Article

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.
Grande aphtose
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Langue : ANGLAIS