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| Korean Journal of Neuromuscular Disorders ; 7 : 45 - 47, June 2015 |
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| Steroid Responsive Encephalopathy in Patient with MELAS Syndrome |
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| Byoung-Jun Yun, MD, Min-Oh Lee, MD, Soo-Hyun Park, MD, Nam-Hee Kim, MD |
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| Department of Neurology, Dongguk University Ilsan Hospital, Dongguk University College of Medicine, Goyang, Korea |
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Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is
a common mitochondrial disease that can cause a wide range of clinical symptoms. We describe a
26-year-old woman diagnosed with MELAS presenting recurrent episodes of seizures, right sided
weakness, visual field defect, and cognitive decline. These recurrent symptoms improved after high
dose corticosteroid treatment, and three months’ maintenance of steroid prevented further recurrence
of encephalopathy. Corticosteroid therapy may be beneficial during acute exacerbation of MELAS.
KEYWORDS
MELAS syndrome,
Steroid,
Mitochondrial disease |
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