Rare missense and exon 10 splicing mutations, which lead to increased levels of tau isoforms with four microtubule binding domains (aka 4-repeat or 4R tau) lead to familial frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17) [2,3], whereas the common MAPT H1 haplotype strongly associates with increased risk of progressive supranuclear palsy (PSP) and corticobasal degeneration (CBD) [4-8]. The gene discussed is MAPT; the disease is Classical progressive supranuclear palsy.