Heliyon (Oct 2024)

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

  • Denise Cerne,
  • Federico Massa,
  • Marco Mora,
  • Silvia Morbelli,
  • Luca Roccatagliata,
  • Giacomo Rebella,
  • Flavio Villani,
  • Federica Bozzano,
  • Antonio Uccelli,
  • Luana Benedetti,
  • Corrado Cabona

Journal volume & issue
Vol. 10, no. 20
p. e39586

Abstract

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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.

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