It is one of the so-called POLG-related disorders due to mutations in the POLG gene, which encompass a large spectrum of conditions ranging from severe cases of the Alpers-Huttenlocher syndrome to myoclonic epilepsy, myopathy, sensory ataxia (MEMSA), ataxic neuropathies, and progressive external ophthalmoplegia [31]. Here, POLG is linked to progressive external ophthalmoplegia.