Ripellino P, Dinacci D, Conti M, Galli G, Frediani L, Rossi P, Gobbi C
Department of Neurology, Neurocenter of Southern Switzerland EOC, Ospedale Regionale di Lugano, Lugano, Switzerland.
Faculty of Biomedical Sciences, Università della Svizzera Italiana, Lugano, Switzerland.
Eur J Neurol. 2025 Aug;32(8):e70308. doi: 10.1111/ene.70308.
Guillain-Barré Syndrome (GBS) is an autoimmune disease that can cause flaccid tetraplegia. Efgartigimod, a monoclonal antibody that inhibits the neonatal receptor (FcRn), increases IgG degradation.
A 60-year-old lady was admitted with acute flaccid tetraparesis, requiring mechanical ventilation within 12 h. Nerve conduction studies were compatible with acute inflammatory demyelinating polyneuropathy (AIDP). No anti-ganglioside or anti-nodal/paranodal antibody could be identified in serum. Despite receiving standard treatment with plasma exchange (PLEX), the patient remained severely tetraparetic, bedridden, and mechanically ventilated. Human immunoglobulin 2 g/kg was given, without obtaining significant improvement. One month after disease onset, the patient received efgartigimod (10 mg/kg intravenously) weekly for four doses.
We report the results of this treatment on muscle strength, patient disability, and nerve conduction studies. The tolerability profile was satisfactory, and the patient was able to walk alone after 4 months from disease onset.
The potential role of efgartigmod in GBS should be explored in future clinical trials.
吉兰 - 巴雷综合征（GBS）是一种可导致弛缓性四肢瘫的自身免疫性疾病。艾加莫德是一种抑制新生儿受体（FcRn）的单克隆抗体，可增加IgG降解。
一名60岁女性因急性弛缓性四肢轻瘫入院，在12小时内需要机械通气。神经传导检查结果与急性炎症性脱髓鞘性多发性神经病（AIDP）相符。血清中未检测到抗神经节苷脂或抗结/旁结抗体。尽管接受了血浆置换（PLEX）的标准治疗，但患者仍严重四肢轻瘫、卧床且需机械通气。给予人免疫球蛋白2g/kg，病情无明显改善。发病1个月后，患者接受艾加莫德（10mg/kg静脉注射），每周1次，共4剂。
我们报告了该治疗对肌肉力量、患者残疾情况及神经传导检查的结果。耐受性良好，发病4个月后患者能够独立行走。
未来的临床试验应探索艾加莫德在GBS中的潜在作用。
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