Department of Neurology, St Josef Hospital, Ruhr University Bochum, Bochum, Germany.
JAMA Neurol. 2013 Aug;70(8):1056-9. doi: 10.1001/jamaneurol.2013.143.
A patient with a Caspr2 autoantibodies-associated syndrome had an unusual clinical triad and an excellent response to B-cell-anergizing therapy using the humanized monoclonal antibody tocilizumab directed against the interleukin 6 (IL-6) receptor.
A 55-year-old man had an atypical clinical triad of epilepsy, dysarthria, and paroxysmal kinesigenic dystonia, and a high titer of Caspr2 antibodies was detected in his serum and cerebrospinal fluid. Screening for underlying neoplasias was negative. With initial methylprednisolone sodium succinate and alternate treatment using plasma exchange and immunoabsorption as well as subsequent IL-6 receptor blockade through tocilizumab, a complete and stable remission of symptoms has been achieved throughout the follow-up period of 7 months.
In our patient, the implementation of a B-cell-anergizing therapy using tocilizumab, a humanized monoclonal antibody against the IL-6 receptor, has shown an excellent response. Larger case series or even controlled studies are needed to confirm the efficacy of tocilizumab in autoimmune synaptic or presynaptic diseases.
一名患有 Caspr2 自身抗体相关综合征的患者出现了不寻常的三联征，并且对靶向白细胞介素 6（IL-6）受体的人源化单克隆抗体托珠单抗的 B 细胞失能治疗有极好的反应。
一名 55 岁男性出现了癫痫、构音障碍和阵发性运动诱发性运动障碍的非典型三联征，其血清和脑脊液中检测到 Caspr2 抗体的高滴度。进行了潜在肿瘤的筛查，结果为阴性。最初使用甲泼尼龙琥珀酸钠治疗，随后使用血浆置换和免疫吸附进行交替治疗，以及随后使用托珠单抗进行 IL-6 受体阻断，在 7 个月的随访期间，症状完全和稳定缓解。
在我们的患者中，使用靶向白细胞介素 6 受体的人源化单克隆抗体托珠单抗进行 B 细胞失能治疗显示出极好的反应。需要更大的病例系列甚至对照研究来证实托珠单抗在自身免疫性突触或突触前疾病中的疗效。
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