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.
Keywords:
Globular glial tauopathy; frontotemporal dementia; neuropathology; primary lateral sclerosis; progressive supranuclear palsy.
Publication types
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Case Reports
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
MeSH terms
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Aged, 80 and over
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Fatal Outcome
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Frontotemporal Dementia / complications
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Frontotemporal Dementia / diagnosis*
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Frontotemporal Dementia / pathology
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Frontotemporal Dementia / physiopathology
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Gait Disorders, Neurologic / diagnosis
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Gait Disorders, Neurologic / etiology
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Gait Disorders, Neurologic / physiopathology
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Humans
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Male
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Motor Neuron Disease / complications
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Motor Neuron Disease / diagnosis*
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Motor Neuron Disease / pathology
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Motor Neuron Disease / physiopathology
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Neuroglia / pathology*
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Ocular Motility Disorders / diagnosis
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Ocular Motility Disorders / etiology
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Ocular Motility Disorders / physiopathology
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Supranuclear Palsy, Progressive / complications
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Supranuclear Palsy, Progressive / diagnosis
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Supranuclear Palsy, Progressive / pathology
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Supranuclear Palsy, Progressive / physiopathology
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Tauopathies / complications
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Tauopathies / diagnosis*
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Tauopathies / pathology
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Tauopathies / physiopathology