Objectives: Behçet's disease is a multisystemic, relapsing, inflammatory disorder of unknown origin. Among Turkish cohorts, 5-15% of patients show involvement of the central nervous system (CNS) at some time during their disease. There are mainly two types of clinical presentation: parenchymal CNS inflammation manifesting mainly as meningoencephalitis of the brainstem, or dural sinus thrombosis. Several drugs like high-dose steroids or immunosuppressive agents, mainly azathioprine, are used in the treatment. For patients who do not respond sufficiently to these agents or are not able tolerate them, other options are needed.
Patients: We are presenting 4 cases with parenchymal neuro-Behçet's disease, where commonly used immunosuppressive drugs could not be continued due to intolerance or inefficacy. However, the patients benefited well from mycophenolate mofetil. The benefit was sustained during 3-7 years of follow-up (median 6.5 years).
Conclusions: Mycophenolate mofetil seems to be an alternative drug in parenchymal neuro-Behçet's disease; however, large controlled studies should be performed for verification of our results.