BRAIN and NERVE－神経研究の進歩73巻5号

2021年05月発行

増大特集 中枢神経の自己免疫性・炎症性疾患ハンドブック

第2章　疾患各論

抗VGKC複合体抗体と辺縁系脳炎

著者： 田中惠子¹²

所属機関： ¹新潟大学脳研究所モデル動物開発分野 ²福島県立医科大学多発性硬化症治療学講座

ページ範囲：P.605 - P.610

文献概要

抗VGKC抗体は，現在，その構成蛋白のLGI1，CASPR2それぞれを特異的に認識する抗体で検出される。LGI1抗体陽性例の頻度が高く，FBDSと辺縁系脳炎の臨床像を呈する。60代に発症し，男性にやや多い。CASPR2抗体はニューロミオトニアとの関連が知られるが，両抗体の併存例が多く，臨床像も重複する。免疫療法が奏効するものの，認知・記憶障害が残存し，再発が多い。認知症との鑑別が重要である。

参考文献

1）Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, et al: Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 75: 1135-1140, 2004

2）Vincent A, Buckley C, Schott JM, Baker I, Dewar BK, et al: Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain 127: 701-712, 2004

3）Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R: Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis. Lancet Neurol 10: 63-74, 2011

4）Irani SR, Alexander S, Waters P, Kleopa KA, Pettingill P, et al: Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan's syndrome and acquired neuromyotonia. Brain 133: 2734-2748, 2010

5）Binks SNM, Klein CJ, Waters P, Pittock SJ, Irani SR: LGI1, CASPR2 and related antibodies: a molecular evolution of the phenotypes. J Neurol Neurosurg Psychiatry 89: 526-534, 2018

6）van Sonderen A, Thijs RD, Coenders EC, Jiskoot LC, Sanchez E, et al: Anti-LGI1 encephalitis: clinical syndrome and long-term follow-up. Neurology 87: 1449-1456, 2016

7）Gadoth A, Pittock SJ, Dubey D, McKeon A, Britton JW, et al: Expanded phenotypes and outcomes among 256 LGI1/CASPR2-IgG-positive patients. Ann Neurol 82: 79-92, 2017

8）Kaneko J, Kanazawa N, Tominaga N, Kaneko A, Suga H, et al: Practical issues in measuring autoantibodies to neuronal cell-surface antigens in autoimmune neurological disorders: 190 cases. J Neurol Sci 390: 26-32, 2018

9）Gao L, Liu A, Zhan S, Wang L, Li L, et al: Clinical characterization of autoimmune LGI1 antibody limbic encephalitis. Epilepsy Behav 56: 165-169, 2016

10）Loane C, Argyropoulos GPD, Roca-Fernández A, Lage C, Sheerin F, et al: Hippocampal network abnormalities explain amnesia after VGKCC-Ab related autoimmune limbic encephalitis. J Neurol Neurosurg Psychiatry 90: 965-974, 2019

11）Binks S, Varley J, Lee W, Makuch M, Elliott K, et al: Distinct HLA associations of LGI1 and CASPR2-antibody diseases. Brain 141: 2263-2271, 2018

12）van Sonderen A, Ariño H, Petit-Pedrol M, Leypoldt F, Körtvélyessy P, et al: The clinical spectrum of Caspr2 antibody-associated disease. Neurology 87: 521-528, 2016

13）Ohkawa T, Fukata Y, Yamasaki M, Miyazaki T, Yokoi N, et al: Autoantibodies to epilepsy-related LGI1 in limbic encephalitis neutralize LGI1-ADAM22 interaction and reduce synaptic AMPA receptors. J Neurosci 33: 18161-18174, 2013