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.
MeSH terms
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Adolescent
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Arginine / therapeutic use*
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Brain / drug effects
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Brain / metabolism
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Brain / physiopathology
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Brain Ischemia / prevention & control*
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Drug Therapy, Combination
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Energy Metabolism / drug effects
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Female
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Glycerol / therapeutic use
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Humans
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Lactic Acid / metabolism
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MELAS Syndrome / diagnosis
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MELAS Syndrome / drug therapy*
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MELAS Syndrome / physiopathology
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Mitochondria / drug effects
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Mitochondria / metabolism
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Nitric Oxide / metabolism
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Prednisolone / therapeutic use
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Seizures / drug therapy
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Seizures / etiology
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Seizures / physiopathology
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Stroke / diagnosis
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Stroke / drug therapy*
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Stroke / etiology
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Treatment Outcome
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Vasodilator Agents / therapeutic use
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Vision Disorders / drug therapy
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Vision Disorders / etiology
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Vision Disorders / physiopathology
Substances
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Vasodilator Agents
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Nitric Oxide
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Lactic Acid
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Arginine
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Prednisolone
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Glycerol