Background/Purpose:   Behçet’s syndrome (BS) is a well-recognized cause of cerebral venous sinus thrombosis (CVST). We assessed the outcome of a large cohort of patients with CVST due to BS attending a single dedicated center.

Methods:   We identified 100 (81 M/19 F) patients with BS who were diagnosed as having CVST. All contacted were called back to the outpatient clinic for a clinical, neurological and ophthalmological examination and cranial MRI /MR venography.

Results:   Results: The mean age of the patients at the onset of the symptoms was 28 ± 10 years. A total of 48 patients developed CVST before or at the onset of ISG fulfillment, while 52 developed CVST after a median 3 [2-8] years of ISG fulfillment. Superior sagittal (n=47) and transverse sinuses (n=46) were most commonly involved followed by sigmoid sinus (n= 26) and jugular vein thrombosis (n= 15). A total of 59 (53 M/ 6 F) patients had vascular involvement in addition to CVST: these were deep vein thrombosis of the lower extremities (n= 47), pulmonary artery involvement (n = 17), Budd-Chiari syndrome (n= 9), vena cava superior thrombosis (n= 6) and major arterial disease (n=3). In about half (32/59), CVST preceded any type of additional vascular involvement. Apart from vascular involvement, eye involvement was seen in 37 patients, parenchymal CNS involvement in 8 (all later than CVST) and gastrointestinal involvement in 5. Seven patients (all male) had died, due to causes unrelated with CVST. By the end of the study (December 2015), all remaining 87 patients were alive and contacted with a median follow-up time of 11 [6-15] years. Only 6 patients had a relapsing CVST course. Information about medical treatment was present in detail in 87 patients of whom 75 received short courses of glucocorticoids with (n=12) or without anti-coagulants. A total of 81 patients received immunosuppressive agents, most commonly azathioprine. Four patients underwent lumbo-peritoneal shunting surgery (1 was successful) and 1 with arterio-venous fistula underwent vascular embolization. By the end of December 2015, a total of 50 patients were re-evaluated at the clinic. None had of symptoms of intracranial hypertension. Ophthalmological examination showed that 17 patients had complications such as bilateral optic atrophy (n= 3), bilateral papilledema (n= 5), bilateral optic disc pallor (n=4) and fibrotic scars around optic disc (n= 5). Sensorineural type hearing loss was detected in 4 patients. Neurological examination was found to be normal among 43 patients with isolated CSVT, whereas abnormal in the remaining 7 patients with concomitant parenchymal CNS involvement. Cranial MR/MR venographies at the end of follow-up, were abnormal in 36 patients showing occlusion/ irregularity/ hypoplasia or collaterals in the sagittal (n=19) or transverse sinus (n=17). In the remaining 14, MR venographies were normal.

Conclusion:   CVST due to BS is closely associated with vascular involvement in the body and may be considered as a risk factor for future vascular involvement. CVST relapses are rare; however, the course is not uneventful: visual acuity or field may be impaired totally or partially because of optic disc atrophy; in addition hearing deficits may occur.  

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/an-outcome-survey-of-100-patients-with-cerebral-venous-sinus-thrombosis-due-to-behcets-syndrome-followed-up-at-a-single-dedicated-center/