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

• Published in: Neurocase Vol. 26; no. 2; pp. 91 - 97
• Main Authors: Liu, Andy J., Chang, Jessica E., Naasan, Georges, Boxer, Adam L., Miller, Bruce L., Spina, Salvatore
• Format: Journal Article
• Language: English
• Published: England Routledge 03.03.2020; Taylor & Francis Ltd
• Subjects: Aged, 80 and over; Astrocytes; Case reports; Dementia; Fatal Outcome; frontotemporal dementia; Frontotemporal Dementia - complications; Frontotemporal Dementia - diagnosis; Frontotemporal Dementia - pathology; Frontotemporal Dementia - physiopathology; Gait; Gait Disorders, Neurologic - diagnosis; Gait Disorders, Neurologic - etiology; Gait Disorders, Neurologic - physiopathology; Globular glial tauopathy; Humans; Male; Motor Neuron Disease - complications; Motor Neuron Disease - diagnosis; Motor Neuron Disease - pathology; Motor Neuron Disease - physiopathology; Motor neuron diseases; Neurodegenerative diseases; Neuroglia - pathology; neuropathology; Ocular Motility Disorders - diagnosis; Ocular Motility Disorders - etiology; Ocular Motility Disorders - physiopathology; Oligodendrocytes; Paralysis; Phenotypes; primary lateral sclerosis; Progressive supranuclear palsy; Supranuclear Palsy, Progressive - complications; Supranuclear Palsy, Progressive - diagnosis; Supranuclear Palsy, Progressive - pathology; Supranuclear Palsy, Progressive - physiopathology; Tau protein; Tauopathies - complications; Tauopathies - diagnosis; Tauopathies - pathology; Tauopathies - physiopathology; frontotemporal dementia; primary lateral sclerosis; neuropathology; Globular glial tauopathy; progressive supranuclear palsy
• ISSN: 1355-4794; 1465-3656; 1465-3656; 1362-4970
• DOI: 10.1080/13554794.2020.1732427

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.