Anti-LGI1 encephalitis and Langerhans cell histiocytosis: Two associated entities? A case report

Heliyon. 2024 Oct 18;10(20):e39586. doi: 10.1016/j.heliyon.2024.e39586. eCollection 2024 Oct 30.

Abstract

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.

Keywords: Case report; IL-6; LGI1 encephalitis; Langerhans cell histiocytosis; Paraneoplastic.

Publication types

  • Case Reports