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Encephalomyelitis

MedGen UID:
8603
•Concept ID:
C0014070
•
Disease or Syndrome
Synonyms: Myeloencephalitides; Myeloencephalitis
SNOMED CT: Encephalomyelitis (62950007)
 
Monarch Initiative: MONDO:0005156

Definition

A disorder characterised by inflammation of both the brain and the spinal cord. [from SNOMEDCT_US]

Professional guidelines

PubMed

Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria.
Banwell B, Bennett JL, Marignier R, Kim HJ, Brilot F, Flanagan EP, Ramanathan S, Waters P, Tenembaum S, Graves JS, Chitnis T, Brandt AU, Hemingway C, Neuteboom R, Pandit L, Reindl M, Saiz A, Sato DK, Rostasy K, Paul F, Pittock SJ, Fujihara K, Palace J
Lancet Neurol 2023 Mar;22(3):268-282. Epub 2023 Jan 24 doi: 10.1016/S1474-4422(22)00431-8. PMID: 36706773
Myalgic Encephalomyelitis/Chronic Fatigue Syndrome: Essentials of Diagnosis and Management.
Bateman L, Bested AC, Bonilla HF, Chheda BV, Chu L, Curtin JM, Dempsey TT, Dimmock ME, Dowell TG, Felsenstein D, Kaufman DL, Klimas NG, Komaroff AL, Lapp CW, Levine SM, Montoya JG, Natelson BH, Peterson DL, Podell RN, Rey IR, Ruhoy IS, Vera-Nunez MA, Yellman BP
Mayo Clin Proc 2021 Nov;96(11):2861-2878. Epub 2021 Aug 25 doi: 10.1016/j.mayocp.2021.07.004. PMID: 34454716
Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient.
Wang CX
Paediatr Drugs 2021 May;23(3):213-221. Epub 2021 Apr 8 doi: 10.1007/s40272-021-00441-7. PMID: 33830467Free PMC Article

Recent clinical studies

Etiology

The role of the complement system in Multiple Sclerosis: A review.
Saez-Calveras N, Stuve O
Front Immunol 2022;13:970486. Epub 2022 Aug 10 doi: 10.3389/fimmu.2022.970486. PMID: 36032156Free PMC Article
Multiple Sclerosis-Minimizing Errors in Radiological Diagnosis.
Boski N, Gulati V, Raj R, Gulati P
Neurol India 2021 Nov-Dec;69(6):1539-1546. doi: 10.4103/0028-3886.333497. PMID: 34979638
Acute Disseminated Encephalomyelitis in Children: An Updated Review Based on Current Diagnostic Criteria.
Cole J, Evans E, Mwangi M, Mar S
Pediatr Neurol 2019 Nov;100:26-34. Epub 2019 Jul 3 doi: 10.1016/j.pediatrneurol.2019.06.017. PMID: 31371120
Inflammatory demyelinating diseases of the central nervous system.
Höftberger R, Lassmann H
Handb Clin Neurol 2017;145:263-283. doi: 10.1016/B978-0-12-802395-2.00019-5. PMID: 28987175Free PMC Article
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 disseminated encephalomyelitis (ADEM) following recent Oxford/AstraZeneca COVID-19 vaccination.
Permezel F, Borojevic B, Lau S, de Boer HH
Forensic Sci Med Pathol 2022 Mar;18(1):74-79. Epub 2021 Nov 4 doi: 10.1007/s12024-021-00440-7. PMID: 34735684Free PMC Article
Assessment and Management of Acute Disseminated Encephalomyelitis (ADEM) in the Pediatric Patient.
Wang CX
Paediatr Drugs 2021 May;23(3):213-221. Epub 2021 Apr 8 doi: 10.1007/s40272-021-00441-7. PMID: 33830467Free PMC Article
Acute Disseminated Encephalomyelitis in Children: An Updated Review Based on Current Diagnostic Criteria.
Cole J, Evans E, Mwangi M, Mar S
Pediatr Neurol 2019 Nov;100:26-34. Epub 2019 Jul 3 doi: 10.1016/j.pediatrneurol.2019.06.017. PMID: 31371120
Acute disseminated encephalomyelitis: Updates on an inflammatory CNS syndrome.
Pohl D, Alper G, Van Haren K, Kornberg AJ, Lucchinetti CF, Tenembaum S, Belman AL
Neurology 2016 Aug 30;87(9 Suppl 2):S38-45. doi: 10.1212/WNL.0000000000002825. PMID: 27572859
A spectrum of inflammation and demyelination in acute disseminated encephalomyelitis (ADEM) of children.
Esposito S, Di Pietro GM, Madini B, Mastrolia MV, Rigante D
Autoimmun Rev 2015 Oct;14(10):923-9. Epub 2015 Jun 14 doi: 10.1016/j.autrev.2015.06.002. PMID: 26079482Free PMC Article

Therapy

B cell depletion therapies in autoimmune disease: advances and mechanistic insights.
Lee DSW, Rojas OL, Gommerman JL
Nat Rev Drug Discov 2021 Mar;20(3):179-199. Epub 2020 Dec 15 doi: 10.1038/s41573-020-00092-2. PMID: 33324003Free PMC Article
Ketogenic diet and fasting diet as Nutritional Approaches in Multiple Sclerosis (NAMS): protocol of a randomized controlled study.
Bahr LS, Bock M, Liebscher D, Bellmann-Strobl J, Franz L, Prüß A, Schumann D, Piper SK, Kessler CS, Steckhan N, Michalsen A, Paul F, Mähler A
Trials 2020 Jan 2;21(1):3. doi: 10.1186/s13063-019-3928-9. PMID: 31898518Free PMC Article
Intermittent Fasting Confers Protection in CNS Autoimmunity by Altering the Gut Microbiota.
Cignarella F, Cantoni C, Ghezzi L, Salter A, Dorsett Y, Chen L, Phillips D, Weinstock GM, Fontana L, Cross AH, Zhou Y, Piccio L
Cell Metab 2018 Jun 5;27(6):1222-1235.e6. doi: 10.1016/j.cmet.2018.05.006. PMID: 29874567Free PMC Article
Role of the innate and adaptive immune responses in the course of multiple sclerosis.
Hemmer B, Kerschensteiner M, Korn T
Lancet Neurol 2015 Apr;14(4):406-19. doi: 10.1016/S1474-4422(14)70305-9. PMID: 25792099
Interleukin-10 and the interleukin-10 receptor.
Moore KW, de Waal Malefyt R, Coffman RL, O'Garra A
Annu Rev Immunol 2001;19:683-765. doi: 10.1146/annurev.immunol.19.1.683. PMID: 11244051

Prognosis

Association between covid-19 vaccination, SARS-CoV-2 infection, and risk of immune mediated neurological events: population based cohort and self-controlled case series analysis.
Li X, Raventós B, Roel E, Pistillo A, Martinez-Hernandez E, Delmestri A, Reyes C, Strauss V, Prieto-Alhambra D, Burn E, Duarte-Salles T
BMJ 2022 Mar 16;376:e068373. doi: 10.1136/bmj-2021-068373. PMID: 35296468Free PMC Article
Ketogenic diet and fasting diet as Nutritional Approaches in Multiple Sclerosis (NAMS): protocol of a randomized controlled study.
Bahr LS, Bock M, Liebscher D, Bellmann-Strobl J, Franz L, Prüß A, Schumann D, Piper SK, Kessler CS, Steckhan N, Michalsen A, Paul F, Mähler A
Trials 2020 Jan 2;21(1):3. doi: 10.1186/s13063-019-3928-9. PMID: 31898518Free PMC Article
Combined central and peripheral demyelination: Clinical features, diagnostic findings, and treatment.
Cortese A, Franciotta D, Alfonsi E, Visigalli N, Zardini E, Diamanti L, Prunetti P, Osera C, Gastaldi M, Berzero G, Pichiecchio A, Piccolo G, Lozza A, Piscosquito G, Salsano E, Ceroni M, Moglia A, Bono G, Pareyson D, Marchioni E
J Neurol Sci 2016 Apr 15;363:182-7. Epub 2016 Feb 10 doi: 10.1016/j.jns.2016.02.022. PMID: 27000248
Role of the innate and adaptive immune responses in the course of multiple sclerosis.
Hemmer B, Kerschensteiner M, Korn T
Lancet Neurol 2015 Apr;14(4):406-19. doi: 10.1016/S1474-4422(14)70305-9. PMID: 25792099
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

Clinical prediction guides

CAR-T Cell-Mediated B-Cell Depletion in Central Nervous System Autoimmunity.
Gupta S, Simic M, Sagan SA, Shepherd C, Duecker J, Sobel RA, Dandekar R, Wu GF, Wu W, Pak JE, Hauser SL, Lim W, Wilson MR, Zamvil SS
Neurol Neuroimmunol Neuroinflamm 2023 Mar;10(2) Epub 2023 Jan 19 doi: 10.1212/NXI.0000000000200080. PMID: 36657993Free PMC Article
Seronegative autoimmune encephalitis: clinical characteristics and factors associated with outcomes.
Lee WJ, Lee HS, Kim DY, Lee HS, Moon J, Park KI, Lee SK, Chu K, Lee ST
Brain 2022 Oct 21;145(10):3509-3521. doi: 10.1093/brain/awac166. PMID: 35512357
Role of lipoic acid in multiple sclerosis.
Xie H, Yang X, Cao Y, Long X, Shang H, Jia Z
CNS Neurosci Ther 2022 Mar;28(3):319-331. Epub 2021 Dec 28 doi: 10.1111/cns.13793. PMID: 34964271Free PMC Article
Gut Microbiome in Progressive Multiple Sclerosis.
Cox LM, Maghzi AH, Liu S, Tankou SK, Dhang FH, Willocq V, Song A, Wasén C, Tauhid S, Chu R, Anderson MC, De Jager PL, Polgar-Turcsanyi M, Healy BC, Glanz BI, Bakshi R, Chitnis T, Weiner HL
Ann Neurol 2021 Jun;89(6):1195-1211. Epub 2021 Apr 30 doi: 10.1002/ana.26084. PMID: 33876477Free 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

Recent systematic reviews

Autoimmune encephalitis associated with COVID-19: A systematic review.
Nabizadeh F, Balabandian M, Sodeifian F, Rezaei N, Rostami MR, Naser Moghadasi A
Mult Scler Relat Disord 2022 Jun;62:103795. Epub 2022 Apr 6 doi: 10.1016/j.msard.2022.103795. PMID: 35472834Free PMC Article
Association of CNS demyelination and COVID-19 infection: an updated systematic review.
Ismail II, Salama S
J Neurol 2022 Feb;269(2):541-576. Epub 2021 Aug 12 doi: 10.1007/s00415-021-10752-x. PMID: 34386902Free PMC Article
Multiple Sclerosis-Minimizing Errors in Radiological Diagnosis.
Boski N, Gulati V, Raj R, Gulati P
Neurol India 2021 Nov-Dec;69(6):1539-1546. doi: 10.4103/0028-3886.333497. PMID: 34979638
Long COVID and Myalgic Encephalomyelitis/Chronic Fatigue Syndrome (ME/CFS)-A Systemic Review and Comparison of Clinical Presentation and Symptomatology.
Wong TL, Weitzer DJ
Medicina (Kaunas) 2021 Apr 26;57(5) doi: 10.3390/medicina57050418. PMID: 33925784Free PMC Article
Systematic review of randomized controlled trials for chronic fatigue syndrome/myalgic encephalomyelitis (CFS/ME).
Kim DY, Lee JS, Park SY, Kim SJ, Son CG
J Transl Med 2020 Jan 6;18(1):7. doi: 10.1186/s12967-019-02196-9. PMID: 31906979Free PMC Article

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