Cerebral vasculitis in Wegener's granulomatosis

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Wegener’s granulomatosis is a necrotizing granulomatous vasculitis of the respiratory tract associated with a focal glomerulonephritis.1–4 Disseminated necrotizing angiitis involving large, medium, and small-sized vessels also comprises a part of the clinical and pathologic spectra. Although the specific pathologic features are well described by Godman and Churg,1 several recent reviews have clarified the renal2 and pulmonary5 histologic findings and discussed improved aspects of treatment.3, 4 There has been less emphasis on the neuropathologic manifestations of Wegener’s granulomatosis. The frequency of nervous system involvement, based upon clinical and pathologic criteria, varies from 22%4 to 54%.6 The purpose of this report is to emphasize neuropathologic involvement in a case of fulminant Wegener’s granulomatosis at autopsy.

Case report

A 53-year-old man was transferred to the Cleve-land Clinic Hospital with diffuse abdominal pain, fever, and pedal edema of 3 weeks’ duration. Proximal muscle weakness and bilateral pedal edema were found on physical examination. Blood pressure was 110/70 mm Hg. The patient was alert and oriented. Roentgenographic examination demonstrated two discrete nodular shadows in the upper right lung field. Urinalysis revealed proteinuria, microhematuria, and numerous granular casts. Hemogram included hemoglobin, 9.3 g/dl; hematocrit, 29.5%; and white blood cell count, 14,800/μl, with a normal differential count. Results of laboratory studies revealed the following values: blood urea nitrogen (BUN), 69 mg/dl and serum creatinine, 5.5mg/dl. Studies from the referring hospital had included electrocardiogram, electroencephalogram, brain scan, skull films, and complete gastrointestinal barium x-ray series. All were normal. During the week following admission, right epididymo-or-chitis, increasing weakness, lethargy, . . .



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