[Cerebral sinus-venous thrombosis in protein S deficiency]

The coagulation inhibitor protein S is a cofactor for the protein C, which inactivates the factors V and VIII. In very rare cases a congenital or acquired protein S deficiency leads to a cerebral sinus or venous thrombosis. Guided by an own case report the other 15 cases of the literature are described. In most cases there was a thrombosis of the sinus sagittalis superior. Usually the active form, which is not bound to protein in plasma, is reduced. Women are more frequently involved than men. Partly there are additional risk factors to inheritance, such as pregnancy, puerperium or anticonceptive pills. In the acute stage the patients have to be treated with heparin, afterwards for a long time with dicumarol.