ABSTRACT
Contactin-1(CNTN1)is a neural cell adhesion molecule belonging to the subgroup of immunoglobulin superfamily.It is anchored on the cell surface through glycosyl phosphatidyl inositol-anchored neural membrane proteins and participates in axon guidance,synapse formation,growth and development of nervous systems,and occurrence of related disea-ses.In addition,CNTN1 can promote inflammatory response and signal communication between microglia and astrocytes through various mechanisms,playing a role in the pathological processes of various neurological and psychiatric disorders.This article discusses the role of CNTN1 in depression and cognitive dysfunction.
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.
ABSTRACT
Objective:To investigate the detecting method and clinical characteristics of anti-nodal/paranodal antibodies in chronic inflammatory demyelinating polyradiculopathy.Methods:Serum samples were collected from 212 patients with chronic inflammatory demyelinating polyradiculopathy who were admitted to Huashan Hospital of Fudan University or from other clinical centers from January 2018 to July 2021. Autoantibodies (anti-NF155, anti-NF186, anti-CNTN1) and IgG subtypes were detected with cell-based assay. According to the test results, patients were divided into anti-NF155 positive group, anti-NF186 positive group and anti-CNTN1 positive group, clinical characteristics of patients in each group, including limb weakness, superficial sensation and proprioception, tremor, cerebrospinal fluid protein level, brachial plexus magnetic resonance (MRI) were retrospectively analyzed and compared.Results:A total of 23 patients (10.8%,23/212) were positive for anti-NF155 antibody, 12 (5.7%,12/212) for anti-NF186 antibody, and 4 (1.9%,4/212) for anti-CNTN1 antibody. IgG 4 was the predominant subtype in anti-NF155 and anti-CNTN1 groups. In the anti-NF186 group, all cases were IgG positive and antibody subtypes could be detected in 4 cases (4/12). In anti-NF155 group, 23 patients (100%,23/23) had limb weakness and deep sensory disturbance, 19 patients (82.6%,19/23) had superficial sensory disturbance, 22 patients (95.7%,22/23) were symmetrically involved, 18 patients (78.3%,18/23) showed tremor, 19 patients (19/19) showed abnormal in brachial plexus MRI. In anti-NF186 group, 12 patients had limb weakness (12/12), 9 patients (9/12) and 6 patients (6/12) had superficial sensory disturbance and deep sensory disturbance respectively, 8 patients (8/12) were asymmetrically involved, and only 1 patient (1/12) showed tremor, 1 (1/7) showed abnormal brachial plexus MRI. In anti-CNTN1 group, 4 cases showed symmetrical limb weakness and sensory disturbance, 3 patients had tremor, and four patients showed brachial plexus MRI abnormality. There were statistically significant differences in onset age, proprioception, tremor and MRI abnormalities of brachial plexus among the 3 groups ( P<0.01). Conclusions:The clinical characteristics of CIDP patients with anti-NF155, anti-NF186 and anti-CNTN1 antibodies are different. Screening anti-nodal/paranodal antibodies is of great significance for accurate diagnosis and treatment of patients with peripheral neuropathy.
ABSTRACT
Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a motor-sensory demyelinating neuropathy mediated by immunity. The pathogenesis is still unclear, and the clinical manifestations are complex. Its diagnoses are difficult, and the treatment effect is not good. At present, most studies consider it to be an autoimmune disease, but the pathogenic antigen and its target to the immune damage mechanism of the disease are still unclear. In this paper, we review the literature on the pathogenesis of CIDP in recent years, and introduce the specific mechanisms which cause CIDP from cellular and humoral immunity.