Progressive supranuclear palsy and primary lateral sclerosis secondary to globular glial tauopathy: a case report and a practical theoretical framework for the clinical prediction of this rare pathological entity.

Journal Article (Journal Article)

Globular glial tauopathy (GGT) is a rare 4-repeat tauopathy characterized by the accumulation of tau globular inclusions in astrocytes and oligodendrocytes. Several clinical phenotypes have been associated with GGT, making the prediction of this rare pathological entity difficult. We report the case of a patient with eye-movement abnormalities and gait instability, reminiscent of progressive supranuclear palsy-Richardson's syndrome (PSP-RS), who later developed upper motor neuron symptoms suggestive of primary lateral sclerosis (PLS). Neuropathological assessment revealed GGT type III pathology. A theoretical framework is proposed to help clinicians predict GGT in subjects with coexistent features of PSP-RS and PLS.

Full Text

Duke Authors

Cited Authors

  • Liu, AJ; Chang, JE; Naasan, G; Boxer, AL; Miller, BL; Spina, S

Published Date

  • April 2020

Published In

Volume / Issue

  • 26 / 2

Start / End Page

  • 91 - 97

PubMed ID

  • 32090696

Pubmed Central ID

  • PMC7197509

Electronic International Standard Serial Number (EISSN)

  • 1465-3656

Digital Object Identifier (DOI)

  • 10.1080/13554794.2020.1732427


  • eng

Conference Location

  • England