The Way Back from Tetraplegia, or Rare Neurological Manifestations of Eosinophil Granulomatosis with Polyangiitis

Central nervous system (CNS) involvement is an extremely rare manifestation in eosinophilic granulomatosis with polyangiitis (EGPA), associated with a poor prognosis. Here we present a case of 50-year-old female patient previously long-lasting treated with asthma who presented initially with extreme eosinophilia (56%) and severe progressive ascending paresis similar to Guillain-Barré syndrome leading to tetraplegia. After navigating through diagnostic mazes; the diagnosis of EGPA was established based on eosinophilia, anti-neutrophil cytoplasmic antibody positivity (MPO-ANCA), asthma, eosinophil granulomatosis in the gastrointestinal tract, and severe peripheral nervous system involvement complicated with rare central nervous granulomatosis and ischaemia. With combined immunosuppressive and immunomodulatory treatment including high-dose corticosteroids, rituximab and intravenous immunoglobulin along with symptomatic treatment and planned rehabilitation over 6 months our patient recovered gradually from tetraplegia and adverse events such as severe infections and osteoporotic fractures. Now from a 2-year perspective we can conclude a successful treatment leading to decrease in all of her symptoms. Due to persistent eosinophilia after steroid tapering, she is currently on mepolizumab maintaining treatment and demonstating continuous improvement of motor and sensory functions. Thanks to periodically repeated rehabilitation, she became self-sufficient and returned to her previous job. Our case represents that EGPA patients should be treated in a centre of expertise due to the rarity of disease and complexity of diagnosis and treatment. Careful multidisciplinary cooperation, huge effort of the patient and supportive environment can show a way back from immune-mediated tetraplegia.