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Published on: April 23, 2021
Cerebral venous thrombosis in Behçet's disease compared to those associated with other etiologies
N Yesilot1, S Bahar, S Yilmazer
1Department of Neurology, Istanbul Medical School, Capa, 34293 Istanbul, Turkey. niluferyes@yahoo.com
Insights
Cerebral venous thrombosis (CVT) associated with Behçet's disease (BD) presents differently than other causes, often with subacute onset and intracranial hypertension. CVT patients with BD generally have better outcomes and rarely develop venous infarction.
Area of Science:
- Neurology
- Rheumatology
- Vascular Medicine
Background:
- Cerebral venous thrombosis (CVT) has diverse causes.
- Behçet's disease (BD) is a significant cause of CVT in Mediterranean and Middle Eastern populations.
Purpose of the Study:
- To compare clinical and radiological characteristics of CVT in patients with and without Behçet's disease.
- To identify distinguishing features of CVT associated with BD.
Main Methods:
- Retrospective evaluation of CVT patients.
- Comparison of clinical, neuroradiological, and follow-up data between BD and non-BD CVT groups.
- Statistical analysis of demographic and clinical variables.
Main Results:
- BD patients were younger (median 26 vs. 39 years) and predominantly male compared to non-BD patients.
- CVT in BD typically presented with subacute onset and isolated intracranial hypertension (94%), unlike acute onset in non-BD.
- Venous infarcts were rare in BD (6%) versus common in non-BD (63%).
- Outcomes were favorable in all BD patients (mRS 0-2 in 97%) versus 91% in non-BD.
Conclusions:
- CVT associated with Behçet's disease exhibits distinct features, including subacute onset, isolated intracranial hypertension, and a low incidence of venous infarction.
- These characteristics differentiate BD-associated CVT from CVT due to other etiologies.
- CVT in BD patients generally has a good prognosis.
Abstract:
Cerebral venous thrombosis (CVT) is caused by various etiologies. In Mediterranean and Middle Eastern countries, Behçet's disease (BD) is one of the leading causes of CVT. We aimed to evaluate any differences in CVT patients with and without BD. All registered patients with CVT were evaluated retrospectively. Clinical, neuroradiological findings and follow-up data were compared between patients with BD and patients with other etiologies. There were 36 patients with CVT and BD, and 32 patients with CVT related to other etiological causes. BD patients were younger (median age at onset 26 vs. 39 years; P < 0.001), and there was a male preponderance (28 males, 8 females) as compared to the non-BD group (10 males, 22 females; P < 0.001). Onset was frequently acute in the non-BD group, and it was subacute or chronic in the BD group. Hemi/quadriparesis, aphasia and seizures were significantly more common (P < 0.001) in the non-BD group. In the BD group 94% of the patients presented with symptoms of isolated intracranial hypertension (P < 0.001). Venous infarcts were observed in 63% of the patients with other causes and in 6% of the patients with BD (P < 0.001). At admission 97% of the patients in the BD group and 41% of the patients in the non-BD group had a modified Rankin score of 0-2. Outcome was good in all of the patients with BD and in 91% of patients with other causes. Clinical recurrences were seen in six patients with BD and in one patient without BD. CVT associated with BD has a subacute onset, mostly presents with signs of isolated intracranial hypertension and venous infarction rarely develops; these features distinguish CVT due to BD from those with other causes.
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