U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Acute necrotizing encephalitis

MedGen UID:
83264
Concept ID:
C0338418
Disease or Syndrome
Synonyms: Acute Necrotizing Encephalitides; Acute Necrotizing Encephalitis; Encephalitides, Acute Necrotizing; Encephalitis, Acute Necrotizing; Necrotizing Encephalitides, Acute; Necrotizing Encephalitis, Acute
SNOMED CT: Acute necrotizing encephalitis (111897007); Acute necrotizing viral encephalitis (111897007)
 
Monarch Initiative: MONDO:0003336

Definition

A fulminant and often fatal demyelinating disease of the brain which primarily affects young adults and children. Clinical features include the rapid onset of weakness, seizures, and coma. It may follow a viral illness or mycoplasma pneumoniae infections but in most instances there is no precipitating event. Pathologic examination reveals marked perivascular demyelination and necrosis of white matter with microhemorrhages. (Adams et al., Principles of Neurology, 6th ed, pp924-5) [from MONDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute necrotizing encephalitis

Professional guidelines

PubMed

Appavu B, Foldes S, Fox J, Shetty S, Oh A, Bassal F, Marku I, Mangum T, Boerwinkle V, Neilson D, Kruer M
J Child Neurol 2021 Jun;36(7):517-524. Epub 2021 Jan 4 doi: 10.1177/0883073820984063. PMID: 33393838
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
Aneja S, Sharma S
Indian J Pediatr 2019 Jan;86(1):70-75. Epub 2018 Sep 19 doi: 10.1007/s12098-018-2775-0. PMID: 30232787

Recent clinical studies

Etiology

Goetz V, Yang DD, Abid H, Roux CJ, Levy R, Kossorotoff M, Desguerre I, Angoulvant F, Aubart M
Eur J Pediatr 2023 Jun;182(6):2615-2624. Epub 2023 Mar 22 doi: 10.1007/s00431-023-04901-9. PMID: 36947244
Nersesjan V, Amiri M, Lebech AM, Roed C, Mens H, Russell L, Fonsmark L, Berntsen M, Sigurdsson ST, Carlsen J, Langkilde AR, Martens P, Lund EL, Hansen K, Jespersen B, Folke MN, Meden P, Hejl AM, Wamberg C, Benros ME, Kondziella D
J Neurol 2021 Sep;268(9):3086-3104. Epub 2021 Jan 13 doi: 10.1007/s00415-020-10380-x. PMID: 33438076Free PMC Article
Hu MH, Wu CT, Hsia SH, Hung PC, Huang GS
J Child Neurol 2020 Oct;35(11):724-730. Epub 2020 Jun 8 doi: 10.1177/0883073820930557. PMID: 32507002
Dadak M, Pul R, Lanfermann H, Hartmann H, Hehr U, Donnerstag F, Michels D, Tryc AB
Clin Neuroradiol 2020 Jun;30(2):243-249. Epub 2019 Jan 4 doi: 10.1007/s00062-018-0756-3. PMID: 30610262
Finelli PF, Foxman EB
Neuroradiol J 2014 Jun;27(3):280-7. Epub 2014 Jun 17 doi: 10.15274/NRJ-2014-10036. PMID: 24976194Free PMC Article

Diagnosis

Goetz V, Yang DD, Abid H, Roux CJ, Levy R, Kossorotoff M, Desguerre I, Angoulvant F, Aubart M
Eur J Pediatr 2023 Jun;182(6):2615-2624. Epub 2023 Mar 22 doi: 10.1007/s00431-023-04901-9. PMID: 36947244
Zamani R, Pouremamali R, Rezaei N
Rev Neurosci 2022 Jun 27;33(4):397-412. Epub 2021 Sep 17 doi: 10.1515/revneuro-2021-0082. PMID: 34536341
Hu MH, Wu CT, Hsia SH, Hung PC, Huang GS
J Child Neurol 2020 Oct;35(11):724-730. Epub 2020 Jun 8 doi: 10.1177/0883073820930557. PMID: 32507002
Bailey HE
Neurodiagn J 2020 Mar;60(1):41-49. doi: 10.1080/21646821.2020.1725864. PMID: 32101112
Br Med J 1967 Sep 30;3(5569):812-3. PMID: 4292085Free PMC Article

Therapy

Pérez-Dueñas B, Ormazábal A, Toma C, Torrico B, Cormand B, Serrano M, Sierra C, De Grandis E, Marfa MP, García-Cazorla A, Campistol J, Pascual JM, Artuch R
Arch Neurol 2011 May;68(5):615-21. doi: 10.1001/archneurol.2011.80. PMID: 21555636
Lee KH, McKie VC, Sekul EA, Adams RJ, Nichols FT
J Pediatr Hematol Oncol 2002 Oct;24(7):585-8. doi: 10.1097/00043426-200210000-00021. PMID: 12368703
Kennedy PG, Adams JH, Graham DI, Clements GB
Neuropathol Appl Neurobiol 1988 Sep-Oct;14(5):395-415. doi: 10.1111/j.1365-2990.1988.tb01141.x. PMID: 3200368
Fearnside MR, Grant JM
J Neurosurg 1972 Apr;36(4):499-502. doi: 10.3171/jns.1972.36.4.0499. PMID: 4335254
Br Med J 1967 Sep 30;3(5569):812-3. PMID: 4292085Free PMC Article

Prognosis

Goetz V, Yang DD, Abid H, Roux CJ, Levy R, Kossorotoff M, Desguerre I, Angoulvant F, Aubart M
Eur J Pediatr 2023 Jun;182(6):2615-2624. Epub 2023 Mar 22 doi: 10.1007/s00431-023-04901-9. PMID: 36947244
Yang X, Wei M, Chu S, Zhang Y
BMC Neurol 2022 Sep 1;22(1):329. doi: 10.1186/s12883-022-02844-6. PMID: 36050642Free PMC Article
Nersesjan V, Amiri M, Lebech AM, Roed C, Mens H, Russell L, Fonsmark L, Berntsen M, Sigurdsson ST, Carlsen J, Langkilde AR, Martens P, Lund EL, Hansen K, Jespersen B, Folke MN, Meden P, Hejl AM, Wamberg C, Benros ME, Kondziella D
J Neurol 2021 Sep;268(9):3086-3104. Epub 2021 Jan 13 doi: 10.1007/s00415-020-10380-x. PMID: 33438076Free PMC Article
Hu MH, Wu CT, Hsia SH, Hung PC, Huang GS
J Child Neurol 2020 Oct;35(11):724-730. Epub 2020 Jun 8 doi: 10.1177/0883073820930557. PMID: 32507002
Wanigasinghe J, Jayathunge T, Ratnayake PD
Neurol India 2017 Jul-Aug;65(4):867-868. doi: 10.4103/neuroindia.NI_1125_16. PMID: 28681764

Clinical prediction guides

Goetz V, Yang DD, Abid H, Roux CJ, Levy R, Kossorotoff M, Desguerre I, Angoulvant F, Aubart M
Eur J Pediatr 2023 Jun;182(6):2615-2624. Epub 2023 Mar 22 doi: 10.1007/s00431-023-04901-9. PMID: 36947244
Garg RK, Paliwal VK, Malhotra HS, Sharma PK
Neurol India 2021 Mar-Apr;69(2):260-271. doi: 10.4103/0028-3886.314531. PMID: 33904434
Nersesjan V, Amiri M, Lebech AM, Roed C, Mens H, Russell L, Fonsmark L, Berntsen M, Sigurdsson ST, Carlsen J, Langkilde AR, Martens P, Lund EL, Hansen K, Jespersen B, Folke MN, Meden P, Hejl AM, Wamberg C, Benros ME, Kondziella D
J Neurol 2021 Sep;268(9):3086-3104. Epub 2021 Jan 13 doi: 10.1007/s00415-020-10380-x. PMID: 33438076Free PMC Article
Hu MH, Wu CT, Hsia SH, Hung PC, Huang GS
J Child Neurol 2020 Oct;35(11):724-730. Epub 2020 Jun 8 doi: 10.1177/0883073820930557. PMID: 32507002
Pérez-Dueñas B, Ormazábal A, Toma C, Torrico B, Cormand B, Serrano M, Sierra C, De Grandis E, Marfa MP, García-Cazorla A, Campistol J, Pascual JM, Artuch R
Arch Neurol 2011 May;68(5):615-21. doi: 10.1001/archneurol.2011.80. PMID: 21555636

Recent systematic reviews

Lee IT, Lin PJ, Yen HH
Int J Infect Dis 2024 Jan;138:29-37. Epub 2023 Nov 7 doi: 10.1016/j.ijid.2023.11.006. PMID: 37944584
Zamani R, Pouremamali R, Rezaei N
Rev Neurosci 2022 Jun 27;33(4):397-412. Epub 2021 Sep 17 doi: 10.1515/revneuro-2021-0082. PMID: 34536341

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Consumer resources

    Recent activity

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...