In primary tauopathies, MAPT mutations are known to cause frontotemporal lobar degeneration (FTLD) with Tau pathology, a heterogeneous term that encompasses diseases such as progressive supranuclear palsy (PSP), corticobasal degeneration, or Pick’s disease, which present clinical and pathological heterogeneity, with clinical manifestations ranging from cognitive decline to movement disorders (6, 7). Here, MAPT is linked to frontotemporal dementia.