Attademo Laura, De Falco Stefano, Rosanova Mario, Esposito Marcello, Mazio Federica, Foschini Francesca, Santaniello Antonio, Fiore Giovanni, Matano Elide, Manganelli Fiore, Carlomagno Chiara
Department of Clinical Medicine and Surgery Department of Neurosciences, Reproductive Sciences and Odontostomatology Department of Advanced Biomedical Sciences, University Federico II, Naples, Italy.
Medicine (Baltimore). 2018 Mar;97(9):e0011. doi: 10.1097/MD.0000000000010011.
Paraneoplastic limbic encephalitis (PLE) is one of the most common causes of neurologic paraneoplastic syndromes, with unclear pathogenesis. While several reports published in the last decades showed the occurrence of PLE in a variety of cancers, only a few cases have been associated with colon cancer.
In February 2017, a 54-year-old man with clinical history of radically resected colon cancer started first line chemotherapy with FOLFOXIRI plus bevacizumab, after radiological diagnosis of multiple liver and bone metastases. During the third cycle of treatment, the patient developed psychomotor agitation and hallucinations followed by severe consciousness level reduction and cognitive impairment.
Magnetic resonance imaging showed hyperintense signals in both hippocampal areas, insula and right cingulate gyrus on fluid attenuated inversion recovery, diffusion weighted imaging, and T2-weighted images, highly suggestive of limbic encephalitis. Other causes (brain metastases, toxicity of chemotherapeutic agents, and infections) were excluded.
Empirical immunosuppressive treatment (high-dose immunoglobulins and corticosteroids) was administered and chemotherapy was resumed.
A slowly progressive improvement in neurological condition has been observed, even though radiological signs of limbic encephalitis are still evident.
The present case highlights the complex diagnostic process of PLE, and the lack of a standard treatment. Moreover, the absence of correlation between PLE and tumor progression or tumor burden, and the opportunity of treating underlying neoplasm is discussed.
副肿瘤性边缘叶脑炎（PLE）是神经系统副肿瘤综合征最常见的病因之一，其发病机制尚不清楚。尽管过去几十年发表的几份报告显示PLE在多种癌症中都有发生，但仅有少数病例与结肠癌相关。
2017年2月，一名有根治性切除结肠癌病史的54岁男性，在经影像学诊断为多发肝转移和骨转移后，开始接受FOLFOXIRI联合贝伐单抗的一线化疗。在第三个治疗周期中，患者出现精神运动性激越和幻觉，随后意识水平严重下降和认知障碍。
磁共振成像显示在液体衰减反转恢复序列、弥散加权成像和T2加权图像上，双侧海马区、岛叶和右侧扣带回均呈高信号，高度提示边缘叶脑炎。排除了其他病因（脑转移、化疗药物毒性和感染）。
给予经验性免疫抑制治疗（高剂量免疫球蛋白和皮质类固醇）并恢复化疗。
尽管边缘叶脑炎的影像学征象仍然明显，但已观察到神经状况有缓慢的逐渐改善。
本病例突出了PLE复杂的诊断过程以及缺乏标准治疗方法。此外，还讨论了PLE与肿瘤进展或肿瘤负荷之间缺乏相关性，以及治疗潜在肿瘤的时机。
