Elective surgery should not be performed during a stroke-like episode

MELAS is a genetic, syndromic, multisystem mitochondrial disorder with stroke-like episodes (SLEs) as a hallmark of the phenotype. SLEs must be clearly differentiated from ischemic stroke, as treatment and outcome vary significantly between the two. MELAS is phenotypically and genotypically heterogeneous, but is due to the variant m.3243A>G in MT-TL1 in 80% of cases. No patient with MELAS due to a SOX5 variant was reported. Due to the genetic and metabolic specifics of the syndrome, diagnostic and therapeutic management require the guidance of a multidsciplinary team familiar with the management of MIDs. Since surgery is associated with stress, it is conceivable that surgery or anaesthesia could exacerbate the MELAS phenotype. Patients with existing SLE should not undergo elective surgery.