The coagulation inhibitor protein S is a cofactor for the protein C, w
hich inactivates the factors V and VIII. In very rare cases a congenit
al or acquired protein S deficiency leads to a cerebral sinus or venou
s thrombosis. Guided by an own case report the other 15 cases of the l
iterature are described. In most cases there was a thrombosis of the s
inus sagittalis superior. Usually the active form, which is not bound
to protein in plasma, is reduced. Women are more frequently involved t
han 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 ti
me with dicumarol.