Autoimmune paranodopathy
10.3760/cma.j.cn113694-20230718-00006
- VernacularTitle:自身免疫性结旁病
- Author:
Zhecheng ZHANG
1
;
Yuying HOU
;
Ju ZHU
Author Information
1. 天津市第三中心医院神经内科，天津 300170
- Keywords:
Autoimmune paranodopathy;
Neurofascin 155;
Contactin-1;
Contactin-associated protein 1
- From:
Chinese Journal of Neurology
2024;57(1):85-91
- CountryChina
- Language:Chinese
-
Abstract:
Autoimmune paranodopathy (APN) has emerged as an independent rare disease,which is medicated by autoimmune antibodies against the essential complex of paranodal region of Ranvier. The antibodies include anti-neurofascin 155 antibody, anti-contactin-1 antibody and anti-contactin-associated protein 1 antibody. Although there are many similarities between APN and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), patients with APN have relatively unique clinical features, pathogenesis, histopathological results and responses to intravenous immune globulin, distinguishing from typical CIDP. The predominant subclass of IgG among pathogenic antibodies is IgG4, meanwhile, other subclasses have been rarely reported. Early detecting the APN related antibodies and their subclasses not only helps to clarify the diagnosis, but also provides valuable clinical information for the selection of precise treatment and prognosis.
