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

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. 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 600mg/m2 of BSA in the 1st, 2nd, 4th, 6th and 8th day followed by a bolus of 600mg/m2 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. 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. 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. Les manifestations neurologiques de la maladie de Behçet représentent entre 4 à 49 % des manifestations et restent la principale cause de morbimortalité. Quarante cas de Neurobehçet remplissant les critères du groupe international d’étude pour la maladie de Behçet, ont été recrutés consécutivement sur une période de juin 2004 au décembre 2010. Les patients ont reçu des corticoïdes et 600mg/kg par m2 par SC de cyclophosphamide intraveineux tous les deux mois pendant deux ans. Les anticoagulants ont été administrés en cas de thrombose veineuse cérébrale. Le suivi et la tolérance au traitement ont été analysés. L’âge moyen était de 34±13ans, avec un sex-ratio de 1,78. Le tableau clinique était dominé par la méningo-encéphalite (48,8 %), la thrombose veineuse cérébrale profonde (43,6 %) et la myélopathie (7,7 %). Les 40 patients ayant reçu un bo