APOE and Alzheimer disease: Alzheimer disease (AD)-like features including amyloid beta-protein accumulation – without plaque formation – and paired-helical filament neurofibrillary tangles (NFTs) are consistently seen in NP-C, mostly in the basal ganglia, hypothalamus, brain stem and spinal cord [75, 76], and are possibly related to dysregulation of cholesterol metabolism and apolipoprotein E genotype [76, 77].