Transient Cerebral Ischemic Attack and Delirium Heralding the Vasculitic Phase of Eosinophilic Granulomatosis With Polyangiitis (Churg-Strauss Syndrome)

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic vasculitis characterized by a prodromic history of allergic asthma and sinusitis for many years, followed by an eosinophilic phase and a vasculitic phase. Central nervous system (CNS) involvement associated with EGPA is very rare, in particular as first sign of the vasculitic phase. We describe a patient with transient cerebral ischemic attack and delirium heralding the vasculitic phase of EGPA. We report a case of EGPA (Churg-Strauss syndrome) in a 76-year-old Italian woman with acute neurologic and neuropsychiatric symptoms and history of asthma and sinusitis. She was admitted in stroke unit with acutely developing aphasia, ataxia and delirium without acute lesions on neuroimaging. When she was transferred in neurorehabilitation unit, an antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis was suspected on the basis of anamnesis, facies (saddle nose) and presence of systemic inflammation. She was finally diagnosed with EGPA because of the presence of subclinical demyelinating polyneuropathy, interstitial pulmonary disease and increased myeloperoxidase-specific ANCA (MPO-ANCA). The patient's neurologic symptoms were, therefore, attributed to a vasculitic process, and they improved with steroid treatment. CNS involvement associated with EGPA is very rare and, in this case it represented the first sign of systemic vasculitis after many years of prodromic phase, and it should be considered in patients with acute cerebrovascular disease associated with systemic inflammation or eosinophilia and anamnestic asthma or sinusitis.