
This study analyzes the clinical and biomarker features of anti-CNTN1+ autoimmune nodopathy (AN) in 31 patients. The majority presented with progressive sensory motor neuropathy, ataxia, and severe disability, with 35% showing kidney involvement. Treatment with intravenous immunoglobulin, corticosteroids, and rituximab was commonly used, with rituximab being highly effective. Anti-CNTN1 titers correlated with clinical severity and were negative after successful treatment. Serum neurofilament light chain (sNfL) and CNTN1 levels were elevated in patients compared to healthy controls and normalized following effective treatment. These biomarkers, along with anti-CNTN1 antibodies, are valuable for monitoring disease status.
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