A case report of influenza-triggered RANBP2-associated acute necrotizing encephalomyelitis in an adult successfully treated with tocilizumab

Here, we report a rare case of a 32-year-old patient presenting with a second episode of influenza A-triggered acute necrotizing encephalomyelitis (ANE) in the context of a Ran-binding protein 2 (RANBP2) -mutation, which was diagnosed after a previous influenza-triggered ANE at the age of eleven. Despite early treatment with corticosteroids, plasmapheresis and intravenous immunoglobulins (IVIG) no meaningful clinical improvement was observed during the first 6 weeks of treatment. As interleukin-6 (IL-6) levels were elevated in both blood and cerebrospinal fluid (CSF), additional therapy with tocilizumab was initiated, leading to clinical improvement from week 11 onward.