The present disclosure provides methods for treating, preventing, ameliorates, inhibits, or delaying the onset of Leigh syndrome as well as methods for ameliorating, inhibiting, ameliorates, inhibits, or delaying the onset of its associated signs and / or symptoms in a subject in need thereof comprising administering to the subject various compounds, mixtures of compounds or compositions, formulations or medicaments derived therefrom. The present disclosure further provides compositions, formulations or medicaments and related uses for the treatment, prevention ameliorating, inhibiting, or delaying the onset of Leigh syndrome in a subject and addressing its associated signs and / or symptoms.