All issues > Volume 35(3); 1992

Case Report

J Korean Pediatr Soc. 1992;35(3):395-398. Published online March 15, 1992.

Subacute Necrotizing Encephalomyelopathy (=Leight's Disease)

So Young SY Kim¹, Heung Ki HK Min¹, Byung Churl BC Lee¹

¹Department of Pediatrics, Catholic University Medical College, Seoul, Korea

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.

Keywords :Subacute Necrotizing Encepholmyelopathy