Atypical presentation of Behçet's disease with central nervous system involvement successfully treated with infliximab

Studer, U; Ruehe, B; Waldegg, G; Vajtai, I; Escher, R; Aeberli, D (2012). Atypical presentation of Behçet's disease with central nervous system involvement successfully treated with infliximab. Rheumatology international, 32(5), pp. 1431-5. Berlin: Springer 10.1007/s00296-011-1915-x

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Central nervous system involvement is a rare and serious complication of Behçet's disease (BD). Herein, we describe a patient with an atypical central lesion, who experienced progressive hypesthesia of the right arm and sensory loss of the trigeminal nerve together with intense headache. A repeated biopsy was necessary to conclusively establish the diagnosis of BD. Therapy with infusions of infliximab led to a remarkable full remission. TNFα-blocking therapy was successfully replaced by azathioprine. The present well-illustrated case demonstrates the difficulty of establishing the diagnosis of BD with central nervous system involvement, the dramatic benefit of short given TNF-α-blocking agent, and the long-term remission with azathioprin.

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