MERRF syndrome (or Myoclonic Epilepsy with Ragged Red Fibers) is a mitochondrial disease maternally-inherited. In this progressive disorder, status epilepticus is common and highly resistant to treatment including generalized anesthesia. We report two cases with Merrf syndrome (sister and brother), presented with status epilepticus refractory even to Thiopental, Propofol and Midazolam. In the s...