Kitazaki Yuki, Shirafuji Norimichi, Takaku Naoko, Yamaguchi Tomohisa, Enomoto Soichi, Ikawa Masamichi, Yamamura Osamu, Nakamoto Yasunari, Hamano Tadanori
Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Fukui, Japan.
Department of Aging and Dementia, Faculty of Medical Sciences, University of Fukui, Fukui, Japan.
eNeurologicalSci. 2021 Nov 24;25:100382. doi: 10.1016/j.ensci.2021.100382. eCollection 2021 Dec.
Autoimmune basal ganglia encephalitis causes neurological symptoms such as parkinsonism associated with basal ganglia lesions. Here, we report a case of autoimmune basal ganglia encephalitis without retinal lesions or malignancy harboring anti-recoverin antibodies. The patient was a 67-year-old Japanese woman who developed anorexia, parkinsonism, and disturbance of consciousness 7 days before admission. Brain magnetic resonance imaging showed hyperintense bilateral basal ganglia lesions on fluid-attenuated inversion recovery images. F-fluorodeoxyglucose-positron emission tomography showed no malignancy in the trunk, and dopamine transporter single-photon emission computed tomography with dopamine transporters revealed reduced radiotracer uptake in the basal ganglia. Further, anti-recoverin IgG antibodies were detected in serum immunoblot. Based on the clinical and imaging findings, the patient was diagnosed with autoimmune basal ganglia encephalitis with anti-recoverin antibodies and administered high-dose immunoglobulins (HD-IVIG), which led to an improvement in clinical symptoms. Anti-recoverin antibodies are paraneoplastic antibodies that explicitly bind to Ca-binding proteins in the retina and cause retinopathy. This pathological sequence is defined as cancer-associated retinopathy (CAR). However, in our case, autoimmune basal ganglia encephalitis developed without CAR syndrome or malignancy. Clinicians should be aware of the possibility of autoimmune basal ganglia encephalitis showing anti-recoverin antibodies but no CAR syndrome or malignancy, which should be treated with HD-IVIG therapy.
自身免疫性基底节脑炎可导致诸如与基底节病变相关的帕金森综合征等神经症状。在此，我们报告一例无视网膜病变或恶性肿瘤但携带抗恢复蛋白抗体的自身免疫性基底节脑炎病例。患者为一名67岁的日本女性，入院前7天出现厌食、帕金森综合征和意识障碍。脑部磁共振成像显示在液体衰减反转恢复图像上双侧基底节病变呈高信号。F-氟脱氧葡萄糖正电子发射断层扫描显示躯干无恶性肿瘤，多巴胺转运体单光子发射计算机断层扫描显示基底节中放射性示踪剂摄取减少。此外，血清免疫印迹检测到抗恢复蛋白IgG抗体。基于临床和影像学表现，该患者被诊断为携带抗恢复蛋白抗体的自身免疫性基底节脑炎，并给予大剂量免疫球蛋白（HD-IVIG）治疗，临床症状有所改善。抗恢复蛋白抗体是副肿瘤抗体，可特异性结合视网膜中的钙结合蛋白并导致视网膜病变。这种病理过程被定义为癌症相关性视网膜病变（CAR）。然而，在我们的病例中，自身免疫性基底节脑炎在无CAR综合征或恶性肿瘤的情况下发生。临床医生应意识到存在显示抗恢复蛋白抗体但无CAR综合征或恶性肿瘤的自身免疫性基底节脑炎的可能性，对此应采用HD-IVIG治疗。
