#5207 central nervous system involvement in eosinophilic granulomatosis with polyangiitis: a diagnostic dilemma

Abstract Background: Central nervous system involvement is extremely rare in eosinophilic granulomatosis with polyangiitis, and associated with very poor prognosis. A high index of suspicion is needed to diagnose this entity and initiate prompt treatment for this life-threatening condition. Case presentation: A 71-year-old man was admitted to our hospital with lower limb weakness, loss of vision in the left eye and diplopia for four weeks. Four months prior to admission he reported unintentional weight loss and wheezing. Laboratory studies revealed anemia, eosinophilia and high antineutrophil cytoplasmic antibodies (ANCA) anti myeloperoxidase (MPO) levels. Brain computerized tomography (CT) was normal. Although corticosteroids were started, addition of other immunosupressants was delayed due to ongoing infection. During the next three days, the patient presented with worsening kidney function and was disoriented, agitated and combative. Lumbar puncture and brain CT scan showed no acute abnormalities. Therefore, plasmapheresis was started. During the third session of plasmapheresis, the patient became drowsy and non-responsive. Brain CT scan revealed subdural hematoma with uncal herniation. Trepanation and initiation of hemodialysis were required. Kidney biopsy revealed focal proliferative glomerulonephritis with fibrocellular crescents. Magnetic resonance imaging and ophthalmological evaluation showed abnormalities compatible with vasculitic disease. Cyclophosphamide was added to prednisolone, with renal and extra renal manifestations improvement. One year later, the patient is autonomous, with right hand paresis, stable kidney function (serum creatinine 2.5 mg/dL) and negative ANCA MPO serology. Conclusion Neurological involvement in GEPA is heterogeneous and requires differential diagnosis with central nervous system infections, adverse effects of corticosteroids and complication of plasmapheresis. The diagnosis is challenging and demands a high level of suspicion. Otherwise, it may lead to delayed, erroneous diagnosis and worse survival.