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Journal of the Korean Pediatric Society 1992;35(3):395-398.
Published online March 15, 1992.
Subacute Necrotizing Encephalomyelopathy (=Leight's Disease)
So Young Kim, Heung Ki Min, Byung Churl Lee
Department of Pediatrics, Catholic University Medical College, Seoul, Korea
아급성 괴사성 뇌척수병증 1례
김소영, 민홍기, 이병철
가톨릭의과대학 소아과학교실
Abstract
Subacute necrotizing encephalomyelopathy or Leigh's disease is a familial neurodegenerative disorder characterized by focal bilateral and usually symmetric lesions of both gray and white matter in the brain and the spinal cord. The lesions are strikingly similar to those of thiamine deficiency encephalopathy. The diagnosis of subacute necrotizing encephalomyelopathy was usually made by typical clinical manifestation and typical bran CT and MRI findings and also brain biopsy. We have experienced a case of subactur necrotizing encephalomyelopathy. The patient, an 8 months old boy, showd failure to weight gain, weakness, irregular respiration delayed motor development, sudden apneas, flaccidity, ataxia and seizure. Brain CT findings revealed bilateral symmetrical hyperintensive basal ganglias especially both putamen area on proton and T2WI and gyral hyperintensities in both cerebral hemispheres.
Key Words: Subacute Necrotizing Encepholmyelopathy


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