Geschwind Syndrome Research Articles

A Narrative Review of the Many Psychiatric Manifestations of Neurosyphilis: The Great Imitator.

Neurosyphilis is an infection of the central nervous system caused by the spirochete, Treponema pallidum. New syphilis infections have been increasing around the world each year. This disease was much of a concern in the pre-penicillin era, where when left untreated many cases progressed to tertiary syphilis which can commonly manifest as neurosyphilis. Of particular interest, neurosyphilis has been linked to masquerading itself as various psychiatric conditions. This narrative review focuses on exploring psychiatric manifestations of neurosyphilis as well as the importance of screening in psychiatric settings and clinicians maintaining high clinical suspicion of the disease. A systematic search was conducted for published articles from 2003 to 2023 using PubMed, EMBASE, and Google Scholar. A total of 66 articles met the criteria and were used for detailed analysis, where psychiatric manifestations and clinical progression of patients were discussed in detail. Psychiatric manifestations that were explored include dementia, delirium, depression, mania, personality changes, and psychosis. One of the most common manifestations of neurosyphilis appears to be severe neurocognitive impairment. There are also rare psychiatric conditions neurosyphilis mimics that have been described in literature such as Capgras syndrome and Geschwind syndrome. A narrative review of the literature revealed a low level of clinical awareness of neurosyphilis as a possible etiology of various psychiatric disorders. This resulted in delayed or inaccurate diagnosis and consequently delayed initiation of adequate treatment. Considering that many psychiatric manifestations of neurosyphilis are reversible with proper treatment, it is imperative to implement routine screening for syphilis among psychiatric patients.

Epilepsy and the "dark" literature of the Greek novelist Demosthenes Voutyras: an outstanding epileptic personality.

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. 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. 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. 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.

Clinical, radiologic, and pathologic features of the globular glial tauopathy subtype of frontotemporal lobar degeneration in right temporal variant frontotemporal dementia with salient features of Geschwind syndrome

ABSTRACT 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. .

Toward a Neurobiological Explanation of Mystical Experience

We have long known that religious or mystical experiences are brain-based phenomena, thanks in part to studies in patients with temporal lobe epilepsy and Gastaut–Geschwind syndrome (1), pharmacological interventions such as psilocybin (2), and demonstrations of unique patterns of neural activity after mystical state induction (3,4). In the current issue of Biological Psychiatry, Ferguson et al. (5) use human lesion evidence to postulate a common neural network for these deeply emotional, self-transcendent mystical experiences.

Can different mechanisms underpin the “Geschwind syndrome” in temporal lobe epilepsy and in temporal lobe variant of frontotemporal degeneration?

Can different mechanisms underpin the “Geschwind syndrome” in temporal lobe epilepsy and in temporal lobe variant of frontotemporal degeneration?

Geschwind Syndrome in frontotemporal lobar degeneration: Neuroanatomical and neuropsychological features over 9 years

Geschwind Syndrome, a characteristic behavioral syndrome frequently described in patients affected by temporal lobe epilepsy (TLE), consists of the following features: hyper-religiosity, hypergraphia, hyposexuality, and irritability. Here we report the 9-year-clinical course of a case of Geschwind Syndrome that developed as a first and salient clinical expression of right temporal lobe variant of frontotemporal lobar degeneration (FTLD). Only one patient affected by frontotemporal dementia has previously been shown to present with Geschwind Syndrome.MS presented at age 73 with 3 years of personality and behavioral symptoms. Her early symptoms primarily included hyper-religiosity, hypergraphia, and poor emotional regulation (irritability, impulsivity, disinhibition, egocentric behavior). Over nine years, other cognitive functions (word retrieval, memory coding and recall, set-shifting, famous face and building recognition) became affected; however, hyper-religiosity, hypergraphia, and scarce emotional control remained her most prominent deficits. Longitudinal cortical thickness and volumetric analyses revealed early atrophy in the right temporal pole, right amygdala, and right hippocampus, which progressively affected homologous regions in the left hemisphere. The present case describes an unusual clinical picture associated with frontotemporal dementia (FTD), in which the most salient symptoms originated and remained consistent with Geschwind Syndrome.

Guru, vriddha, rishi and siddha grahonmaada: Geschwind syndrome?

Background: “Bhuta vidya” (Ayurvedic psychiatry) is one of the eight branches of Ayurveda. The person afflicted with bhuta/graha gets grahonmaada/bhutonmaada and he will exhibit superhuman abilities or qualities without any nown/visible etiopathology. Among the 18 types of bhutonamaada's explained by Vagbhata, “Guru, Vriddha, Rishi and Siddha grahonmaada” (GVRSG) is one. Till date, there is no clear understanding about etiopathology, symptomatology, and management of GVRSG. No works have been conducted on this topic. Aim and Objective: The present article aims at better understanding of GVRSG. Discussion: Geschwind syndrome is characterized by hypergraphia, hyper religiosity, hyposexuality, circumstantiality, and intensified mental life. People like Guru, Vriddha, Rishi, and Siddha in ancient India are known to have qualities such as, “knowledge,” “teaching,” “moralistic,” “disciplined,” “religious,” “ethical,” “experienced,” “having super natural owers,” “counselor,” “guide,” and “following celibacy,” which resembles with the symptomatology of Geschwind syndrome such as, hyper-religiosity, hypergraphia, hyposexuality, emotional liability, grandiosity, and obsessive-compulsive like symptoms. Conclusion: There is marked similarity found between GVRSG and Geschwind syndrome and Ayurvedic diagnosis of Guru, Vriddha, Rishi, and Siddha bhootonmada/grahonmada is suitable for Geschwind syndrome.

Norman Geschwind: career and works

Norman Geschwind was an outstanding American neurologist. He first majored in psychology and then medicine at Harvard University. After graduation, he received neurological training at Beth Israel Hospital, the National Hospital at Queen Square in London, and Boston City Hospital, under the guidance of Denny-Brown, Charles Symonds, and Fred Quadfasel. He later moved to the Boston Veterans Administration Hospital, and established the Boston University Aphasia Research Center with Edith Kaplan. This center played a cardinal role in aphasia research in the United States. In 1969, Geschwind became a Professor at Harvard Medical School. Disconnection syndromes, which made Geschwind's name famous worldwide in the field of neurology, are reviewed here in regard to pure alexia, conduction aphasia, pure word deafness, isolation of the speech area, and apraxia, with reference to the Wernicke-Geschwind model. Other contributions by Geschwind are also described, such as behavioral changes in temporal lobe epilepsy (Geschwind syndrome) and cerebral lateralization, including the Geschwind-Galaburda hypothesis.

Exaltation in temporal lobe epilepsy: neuropsychiatric symptom or portal to the divine?

Religiosity is a prominent feature of the Geschwind syndrome, a behavioural pattern found in some cases of temporal lobe epilepsy. Since the 1950s, when Wilder Penfield induced spiritual feelings by experimental manipulation of the temporal lobes, development of brain imaging technology has revealed neural correlates of intense emotional states, spurring the growth of neurotheology. In their secular empiricism, psychiatry, neurology and psychology are inclined to pathologise deviant religious expression, thereby reinforcing the dualism of objective and phenomenal worlds. Considering theological perspectives and the idea of cosmic consciousness, the authors urge a holistic approach to the spiritual events of epileptic aura, potentially leading to a deeper understanding of the mind and its transcendent potential.

Friedrich Nietzsche and his Illness: A Neurophilosophical Approach to Introspection

There are some arguments that Friedrich Nietzsche suffered from the autosomal dominant vascular microangiopathy: Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy (CADASIL). Here, a hypothesis is formulated supporting that CADASIL presenting with symptoms of bipolar disorder and Gastaut-Geschwind syndrome would contribute to the increased insight and creativity of a philosopher whose perceptions and intuitions often bear out the results of modern neuroscience. Alterations of the brain default and reward networks would account for such an increased level of introspection and creativity. A new framework on approaching illness is proposed, which, in conformity with Nietzsche's positive view, outlines the enabling aspects of some otherwise highly disabling neuropsychiatric disorders.

A Novel Study of Comorbidity between Schizoaffective Disorder and Geschwind Syndrome

Geschwind syndrome has been described in patients with temporal lobe epilepsy and is characterized by sexual behavioural disorders, hyperreligiosity, hypergraphia, and viscosity. Presented here is a case of a 53-year-old man with clinical findings consistent with Geschwind syndrome in the setting of a known diagnosis of schizoaffective disorder, with no identifiable comorbid illness of temporal lobe epilepsy or frontotemporal dementia. Brain MRI showed bilateral temporal lobe atrophy greater than would be expected for age and more prominent on the left side than the right. It is likely that these structural abnormalities may be related to this patient's clinical presentation of Geschwind syndrome. To our knowledge, this is the first reporting of a case of Geschwind syndrome in the setting of schizoaffective disorder. These symptoms of Geschwind syndrome were present irrespective of mental state status. The report highlights the importance in correct identification of underlying cause and differentiation between Geschwind syndrome and schizoaffective disorder in order to avoid mistreatment and consequent iatrogenic adverse events.

Spiritual experiences in temporal lobe epilepsy: a literature review

Profound spiritual experiences have been observed as a trait of temporal lobe epilepsy (TLE), as described in the Geschwind syndrome. This systematic review considers the evidence for ictal and interictal religiosity, and the neurological, psychiatric and cultural factors. Research in this area is at an early stage of development, with indications that a temporal focus is an important factor in spiritual experiences of epilepsy, albeit as a relatively rare and nebulous trait. The literature indicates that spiritual feelings may arise from a reciprocity of biological and psychosocial factors, but nurseq should afford the possibility of a genuinely transcendent experience. Further research may help to explain whether the complex, partial seizures of TLE are a cause of abnormal religiosity or an accessory to spiritual enlightenment.

“Tell me how do I feel” – Emotion recognition and theory of mind in symptomatic mesial temporal lobe epilepsy

Specific interictal personality characteristics in epilepsy, sometimes referred to as “Waxman–Geschwind Syndrome”, have been recognized for centuries and extensively described. Despite the persevering clinical impression that patients with mesial temporal lobe epilepsies (MTLE) suffer from problems in communication and interpersonal relations, uncertainties and controversies remain as to the precise origin of these psychosocial difficulties. Here, we investigated social-cognitive and decision-making abilities using a set of tasks that combine behavioural and psychological measures of social and emotional variables to answer the question of whether patients with MTLE are specifically impaired in social cognition compared to both an epilepsy and a healthy control group. MTLE patients, an epilepsy control group (extra-MTLE; patients with epilepsy, not originating within the frontal or mesial temporal lobe) and healthy controls (HC) were assessed according to their general cognitive status as well as with our Social Cognition Battery, which included tests of basic processes of social cognition, theory of mind, decision making, and various aspects of psychopathology and quality of life. MTLE patients were significantly impaired compared to HC on most measures of the Social Cognition Battery. MTLE patients were predominantly impaired in general emotion recognition compared to extra-MTLE patients. Performance in the epilepsy control group, although not significantly differing from performance in either the MTLE or the healthy control group, lay between these two groups. MTLE can be considered a significant risk factor for the development of deficits in social cognition beyond weaknesses that might be associated with epilepsy as a stigmatized chronic neurological disorder. The presence of deficits in social cognition may explain various behavioural symptoms that have historically driven concepts such as “epileptic personality” or “interictal personality disorder” and may indicate new routes for therapeutic interventions.

The Temporolimbic Personality: A cross-national study

Background and objectives: Early investigators claimed that temporal lobe epilepsy (TLE) was associated with a personality traits and psychiatric symptoms collec- tively known as the interictal behavioral syndrome or Geschwind's syndrome. Interictal behavioral alterations associated with TLE included affective dysregulation; irritability and impulsive aggression; anxiety and obsessive-compulsive symptoms; paranoia; abnor- mal patterns of social interaction; schizophrenic-like symptoms and dissociative states; hypergraphia; and hyperreligiosity. A number of psychiatric disorders are known to have subclinical variants. Are recurrent temporolimbic seizure-like events (as determined by a self-report symptom inventory—the LSCL-Limbic System Checklist) among non-clinical subjects also associated with TLE-related psychiatric symptoms/ syndromes and personality features? Methods: To test this, we examined the clinical/personality profiles of students who self-reported symptoms associated with temporolimbic seizures. Results: In two separate studies, we found that American and Turkish students report- ing temporolimbic seizure-like symptoms had clinical/personality profiles resembling interictal clinical/personality features. Conclusions: Findings do not imply that high- or median-LSCL scorers are afflicted with an undiagnosed TL seizure disorder. Rather, the temporolimbic personality may be found, albeit in milder form, among individuals free of neurologic disease.

Fronto-temporal dementia presenting as Geschwind's syndrome

Geschwind described a syndrome (Geschwind syndrome, GS) in patients with temporal lobe epilepsy, characterized by sexual behavioural disorders, hyper-religiosity, hypergraphia and viscosity. In this report we describe a patient affected by fronto-temporal dementia (FTD), who showed all the personality changes of GS without having epilepsy, and suggest that clinicians should be aware of several other features in FTD, such as hyposexuality and hypergraphia, which are usually not noted during the diagnostic evaluation.

An investigation of religiosity and the Gastaut–Geschwind syndrome in patients with temporal lobe epilepsy

We examined the religious experiences of 28 patients with epilepsy and religiosity, 22 patients with epilepsy and no expressed interest in religion, and 30 volunteer regular churchgoers. We profiled the experiences of the first group, revealing more of their phenomenology, but also their bipolarity, and demonstrated that members of the religious group were significantly more likely to have had past episodes of postictal psychosis, and to have bilateral cerebral dysfunction. We added further data to support the validity of the Bear–Fedio Inventory, and noted that although the experiences of patients with epilepsy are different in content and intensity from the experiences of regular churchgoers, the patients with epilepsy and religiosity conform to those who William James referred to as having, with respect to religion, “an acute fever.”

Classics in Epilepsy and Behavior: Commentary

The article by Waxman and Geschwind [1] may be described as a classic, but it is also emblematic of a marked change in the history of neurology and psychiatry. They were describing a chronic personality change seen in people with epilepsy that was attributed to neurological dysfunction in the temporal lobes. It must be remembered that when this article was published, the prevailing view was that any psychopathology associated with epilepsy was secondary to the consequences of the disorder, rather than integral to the epilepsy itself. This was largely a result of the important contributions of William and Margaret Lennox (1960), who stated broadly that head injuries, sedative antiepileptic drugs, and social stigma were the problems that people with epilepsy suffered and that, if seizures could be controlled, the psychiatric problems would resolve [2]. The idea that alteration of function of the temporal lobes could lead to an organically induced personality change was quite alien to that generation. Why it was is difficult to fathom. By then, it was well established that patients could present with one of a variety of frontal lobe syndromes, which were essentially behavior changes brought about by altered function of the frontal cortex and its connections. Further, MacLean (1958) and others had shown that the limbic system was not simply a rhinencephalon, related to smell and not of great use to Homo sapiens, but was linked to several brain structures that formed circuits for the modulation of emotions [3]. Indeed, some neurological texts would have been bold enough to acknowledge such syndromes, although the term Kluver–Bucy syndrome was still largely recondite. Neurology and psychiatry were divided by the chasm of metaphysics with its ever-flowing cartesian stream, and there seemed no easy way to harmonize the two at that time. Then along came Norman Geschwind and his followers. He gave us the term behavioral neurology, essentially an offshoot of 19th-century neuropsychiatry, a world he reintroduced us to. Thus, he was familiar with the elegant and extensive writings of the French and German neuropsychiatrists of that time, leading him to write another classic, his article on disconnection syndromes [4,5]. This was an attack on modular thinking in neurology, an introduction to connectivity and circuits as explanations for behavioral changes, and an extension of neurological practice into the territory of psychiatry—aberrant behavior caused by neurological disease. However, the article on hypergraphia pushed the concept much further, as it touched on behaviors not usually discussed in neurological circles, for example, religiosity. The characteristics of Gastaut–Geschwind syndrome are now very well known, and yet there are many neurologists, even epileptologists, who find it hard to see the specimen when it flutters before them. They include such symptoms as alterations in sexual behavior, irritability, and viscosity, the last being a tendency toward slow, labored thinking, as if thoughts are emerging from treacle. This sometimes is revealed as circumstantiality, to quote the psychopathologist Frank Fish: Here thinking proceeds slowly with many unnecessary trivial details but the point is finally reached. The goal of thought is never completely lost and thinking proceeds towards it by an intricate and devious path. This disorder has been explained as the result of a weakness of judgement and egocentricity. It is an outstanding feature of the epileptic personality [6].

Psychopathological profile in patients with severe bilateral hippocampal atrophy and temporal lobe epilepsy: evidence in support of the Geschwind syndrome?

Bilateral symmetrical hippocampal atrophy (BHA) has been implicated as a possible causal element in various neuropsychiatric disorders, in particular depressive disorder and schizophrenia. To test the hypothesis that bilateral symmetrical severe volume loss of the hippocampi is of causal relevance to these psychiatric syndromes rather than an epiphenomenon we assessed the psychopathology in a group of patients with temporal lobe epilepsy (TLE) and very severe bilateral symmetrical hippocampal atrophy and compared it with that of a patient control group. Patients with TLE and hippocampal volumes smaller than three standard deviations below the mean of a control population were identified and compared with a matched patient population with normal hippocampal volumes. Psychopathology was assessed by blinded trained psychiatrists using the Present State Examination and Neurobehavioral Inventory. The prevalence of psychiatric syndromes was high in both patient groups; however, there was no significant difference between the two groups. With use of the more specific Neurobehavioral Inventory a psychopathological pattern reminiscent of the Geschwind syndrome emerged when patients with BHA were characterized by caregivers. While BHA does not result in an increased prevalence of specific psychiatric syndromes, specific symptoms that characterize the Geschwind syndrome like hypergraphia and hyposexuality might be pathogenically related to hippocampal atrophy.

Textbook of Clinical Neuropsychiatry

Textbook of Clinical Neuropsychiatry

Personality disorders and epilepsy.

The relationship between epilepsy and personality disorder is viewed. The historical links are discussed, and it is pointed out that the position has moved from that of assuming that everybody with epilepsy will undergo personality change, to noting in particular changes that may be associated with temporal lobe, especially limbic system, abnormalities. The Geschwind syndrome is briefly described, and the association between aggression and epilepsy explored. It is concluded that some aspects of personality may improve following temporal lobectomy, especially if seizures also improve.