Overlapping central and peripheral nervous system syndromes in MOG antibody-associated disorders.
Adult
Aged
Autoantibodies
/ immunology
Autoantigens
/ immunology
Cohort Studies
Demyelinating Autoimmune Diseases, CNS
/ complications
Female
Humans
Male
Middle Aged
Myelin-Oligodendrocyte Glycoprotein
/ immunology
Myelitis, Transverse
/ complications
Optic Neuritis
/ complications
Peripheral Nervous System Diseases
/ epidemiology
Journal
Neurology(R) neuroimmunology & neuroinflammation
ISSN: 2332-7812
Titre abrégé: Neurol Neuroimmunol Neuroinflamm
Pays: United States
ID NLM: 101636388
Informations de publication
Date de publication:
01 2021
01 2021
Historique:
received:
12
07
2020
accepted:
16
10
2020
entrez:
4
12
2020
pubmed:
5
12
2020
medline:
13
10
2021
Statut:
epublish
Résumé
Antibodies to myelin oligodendrocyte glycoprotein (MOG) are associated with CNS demyelination inclusive of optic neuritis (ON) and transverse myelitis (TM). To examine whether peripheral nervous system (PNS) involvement is associated with MOG antibody-associated disorders (MOGAD), we performed detailed characterization of an Australasian MOGAD cohort. Using a live cell-based assay, we diagnosed 271 adults with MOGAD (2013-2018) and performed detailed clinical and immunologic characterization on those with likely PNS involvement. We identified 19 adults with MOGAD and PNS involvement without prior TM. All patients had CNS involvement including ON (bilateral [n = 3], unilateral [n = 3], and recurrent [n = 7]), a cortical lesion (n = 1), meningoencephalitis (n = 1), and subsequent TM (n = 4). Clinical phenotyping and neurophysiology were consistent with acute inflammatory demyelinating polyneuropathy (n = 1), myeloradiculitis (n = 3), multifocal motor neuropathy (n = 1), brachial neuritis (n = 2), migrant sensory neuritis (n = 3), and paresthesia and/or radicular limb pain (n = 10). Onset MRI spine was consistent with myeloradiculitis with nerve root enhancement in 3/19 and normal in 16/19. Immunotherapy resulted in partial/complete PNS symptom resolution in 12/15 (80%) (steroids and/or IV immunoglobulin n = 9, rituximab n = 2, and plasmapheresis n = 1). We identified serum antibodies targeting neurofascin 155, contactin-associated protein 2, or GM1 in 4/16 patients with MOGAD PNS compared with 0/30 controls ( Myeloradiculitis, combined central and peripheral demyelination syndromes, and inflammatory neuropathies may be associated with MOGAD and may be immunotherapy responsive. We identified a subgroup who may have pathology mediated by coexistent autoantibodies.
Identifiants
pubmed: 33272955
pii: 8/1/e924
doi: 10.1212/NXI.0000000000000924
pmc: PMC7803332
pii:
doi:
Substances chimiques
Autoantibodies
0
Autoantigens
0
Myelin-Oligodendrocyte Glycoprotein
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Subventions
Organisme : Medical Research Council
ID : MR/P008399/1
Pays : United Kingdom
Organisme : Wellcome Trust
Pays : United Kingdom
Investigateurs
Helmut Butzkueven
(H)
Clare L Fraser
(CL)
Victor S C Fung
(VSC)
Andrew P D Henderson
(APD)
Matthew C Kiernan
(MC)
Jeannette Lechner-Scott
(J)
Mark P Marriott
(MP)
Pamela McCombe
(P)
Mastura Monif
(M)
John D E Parratt
(JDE)
Peter W Rowe
(PW)
Neil Shuey
(N)
Mark Slee
(M)
Ian Sutton
(I)
Esther Tantsis
(E)
Benjamin Trewin
(B)
Anneke Van der Walt
(A)
Owen B White
(OB)
Con Yiannikas
(C)
Informations de copyright
Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
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