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From the Department of Pediatrics and Child Health (Drs. Koga, Akita, Nishioka, Yatsuga, Povalko, and Matsuishi), Kurume University School of Medicine, Fukuoka, Japan; Department of Pediatric Neurology (Dr. Tanabe), Chiba Childrens Hospital, Chiba, Japan; Department of Pediatrics (Dr. Fujimoto), Nagoya City University School of Medicine, Nagoya, Japan; and Research Centre for Medical Genetics RAMNS (Dr. Povalko), Moscow, Russia.
Address correspondence and reprint requests to Dr. Yasutoshi Koga, Department of Pediatrics and Child Health, Kurume University School of Medicine, 67 Asahi-Machi, Kurume City, Fukuoka 830-0011, Japan; e-mail: yasukoga{at}med.kurume-u.ac.jp
Based on the hypothesis that mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS) are caused by impaired vasodilation in an intracerebral artery, the authors evaluated the effects of administering l-arginine, a nitric oxide precursor. Patients were administered l-arginine intravenously at the acute phase or orally at the interictal phase. l-Arginine infusions significantly improved all strokelike symptoms, suggesting that oral administration within 30 minutes of a stroke significantly decreased frequency and severity of strokelike episodes.
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