title: ANCA-Negative Churg-Strauss Syndrome Presenting as Acute Multiple Cerebral Infarcts: A Case Report.
abstract: Eosinophilic granulomatosis with polyangiitis (EGPA, previously named Churg-Strauss syndrome) is a form of necrotizing vasculitis occurring in patients with asthma and eosinophilia. Ischemic stroke is a relatively rare complication of the disease. We report a case of a 63-year-old woman with multiple embolic infarcts, hypereosinophilia (for >7 years), and skin rash. Elevated cardiac enzymes and cardiac magnetic resonance imaging were consistent with endomyocarditis. The simultaneous presence of history of asthma, sinusitis, hypereosinophilia, and vasculitis led to the diagnosis of EGPA. This case contributes to the recent debate of the 2 possible presentations of the disease according to the ANCA (antineutrophil cytoplasmic antibodies) status. We furthermore underscore the need for careful differential diagnosis of the "ANCA negative" cases with persistent hypereosinophilia from the idiopathic hypereosinophilic syndrome.
authors.affiliation: Stroke Unit, Mediterraneo Hospital, Athens, Greece. Electronic address: apsychoyio@yahoo.gr.
authors.forename: Klearchos
authors.initials: K
authors.lastname: Psychogios
authors.affiliation: Department of Thrombosis and Hemostasis, Mediterraneo Hospital, Athens, Greece.
authors.forename: Ilias
authors.initials: I
authors.lastname: Evmorfiadis
authors.affiliation: Department of Cardiology, Mediterraneo Hospital, Athens, Greece.
authors.forename: Spyros
authors.initials: S
authors.lastname: Dragomanovits
authors.affiliation: Stroke Unit, Mediterraneo Hospital, Athens, Greece.
authors.forename: Athanasios
authors.initials: A
authors.lastname: Stavridis
authors.affiliation: Stroke Unit, Mediterraneo Hospital, Athens, Greece.
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Takis
authors.affiliation: Department of Pathology, Onassis Cardiac Surgery Center, Athens, Greece.
authors.forename: Loukas
authors.initials: L
authors.lastname: Kaklamanis
authors.affiliation: Stroke Unit, Mediterraneo Hospital, Athens, Greece.
authors.forename: Pantelis
authors.initials: P
authors.lastname: Stathis
mesh_terms: D002544:Cerebral Infarction / Q000000981:diagnostic imaging / Q000503:physiopathology*; D015267:Churg-Strauss Syndrome / Q000000981:diagnostic imaging*; D038524:Diffusion Magnetic Resonance Imaging; D005260:Female; D006801:Humans; D008875:Middle Aged; D018366:Vasculitis, Leukocytoclastic, Cutaneous / Q000000981:diagnostic imaging / Q000503:physiopathology
keywords: Churg–Strauss syndrome; Stroke; hypereosinophilia; vasculitis