A member of a family with an autosomal dominant pattern of frontotemporal dementia (FTD) with a TDP-43 pathological substrate in other members and no mutations in FTD-associated genes developed behavioral variant FTD followed by Progressive Supranuclear Palsy. Autopsy revealed a pure tauopathy of PSP pattern. Conclusions: The findings raise the possibility of shared pathogenic pathways and a proximal genetic abnormality between PSP and FTLD-43.
- Progressive Supranuclear Palsy
- frontotemporal dementia
ASJC Scopus subject areas
- Arts and Humanities (miscellaneous)
- Clinical Neurology