Rapid, complete and sustained response to corticosteroids and pulse cyclophosphamide therapy in a patient with behçet and central nervous disease.

BACKGROUND: Approximately eighty-percent of patients with neuro-Behçet have parenchyma CNS disease, probably due to small-vessel vasculitis. The most used treatment is high-dose corticosteroids followed by immunosuppressive medication but efficacy of this combination has not been proven to date.

METHODS: A 23 year-old male Hispanic patient with diagnosis of Behçet's disease, with onset at the age of 18, developed a bout of oral and genital ulcers, fever 39°C to 40°C, joint inflammation, cephalea, diplopia, convergent strabismus and drowsiness. A brain MRI has shown a large T2-weighted hyperintense signal in the right subthalamic nucleus and pontobulbar area. The symptoms remitted after treatment with oral prednisone and monthly pulses of cyclophosphamide and the patient had a complete and sustained neurological recovery up to twelve months of follow-up.

RESULTS: We present here the rapid, complete and sustained response to corticosteroids and pulse cyclophosphamide therapy in a patient with Behçet´s disease and subacute central nervous system (CNS) disease.

CONCLUSIONS: The early institution of an aggressive scheme combining high dose corticosteroids and monthly pulse cyclophosphamide therapy in a patient with Behçet disease with CNS manifestations is critical for a rapid, complete and potentially sustained response and to prevent permanent CNS damage.