AChR myasthenia gravis switching to MuSK or double antibody positive myasthenia gravis in two children and literature review.
Achr antibody
Children
Musk antibody
Myasthenia gravis
Journal
Neuromuscular disorders : NMD
ISSN: 1873-2364
Titre abrégé: Neuromuscul Disord
Pays: England
ID NLM: 9111470
Informations de publication
Date de publication:
07 2020
07 2020
Historique:
received:
10
07
2019
revised:
14
02
2020
accepted:
23
03
2020
pubmed:
11
5
2020
medline:
12
8
2021
entrez:
11
5
2020
Statut:
ppublish
Résumé
Muscle-specific tyrosine kinase antibody (MuSK-Ab) and acetylcholine receptor antibody (AChR-Ab) coexistence in myasthenia gravis (MG) is very rare. In this report, two children with AChR-Ab switching to double antibody positive MG (DP-MG) or MuSK-Ab positive MG (MuSK-MG) are described. Six similar cases were found in the literature via online database search. Therefore, this study describes eight patients in total, six female and two male. The average age of onset was 7.25 ± 5.95 years. Four AChR-MG patients switched to DP-MG with no known precipitating factor and four switched after thymectomy (two to MuSK-MG and two to DP-MG). After the serological switch, the patients transitioned to the phenotype of MuSK-MG and responded poorly to cholinesterase inhibitors and well to corticosteroids and plasma exchange.
Identifiants
pubmed: 32387283
pii: S0960-8966(20)30085-7
doi: 10.1016/j.nmd.2020.03.012
pii:
doi:
Substances chimiques
Autoantibodies
0
Cholinesterase Inhibitors
0
Receptors, Cholinergic
0
MUSK protein, human
EC 2.7.10.1
Receptor Protein-Tyrosine Kinases
EC 2.7.10.1
Types de publication
Case Reports
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
534-538Commentaires et corrections
Type : CommentIn
Informations de copyright
Copyright © 2020 Elsevier B.V. All rights reserved.