Although unusual, some patients with genetically-confirmed OPA1 mutations have been described with acute and even reversible visual loss (Cornille et al., 2008; Nochez et al., 2009), and a subgroup of LHON patients can present with a slowly progressive optic neuropathy, more suggestive of DOA (Barboni et al., 2006). Here, OPA1 is linked to Optic neuropathy.