The National Institute on Aging—Alzheimer’s Association (NIA-AA) guidelines for the neuropathological evaluation of Alzheimer’s disease (AD), consider extracellular amyloid-β (Aβ) plaques and intracellular neurofibrillary tangles (NFTs) of tau essential for the neuropathologic diagnosis of AD [1]. The gene discussed is MAPT; the disease is early-onset autosomal dominant Alzheimer disease.