[长期使用利妥昔单抗后严重且依赖静脉注射免疫球蛋白的多灶性运动神经病伴传导阻滞的改善情况]

[Improvement of severe and intravenous immunoglobulin-dependent multifocal motor neuropathy with conduction block after long-term rituximab].

作者信息

Michaud A, Delmont E, Jeandel P-Y, Desnuelle C

机构信息

Centre de référence des maladies neuromusculaires et SLA, hôpital de l'Archet, 1, route Saint-Antoine-de-Ginestière, BP 3979, 06202 Nice, cedex 3, France.

出版信息

Rev Neurol (Paris). 2011 Dec;167(12):916-20. doi: 10.1016/j.neurol.2011.02.042. Epub 2011 Jul 12.

DOI:10.1016/j.neurol.2011.02.042

PMID:21752414

Abstract

INTRODUCTION

Some patients suffering from multifocal motor neuropathy with conduction blocks (MMNCB) are still disabled after treatment with intravenous immunoglobulin (IVIg).

CASE REPORT

We report the benefits of a combination of rituximab (RTX) and IVIg in the case of a 72-year-old man with MMNCB.

DISCUSSION

Despite an IVIg treatment, the patient had severe motor weakness of the four limbs which limited daily living activity. Azathioprine, mycophenolate mofetyl and cyclophosphamid did not improve the patient's status. Adjunction of rituximab to IVIg therapy increased muscle strength measured on MRC sum score and reduced disability evaluated on ONLS (Overall Neuropathy Limitation Scale) score in the long term (37 months). In spite of the improvement of his neurological status, the patient remained dependent on IVIg.

CONCLUSION

RTX could be proposed as a long-term complementary treatment for some severe cases of IVIg-dependent NMMBC. These results must be confirmed in a randomized controlled study.

摘要

引言

一些患有多灶性运动神经病伴传导阻滞（MMNCB）的患者在接受静脉注射免疫球蛋白（IVIg）治疗后仍有残疾。

病例报告

我们报告了利妥昔单抗（RTX）与IVIg联合治疗一名72岁MMNCB男性患者的疗效。

讨论

尽管接受了IVIg治疗，该患者仍有严重的四肢运动无力，限制了日常生活活动。硫唑嘌呤、霉酚酸酯和环磷酰胺均未改善患者状况。长期（37个月）来看，在IVIg治疗中加用利妥昔单抗可提高医学研究委员会（MRC）总分评估的肌肉力量，并降低总体神经病变限制量表（ONLS）评分评估的残疾程度。尽管其神经状态有所改善，但该患者仍依赖IVIg。