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Globular Glial Tauopathies and 4-Repeat Tauopathies
1. A D E W I J A Y A , M D – A P R I L 2 0 2 3
Globular Glial Tauopathies
2. Ahmed Z, Bigio EH, Budka H, Dickson DW, Ferrer I, Ghetti B, Giaccone G, Hatanpaa KJ, Holton JL, Josephs KA, Powers J.
Globular glial tauopathies (GGT): consensus recommendations. Acta neuropathologica. 2013 Oct;126:537-44.
3. 4-repeat Tauopathies
Progressive supranuclear palsy
Corticobasal degeneration
Argyrophilic grain disease
Glial globular tauopathy
A group of neurodegenerative diseases defined by cytoplasmic inclusions
predominantly composed of tau protein isoforms with four microtubule-
binding domains
Roesler TW, Marvian AT, Brendel M, Nykaenen NP, Hoellerhage M, Schwarz SC, Hopfner F, Koeglsperger T, Respondek G, Schweyer K, Levin J. Four-repeat
tauopathies. Progress in neurobiology. 2019 Sep 1;180:101644.
4. Classification
Ahmed Z, Bigio EH, Budka H, Dickson DW, Ferrer I, Ghetti B, Giaccone G, Hatanpaa KJ, Holton JL, Josephs KA, Powers J.
Globular glial tauopathies (GGT): consensus recommendations. Acta neuropathologica. 2013 Oct;126:537-44.
5. Histopathology
Ahmed Z, Bigio EH, Budka H, Dickson DW, Ferrer I, Ghetti B, Giaccone G, Hatanpaa KJ, Holton JL, Josephs KA, Powers J.
Globular glial tauopathies (GGT): consensus recommendations. Acta neuropathologica. 2013 Oct;126:537-44.
6. Ahmed Z, Bigio EH, Budka H, Dickson DW, Ferrer I, Ghetti B, Giaccone G, Hatanpaa KJ, Holton JL, Josephs KA, Powers J.
Globular glial tauopathies (GGT): consensus recommendations. Acta neuropathologica. 2013 Oct;126:537-44.
7. Ahmed Z, Bigio EH, Budka H, Dickson DW, Ferrer I, Ghetti B, Giaccone G, Hatanpaa KJ, Holton JL, Josephs KA, Powers J.
Globular glial tauopathies (GGT): consensus recommendations. Acta neuropathologica. 2013 Oct;126:537-44.
8. Summary
4-R Tauopathies
Clinical Presentation: FTD and/or MND and/or
extrapyramidal features
GGTs are characterised neuropathologically by tau-
positive GOIs and GAIs, the latter of which are mostly
Gallyas-negative. These characteristic features help GGT
to be distinguished from other 4R-tauopathies, in
particular PSP