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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 autoantibody against NF186 in acute‐onset neuropathy is recommended. [ABSTRACT FROM AUTHOR]