with CVT and associated parenchymal brain lesions who present with one or more seizures in the acute phase, antiseizure medications should be continued until seizure-free for a defined duration (eg, one year). The risk of epilepsy after CVT ranges from 5 to 11 percent of patients [15,62-64]. The risk is higher in those with seizures in the acute phase, with hemorrhagic parenchymal lesions, and who survived a decompressive hemicraniectomy [63,64]. Late-onset seizures indicate an even higher risk of epilepsy. In one study of 123 CVT patients with late seizures (occurring more than seven days after CVT diagnosis), 70 percent had a recurrent seizure during a mean two-year follow-up [64]. General recommendations for the selection and withdrawal of antiseizure medications are discussed separately. (See "Overview of the management of epilepsy in adults", section on 'Antiseizure medication therapy' and "Overview of the management of epilepsy in adults", section on 'Discontinuing antiseizure medication therapy' and "Seizures and epilepsy in children: Initial treatment and monitoring", section on 'Selection of an antiseizure medication'.) Headaches Chronic headaches have been reported to occur in more than half of patients with prior CVT [65]. Headaches severe enough to require bed rest or hospital admission afflict 14 percent of patients with CVT [15]. Repeated brain MRI and magnetic resonance venography (MRV) are necessary to exclude the rare case of recurrent CVT. MRV may depict stenosis of a previously occluded sinus [66,67]. https://www.uptodate.com/contents/cerebral-venous-thrombosis-treatment-and-prognosis/print 12/33 7/6/23, 12:28 PM Cerebral venous thrombosis: Treatment and prognosis - UpToDate Lumbar puncture may be necessary to exclude chronically elevated intracranial pressure (ICP) if headache persists and brain MRI and MRV are normal. In such cases, therapeutic options to treat elevated ICP include acetazolamide (500 mg twice daily) or topiramate (for patients who cannot tolerate acetazolamide), but efficacy is unproven [39]. Additional options if severe headache associated with increased intracranial hypertension persists include repeated lumbar punctures, a lumboperitoneal shunt, or stenting of sinus stenosis [68-70], but efficacy after CVT is also unproven. Visual loss Severe visual loss due to CVT is fortunately a rare event [71-73]. Nevertheless, elevated intracranial pressure must be rapidly ruled out and managed accordingly if visual acuity decreases during follow-up and is not explained by ocular causes. Fenestration of the optic nerve sheath has also been used to relieve pressure and prevent optic nerve atrophy, but efficacy is not established [39,74]. Because of the potential for visual loss caused by severe or long-standing elevation of intracranial pressure, serial assessment of visual fields and visual acuity is recommended for children with CVT during follow-up, particularly during the first year [39,42]. It is reasonable to do the same for adults with visual complaints, chronic headaches, or papilledema. Cognitive and psychiatric complications Despite the apparent general good recovery in most patients with CVT, approximately one-half of the survivors feel depressed or anxious, and minor cognitive or language deficits may preclude them from resuming their previous jobs [75,76]. Patients should be reassured of the very low level of risk of recurrence of CVT and be encouraged to return to previous occupations and hobbies. In some cases, antidepressants may be necessary. Subsequent pregnancy We suggest prophylaxis with LMWH during pregnancy and puerperium for those with a previous history of CVT to reduce the risk of recurrent CVT and other venous thromboembolic events, in accord with guidelines from the European Stroke Organization and the American Heart Association/American Stroke Association [37,39,77]. For pregnant patients with a history of CVT, we suggest temporary prophylactic anticoagulation with subcutaneous LMWH throughout pregnancy and continuing up to eight weeks postpartum. (See "Deep vein thrombosis and pulmonary embolism in pregnancy: Prevention".) Pregnancy and the puerperium are known risk factors for CVT (see "Cerebral venous thrombosis: Etiology, clinical features, and diagnosis", section on 'Risk factors and associated conditions'). The absolute risk of complications during subsequent pregnancy among those who have a history of CVT appears to be low, although the relative risks of recurrent CVT and noncerebral VTE are quite elevated compared with the general population. Supporting evidence comes from https://www.uptodate.com/contents/cerebral-venous-thrombosis-treatment-and-prognosis/print 13/33 7/6/23, 12:28 PM Cerebral venous thrombosis: Treatment and prognosis - UpToDate a 2016 systematic review of 13 observational studies evaluating the frequency of CVT or noncerebral VTE associated with pregnancy and the puerperium in those with a history of previous CVT [78]. The following observations were reported: Recurrent CVT occurred in 2 of 217 pregnancies (9 per 1000 pregnancies, 95% CI 3-33 per 1000), a rate that was more than 80-fold higher than the previously reported incidence in the general population. Noncerebral VTE occurred in 5 of 186 pregnancies (27 per 1000, 95% CI 12-61 per 1000), a rate that was approximately 16-fold higher than the incidence previously described in the general population. Spontaneous abortion occurred in 33 of 186 pregnancies (18 percent, 95% CI 13-24 percent), a rate similar to the estimated rate in the general population. (See "Pregnancy loss (miscarriage): Terminology, risk factors, and etiology", section on 'Incidence'.) There was no significant difference in the rate of spontaneous abortion for patients treated or not treated with antithrombotic therapy (11 versus 19 percent). Thus, based upon the available evidence, a history of CVT, including pregnancy- or puerperium- related CVT, is not a contraindication for future pregnancy. Patients should be advised not to become pregnant while on warfarin because of its teratogenic effects and increased risk of fetal bleeding. (See "Use of anticoagulants during pregnancy and postpartum", section on 'Already taking warfarin'.) Oral contraceptives Because it is a risk factor for CVT, female patients with prior CVT should be informed about the risks of combined estrogen-progestin hormonal contraception and advised against its use [37,42]. Risk is associated with the dose of ethinyl estradiol (less risk with doses <50 mcg). The type of progestin is also associated with VTE risk; in general, the lowest risk is seen with combined oral contraceptives that contain a second-generation progestin such as levonorgestrel. These risks are discussed in greater detail separately. (See "Combined estrogen- progestin contraception: Side effects and health concerns", section on 'Venous thromboembolism'.) PROGNOSIS CVT can result in death or permanent disability but usually has a favorable prognosis. https://www.uptodate.com/contents/cerebral-venous-thrombosis-treatment-and-prognosis/print 14/33 7/6/23, 12:28 PM Cerebral venous thrombosis: Treatment and prognosis - UpToDate Early deterioration and death Approximately 5 percent of patients die in the acute phase of the disorder [79,80]. Most of the early deaths are a consequence of CVT. A systematic review found that mortality rates among patients with CVT declined since the 1960s; increased detection of less severe cases with advances in neuroimaging along with improved hospital care may have accounted for some or all of the decreased mortality [81]. In the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT) that evaluated 624 patients (age >15 years) with CVT, 27 patients (4.3 percent) died during hospitalization for the acute phase, including 21 patients (3.4 percent) who died within 30 days from symptom onset [79]. Predictors of mortality at 30 days in the ISCVT were as follows [79]: Depressed consciousness Altered mental status Thrombosis of the deep venous system Right hemisphere hemorrhage Posterior fossa lesions Early mortality in children with CVT is similar to that in adults. In a European cohort of 396 children with CVT (median age 5.2 years), death in the first two weeks after presentation occurred in 12 patients (3 percent) [82]. The main cause of acute death with CVT is transtentorial herniation secondary to a large hemorrhagic lesion [79]. Other causes of early death include herniation due to multiple lesions or to diffuse brain edema, status epilepticus, medical complications, and pulmonary embolism [2]. Long-term outcome Mortality after the acute phase of CVT is predominantly related to underlying conditions. The main ISCVT report [15] performed a meta-analysis of seven prospective series and found that acute CVT was associated with a 15 percent overall death or dependency rate at the end of follow-up, which varied from 3 to 78 months. In the ISCVT study, complete recovery at the end of follow-up (median 16 months) was noted in 79 percent of the entire cohort of patients, while death was the outcome in 8 percent [15]. Predictors of poor long-term prognosis in the ISCVT were as follows [15]: Central nervous system infection Any malignancy Thrombosis of the deep venous system https://www.uptodate.com/contents/cerebral-venous-thrombosis-treatment-and-prognosis/print 15/33 7/6/23, 12:28 PM Cerebral venous thrombosis: Treatment and prognosis - UpToDate Hemorrhage on head CT or MRI Glasgow coma scale score <9 on admission ( table 3) Mental status abnormality Age >37 years Male sex The CVT risk score was designed to estimate the functional prognosis at six months after CVT onset; the score was derived using data from the original ISCVT cohort of 624 patients and validated in two smaller cohorts [83]. The score is tallied as follows: Presence of malignancy 2 points Coma on admission 2 points Thrombosis involving the deep venous system 2 points Mental status disturbance on admission 1 point Male sex 1 point Intracranial hemorrhage on admission 1 point A CVT risk score 3 was associated with a poor outcome, defined as a modified Rankin Scale ( table 4) score of >2 (dependency or death), with a high sensitivity but poor specificity (96 and 14 percent, respectively) [83]. In the ISCVT, complete recovery at six months was significantly more common for female than male patients (81 versus 71 percent), while dependency or death was less likely for females than males (12 versus 20 percent) [84]. These differences were driven entirely by the more favorable outcome for the subgroup of females who had sex-specific risk factors (mainly oral contraceptives, pregnancy, or puerperium) for CVT. Females without sex-specific risk factors had outcomes similar to males. (See "Cerebral venous thrombosis: Etiology, clinical features, and diagnosis", section on 'Epidemiology'.) Intracerebral hemorrhage present at the time of CVT diagnosis was identified in 245 patients (39 percent) of the ISCVT cohort [85]. In this subgroup with early intracerebral hemorrhage, predictors of poor prognosis at six months were older age, male sex, thrombosis of the deep cerebral venous system or of the right lateral sinus, and a motor deficit [85]. By contrast, several studies have found that a good outcome after CVT is predicted when symptoms of intracranial hypertension are the only manifestations of CVT at the time of diagnosis [46,86]. In patients with CVT presenting with isolated intracranial hypertension, a subgroup analysis of the ISCVT cohort found that poor outcome was associated with a longer diagnostic delay [87]. https://www.uptodate.com/contents/cerebral-venous-thrombosis-treatment-and-prognosis/print 16/33 7/6/23, 12:28 PM Cerebral venous thrombosis: Treatment and prognosis - UpToDate Recurrence The risk of recurrent CVT is approximately 2 to 4 percent, while the risk of recurrent venous thromboembolism (VTE) in other locations after CVT ranges from 4 to 7 percent. In the ISCVT study, which evaluated 624 patients with CVT over a median 16 months of follow-up, 14 (2 percent) had a recurrent CVT and 27 (4 percent) had other thrombotic events during follow-up [15]. The risks of recurrent CVT or any venous thrombosis were 1.5 and 4.1 per 100 person-years, respectively [88]. Observational data also suggest longer-term CVT recurrence rates appear to be lower than rates of recurrent VTE. In a retrospective cohort study of 706 patients with a first CVT who were followed for 6 to 297 months (median 40 months), CVT recurred in 31 patients (4 percent), and VTE in a different site occurred in 46 patients (7 percent) [89]. In another study that prospectively followed 145 patients with CVT for a median of six years after discontinuation of anticoagulant therapy, a recurrent CVT developed in five patients (3 percent), and other types of VTE developed in another 10 patients (6 percent) [90]. The risks of recurrent CVT or other types of VTE were 0.5 and 2.0 per 100 person-years, respectively. Risk factors for CVT recurrence in adults include the following [88-91]: History of prior VTE Polycythemia/thrombocythemia Clinical history or laboratory evidence of thrombophilia Male sex Black race Data on CVT recurrence and risk factors in children are limited. In the European cohort of 396 children with CVT (median age five years) followed for a median of 36 months, recurrent venous thrombosis occurred in 22 children at a median of six months, including CVT in 13 children (3 percent) [82]. There were no recurrences of CVT among children younger than 25 months. Factors independently associated with recurrent cerebral and systemic venous thrombosis in children were nonadministration of anticoagulant therapy before relapse (hazard ratio [HR] 11.2, 95% CI 3.4-37.0), persistent occlusion on repeat venous imaging (HR 4.1, 95% CI 1.1-14.8), and heterozygosity for the prothrombin (factor II) G20210A variant (HR 4.3, 95% CI 1.1-16.2) [82]. Recanalization Most patients with CVT achieve some degree of cerebral vein and sinus recanalization. A 2018 systematic review and meta-analysis identified 19 studies, mostly retrospective, that reported recanalization rates for adult patients with CVT who were treated with anticoagulation [92]. The overall recanalization rate was 85 percent among 818 patients who received follow-up imaging, with partial recanalization achieved by 35 percent and complete recanalization by 49 percent of patients. In the few studies with available data, the https://www.uptodate.com/contents/cerebral-venous-thrombosis-treatment-and-prognosis/print 17/33 7/6/23, 12:28 PM Cerebral venous thrombosis: Treatment and prognosis - UpToDate recanalization rate increased with time, and approximately three-quarters of patients had achieved recanalization at three months. Positive predictors of recanalization were thrombosis of the superior sagittal sinus and female sex; negative predictors of recanalization were multiple thromboses, hormonal therapy, older age, and lack of identified risk factors for CVT. The meta-analysis also found that recanalization was associated with functional recovery [92]. A favorable outcome, defined as a modified Rankin Scale score ( table 4) of 0 (no symptoms) or 1 (no significant disability despite symptoms), was achieved in 319 of 357 (89 percent) patients with recanalization and in 42 of 59 (71 percent) patients without recanalization; the pooled odds ratio for a favorable outcome with recanalization was 3.3 (95% CI 1.2-8.9). In subsequent prospective cohort study of 68 patients with newly diagnosed CVT treated with anticoagulation and followed with MRI and magnetic resonance venography, early venous recanalization (confirmed by imaging on day 8 after starting anticoagulation) was associated with both regression and a reduced risk of enlargement of nonhemorrhagic lesions, including those with venous infarction [93]. SOCIETY GUIDELINE LINKS Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Stroke in adults" and "Society guideline links: Stroke in children".) SUMMARY AND RECOMMENDATIONS Acute anticoagulation For adults with symptomatic cerebral venous thrombosis (CVT), with or without hemorrhagic venous infarction, we recommend initial anticoagulation therapy with subcutaneous low molecular weight heparin (LMWH) or intravenous heparin (Grade 1C). For children with CVT, with or without significant secondary hemorrhage, we suggest initial anticoagulation therapy with subcutaneous LMWH or intravenous heparin (Grade 2C). (See 'Initial anticoagulation' above.) Management of acute complications Complications that require intervention during the acute phase of CVT include elevated intracranial pressure, brain swelling, seizures, and infection. (See 'Other acute management issues' above.) Measures to control acutely increased intracranial pressure and impending herniation, including decompressive surgery, may be required in patients with CVT. https://www.uptodate.com/contents/cerebral-venous-thrombosis-treatment-and-prognosis/print 18/33 7/6/23, 12:28 PM Cerebral venous thrombosis: Treatment and prognosis - UpToDate For patients with CVT who have both seizures at presentation and focal cerebral supratentorial lesions (eg, edema, infarction, or hemorrhage on admission computed tomography or magnetic resonance imaging), we recommend seizure prophylaxis with an antiseizure medication (Grade 1B). For patients with a single early symptomatic seizure due to CVT in the absence of a supratentorial cerebral lesion, the benefit of seizure prophylaxis is uncertain due to low likelihood of recurrence. Seizure prophylaxis is avoided for those who have focal cerebral lesions without seizures. (See 'Seizures' above.) Antibiotic treatment is mandatory whenever there is meningitis or other intracranial infection or an infection of a neighboring structure, such as otitis or mastoiditis. Selection and duration of anticoagulation After the acute phase of CVT, we suggest anticoagulation for most patients with either a direct oral anticoagulant or warfarin for 3 to 12 months (Grade 2C). Exceptions include patients with comorbid malignancy (for whom LMWH is preferred), antiphospholipid syndrome or chronic kidney disease (for whom warfarin is preferred), and those who are pregnant (for whom heparins are preferred). (See 'Long-term anticoagulation' above and 'Selection of anticoagulant' above.) It is reasonable to continue anticoagulation for three to 6 months for patients with a provoked CVT associated with a transient risk factor ( table 2) and for 6 to 12 months for patients with an unprovoked CVT. Indefinite oral anticoagulation is reserved for patients with recurrent CVT, extracerebral venous thromboembolism after CVT, or those with an associated severe thrombophilia. (See 'Duration of anticoagulation' above.) Managing the risk of recurrence For pregnant patients with a history of CVT, we suggest temporary prophylactic anticoagulation with subcutaneous LMWH throughout pregnancy and continuing up to eight weeks postpartum (Grade 2C). For adolescent and adult females with a history of CVT, we advise not using combined oral contraceptives. Prognosis CVT is associated with a good outcome in close to 80 percent of patients. Approximately 5 percent of patients die in the acute phase of the disorder, and longer-term mortality is nearly 10 percent. The main cause of acute death with CVT is neurologic, most often from brain herniation. After the acute phase, most deaths are related to underlying disorders such as cancer. (See 'Prognosis' above.) 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Topic 1109 Version 45.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-treatment-and-prognosis/print 26/33 7/6/23, 12:28 PM Cerebral venous thrombosis: Treatment and prognosis - UpToDate GRAPHICS Placebo-controlled randomized trials of anticoagulants in acute cerebral venous thrombosis Einh upl et al 1991, intravenous heparin versus placebo Heparin versus placebo Outcome at three months Heparin group (n = 10) Total recovery 8 patients Residual motor deficit 2 patients Placebo group (n = 10) Total recovery 1 patient Minor residual deficit 6 patients Deaths 3 patients De Bruijn and Stam 1999, subcutaneous nadroparin versus placebo Outcome difference Nadroparin (n = 30) Placebo (n = 29) Risk difference At three weeks Deaths 2 4 BI score <15 4 3 Death or BI <15 6 (20 percent) 7 (24 percent) 4 percent (95% CI, 25 to 17 percent) At 12 weeks Deaths 2 4 Dependent (OHS 2 2 3 to 5) Death or dependent 4 (13 percent) 6 (21 percent) 7 percent (95% CI, 26 to 12 percent) BI: Barthel Index; OHS: Oxford Handicap Score. Data from: 1. Einh upl KM, Villringer A, Meister W, et al. Heparin treatment in sinus venous thrombosis. Lancet 1991; 338:597. 2. de Bruijn SF, Stam J. Randomized, placebo-controlled trial of anticoagulant treatment with low-molecular-weight heparin for cerebral sinus thrombosis. Stroke 1999; 30:484. Graphic 69484 Version 7.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-treatment-and-prognosis/print 27/33 7/6/23, 12:28 PM Cerebral venous thrombosis: Treatment and prognosis - UpToDate Systemic and local conditions increasing the risk of cerebral venous thrombosis Transient risk factors Infection Central nervous system Ear, sinus, mouth, face, and neck Systemic infectious disease Pregnancy and puerperium Dehydration Mechanical precipitants Head injury Lumbar puncture Neurosurgical procedures Jugular catheter occlusion Drugs Oral contraceptives Hormone replacement therapy Androgens Asparaginase Tamoxifen Glucocorticoids Permanent risk factors Inflammatory diseases Systemic lupus erythematosus Beh et disease Granulomatosis with polyangiitis Thromboangiitis obliterans Inflammatory bowel disease Sarcoidosis Malignancy Central nervous system