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. 2022 Dec;29(12):3611-3622.
doi: 10.1111/ene.15553. Epub 2022 Sep 25.

Efficacy of rituximab in anti-myelin-associated glycoprotein demyelinating polyneuropathy: Clinical, hematological and neurophysiological correlations during 2 years of follow-up

Mattia Parisi  1 , Irene Dogliotti  2 , Michele Clerico  3   4 , Davide Bertuzzo  5 , Giulia Benevolo  4 , Lorella Orsucci  6 , Irene Schiavetti  7 , Roberto Cavallo  8 , Federica Cavallo  3   4 , Simone Ragaini  3   4 , Alessandra Di Liberto  8 , Martina Ferrante  3 , Giulia Bondielli  3 , Carlo Alberto Artusi  1 , Daniela Drandi  3 , Leonardo Lopiano  1 , Bruno Ferrero  1 , Simone Ferrero  3   4
Affiliations

Efficacy of rituximab in anti-myelin-associated glycoprotein demyelinating polyneuropathy: Clinical, hematological and neurophysiological correlations during 2 years of follow-up

Mattia Parisi et al. Eur J Neurol. 2022 Dec.

Abstract

Background and purpose: We evaluated the clinical and neurophysiological efficacy of rituximab (RTX) in a neurophysiologically homogeneous group of patients with monoclonal gammopathy and immunoglobulin M (IgM) anti-myelin-associated glycoprotein antibody (anti-MAG) demyelinating polyneuropathy.

Methods: Twenty three anti-MAG-positive polyneuropathic patients were prospectively evaluated before and for 2 years after treatment with RTX 375 mg/m2 . The Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale (INCAT-ds), modified INCAT sensory score (mISS), Medical Research Council sum score, Patients' Global Impression of Change scale were used, IgM levels were assessed and extensive electrophysiological examinations were performed before (T0) and 1 year (T1) and 2 years (T2) after RTX treatment.

Results: At T1 and T2 there was a significant reduction from T0 both in mISS and in INCAT-ds, with a p value < 0.001 in the inferential Friedman's test overall analysis. Ulnar nerve Terminal Latency Index and distal motor latency significantly changed from T0 to T1 and in the overall analysis (p = 0.001 and p = 0.002), and ulnar nerve sensory nerve action potential (SNAP) amplitude was significantly increased at T2 from T1, with a p value < 0.001 in the overall analysis. Analysis of the receiver-operating characteristic curves showed that a 41.8% increase in SNAP amplitude in the ulnar nerve at T2 from T0 was a fair predictor of a mISS reduction of ≥2 points (area under the curve 0.85; p = 0.005; sensitivity: 90.9%, specificity: 83.3%).

Conclusions: This study suggests that RTX is effective in patients with clinically active demyelinating anti-MAG neuropathy over 2 years of follow-up, and that some neurophysiological variables might be useful for monitoring this efficacy.

Keywords: clinical neurophysiology; haematological disorders; immunomodulatory therapy; polyneuropathy.

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Conflict of interest statement

The authors declare no conflicts of interests.

Figures

FIGURE 1
FIGURE 1
Flow chart showing selection of patients included in this study. anti‐MAG, anti‐myelin‐associated glycoprotein antibody; BTU, Bühlmann titer unit; CIDP, chronic inflammatory demyelinating polyradiculoneuropathy; IgM MG, immunoglobulin M monoclonal gammopathy; MGUS, monoclonal gammopathy of undetermined significance; PDN, paraproteinemic demyelinating immunoglobulin M‐related neuropathy; PN, peripheral neuropathy; WM, Waldenström macroglobulinemia
FIGURE 2
FIGURE 2
Receiver‐operating characteristic (ROC) curve analysis of ulnar nerve sensory nerve action potential (SNAP) amplitude percent increase at 2 years compared with baseline value (%Δ‐SNAP) for prediction of modified inflammatory neuropathy cause and treatment sensory score (mISS) reduction ≥2 points at 2 years from baseline evaluation. AUC, area under the curve
FIGURE 3
FIGURE 3
Ulnar nerve Terminal Latency Index (TLI) and sensory nerve action potential (SNAP) amplitude variation (mean ± SD) during 2‐year follow‐up of 23 patients affected by demyelinating polyneuropathy with anti‐myelin‐associated glycoprotein antibody immunoglobulin M monoclonal gammopathy treated with rituximab
See this image and copyright information in PMC

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