Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
Department of Neurology, Graduate School of Medicine, Chiba University, Chiba, Japan.
J Neuroimmunol. 2020 Dec 15;349:577424. doi: 10.1016/j.jneuroim.2020.577424. Epub 2020 Oct 9.
Myasthenia gravis (MG) is an autoantibody-mediated disease of the neuromuscular junction. The neuromuscular junction damage associated with MG is caused by anti-acetylcholine receptor (AChR) antibody and complements. Recently, eculizumab (an anti-C5 monoclonal antibody) was approved for patients with anti-AChR antibody-positive generalized refractory MG. Here, we report a Japanese man with MG who well responded to eculizumab, but experienced acute severe worsening of myasthenic symptoms 2 months after its discontinuation. Plasmapheresis did not improve his symptoms; hence, eculizumab was re-administered, resulting in a dramatic response within a week. This is an informative case because eculizumab discontinuation in patients with MG has been very rarely reported. If eculizumab treatment is clinically well effective and AChR antibody titer does not decrease, clinicians should be aware that acute and critical deterioration of MG may occur after the eculizumab discontinuation.
重症肌无力（MG）是一种神经肌肉接头的自身抗体介导的疾病。与 MG 相关的神经肌肉接头损伤是由抗乙酰胆碱受体（AChR）抗体和补体引起的。最近，依库珠单抗（一种抗 C5 单克隆抗体）被批准用于抗 AChR 抗体阳性的全身性难治性 MG 患者。在这里，我们报告了一例日本男性 MG 患者，他对依库珠单抗反应良好，但在停用依库珠单抗 2 个月后出现急性严重肌无力症状恶化。血浆置换未能改善他的症状；因此，重新给予依库珠单抗，在一周内出现显著反应。这是一个信息丰富的病例，因为 MG 患者停用依库珠单抗的情况非常罕见。如果依库珠单抗治疗在临床上非常有效，且 AChR 抗体滴度没有下降，临床医生应该意识到在停用依库珠单抗后，MG 可能会发生急性和严重恶化。
