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慢性运动神经病:传统治疗失败后对β-1a干扰素的反应

Chronic motor neuropathies: response to interferon-beta1a after failure of conventional therapies.

作者信息

Martina I S, van Doorn P A, Schmitz P I, Meulstee J, van der Meché F G

机构信息

Department of Neurology, University Hospital Rotterdam, Dr Molewaterplein, The Netherlands.

出版信息

J Neurol Neurosurg Psychiatry. 1999 Feb;66(2):197-201. doi: 10.1136/jnnp.66.2.197.

Abstract

OBJECTIVES

The effect of interferon-beta1a (INF-beta1a; Rebif) was studied in patients with chronic motor neuropathies not improving after conventional treatments such as immunoglobulins, steroids, cyclophosphamide or plasma exchange.

METHODS

A prospective open study was performed with a duration of 6-12 months. Three patients with a multifocal motor neuropathy and one patient with a pure motor form of chronic inflammatory demyelinating polyneuropathy were enrolled. Three patients had anti-GM1 antibodies. Treatment consisted of subcutaneous injections of IBF-beta1a (6 MIU), three times a week. Primary outcome was assessed at the level of disability using the nine hole peg test, the 10 metres walking test, and the modified Rankin scale. Secondary outcome was measured at the impairment level using a slightly modified MRC sumscore.

RESULTS

All patients showed a significant improvement on the modified MRC sumscore. The time required to walk 10 metres and to fulfil the nine hole peg test was also significantly reduced in the first 3 months in most patients. However, the translation of these results to functional improvement on the modified Rankin was only seen in two patients. There were no severe adverse events. Motor conduction blocks were partially restored in one patient only. Anti-GM1 antibody titres did not change.

CONCLUSION

These findings indicate that severely affected patients with chronic motor neuropathies not responding to conventional therapies may improve when treated with INF-beta1a. From this study it is suggested that INF-beta1a should be administered in patients with chronic motor neuropathies for a period of up to 3 months before deciding to cease treatment. A controlled trial is necessary to confirm these findings.

摘要

目的

研究干扰素β-1a(INF-β-1a;利比)对经免疫球蛋白、类固醇、环磷酰胺或血浆置换等传统治疗后仍无改善的慢性运动神经病患者的疗效。

方法

进行了一项为期6至12个月的前瞻性开放研究。纳入3例多灶性运动神经病患者和1例纯运动型慢性炎症性脱髓鞘性多发性神经病患者。3例患者有抗GM1抗体。治疗方法为皮下注射INF-β-1a(6 MIU),每周3次。主要结局通过九孔插针试验、10米步行试验和改良Rankin量表评估残疾程度。次要结局通过稍加修改的医学研究委员会(MRC)总分在损伤水平进行测量。

结果

所有患者的改良MRC总分均有显著改善。大多数患者在最初3个月内,10米步行时间和完成九孔插针试验所需时间也显著缩短。然而,仅2例患者在改良Rankin量表上的功能有所改善。未出现严重不良事件。仅1例患者的运动传导阻滞部分恢复。抗GM1抗体滴度未改变。

结论

这些发现表明,对传统治疗无反应的重度慢性运动神经病患者,使用INF-β-1a治疗可能会有所改善。本研究表明,对于慢性运动神经病患者,在决定停止治疗前,应给予INF-β-1a治疗长达3个月。需要进行对照试验以证实这些发现。

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