Behcet’s Disease Should Be Considered a Causative Factor of Cerebral Venous Sinus Thrombosis

Behcet’s disease (BD) was found in approximately 9% of patients with cerebral venous sinus thrombosis (CVST) and should be considered an initial causative factor of CVST in men between 18 and 36 years, particularly those residing in countries in which the disease is common, according to an analysis of data from the retrospective, prospective, national, multicenter, observational VENOST study, published in Rheumatology.

The investigators sought to determine the rate of CVST in cases of BD that were evaluated in the VENOST study. A total of 1144 patients with CVST were enrolled in the study. Patients were diagnosed with CVST at 35 different centers between June 2000 and June 2015, and were grouped according to etiologic factors, time of onset of CVST symptoms, sinus involvement, treatment approach, and overall prognosis.

Of the 1144 enrollees in the VENOST study, 9.4% of patients with CVST were affected by Behcet’s disease, as indicated by clinical and imaging characteristics and confirmed by expert neurologists. The mean age of the participants in the BD group was 35.27 years and 68.5% were men. In contrast, in patients who were not affected by BD, the mean patient age was 40.57 years, and 28.3% were men (P <.001). 

Of the etiologic factors for patients 18 to 36 years, Behcet’s disease was predominant in men and puerperium was predominant in women. In the BD group, symptom onset was consistent with the subacute form of the disease. The most common sites of thrombosis in the BD group were the transverse sinuses (70.4%) and the superior sagittal sinuses (44.4%). The most common symptoms in the BD group were headache (96.2%) and visual field defects (38%).

Symptom onset in the BD group was consistent with the subacute form of the disease (43.4%), whereas the acute form of the disease was significantly more common in patients who were not affected by Behcet’s disease (P <.001). Epileptic seizures, focal neurologic deficits, and altered consciousness were significantly more common in patients who were not affected by BD vs patients in the BD group (P <.001, P =.005, and P <.001, respectively), but visual field defects were significantly more common in the BD group compared with patients who were not affected by BD (P =.005).

Patients with Behcet’s disease were generally younger and demonstrated a male predominance. The functional outcome of CVST in patients with BD was good, and only 12% of patients who presented with cranial nerve involvement and altered consciousness at the study initiation exhibited a poor outcome.

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Reference

Uluduz D, Midi I, Duman T, et al. Behçet’s disease as a causative factor of cerebral venous sinus thrombosis: subgroup analysis of data from the VENOST study [published online July 9, 2018]. Rheumatology (Oxford). doi:10.1093/rheumatology/key153