Neuro-Behçet's syndrome: differential diagnosis and management

Behçet's syndrome (BS) is a multisystem disease of unknown etiology, characterized by chronic relapsing cardinal symptoms of orogenital ulcers, uveitis, and different skin lesions. Its major pathologic feature is vasculitis. Neuro-Behçet's syndrome (NBS) is defined as a constellation of neurologic manifestations with characteristic neuropathologic findings, usually confirmed by ancillary investigations, in patients who meet the diagnostic criteria for BS. Neurologic manifestations of the syndrome are more common in male patients and have been reported to occur in anywhere from 5% to 50% of BS patients, depending on their geographical region. NBS primarily affects the central nervous system and includes parenchymal and nonparenchymal involvement. Peripheral neuropathy and myopathy are rare. Immunosuppression is widely used for treatment.