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

被引:26
作者
Ben Haddou, E. H. Ait [1 ]
Imounan, F. [1 ]
Regragui, W. [1 ]
Mouti, O.
Benchakroune, N. [4 ]
Abouqal, R. [2 ,3 ]
Benomar, A. [1 ,2 ,3 ]
Yahyaoui, M. [1 ]
机构
[1] CHU Rabat Sale, ONO, Hop Specialites, Serv Neurol & Neurogenet B,Rabat Inst, Rabat, Morocco
[2] Fac Med & Pharm, CRECET, Rabat, Morocco
[3] Fac Med & Pharm, LBRCE, Rabat, Morocco
[4] CHU Rabat Sale, ONO, Hop Specialites, Serv Ophtalmol B,Rabat Inst, Rabat, Morocco
关键词
Cerebral deep venous system thrombosis; Cyclophosphamide; Meningo-encephalitis; Myelopathy; Neurobehcet disease; VASCULITIS;
D O I
10.1016/j.neurol.2011.09.006
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Introduction. - Neurological manifestations in Behcet'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 Neurobehcet cases fulfilling the International Study Group criteria for Behcet'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 neurobehcet'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. (C) 2011 Elsevier Masson SAS. All rights reserved.
引用
收藏
页码:344 / 349
页数:6
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