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

Neurocase. 2022 Aug;28(4):375-381. doi: 10.1080/13554794.2022.2130805. Epub 2022 Oct 17.

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. .

Keywords: GGT; Geschwind syndrome; Globular glial tauopathy type III; frontotemporal lobar degeneration; right temporal variant frontotemporal dementia.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Research Support, N.I.H., Extramural

MeSH terms

  • Atrophy / pathology
  • Brain / pathology
  • Female
  • Frontotemporal Dementia* / diagnostic imaging
  • Frontotemporal Dementia* / pathology
  • Frontotemporal Lobar Degeneration* / diagnostic imaging
  • Frontotemporal Lobar Degeneration* / pathology
  • Humans
  • Pick Disease of the Brain* / pathology
  • Tauopathies* / diagnostic imaging
  • Tauopathies* / pathology
  • tau Proteins / metabolism

Substances

  • tau Proteins