Abstract | 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.
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Authors | E H Ait Ben Haddou, F Imounan, W Regragui, O Mouti, N Benchakroune, R Abouqal, A Benomar, M Yahyaoui |
Journal | Revue neurologique
(Rev Neurol (Paris))
Vol. 168
Issue 4
Pg. 344-9
(Apr 2012)
ISSN: 0035-3787 [Print] France |
PMID | 22136880
(Publication Type: Evaluation Study, Journal Article)
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Copyright | Copyright © 2011 Elsevier Masson SAS. All rights reserved. |
Chemical References |
- Anti-Inflammatory Agents
- Cyclophosphamide
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Topics |
- Adolescent
- Adult
- Aged
- Anti-Inflammatory Agents
(therapeutic use)
- Behcet Syndrome
(complications, drug therapy, epidemiology, psychology)
- Cyclophosphamide
(therapeutic use)
- Disease Progression
- Female
- Humans
- Male
- Middle Aged
- Nervous System Diseases
(drug therapy, epidemiology, etiology)
- Retrospective Studies
- Treatment Outcome
- Young Adult
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