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
Affiliations
Denise Cerne
Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy; Corresponding author.
Federico Massa
Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DINOGMI), University of Genoa, Genoa, Italy; IRCCS, Ospedale Policlinico San Martino, Genoa, Italy
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.
