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Rheumatol Int. 2012 May;32(5):1431-5. doi: 10.1007/s00296-011-1915-x. Epub 2011 Mar 29.

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

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Department of Rheumatology and Clinical Immunology/Allergology, Bern University Hospital, Bern, Switzerland.


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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