A Comparative Study of Clinical Features and Outcomes in Children With Acute Fulminant Cerebral Edema and Acute Necrotizing Encephalopathy.

BACKGROUND

To compare clinical characteristics, laboratory parameters, and prognosis between children with acute fulminant cerebral edema (AFCE) and acute necrotizing encephalopathy (ANE).

METHODS

A single-center retrospective study was conducted between June 2021 and June 2023. Demographics, etiology, complications, laboratory markers, and outcomes were compared. Survival was estimated by Kaplan-Meier analysis. Receiver operating characteristic curves assessed biomarkers for differentiating AFCE from ANE.

RESULTS

A total of 36 children (13 AFCE, 23 ANE) were included. Both conditions were associated with influenza and severe acute respiratory syndrome coronavirus 2, with no age/sex differences. AFCE patients had significantly higher incidences of multiple organ dysfunction syndrome (92.3% vs 34.8%, P = 0.001), disseminated intravascular coagulation (92.3% vs 30.4%, P < 0.001), and brain herniation (92.3% vs 34.8%, P = 0.001). Procalcitonin and interleukin-6 were significantly elevated in AFCE patients. Prognosis was substantially worse in AFCE: 7-day mortality (76.9% vs 30.4%, P = 0.014), 30-day mortality (84.6% vs 47.8%, P = 0.039), 2-year mortality (92.3% vs 52.2%, P = 0.025), and worse modified Rankin Scale distribution (P = 0.034). In this exploratory analysis, procalcitonin showed the highest discriminative ability, with an area under the curve of 0.818 (95% confidence interval: 0.659-0.978). A procalcitonin cutoff of 38.89 ng/mL yielded a sensitivity of 76.9% and a specificity of 81.8% for identifying AFCE.

CONCLUSIONS

These exploratory findings suggest that AFCE may represent a more severe phenotypic presentation than ANE within the infection-triggered encephalopathy syndromes spectrum, characterized by hyperinflammation, multi-organ injury, and poor outcomes. However, given the small sample size and lack of adjustment for multiple comparisons, the results should be interpreted as hypothesis-generating and require prospective validation.