Eosinophilic granulomatosis with polyangiitis (EGPA), or traditionally termed Churg Strauss syndrome was first described in 1951 by Churg and Strauss. It is a rare primary systemic necrotizing vasculitis affecting small and medium sized vessels .Perinuclear antineutrophil cytoplasmic antibody (p-ANCA) is positive in up to 40%–50% of cases [1]. This multisystem disease is typically characterized by 3 phases which include prodromal phase (with atopic disease, allergic rhinitis, and bronchial asthma), eosinophilic phase (peripheral blood eosinophilia or eosinophilic infiltration of multiple organs) and a vasculitic phase of which constitutional symptoms is a presage. Asthma and lung disease are the cardinal clinical features of EGPA and are present in more than 90% of the cases. Neurological involvement in EGPA is rare. Even in the presence of infrequent involvement of CNS in EGPA, it is usually seen in the later phase of the disease [1]
. CNS is affected in 6% to 10% of patients with EGPA and it causes encephalopathy, ischemic infarcts or haemorrhages. (Murthy et al.,
2013)[2].This is a unique case of a patient with EGPA presenting with pan CNS and gut involvement at the diagnosis without lung or upper airway
involvement.