Malter M P, Frisch C, Schoene-Bake J C, Helmstaedter C, Wandinger K P, Stoecker W, Urbach H, Surges R, Elger C E, Vincent A V, Bien C G
Department of Epileptology, University of Bonn Medical Center, Bonn, Germany,
J Neurol. 2014 Sep;261(9):1695-705. doi: 10.1007/s00415-014-7408-6. Epub 2014 Jun 17.
In limbic encephalitis (LE) with antibodies (Abs) to the voltage-gated potassium channel complex (VGKC), the Abs are mainly directed to the VGKC-complex proteins, leucine-rich, glioma inactivated 1 protein (LGI1) or contactin-associated protein-like 2 (CASPR-2) or neither. Here, we relate the outcomes of VGKC-LE patients to the presence of Abs to LGI1, CASPR-2 or neither antigen (LGI1/CASPR-2-Ab(-)). Clinical, neuropsychology and MRI data were obtained from patient records for all LE patients from the Bonn Epilepsy Centre positive for VGKC-Abs by radioimmunoprecipitation assay between 2002 and 2011. Eighteen VGKC-LE patients were identified: nine patients (50 %) had LGI1-Abs, three (16 %) had CASPR-2-Abs; and six (33 %) were negative for both LGI1- and CASPR-2-Abs. At first assessment, the groups did not differ clinically or radiologically, but faciobrachial dystonic seizures were only observed in two LGI1-Ab(+) patients. All patients received monthly intravenous methylprednisolone (MP) pulses. At the most recent follow up (median 26 months), thirteen (72 %) were seizure-free, and seizure-freedom rates did not differ between the Ab groups. Hippocampal atrophy had developed in 7/9 LGI1-Ab(+) patients, but in none of the CASPR-2-Ab(+) or LGI/CASPR-2-Ab(-) patients (p = 0.003). While all subgroups improved, memory scores only normalized in six patients (33 %) and LGI1-Ab(+) patients were left with significantly poorer memory than the other two subgroups. Most VGKC-LE patients become seizure-free with pulsed monthly MP, but memory outcome is less favourable. Hippocampal atrophy and poor memory recovery is common in patients with LGI1-Abs and suggests permanent functional damage. More intense immunotherapies could improve outcomes in LGI1-Ab(+)-LE.
在伴有针对电压门控钾通道复合物（VGKC）抗体（Abs）的边缘叶脑炎（LE）中，这些抗体主要针对VGKC复合物蛋白、富含亮氨酸的胶质瘤失活1蛋白（LGI1）或接触蛋白相关蛋白样2（CASPR-2），或者两者都不针对。在此，我们将VGKC-LE患者的预后与针对LGI1、CASPR-2的抗体或两种抗原均不存在（LGI1/CASPR-2-Ab(-)）的情况相关联。通过放射免疫沉淀法对2002年至2011年间波恩癫痫中心所有VGKC-Abs呈阳性的LE患者的临床、神经心理学和MRI数据进行了回顾。共确定了18例VGKC-LE患者：9例患者（50%）有LGI1-Abs，3例（16%）有CASPR-2-Abs；6例（33%）LGI1-Abs和CASPR-2-Abs均为阴性。在首次评估时，各亚组在临床或影像学上无差异，但仅在2例LGI1-Ab(+)患者中观察到面臂肌张力障碍性癫痫发作。所有患者均接受每月一次的静脉注射甲泼尼龙（MP）冲击治疗。在最近一次随访（中位时间26个月）时，13例（72%）患者无癫痫发作，各抗体亚组的无癫痫发作率无差异。7/9例LGI1-Ab(+)患者出现了海马萎缩，但CASPR-2-Ab(+)或LGI/CASPR-2-Ab(-)患者均未出现（p = 0.003）。虽然所有亚组均有改善，但仅6例患者（33%）的记忆评分恢复正常，且LGI1-Ab(+)患者的记忆明显比其他两个亚组差。大多数VGKC-LE患者通过每月一次的MP冲击治疗可实现无癫痫发作，但记忆预后较差。LGI1-Abs患者常见海马萎缩和记忆恢复不佳，提示存在永久性功能损害。更强化的免疫治疗可能改善LGI1-Ab(+)-LE患者的预后。
