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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

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
Acute Necrotizing Encephalopathy.
Jan F, Jafri SK, Ibrahim SH
J Coll Physicians Surg Pak 2019 Jul;29(7):649-653. doi: 10.29271/jcpsp.2019.07.649. PMID: 31253217
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
Acute disseminated encephalomyelitis.
Tenembaum S, Chitnis T, Ness J, Hahn JS; International Pediatric MS Study Group
Neurology 2007 Apr 17;68(16 Suppl 2):S23-36. doi: 10.1212/01.wnl.0000259404.51352.7f. PMID: 17438235

Diagnosis

Genetic Acute Necrotizing Encephalopathy Associated with RANBP2: Clinical and Therapeutic Implications in Pediatrics.
Levine JM, Ahsan N, Ho E, Santoro JD
Mult Scler Relat Disord 2020 Aug;43:102194. Epub 2020 May 15 doi: 10.1016/j.msard.2020.102194. PMID: 32426208Free PMC Article
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
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
Acute necrotizing encephalopathy.
Yadav S, Das CJ, Kumar V, Lodha R
Indian J Pediatr 2010 Mar;77(3):307-9. Epub 2009 Nov 20 doi: 10.1007/s12098-009-0261-4. PMID: 19936654

Therapy

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
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
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

Genetic Acute Necrotizing Encephalopathy Associated with RANBP2: Clinical and Therapeutic Implications in Pediatrics.
Levine JM, Ahsan N, Ho E, Santoro JD
Mult Scler Relat Disord 2020 Aug;43:102194. Epub 2020 May 15 doi: 10.1016/j.msard.2020.102194. PMID: 32426208Free PMC Article
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
Acute disseminated encephalomyelitis.
Tenembaum S, Chitnis T, Ness J, Hahn JS; International Pediatric MS Study Group
Neurology 2007 Apr 17;68(16 Suppl 2):S23-36. doi: 10.1212/01.wnl.0000259404.51352.7f. PMID: 17438235
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

Clinical features and imaging manifestations of acute necrotizing encephalopathy in children.
Wu L, Peng H, Jiang Y, He L, Jiang L, Hu Y
Int J Dev Neurosci 2022 Aug;82(5):447-457. Epub 2022 Jun 22 doi: 10.1002/jdn.10201. PMID: 35688614
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
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
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

Dengue-Associated Acute Necrotizing Encephalopathy Is an Acute Necrotizing Encephalopathy Variant Rather than a Mimic: Evidence From a Systematic Review.
Barron S, Han VX, Gupta J, Lingappa L, Sankhyan N, Thomas T
Pediatr Neurol 2024 Dec;161:208-215. Epub 2024 Sep 27 doi: 10.1016/j.pediatrneurol.2024.09.021. PMID: 39442246
Acute hemorrhagic leukoencephalitis: a case report and systematic review of factors associated with severe disability and death.
Tan Z, Lin SZZ, Foong WD, Yong MH
Neurol Sci 2024 Dec;45(12):5859-5874. Epub 2024 Aug 8 doi: 10.1007/s10072-024-07715-1. PMID: 39115612
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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