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Early High-Dose Methylprednisolone Therapy Is Associated with Better Outcomes in Children with Acute Necrotizing
Han-Pi Chang1,2, Shao-Hsuan Hsia1,2, Jainn-Jim Lin1,2
1Division of Pediatric Critical Care Medicine, Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan 33305, Taiwan.
Insights
Early pulse steroid therapy for acute necrotizing encephalopathy (ANE) may improve outcomes. Prompt treatment within 24 hours of symptom onset is crucial for better neurologic recovery in ANE patients.
Area of Science:
- Pediatric Neurology
- Critical Care Medicine
- Neuroinflammation
Background:
- Acute necrotizing encephalopathy (ANE) is a severe neurologic condition with high mortality (up to 40%) and significant long-term disability.
- Current treatments, including steroids and immunoglobulin, have controversial efficacy for ANE.
- Understanding factors influencing ANE outcomes is critical for developing effective therapeutic strategies.
Purpose of the Study:
- To evaluate the effectiveness of pulse steroid therapy and intravenous immunoglobulin in treating acute necrotizing encephalopathy (ANE).
- To investigate the impact of early treatment initiation on neurologic outcomes in ANE patients.
- To identify prognostic factors associated with mortality and neurologic deficits in ANE.
Main Methods:
- Retrospective review of medical records for 26 pediatric patients diagnosed with ANE.
- Patients were analyzed based on the presence or absence of brainstem involvement.
- Treatment protocols included pulse steroid therapy (methylprednisolone) and intravenous immunoglobulin, with analysis of treatment timing relative to symptom onset.
Main Results:
- Overall mortality rate was 42.3%; non-survivors had higher initial lactate and ferritin levels, and increased need for inotropic agents, particularly with brainstem involvement.
- Early pulse steroid therapy (within 24 hours of ANE onset) was significantly associated with better outcomes (p=0.039).
- Patients without brainstem involvement receiving early pulse methylprednisolone showed good neurologic outcomes in 87.5% of cases.
Conclusions:
- Administering pulse steroid therapy within 24 hours of acute necrotizing encephalopathy (ANE) onset may correlate with improved neurologic prognosis.
- Early intervention with pulse steroid therapy appears particularly beneficial for patients without brainstem involvement.
- Further research is required to establish definitive treatment guidelines for this critical neurologic condition.
Background:
The neurologic outcomes of acute necrotizing encephalopathy (ANE) are very poor, with a mortality rate of up to 40% and fewer than 10% of patients surviving without neurologic deficits. Steroid and immunoglobulin treatments have been the most commonly used options for ANE, but their therapeutic efficacy is still controversial.
Method:
We retrospectively reviewed the medical records of 26 children diagnosed with ANE. We also divided these patients into two groups: 21 patients with brainstem involvement and 8 patients without brainstem involvement. Pulse steroid therapy (methylprednisolone at 30 mg/kg/day for 3 days) and intravenous immunoglobulin (2 g/kg for 2-5 days) were administered to treat ANE.
Results:
The overall mortality rate was 42.3%, and patients who did not survive had significantly higher initial lactate and serum ferritin levels, as well as higher rates of inotropic agent use with brainstem involvement. There were no significant differences in the outcomes of pulse steroid therapy or pulse steroid plus immunoglobulin between survivors and non-survivors. When analyzing the time between symptom onset and usage of pulse steroid therapy, pulse steroid therapy used within 24 h after the onset of ANE resulted in significantly better outcomes (p = 0.039). In patients with brainstem involvement, the outcome was not correlated with pulse steroid therapy, early pulse steroid therapy, or pulse steroid therapy combined with immunoglobulin. All patients without brainstem involvement received "early pulse methylprednisolone" therapy, and 87.5% (7/8) of these patients had a good neurologic outcome.
Conclusion:
Pulse steroid therapy (methylprednisolone at 30 mg/kg/day for 3 days) administered within 24 h after the onset of ANE may be correlated with a good prognosis. Further studies are needed to establish a consensus guideline for this fulminant disease.
