Churg-Strauss Syndrome: An Uncommon Cause of Intracerebral Hemorrhage

2013 
A58-year-oldmanwithasthma, chronic sinusitis, andnasal polyposis presented with a 1-day history of headache, blurry vision, and vomiting. His blood pressure was 170/88mmHg. Neurological examinationwas significant for right homonymous hemianopsia. Initial laboratory resultswere remarkable for peripheral bloodeosinophilia (25%; normal, 0%-8.5%). A computed tomographic scan of theheadshowedan intracranialhemorrhage in the leftoccipital lobe (Figure1A).Emergentevacuationof the intracranialhemorrhagewas performed, and brain tissue was sent for routine biopsy. On postoperativeday2, hedevelopedcoughand shortness of breath.Oxygen saturation was 90%. A general examination revealed coarse crackles bilaterally on chest auscultation. Diffuse alveolar hemorrhage was noted on a computed tomographic scan of the chest (Figure 1B).Hewas intubated for respiratory failure. Furtherworkup revealed an elevated myeloperoxidase–antineutrophil cytoplasmic antibody (>1000EU; normal, 0-21 EU). Brain tissue pathologic study results showed an intense eosinophilic vasculitis (Figure 2A andB).AdiagnosisofChurg-StraussSyndrome(CSS)wasmade.The patientreceivedpulsecyclophosphamide infusionanda5-daycourse of pulse intravenous methylprednisone (1000 mg/d). His respiratory parameters improved, and he was weaned off the ventilator. Hewasdischargedandprescribedoral prednisoneandmonthly cyclophosphamide infusions. Brain magnetic resonance imaging (Figure 2C) obtained 6weeks later showed a small residual left occipital hematoma,withnoevidenceof chronic ischemic sequelaeor microhemorrhages. At the 3-month follow-up, the patient’s neuro-
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