60-year-old gentleman with medical history of hypertension, thyroid nodules, who various months prior to initial presentation developed chronic sinusitis, followed weeks after by left eye uveitis, in the setting of positive MPO/C-ANCA antibodies. Granulomatosis with Polyangiitis (GPA) suspected, patient started on immunosuppressive therapy. Treatment was eventually discontinued due to Herpes Zoster Virus Infection and recently identified latent Tuberculosis.  
Months after, patient developed intractable headache with horizontal diplopia. Examination revealed right oculomotor, trochlear, and abducens paresis, complicated with ipsilateral optic neuritis.  Two months after patient developed contralateral eye involvement consisting of oculomotor, trochlear, and abducens paresis with optic neuritis.

On neuroimaging patient with bilateral mastoiditis, dural meningeal enhancement and thickening. Lumbar puncture revealed elevated opening pressure and inflammatory changes (WBC 27, Protein 165 mg/dL, Glucose 86 mg/dL), negative cytology, negative CSF Acid Fast, and Lyme titers. Additional lab work demonstrated elevated inflammatory markers, positive MPO/C-ANCA, normal IgG4, negative syphilis antibodies, ANA titers, and rheumatoid factor.

Patient underwent meningeal biopsy that showed fibrous thickening with chronic inflammatory changes, no evidence of vasculitis, granulomatous changes or malignancy. Temporal artery biopsy normal. Patient managed with intravenous steroids with partial improvement of symptoms.