Gazzola Sébastien, Delmont Emilien, Franques Jérôme, Boucraut José, Salort-Campana Emmanuelle, Verschueren Annie, Sagui Emmanuel, Hubert Anne-Michèle, Pouget Jean, Attarian Shahram
Referral Centre for Neuromuscular Disorders and ALS, Hospital La Timone, Marseille, France; Department of Neurology, Hospital Saint-Anne, Toulon, France.
Referral Centre for Neuromuscular Disorders and ALS, Hospital La Timone, Marseille, France; Aix-Marseille University, CNR2M, CNRS UMR 7286, Medicine Faculty, Marseille, France.
J Neurol Sci. 2017 Jun 15;377:144-148. doi: 10.1016/j.jns.2017.04.015. Epub 2017 Apr 12.
To identify factors associated with efficacy of rituximab (RTX) infusions in patients with anti-myelin associated glycoprotein (MAG) neuropathy.
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.
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.
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.
确定与抗髓鞘相关糖蛋白(MAG)神经病患者中利妥昔单抗(RTX)输注疗效相关的因素。
对33例接受RTX治疗的抗MAG神经病患者进行回顾性评估。所有患者均接受了神经学、生物学和电生理检查。良好反应定义为在6个月或最后一次随访时总体神经病变限制量表(ONLS)至少提高1分。如果在治疗前一年ONLS至少增加2分,则疾病进展定义为亚急性。
10例患者(30%)在RTX治疗6个月后病情改善,20例中的6例(30%)在最后一次随访时(平均42个月)病情改善。对RTX的反应与疾病发作时的亚急性进展和下肢近端无力显著相关。改善与电生理数据和抗MAG抗体滴度无关。
本研究表明,RTX可能对一部分抗MAG神经病患者有效,特别是那些下肢近端无力或亚急性进展的患者。