依加替莫德治疗静脉注射免疫球蛋白反应不良的格林-巴-罗综合征1例报告。

IF 2.2 3区 医学 Q3 CLINICAL NEUROLOGY
Bai Qi Hu, Sen Xiang Wu, Qiao Wen Tong, Hua Ye, Ping Ping Jin
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引用次数: 0

摘要

背景:格林-巴勒综合征(GBS)是一种由免疫球蛋白G (IgG)自身抗体介导的周围神经病变,可引起急性弛缓性麻痹。Miller-Fisher综合征(MFS)是GBS的一种变体,在某些情况下,MFS可能与GBS重叠(MFS-GBS重叠综合征),可能延缓疾病的进展。除了静脉注射免疫球蛋白(IVIg)和血浆交换(PLEX),这是目前的临床护理标准。然而,至少20-30%的患者在IVIg或PLEX期间或之后不久出现急性呼吸衰竭,约3-7%的患者死亡。Efgartigimod是一种靶向新生儿Fc受体(FcRn)的拮抗剂,在治疗GBS方面显示出前景。病例介绍:我们提出一个病例与MFS-GBS重叠综合征谁检测阳性血清神经节苷脂IgG抗体。尽管完成了规定的IVIg药物治疗,但患者的症状继续恶化,最终出现呼吸衰竭。随后,艾夫加替莫用于治疗,剂量为800毫克,共给药4次。患者症状缓解,仅剩眼外麻痹。此外,使用依加替莫可使血清IgG水平逐渐下降。这种治疗方案是首次报道。结论:对于IVIg反应较差的MFS-GBS重叠综合征患者,我们发现依夫加替莫德(800 mg, 4次)序贯治疗是安全有效的,并且可以缩短病程。Efgartigimod有望通过持续、彻底的基础和临床研究,在治疗GBS方面取得重大进展。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Efgartigimod for treating Guillain-Barré syndrome with poor response to intravenous immunoglobulin: a case report.

Background: Guillain-Barré syndrome (GBS) is a peripheral neuropathy mediated by immunoglobulin G (IgG) autoantibodies, which can cause acute flaccid paralysis. Miller-Fisher syndrome (MFS) is a variant of GBS, and in some cases, MFS may overlap with GBS (MFS-GBS overlap syndrome), potentially delaying the disease's progression. Apart from intravenous immunoglobulin (IVIg) and plasma exchange (PLEX), which are currently the clinical standard of care. However, at least 20-30% of patients develop acute respiratory failure during or shortly after IVIg or PLEX, and about 3-7% of patients die. Efgartigimod is an antagonist that targets the neonatal Fc receptor (FcRn) and shows promise in the treatment of GBS.

Case presentation: We present a case with MFS-GBS overlap syndrome who tested positive for serum ganglioside IgG antibodies. Despite completing a prescribed course of IVIg medication, the patient's symptoms continued to worsen, and she eventually developed respiratory failure. Subsequently, efgartigimod was used for treatment at a dose of 800 mg, administered four times in total. The patient's symptoms were relieved, leaving only external ophthalmoplegia. Additionally, using efgartigimod caused a gradual drop in the serum IgG level. This treatment regimen is the first reported.

Conclusion: For MFS-GBS overlap syndrome patients with poor IVIg response, we discovered that sequential treatment with efgartigimod (800 mg, four doses) was safe, effective, and could reduce the course of the disease. Efgartigimod is anticipated to represent a significant advancement in treating GBS through ongoing, thorough basic and clinical research.

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来源期刊
BMC Neurology
BMC Neurology 医学-临床神经学
CiteScore
4.20
自引率
0.00%
发文量
428
审稿时长
3-8 weeks
期刊介绍: BMC Neurology is an open access, peer-reviewed journal that considers articles on all aspects of the prevention, diagnosis and management of neurological disorders, as well as related molecular genetics, pathophysiology, and epidemiology.
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