Lewy body pathology is not restricted to Parkinson’s disease (PD), PD dementia (PDD), and dementia with Lewy bodies (DLB), but is also observed in several neurodevelopmental and neurometabolic disorders, such as PLA2G6-associated neurodegeneration (i.e., infantile neuroaxonal dystrophy, atypical neuroaxonal dystrophy, adult-onset dystonia-parkinsonism, and autosomal recessive early-onset parkinsonism), POLG-associated neurodegeneration, Niemann-Pick type C1, and Krabbe disease [5]. Here, POLG is linked to Parkinson disease.