Although extrapyramidal features are relatively rare in patients with mitochondrial disorders there have been a number of reports of Parkinsonism associated with mutations within POLG. In these cases there is often pigmented SN neuron loss and on occasions Lewy body pathology (Betts-Henderson et al., 2009; Luoma et al., 2004) and however we have detected Lewy body pathology in a POLG patient with no discernible cell loss or PD-like symptoms (Reeve et al., 2013). This evidence concerns the gene POLG and Parkinson disease.