Melas syndrome: a diagnostic and therapeutic approach - a report of two cases Hospital de San José, Bogotá DC.

Melas: aproximación diagnóstica y experiencia terapéutica reporte de dos casos. Hospital de San José, Bogotá DC.

Mitochondrial encephalomyopathy, lactic acidosis and stroke-Iike episodes (MELAS) is a rare syndrome featured by encephalopathy with seizures, lactic acidosis and stroke-Iike episodes which constitute a cause of cerebrovascular disease (CVC) in young adults. MELAS has extreme variability of clinical expressions in members of the same family. Its pathophysiology is based on cellular ATP depletion caused by impaired oxidative phosphorylation secondary to a mtDNA mutation. A mutation of nucleotide pair 3243 A>G is found in most cases. An adequate treatment protocol for MELAS has not yet been established and recommendationsare based on case reports or open randomized clinical trials. Coexisting conditions must receive adequate treatment considering that sorne medications can exacerbate the disease.

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