In support of this hypothesis, in the single DLB case with sufficient tau pathology to have coexisting Alzheimer’s disease we observed a similar increase in ATP13A2 immunostaining in cortical neurons, with occasional cortical neurons displaying cytoplasmic ATP13A2 aggregates, a finding associated with abnormal or reduced nuclear nucleic acid staining or neurofibrillary tangle-like structures (Additional file 1: Figure S2A). Here, ATP13A2 is linked to early-onset autosomal dominant Alzheimer disease.