Leigh Syndrome Mimicking Wernicke's Encephalopathy: A Case Report
오지수, 최진옥, 김수정, 유은애,
소속 상세정보
오지수 ( Oh Ji-Soo )
Presbyterian Medical Center Department of Radiology
최진옥 ( Choi Jin-Ok )
Presbyterian Medical Center Department of Radiology
김수정 ( Kim Soo-Jung )
Presbyterian Medical Center Department of Radiology
유은애 ( Yoo Eun-Ae )
Presbyterian Medical Center Department of Radiology
Abstract
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.
키워드
Leigh Syndrome; Wernicke's Encephalopathy; Mitochondria
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