Long Term Follow-Up of Recurrent Status Epilepticus and Stroke-Like Episodes in a Melas Family

Background: Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) is a disorder commonly caused by the A3243G/tRNALeu mutation of mitochondrial DNA (mtDNA). MELAS patients are at high risk of developing status epilepticus (SE) during stroke-like episodes (SLEs). We describe the long-term follow-up of 2 affected members of a MELAS family with recurrent focal SE associated with SLEs. SE treatment is discussed.