MAPT-Associated Familial Progressive Supranuclear Palsy with Typical Corticobasal Degeneration Neuropathology: A Clinicopathological Report

Abstract

Corticobasal degeneration (CBD) is a rare, 4-repeat (4R) tauopathy characterized by astrocytic plaque neuropathology. Although ~25% of sporadic CBD cases present with progressive supranuclear palsy-Richardson's syndrome (PSP-RS),1 only one other case of microtubule-associated protein tau gene (MAPT)- related CBD with a PSP-like phenotype has been reported.2 We aim to highlight important issues regarding the classification of MAPT-associated tauopathies and the implications for clinical research

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