Autoimmune nodopathy is a peripheral neuropathy characterized by acquired motor and sensory deficit with autoantibodies against the node of Ranvier or paranodal region in the peripheral nervous system. The clinical and pathological characteristics of the disease are distinct from that of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and the standard treatment for CIDP is partially effective. Rituximab is a chimeric monoclonal antibody which binds and depletes B cells in peripheral blood. This prospective observational study included 19 patients with autoimmune nodopathy. Participants received intravenous rituximab treatment 100 mg the first day and 500 mg the next day and given every 6 months. The Inflammatory Neuropathy Cause and Treatment (INCAT) disability score, Inflammatory Rasch-Built Overall Disability Scale (I-RODS), Medical Research Council (MRC) sum score, and Neuropathy Impairment Score (NIS) were collected at entry and before the rituximab infusion every 6 months. At the last visit, 94.7% (18/19) of the patients showed clinical improvement on either the INCAT, I-RODS, MRC, or NIS scale. After the first infusion, 9 patients (47.7%) showed improvement on the INCAT score, and 11 patients (57.9%) on cI-RODS. In patients who received more than one rituximab infusion, the improvement of INCAT score and cI-RODS at the last assessment was higher than that after the first infusion. We also observed tapered or withdrawn concomitant oral medications in these patients.
Keywords: Chronic inflammatory demyelinating polyradiculoneuropathy; Peripheral neuropathy; Prospective observational study; Rituximab; Treatment.
© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany.