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

Andy J. Liu, Jessica E. Chang, Georges Naasan, Adam L. Boxer, Bruce L. Miller, Salvatore Spina

Research output: Contribution to journalArticlepeer-review

11 Scopus citations

Abstract

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.

Original languageEnglish
Pages (from-to)91-97
Number of pages7
JournalNeurocase
Volume26
Issue number2
DOIs
StatePublished - 3 Mar 2020
Externally publishedYes

Keywords

  • Globular glial tauopathy
  • frontotemporal dementia
  • neuropathology
  • primary lateral sclerosis
  • progressive supranuclear palsy

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