Wegener granulomatosis manifesting as meningitis

Abstract

The authors describe a unique presentation of Wegener granulomatosis (WG) manifesting predominantly as meningitis. Magnetic resonance imaging demonstrated diffuse meningeal enhancement, including the pia mater, in a 28-year-old man with meningitis. A diagnosis of atypical WG was based on the findings of a dural biopsy sample and an elevated cytoplasmic antineutrophil cytoplasmic antibody (cANCA) titer, although the patient did not have any of the lesions common to WG. Immunosuppressive therapy was quite effective. With treatment, the meningeal enhancement resolved and the cANCA titer normalized. Meningeal granulomatosis as the sole lesion in WG has never been reported in the literature. This atypical course of WG should be noted.