Progressive supranuclear palsy (PSP) is a neuropathologically defined entity that is challenging to diagnose antemortem. In 2017, the Movement Disorders Society put forward new clinical criteria for the diagnosis of PSP recognizing diverse PSP phenotypes. 

Patients with clinical parkinsonism, clinical and/or neuropathological diagnosis of PSP were identified from the Cure PSP brain bank, Mayo Clinic Florida. All patients had neuropathologic diagnoses and detailed clinical examination performed by a specialist in Movement Disorders or Behavioral Neurology at one of the three Mayo Clinic sites located in Florida, Arizona and Minnesota. Clinical data on symptoms and signs were abstracted retrospectively in a blinded fashion and used to determine whether or not patients met NINDS or MDS PSP criteria. Patients

were divided into early and late disease stage groups using a 3-year cut off. Sensitivity and specificity were calculated for the entire cohort and for early versus late time points. 

A total of 129 patients were included of which 66 (51%) had PSP. The remainder had other neurodegenerative diseases including corticobasal degeneration, Parkinson’s disease, multiple system atrophy, frontotemporal lobar degeneration with transactive DNA binding protein 43 and globular glial tauopathy. The overall

sensitivity of the MDS criteria was 87.9% compared to 45.5% for NINDS, whereas the specificity of the MDS probable PSP criteria was 85.7% and 90.5% for NINDS criteria. Patients were noted to have features of multiple PSP phenotypes. 

The MDS criteria for the diagnosis of PSP recognizes several phenotypes of PSP and hence has a higher sensitivity than the NINDS criteria. False positive diagnoses resulted predominantly from other tauopathies.