PT - JOURNAL ARTICLE AU - Janev Fehmi AU - Tom Vale AU - Stephen Keddie AU - Simon Rinaldi TI - Nodal and paranodal antibody-associated neuropathies AID - 10.1136/practneurol-2021-002960 DP - 2021 Aug 01 TA - Practical Neurology PG - 284--291 VI - 21 IP - 4 4099 - http://pn.bmj.com/content/21/4/284.short 4100 - http://pn.bmj.com/content/21/4/284.full SO - Pract Neurol2021 Aug 01; 21 AB - Within the last decade, antibodies targeting the node and paranode of myelinated peripheral nerves have been increasingly identified in patients with acquired immune-mediated neuropathies, commonly termed ‘nodo-paranodopathies’. Crucially, these patients often present with additional clinical features not usually seen with the most common immune-mediated neuropathies, Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy, and respond poorly to conventionally used immunomodulatory therapies. Emerging evidence that these are pathologically distinct diseases has further prompted the use of more targeted treatment, such as the B cell depleting monoclonal antibody rituximab, which has been reported to significantly improve functional outcomes in this subset of patients. We provide an overview of the emerging clinical and serological phenotypes in patients with specific nodal/paranodal antibodies, the practicalities of antibody testing and current evidence supporting the use of non-standard therapies.