Granulomatosis with CNS involvement: A neuroimaging clinicopathologic correlation

BACKGROUND AND PURPOSE: To present a rare case of a rheumatologic disorder with a central nervous system manifestation. METHODS: A 48-year-old female with history of nasal and auricular pain and inflammation developed bilateral intermittent scleritis that was refractory to several immunosuppressive medications. She later presented with headaches and right upper extremity parasthesias. Brain magnetic resonance imaging (MRI) was obtained and she was referred to a neurosurgeon. RESULTS: Neurological examination did not reveal any focal deficits. Laboratory analysis demonstrated an elevated erythrocyte sedimentation rate (ESR). Brain MRI with and without gadolinium contrast revealed an extra-axial, enhancing, left frontoparietal convexity lesion. The patient was taken to the operating room for biopsy and resection of the mass. Final pathology demonstrated dural granulomatosis with vasculitis, most consistent with intracranial Wegener's granulomatosis. The patient was discharged to follow-up with her rheumatologist for further medical management. CONCLUSIONS: Enhancing, intracranial dural lesions are associated with a variety of disease entities. Wegener's granulomatosis is one such entity and should be considered in the correct context.