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  • The following term was not found in MedGen: acyl-homoserine.

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

Biallelic variants in ribonuclease inhibitor (RNH1), an inflammasome modulator, are associated with a distinctive subtype of acute, necrotizing encephalopathy.
Shashi V, Schoch K, Ganetzky R, Kranz PG, Sondheimer N, Markert ML, Cope H, Sadeghpour A, Roehrs P, Arbogast T, Muraresku C; Undiagnosed Diseases Network, Tyndall AV, Esser MJ, Woodward KE, Ping-Yee Au B, Parboosingh JS, Lamont RE, Bernier FP, Wright NAM, Benseler SM, Parsons SJ, El-Dairi M, Smith EC, Valdez P, Tennison M, Innes AM, Davis EE
Genet Med 2023 Sep;25(9):100897. Epub 2023 May 13 doi: 10.1016/j.gim.2023.100897. PMID: 37191094Free PMC Article
Acute haemorrhagic leukoencephalitis (AHLE) - our experience and a short review.
Pujari SS, Kulkarni RV, Ojha P, Gursahani R, Nadgir D, Patil S, Soni G, Bangar S, Harshe A, Mandolkar M, Joshi A, Kadam S, Goyal A
J Neuroimmunol 2021 Dec 15;361:577751. Epub 2021 Oct 13 doi: 10.1016/j.jneuroim.2021.577751. PMID: 34739912
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
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

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
Paediatric acute haemorrhagic leukoencephalitis.
Bamnawat H, Khera D, Didel S, Tiwari S
BMJ Case Rep 2022 Jun 9;15(6) doi: 10.1136/bcr-2021-246654. PMID: 35680284Free PMC Article
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 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
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

Therapy

Acute haemorrhagic leukoencephalitis (AHLE) - our experience and a short review.
Pujari SS, Kulkarni RV, Ojha P, Gursahani R, Nadgir D, Patil S, Soni G, Bangar S, Harshe A, Mandolkar M, Joshi A, Kadam S, Goyal A
J Neuroimmunol 2021 Dec 15;361:577751. Epub 2021 Oct 13 doi: 10.1016/j.jneuroim.2021.577751. PMID: 34739912
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
Encephalitis and postinfectious encephalitis.
Greenlee JE
Continuum (Minneap Minn) 2012 Dec;18(6 Infectious Disease):1271-89. doi: 10.1212/01.CON.0000423847.40147.06. PMID: 23221841
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

Prognosis

Paediatric acute haemorrhagic leukoencephalitis.
Bamnawat H, Khera D, Didel S, Tiwari S
BMJ Case Rep 2022 Jun 9;15(6) doi: 10.1136/bcr-2021-246654. PMID: 35680284Free PMC Article
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 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
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

Clinical prediction guides

Biallelic variants in ribonuclease inhibitor (RNH1), an inflammasome modulator, are associated with a distinctive subtype of acute, necrotizing encephalopathy.
Shashi V, Schoch K, Ganetzky R, Kranz PG, Sondheimer N, Markert ML, Cope H, Sadeghpour A, Roehrs P, Arbogast T, Muraresku C; Undiagnosed Diseases Network, Tyndall AV, Esser MJ, Woodward KE, Ping-Yee Au B, Parboosingh JS, Lamont RE, Bernier FP, Wright NAM, Benseler SM, Parsons SJ, El-Dairi M, Smith EC, Valdez P, Tennison M, Innes AM, Davis EE
Genet Med 2023 Sep;25(9):100897. Epub 2023 May 13 doi: 10.1016/j.gim.2023.100897. PMID: 37191094Free PMC Article
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
Spectrum of Neurological Manifestations in Covid-19: A Review.
Garg RK
Neurol India 2020 May-Jun;68(3):560-572. doi: 10.4103/0028-3886.289000. PMID: 32643664
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

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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