Niño Uribe Maria Fernanda, Van Cutsem Gilles, Kerschen Philippe
Neurology Department, Centre Hospitalier de Luxembourg, Luxembourg ville, Luxembourg
University of Luxembourg, Esch-sur-Alzette, Luxembourg.
BMJ Case Rep. 2025 Apr 2;18(4):e261902. doi: 10.1136/bcr-2024-261902.
Our patient is a woman who developed a subacute amnestic syndrome, followed by cognitive impairment, headache, temporal lobe epilepsy and hyponatraemia. An early-stage asymptomatic breast cancer was identified on positron emission tomography (PET) scan. After treatment with immunotherapy with high-dose corticosteroids, intravenous immunoglobulins, upfront rituximab and excision of the tumour, the patient gradually recovered. Partial retrograde and anterograde amnesia persisted during the initial weeks, followed by slow and steady improvement. Autoimmune encephalitis is a severe neurological disorder associated with antibodies against neuronal cell-surface or intracellular onconeural proteins. Anti-leucine-rich glioma inactivated 1 (LGI1) predominantly affects males in the seventh decade of life, and less than 10% of all cases are associated with cancer. Paraneoplastic cases have been associated with malignant thymoma, neuroendocrine tumours and mesothelioma and usually had Morvan syndrome, in which serum antibodies are more frequently directed against CASPR2 than against LGI1. We report the first well-documented case of anti-LGI1 limbic encephalitis in a woman with newly diagnosed breast cancer.
我们的患者是一名女性,她先是出现了亚急性遗忘综合征,随后出现认知障碍、头痛、颞叶癫痫和低钠血症。正电子发射断层扫描(PET)发现了早期无症状性乳腺癌。在接受大剂量皮质类固醇免疫治疗、静脉注射免疫球蛋白、早期使用利妥昔单抗以及肿瘤切除术后,患者逐渐康复。最初几周仍存在部分逆行性和顺行性遗忘,随后缓慢而稳定地好转。自身免疫性脑炎是一种严重的神经系统疾病,与针对神经元细胞表面或细胞内肿瘤相关神经蛋白的抗体有关。抗富含亮氨酸胶质瘤失活1(LGI1)抗体主要影响70岁左右的男性,所有病例中不到10%与癌症相关。副肿瘤性病例与恶性胸腺瘤、神经内分泌肿瘤和间皮瘤有关,通常患有莫尔旺综合征,其中血清抗体更常针对接触蛋白相关蛋白2(CASPR2)而非LGI1。我们报告了首例有充分记录的抗LGI1边缘性脑炎病例,患者为一名新诊断的乳腺癌女性。