Neurological manifestations of Behçet's disease: evaluation of 40 patients treated by cyclophosphamide.

INTRODUCTION

Neurological manifestations in Behçet's disease represent between 4 to 49% of systemic manifestations and remain, in the long term, the leading cause of morbidity and mortality.

METHODS

Retrospective series of 40 severe Neurobehçet cases fulfilling the International Study Group criteria for Behçet's disease were consecutively recruited over a period from June 2004 to December 2010. All patients had clinical and ophthalmologic examinations; they underwent laboratory and imaging investigations. They received corticosteroids and cyclophosphamide as initial bolus of 600 mg/m(2) of BSA in the 1st, 2nd, 4th, 6th and 8th day followed by a bolus of 600 mg/m(2) BSA every 2 months for 2 years. Antithrombotic therapy was given to patients with cerebral deep venous thrombosis. Patient follow-up and tolerance to treatment were analyzed.

RESULTS

The average age at diagnosis was 34±13 years, with a sex-ratio of 1.78. The clinical presentation was dominated by the meningoencephalitis in 48.8% of cases, cerebral deep venous thrombosis in 43.6% of cases and myelopathy in 7.7% of cases. The 40 patients receiving cyclophosphamide bolus, despite two aggravated cases, evolved positively with clinical improvement and good tolerance.

CONCLUSION

The demographic and clinical aspects of our series are similar to those reported in the literature. In contrast to previously reported cases of a poor prognosis in severe neurobehçet's disease, our study suggests that immediate and aggressive treatment by cyclophosphamide may ameliorate the prognosis. However, a multicenter study is needed to confirm the possible efficacy of cyclophosphamide and further assess the long-term tolerance.