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Cerebral venous thrombosis in Behçet's disease
D Saadoun1, B Wechsler, M Resche-Rigon
1Hôpital Pitié-Salpétrière and Pierre et Marie Curie-Paris VI University, Paris, France. david.saadoun@psl.aphp.fr
Insights
Cerebral venous thrombosis (CVT) affects 7.8% of Behçet's disease (BD) patients, often presenting with extraneurologic lesions. Anticoagulation is a safe and effective treatment for CVT in BD.
Area of Science:
- Neurology
- Rheumatology
- Vascular Medicine
Background:
- Behçet's disease (BD) is a multisystem inflammatory disorder.
- Cerebral venous thrombosis (CVT) is a rare but serious complication of BD.
Purpose of the Study:
- To analyze the clinical characteristics, treatment, and outcomes of CVT in a large cohort of BD patients.
- To determine the prevalence of CVT in BD and identify factors influencing prognosis.
Main Methods:
- A retrospective analysis of 64 consecutive BD patients with CVT.
- Comparison of clinical findings between BD patients with and without CVT.
- Multivariate analysis to identify prognostic factors.
Main Results:
- CVT was present in 7.8% of 820 BD patients.
- CVT patients showed less parenchymal CNS involvement but more extraneurologic vascular lesions.
- Anticoagulation was effective in 90% of cases with no severe hemorrhagic complications.
- Papilledema and concurrent prothrombotic risk factors were associated with sequelae.
- Relapse was linked to prothrombotic factors and peripheral venous thrombosis.
Conclusions:
- CVT in BD can lead to severe neurological outcomes.
- Anticoagulation is a safe and effective treatment for CVT in BD.
- Investigating prothrombotic disorders is crucial in BD patients with CVT.
Objective:
To analyze the clinical findings, treatment, outcome, and prevalence of cerebral venous thrombosis (CVT) in a large cohort of patients with Behçet's disease (BD) from a single center.
Methods:
We reported a series of 64 consecutive patients with CVT who fulfilled the international criteria for BD. Multivariate analysis was performed to define factors that affect prognosis.
Results:
Among a cohort of 820 patients with BD, CVT was present in 64 (7.8%). Compared with BD patients without CVT, those with CVT had lower parenchymal central nervous system involvement (4.7% versus 28.7%; P = 0.0001) and higher extraneurologic vascular lesions (62.5% versus 38.8%; P = 0.03). Up to 90% of patients responded to anticoagulation therapy without severe hemorrhagic complications. Neither steroid nor immunosuppressant use provided better outcome. Severe visual loss due to optic atrophy was the main complication of CVT, being found in 15% of patients. In multivariate analysis, papilledema (odds ratio [OR] 7.1, 95% confidence interval [95% CI] 1.6-31.9) and concurrent prothrombotic risk factors (OR 4.6, 95% CI 1.1-20.2) were independently associated with the occurrence of sequelae. Factors associated with relapse of thrombosis were concurrent prothrombotic risk factors (hazard ratio [HR] 4.9, 95% CI 1.5-15.4) and a peripheral venous thrombosis (HR 2.8, 95% CI 0.7-10.5). After a mean +/- SD followup of 8.2 +/- 6.9 years, 4 deaths unrelated to CVT were noted.
Conclusion:
CVT in patients with BD may result in serious neurologic outcomes. Anticoagulation represents a safe and effective therapy. Extensive investigation of prothrombotic disorders should be considered.
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