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Acute hemorrhagic leukoencephalitis

MedGen UID:
4937
•Concept ID:
C0014077
•
Disease or Syndrome
Synonyms: Acute Hemorrhagic Encephalomyelitides; Acute Hemorrhagic Encephalomyelitis; Acute Hemorrhagic Leukoencephalitides; Acute Hemorrhagic Leukoencephalitis; Disease, Hurst; Disease, Hurst's; Encephalomyelitides, Acute Hemorrhagic; Encephalomyelitis, Acute Hemorrhagic; Encephalomyelitis, Acute Necrotizing Hemorrhagic; Hemorrhagic Encephalomyelitides, Acute; Hemorrhagic Encephalomyelitis, Acute; Hemorrhagic Leukoencephalitides, Acute; Hemorrhagic Leukoencephalitis, Acute; Hurst Disease; Hurst Syndrome; Hurst's Disease; Hursts Disease; Leukoencephalitides, Acute Hemorrhagic; Leukoencephalitis Acuta Hemorrhagica; Leukoencephalitis, Acute Hemorrhagic; Syndrome, Hurst
SNOMED CT: Acute hemorrhagic leukoencephalitis (72986009); Hurst's disease (72986009); Acute hemorrhagic leukoencephalitis of Weston Hurst (72986009)
 
Monarch Initiative: MONDO:0011716
OMIM®: 606752

Definition

A degenerative brain disorder presenting with acute neurological manifestations. It usually associated with an acute infection or vaccination. It is characterized by brain hemorrhage, vascular necrosis and demyelination. Signs and symptoms include fever, headache, vomiting, convulsions and loss of consciousness. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAcute hemorrhagic leukoencephalitis

Professional guidelines

PubMed

Acute Hemorrhagic Leukoencephalitis (AHLE): A Comprehensive Review on Causes, Symptoms, Link with COVID-19, Diagnosis, and Treatment.
Alromaihi M
Biomed Res Int 2022;2022:6008375. Epub 2022 Nov 15 doi: 10.1155/2022/6008375. PMID: 36425337Free PMC Article
Update on investigation and management of postinfectious encephalitis.
Sonneville R, Klein IF, Wolff M
Curr Opin Neurol 2010 Jun;23(3):300-4. doi: 10.1097/WCO.0b013e32833925ec. PMID: 20442573
Post-infectious encephalitis in adults: diagnosis and management.
Sonneville R, Klein I, de Broucker T, Wolff M
J Infect 2009 May;58(5):321-8. Epub 2009 Apr 14 doi: 10.1016/j.jinf.2009.02.011. PMID: 19368974Free PMC Article

Recent clinical studies

Etiology

De novo RANBP2 variant in a fetal demise case with cerebral intraparenchymal hemorrhage.
Meroni A, Kalantari S, Arossa A, Spinillo A, Melito C, Scatigno AL, Cesari S, Giorgio E, Furione M, Homfray T, Sirchia F
Am J Med Genet A 2023 Jul;191(7):1973-1977. Epub 2023 Apr 27 doi: 10.1002/ajmg.a.63223. PMID: 37102432
Clinical and Radiologic Findings of Acute Necrotizing Encephalopathy in Young Adults.
Vanjare HA, Selvi BT, Karuppusami R, Manesh A, Gunasekaran K, Prabhakar AT, Mannam P, Jasper A
AJNR Am J Neuroradiol 2020 Dec;41(12):2250-2254. Epub 2020 Oct 29 doi: 10.3174/ajnr.A6803. PMID: 33122207Free PMC Article
Neuropsychological outcomes of childhood acute necrotizing encephalopathy.
Williams TA, Brunsdon RK, Burton KLO, Drevensek S, Brady C, Dale RC, Mohammad SS
Brain Dev 2019 Nov;41(10):894-900. Epub 2019 Jul 31 doi: 10.1016/j.braindev.2019.07.007. PMID: 31376945
A severity score for acute necrotizing encephalopathy.
Yamamoto H, Okumura A, Natsume J, Kojima S, Mizuguchi M
Brain Dev 2015 Mar;37(3):322-7. Epub 2014 Jun 12 doi: 10.1016/j.braindev.2014.05.007. PMID: 24931733
The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review.
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923

Diagnosis

Acute necrotizing encephalopathy: A case report.
Jackson EL, Eilbert W, Hathcock AM
Am J Emerg Med 2021 Mar;41:263.e5-263.e7. Epub 2020 Aug 22 doi: 10.1016/j.ajem.2020.08.026. PMID: 32861530
Genetic chameleons: remember the relapsing disorders.
Thomas RH, Thomas NJP
Pract Neurol 2019 Aug;19(4):282-283. Epub 2019 Apr 4 doi: 10.1136/practneurol-2018-002181. PMID: 30948557
Weston-Hurst syndrome with acute hemorrhagic cerebellitis.
Bonduelle T, Stricker J, Minéo JF, Massri A, Guesdon C, Barroso B, Bonnan M
Clin Neurol Neurosurg 2018 Oct;173:118-119. Epub 2018 Aug 11 doi: 10.1016/j.clineuro.2018.08.007. PMID: 30121019
Atypical inflammatory demyelinating lesions and atypical multiple sclerosis.
Ayrignac X, Carra-Dallière C, Labauge P
Rev Neurol (Paris) 2018 Jun;174(6):408-418. Epub 2018 Apr 16 doi: 10.1016/j.neurol.2018.03.007. PMID: 29673573
Acute Hemorrhagic Leucoencephalitis.
Chellathurai A, Ponnusamy S, Periakaruppan A, Gopinathan K, Philson J
Indian J Pediatr 2016 Mar;83(3):276-7. Epub 2015 Jul 23 doi: 10.1007/s12098-015-1840-1. PMID: 26199077

Therapy

Tocilizumab in acute necrotizing encephalopathy (ANE): How much, how soon, and will it improve outcomes beyond survival?
Han VX, Lim M
Eur J Paediatr Neurol 2024 Sep;52:A3-A4. Epub 2024 Sep 4 doi: 10.1016/j.ejpn.2024.08.010. PMID: 39244415
Factors associated with outcomes of severe acute necrotizing encephalopathy: A multicentre experience in Malaysia.
Lee VWM, Khoo TB, Teh CM, Heng HS, Li L, Yusof YLM, Yahaya NA, Dharshini S, Wong SW, Nickson T; ANE Malaysia Outcome Study Group
Dev Med Child Neurol 2023 Sep;65(9):1256-1263. Epub 2023 Feb 7 doi: 10.1111/dmcn.15536. PMID: 36748407
Weston-Hurst syndrome with acute hemorrhagic cerebellitis.
Bonduelle T, Stricker J, Minéo JF, Massri A, Guesdon C, Barroso B, Bonnan M
Clin Neurol Neurosurg 2018 Oct;173:118-119. Epub 2018 Aug 11 doi: 10.1016/j.clineuro.2018.08.007. PMID: 30121019
Influenza-associated encephalopathy in Japan.
Sugaya N
Semin Pediatr Infect Dis 2002 Apr;13(2):79-84. doi: 10.1053/spid.2002.122993. PMID: 12122956
Neurodegenerative disorders and hyperaldosteronism.
Simopoulos AP, Delea CS, Bartter FC
J Pediatr 1971 Oct;79(4):633-41. doi: 10.1016/s0022-3476(71)80312-8. PMID: 4328652

Prognosis

Association of O blood type with the prognosis of acute necrotizing encephalopathy in childhood: A single-center cohort study.
Wang Y, Qian S, Li K, Yang Y, Fan C, Wang L
Brain Dev 2022 Feb;44(2):131-138. Epub 2021 Sep 22 doi: 10.1016/j.braindev.2021.09.004. PMID: 34563416
Atypical inflammatory demyelinating lesions and atypical multiple sclerosis.
Ayrignac X, Carra-Dallière C, Labauge P
Rev Neurol (Paris) 2018 Jun;174(6):408-418. Epub 2018 Apr 16 doi: 10.1016/j.neurol.2018.03.007. PMID: 29673573
Acute Hemorrhagic Leucoencephalitis.
Chellathurai A, Ponnusamy S, Periakaruppan A, Gopinathan K, Philson J
Indian J Pediatr 2016 Mar;83(3):276-7. Epub 2015 Jul 23 doi: 10.1007/s12098-015-1840-1. PMID: 26199077
The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review.
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923
Influenza-associated encephalopathy in Japan.
Sugaya N
Semin Pediatr Infect Dis 2002 Apr;13(2):79-84. doi: 10.1053/spid.2002.122993. PMID: 12122956

Clinical prediction guides

Factors associated with outcomes of severe acute necrotizing encephalopathy: A multicentre experience in Malaysia.
Lee VWM, Khoo TB, Teh CM, Heng HS, Li L, Yusof YLM, Yahaya NA, Dharshini S, Wong SW, Nickson T; ANE Malaysia Outcome Study Group
Dev Med Child Neurol 2023 Sep;65(9):1256-1263. Epub 2023 Feb 7 doi: 10.1111/dmcn.15536. PMID: 36748407
Association of O blood type with the prognosis of acute necrotizing encephalopathy in childhood: A single-center cohort study.
Wang Y, Qian S, Li K, Yang Y, Fan C, Wang L
Brain Dev 2022 Feb;44(2):131-138. Epub 2021 Sep 22 doi: 10.1016/j.braindev.2021.09.004. PMID: 34563416
A severity score for acute necrotizing encephalopathy.
Yamamoto H, Okumura A, Natsume J, Kojima S, Mizuguchi M
Brain Dev 2015 Mar;37(3):322-7. Epub 2014 Jun 12 doi: 10.1016/j.braindev.2014.05.007. PMID: 24931733
The diagnosis of multiple sclerosis and the various related demyelinating syndromes: a critical review.
Karussis D
J Autoimmun 2014 Feb-Mar;48-49:134-42. Epub 2014 Feb 10 doi: 10.1016/j.jaut.2014.01.022. PMID: 24524923
Novel human reovirus isolated from children with acute necrotizing encephalopathy.
Ouattara LA, Barin F, Barthez MA, Bonnaud B, Roingeard P, Goudeau A, Castelnau P, Vernet G, Paranhos-Baccalà G, Komurian-Pradel F
Emerg Infect Dis 2011 Aug;17(8):1436-44. doi: 10.3201/eid1708.101528. PMID: 21801621Free PMC Article

Recent systematic reviews

Pediatric neuroimaging findings and clinical presentations of COVID-19: A systematic review.
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
Acute Disseminated Encephalomyelitis and Acute Hemorrhagic Leukoencephalitis Following COVID-19: Systematic Review and Meta-synthesis.
Manzano GS, McEntire CRS, Martinez-Lage M, Mateen FJ, Hutto SK
Neurol Neuroimmunol Neuroinflamm 2021 Nov;8(6) Epub 2021 Aug 27 doi: 10.1212/NXI.0000000000001080. PMID: 34452974Free PMC Article

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