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慢性炎症性脱髓鞘性多发性神经根神经病患者接受皮下免疫球蛋白治疗的长期神经生理学和临床反应。

Long-term neurophysiological and clinical response in patients with chronic inflammatory demyelinating polyradiculoneuropathy treated with subcutaneous immunoglobulin.

机构信息

I Division of Neurology and Neurophysiopathology, University of Campania "Luigi Vanvitelli", Naples, Italy.

I Division of Neurology and Neurophysiopathology, University of Campania "Luigi Vanvitelli", Naples, Italy.

出版信息

Clin Neurophysiol. 2018 May;129(5):967-973. doi: 10.1016/j.clinph.2018.01.070. Epub 2018 Feb 19.

DOI:10.1016/j.clinph.2018.01.070
PMID:29554579
Abstract

OBJECTIVE

To assess the long-term effects of subcutaneous immunoglobulin (SCIg) on neurophysiological and clinical parameters in patients affected by chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

METHODS

16 drug-naïve CIDP patients, fulfilling the clinical and neurophysiological criteria for typical CIDP, were treated with one cycle of intravenous immunoglobulin (IVIg) (0.4 g/kg/day for 5 consecutive days) and then shifted to the subcutaneous treatment (SCIg) (0.4 g/kg/week) after 4 weeks. Patients were evaluated (i) at the diagnosis (t), (ii) after 4 weeks since the last IVIg infusion (t), and (iii) after long-term treatment with SCIg (t = 12 months; t = 24 months) by (1) neurophysiological parameters of nerve conduction studies and (2) clinical assessment scales for evaluation of (a) strength, by medical research council (MRC) sum score, (b) sensory functions, by inflammatory neuropathy care and treatment (INCAT) sensory sum score and (c) disability, by overall disability sum score (ODSS).

RESULTS

Long-term SCIg treatment induced a significant improvement of neurophysiological parameters, in particular primary demyelinating features of nerve conduction, and clinical variables. Correlation analysis showed (1) a direct positive correlation between the MRC sum score and the amplitude of the distal compound muscle action potential (dCMAP) amplitude, (2) an inverse correlation between dCMAP amplitude and ODSS score, and (3) an inverse correlation between sensory nerve action potential (SNAP) amplitude and the INCAT sensory sum score.

CONCLUSIONS

Our findings revealed a significant long-term effect of the SCIg treatment on the neurophysiological parameters, associated with improvement of global strength, sensory deficits and overall disability in patients with CIDP.

SIGNIFICANCE

Early start of the SCIg treatment after IVIg infusions induced a significant and long-term improvement of clinical and neurophysiological parameters in CIDP patients.

摘要

目的

评估皮下免疫球蛋白(SCIg)对慢性炎症性脱髓鞘性多发性神经病(CIDP)患者神经生理和临床参数的长期影响。

方法

16 名初治 CIDP 患者,符合典型 CIDP 的临床和神经生理标准,接受一个周期的静脉免疫球蛋白(IVIg)(0.4g/kg/天,连续 5 天)治疗,然后在 4 周后转为皮下治疗(SCIg)(0.4g/kg/周)。患者在(1)诊断时(t)、(2)最后一次 IVIg 输注后 4 周(t)和(3)长期 SCIg 治疗后(t=12 个月;t=24 个月)进行评估,通过(1)神经传导研究的神经生理参数和(2)用于评估(a)力量的临床评估量表,即肌研究理事会(MRC)总分、(b)感觉功能,即炎症性神经病护理和治疗(INCAT)感觉总分和(c)残疾,即总体残疾总分(ODSS)。

结果

长期 SCIg 治疗可显著改善神经生理参数,特别是神经传导的原发性脱髓鞘特征和临床变量。相关性分析显示(1)MRC 总分与远端复合肌肉动作电位(dCMAP)振幅之间存在直接正相关,(2)dCMAP 振幅与 ODSS 评分之间存在负相关,(3)感觉神经动作电位(SNAP)振幅与 INCAT 感觉总分之间存在负相关。

结论

我们的发现表明,SCIg 治疗对 CIDP 患者的神经生理参数具有显著的长期影响,与整体力量、感觉缺陷和总体残疾的改善相关。

意义

在 IVIg 输注后早期开始 SCIg 治疗可显著改善 CIDP 患者的临床和神经生理参数。

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