Abstract
Two autopsy cases of infantile subacute necrotizing encephalopathy were reported. The first case was a 7 month old boy, who showed psychomotor retardation at the age of 4 months, developed infantile spasms with hypsarrhythmia, and died suddenly with unexplained fever and dyspnea. The postmortem examination of the brain revealed symmetrical necrosis of bilateral lenticular and caudate nuclei. Sharply demarcated and symmetrical foci of necrosis were noted in the tegmentum of the midbrain and the pons. Histologically, the lesions were characterized by spongy degeneration, cavity formation, and proliferation of small blood vessels, being consistent with Leigh's subacute necrotizing encephalomyelopathy. The mammillary body was intact, though a necrotizing lesion was found around the third ventricle.
