• Can clinically present with bvFTD (Type 1), PSP/CBS with MND/PLS spectrum (Type 2) and mixed (Type 3), based on topographic location of white matter deposits of tau immunoreactive globular glial inclusions (117) • Atypical PSP with marked upper motor neuron (UMN) signs (PSP-PLS phenotype) can be a clinical clue • Other phenotypes: PPA-G with chorea (118), PPA-S (119), Mill's hemiplegic variant of MND (120). The gene discussed is MAPT; the disease is supranuclear palsy, progressive, 1.