Kelch-like蛋白11抗体（KLHL11-IgG）在2019年首次被描述为副肿瘤性神经系统（PNS）的标志物。它们主要与睾丸生殖细胞瘤（tGCT）相关联。报告了两名KLHL11-IgG脑炎患者，并对文献进行了全面的回顾。患者1在10年前已摆脱tGCT，但他逐渐出现了癫痫性眩晕和复视。症状出现几天后，开始使用类固醇（CS）治疗。患者2曾短暂出现双重视觉，但自行缓解。视觉问题持续了7个月，之后他又出现了渐进性小脑综合症。发病1年后，开始使用CS治疗。两个患者的最初磁共振成像（MRI）均无异常，但脑脊液分析显示慢性炎症。两个患者均为阳性KLHL11-IgG（患者1仅在CSF中，患者2在血清中）。到目前为止，恶性肿瘤筛查尚未发现任何活动潜在恶性肿瘤的迹象。我们发现总共有112名患有KLHL11-IgG脑炎的患者的15篇论文。大多数患者（n=82）具有小脑综合症，伴随或无中脑桥炎症状。最常见的症状是共济失调（n=82）和眩晕（n=47），其次是眼动障碍（n=35）和听力障碍（n=31）。84名患者中的80位患有GCT作为潜在的肿瘤。我们的病例表明了KLHL11-IgG脑炎的典型症状。早期诊断和治疗是必不可少的。与其他PNS一样，需要临床意识，并需要进一步研究，特别是针对治疗管理方面。本文受版权保护。保留一切权利。

Kelch-like protein 11 antibodies (KLHL11-IgG) were first described in 2019 as a marker of paraneoplastic neurological syndromes (PNS). They have mostly been associated with testicular germ cell tumours (tGCT).Report of two patients with KLHL11-IgG encephalitis and comprehensive review of literature.Patient 1 had been in remission from a tGCT 10 years prior. He developed episodic vertigo and diplopia progressing over a few days. Treatment with corticosteroids (CS) was started few days after symptom-onset. Patient 2 had transient diplopia which resolved spontaneously. Visual problems persisted for 7 months, when he additionally developed a progressive cerebellar syndrome. 1 year after onset, CS treatment was started. Initial MRIs were unremarkable in both patients, but analysis of cerebrospinal fluid (CSF) revealed chronic inflammation. KLHL11-IgG was positive in both patients (Patient 1 only in CSF, patient 2 in serum). Neoplastic screening has so far not revealed any signs of active underlying malignancy. We found 15 publications of 112 patients in total with KLHL11-IgG encephalitis. Most patients (n=82) had a cerebellar syndrome with or without signs of rhombencephalitis. The most common symptoms were ataxia (n=82) and vertigo (n=47), followed by oculomotor disturbances (n=35) and hearing disorders (n=31). 80 of 84 patients had a GCT as an underlying tumour.Our cases demonstrate classical symptoms of KLHL11-IgG encephalitis. An early diagnosis and therapy is imperative. As with other PNS, clinical awareness is needed and further studies are required especially in regard to therapeutic management.This article is protected by copyright. All rights reserved.