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

Autor: Sylvia Josephy-Hernandez, Michael Brickhouse, Samantha Champion, David Dongkyung Kim, Alexandra Touroutoglou, Matthew Frosch, Bradford C. Dickerson
Rok vydání: 2022
Předmět:
Zdroj: Neurocase. 28:375-381
ISSN: 1465-3656
1355-4794
DOI: 10.1080/13554794.2022.2130805
Popis: 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. .
Databáze: OpenAIRE
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