Clinical Practice Guidelines: Acute Necrotizing Encephalopathy

CLINICAL PRACTICE GUIDELINES (FULL LIST)

ACUTE NECROTIZING ENCEPHALOPATHY CPG

Acute necrotizing encephalopathy (ANE) is a rare and severe form of acute encephalopathy, usually preceded by a virus-associated (commonly influenza) febrile illness and characterized by rapid clinical deterioration and high risk of morbidity and mortality. Given severity and rapid progression, early identification and timely treatment is imperative. Inclusion: Patients presenting to the pediatric emergency department with known or suspected viral infection and acute encephalopathy (as determine by clinical assessment OR GCS < 14)

Authors:

  • Kristina Betters (PICU Medical Director)
  • Michael Wolf (Neurocritical Care)
  • Lindsay Pagano (ICU Neurology)
  • Asha Sarma (Neuroradiology)
  • Ritu Banarjee (ID)
  • Brent Graham (Rheumatology)
  • Bryce Schuler (Genetics)
  • Kathy Jabs (Nephrology)
  • Barbara Solomon (Emergency Medicine)
  • Nick Jones (Emergency Medicine)
  • James Antoon (Hospital Medicine)

References:

  1. Li K, Zhang T, Liu G, Jin P, Wang Y, Wang L, Xu M, Liu C, Liu Y, Zhou T, Xu Y, Yang Y, Fang B, Yang X, Liu C, Qian S. Plasma exchange therapy for acute necrotizing encephalopathy of childhood. Pediatr Investig. 2021 Jun 18;5(2):99-105. doi: 10.1002/ped4.12280. PMID: 34179705; PMCID: PMC8212728.
  2. Azmi A, Zainal Abidin AS. Good Outcome With Respect to Acute Necrotizing Encephalitis in Children Associated With Post-infectious SARS-CoV-2. Cureus. 2023 Aug 9;15(8):e43198. doi: 10.7759/cureus.43198. PMID: 37692682; PMCID: PMC10486289.
  3. Sarigecili E, Ucar HK, Havali C, Cansu A, Aydin K. Acute necrotizing encephalopathy associated with RANBP2 mutation: value of MRI findings for diagnosis and intervention. Acta Neurol Belg. 2023 Apr;123(2):571-582. doi: 10.1007/s13760-022-02166-x. Epub 2022 Dec 26. PMID: 36572756; PMCID: PMC9792159.
  4. Uyeki TM. Pediatric Influenza-Associated Acute Necrotizing Encephalopathy—Gaps Need to Be Addressed. JAMA. 2025;334(8):677–679. doi:10.1001/jama.2025.13003
  5. Fazal A, Reinhart K, Huang S, et al. Reports of Encephalopathy Among Children with Influenza-Associated Mortality — United States, 2010–11 Through 2024–25 Influenza Seasons. MMWR Morb Mortal Wkly Rep 2025;74:91–95. DOI: http://dx.doi.org/10.15585/mmwr.mm7406a3
  6. Influenza-Associated Acute Necrotizing Encephalopathy (IA-ANE) Working Group. Influenza-Associated Acute Necrotizing Encephalopathy in US Children. JAMA. 2025;334(8):692–701. doi:10.1001/jama.2025.11534
  7. Paktinat M, Hessami K, Inaloo S, Nemati H, Katibeh P, Nejabat M, Darabi MH, Bereshneh AH. Case Report of RANBP2 Mutation and Familial Acute Necrotizing Encephalopathy. Int J Pediatr. 2021 Mar 13;2021:6695119. doi: 10.1155/2021/6695119. PMID: 33777149; PMCID: PMC7981175.