Clinical Profile of Autoimmune Nodopathy with Anti‐neurofascin 186 Antibody

Objective: Nodal/paranodal autoantibodies identified a group of peripheral neuropathies independent from chronic inflammatory demyelinating polyneuropathy (CIDP). However, nodopathy with antibody against neurofascin 186 (NF186) was rarely reported. We presented a cohort of patients with anti-NF186 antibody and described the clinical profile of them. Methods: In this retrospective study, 195 patients diagnosed with CIDP and immune mediated idiopathic neuropathies were enrolled. Cell-based assay was used to screen anti-NF186 and anti-NF155 antibodies in serum samples. Teased-fiber immunofluorescence were used as a confirmatory assay. Clinical data of seropositive patients were collected and analyzed. Results: Among the patients with anti-NF186 antibody, seven patients (58.3%) presented acute or subacute disorder onset. Four patients (33.3%) were found to have asymmetric weakness or numbness. Distal weakness and/or numbness was the core feature. Sensory ataxia, tremor and central nervous system demyelination were rarely observed. Nerve conduction studies revealed predominant demyelinating with/without axonal loss. Brachial plexus MRI was normal in the majority of patients (6/7, 85.7%). Five patients (5/9, 55.6%) showed response to intravenous immunoglobulin. Eight patients (8/10, 80.0%) improved after corticosteroids. All patients (3/3,100%) responded to rituximab. Interpretation: In the study, we depicted the clinical profile of nodopathy with anti-NF186 antibody. The diversity of clinical features, electrophysiology results and pathological findings was specific in nodopathy with anti-NF186 antibody. Screening of auto antibody against NF186 in acute-onset neuropathy is recommended.