Behçet’s Syndrome and the Nervous System Involvement

Behcet’s syndrome (BS) is a chronic, recurrent relapsing multisystem vascular-inflammatory syndrome of unknown etiology that predominantly affects veins. Although BS was initially described by Turkish physician Hulusi Behçet in 1937 as a triad of aphthous stomatitis, genital ulcers, and recurrent uveitis, meantime it is considered a multisystemic disease that extends beyond these symptoms, manifesting with a wide range of clinical findings. The neurologic involvement of BS is termed as Neuro-Behçet’s Syndrome (NBS). These nervous system findings may be associated with either primary nervous system (parenchymal NBS and cerebral venous sinus thrombosis) involvement implying directly related to BS or secondary nervous system involvement indirectly related to BS or its treatment. In approximately 75% of patients with neurological involvement, parenchymal involvement is observed, while cerebral venous sinus involvement is seen in 20–25% of cases. Headache, cranial neuropathy, dysarthria, ataxia, and hemiparesis are the most prominent symptoms. The most common areas affected in p-NBS are the mesodiencephalic junction (MDJ), pons, and medulla oblongata. MDJ lesions tend to extend upward to involve the diencephalic structures and downward to involve the pontobulbar region which is the most common radiological finding observed in p-NBS. The only drug that has been shown to be effective based on the Class IV evidence is infliximab for the treatment of p-NBS.