A heterozygous recessive variant (p.G268A) in POLG has been linked to autosomal recessive/sporadic progressive external ophthalmoplegia (PEO) in compound heterozygous or homozygous form [43,44], but also described as single-heterozygous in a child with a syndrome including Parkinsonism born from consanguineous parents (probable autosomal recessive mode of inheritance) [45]. Here, POLG is linked to Parkinsonism.