Outcome and risk of recurrence in a large cohort of idiopathic longitudinally extensive transverse myelitis without AQP4/MOG antibodies.

Adolescent Adult Aged Aged, 80 and over Aquaporin 4 / immunology Autoantibodies / blood Cohort Studies Female Humans Immunoglobulins, Intravenous / therapeutic use Immunosuppressive Agents / therapeutic use Male Middle Aged Myelin-Oligodendrocyte Glycoprotein / immunology Myelitis, Transverse / immunology Plasmapheresis / methods Prognosis Recovery of Function Recurrence Risk Factors Young Adult

Longitudinally extensive transverse myelitis Neuromyelitis optica Outcome Seronegative Treatment

Journal

Journal of neuroinflammation

ISSN: 1742-2094

Titre abrégé: J Neuroinflammation

Pays: England

ID NLM: 101222974

Informations de publication

Date de publication:
23 Apr 2020

Historique:

received: 10 01 2020

accepted: 16 03 2020

entrez: 25 4 2020

pubmed: 25 4 2020

medline: 19 3 2021

Statut: epublish

Résumé

Longitudinally extensive transverse myelitis (LETM) is classically related to aquaporin (AQP4)-antibodies (Ab) neuromyelitis optica spectrum disorders (NMOSD) or more recently to myelin oligodendrocyte glycoprotein (MOG)-Ab associated disease. However, some patients remain negative for any diagnosis, despite a large work-up including AQP4-Ab and MOG-Ab. Data about natural history, disability outcome, and treatment are limited in this group of patients. We aimed to (1) describe clinical, biological, and radiological features of double seronegative LETM patients; (2) assess the clinical course and identify prognostic factors; and (3) assess the risk of recurrence, according to maintenance immunosuppressive therapy. Retrospective evaluation of patients with a first episode of LETM, tested negative for AQP-Ab and MOG-Ab, from the French nationwide observatory study NOMADMUS. Fifty-three patients (median age 38 years (range 16-80)) with double seronegative LETM were included. Median nadir EDSS at onset was 6.0 (1-8.5), associated to a median EDSS at last follow-up of 4.0 (0-8). Recurrence was observed in 24.5% of patients in the 18 following months, with a median time to first relapse of 5.7 months. The risk of recurrence was lower in the group of patients treated early with an immunosuppressive drug (2/22, 9%), in comparison with untreated patients (10/31, 32%). A first episode of a double seronegative LETM is associated to a severe outcome and a high rate of relapse in the following 18 months, suggesting that an early immunosuppressive treatment may be beneficial in that condition.

Sections du résumé

BACKGROUND BACKGROUND

METHODS METHODS

Retrospective evaluation of patients with a first episode of LETM, tested negative for AQP-Ab and MOG-Ab, from the French nationwide observatory study NOMADMUS.

RESULTS RESULTS

Fifty-three patients (median age 38 years (range 16-80)) with double seronegative LETM were included. Median nadir EDSS at onset was 6.0 (1-8.5), associated to a median EDSS at last follow-up of 4.0 (0-8). Recurrence was observed in 24.5% of patients in the 18 following months, with a median time to first relapse of 5.7 months. The risk of recurrence was lower in the group of patients treated early with an immunosuppressive drug (2/22, 9%), in comparison with untreated patients (10/31, 32%).

CONCLUSIONS CONCLUSIONS

A first episode of a double seronegative LETM is associated to a severe outcome and a high rate of relapse in the following 18 months, suggesting that an early immunosuppressive treatment may be beneficial in that condition.

Identifiants

DOI: 10.1186/s12974-020-01773-w PMID: 32326965 PMC: PMC7178729

pubmed: 32326965

doi: 10.1186/s12974-020-01773-w

pii: 10.1186/s12974-020-01773-w

pmc: PMC7178729

doi:

Substances chimiques

AQP4 protein, human 0

Aquaporin 4 0

Autoantibodies 0

Immunoglobulins, Intravenous 0

Immunosuppressive Agents 0

MOG protein, human 0

Myelin-Oligodendrocyte Glycoprotein 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

Pagination

128

Références

BMC Neurol. 2013 Apr 08;13:33

pubmed: 23566260

Neurol Neuroimmunol Neuroinflamm. 2018 Sep 26;5(6):e504

pubmed: 30345331

J Neuroophthalmol. 2019 Mar;39(1):3-7

pubmed: 30015656

Neurology. 2013 Jun 11;80(24):2194-200

pubmed: 23658379

Mult Scler. 2016 Mar;22(3):312-9

pubmed: 26209592

Neurol Neuroimmunol Neuroinflamm. 2014 Apr 24;1(1):e4

pubmed: 25340060

J Neuroinflammation. 2016 Nov 1;13(1):282

pubmed: 27802824

Mult Scler. 2017 Sep;23(10):1377-1384

pubmed: 27885065

Ann Neurol. 2016 Feb;79(2):206-16

pubmed: 26537743

Neurology. 2002 Aug 27;59(4):499-505

pubmed: 12236201

Neurol Neuroimmunol Neuroinflamm. 2016 Apr 14;3(3):e225

pubmed: 27144216

Curr Opin Neurol. 2014 Jun;27(3):279-89

pubmed: 24792338

J Neurol Neurosurg Psychiatry. 2019 Apr;90(4):488-490

pubmed: 30032117

Mult Scler. 2015 May;21(6):710-7

pubmed: 25305252

JAMA Neurol. 2013 Nov;70(11):1375-81

pubmed: 23999580

Neurology. 2015 Jul 14;85(2):177-89

pubmed: 26092914

JAMA Neurol. 2019 Jan 1;76(1):56-63

pubmed: 30264146

J Neuroradiol. 2015 Jun;42(3):133-40

pubmed: 25660217

Sci Rep. 2019 Jul 17;9(1):10371

pubmed: 31316082

Neurol Neuroimmunol Neuroinflamm. 2017 Feb 22;4(3):e334

pubmed: 28255575

Ann Neurol. 2006 Mar;59(3):566-9

pubmed: 16453327

Neurology. 2018 Jan 9;90(2):e96-e102

pubmed: 29247071

Eur J Neurol. 2018 Nov;25(11):1378-1383

pubmed: 30004610

Neurology. 2018 May 22;90(21):e1858-e1869

pubmed: 29695592

Ther Adv Neurol Disord. 2016 May;9(3):180-8

pubmed: 27134673

Rev Neurol (Paris). 2014 Jan;170(1):6-12

pubmed: 24269116

JAMA Neurol. 2014 Mar;71(3):324-30

pubmed: 24445513

Mult Scler. 2013 May;19(6):742-8

pubmed: 23037550

Acad Radiol. 2014 May;21(5):675-84

pubmed: 24703482