Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a common mitochondrial disease that can cause a wide range of clinical symptoms. We describe a 26-year-old woman diagnosed with MELAS presenting recurrent episodes of seizures, right sided weakness, visual field defect, and cognitive decline. These recurrent symptoms improved after high dose corticosteroid treatment, and three months’ maintenance of steroid prevented further recurrence of encephalopathy. Corticosteroid therapy may be beneficial during acute exacerbation of MELAS.
