Clinical and immunological features of auto-immune nodopathies.

OBJECTIVE

We aimed to evaluate the detection rates of antibodies against nodal-paranodal junction proteins in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and to analyze the clinical, laboratory, and neuroelectrophysiological characteristics of antibody-positive patients.

METHODS

A total of 45 patients with CIDP were enrolled. Serum samples were collected, and cell-based assay (CBA) was used to detect IgG against human neurofascin-155 (NF155), neurofascin-186 (NF186), contactin-1 (CNTN1), contactin-2 (CNTN2) and contactin-associated protein 1 (CASPR1).

RESULTS

Six patients (13.3%) had IgG against NF155, 2 (4.4%) against CASPR1, 2 (4.4%) against NF186 and 1 (2.2%) against CNTN1. Seropositive patients had elevated cerebrospinal fluid (CSF) protein, higher CSF cells than seronegative patients. Patients seropositive for NF155-IgG exhibited an earlier disease onset and demonstrated higher prevalence of limb tremors and sensory ataxia compared to their seronegative counterparts. Among 11 seropositive patients, intravenous immunoglobins (IVIG) achieved good response in 1/7 (14.3%) cases. Rituximab showed good response in 6/8 (75.0%) cases.

CONCLUSIONS

Auto-immune nodopathies represent a distinct clinical entity from CIDP.