Acute necrotizing encephalopathy (ANE) is a rare condition associated with rapid progression to coma and high incidence of morbidity and mortality.
Methods:
Clinical, electroencephalographic (EEG), and brain magnetic resonance imaging (MRI) characteristics and immunomodulatory therapy timing were retrospectively analyzed in children with ANE. ANE severity scores (ANE-SS) and MRI scores were also assessed. The associations of patient characteristics with 6-month modified Rankin scale (mRS) and length of hospitalization were determined using either univariate linear regression or one-way analysis of variance.
Results:
7 children were retrospectively evaluated. Normal EEG sleep spindles (P = .024) and early treatment (R2 = .57, P = .030) were associated with improved outcomes (ie, decreased mRS). Higher ANE-SS (R2 = .79, P = .011), higher age (R2 = .62, P = .038), and presence of brainstem lesions (P = .015) were associated with longer length of hospitalization. Other patient characteristics were not significantly associated with mRS or length of hospitalization.
Conclusion:
Early immunomodulatory therapy and normal sleep spindles are associated with better functional outcome in children with ANE.
MizutaRIzumiH, et al.A case of Reye’s syndrome with elevation in influenza A, CF antibody. Jap J Pediatr. 1979;32:2144–2149.
2.
MizuguchiM. Acute necrotizing encephalopathy of childhood: a novel form of acute encephalopathy prevalent in Japan and Taiwan. Brain Dev. 1997;19(2):81–92.
3.
KimJHKimIO, et al.Acute necrotizing encephalopathy in Korean infants and children: imaging findings and diverse clinical outcome. Korean J Radiol. 2004;5(3):171–177.
4.
NeilsonDEAdamsMDOrrCM, et al.Infection-triggered familial or recurrent cases of acute necrotizing encephalopathy caused by mutations in a component of the nuclear pore, RANBP2. Am J Hum Genet. 2009;84(1):44–51.
5.
OkumuraAMizuguchiM, et al.Outcome of acute necrotizing encephalopathy in relation to treatment with corticosteroids and gammaglobulin. Brain Dev. 2009;31(3):221–227.
6.
KohJCMurugasuAKrishnappaJThomasT. Favorable outcomes with early interleukin 6 receptor blockade in severe acute necrotizing encephalopathy of childhood. Pediatr Neurol. 2019;98:80–84.
7.
MizuguchiMAbeJ, et al.Acute necrotising encephalopathy of childhood: a new syndrome presenting with multifocal, symmetric brain lesions. J Neurol Neurosurg Psychiatry. 1995;58(5):555–561.
8.
WongAMSimonEMZimmermanRAWangHSTOhCHNgSH. Acute necrotizing encephalopathy of childhood: correlation of MR findings and clinical outcome. AJNR Am J Neuroradiol. 2006;27(9):1919–1923.
9.
MastroyianniSDGionnisD, et al.Acute necrotizing encephalopathy of childhood in non-Asian patients: report of three cases and literature review. J Child Neurol. 2006;21(10):872–879.
10.
AlbayramSBilgiZ, et al.Diffusion-weighted MR imaging findings of acute necrotizing encephalopathy. Am J Neuroradiol. 2004;25(5):792–797.
11.
OkumuraAKidokoroH, et al.The mildest form of acute necrotizing encephalopathy associated with influenza A. Neuropediatrics. 2006;37(4):261–263.
12.
YamamotoHOkamuraATasumeJKojimaSMizuguchiM. A severity score for acute necrotizing encephalopathy. Brain Dev. 2015;37:322–327.
13.
Van SwietenJCKoudstaalPJVisserMCSchoutenHJvan GijnJV. Interobserver agreement for the assessment of handicap in stroke patients. Stroke. 1998;19(5):604–607.
KellerSSGerdesJSMohammadiS, et al.Volume estimation of the thalamus using FreeSurfer and stereology: consistency between methods. Neuroinformatics. 2012;10(4):341–350.
SkeltonBWHollingsheadMCSleddATPhillipsCDCastilloM. Acute necrotizing encephalopathy of childhood: typical findings in an atypical disease. Pediatr Radiol. 2008;38(7):810–813.
20.
YonJBRemigioCMilliganTDelineC. Acute necrotizing encephalopathy in a child with H1N1 influenza infection. Pediatr Radiol. 2010;40(2):200–205.
21.
MartinAReadeEP. Acute necrotizing encephalopathy progressing to brain death in a pediatric patient with novel influenza A (H1N1) infection. Clin Infect Dis. 2010;50(8):e50–e52.
22.
OkamuraAMizuguchiMAibaHTanabeTTsujiTOhnoA. Delirious behavior in children with acute necrotizing encephalopathy. Brain Dev. 2009;31(8):597–599.
HuangSMChenCCChiuPCChengMFLaiPHHsiehKS. Acute necrotizing encephalopathy of childhood associated with influenza type B virus infection in a 3-year-old girl. J Child Neurol. 2004;19(1):64–67.
25.
BergaminoLCapraVBiancheriR, et al.Immunomodulatory therapy in recurrent acute necrotizing encephalopathy ANE1: is it useful?Brain Dev. 2012;34(5):384–391.
26.
ManaraRFranzoiMCogoPBattistellaPA. Acute necrotizing encephalopathy: combined therapy and favorable outcome in a new case. Child’s Nerv Syst. 2006;22(10):1231–1236.
27.
BassukAGBurrowesDMMcRaeW. Acute necrotizing encephalopathy of childhood with radiographic progression over 10 hours. Neurology. 2003;60(9):1552–1553.
28.
MunakataMKatoRYokoyamaH, et al.Combined therapy with hypothermia and anticytokine agents in influenza A encephalopathy. Brain Dev. 2000;22(6):373–377.
29.
SteriadeM. The corticothalamic system in sleep. Front Biosci. 2003;8:d878–d899.
30.
Ducharme-CrevierPress CAKurzJEMillsMGGoldsteinJLWainwrightMS. Early presence of sleep spindles on electroencephalography is associated with good outcome after pediatric cardiac arrest. Pediatr Crit Care Med. 2017;18(5):452–460.
31.
BassukAGBurrowesDMMcRaeW. Acute necrotizing encephalopathy of childhood with radiographic progression over 10 hours. Neurology. 2003;60(9).
32.
ChowCKMaCKLM. Presentation and outcome of acute necrotizing encephalopathy in childhood: a 10-year single-center retrospective study from Hong Kong. J Child Neurol. 2020[Epub ahead of print].
33.
MarcoEJAndersonJENeilsonDEStroberJB. Acute necrotizing encephalopathy in 3 brothers. Pediatrics. 2010;125(3):e693–e698.
34.
LeeJHLeeMLeeJ. Recurrent acute necrotizing encephalopathy in a Korean child: the first non-Caucasian case. J Child Neurol. 2012;27(10):1343–1347.
