Background and objective: Central nervous system (CNS) involvement, one of the most serious manifestations of Behcet disease (BD), is uncommon in children. We describe a teenager diagnosed with BD following cerebral sinus vein thrombosis and review the available literature to summarize the clinical characteristics of children with cerebral sinus vein thrombosis associated with BD.
Methods: A 12-year old boy with cerebral sinus vein thrombosis demonstrated by magnetic resonance venography, and associated with skin rash and oral ulcers is described and the relevant literature based on a MEDLINE search from 1966 to July 2014 is reviewed. The keywords used were "cerebral venous thrombosis" or "sinus thrombosis" and "Behcet disease".
Results: Twenty-five well documented reports of childhood cerebral venous thrombosis as part of neuro-Behcet syndrome (NBS) have been identified. The male to female ratio was 1.8:1 and the age range was 4-18. The clinical course was progressive in most cases, with only 3 cases presenting with concurrent parenchymal central nervous system involvement. Management differed between reports in terms of immunosuppression and anticoagulation treatment.
Conclusion: Cerebral sinus vein thrombosis in children is a rare manifestation of NBS with male predominance. Overall, neurological prognosis is good. Therapeutic approach is not settled yet, however, we support the use of long-term anticoagulation, particularly in patients with detected thrombophilic risk factors.
