Epilepsy Center Bethel (M.D.O., C.B., M.B.-H., C.G.B.), Krankenhaus Mara, Bielefeld; Department of Neurology (M.D.O.), University Hospital Erlangen; Department of Neurology (K.S.G., H.L., H.W., N.M.), University of Münster; Department of Nephrology (M.A.-T., R.V.), Bethel-EvKB, Bielefeld; Department of Medicine D (M.B., H.P., G.T.), Division of General Internal Medicine, Nephrology and Rheumatology, University Hospital of Münster; and Laboratory Krone (D.M.), Bad Salzuflen, Germany.
Neurol Neuroimmunol Neuroinflamm. 2016 Feb 26;3(2):e207. doi: 10.1212/NXI.0000000000000207. eCollection 2016 Apr.
It was hypothesized that in encephalitides with autoantibodies directed to CNS surface antigens an antibody-removing intervention might speed up recovery.
The outcome of autoimmune encephalitis in 19 patients with antibodies against surface antigens (leucine-rich, glioma inactivated 1 [LGI1], n = 3; contactin-associated protein-2 [CASPR2], n = 4; NMDA receptor [NMDAR], n = 7) and intracellular antigens (glutamic acid decarboxylase [GAD], n = 5) after immunoadsorption in addition to corticosteroid therapy was evaluated retrospectively. Modified Rankin scale (mRS) scores and data on seizures, memory, and antibody titers directly after immunoadsorption (early follow-up) and after a median of 4 months (late follow-up) were compiled.
Immediately after immunoadsorption, 9 of 14 patients with antibodies against LGI1, CASPR2, or NMDAR (64%), but none with GAD antibodies, had improved by at least one mRS point. Five of the 7 patients with LGI1 or CASRP2 antibodies had become seizure-free, and 2 patients with NMDAR antibodies had a memory improvement of more than 1 SD of a normal control population. At late follow-up, 12 of 14 patients with surface antibodies had improved (86%), and none of the patients with GAD antibodies.
It is suggested that addition of immunoadsorption to immunosuppression therapy in patients with surface antibodies may accelerate recovery. This supports the pathogenic role of surface antibodies.
This study provides Class IV evidence that immunoadsorption combined with immunosuppression therapy is effective in patients with autoimmune encephalitis with surface antibodies.
据推测，在针对中枢神经系统表面抗原的自身抗体脑炎中，抗体清除干预可能会加速恢复。
回顾性评估了 19 例针对表面抗原（富含亮氨酸的胶质瘤失活蛋白 1 [LGI1]，n = 3；接触蛋白相关蛋白 2 [CASPR2]，n = 4；NMDA 受体 [NMDAR]，n = 7）和细胞内抗原（谷氨酸脱羧酶 [GAD]，n = 5）自身抗体的自身免疫性脑炎患者在免疫吸附联合皮质类固醇治疗后的结果。编译了免疫吸附后（早期随访）和中位数为 4 个月（晚期随访）的改良 Rankin 量表（mRS）评分以及癫痫发作、记忆和抗体滴度的数据。
在免疫吸附后，14 例针对 LGI1、CASPR2 或 NMDAR 的抗体患者中有 9 例（64%）至少改善了 1 个 mRS 点，而 7 例 LGI1 或 CASPR2 抗体患者中有 5 例无癫痫发作，2 例 NMDAR 抗体患者的记忆改善超过正常对照人群的 1 个标准差。在晚期随访时，14 例表面抗体患者中有 12 例（86%）有所改善，而 GAD 抗体患者则没有。
建议在表面抗体患者中，将免疫吸附与免疫抑制治疗联合使用，可能会加速恢复。这支持了表面抗体的致病作用。
这项研究提供了 IV 级证据，表明免疫吸附联合免疫抑制疗法对具有表面抗体的自身免疫性脑炎患者有效。
