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Myelitis

MedGen UID:
10230
Concept ID:
C0026975
Disease or Syndrome
Synonyms: Inflammation, Spinal Cord; Inflammations, Spinal Cord; Inflammatory Myelopathies; Inflammatory Myelopathy; Myelitides; Myelopathies, Inflammatory; Myelopathy, Inflammatory; Spinal Cord Inflammation; Spinal Cord Inflammations
SNOMED CT: Myelitis (41370002)
 
HPO: HP:0012486
Monarch Initiative: MONDO:0002565

Definition

Inflammation of the spinal cord. [from HPO]

Conditions with this feature

Systemic lupus erythematosus 17
MedGen UID:
1804329
Concept ID:
C5676884
Disease or Syndrome
Systemic lupus erythematosus-17 (SLE17) is an X-linked dominant autoimmune disorder characterized by onset of systemic autoinflammatory symptoms in the first decades of life. Only affected females have been reported. Features may include classic features of SLE, such as malar rash and arthralgias, or can include less common entities such as hemiplegia and neuromyelitis optica (NMO). Laboratory studies show the presence of autoantibodies and enhanced NFKB (164011) signaling, the latter being consistent with a gain-of-function effect (Brown et al., 2022). For a phenotypic description and a discussion of genetic heterogeneity of systemic lupus erythematosus (SLE), see 152700.

Professional guidelines

PubMed

Jarius S, Aktas O, Ayzenberg I, Bellmann-Strobl J, Berthele A, Giglhuber K, Häußler V, Havla J, Hellwig K, Hümmert MW, Kleiter I, Klotz L, Krumbholz M, Kümpfel T, Paul F, Ringelstein M, Ruprecht K, Senel M, Stellmann JP, Bergh FT, Tumani H, Wildemann B, Trebst C; Neuromyelitis Optica Study Group (NEMOS)
J Neurol 2023 Jul;270(7):3341-3368. Epub 2023 Apr 6 doi: 10.1007/s00415-023-11634-0. PMID: 37022481Free PMC Article
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
McGinley MP, Goldschmidt CH, Rae-Grant AD
JAMA 2021 Feb 23;325(8):765-779. doi: 10.1001/jama.2020.26858. PMID: 33620411

Recent clinical studies

Etiology

Douglas AG, Xu DJ, Shah MP
Neurol Clin 2022 Feb;40(1):133-156. doi: 10.1016/j.ncl.2021.08.009. PMID: 34798966
Lopez Chiriboga S, Flanagan EP
Continuum (Minneap Minn) 2021 Feb 1;27(1):62-92. doi: 10.1212/CON.0000000000000900. PMID: 33522737
Dubey D, Pittock SJ, Krecke KN, Morris PP, Sechi E, Zalewski NL, Weinshenker BG, Shosha E, Lucchinetti CF, Fryer JP, Lopez-Chiriboga AS, Chen JC, Jitprapaikulsan J, McKeon A, Gadoth A, Keegan BM, Tillema JM, Naddaf E, Patterson MC, Messacar K, Tyler KL, Flanagan EP
JAMA Neurol 2019 Mar 1;76(3):301-309. doi: 10.1001/jamaneurol.2018.4053. PMID: 30575890Free PMC Article
Rodríguez Y, Rojas M, Pacheco Y, Acosta-Ampudia Y, Ramírez-Santana C, Monsalve DM, Gershwin ME, Anaya JM
Cell Mol Immunol 2018 Jun;15(6):547-562. Epub 2018 Jan 29 doi: 10.1038/cmi.2017.142. PMID: 29375121Free PMC Article
Transverse Myelitis Consortium Working Group
Neurology 2002 Aug 27;59(4):499-505. doi: 10.1212/wnl.59.4.499. PMID: 12236201

Diagnosis

Tisavipat N, Flanagan EP
Expert Rev Neurother 2023 Apr;23(4):389-411. Epub 2023 Apr 7 doi: 10.1080/14737175.2023.2195095. PMID: 37026545
Douglas AG, Xu DJ, Shah MP
Neurol Clin 2022 Feb;40(1):133-156. doi: 10.1016/j.ncl.2021.08.009. PMID: 34798966
Lopez Chiriboga S, Flanagan EP
Continuum (Minneap Minn) 2021 Feb 1;27(1):62-92. doi: 10.1212/CON.0000000000000900. PMID: 33522737
West TW
Discov Med 2013 Oct;16(88):167-77. PMID: 24099672
West TW, Hess C, Cree BA
Semin Neurol 2012 Apr;32(2):97-113. Epub 2012 Sep 8 doi: 10.1055/s-0032-1322586. PMID: 22961185

Therapy

Misery L, Genestet S, Zagnoli F
Dermatology 2022;238(5):823-828. Epub 2022 Mar 4 doi: 10.1159/000522168. PMID: 35249011
Pittock SJ, Zekeridou A, Weinshenker BG
Nat Rev Neurol 2021 Dec;17(12):759-773. Epub 2021 Oct 28 doi: 10.1038/s41582-021-00568-8. PMID: 34711906
Carnero Contentti E, Correale J
J Neuroinflammation 2021 Sep 16;18(1):208. doi: 10.1186/s12974-021-02249-1. PMID: 34530847Free PMC Article
Asundi A, Cervantes-Arslanian AM, Lin NH, Barbosa F
Semin Neurol 2019 Aug;39(4):472-481. Epub 2019 Sep 18 doi: 10.1055/s-0039-1688923. PMID: 31533188
Li XY, Xiao HB, Pai P
J Clin Neurosci 2017 Oct;44:18-22. Epub 2017 Jul 8 doi: 10.1016/j.jocn.2017.06.003. PMID: 28694045

Prognosis

Grasso EA, Pozzilli V, Tomassini V
Handb Clin Neurol 2023;196:101-117. doi: 10.1016/B978-0-323-98817-9.00020-X. PMID: 37620065
Mikami T, Liaw B, Asada M, Niimura T, Zamami Y, Green-LaRoche D, Pai L, Levy M, Jeyapalan S
J Neurooncol 2021 Mar;152(1):135-144. Epub 2021 Jan 9 doi: 10.1007/s11060-020-03687-2. PMID: 33423151
Jarius S, Paul F, Weinshenker BG, Levy M, Kim HJ, Wildemann B
Nat Rev Dis Primers 2020 Oct 22;6(1):85. doi: 10.1038/s41572-020-0214-9. PMID: 33093467
Absoud M, Greenberg BM, Lim M, Lotze T, Thomas T, Deiva K
Neurology 2016 Aug 30;87(9 Suppl 2):S46-52. doi: 10.1212/WNL.0000000000002820. PMID: 27572861
West TW
Discov Med 2013 Oct;16(88):167-77. PMID: 24099672

Clinical prediction guides

Tisavipat N, Flanagan EP
Expert Rev Neurother 2023 Apr;23(4):389-411. Epub 2023 Apr 7 doi: 10.1080/14737175.2023.2195095. PMID: 37026545
Dubey D, Pittock SJ, Krecke KN, Morris PP, Sechi E, Zalewski NL, Weinshenker BG, Shosha E, Lucchinetti CF, Fryer JP, Lopez-Chiriboga AS, Chen JC, Jitprapaikulsan J, McKeon A, Gadoth A, Keegan BM, Tillema JM, Naddaf E, Patterson MC, Messacar K, Tyler KL, Flanagan EP
JAMA Neurol 2019 Mar 1;76(3):301-309. doi: 10.1001/jamaneurol.2018.4053. PMID: 30575890Free PMC Article
Absoud M, Greenberg BM, Lim M, Lotze T, Thomas T, Deiva K
Neurology 2016 Aug 30;87(9 Suppl 2):S46-52. doi: 10.1212/WNL.0000000000002820. PMID: 27572861
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
Carod-Artal FJ
Expert Rev Anti Infect Ther 2010 Nov;8(11):1307-18. doi: 10.1586/eri.10.111. PMID: 21073294

Recent systematic reviews

Wen X, Xu D, Yuan S, Zhang J
Autoimmun Rev 2022 Jun;21(6):103103. Epub 2022 Apr 20 doi: 10.1016/j.autrev.2022.103103. PMID: 35452852
Marini A, Bernardini A, Gigli GL, Valente M, Muñiz-Castrillo S, Honnorat J, Vogrig A
Neurology 2021 Apr 20;96(16):754-766. Epub 2021 Mar 2 doi: 10.1212/WNL.0000000000011795. PMID: 33653902
Agmon-Levin N, Kivity S, Szyper-Kravitz M, Shoenfeld Y
Lupus 2009 Nov;18(13):1198-204. doi: 10.1177/0961203309345730. PMID: 19880568
Rafailidis PI, Mourtzoukou EG, Varbobitis IC, Falagas ME
Virol J 2008 Mar 27;5:47. doi: 10.1186/1743-422X-5-47. PMID: 18371229Free PMC Article
Cree BA, Goodin DS, Hauser SL
Semin Neurol 2002 Jun;22(2):105-22. doi: 10.1055/s-2002-36534. PMID: 12524556

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