Stroke revealing granulomatosis

Central nervous system involvement is rare in Wegener granulomatosis. Stroke is the most common event suggestive of the disease. A 35-year-old woman, who was followed for rhinitis and mild asthma, described gradual decline of visual acuity in the right eye over two months, persistent nasal obstruction and fronto-orbital headache since a few weeks. She presented left hemiparesis due to a stroke, associated with exophthalmos and deficits of the optic nerve and abducens of the right eye. The otolaryngological examination found signs of crusty rhinitis and right nasal stenosis. The diagnosis of Wegener's granulomatosis was established on the basis of the clinical findings, radiological aspects and the presence of ANCA. The patient was treated by antiplatelet agents and high-dose corticosteroids associated with immunosuppressive drugs including cyclophosphamide in a monthly bolus. This case illustrates two of the three pathogenic mechanisms that may account for central nervous system involvement in Wegener granulomatosis: vasculitis, extension by contiguity of granulomatous tissue from the nasal cavity or sinuses, and in situ formation of a granuloma into the brain parenchyma or meninges.