Acute necrotizing encephalopathy in Korean infants and children: imaging findings and diverse clinical outcome

Korean J Radiol. 2004 Jul-Sep;5(3):171-7. doi: 10.3348/kjr.2004.5.3.171.


Objective: The purpose of our study was to describe acute necrotizing encephalopathy in Korean infants and children, and we sought to evaluate the prognostic factors.

Materials and methods: Acute necrotizing encephalopathy was diagnosed in 14 Korean infants and children. We retrospectively analyzed the neuroimaging findings including the follow-up changes. The clinical course of the disease was graded, and we evaluated prognostic factors including age, serum level of the aminotransferase, hemorrhage, and localized atrophy of the brain.

Result: This encephalopathy predominantly affected the bilateral thalami (n = 14), pons (n = 12), and midbrain (n = 10) in a symmetrical pattern. Hemorrhage was observed in eight patients (57%). On the follow-up images (n = 12), the brain lesions were reduced in extent for all patients, and generalized atrophy was seen in six patients. Localized tissue loss was observed in five patients and a complete resolution occurred for one patient. All the patients survived and two recovered completely; mild (n = 6) to severe (n = 6) neurological deficits persisted in the remaining 12 patients. The significant prognostic factors identified in this study were the presence of hemorrhage (p = 0.009) and localized atrophy (p = 0.015).

Conclusion: Acute necrotizing encephalopathy in Korean patients showed the characteristic patterns of the post-infectious encephalopathy as described in the literature. The high survival rate and the relatively favorable clinical course observed for the present study suggest a more diverse spectrum of disease severity than was previously described. The presence of hemorrhage and localized tissue loss on MR images may suggest a poor prognosis.

MeSH terms

  • Brain / pathology
  • Child
  • Child, Preschool
  • Female
  • Humans
  • Infant
  • Korea
  • Leukoencephalitis, Acute Hemorrhagic / complications
  • Leukoencephalitis, Acute Hemorrhagic / pathology*
  • Magnetic Resonance Imaging*
  • Male
  • Prognosis
  • Retrospective Studies