| Summary: | <p>Although there are many diseases which can cause cavernous sinus syndrome, granulomatosis with polyangiitis (GPA) is rare. This paper reports a case of GPA in cavernous sinus to illustrate the important role of angiitis in cavernous sinus syndrome, which neurologists should pay more attention to. A 37-year-old woman presented palsy of left cranial nerves (CN) II, III, IV, VI and the branches of CN V. MRI showed an enhanced mass in the left cavernous sinus. Laboratory tests showed cytoplasmic ANCA was positive. The nasal mucosa biopsy revealed an inflammatory reaction, while a former renal biopsy had confirmed crescentic glomerulonephritis. The patient was diagnosed as cavernous sinus syndrome with GPA. After the therapy of pulse corticosteroids, the patient was given cyclophosphamide and tacrolimus as a long-term sequential therapy, which achieved a good response. GPA is not a common cause of cavernous sinus syndrome. Clinicians should have the insight into the potential pathogeny of cavernous sinus syndrome. Especially for female patients, the ANCA test may have more important value. Corticosteroids and immunosuppressants are effective treatment methods for GPA.</p><p> </p><p><strong>DOI: </strong>10.3969/j.issn.1672-6731.2015.05.015</p>
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