Treatment of patients with multifocal motor neuropathy with immunoglobulins in clinical practice: the SIGNS registry

Abstract

Objectives:The management of patients with multifocal motor neuropathy (MMN) under everyday clinical conditions has been insufficiently studied. We therefore collected comprehensive observational data on patients with MMN who received intravenous (IV) or subcutaneous (SC) immunoglobulins (IGs) as maintenance therapy. Methods: This was a prospective, noninterventional study (registry) in neurological centres (hospitals and offices) throughout Germany. Results: As of 1 December 2015, 80 patients with MMN were included (mean age 55.4 ± 9.8 years, 67% males, mean disease duration 10.7 ± 10.2 years). The affected limb regions were predominantly distal muscle groups of the upper extremities. On the inflammatory neuropathy cause and treatment (INCAT) scale, 94% of the patients had some disability in the arms and 61% in the legs. At inclusion, 98.8% received IVIG and 1.3% SCIG. Substantial variation was observed between IVIG treatment intervals (every 0.7 to 17.3 weeks) and dosage (0.2-2.1 g/kg body weight received during a single administration; mean monthly dosage, 0.9 g/kg body weight). However, the mean monthly dosage was steady over time. At 1-year follow up, improvement was seen in muscle strength, INCAT and quality of life (QoL) scores (SF-36 questionnaire). Conclusions:The management of patients with MMN in everyday clinical practice demonstrates a wide range of absolute dosages and treatment intervals of IG, supporting the recommended practice of determining treatment dose on an individual patient basis. The improvements in muscle strength and reduction in disability, accompanied by increased QoL, strengthen the case for use of IG as a maintenance treatment for MMN.

Management of patients with malignancies and secondary immunodeficiencies treated with immunoglobulins in clinical practice: Long‐term data of the SIGNS study

“…Forty‐eight centres throughout Germany enrolled SID patients into the study, comprising university and community hospitals, as well as office‐based physicians. Also, patients with PID and neurological autoimmune diseases on IgG treatment were documented (reported elsewhere) …”

IqYmune® is an effective maintenance treatment for multifocal motor neuropathy: A randomised, double‐blind, multi‐center cross‐over non‐inferiority study vs Kiovig®—The LIME Study

“…Secondary efficacy outcomes included the MMRC new 10‐sum score developed specifically for this study, the Rasch‐built MMRC 10‐sum score, the MMRC 14‐sum score (Table ), grip strength measured with a dynamometer for the most affected hand and disability in daily activities, as assessed with the inflammatory neuropathy course and treatment (INCAT) disability score as used in MMN by Stangel et al Total score on this scale ranges from 0 (no signs of disability) to 10 (most severe disability score) and the upper‐limb subscore ranges from 0 to 5.…”

“…Stangel et al 9 Total score on this scale ranges from 0 (no signs of disability) to 10 (most severe disability score) and the upper-limb subscore ranges from 0 to 5.…”

Multifocal Motor Neuropathy: A clinical case study

“…It contains typical IgG antibodies with a broad spectrum of antibody activity and is used to treat patients requiring antibody replacement as well as autoimmune, hematological and neurological disorders (Australian Red Cross, 2018). The mechanism of action of IVIg is not fully understood, however it is thought the antibodies work against the mechanisms of the classical complement pathway in order to prevent the membrane attack (Stangel et al 2016). IVIg neutralises pathogenic antibodies, inhibits antibody production by B cells and suppresses inflammatory mediators produced by T cells (Léger, Guimarães-Costa & Iancu Ferfoglia, 2015).…”