Wegener’s Granulomatosis presenting with multiple cranial neuropathies.

A 41–year-old man had headaches, hearing loss, and left facial palsy for five months. Examination was remarkable for left seventh nerve palsy, left sided hearing loss and hemi tongue atrophy. MRI showed left cerebellopontine angle dural thickening.(Figure1and 2). Laboratory studies (CSF and blood) were unremarkable for an infectious, neoplastic and autoimmune etiology except for an elevated Anti-neutrophil cytoplasmic antibody titer. A dural biopsy showed chronic inflammation with granuloma formation. He was started on prednisone with plans to start immunosuppresants as outpatient.

Wegener’s Granulomatosis can present with multiple cranial neuropathies as initial clinical manifestation1 due to focal meningeal involvement 2.

Figure1. Coronal MRI T1–weighted image with gadolinium (A and B) showing dural thickening and enhancement along the left cerebellopontine angle and surrounding the left seventh and eighth nerves.

Figure 2. Axial MRI T1–weighted image with gadolinium (A and B) showing dural thickening and enhancement along the left cerebellopontine angle. There is also noted surrounding inflammation extending into the left external auditory canal.