The most frequent pathological findings in PCA are tau neurofibrillary tangles and beta-amyloid neuritic plaques which are characteristic of Alzheimer’s disease [4], although other pathologies have been described in PCA including corticobasal degeneration, diffuse Lewy body disease, and Creutzfeldt-Jakob disease [5–7]; frontotemporal dementia with progranulin mutation has also been reported [8]. Here, MAPT is linked to early-onset autosomal dominant Alzheimer disease.