en Neurology Neurology case report case report 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. MELAS syndrome, mitochondrial disorders. 181 184 http://mjiri.iums.ac.ir/browse.php?a_code=A-10-298-339&slc_lang=en&sid=1 M GHOFRANI 20031947532846004454 20031947532846004454 Yes From the Dept. of Child Neurology, Mofid Children S Hospital, Shahid Beheshti University of Medical Sciences, Tehran GR ZAMANI 20031947532846004455 20031947532846004455 No the Department of Child Neurology, Ahwaz University of Medical Sciences, Ahwaz, I.R: Iran.