[Cerebral sinus-venous thrombosis in protein S deficiency]
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B Neundörfer1, F Erbguth, W Scheidler
1Neurologische Klinik mit Poliklinik der Universität Erlangen-Nürnberg.
Insights
Protein S deficiency, a rare condition, can cause cerebral sinus thrombosis. This condition often affects women and may be linked to factors like pregnancy or oral contraceptives.
Area of Science:
- Hematology
- Neurology
- Vascular Medicine
Background:
- Protein S acts as a cofactor for protein C, crucial for inactivating factors V and VIII in the coagulation cascade.
- Deficiency in protein S, whether congenital or acquired, is a rare but significant risk factor for cerebral sinus and venous thrombosis.
Observation:
- This report details 15 cases from literature, alongside an author's own case, focusing on cerebral sinus thrombosis.
- The superior sagittal sinus was the most commonly affected site in these thrombosis cases.
- Reduced levels of the active, unbound form of protein S in plasma were frequently observed.
Findings:
- Cerebral sinus thrombosis associated with protein S deficiency predominantly affects women.
- Inherited protein S deficiency is often compounded by additional risk factors such as pregnancy, puerperium, or the use of oral contraceptive pills.
- The active form of protein S, unbound in plasma, is typically reduced in affected individuals.
Implications:
- Early diagnosis and management of protein S deficiency are critical to prevent potentially life-threatening cerebral venous thrombosis.
- Understanding the role of protein S in coagulation is vital for developing targeted therapies for thrombotic disorders.
- Anticoagulant therapy, initially with heparin and long-term with dicumarol, is the standard treatment for acute stages and long-term management.
Abstract:
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.