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. Issue 2 (3rd March 2020)

• Record Type: Journal Article
• Authors: Liu, Andy J.; Chang, Jessica E.; Naasan, Georges; Boxer, Adam L.; Miller, Bruce L.; Spina, Salvatore
• Is Part Of: Neurocase. Volume 26:Issue 2(2020)
• Page Start: 91
• Page End: 97
• Publication Date: 2020-03-03
• Subjects: Globular glial tauopathy -- primary lateral sclerosis -- frontotemporal dementia -- progressive supranuclear palsy -- neuropathology; Neurology -- Periodicals; Neuropsychology -- Case Report; Mental Disorders -- Case Report; Nervous System Diseases -- Case Report; 616.8
• DOI: 10.1080/13554794.2020.1732427
• Languages: English
• ISSNs: 1355-4794

Abstract

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.