Cerne Denise, Massa Federico, Mora Marco, Morbelli Silvia, Roccatagliata Luca, Rebella Giacomo, Villani Flavio, Bozzano Federica, Uccelli Antonio, Benedetti Luana, Cabona Corrado
Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy.
IRCCS, Ospedale Policlinico San Martino, Genoa, Italy.
Heliyon. 2024 Oct 18;10(20):e39586. doi: 10.1016/j.heliyon.2024.e39586. eCollection 2024 Oct 30.
Autoimmune encephalitis (AE) is an immune-mediated condition that induces brain inflammation due to several neural-specific autoantibodies. The main triggering and predisposing factors are infections, genetics, the use of immune checkpoint inhibitors and tumors. We report a case of a 57-year-old male with a biopsy-confirmed Langerhans cell histiocytosis (LCH) and a concomitant anti-LGI1 encephalitis discussing a possible relationship in the pathogenesis of these phenomena. Only sporadic cases of AE developing in the context of histiocytic neoplasms have been described and there are no other reports on the relationship between LGI1-AE and LCH worldwide due to small number of cases.
自身免疫性脑炎(AE)是一种免疫介导的疾病,由多种神经特异性自身抗体引起脑部炎症。主要的触发因素和易感因素包括感染、遗传、免疫检查点抑制剂的使用和肿瘤。我们报告了一例57岁男性病例,经活检确诊为朗格汉斯细胞组织细胞增多症(LCH),并伴有抗LGI1脑炎,探讨了这些现象发病机制中可能存在的关系。仅描述了在组织细胞肿瘤背景下发生的散发性AE病例,由于病例数量少,全球尚无关于LGI1-AE与LCH之间关系的其他报道。