Sébastien Gazzola1, Emilien Delmont2, Jérôme Franques3, José Boucraut4, Emmanuelle Salort-Campana3, Annie Verschueren3, Emmanuel Sagui5, Anne-Michèle Hubert6, Jean Pouget3, Shahram Attarian3. 1. Referral Centre for Neuromuscular Disorders and ALS, Hospital La Timone, Marseille, France; Department of Neurology, Hospital Saint-Anne, Toulon, France. 2. Referral Centre for Neuromuscular Disorders and ALS, Hospital La Timone, Marseille, France; Aix-Marseille University, CNR2M, CNRS UMR 7286, Medicine Faculty, Marseille, France. Electronic address: emilien.delmont@ap-hm.fr. 3. Referral Centre for Neuromuscular Disorders and ALS, Hospital La Timone, Marseille, France. 4. Aix-Marseille University, CNR2M, CNRS UMR 7286, Medicine Faculty, Marseille, France; Department of Immunology, Hospital La Conception, Marseille, France. 5. Department of Neurology, Hospital Laveran, Marseille, France. 6. Department of Immunology, Hospital La Conception, Marseille, France.
Abstract
OBJECTIVE: To identify factors associated with efficacy of rituximab (RTX) infusions in patients with anti-myelin associated glycoprotein (MAG) neuropathy. METHODS: 33 patients with anti-MAG neuropathy treated with RTX were retrospectively evaluated. All patients underwent neurological, biological, and electrophysiological examinations. Good response was defined as an improvement of at least one point of the Overall Neuropathy Limitation Scale (ONLS) at 6months or at the last follow-up. Disease evolution was defined as sub-acute if the ONLS increased by at least 2 points the year before therapy. RESULTS: Ten patients (30%) were improved 6months after RTX and 6/20 (30%) at the last follow-up (mean 42months). Response to RTX was significantly associated with subacute evolution and proximal weakness of the lower limbs at the onset of disease. Improvement was not correlated with electrophysiological data and anti-MAG antibodies titers. DISCUSSION: This study suggests that RTX may be efficacious in a sub-population of patients with anti-MAG neuropathy, particularly in those with proximal weakness of the lower limbs or sub-acute evolution.
OBJECTIVE: To identify factors associated with efficacy of rituximab (RTX) infusions in patients with anti-myelin associated glycoprotein (MAG) neuropathy. METHODS: 33 patients with anti-MAG neuropathy treated with RTX were retrospectively evaluated. All patients underwent neurological, biological, and electrophysiological examinations. Good response was defined as an improvement of at least one point of the Overall Neuropathy Limitation Scale (ONLS) at 6months or at the last follow-up. Disease evolution was defined as sub-acute if the ONLS increased by at least 2 points the year before therapy. RESULTS: Ten patients (30%) were improved 6months after RTX and 6/20 (30%) at the last follow-up (mean 42months). Response to RTX was significantly associated with subacute evolution and proximal weakness of the lower limbs at the onset of disease. Improvement was not correlated with electrophysiological data and anti-MAG antibodies titers. DISCUSSION: This study suggests that RTX may be efficacious in a sub-population of patients with anti-MAG neuropathy, particularly in those with proximal weakness of the lower limbs or sub-acute evolution.