Abstract

A 27-year-old singer presented with difficulty singing. A left-sided vocal cord palsy was noted and he was treated with steroids and antibiotics with some improvement. A few weeks later he developed diplopia and dysphagia. Examination revealed left-sided VI, IX and X cranial nerve palsies. Blood results demonstrated raised CRP. Cranial MRI revealed basal meningo-dural enhancement with marrow oedema of the left petrous bone and surrounding soft tissue swelling. A left sigmoid sinus thrombosis was present. The abnormalities were thought to be in keeping with skull base osteomyelitis. CSF examination revealed normal constituents. An open left mastoid-exploration failed to find evidence of infection. Despite broad-spectrum antibiotics, progression continued with left-sided VIII and bilateral XII nerve involvement. Repeat MRI showed a basal meningitis. An auto-immune screen on admission was normal but ANCAs had not been included. The possibility of Wegner's granulomatosis (WG) was considered and his c-ANCA was strongly positive with anti-proteinase-3 of 39. Warfarin therapy precluded an early biopsy. He was treated with IV steroids, followed by IV Cyclophosphamide and Rituximab for localised WG. The patient's VIII, IX, X, XII nerve involvement has improved. Localised WG with pachymeningits is rare but treatable and all patients presenting with multiple cranial nerve palsies should have ANCA requested.