Behçet's disease as a causative factor of cerebral venous sinus thrombosis: subgroup analysis of data from the VENOST study

Rheumatology (Oxford). 2019 Apr 1;58(4):600-608. doi: 10.1093/rheumatology/key153.


Objective: This study was performed to determine the rate of cerebral venous sinus thrombosis (CVST) among cases of Behçet's disease (BD) included in a multicentre study of cerebral venous sinus thrombosis (VENOST).

Methods: VENOST was a retrospective and prospective national multicentre observational study that included 1144 patients with CVST. The patients were classified according to aetiologic factors, time of CVST symptom onset, sinus involvement, treatment approach and prognosis.

Results: BD was shown to be a causative factor of CVST in 108 (9.4%) of 1144 patients. The mean age of patients in the BD group was 35.27 years and 68.5% were men, whereas in the non-BD CVST group, the mean age was 40.57 years and 28.3% were men (P < 0.001). Among the aetiologic factors for patients aged 18-36 years, BD was predominant for men, and puerperium was predominant for women. The onset of symptoms in the BD group was consistent with the subacute form. The transverse sinuses were the most common sites of thrombosis, followed by the superior sagittal sinuses. The most common symptom was headache (96.2%), followed by visual field defects (38%).

Conclusions: BD was found in 9.4% of patients in our VENOST series. Patients with BD were younger and showed a male predominance. The functional outcome of CVST in patients with BD was good; only 12% of patients presenting with cranial nerve involvement and altered consciousness at the beginning had a poor outcome (modified Rankin Score ⩾2).

Keywords: Behçet’s disease; VENOST study; cerebral venous sinus thrombosis; outcome; subgroup analysis.

Publication types

  • Multicenter Study
  • Observational Study

MeSH terms

  • Adult
  • Age Factors
  • Behcet Syndrome / complications*
  • Behcet Syndrome / pathology
  • Female
  • Humans
  • Male
  • Middle Aged
  • Prognosis
  • Prospective Studies
  • Retrospective Studies
  • Risk Factors
  • Sex Factors
  • Sinus Thrombosis, Intracranial / etiology*
  • Sinus Thrombosis, Intracranial / pathology