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静脉注射免疫球蛋白作为多灶性运动神经病的短期和长期治疗:40例患者对静脉注射免疫球蛋白反应及其预测标准的回顾性研究

Intravenous immunoglobulin as short- and long-term therapy of multifocal motor neuropathy: a retrospective study of response to IVIg and of its predictive criteria in 40 patients.

作者信息

Léger J-M, Viala K, Cancalon F, Maisonobe T, Gruwez B, Waegemans T, Bouche P

机构信息

Department of Neurology, Hôpital de la Salpêtriere, Paris, France.

出版信息

J Neurol Neurosurg Psychiatry. 2008 Jan;79(1):93-6. doi: 10.1136/jnnp.2007.121756.

Abstract

OBJECTIVE

To document short-term and long-term responses to a single type of intravenous immunoglobulin (IVIg) in a large cohort of patients with multifocal motor neuropathy (MMN).

METHODS

A retrospective study was conducted in 40 patients with MMN included on ENMC Workshop criteria, and treated with periodic IVIg infusions between 1995 and 2003. The short-term response was defined as improvement of at least 1 point on the MRC score in at least two affected muscles at 6 months. The population comprised 22 treatment-naïve patients (who had never received IVIg before inclusion), and 18 previously treated patients. For the long-term evaluation (>6 months), the patients were classified into three groups according to the dependency or not on periodic IVIg. In addition, changes in conduction block (CB) and predictive criteria for response to IVIg were explored.

RESULTS

The MRC score significantly improved (p<0.0001) in 14 (70%; 95% CI 0.46 to 0.88) of the 20 treatment-naïve patients (missing data for 2 patients). None of the predictive criteria studied were found to be significant. At the end of follow-up (mean of 2.2+/-2.0 years), only 8 of the 40 patients (22%) had significant remission, whereas 25 patients (68%) were dependent on periodic IVIg infusions. The number of CBs decreased or remained unchanged in 12 treatment-naïve patients and increased in 2 such patients.

CONCLUSIONS

This study confirmed a significantly high short-term response to IVIg of patients with MMN, but showed contrasted results in long-term follow-up. No predictive factors for response to IVIg were found.

摘要

目的

记录一大群多灶性运动神经病(MMN)患者对单一类型静脉注射免疫球蛋白(IVIg)的短期和长期反应。

方法

对40例符合欧洲神经肌肉中心(ENMC)研讨会标准且在1995年至2003年间接受定期IVIg输注治疗的MMN患者进行了一项回顾性研究。短期反应定义为在6个月时至少两个受影响肌肉的医学研究委员会(MRC)评分至少提高1分。该人群包括22例初治患者(纳入前从未接受过IVIg治疗)和18例既往接受过治疗的患者。对于长期评估(>6个月),根据是否依赖定期IVIg将患者分为三组。此外,还探讨了传导阻滞(CB)的变化以及IVIg反应的预测标准。

结果

20例初治患者中的14例(70%;95%置信区间0.46至0.88)MRC评分显著改善(p<0.0001)(2例患者数据缺失)。所研究的预测标准均未发现具有显著性。随访结束时(平均2.2±2.0年),40例患者中只有8例(22%)有显著缓解,而25例患者(68%)依赖定期IVIg输注。12例初治患者的CB数量减少或保持不变,2例患者的CB数量增加。

结论

本研究证实MMN患者对IVIg有显著较高的短期反应,但长期随访结果显示存在差异。未发现IVIg反应的预测因素。

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