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Short and long-term effect of IVIg in demyelinating neuropathy associated with MGUS, experience of a monocentric
M Théaudin1, P Lozeron, C Lacroix
1Service de neurologie adultes, CHU Bicêtre, 78, rue du Général-Leclerc, 94270 Le Kremlin-Bicêtre, France. marie.theaudin@bct.aphp.fr
Background:
The optimal treatment for demyelinating neuropathy associated with MGUS and anti-MAG neuropathy is not known.
Methods:
We retrospectively studied the efficacy of IVIg in 14 patients with DN-MGUS (seven IgM and seven IgG/A) and seven with anti-MAG neuropathies, treated in our reference center between 2002 and 2007. Patients were clinically evaluated before the first infusion, after the first infusion, and after the last IVIg treatment.
Results:
Anti-MAG neuropathy: after a single infusion, one patient improved and six were stable. At last follow-up (mean: 15.6months [range: 3.5-31], mean number of IVIg courses: 8 [2-33]), one patient maintained her improvement from baseline. DN-MGUS: after a single infusion, nine patients improved (64%), four were stable and one deteriorated further. The factor predictive of short-term response to IVIg was relapsing neuropathy responding better in the walking score analysis (Fisher exact test: P=0.005). At last follow-up (mean: 22.6months [range 2-72], mean number of IVIg courses: seven [1-24]), neurological status improved in four patients, five patients remained stable, including three who are still under regular IVIg, and four had deteriorated. Improvement from baseline persisted for a prolonged period in two patients after IVIg were stopped. Patients who were responders on Norris after the first IVIg course were significantly better responders at long-term follow-up than the others (P=0.001). We report no serious adverse effect.
Conclusion:
IVIg are not very efficient in the management of anti-MAG neuropathies. Nevertheless, they have a frequent short-term beneficial effect in DN-MGUS, which was maintained at long-term follow-up in one-third of our patients. When a DN-MGUS patient is regularly treated by IVIg courses, frequent periodic clinical evaluations must be performed to determine when to stop treatment and switch to another one.
Insights
Intravenous immunoglobulin (IVIg) shows limited efficacy for anti-MAG neuropathies but offers short-term benefits for demyelinating neuropathy with monoclonal gammopathy of undetermined significance (DN-MGUS), with some long-term maintenance. Regular monitoring is crucial for DN-MGUS patients undergoing IVIg treatment.
Area of Science:
- Neurology
- Immunology
- Clinical Therapeutics
Background:
- Optimal treatment for demyelinating neuropathy (DN) associated with monoclonal gammopathy of undetermined significance (MGUS) and anti-MAG neuropathy remains unknown.
- Investigating the efficacy of intravenous immunoglobulin (IVIg) in these specific neurological conditions is critical for patient management.
Purpose of the Study:
- To retrospectively evaluate the efficacy of IVIg in patients diagnosed with DN-MGUS and anti-MAG neuropathies.
- To identify factors predicting treatment response and long-term outcomes in these patient groups.
Main Methods:
- Retrospective study of 14 DN-MGUS and 7 anti-MAG neuropathy patients treated with IVIg between 2002 and 2007.
- Clinical evaluations were performed before, after the first infusion, and after the last IVIg treatment course.
Main Results:
- IVIg demonstrated limited efficacy in anti-MAG neuropathies, with only one patient showing sustained improvement.
- In DN-MGUS patients, IVIg provided a frequent short-term beneficial effect (64% improved after single infusion), with sustained improvement in one-third at long-term follow-up.
- Relapsing neuropathy predicted better short-term response, and initial responders showed significantly better long-term outcomes (P=0.001). No serious adverse effects were reported.
Conclusions:
- IVIg is not highly efficient for managing anti-MAG neuropathies.
- IVIg offers a frequent short-term benefit in DN-MGUS, maintained long-term in a subset of patients.
- Regular clinical evaluations are essential for DN-MGUS patients on IVIg to optimize treatment duration and consider alternative therapies.
