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Two Cases of Leigh Disease

Journal of the Korean Pediatric Society 1998;41(3):405-409.
Published online March 15, 1998.
Two Cases of Leigh Disease
Seong Hun Kim, Soo En Park, Ju Seok Lee, Sang Ook Nam, Yeong Tak Lim
Department of Pediatrics, P usan National University College of M edicine, Pusan, Korea
Leigh Disease 2예
김성훈, 박수은, 이주석, 남상욱, 임영탁
부산대학교 의과대학 소아과학교실
Seong Hun Kim, Email: 1
Leigh Disease, or subacute necrotizing encephalopathy(SNE), is a degenerative disorder characterized by lesions of the gray and white matter in the bran and spinal cord. The pathogenesis was known as mitochondrial enzyme defect of the respiratory chain system. We experienced 2 cases of Leigh disease. The first case, a seven-month old girl who was presented with weak respiration and failure to thrive, showed lactic acidemia and increased lactic acid in CSF fluid, high signal intensity in the bilateral putamen and head of caudate of nucleus at T2 weighted MR imaging. The second case, a 3-year-old girl with ataxic gait and bilateral ptosis also showed lactic acidemia, increased lactic acid in CSF fluid and high signal intensity in the bilateral basal ganglia. Respiratory difficuly developed in both cases and died within 1 month after visiting our hospital. The diagnosis was made by lactic acidosis and specific MRI finding. We report these cases with a brief review of its related literature.
Key Words: Leigh disease, Subacute necrotizing encephalopathy

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