Lethal sinus vein thrombosis in a patient with mixed connective tissue disease and antiphospholipid syndrome

Background: The antiphospholipid (Huges) syndrome is a complication of conn ective tissue diseases characterized by thromboembolic occlusions of arteri al and venous blood vessels. Case Report: At the age of 13, the patient developed connective tissue dise ase with arthritis and myositis. The course of her disease was characterize d by frequent relapses despite immunosuppressive treatment. She developed d eep venous thrombosis of her right leg as a manifestation of secondary anti phospholipid antibody syndrome at the age of 15 and was subsequently starte d on oral anticoagulation therapy. Approximately 10 months later, however, she decided to try alternative medicine and stopped both anticoagulation an d immunosuppressive therapy. Only after 4 weeks she developed seizures foll owed by respiratory arrest with the need for cardiopulmonary resuscitation. Despite intensive care she died 2 days later with the signs of severe cere bral edema causing herniation of the brainstem. Autopsy confirmed the diagn osis of severe edema of the brain as a result of extensive thrombosis of al l sinus veins. Conclusion: A complete sinus vein thrombosis is a rare manifestation of ant iphospholipid antibody syndrome. The lethal thrombosis in this case occurre d during a period of reactive hypercoagulability after termination of immun osuppressive and/or anticoagulation therapy. This case report underlines th e need for long-term anticoagulation in patients with the antiphospholipid syndrome.