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Acute necrotizing encephalopathy of childhood

MedGen UID:
1641610
Concept ID:
C4706387
Disease or Syndrome
Synonyms: acute necrotizing encephalopathy of childhood; ANEC; Isolated acute necrotizing encephalopathy; isolated acute necrotizing encephalopathy; Isolated ANE; isolated ANE
SNOMED CT: Isolated acute necrotizing encephalopathy (763310000); Acute necrotizing encephalopathy of childhood (763310000)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0016991
Orphanet: ORPHA263524

Definition

A rare neurologic disease with characteristics of rapid onset of seizures, an altered state of consciousness, neurologic decline, and variable degrees of hepatic dysfunction following a respiratory or gastrointestinal infection (e.g. mycoplasma, influenza virus) in a previously healthy child. Brain MRI of patients reveals bilateral, multiple, symmetrical lesions predominantly observed in thalami and brainstem, but also in periventricular white matter and cerebellum in some cases. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute necrotizing encephalopathy of childhood

Professional guidelines

PubMed

Mizuguchi M, Ichiyama T, Imataka G, Okumura A, Goto T, Sakuma H, Takanashi JI, Murayama K, Yamagata T, Yamanouchi H, Fukuda T, Maegaki Y
Brain Dev 2021 Jan;43(1):2-31. Epub 2020 Aug 20 doi: 10.1016/j.braindev.2020.08.001. PMID: 32829972
McGlennan C, Ganesan V
Dev Med Child Neurol 2008 Jul;50(7):537-40. doi: 10.1111/j.1469-8749.2008.03012.x. PMID: 18611205
Packer RJ, Meadows AT, Rorke LB, Goldwein JL, D'Angio G
Med Pediatr Oncol 1987;15(5):241-53. doi: 10.1002/mpo.2950150505. PMID: 3309606

Recent clinical studies

Etiology

Li K, Wang L, Liu G, Jin P, Fan C, Zhang T, Xu M, Zhou T, Liu C, Wang Y, Liu C, Qian S
Chin Med J (Engl) 2023 Mar 20;136(6):743-745. doi: 10.1097/CM9.0000000000002271. PMID: 36806243Free PMC Article
Ho JHY, Lee CYM, Chiong YK, Aoyama R, Fan LJ, Tan AHS, Han VX
Pediatr Neurol 2023 Feb;139:65-69. Epub 2022 Nov 25 doi: 10.1016/j.pediatrneurol.2022.11.010. PMID: 36529001Free PMC Article
Chatur N, Yea C, Ertl-Wagner B, Yeh EA
Dev Med Child Neurol 2022 Aug;64(8):1008-1016. Epub 2022 Feb 2 doi: 10.1111/dmcn.15165. PMID: 35108406
Chow CK, Ma CKL
J Child Neurol 2020 Sep;35(10):674-680. Epub 2020 Jun 4 doi: 10.1177/0883073820927915. PMID: 32493103
Wong AM, Simon EM, Zimmerman RA, Wang HS, Toh CH, Ng SH
AJNR Am J Neuroradiol 2006 Oct;27(9):1919-23. PMID: 17032866Free PMC Article

Diagnosis

Aksoy E, Öztoprak Ü, Çelik H, Özdemir FMA, Özkan M, Kayılıoğlu H, Danış A, Kucur Ö, Kesici S, Uysal Yazıcı M, Azapağası E, Taşcı Yıldız Y, Ceylan N, Şenel S, Yüksel D
Turk J Med Sci 2021 Apr 30;51(2):706-715. doi: 10.3906/sag-2102-47. PMID: 33754655Free PMC Article
Biswas A, Varman M, Gunturi A, Yoganathan S, Gibikote S
Neurology 2018 Jan 9;90(2):e177-e178. doi: 10.1212/WNL.0000000000004800. PMID: 29311376
Weng WC, Peng SS, Lee WT
J Child Neurol 2010 Dec;25(12):1539-41. Epub 2010 Sep 7 doi: 10.1177/0883073810374877. PMID: 20823028
Wong AM, Simon EM, Zimmerman RA, Wang HS, Toh CH, Ng SH
AJNR Am J Neuroradiol 2006 Oct;27(9):1919-23. PMID: 17032866Free PMC Article
Mastroyianni SD, Gionnis D, Voudris K, Skardoutsou A, Mizuguchi M
J Child Neurol 2006 Oct;21(10):872-9. doi: 10.1177/08830738060210101401. PMID: 17005104

Therapy

Hosie PH, Lim C, Scott TRD, Cardamone M, Farrar MA, Frith C, Andrews PI, Pinner J, Pillai S
Brain Dev 2023 Aug;45(7):401-407. Epub 2023 Mar 24 doi: 10.1016/j.braindev.2023.03.002. PMID: 36967317
Li K, Wang L, Liu G, Jin P, Fan C, Zhang T, Xu M, Zhou T, Liu C, Wang Y, Liu C, Qian S
Chin Med J (Engl) 2023 Mar 20;136(6):743-745. doi: 10.1097/CM9.0000000000002271. PMID: 36806243Free PMC Article
Koh JC, Murugasu A, Krishnappa J, Thomas T
Pediatr Neurol 2019 Sep;98:80-84. Epub 2019 Apr 25 doi: 10.1016/j.pediatrneurol.2019.04.009. PMID: 31201070

Prognosis

Ho JHY, Lee CYM, Chiong YK, Aoyama R, Fan LJ, Tan AHS, Han VX
Pediatr Neurol 2023 Feb;139:65-69. Epub 2022 Nov 25 doi: 10.1016/j.pediatrneurol.2022.11.010. PMID: 36529001Free PMC Article
Chatur N, Yea C, Ertl-Wagner B, Yeh EA
Dev Med Child Neurol 2022 Aug;64(8):1008-1016. Epub 2022 Feb 2 doi: 10.1111/dmcn.15165. PMID: 35108406
Chow CK, Ma CKL
J Child Neurol 2020 Sep;35(10):674-680. Epub 2020 Jun 4 doi: 10.1177/0883073820927915. PMID: 32493103
Wong AM, Simon EM, Zimmerman RA, Wang HS, Toh CH, Ng SH
AJNR Am J Neuroradiol 2006 Oct;27(9):1919-23. PMID: 17032866Free PMC Article
Mizuguchi M
Brain Dev 1997 Mar;19(2):81-92. doi: 10.1016/s0387-7604(96)00063-0. PMID: 9105653

Clinical prediction guides

Ho JHY, Lee CYM, Chiong YK, Aoyama R, Fan LJ, Tan AHS, Han VX
Pediatr Neurol 2023 Feb;139:65-69. Epub 2022 Nov 25 doi: 10.1016/j.pediatrneurol.2022.11.010. PMID: 36529001Free PMC Article
Chatur N, Yea C, Ertl-Wagner B, Yeh EA
Dev Med Child Neurol 2022 Aug;64(8):1008-1016. Epub 2022 Feb 2 doi: 10.1111/dmcn.15165. PMID: 35108406
Aksoy E, Öztoprak Ü, Çelik H, Özdemir FMA, Özkan M, Kayılıoğlu H, Danış A, Kucur Ö, Kesici S, Uysal Yazıcı M, Azapağası E, Taşcı Yıldız Y, Ceylan N, Şenel S, Yüksel D
Turk J Med Sci 2021 Apr 30;51(2):706-715. doi: 10.3906/sag-2102-47. PMID: 33754655Free PMC Article
Chow CK, Ma CKL
J Child Neurol 2020 Sep;35(10):674-680. Epub 2020 Jun 4 doi: 10.1177/0883073820927915. PMID: 32493103
Wong AM, Simon EM, Zimmerman RA, Wang HS, Toh CH, Ng SH
AJNR Am J Neuroradiol 2006 Oct;27(9):1919-23. PMID: 17032866Free PMC Article

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