Acute Necrotizing Encephalopathy in Korean Infants and Children: Imaging Findings and Diverse Clinical Outcome

Abstract

ObjectiveThe 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 MethodsAcute 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.ResultsThis 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).ConclusionAcute 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.

Acute Necrotizing Encephalopathy in a Child during the 2009 Influenza A(H1N1) Pandemia: MR Imaging in Diagnosis and Follow-Up

“…8 Sporadic cases have been documented worldwide. 2 To our knowledge, this is the first case of ANE reported in Europe during the 2009 influenza A(H1N1) pandemia and the only one resulting in patient survival.…”

“…The importance of this imaging technique, eventually associated with DTI, should be advocated as well in the follow-up of these patients to guide the clinical approach and predict the final outcome. 3,8 …”

Acute necrotizing encephalopathy

“…1,2 The presence of hemorrhage and localized tissue loss on MRI may suggest a poor prognosis. 9 Reversion of imaging changes to as normal as possible usually suggests a good prognosis. 9 Okumura et al 10 concluded that administration of steroid within 24 hours after the onset was related to better outcome of children with ANEC without brainstem lesions.…”

Efficacy of Pulse Steroid Therapy in Acute Viral Encephalopathy (Case Report)

“…Acute viral encephalopathy is often associated with influenza A infection but also has been described in human herpes virus 6 (HHV6), herpes simplex virus (HSV), mycoplasma and measles [2][3][4][5][6][7]. The pathogenesis of viral encephalopathy is not entirely explained but recent studies showed that high levels of cytokines (IL-6, TNF-ɑ), nitrogen oxides (NOx) and histiocytosis in some organs were detected as the cause of inflammation [8,9].…”