Toro Jaime, Cuellar-Giraldo David, Duque Alejandra, Minota Karla, Patiño Jorge, García Manuel
*Department of Neurology, University Hospital of the Fundación Santa Fe de Bogotá †School of Medicine, El Bosque University ‡School of Medicine, University of Los Andes, Bogotá, Colombia.
Cogn Behav Neurol. 2017 Sep;30(3):125-128. doi: 10.1097/WNN.0000000000000134.
Paraneoplastic limbic encephalitis is an autoimmune syndrome characterized by the acute or subacute onset of encephalopathy, memory loss, confusion, temporal lobe seizures, and behavioral and mood changes. Although most patients with paraneoplastic limbic encephalitis have antineuronal antibodies, advances in the field now permit the diagnosis without autoantibody test results. In this case illustrating the new diagnostic criteria, we report a 70-year-old woman who was brought to the emergency room after the acute onset of cognitive impairment, altered mental status, and choreoathetoid movements. Brain magnetic resonance imaging showed hyperintense signals in both temporal lobes, and a chest computed tomogram revealed a thymoma. Because the patient met current diagnostic criteria for autoimmune limbic encephalitis, we were able to start treatment before her antibody tests were processed. The patient received immunotherapy and her tumor was resected. Her choreoathetoid movements disappeared and her other neurologic symptoms improved. Her cerebrospinal fluid proved to be negative for paraneoplastic limbic encephalitis antibodies. Most but not all patients with paraneoplastic limbic encephalitis associated with thymoma have evidence of paraneoplastic antibodies. Prompt management of the underlying malignancy determines whether patients survive and may minimize future cognitive and functional impairment. Practicing neurologists and psychiatrists should be aware of this diagnosis.
副肿瘤性边缘叶脑炎是一种自身免疫综合征，其特征为急性或亚急性起病的脑病、记忆力减退、意识模糊、颞叶癫痫发作以及行为和情绪改变。尽管大多数副肿瘤性边缘叶脑炎患者存在抗神经元抗体，但该领域的进展使得现在即使没有自身抗体检测结果也能做出诊断。在这个说明新诊断标准的病例中，我们报告了一名70岁女性，她在急性出现认知障碍、精神状态改变和舞蹈手足徐动症后被送往急诊室。脑部磁共振成像显示双侧颞叶有高信号，胸部计算机断层扫描显示有胸腺瘤。由于该患者符合自身免疫性边缘叶脑炎的当前诊断标准，我们在其抗体检测结果出来之前就能够开始治疗。患者接受了免疫治疗，其肿瘤也被切除。她的舞蹈手足徐动症消失，其他神经症状有所改善。她的脑脊液检测结果显示副肿瘤性边缘叶脑炎抗体为阴性。大多数但并非所有与胸腺瘤相关的副肿瘤性边缘叶脑炎患者都有副肿瘤抗体的证据。对潜在恶性肿瘤的及时处理决定了患者是否能存活，并可能将未来的认知和功能损害降至最低。执业神经科医生和精神科医生应了解这一诊断。
