MELAS SYNDROME IN TWO IRANIAN CHILDREN

MELAS syndrome is a mitochondrial disorder with progressive nature, because adequate treatment is not available. Diagnosis of this mitochondrial disorder depends initially on clinical suspicion, which is strengthened by additional metabolic evidence of impaired oxidative metabolism such as high serum or C.S.F. lactate levels and confirmed by demonstration of mitochondrial abnormalities-in muscle biopsy. Here we present the clinical course and management of two children with MELAS syndrome who exhibited progressive neurologic deterioration.