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KMID : 1103720200810061478
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Journal of the Korean Society of Radiology
2020 Volume.81 No. 6 p.1478 ~ p.1485
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Leigh Syndrome Mimicking Wernicke's Encephalopathy: A Case Report
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Oh Ji-Soo
Choi Jin-Ok
Kim Soo-Jung
Yoo Eun-Ae
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Abstract
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Leigh syndrome or subacute necrotizing encephalomyelopathy is a rare, rapidly progressive neurodegenerative disorder. In general, symptoms such as shortness of breath and decreased cardiac function usually occur within 1 year of life. It is a serious disease with a mortality rate of 75% in 2?3 years. The cause of Leigh syndrome is DNA mutation. Approximately 75% of patients have nuclear DNA mutations while 25% have mitochondrial DNA mutations. Clinical symptoms vary depending on the affected brain area. Neuroimaging plays an important role in diagnosing patients with Leigh syndrome. Late-onset Leigh syndrome is rarer and progresses more slowly compared to the classic form. Here, we report a case of late-onset Leigh's syndrome mimicking Wernicke's encephalopathy.
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KEYWORD
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Leigh Syndrome, Wernicke's Encephalopathy, Mitochondria
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