars, people interested in comparative religion, philosophy of religion, cultural evolution, and personal self-transformation will find an account of how such transformation is accomplished within religious contexts.
Globular Glial Tauopathy (GGT) is a rare form of Frontotemporal Lobar Degeneration (FTLD) consisting of 4-repeat tau globular inclusions in astrocytes and oligodendrocytes. We present the pathological findings of GGT in a previously published case of a 73-year-old woman with behavioral symptoms concerning for right temporal variant frontotemporal dementia with initial and salient features of Geschwind syndrome. Clinically, she lacked motor abnormalities otherwise common in previously published GGT cases. Brain MRI showed focal right anterior temporal atrophy (indistinguishable from five FTLD-TDP cases) and subtle ipsilateral white matter signal abnormalities. Brain autopsy showed GGT type III and Alzheimer's neuropathologic changes. .
Obsessive-compulsive disorder (OCD) has long been associated with epilepsy. The link with temporal lobe (usually refractory) epilepsy (TLE) is particularly prominent. Of TLE patients, 10% to 22% of patients may have OCD, often underdiagnosed in the outpatient clinic. Data on the links include case reports, case series, and controlled studies. Three larger, controlled studies in TLE patients, using comprehensive epilepsy and OCD classifications, in aggregate, have noted the obsessive qualities of washing, symmetry/exactness, and ordering, with a greater preoccupation with certain aspects of religion, compared with controls or patients with idiopathic generalized epilepsy. TLE foci may be either left- or right-sided. Social and neurobiological factors are involved in OCD in TLE. The neurobiology implicates a pathophysiological or structural impairment of the orbitofrontal-thalamic, and frontothalamic-pallidal-striatal-anterior cingulate-frontal circuits. Discrete anatomic lesions in these pathways, or their surgical removal, may induce (or conversely) improve OCD in TLE patients.
St Teresa of Avila (1515–82), known as an ecstatic mystic nun, experienced a rich palette of feelings and internal sensations, that were precisely documented in her writings. Authors with diverse fields of expertise, have attributed these manifestations to religious feelings, neurological and psychiatric symptoms, even suggesting she may have been an ‘illustrious patroness of hysterics’. We review here major themes from the writings of St Teresa in the light of present neurological and psychiatric knowledge, wondering whether her ecstasies may correspond to epileptic seizures initiated in limbic system and how her religious personality may be needed to explain the range of feelings and sensations she described.
Ecstasies in epilepsy
Ecstasy is a rare but intriguing symptom in epilepsy. Patients with limbic or temporal lobe epilepsies may describe intense positive emotions, absolute well-being and hyper-perception of themselves in the environment during a seizure.¹ These feelings may be associated with other phenomena including thymic lability and the Geschwind syndrome of hyperreligiosity, hypergraphy, hyposexuality or ethical and moral concerns.² Ecstatic seizures with religious character, while rare, are historically important since they strengthen links between epilepsy and the divine. They seem likely to involve the right temporal region and the anterior insular cortex.¹ Insular regions support a ‘sentient self’, providing a capacity to experience feelings and sensations.³ Ecstatic seizures are now thought to arise from the anterior insula, according to studies with depth intracranial recordings.⁴ The insula appears to generate feelings of beauty, certainty and ecstasy when a seizure is initiated, possibly due to influences on more distant and connected brain regions.
Besides dementia or mild cognitive disorder, some other organic mental disorders, due to brain damage and brain dysfunction or to physical diseases, may affect any person, especially persons with intellectual disability (ID) or autism spectrum disorder (ASD), whose rate of physical health problems is more than double that of the general population. Moreover, in some etiologically defined forms of ID or ASD, mainly in genetic syndromes, certain psychiatric manifestations are very prominent or characteristic and strictly associated with physical diseases. Clinical manifestations of organic mental disorders are often very similar to those of disorders not regarded as organic and a thorough physical assessment is fundamental to distinguish. The knowledge in this area is likely to increase over the next decades.This chapter also addresses issues related to epilepsy in persons with ID and/or ASD, both in general and with respect to the connection with psychiatric disorders, which involves more than overlapping symptoms and mistaken diagnoses.In persons with ID and/or ASD, the prevalence of epilepsy is higher than in general population, and the clinical management is more complex. Pharmacokinetic or pharmacodynamic characteristics of antiepileptic drugs (AEDs) are important factors in determining their action on behavioral disturbances in persons with ID and/or ASD. Rehabilitation of these persons should have a multidisciplinary approach requiring the involvement of different operators, family members, and caregivers specially trained in the management of these complex clinical conditions.KeywordsOrganic mental disorderOrganic hallucinosisOrganic catatonic disorderOrganic delusional [schizophrenia-like] disorderOrganic mood [affective] disorderOrganic anxiety disorderOrganic dissociative disorderOrganic personality disordersPostencephalitic syndromePost-concussion syndromeGenetic syndromesEpilepsySeizuresIntellectual disabilityPsychopathologyAutism spectrum disorderPharmacological treatmentPsychosocial treatment
Religious experiences in epilepsy patients have provoked much interest with suggestions that hyperreligiosity is associated with temporal lobe seizures. Extreme varieties of religious behavior may be more frequent in epilepsy patients during ictal activity or during post-ictal psychotic episodes. We report a 75 year-old man with epilepsy who developed a progressive decline in cognition and behavior following a religious conversion 15 years earlier. He subsequently developed religious delusions of increasing severity and symptoms of Capgras syndrome. Brain imaging revealed bilateral posterior cortical atrophy, chronic right parieto-occipital encephalomalacia, and right mesial temporal sclerosis. Electroencephalograms and neuropsychological testing revealed initial right temporal lobe abnormalities followed by progressive frontal and bilateral dysfunction. The case highlights how a history of seizures, superimposed on sensory deprivation and a progressive impairment of right posterior and bilateral anterior brain function, contributed to religious conversion, which was followed by dementia and delusions with religious content.
There is a lot of evidence that a great Russian writer and essayist Fyodor Mikhailovich Dostoevsky suffered from epilep‑ sy. Not only this disease had influence on Dostoevsky’s personality and social life, but also affected his creative work. Dostoevsky is believed to suffer from mesiobasal temporal lobe epilepsy with an ecstatic aura – a form named after him afterwards.
Any attempt to compare the definitions of symptoms listed for “primary psychoses” with those adopted in studies of psychoses in patients with epilepsy (PWE) will encounter problems of heterogeneity within both conditions. In this manuscript, five psychotic illnesses listed in Diagnostic and Statistical Manual of Mental Disorders-5th Edition (DSM-5), that is, brief psychotic illness, schizophreniform disorder, schizophrenia, delusional disorder, and schizoaffective disorder are compared with postictal (or periictal) and interictal psychotic disorders in PWE. After examining definitions of primary psychoses, definitions of psychoses adopted in the papers dealing with postictal and interictal psychoses are summarized. Further, diagnostic criteria of five types of psychotic disorders in PWE proposed in 2007 by Krishnamoorthy et al. are also discussed, which include postictal psychosis, comorbid schizophrenia, iatrogenic psychosis caused by antiepileptic drugs (AEDs) (AED-induced psychotic disorder: AIPD), and forced normalization. Evidently, a comparison between postictal psychosis and schizophrenia is pointless. Likewise, schizophrenia may not be an appropriate counterpart of forced normalization and AIPD, given their acute or subacute course.
This review provides responses to four questions on epilepsy, religion, and spirituality. Firstly, have early religious beliefs and writings stigmatized and discriminated against epilepsy and if so, what has been done to correct this? We provide textual evidence suggesting an affirmative response. Secondly, which religious luminaries, gods, saints, and religious symbols have connections with epilepsy? We argue that the evidence to suggest that St Paul, Joan of Arc, the Prophet Mohammed, and others had epilepsy is weak and emphasizes the limitations of imposing contemporary neurological frameworks upon them. Furthermore, we discuss how different faith traditions identify Divine figures, as associated with epilepsy, and the use of religious symbols in healing. Thirdly, why is religiosity associated with having epilepsy? We review empirical studies focusing upon the epileptic personality, religiosity, mysticism, and religious conversion and find that, while some studies suggest that religious experience may be associated with epilepsy, this cannot be taken as proven. Fourthly, in what ways has religion been a force for good for those with epilepsy? We discuss the role of Christian social reform in caring for individuals with epilepsy and that of religion in coping with the condition. We conclude by arguing that the relationship between religion and epilepsy has been overstated in the academic literature.
In the 19th and early 20th century, epilepsy was one of the most investigated disorders in forensic psychiatry and psychology. The possible subsidiary symptoms of epilepsy (such as temporal confusion, alterations of consciousness, or increased aggression) played pivotal roles in early forensic and criminal psychological theories that aimed to underscore the problematic medical, social and legal status of epileptic criminals. These criminals were considered extremely violent and capable of committing sudden, brutal acts. Although the theory of “epileptic criminality” was refuted due to 20th‐century developments in medicine, forensic psychiatry, and criminal psychology, some suppositions related to the concept of epileptic personality have lingered. This paper explores the lasting influence of the theory of epileptic personality by examining the evolution of the theories of epileptic criminality both in the international and the Hungarian context. Specifically, it calls attention to the twentieth‐century revival of the theory of epileptic personality in the works of Leopold Szondi, István Benedek and Norman Geschwind. The paper shows that the issue of epileptic personality still lingers in neuropsychology. In doing so, biological reductionist trends in medical‐psychological thinking are traced, and attention is drawn to questions that arise due to changing cultural and medical representations.
Background
Over 80% of the global population consider themselves religious with even more identifying as spiritual, but the neural substrates of spirituality and religiosity remain unresolved.
Methods
In two independent brain lesion datasets (N1=88; N2=105), we apply lesion network mapping to test whether lesion locations associated with spiritual and religious belief map to a specific human brain circuit.
Results
We found that brain lesions associated with self-reported spirituality map to a brain circuit centered on the periaqueductal grey. Intersection of lesion locations with this same circuit aligned with self-reported religiosity in an independent dataset, as well as prior reports of lesions associated with hyper-religiosity. Lesion locations causing delusions and alien limb syndrome also intersected this circuit.
Conclusions
These findings suggest that spirituality and religiosity map to a common brain circuit centered on the periaqueductal grey, a brainstem region previously implicated in fear conditioning, pain modulation, and altruistic behavior.
Temporal lobe epilepsy may lead to misdiagnosis due to affective, psychotic and/or personality abnormalities. Here, Dr Côrte‐Real et al. investigate such a case of an epileptic patient, initially diagnosed with bipolar disorder whose diagnosis was later changed to focal epilepsy and major neurocognitive disorder highlighting the importance of differential diagnosis in atypical cases.
Background
In patients with temporal lobe epilepsy (TLE), studies demonstrate frequent comorbidity with mood disorders, personality disorders (especially obsessive-compulsive disorder) and major depression, but there are conflicting findings. This study aimed to investigate psychiatric comorbidities and affective temperament among TLE patients and to explore the relationships between obsessive compulsive disorder, other personality disorders, major depression and affective temperament in order to clarify the mediator effect of TLE in these relationships.
Methods
Thirty patients with TLE and 30 healthy volunteers were included. The Structured Clinical Interview for DSM-IV Axis I Disorders (SCID-I), the Structured Clinical Interview for DSM III-R Axis II Disorders (SCID-II), Hamilton Anxiety (HAM-A) scale, Hamilton Depression (HAM-D) scale, Beck Suicidal Ideation Scale (BSSI) and Yale Brown Obsession Compulsion Scale (YBOCS) were applied and evaluated by a psychiatrist. Additionally, all individuals completed The Temperament Evaluation of Memphis, Pisa, Paris and San Diego (TEMPS-A).
Results
Patients with temporal lobe epilepsy had higher scores in TEMPS-A, HAM-A, HAM-D, YBOCS and BSSI. Major depression, obsessive compulsive disorder and dependent and antisocial personality disorders were prevalent in patients. With respect to affective temperaments, depressive, cyclothymic and anxious temperaments were associated with obsessive compulsive disorder comorbidity; whereas, depressive and anxious temperaments were found to be associated with major depression comorbidity in patients with TLE. Furthermore, cluster A and cluster C personality disorders were associated with affective temperaments in patients with TLE. Affective temperaments had no correlation with illness duration, seizure frequency, depression severity and suicidal thoughts, but obsessions and compulsions. Suicidal thoughts were associated with obsessions and compulsions.
Conclusion
Affective temperaments are core personality traits with biological background and they may provide a foundation for psychiatric disorders, especially mood disorders. Considering that TLE originates from abnormalities in brain circuitry, it may form a basis for psychiatric disorders. Therefore, psychiatric evaluation to determine comorbidities may be beneficial to increase the quality of life of patients with TLE.
Reports on social cognition in patients with developmental amnesia resulting from bilateral hippocampal lesions are rare, although the link between social cognition and temporal lobe structures is well established. We present the case of a 23-year-old male epilepsy patient, BM, with developmental amnesia due to perinatal cerebral hypoxia. The patient was examined with neuroimaging and neuropsychological methods and compared to IQ-matched patients with epilepsy to control for effects of epilepsy. In addition, we used a test battery that evaluates emotion recognition and theory of mind to study his social cognition abilities. Structural high-resolution magnetic resonance imaging showed bilateral hippocampal atrophy. The comparison to controls showed that, in addition to the well-documented memory disorders in developmental amnesia, BM showed remarkable deficits in 9 out of 17 social cognitive tasks assessing emotion recognition and theory of mind. In contrast, BM’s performance on tasks of executive functions was largely preserved. The relevance of deficits in social cognition for patients with developmental amnesia is discussed.
Introduction
The incidence of mood disorders and psychopathology is more frequent in patients with epilepsy (PWE) than in the general population. Also, it has been reported that PWE suffer more seizures during certain phases of their menstrual cycle (MC). Still, limited information exists regarding the relationship between the physical and emotional changes during the MC in PWE. Therefore, in this study, we aimed to evaluate the mood and personality traits of PWE during their MC and to compare them with controls.
Methods
A cohort of 22 PWE and nine controls was gathered. All the participants underwent psychiatric, electroencephalographic, and gynecological evaluations.
Results
Overall, PWE scored higher in depression compared with controls (p < 0.05), PWE also obtained higher scores for the personality traits of neuroticism and self-isolation (p < 0.05). During the evaluation of the symptoms of premenstrual syndrome (PMS), PWE were more symptomatic during the early follicular (EF) phase in comparison with the rest of the phases of their MC, whereas the control group showed the known premenstrual pattern of symptoms during the late luteal (LL) phase. The frequency of seizures (40.6%) and electroencephalographic abnormalities (34.8%) was also higher during the EF phase of the MC when compared with the other phases of the MC (p < 0.05).
Conclusions
The results of this study suggest that unlike the PMS present in women without epilepsy, PWE appear to show a “menstrual syndrome” that consists of similar mood changes and physical symptoms. This arrangement of symptoms seems to have an impact on the increase in seizure activity.
Behavioral and personality disorders in temporal lobe epilepsy (TLE) have been a topic of interest and controversy for decades, with less attention paid to alterations in normal personality structure and traits. In this investigation, core personality traits (the Big 5) and their neurobiological correlates in TLE were explored using the Neuroticism Extraversion Openness-Five Factor Inventory (NEO-FFI) and structural magnetic resonance imaging (MRI) through the Epilepsy Connectome Project (ECP). NEO-FFI scores from 67 individuals with TLE (34.6 ± 9.5 years; 67% women) were compared to 31 healthy controls (32.8 ± 8.9 years; 41% women) to assess differences in the Big 5 traits (agreeableness, openness, conscientiousness, neuroticism, and extraversion). Individuals with TLE showed significantly higher neuroticism, with no significant differences on the other traits. Neural correlates of neuroticism were then determined in participants with TLE including cortical and subcortical volumes. Distributed reductions in cortical gray matter volumes were associated with increased neuroticism. Subcortically, hippocampal and amygdala volumes were negatively associated with neuroticism. These results offer insight into alterations in the Big 5 personality traits in TLE and their brain-related correlates.
Limbic encephalitis is an inflammatory process of the limbic structures, with polymorphic clinical features, caused by paraneoplastic and nonparaneoplastic conditions and infections. We describe a case of neurosyphilis limbic encephalitis, presenting with reversible Geschwind syndrome (hyperreligiosity and hypergraphia) and mood disorder due to the predominant involvement of left mesial temporal structures in a previously healthy 34-year-old, left-handed woman. Because neurosyphilis can mimic common neuropsychiatric syndromes, it should be included in the differential diagnosis of psychiatric disorders with suspected general medical causes. This case of nondominant limbic encephalopathy caused by syphilis infection highlights the relevance of a careful investigation for secondary psychotic, mood, and personality disorders when assessing new-onset psychiatric illness and the importance of a multidisciplinary approach to provide a better outcome in patients with neurosyphilis.
Abstract
The current article is a critical review of the neuropsychological study of religious experience. First, we analyse the philosophical and theological roots of the term and its characterization as a sui generis and unique phenomenon. We subsequently describe the adoption of the concept in psychology, as well as the emergence of alternative approaches that emphasize the role of categorization of experiences as religious. We then move on to explore how both approaches have influenced the neuropsychological study of religious experience, consequently producing different models: sui generis, attributional and others that consider the variety of factors and the heterogeneity of experiences associated with religious practices.
Resumen
El presente artículo es una revisión crítica del estudio neuropsicológico de la experiencia religiosa. Comenzaremos analizando las raíces filosóficas y teológicas del concepto, y su caracterización en tanto fenómeno sui generis y único. Proseguiremos describiendo el traslado de dicha concepción a la psicología, así como el surgimiento de una concepción crítica al modelo sui generis, a través de enfoques alternativos que enfatizan en el rol de la categorización de las experiencias en tanto religiosas. Veremos cómo ambas concepciones son trasladadas al estudio neuropsicológico de la experiencia religiosa, produciendo distintos modelos sui generis, atribucionistas, así como otros que contemplan la variedad de factores y heterogeneidad de experiencias relacionadas a las prácticas religiosas.
This essay provides a medical theory that could clarify enigmas surrounding the historical Socrates. It offers textual evidence that Socrates had temporal lobe epilepsy and that its two types of seizure manifested as recurrent voices and peculiar behaviour, both of which were notorious hallmarks of Socrates. Common and immediate criticisms against the methodology of retrospective diagnosis are addressed first. Next, the diagnostic reasoning is presented in detail. The possibility of temporal lobe personality in Socrates is also considered. The important implication of this theory is that one of the charges against Socrates, introducing new divinities, was a now well-known neurologic symptom.
Objectives:
The findings of previous studies focused on personality disorders in epileptic patients are difficult to interpret due to nonhomogeneous samples and noncomparable methods. Here, we aimed at studying the personality profile in patients with mild temporal lobe epilepsy (mTLE) with psychiatric comorbidity.
Materials and methods:
Thirty-five patients with mTLE (22 males, mean age 40.7 ± 12.1) underwent awake and sleep EEG, 3T brain MRI, and an extensive standardized diagnostic neuropsychiatric battery: Temperament and Character Inventory-Revised (TCI-R), Beck Depression Inventory-2, and State-Trait Anxiety Inventory. Drug history was collected in detail. Hierarchical Cluster Analysis was performed on TCI-R data, while all other clinical and psychological variables were compared across the resulting clusters.
Results:
Scores of Harm Avoidance (HA), Reward Dependence (RD), Persistence (P), Cooperativeness (C), and Self-Transcendence (ST) allowed the identification of two clusters, describing different personality subtypes. Cluster 1 was characterized by an early onset, more severe anxiety traits, and combined drug therapy (antiepileptic drug and Benzodiazepine/Selective Serotonin Reuptake Inhibitors) compared to Cluster 2.
Conclusions:
Our findings suggest that different personality traits may play a role in determining the clinical outcome in patients with mTLE. Specifically, lower scores of HA, RD, P, C, and ST were associated with worse clinical outcome. Thus, personality assessment could serve as an early indicator of greater disease severity, improving the management of mTLE.
Fyodor Mikhailovich Dostoevsky is one of the best Russian novelists. It is also known that he had been suffering from epilepsy―one can find many descriptions of this particular condition in Dostoevsky’s novels. These writings are most probably based on his personal experience. There are numerous neurological hypotheses about the type of epilepsy with which Dostoevsky suffered, the most notorious feature of his type of epilepsy being the so-called “ecstatic aura.” In fact, the type of epilepsy Dostoevsky experienced is often termed “Dostoevsky’s epilepsy with ecstatic aura.” In thecurrent article, I offer a review of the literature on Dostoevsky’s epilepsy. Subsequently, the notorious feature “ecstatic aura” is compared with mystical experience, and a conclusion is reached: the two states are in fact identical in the sense that mystical experience can occur during ecstatic aura. A neuroscientific explanation of the experience is presented as well. Finally, a philosophical analysis is performed.
Experimental evidence supports the concept of the hypothalamus as the primary neural substrate for neuroendocrine regulation of hypophyseal hormone secretion. However, research over the last few decades has established the importance of the limbic system in the mediation of endocrine expression. Functioning as an integrative mechanism for interoceptive, exteroceptive and emotional information, the limbic system communicates with lower brain stem structures and the higher neopallial systems through extensive afferent and efferent connections. Therefore, in consideration of the enormous variety of autonomic and behavioral responses elicited by external factors or experimental manipulation of the various components of the limbic system, and of the fundamental role of the endocrines in the maintenance of homeostasis, it is not surprising that activation of the endocrine system should follow disturbances to limbic structures. Research over the last ten years has focused on elucidating the nature of the interactions of various limbic structures with the hypothalamus.
A case is reported of right temporal lobectomy performed for local epilepsy and resulting in a severe and persisting amnesic state without general dementia. Pre-operative and post-operative electroencephalography, air encephalography, angiography, and clinical examination failed to provide evidence of a cerebral lesion on the unoperated side. Possible explanations of this observation are briefly discussed.RésuméPrésentation d'un cas de lobectomie temporale droite pratiquée pour épilepsie focale, et de l'état amnésique grave et persistant sans démence globale qui en résulta. Les examens pré—et post—opératoires: électroencéphalographie, encéphalographie gazeuse, artériographie et examens cliniques ne fournirent aucune preuve d'une atteinte cérébrale du côté non opéré.Les explications possibles de cette observation sont briévement discutées.ZusammenfassungVorstellung eines Falles einer rechten temporalen Lobectomie, für fokale Epilepsie, bei dem es einen amnesischen Zustand in Folge der Operation gab. Dieser Zustand war schwer und andauernd aber ohne totale Demens. Die Teste (EEG Angiographie, Pheumoencephalographie) die vor une nach der Operation gemacht worden sind beweisen dass die rechte nicht operierte Seite gesund ist.Die möglichen Erklärungen dieses Falles wurden kurz besprochen.
In 1953, in an article intended for the general practitioner, Denis Hill made a brief reference to the chronic paranoid psychoses which may develop in association with temporal lobe epilepsy. He described the condition as likely to come on when the seizures were diminishing in frequency, as appearing gradually with onset in middle age, and as resembling a paranoid schizophrenic state. In 1957, D. A. Pond, from the same department of applied electro-physiology at the Maudsley Hospital, gave a more detailed account of the clinical features. He described the psychotic states as closely resembling schizophrenia, with paranoid ideas which might become systematized, ideas of influence, auditory hallucinations often of a menacing quality; and occasional frank thought disorder with neologisms, condensed words and inconsequential sentences. There were, however, also some points of difference, of a quantitative rather than qualitative kind: a religious colouring of the paranoid ideas was common; the affect tended to remain warm and appropriate; and there was no typical deterioration to the hebephrenic state. All the patients had epilepsy arising from the temporal lobe region with complex auras; occasional major seizures occurred in sleep only. EEG foci, always present, were sometimes only to be demonstrated in sleep-sphenoidal records. The epilepsy began some years before the psychotic symptoms, usually in the late teens or the twenties; and the latter often seemed to begin as the epileptic attacks were diminishing in frequency, either spontaneously or with drug treatment.
In 1888, Sanger-Brown and Schafer1 described an apparent disturbance of memory in monkeys after the partial or total removal of both temporal lobes. Two years later, Bechterew2 in a very brief report, described a patient who had shown an extraordinary degree of memory disturbance, as well as considerable apathy for many years; in the region of the gyrus uncinatus and Ammon's horn in each temporal lobe, the cortex and underlying structures were said to be softened. The matter then lay dormant until 1954, when Scoville3 described a grave loss of recent memory in 2 patients after resection of the medial parts of both temporal lobes. The ablations extended posteriorly along the medial surfaces for a distance of about 8 cm. from the tips of the temporal lobes, and probably destroyed the anterior two-thirds of the hippocampus and hippocampal gyrus bilaterally, as well as the amygdala. Later, these
SERUM creatine phosphokinase (CPK) and aldolase activities are increased in many acutely psychotic patients of a variety of diagnostic types.1-3 CPK exists in three molecular forms (isoenzymes). Type I is found almost exclusively in the brain, type II in cardiac muscle, and type III in skeletal and cardiac muscle.4 Much smaller amounts are present in many tissues.5 Only the type III isoenzyme of CPK was found in the sera of psychotic patients.1,2 A number of possible causes of an increased activity in serum of type III CPK have been evaluated: orally or intramuscularly administered psychotropic medications2,6; physical activity2,7; severe emotional distress not part of a psychotic process2 weight loss2,8; hypersecretion of steroids2 or epinephrine8; and muscle tension.7 These have been considered to be not responsible for the increased serum enzyme activity in most acutely psychotic patients. Increased activity of serum
The ‘religiosity’ of the epileptic has been recognized since the time of Esquirol (1838) and Morel (1860). These, and later French workers (including Marchand and Ajuriaguerra, 1948), have sought to explain the epileptic's religiosity as being the result of his disability, social isolation and his enhanced need for the consolation of religion. A specific conversion experience after a fit was reported by Howden (1872–73). The patient believed that he was in Heaven. He would appear to have been depersonalized, as it took three days for his body to be reunited with his soul. ‘He maintained that God had sent it to him as a means of conversion, that he was now a new man, and had never before known what true peace was…. He assured me that he was a converted man and that he was convinced he would have no more fits.’ Howden also reported on John Engellerecht who, after many years of depression, attempted suicide and appeared to die. After visiting Hell and Heaven, he cast off his depression, and acquired a state of religious ecstasy accompanied by visual and auditory hallucinations. Boven (1919) mentions a 14-year-old boy who after a seizure ‘saw the good God and the angels, and heard a celestial fanfare of music’. He regards the religiosity of the epileptic character as being due to experiences acquired in the course of an epileptic aura and in the subsequent confusional state. Eventually the patient becomes preoccupied with a cure which he believes depends entirely on God, and this belief is the basis of his euphoria. Boven stresses the intensified piety of the epileptic after a severe seizure.
The phenomenon of hypergraphia, or the tendency toward extensive and, in some cases, compulsive writing, in temporal lobe epilepsy is described in seven patients, in each of whom there was electroencephalographic demonstration of a temporal lobe focus. Unusually detailed and strikingly copious writing was evidenced in each patient. Six patients provided documentation of their extensive writing, which often was concerned with religious or moral issues. A seventh patient claimed to have written extensively, but refused to exhibit his writings. Aggressiveness, religiosity, and changes in sexual behavior in temporal lobe disorders have been described previously. The hypergraphia of temporal lobe epilepsy appears to be part of a specific behavioral syndrome of special interest because of its association with dysfunction at specific anatomic loci.
A series of divers experiments in mammalian cortex and isolated neurons have yielded data suggesting a possible mechanism for the generation of the interictal epileptic spike, the simplest type of paroxysmal neuronal activity.We have outlined the hypothesis that penicillin, the most commonly used epileptogenic agent, brings about the following effects. Excitatory feedback actions in the cortex are enhanced by a decrease in the threshold for impulse initiation in neurons within these pathways and/or by an increase in the potency of excitatory synaptic actions. An increase in the gain of positive feedback systems in the cortex could lead to the production of the explosive and synchronous ‘epileptic spike’ with its cellular correlate, the ‘paroxysmal depolarizing shift’, which is believed to be an EPSP. The termination of the interictal spike has been suggested to depend, at least in part, on the delayed and enduring operation of recurrent inhibition.
This article has no abstract; the first 100 words appear below.
When Mr. Murray Falconer moved from New Zealand to London, his neurosurgical unit based jointly in two major London teaching hospitals and at the Maudsley Hospital, one of the leading psychiatric centers of the world, began a methodical study of surgery in the control of temporal-lobe epilepsy. Although the primary indication for surgery was uncontrolled seizures, the presence of behavioral changes in a large fraction of the 250 patients operated on has permitted the accumulation of considerable data on the clinical pictures of these personality changes and the effects of surgery. This study has had the advantage of independent assessment . . .
Norman Geschwind, M.D.
Boston City Hospital Boston, Mass. 02118
Aggression and psychosis were frequent preoperative complications in over 250 patients with temporal-lobe epilepsy who underwent unilateral temporal lobectomy. Pathological findings in the resected specimens, which included the hippocampus and the lateral amygdala, were mesial temporal sclerosis in about 1/2, hamartomas in 1/4, miscellaneous lesions such as scars and infarcts in 1/10, and nonspecific lesions in the remainder. Follow-up evaluations after six to 20 years were carried out independently by psychiatrists. In a series of 100 patients, 27 had aggression, which was relieved most often after operation in patients who had mesial temporal sclerosis (seven of 13). In the first 100 patients psychosis complicated long standing temporal-lobe epilepsy due to many substrates in 12 cases and was benefited by operation in seven. When a schizophrenia-like state supervenes, the substrate is likely to be a hamartoma. In patients with schizophrenia, operation relieved epilepsy, but the psychosis persis...
THERE is considerable evidence of neuromuscular dysfunction in most
patients with all types of the functional psychoses. Examples of such
dysfunction are (1) increased activity of skeletal-muscle-type creatine
phosphokinase (CPK) in serum in the acute stage of
psychosis1-4 (2) abnormally large increases in serum CPK
activity with a standardised exercise test performed by patients in
symptomatic remission5; (3) morphological abnormalities in
skeletal muscle fibres at biopsy, particularly scattered atrophic fibres
and extensive areas of Z-band streaming6-8 and (4)
abnormalities of eye movements in tracking a pendulum9. The
skeletal muscle fibre abnormalities found in psychotic patients are
likely to be neurogenic in origin10.
IN THE course of previous investigations designed to identify more specifically the nature of mental aberrations peculiar to patients with psychomotor epilepsy, we were not a little surprised to find that our psychological and psychiatric evaluations failed to differentiate psychomotor from nonpsychomotor seizure patients who were otherwise matched for age, sex, seizure frequency, general intelligence, and economic background.1
In a second study undertaken with different techniques, patients, and collaborators, only one measure significantly differentiated a group of psychomotor epilepsy patients from subjects with "centrencephalic" grand and petit mal seizures. This test, a continuous performance task, indicated that the "centrencephalic" group performed less well than did the psychomotor patients.2 Although our findings are consistent with those of the few other controlled studies that appear in the literature,3-5 our conclusions, namely that the psychomotor epilepsies are not associated with an
The genesis of independent secondary epileptic foci from artificially induced primary epileptic lesions has been demonstrated in animals which are representative of different stages of evolutionary development. These data suggest that the length of time necessary for the occurrence of an autonomously discharging secondary epileptic focus following the artificial creation of a primary epileptic lesion varies directly with the differentiation and organizational complexity of the forebrain of the animal selected as representative of the particular stage of evolutionary development.
The data also indicate that secondary epileptogenesis results from an alteration of normal physiological processes and that the propensity for epileptogenesis is inherent in vertebrate neural tissue. The data presented are discussed in relation to the question of secondary epileptogenesis in humans with focal epilepsy.
To the authors' knowledge this study is the first which has used the opossum and cayman as models for the study of epileptogenesis. The relative advantages are mentioned.
RÉSUMÉ
Ľexistence de foyers áepileptiques secondaires indáependants de láesions áepileptogàenes expáerimentales primaires a été prouváee chez des animaux àa diffáerents etages du developpement phylogáenáetique. Les donnáees recueillies suggàerent que le temps náecessaire pour ľapparition des dáecharges secondaires en relation avec le foyer áepileptogene primaire varie en fonction directe de la differentiation et de la complexitáe dans ľorganisation du táelencáephale des animaux en relation avec leur situation dans le phylum.
Cette áetude qui est la premiáere,à a la connaissance des auteurs, àa utiliser ľOpossum et la Caiman comme modàele pour áetudier ľáepileptogáenàese, est utilisáee pour discuter le problàeme de ľáepileptogáenàese secondaire chez des Hommes souffrant ďune áepilepsie focale.
Ces données suggèrent également que ľepileptogénàese secondaire résulte ďune altáeration des processus physiologiques normaux et qu'elle est inherente aux tissus nerveux des vertáebráes.
Brief bursts of nonpolarizing electrical brain stimulation were presented once each day at constant intensity. At first the stimulation had little effect on behavior and did not cause electrographic afterdischarge. With repetition the response to stimulation progressively changed to include localized seizure discharge, behavioral automatisms and, eventually, bilateral clonic convulsions. Thereafter, the animal responded to each daily burst of stimulation with a complete convulsion. The effect was obtained from bipolar stimulation of loci associated with the limbic system, but not from stimulation of many other regions of the brain. Parametric studies and control observations revealed that the effect was due to electrical activation and not to tissue damage, poison, edema, or gliosis. The changes in brain function were shown to be both permanent and trans-synaptic in nature. Massed-trial stimulation, with short inter-burst intervals, rarely led to convulsions. The number of stimulation trials necessary to elicit the first convulsion decreased as the interval between trials approached 24 hours. Further increase in the inter-trial interval had little effect on the number of trials to first convulsion. High-intensity stimulation studies revealed that the development of convulsions was not based simply on threshold reduction, but involved complex reorganization of function. Experiments with two electrodes in separate parts of the limbic system revealed that previously established convulsions could facilitate the establishment of a second convulsive focus, but that the establishment of this second convulsive focus partially suppressed the otherwise permanent convulsive properties of the original focus.
The most common sexual abnormality in temporal lobe epilepsy is a marked lack of libidinal and genital arousal. The author documents the occurrence of hypersexual behavior in 50 temporal lobe epileptics, 42 of whom had unilateral temporal lobectomy. Twenty-nine (58 percent) were clearly hyposexual prior to surgery; seven (14 percent) had distinct episodes of hypersexuality. In six patients the hypersexual episodes followed the abrupt cessation of temporal lobe seizure activity following either temporal lobectomy or seizures. These findings document the role of the temporal limbic structures in the regulation of sexual arousal.
THE MAMMALIAN hypothalamus interposes itself between the rest of the brain and the endocrine system, and interrelates to each to regulate a large part of the organism's instinctive behavior and endocrine function. A rich experimental literature describes what happens to animal behavior and homeostasis when various parts of the hypothalamus are selectively stimulated or destroyed, and inferences from these observations are commonly extrapolated to man since opportunities rarely present themselves to study precise hypothalamic lesions in humans. The anatomy of the small but complex hypothalamus changes little between lower animals and man, lending confidence that comparisons of direct hypothalamic-endocrine functions may be valid. But the cerebral hemispheres of man are far more complex than in animals, so that detailed studies of humans with hypothalamic disease become particularly valuable in establishing the relevance of the behavioral aspects of the experimental studies.
This is a report of a young woman who underwent
DISTURBANCES of color identification in association with the syndrome of pure alexia without agraphia have been repeatedly recognized in the past. Lange1 reviewed these extensively. More recently Critchley2 has reviewed the literature on such disturbances of color identification. He expressed skepticism as to the existence of the syndrome of aphasia for color names and advanced the view that such cases probably represented the combination of a perceptual deficit with a minimal aphasia.
We have recently had the opportunity to clarify the problem of color-name aphasia by repeated study of a patient with the syndrome of pure alexia without agraphia over several months. On the basis of these studies, we believe that the evidence is now clear that a disturbance of color-naming, or more correctly, of matching the spoken name of the color to the seen color, can be shown to exist in the demonstrated absence of any
Bilateral lesions produced by electrocoagulation in the basolateral amygdaloid nuclear complex were found to affect plasma and pituitary levels of luteinizing hormone (LH) in the male deermouse (Peromyscus maniculatus bairdii). Within 1 week after lesions were produced in the basolateral amygdaloid group, pituitary levels of LH increased significantly by 170% from the level in intact controls of 0·17–0·47 m-u./mg. During the same period, plasma levels rose by approximately 220% from 0·14–0·45 m-u./ml. The rise in LH continued and was at 0·55 m-u./mg. in the pituitary and 0·54 m-u./ml. in plasma 3 weeks after the lesions had been placed. The rise in plasma LH was reflected in the weight of the testes, prostates and seminal vesicles which significantly increased in all animals with lesions.
Introduction
In recent years a physicochemical basis for neuronal memory patterns has been hypothesized on the basis of intracellular reversible protoplasmic reactions.10 This thesis explains satisfactorily the perception of sensory phenomena in the primary cortical areas. However, there is increasing evidence that many perceptual analyses are formulated in cortical or subcortical neuronal circuits other than primary sensory centers.One of these cellular constellations important in certain memory processes would seem to be related to the temporal lobe. This has been demonstrated both in experimental animals and in man. Recent experimental studies in monkeys have revealed defects in visual discrimination due to damage to the inferior temporal cortex. This deficit is greater in visual learning than in retention of old visual habits, a finding which might be construed as representing an impairment in visual recent memory.1In man, the evidence that the temporal lobe plays an important role in
Acute epileptogenic foci have been produced in the cerebral cortex of cats (cerveau isolé or lightly anesthetized preparations) with topical application of penicillin, and the interictal manifestations have been analyzed by means of simultaneous intracellular and surface recording. Within any given neuronal population, individual elements display different degrees of involvement but the most common behavioral pattern of the “epileptic” neuron appears to be a paroxysmal depolarization shift (PDS), often followed by a long-lasting hyperpolarization. The main characteristic features and variations of this pattern are described. Both an excessive synaptic bombardment and (undetermined) changes in the intrinsic properties of the neuronal membrane are likely to contribute to the production of the PDS. This may prevent the soma from being invaded by the spikes but the latter can fire repetitively along the axon. The hyperpolarization shift is probably a true IPSP. Its presence could be one of the limiting factors for the maximal repetition frequency at which the interictal spontaneous discharges can occur.
Certain disorders in cerebral function have been described in patients with psychomotor‐temporal lobe epilepsy which predispose to interictal psychosis. These are predominantly a disturbance of thought processes with loss of trains of associations, word‐finding difficulties and fluctuations in the accuracy of perceptions. The psychotic states which may develop, usually after the seizure disorder had existed for several years, include inappropriate, bizarre behavior, delusions and paranoid and depressive reactions, hallucinatory activity, denial and excessive religiosity. The continued efforts to be in contact with reality and lack of autism, archaic thinking, withdrawal, or peculiar symbolism differentiate these reactions from a schizophrenic process. Electroencephalographic correlates with interictal psychotic episodes are only occasional and then usually consist of more intense focal temporal or bilateral paroxysmal discharge. At times, however, the electroencephalogram improves or becomes normal during a psychotic state. However, it is possible that these interictal personality and behavior disturbances may be due at least in part to subictal cerebral excitation.
RÉSUMÉ
Description de quelques troubles de la fonction cérébrate chez des malades atteints d'épilepsie psychomotrice‐temporale qui prédisposent à une psychose interictale. Ceci est principalement un trouble des procès de la pensée avec perte de l'enchaînement des associations, difficultés à trouver les mots et fluctuations dans l'exactitude des perceptions. Les états de psychose, se développant généralement quand l'épilepsie date de plusieurs années, comprennent un comportement inapproprié et bizarre, des illusions et des réactions paranoïaques et dépressives, une activité hallucinatoire, une religiosité excessive et du reniement. Des efforts continus pour être en contact avec la réalité et un manque d'autisme, d'une façon de penser achaïque, de la retraite ou d'un symbolisme singulier différencient ces réactions d'un procès schizophrénique. Des corrélations électroencéphalographiques avec des épisodes de psychose interictale ne sont qu'occasionnelles et consistent habituellement en une décharge focaletemporale ou paroxysmale bilatérale plus intense. Parfois, cependant, l'électroencé‐phalogramme s'améliore ou devient normal pendant un état de psychose. Il est toutefois possible que cette personnalité interictale et ces troubles du comportement soient dus, pour une part tout au moins, à une excitation cérébrale subictale.
A neurophysiological study has been made in rabbits of the effects of sex steroids and pituitary and placcntal hormones on sex behavior and pituitary function as related to changes in thresholds of two opposing cerebral systems: the EEG arousal threshold involving the brain stem rcticular formation (I), and the EEG after-reaction threshold involving the rhinencephalon and hypothalamus (TI). In the estrous or estrogen-primed female rabbit, progesterone at first lowers both thresholds for a few hours and subsequently elevates them to supranormal levels until withdrawal or rebound brings them clown again. The early phase is related to estrus and a lowered threshold of pituitary activation, whereas the elevated thresholds correlate with anestrus and pituitary inhibition. Testosterone, certain pituitary and placental hormones, seasonal influences and even prolonged treatment with female sex steroids affect the two thresholds differentially. Relating the effects on thresholds to behavior and to known thresholds of pituitary activation, one may conclude that changes in 1 are more closely related to sexual behavior, whereas alterations in II arc correlated with pituitary thresholds for the release of ovulating hormone. It is concluded that both the facilitatory and inhibitory effects of sex steroids on pituitary activation, as well as the influences on behavior, arc mediated by means of altered thresholds of cerebral activity.
A neurophysiological study has been made in rabbits of the effects of sex steroids and pituitary and placcntal hormones on sex behavior and pituitary function as related to changes in thresholds of two opposing cerebral systems: the EEG arousal threshold involving the brain stem rcticular formation (I), and the EEG after-reaction threshold involving the rhinencephalon and hypothalamus (TI). In the estrous or estrogen-primed female rabbit, progesterone at first lowers both thresholds for a few hours and subsequently elevates them to supranormal levels until withdrawal or rebound brings them clown again. The early phase is related to estrus and a lowered threshold of pituitary activation, whereas the elevated thresholds correlate with anestrus and pituitary inhibition. Testosterone, certain pituitary and placental hormones, seasonal influences and even prolonged treatment with female sex steroids affect the two thresholds differentially. Relating the effects on thresholds to behavior and to known thresholds of pituitary activation, one may conclude that changes in 1 are more closely related to sexual behavior, whereas alterations in II arc correlated with pituitary thresholds for the release of ovulating hormone. It is concluded that both the facilitatory and inhibitory effects of sex steroids on pituitary activation, as well as the influences on behavior, arc mediated by means of altered thresholds of cerebral activity.
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