PDF(Pubmed)
Abstract：
Patients with anti-GABA-A receptor encephalitis characteristically experience therapy-refractory epileptic seizures. General anesthesia is often required to terminate refractory status epilepticus. The immunologic mechanisms leading to antibody formation remain to be elucidated. Described triggers of anti-GABA-A autoimmunity are tumors, mainly thymomas, and herpes simplex encephalitis.
We present a young woman with prediagnosis of relapse remitting multiple sclerosis (MS), treated with interferons, natalizumab, and alemtuzumab. Six months after one and only cycle of alemtuzumab, speech arrest and behavioral changes with aggressive and anxious traits appeared. She showed increasing motor convulsions resulting in focal status epilepticus.
Anti-GABA-A receptor antibodies in CSF and serum were confirmed in different external laboratories, in a more extensive analysis after antibodies against NMDAR, CASPR2, LGI1, GABABR, and AMPAR were ruled out during in-house examination. Clinical condition improved temporarily with cortisone therapy, plasmapheresis, and IVIG but deteriorated rapidly after steroid discontinuation, resulting in brain biopsy. On histopathologic confirmation consistent with anti-GABA-A receptor antibody-associated CNS inflammation, completing the first rituximab cycle, continuing oral corticosteroids and supplementing immunosuppression with cyclosporine A led to quick recovery.
Our case describes a severe autoantibody-induced encephalitis in a young patient with MS, with alemtuzumab as a potential trigger for anti-GABA-A receptor encephalitis.
We present a young woman with prediagnosis of relapse remitting multiple sclerosis (MS), treated with interferons, natalizumab, and alemtuzumab. Six months after one and only cycle of alemtuzumab, speech arrest and behavioral changes with aggressive and anxious traits appeared. She showed increasing motor convulsions resulting in focal status epilepticus.
Anti-GABA-A receptor antibodies in CSF and serum were confirmed in different external laboratories, in a more extensive analysis after antibodies against NMDAR, CASPR2, LGI1, GABABR, and AMPAR were ruled out during in-house examination. Clinical condition improved temporarily with cortisone therapy, plasmapheresis, and IVIG but deteriorated rapidly after steroid discontinuation, resulting in brain biopsy. On histopathologic confirmation consistent with anti-GABA-A receptor antibody-associated CNS inflammation, completing the first rituximab cycle, continuing oral corticosteroids and supplementing immunosuppression with cyclosporine A led to quick recovery.
Our case describes a severe autoantibody-induced encephalitis in a young patient with MS, with alemtuzumab as a potential trigger for anti-GABA-A receptor encephalitis.
摘要：
目的：抗GABA-A受体脑炎患者特征性经历治疗难治性癫痫发作。通常需要全身麻醉来终止难治性癫痫持续状态。导致抗体形成的免疫机制仍有待阐明。描述的抗GABA-A自身免疫的触发因素是肿瘤，主要是胸腺瘤,和单纯疱疹性脑炎.
方法：我们介绍了一名年轻女性，患有复发缓解型多发性硬化症（MS），用干扰素治疗，那他珠单抗,和阿仑珠单抗。在一个也是唯一一个阿仑珠单抗周期后六个月，出现了具有攻击性和焦虑特征的言语停滞和行为改变。她表现出增加的运动性惊厥导致局灶性癫痫持续状态。
结果：在不同的外部实验室中证实了CSF和血清中的抗GABA-A受体抗体，在抗NMDAR抗体之后的更广泛的分析中，CASPR2,LGI1,GABABR,在内部检查中排除了AMPAR。可的松治疗的临床状况暂时改善，血浆置换,和IVIG，但在类固醇停药后迅速恶化，导致脑活检。组织病理学证实与抗GABA-A受体抗体相关中枢神经系统炎症一致，完成第一个利妥昔单抗周期，继续口服皮质类固醇和补充环孢素A免疫抑制导致快速恢复。
结论：我们的病例描述了一名年轻MS患者的严重自身抗体诱导的脑炎，阿仑珠单抗作为抗GABA-A受体脑炎的潜在触发因素。
方法：我们介绍了一名年轻女性，患有复发缓解型多发性硬化症（MS），用干扰素治疗，那他珠单抗,和阿仑珠单抗。在一个也是唯一一个阿仑珠单抗周期后六个月，出现了具有攻击性和焦虑特征的言语停滞和行为改变。她表现出增加的运动性惊厥导致局灶性癫痫持续状态。
结果：在不同的外部实验室中证实了CSF和血清中的抗GABA-A受体抗体，在抗NMDAR抗体之后的更广泛的分析中，CASPR2,LGI1,GABABR,在内部检查中排除了AMPAR。可的松治疗的临床状况暂时改善，血浆置换,和IVIG，但在类固醇停药后迅速恶化，导致脑活检。组织病理学证实与抗GABA-A受体抗体相关中枢神经系统炎症一致，完成第一个利妥昔单抗周期，继续口服皮质类固醇和补充环孢素A免疫抑制导致快速恢复。
结论：我们的病例描述了一名年轻MS患者的严重自身抗体诱导的脑炎，阿仑珠单抗作为抗GABA-A受体脑炎的潜在触发因素。
