[P1-143] Beneficial Effect of Intravenous L-arginine in a Pediatric Patient with MELAS
Several case reports and limited case series have indicated a possible role for L-arginine in acute metabolic strokes seen in mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS). We present a 9 year old boy with MELAS who was treated with recurrent intravenous (IV) L-arginine for multiple stroke-like episodes. He experienced nausea, vomitting at 8 years and was diagnosed as viral encephalitis. He was treated with acyclovir, however ten days after discharge, his complaints had recurred, he received another course of acyclovir and was reffered to our hospital. His MRI was remarkable for cerebellar atrophy and cortical lesions suggestive of MELAS. A point mutation at nucleotide 3243 in the tRNALeu(UUR) was detected in mitochondrial DNA. L-arginine (0.2 mg/kg/day PO) was started, in addition to coctail. After 6 months, he presented with a generalized tonic clonic seizure and severe headache associated with a stroke; he was started on levetirecetam. After a month, he complained of weakness in the right hand. The third episode occurred 10 days later, when the patient complained of progressive weakness in the right side, accompanied by difficulty in swallowing. L-arginine (0.25 g/kg/day IV) was administered for 2 days resulting in complete recovery. However on the second day, he had blurred in vision and lethargy; L-arginine (0.5 g/kg/day IV) was given for additional 5 days. No adverse effects were detected during IV L-arginine treatment. We conclude that IV L-arginine administration is well tolerated and may improve neurological deficits occured during stroke-like episodes in children with MELAS.