2. Cerebral Vein Thrombosis Morning Report Sima Patel 5/13/09

3. CEREBRAL VEIN and SINUS THROMBOSIS Thrombosis of the dural venous sinuses and associated veins First described by French physician Ribes in 1825 who observed thrombosis of the saggital sinus and cerebral veins.

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5. EPIDEMIOLOGY 3-4 cases per million in adults Most common in 3 rd decade of live 75% are female Older studies show no difference in gender and it is believed that use of oral contraceptive have played a role Accounts for 50% of strokes during pregnancy and peripartum period

6. RISK FACTORS Thrombotic disorders ▫Factor V leiden ▫Protein C deficiency ▫Protein S deficiency ▫Antithrombin III deficiency ▫Antiphospholipid syndrome ▫Lupus anticoagulant

7. RISK FACTORS Nephrotic syndrome Chronic inflammatory diseases such as IBD (felt to be mediated by endotoxins and abnormalities of the fibrinolytic system, platelet count and functions) Collagen vascular diseases – Lupus, Wegeners, Behcet syndrome Hyperhomocysteinemia

8. RISK FACTORS Pregnancy and peripartum period Polycythemia Vera Paroxysmal Noctural Hemoglobinuria Oral Contraception use (estrogen containing) Infection - Meningitis, otitis media, sinusitis, mastoiditis Direct injury to the venous sinuses

9. PATHOGENESIS Formation of blood clots in the veins as well as venous sinuses of the brain Thrombosis of the veins cause infarcts to associated brain tissue and can result in cerebral edema. Infarcts can lad to hemorrhages (40% can progress to hemorrhagic infarcts) Sinus thrombosis leads to decreased resorption of the CSF leading to increased intracranial pressure Most cases of cerebral venous sinus thrombosis are due to hypercoagulability Thrombosis can lead to embolization and PE (occurs in 10% of patients)

10. CLINICAL PRESENTATION Headache (sudden or gradual) – in 90% of patients Symptoms of stroke – in 50% of patients Seizures (usually affect only one part of the body) – in 40% of patients AMS Increased intracranial pressure  visual changes If cavernous sinus thrombosis (3%) then can present with proptosis and painful ophthalmoplegia Other: nausea/vomiting, vestibular neuropathy, pulsatile tinnitus, double vision, unilateral deafness, facial weakness, visual changes

11. EVALUATION CBC Antiphospholipid and anticardiolipin antibodies Protein S Protein C Antithrombin III Lupus anticoagulant Factor V Leiden Hb Electrophoresis ESR ANA Urine protein LFT

12. DIAGNOSIS Based on symptoms (headache, signs of increased ICP, neurologic deficits) CT MRI Angiography – may be able to demonstrate smaller clots than MRI and CT Most sensitive  MRI and MRV

15. TREATMENT Heparin or low molecular weight heparin for initial treatment followed by coumadin If extensive hemorrhage, repeat imaging in 7-10 days to determine if anticoagulation safe. If thrombosis occurred under temporary conditions, the patient will need 3 months of anticoagulation

16. TREATMENT If no clear cause or condition unprovoked then needs 6-12 months anticoagulation If underlying thrombotic disorder then needs lifelong anticoagulation Thrombolysis is restricted to those with a poor prognosis

17. PROGNOSIS Mortality 5.6% during hospitalization Mortality 50% in children 88% have total or near complete recovery Rate of recurrence 2.8%

18. REFERENCES Ida Martinelli, Sacchi E, Landi G, Taioli E, Duca F. High Risk of Cerebral Vein Thrombosis in Carriers of Prothrombin-Gene Mutation and in users of Oral Contraceptives. New England Journal of Medicine June 18, 1998; Volume 338: 1793-1979 Prakash C, Bansal BC. Cerebral Venous Thrombosis. Journal of Indian Academy of Clinical Medicine. www.uptodate.com