Leigh syndrome, French-Canadian type (LSFC, MIM#220111) is a neurogenetic degenerative disorder caused by mutations in the nuclear gene leucine-rich pentatricopeptide repeat-containing (LRPPRC) localized on 2p16. The gene discussed is LRPPRC; the disease is congenital lactic acidosis, Saguenay-Lac-Saint-Jean type.