Widening of myelin lamellae in polyneuropathy with immunoglobulin-M monoclonal gammopathy, without activity against myelin-associated glycoprotein, responsive to treatment.
Anti-MAG
Inter-nodopathy
Nodo-paranodopathy
Polyneuropathy
Rituximab
Journal
Neuromuscular disorders : NMD
ISSN: 1873-2364
Titre abrégé: Neuromuscul Disord
Pays: England
ID NLM: 9111470
Informations de publication
Date de publication:
08 2022
08 2022
Historique:
received:
07
03
2022
revised:
04
05
2022
accepted:
09
05
2022
pubmed:
27
5
2022
medline:
19
8
2022
entrez:
26
5
2022
Statut:
ppublish
Résumé
We report the case of a patient with a very severe predominantly demyelinating sensorimotor polyneuropathy (with axonal loss) that had developed over several months, along with an immunoglobulin-M monoclonal gammopathy without anti-myelin associated glycoprotein antibodies (or other antibodies against myelin). Widening of myelin lamellae were frequently observed by electron microscopic examination of a nerve biopsy: immunoglobulin-M targeting an unknown myelin antigen appears to be responsible for the nerve lesions similar to those observed in anti-myelin associated glycoprotein polyneuropathy. Usually, if in anti-myelin associated glycoprotein neuropathy the response to immunotherapies is not optimal, in this case the combination of plasma exchanges and rituximab was effective, suggesting an autoimmune origin.
Identifiants
pubmed: 35618575
pii: S0960-8966(22)00138-9
doi: 10.1016/j.nmd.2022.05.001
pii:
doi:
Substances chimiques
Antibodies, Monoclonal
0
Immunoglobulin M
0
Myelin-Associated Glycoprotein
0
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
678-681Informations de copyright
Copyright © 2022. Published by Elsevier B.V.
Déclaration de conflit d'intérêts
Declaration of Competing Interest The authors declare that they have no conflict of interest.