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

Abstract

Geschwind Syndrome, a characteristic behavioral syndrome frequently described in patients affected by temporal lobe epilepsy, 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. 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, in which the most salient symptoms originated and remained consistent with Geschwind Syndrome.

A category-selective semantic memory deficit for animate objects in semantic variant primary progressive aphasia

“…Future studies should include longitudinal investigations to improve our understanding of how the localization, magnitude, and spread of atrophy in svPPA relates to the presence and magnitude of a CSSD for animate objects or other categories of information, including emotionally-charged information. 136 , 137 These next steps will improve our understanding of brain systems that subserve dissociable categories of semantic memory and how their degeneration impacts patients’ understanding of the world around them.…”

Dreamy States and Cosmic Wanderings

“…22 As such, they are deemed neither authentic nor appropriate to the hegemony of neurotypical functioning, and the conventional reaction of the medical profession to an individual reporting such an experience is to pathologize it, 23 regarding it as, at best, delusional or hallucinatory 24 and, at worst, symptomatic of Geschwind syndrome, a contested behavioral diagnosis still used by clinicians. 25 It is identified by hypergraphia, an intense mental life, hyposexuality, irritability, and hyper-religiosity. 26 William James found this materialist understanding of EFEs to be too reductive.…”

Social Cognition and Behavioral Assessments Improve the Diagnosis of Behavioral Variant of Frontotemporal Dementia in Older Peruvians With Low Educational Levels

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