Semantic dementia: a complex and culturally influenced presentation.

SUMMARY: The variants of frontotemporal dementia (FTD) require careful differentiation from primary psychiatric disorders as the neuropsychiatric manifestations can overshadow the unique cognitive deficits. The language variants of FTD are less readily recognised by trainees despite making up around 43% of cases. This educational article presents an anonymised case of one of the language variants: semantic dementia. The cognitive deficits and neuropsychiatric manifestations (delusions and hyperreligiosity) are explored in terms of aetiology and management. By the end of the article, readers should be able to differentiate FTD from Alzheimer's disease, understand the principles of management and associated risks, and develop a multifaceted approach to hyperreligiosity in dementia.

Religious conversion in an older male with longstanding epilepsy.

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, may have contributed to religious conversion, which was followed by dementia and delusions involving religious content.

A Neural Circuit for Spirituality and Religiosity Derived From Patients With Brain Lesions.

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 applied 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 gray. Intersection of lesion locations with this same circuit aligned with self-reported religiosity in an independent dataset and previous 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 gray, a brainstem region previously implicated in fear conditioning, pain modulation, and altruistic behavior.

Hyper-religiosity and visual hallucinations in a patient with frontotemporal dementia carrying a double variant in GRN gene.

Introduction: Hyper-religiosity has been reported in patients affected by frontotemporal dementia (FTD) with asymmetrical, predominantly right-sided frontotemporal atrophy. Case report: We report a FTD patient carrying a double genetic variant (p.Cys139Arg and c.*78C > T) in the progranulin (GRN) gene who showed an unusual clinical phenotype characterized by hyper-religiosity behavior and visual hallucinations with exclusively religious content. Noteworthy, this patient exhibited a slow clinical and radiological rate of disease progression and a predominantly left-sided frontotemporal atrophy. Discussion and conclusion: The simultaneous presence of these GRN variants in our FTD patient with predominant atrophy in the left (dominant) hemisphere could determine the unusual phenotype with hyper-religiosity and visual hallucinations with exclusively religious content and influence the slow rate of disease progression.

A Case of Attempted Bilateral Self-Enucleation in a Patient with Bipolar Disorder.

Attempted and completed self-enucleation, or removal of one's own eyes, is a rare but devastating form of self-mutilation behavior. It is often associated with psychiatric disorders, particularly schizophrenia, substance induced psychosis, and bipolar disorder. We report a case of a patient with a history of bipolar disorder who gouged his eyes bilaterally as an attempt to self-enucleate himself. On presentation, the patient was manic with both psychotic features of hyperreligous delusions and command auditory hallucinations of God telling him to take his eyes out. On presentation, the patient had no light perception vision in both eyes and his exam displayed severe proptosis, extensive conjunctival lacerations, and visibly avulsed extraocular muscles on the right side. An emergency computed tomography scan of the orbits revealed small and irregular globes, air within the orbits, and intraocular hemorrhage. He was taken to the operating room for surgical repair of his injuries. Attempted and completed self-enucleation is most commonly associated with schizophrenia and substance induced psychosis, but can also present in patients with bipolar disorder. Other less commonly associated disorders include obsessive-compulsive disorder, depression, mental retardation, neurosyphilis, Lesch-Nyhan syndrome, and structural brain lesions.

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.

Hyperreligiosity in malignant brain tumors: a case report and accompanying bibliographic review.

Religion is a complex cognitive process with biopsychosocial and cultural dimensions, product of the activation of different circuits of the neocortex. In some cases, religiosity can appear as a pathological correlate in patients with brain lesions in the areas involved. We present the clinical case of a patient with an astrocytoma in the right prefrontal region, with apparent inflammatory involvement of the right temporal lobe. This tumor debuted almost exclusively as an alteration of personality consisting of hyperreligiosity, logorrhea, and mystical experiences. A review of the literature has been conducted and possible pathophysiological mechanisms are proposed.

Cotard Delusion in the Context of Schizophrenia: A Case Report and Review of the Literature.

BACKGROUND: The Cotard delusion (CD) is one of a variety of narrowly defined monothematic delusions characterized by nihilistic beliefs about the body's existence or life itself. The presence of CD within the context of schizophrenia is rare (<1%), and remains understudied. CASE: 'Mr. C' is a 58-year-old veteran with a prior diagnosis of schizophrenia, who presented with CD in the context of significant depression, suicidal ideation, violence, and self-harm behavior. He perseverated in his belief that he was physically dead and possessed by demons for several weeks. This delusion was reinforced by his religious belief that life was an attribute of God, and by inference, he as a human, was dead. His condition gradually improved over the course of treatment with Divalproex and quetiapine with discussions about the rationale for his belief. Upon discharge, Mr. C. demonstrated awareness of his fixation on death and an ability to redirect himself. DISCUSSION: This case highlights the need to better understand the co-occurrence of CD in schizophrenia, their differentiation, the increased risk of violence and self-harm behavior in this presentation, and how specific events and religious factors can influence delusional themes of CD. Pharmacotherapy and aspects of cognitive-behavioral therapy may be effective in ameliorating these symptoms in CD.

Epilepsia, psicose e religiosidade – Caso clínico / Epilepsy, psychosis and religiosity – A case report

Os autores apresentam o caso de uma paciente de 61 anos, com antecedentes depressivos e história de síncopes, que desenvolve, no espaço de meio ano, alterações de comportamento pautadas por hiper-religiosidade, ideias delirantes de grandiosidade e fenômenos compatíveis com déjà vu. Nesse contexto, foi referenciada pelo médico de família para a urgência de psiquiatria, tendo sido realizada investigação orgânica e identificada epilepsia do lobo temporal após realização de eletroencefalograma. Foi medicada com valproato de sódio, na dose de 750 mg/dia, com esbatimento progressivo da sintomatologia psicótica. Com este trabalho, os autores pretendem sublinhar a importância da exploração orgânica dos sintomas neuropsiquiátricos, antes de atribuir um diagnóstico psiquiátrico ao paciente.

The authors report the case of a 61 years-old woman, with history of depressive episodes and syncope, who developed hyperreligiosity, grandiose delusions and déjà vu. She was admitted to a psychiatry emergency and the electroencephalogram identified temporal lobe epilepsy. She was medicated with valproate sodium 750 mg/day with remission of psychotic symptoms and return to premorbid function. The purpose of this work is to highlight the importance of organic investigation in the assessment of neuropsychiatric symptoms.

Carbamazepine in interictal hyperreligiosity: three case reports.

Three patients with manifestations of hyperreligiosity during the post ictal period are presented- The compute clearance of these manifestations on carbamazepine monotherapy is highlighted.