Desai Krisha, Aneja Ankur, Luthra Munish
Department of Medicine, School of Medicine, J. Willis Hurst Internal Medicine Residency Program, Emory University, Atlanta, Georgia.
Department of Medicine, Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, School of Medicine, Emory University, Atlanta, Georgia.
Lung India. 2021 Nov-Dec;38(6):577-580. doi: 10.4103/lungindia.lungindia_893_20.
Paraneoplastic and autoimmune encephalitis (AIE) syndromes describe a range of inflammatory disorders of the brain. "Classic" paraneoplastic encephalitis syndromes occur due to a remote neoplasm and are associated with antibodies that target intracellular neuronal proteins while the more recently described AIE syndromes are not always paraneoplastic and occur in association with antibodies that target cell-surface neuronal receptors (e.g., anti-NMDA receptor, anti-LGI1, anti-GABA receptor). Diagnosis can be difficult and delayed due to nonspecific clinical, imaging, and laboratory findings, and in those syndromes associated with a neoplasm, the neurologic syndromes often precede the cancer diagnosis. We present a case of a 64-year-old patient diagnosed with anti-GABA receptor encephalitis that subsequently revealed an underlying small cell lung cancer without a primary lung tumor. This case highlights the clinical challenge in diagnosing immune-mediated encephalitis, its methodical work up, and subsequent management.
副肿瘤性和自身免疫性脑炎(AIE)综合征描述了一系列脑部炎症性疾病。“经典”副肿瘤性脑炎综合征由远处肿瘤引起,与靶向细胞内神经元蛋白的抗体相关,而最近描述的AIE综合征并不总是副肿瘤性的,且与靶向细胞表面神经元受体的抗体(如抗N-甲基-D-天冬氨酸受体、抗富含亮氨酸胶质瘤失活蛋白1、抗γ-氨基丁酸受体)相关。由于非特异性的临床、影像学和实验室检查结果,诊断可能困难且延迟,在那些与肿瘤相关的综合征中,神经综合征往往先于癌症诊断出现。我们报告一例64岁诊断为抗γ-氨基丁酸受体脑炎的患者,随后发现其存在潜在的小细胞肺癌,但未发现原发性肺肿瘤。该病例突出了免疫介导性脑炎诊断中的临床挑战、其系统的检查方法及后续管理。