副肿瘤性 Kelch 样蛋白 11 抗体相关小脑和边缘脑炎伴转移性“耗竭性”精原细胞瘤——一种可怕的现象。
Paraneoplastic Kelch-like protein 11 antibody-associated cerebellar and limbic encephalitis caused by metastatic "burned-out" seminoma - A scar(r)y phenomenon.
机构信息
Department of Pathology, Forensic and Insurance Medicine, Semmelweis University, Budapest, Hungary.
Department of Neurology, Semmelweis University, Budapest, Hungary; János Szentágothai Doctoral School of Neurosciences, Semmelweis University, Budapest, Hungary.
出版信息
J Neuroimmunol. 2023 May 15;378:578073. doi: 10.1016/j.jneuroim.2023.578073. Epub 2023 Mar 23.
INTRODUCTION
The diagnosis of paraneoplastic neurologic syndromes is challenging when the primary tumor masquerades as scar tissue (i.e. "burned-out").
METHODS
Case report.
RESULTS
A 45-year-old male patient presented with progressive cerebellar symptoms and hearing loss. Initial screening for malignancy and extensive testing of paraneoplastic and autoimmune neuronal antibodies gave negative results. Repeated whole-body FDG-PET CT revealed a single paraaortic lymphadenopathy, metastasis of a regressed testicular seminoma. Anti-Kelch-like protein-11 (KLHL11) encephalitis was finally diagnosed.
CONCLUSION
Our case highlights the importance of continued efforts to find an often burned-out testicular cancer in patients with a highly unique clinical presentation of KLHL11 encephalitis.
简介
当原发性肿瘤伪装成疤痕组织（即“耗竭性”）时，副肿瘤性神经综合征的诊断具有挑战性。
方法
病例报告。
结果
一名 45 岁男性患者出现进行性小脑症状和听力损失。最初对恶性肿瘤进行筛查以及对副肿瘤性和自身免疫性神经元抗体进行广泛检测均呈阴性结果。重复全身 FDG-PET CT 显示单个主动脉旁淋巴结病，睾丸精原细胞瘤消退转移。最终诊断为抗 Kelch 样蛋白 11（KLHL11）脑炎。
结论
我们的病例强调了在具有高度独特 KLHL11 脑炎临床表现的患者中，继续努力寻找常常被“耗竭”的睾丸癌的重要性。
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