Elsevier

Brain and Development

Volume 26, Issue 7, October 2004, Pages 481-483
Brain and Development

Case report
Beneficial effect of l-arginine for stroke-like episode in MELAS

https://doi.org/10.1016/j.braindev.2004.01.006Get rights and content

Abstract

We here reported the clinical course and therapeutic details of a 16-year-old girl with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) who had had five stroke-like episodes (two episodes were clinically mild, while the three subsequent episodes were severe). Among the three episodes, the symptoms improved earliest and magnetic resonance spectroscopy abnormality was minimal when given l-arginine in addition to prednisolone, glycerol and edalavone. l-arginine administration during the acute phase of MELAS might be a potential therapy to reduce brain damage due to mitochondrial dysfunction.

Introduction

Although MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) is a systemic disorder of mitochondrial dysfunction caused by mitochondrial DNA mutation, the pathogenesis of stroke-like episodes is not yet clear. It is regarded that stroke-like episodes are not simple infarcts but the results of transient oxidative-phosphorylation failure within the brain. Although prophylactic or supportive therapies for each symptom were reported to be partially effective [1], [2], [3], [4], the disease is basically progressive. Once stroke-like episodes have begun to occur frequently, the general status of the patient tends to deteriorate progressively. Recently, Koga et al. [5] reported that l-arginine therapy improved microcirculation of the brain and thus reduced tissue injury from ischemia during the acute phase of stroke-like episodes in MELAS. l-arginine is a potent donor of nitric oxide, which may reduce ischemic damage in the acute phase of focal brain ischemia. We here reported the clinical course and therapeutic details of a 16-year-old girl with MELAS who had had five stroke-like episodes and discuss the efficacy of l-arginine therapy as demonstrated by MRS (proton magnetic resonance spectroscopy).

Section snippets

Case report

The diagnosis of MELAS was confirmed by muscle biopsy findings including ragged red fibers and gene analysis (point mutation of A3243G in the mitochondrial tRNALeu(UUR) gene), at the age of 8 years when the patient developed limb weakness and easy fatigability. Starting at 9 years of age, dichloroacetate (DCA) and vitamin B1 administration lowered the elevated levels of lactate and pyruvate in the serum and CSF and ameliorated abdominal pain, headache, and improved school performance and

Discussion

The first two stroke-like episodes were clinically mild, while the three subsequent episodes were severe. Durations of hospitalization of the last three episodes were 25, 8 and 4 days for the third, fourth and fifth episodes, respectively. Although the severity of disability was almost the same among these three episodes, the symptoms improved earliest after the fifth episode compared to the other two episodes. Steroid and glycerol were used for all three episodes and edalavone for the last two

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