Lastly, although amygdala LB pathology has been reported to occur frequently in concomitance with other protein misfolding disorders (e.g., AD and other tauopathies [46, 62]), we found no cases of the amygdala-predominant LBD variant in our CJD cohort, not even in sCJD subtypes with significant pathological involvement of the amygdala (i.e., VV2, VV1, and MV2K) [5, 6], supporting the prominent role of AD-related tau pathology in determining this subtype of focal LBD pathology. Here, MAPT is linked to Alzheimer disease.