They are particularly frequent in frontotemporal lobar degeneration (FTLD-tau) disorders including Pick’s disease and corticobasal degeneration which, interestingly, show prominent white matter/axonal involvement, when compared to another tauopathy, progressive supranuclear palsy, in which the white matter is relatively spared and the ballooned neurons not particularly numerous [7]. The gene discussed is MAPT; the disease is frontotemporal dementia.