TARDBP and supranuclear palsy, progressive, 1: Nine subjects in the CBS group came to autopsy with pathological diagnoses of pure CBD in five, mixed disease - dementia with agyrophilic grains + CBD + cerebral amyloid angiopathy in one, PSP in two, and FTLD-U/TDP43 proteinopathy due to mutation in the progranulin gene (GRN) in one [56].