Abstract：
Contactin-associated protein-like 2 (CASPR2) and leucine-rich glioma-inactivated 1 (LGI1) are essential components of the voltage-gated Kv1 potassium channel complex and are extensively expressed in both central and peripheral nervous system. Autoimmune CASPR2 and LGI1 disorders commonly present with Morvan syndrome (Mos) and/or limbic encephalitis, but whether Guillain-Barré syndrome (GBS) is a specific clinical phenotype is unknown. Here, we first reported an adult patient with dual CASPR2 and LGI1 antibodies in both serum and cerebrospinal fluid, who initially presented with a GBS-like syndrome and developed a typical MoS and respiratory paralysis, with a rapid resolution of his neurological symptoms and disappearance of autoantibodies after treatment with plasma exchange. Additionally, we also provided an overview of the previously reported GBS cases associated with CASPR2 or LGI1 antibodies. These cases expand the phenotypic spectrum of CASPR2 and LGI1 autoimmune syndromes, implying that these two antigens, especially CASPR2, are likely to participate in the etiology of GBS as a potential new target antigen, which deserves further exploration.
摘要：
接触素相关蛋白样2（CASPR2）和富含亮氨酸的神经胶质瘤灭活1（LGI1）是电压门控Kv1钾通道复合物的必需成分，并在中枢和周围神经系统中广泛表达。自身免疫性CASPR2和LGI1疾病通常伴有Morvan综合征（Mos）和/或边缘叶脑炎，但格林-巴利综合征(GBS)是否是一种特定的临床表型尚不清楚.这里,我们首先报道了一名成人患者，血清和脑脊液中同时存在双重CASPR2和LGI1抗体，最初表现为GBS样综合征，并发展为典型的MoS和呼吸麻痹，在血浆置换治疗后，他的神经系统症状迅速消退，自身抗体消失。此外,我们还概述了以前报道的与CASPR2或LGI1抗体相关的GBS病例.这些病例扩大了CASPR2和LGI1自身免疫综合征的表型谱，暗示这两种抗原，尤其是CASPR2,很可能作为潜在的新靶抗原参与GBS的病因学,值得进一步探索。
