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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.

Publication ,  Journal Article
Liu, AJ; Chang, JE; Naasan, G; Boxer, AL; Miller, BL; Spina, S
Published in: Neurocase
April 2020

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.

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Published In

Neurocase

DOI

EISSN

1465-3656

Publication Date

April 2020

Volume

26

Issue

2

Start / End Page

91 / 97

Location

England

Related Subject Headings

  • Tauopathies
  • Supranuclear Palsy, Progressive
  • Ocular Motility Disorders
  • Neuroglia
  • Motor Neuron Disease
  • Male
  • Humans
  • Gait Disorders, Neurologic
  • Frontotemporal Dementia
  • Fatal Outcome
 

Citation

APA
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MLA
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Liu, A. J., Chang, J. E., Naasan, G., Boxer, A. L., Miller, B. L., & Spina, S. (2020). 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. Neurocase, 26(2), 91–97. https://doi.org/10.1080/13554794.2020.1732427
Liu, Andy J., Jessica E. Chang, Georges Naasan, Adam L. Boxer, Bruce L. Miller, and Salvatore Spina. “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.Neurocase 26, no. 2 (April 2020): 91–97. https://doi.org/10.1080/13554794.2020.1732427.

Published In

Neurocase

DOI

EISSN

1465-3656

Publication Date

April 2020

Volume

26

Issue

2

Start / End Page

91 / 97

Location

England

Related Subject Headings

  • Tauopathies
  • Supranuclear Palsy, Progressive
  • Ocular Motility Disorders
  • Neuroglia
  • Motor Neuron Disease
  • Male
  • Humans
  • Gait Disorders, Neurologic
  • Frontotemporal Dementia
  • Fatal Outcome