Whereas most tauopathies such as Pick's disease, corticobasal degeneration, progressive supranuclear palsy and Alzheimer's disease (AD) are not linked to mutations, frontotemporal dementia with parkinsonism linked to chromosome 17 is caused by autosomal dominant mutations in the MAPT gene [4]. Here, MAPT is linked to early-onset autosomal dominant Alzheimer disease.