Recurrent Cerebral Venous Thrombosis Research Articles

Clinical observation of cerebral venous thrombosis and reversible vasoconstriction syndrome in a young patient

Despite the capabilities and availability of modern diagnostic methods, due to the low alertness of doctors, a number of cerebrovascular disorders are diagnosed late, which can lead to serious consequences, illness leading to disability and death in patients of working age. A clinical case of a young patient with recurrent cerebral venous thrombosis and reversible cerebral vasoconstriction syndrome is presented.

Effectiveness of rivaroxaban in preventing cerebral venous thromboembolism: a systematic review and meta-analysis.

Cerebral venous thromboembolism (CVT) poses a significant risk of venous infarction and haemorrhage, which can lead to neurological deficits and, in severe cases, even death. The optimal treatment regimen for patients with CVT remains unclear. MEDLINE, Embase, Google Scholar, Web of Science (WoS), and Cochrane Central databases were searched for randomized controlled trials (RCTs) and observational studies assessing the efficacy and safety of rivaroxaban in patients with CVT. All-site venous thromboembolism (VTE), risk of clinically relevant non-major bleeding, incidence of partial recanalization, complete recanalization and major haemorrhage were among outcomes of interest. Mantel-Haenszel weighted random-effects model was used to calculate relative risks (RRs) with 95% CIs. The analysis included 1 RCT and 3 observational studies containing 211 patients. Compared to vitamin K antagonists (VKAs), rivaroxaban did not significantly decrease the all-site VTE [RR 0.31 (95% CI 0.01, 8.43); P=0.49, I2=0%]. Compared with VKAs, patients on rivaroxaban did not show a significantly reduced risk of recurrent cerebral venous thrombosis. In terms of incidence of partial recanalization, there was no discernible difference between rivaroxaban and VKAs [RR 0.90 (95% CI 0.66, 1.22); P=0.49, I2=0%]. There was no discernible difference in incidence of complete recanalization [RR 0.98 (95% CI 0.32, 3.03); P=0.97, I2=28%] and incidence of major haemorrhage [RR 0.19 (95% CI 0.01, 4.54); P=0.30]. Rivaroxaban was found to have similar efficacy to VKAs. Due to its lower risk of severe bleeding and no need for INR monitoring, rivaroxaban may be a preferable treatment option for CVT.

Dabigatran etexilate versus warfarin in cerebral venous thrombosis in Chinese patients (CHOICE-CVT): An open-label, randomized controlled trial.

The efficacy and safety of dabigatran etexilate for Chinese patients with cerebral venous thrombosis (CVT) has not been well established. CHOICE-CVT was an exploratory, single-center, randomized, open-label study in the National Center for Neurological Disorders involving Chinese patients with CVT aged 18 to 80 years who were randomly assigned (1:1) to either dabigatran etexilate or warfarin. Oral anticoagulants were initiated after 10-15 days of LMWH. The primary efficacy and safety endpoints included the number of patients with recurrent CVT and/or deep venous thrombosis (DVT) and major clinical bleeding within 180 days. Secondary efficacy endpoints included venous recanalization and change in papilledema at day 180. Secondary safety outcomes comprised death, clinical nonmajor bleeding, and any bleeding. The study was registered with ClinicalTrials.gov under NCT03930940. Between October 2017 and February 2023, a total of 89 patients were enrolled and randomly assigned to receive either dabigatran etexilate (n = 44) or warfarin (n = 45). At day 180, the dabigatran etexilate group showed a statistically nonsignificant but likely clinically significant number of patients with recurrent CVT and/or DVT (8 (18.2%; 95% CI, 6.3-30.0) vs 3 (6.7%; 95% CI, 0.0-14.2), p = 0.099, with a power (1-β) of 38.401%) compared with the warfarin group. The dabigatran etexilate group showed a comparable number of patients with clinical major bleeding (0 (0) vs 0 (0) p = 1.000), and clinical nonmajor bleeding (1 (2.3%; 95% CI, 0.0-6.9) vs 1 (2.2%; 95% CI, 0.0-6.7)) but demonstrated a lower risk of any bleeding (1 (2.3%; 95% CI, 0.0-6.9) vs 9 (20.0%; 95% CI, 7.8-32.2)) compared with the warfarin group. Most patients in both groups achieved venous recanalization according to the Modified Qureshi scale (27 (75%; 95% CI, 60.1-89.9) in the dabigatran etexilate group vs 34 (82.9%; 95% CI, 70.9-95.0) in the warfarin group) and exhibited improvement in papilledema as per the Frisén classification (35 (97.2%; 95% CI, 91.6-100.0) in the dabigatran etexilate group vs 37 (88.1%, 95% CI, 77.9-98.3) in the warfarin group). These findings regarding efficacy and safety support the consideration of dabigatran etexilate therapy as a viable treatment option for Chinese patients with CVT.

Abstract TMP82: Comparison Of Direct Oral Anticoagulants To Vitamin K Antagonists For Treatment Of Cerebral Venous Sinus Thrombosis

Introduction: Direct oral anticoagulants (DOACs) have gained popularity in treating cerebral venous thrombosis (CVT). However, studies comparing the use of DOACs to Vitamin K antagonists (VKA) among patients with CVT are limited. Methods: We conducted a single-center retrospective cohort study comparing VKA to DOAC-treated CVT patients. Clinical, radiographic findings and outcomes were compared. Continuous and categorical variables were compared using t-test or Wilcoxon test and Chi-square or Fisher's exact test, as appropriate. Results: 82 CVT patients were included in final analysis (mean age 41.3±16.3, 76.8% women). Thirty (37%) were treated with DOACs. There was no difference in clinical or radiographic characteristics between the two groups. There was no death and majority of patents were discharged home (p=0.11). Sixty-one patients (74.4%) had follow-up imaging within a year. Fifteen, thirty-seven and nine patients had complete, partial, and no vessel recanalization, respectively. There was no difference in recanalization status between the DOAC and VKA groups (p=0.53). 68 patients (82.3%) had follow-up data on headache status: 21(31%) reported resolution and 45(66%) partial improvement with no difference between DOAC and VKA groups (p=0.81). One patient in the DOAC group had a recurrent CVT. One patient in the VKA group had a major hemorrhage within 3 months. Conclusion: We found no significant difference in venous recanalization or outcomes in patients with CVT treated with DOAC vs VKA. DOAC appears to be a safe alternative to VKA. Large multicenter studies are needed to better evaluate the efficacy and safety of DOAC in CVT.

Cerebral Venous Thrombosis and Myeloproliferative Neoplasms: A Three-Center Study of 74 Consecutive Cases

▪BackgroundThe recent association of cerebral venous thrombosis (CVT) with COVID-19 vaccinations (JAMA; 2021; 325, N Engl J Med 2021; 384) motivated the current review of CVT and MPN. Our objectives were, i) provide an estimation of the incidence of CVT in the context of MPN, followed by a description of clinical phenotype and therapeutic strategies, ii) determine long term outlook in terms of recurrent thromboses, hemorrhage, and survival, iii) identify salient features which distinguish MPN associated from COVID vaccine- related CVT.Methods74 consecutive MPN patients with CVT that underwent evaluation at the Mayo Clinic, Rochester MN, USA (n=36), Catholic University, Rome, Italy, (n=23), and University of Florence, Italy (n=15) between 1991 and 2021 were included. The cohort from a previously published multi-center study that included 42 MPN cases with CVT, which were not included in the current study, was used for comparison of observations. Diagnosis of CVT was established with computed tomography or magnetic resonance imaging with venography.Results •Patient characteristics at time of CVTAmong 74 patients with CVT and MPN (median age 44 years, range 15-85; 61% females); disease-specific frequencies were 1.3% (39/2,893), 1.2% (21/1,811) and 0.2% (3/1,888) for essential thrombocythemia (ET), polycythemia vera (PV) and primary myelofibrosis (PMF), respectively.CVT occurred prior to (n=20, 27%, median time to MPN diagnosis 16.5 months), at (n=32, 44%) or after (n=21, 29%, median time to CVT 26 months) MPN diagnosis. 72% of patients presented with headaches, 22% visual changes, 12% nausea/vomiting, 8% neurological deficits, and 6% seizures. Transverse (51%), sagittal (43%) and sigmoid (35%) sinuses were involved with central nervous system hemorrhage in 10 (14%) patients.MPN phenotype included ET (n=39, 53%), PV (n=21, 28%), pre-fibrotic MF (n=6, 8%), MPN-unclassified (n=4, 5%), PMF (n=3, 4%) and post-PV MF (n=1, 1%). Driver mutation testing was performed in 65 patients: 91% harbored JAK2V617F, 3% CALR type 1, 2% MPL, 5% triple negative; moreover, JAK2V617F was mutated in 27/33 (82%) ET patients. An underlying thrombophilia was identified in 19 (31%) cases. No patient had thrombocytopenia. (Table 1). Notably, one patient received the Ad26.COV2.S vaccine, five days prior to presenting with CVT, not associated with thrombosis in other sites, thrombocytopenia or platelet factor 4 antibodies. A history of thrombosis was documented in 10 (14%) patients with three splanchnic venous events. These observations were similar to those noted in our comparative group from a previously published report that included 42 cases; (ET (n=25, 60%), PV (n=11, 26%), PMF (n=5, 12%); median age 51 years, range 16-84; 55% females; 81% JAK2V617F mutated). Prior thrombosis occurred in 8(19%) patients with four splanchnic venous events.Treatment for CVT included systemic anticoagulation alone in 27 (36%) patients or in conjunction with aspirin (n=24, 32%), cytoreductive therapy (n=14, 19%), or both aspirin and cytoreduction (n=9, 12%). 5/21 (24%) patients with CVT post MPN diagnosis, were on anticoagulation at the time of CVT.1.Outcome following CVTAt a median follow-up of 5.1 years (range; 0.1-28.6), recurrent CVT was documented in 3 (4%) patients; incidence rates for other arterial and venous thromboses and hemorrhage were 11% (2 per 100 patient-years), 9% (1.9 per 100 patient-years) and 14% (3 per 100 patient-years), respectively. 3 of 7 (43%) venous thromboses were splanchnic events. Antithrombotic therapy was ongoing in 53% and 80% of patients with thrombotic recurrences and hemorrhage, respectively. A higher incidence of venous thrombosis was noted in the aforementioned previously published cohort (12 (29%) vs 7 (9%), p=0.01); with 5/12 (42%) splanchnic events. Incidence rates for arterial thrombosis and major hemorrhage were similar. Fibrotic and leukemic transformation occurred in 5 (8%) and 1(1%) patient, respectively, with five (7%) deaths unrelated to CVT.ConclusionsThe current study highlights close association of CVT with JAK2V617F, younger age, and female gender. Clinical features distinguishing COVID vaccine-related from MPN-associated CVT include lower likelihood of concomitant non-CVT venous thromboses with the latter; moreover, the absence of thrombocytopenia resulted in a lower rate of intracerebral hemorrhage in MPN cases; as a result, MPN-CVT was not fatal. [Display omitted] DisclosuresVannucchi: AbbVie: Membership on an entity's Board of Directors or advisory committees; Incyte Corporation: Honoraria, Membership on an entity's Board of Directors or advisory committees; Bristol Myers Squibb: Honoraria, Membership on an entity's Board of Directors or advisory committees; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees.

Cerebral venous thrombosis and myeloproliferative neoplasms: A three-center study of 74 consecutive cases.

The recent association of cerebral venous thrombosis (CVT) with COVID‐19 vaccinations prompted the current retrospective review of 74 cases of CVT (median age = 44 years, range 15–85; 61% females) associated with myeloproliferative neoplasms (MPNs), seen at the Mayo Clinic, Catholic University of Rome, and University of Florence, between 1991 and 2021. Disease‐specific frequencies were 1.3% (39/2893), 1.2% (21/1811) and 0.2% (3/1888) for essential thrombocythemia, polycythemia vera and primary myelofibrosis, respectively. Cerebral venous thrombosis occurred either prior to (n = 20, 27%), at (n = 32, 44%) or after (n = 22) MPN diagnosis. A total of 72% of patients presented with headaches. Transverse (51%), sagittal (43%) and sigmoid sinuses (35%) were involved with central nervous system hemorrhage noted in 10 (14%) patients. In all, 91% of tested patients harbored JAK2V617F. An underlying thrombophilic condition was identified in 19 (31%) cases and history of thrombosis in 10 (14%). Treatment for CVT included systemic anticoagulation alone (n = 27) or in conjunction with aspirin (n = 24), cytoreductive therapy (n = 14), or both (n = 9). At a median follow‐up of 5.1 years (range 0.1–28.6), recurrent CVT was documented in three (4%) patients while recurrent arterial and venous thromboses and major hemorrhage were recorded in 11%, 9% and 14%, respectively. Follow‐up neurological assessment revealed headaches (n = 9), vision loss (n = 1) and cognitive impairment (n = 1). The current study lends clarity to MPN‐associated CVT and highlights its close association with JAK2V617F, younger age and female gender. Clinical features that distinguish COVID vaccine‐related CVT from MPN‐associated CVT include, in the latter, lower likelihood of concurrent venous thromboses and intracerebral hemorrhage; as a result, MPN‐associated CVT was not fatal.

Chronic intracranial hypertension after cerebral venous and sinus thrombosis \u2013 frequency and risk factors

BackgroundCerebral venous sinus thrombosis (CVST) can infrequently lead to chronical intracranial hypertension (IH) due to the altered venous drainage. The aim of this study was to ascertain the risk of IH after CVST and to stratify underlying risk factors.MethodsWe performed a retrospective cohort analysis of all cases treated for acute CVST at our department between 2013 and 2019. IH was diagnosed at follow-up according to the modified Dandy criteria. CVST-patients with and without IH were descriptively compared conforming to available clinical and radiological data as well as outcomes.ResultsOur study included 102 patients with acute CVST. In 70 cases complete follow-up data was available (68.6%). Seven of these patients developed symptomatic intracranial hypertension (10%; N = 7, n = 70) within a median follow-up of 6 months. Four of these patients (57.1% (N = 4, n = 7) vs. 3.2% (N = 2, n = 63); p < 0.001) presented recurrent sinus thrombosis in the further course. There were no significant differences between patients with or without IH concerning gender, age, risk factors, occluded vessels and treatment for their CVST. However the presence of visual deterioration at initial admission was higher in patients who developed IH afterwards (57.1% (N = 4, n = 7) vs. 20.6% (N = 13, n = 63); p = 0.03). Patients with chronic IH after CVST showed significantly less likely recanalization of the occluded vessel on follow-up MRI (no recanalization in 28.6% (N = 2, n = 7) vs. 4.8% (N = 3, n = 63); p = 0.02). All patients with IH had a good outcome (mRS 0–2) at discharge and follow-up.ConclusionIH occurred in around 10% after CVST. Insufficient recanalization status may facilitate IH. Patients with visual disturbances seem to develop more likely IH afterwards. Patients who present IH after CVST may develop recurrent cerebral venous thrombosis.

Direct oral anticoagulants in treatment of cerebral venous thrombosis: a systematic review

ObjectivesCurrent guidelines do not recommend direct oral anticoagulants (DOACs) to treat cerebral venous thrombosis (CVT) despite their benefits over standard therapy. We performed a systematic review to summarise the published...

Preliminary findings regarding the use of direct oral anticoagulants in cerebral venous thrombosis

BackgroundDirect oral anticoagulants (DOACs) have entered the treatment paradigms of various conditions based upon large randomized controlled trials. However, use of DOACs for thrombosis at unusual sites, such as cerebral venous thrombosis (CVT), is less clear as the ability to conduct large randomized controlled trials is limited by its rarity. Furthermore, its use in the setting of malignancy or in the elderly remains an area of ongoing research. ObjectiveOur aim was to assess the outcomes in CVT patients treated with DOACs compared to warfarin. We also sought to elucidate whether its use was safe in the setting of malignancy or in the elderly. MethodsRetrospectively assess the differences in clinical outcomes in patients hospitalized with CVT in the Lifespan Health System. ResultsBetween 1 July 2004 and 1 March 2020, 46 adult patients with CVT fulfilled inclusion criteria. No significant differences in outcomes were observed between the DOAC (N = 8) and vitamin K antagonist (VKA) (N = 38) cohorts. The use of DOACs did not result in an increased rate of acute complications, recurrent venous thromboembolism (VTE) and/or CVT, World Health Organization (WHO) grade 3 or 4 bleeding rates, or differences in clinical improvement per the modified Rankin scale. Furthermore, in patients with underlying metastatic cancer (N = 2) or in patients > 80 (N = 1) who received a DOAC, there was no increase in adverse events or significant differences in outcome when compared to warfarin. ConclusionPatients who received a DOAC in the treatment of CVT demonstrated no differences in outcomes when compared to warfarin.

Safety and Efficacy of Dabigatran Etexilate vs Dose-Adjusted Warfarin in Patients With Cerebral Venous Thrombosis

Patients with cerebral venous thrombosis (CVT) are at risk of recurrent venous thrombotic events (VTEs). Non-vitamin K oral anticoagulants have not been evaluated in randomized controlled trials in CVT. To compare the efficacy and safety of dabigatran etexilate with those of dose-adjusted warfarin in preventing recurrent VTEs in patients who have experienced a CVT. RE-SPECT CVT is an exploratory, prospective, randomized (1:1), parallel-group, open-label, multicenter clinical trial with blinded end-point adjudication (PROBE design). It was performed from December 21, 2016, to June 22, 2018, with a follow-up of 25 weeks, at 51 tertiary sites in 9 countries (France, Germany, India, Italy, the Netherlands, Poland, Portugal, Russia, and Spain). Adult consecutive patients with acute CVT, who were stable after 5 to 15 days of treatment with parenteral heparin, were screened for eligibility. Patients with CVT associated with central nervous system infection or major trauma were excluded, but those with intracranial hemorrhage from index CVT were allowed to participate. After exclusions, 120 patients were randomized. Data were analyzed following the intention-to-treat approach. Dabigatran, 150 mg twice daily, or dose-adjusted warfarin for a treatment period of 24 weeks. Primary outcome was a composite of patients with a new VTE (recurrent CVT, deep vein thrombosis of any limb, pulmonary embolism, and splanchnic vein thrombosis) or major bleeding during the study period. Secondary outcomes were cerebral venous recanalization and clinically relevant non-major bleeding events. In total, 120 patients with CVT were randomized to the 2 treatment groups (60 to dabigatran and 60 to dose-adjusted warfarin). Of the randomized patients, the mean (SD) age was 45.2 (13.8) years, and 66 (55.0%) were women. The mean (SD) duration of exposure was 22.3 (6.16) weeks for the dabigatran group and 23.0 (5.20) weeks for the warfarin group. No recurrent VTEs were observed. One (1.7%; 95% CI, 0.0-8.9) major bleeding event (intestinal) was recorded in the dabigatran group, and 2 (3.3%; 95% CI, 0.4-11.5) (intracranial) in the warfarin group. One additional patient (1.7; 95% CI, 0.0-8.9) in the warfarin group experienced a clinically relevant non-major bleeding event. Recanalization occurred in 33 patients in the dabigatran group (60.0%; 95% CI, 45.9-73.0) and in 35 patients in the warfarin group (67.3%; 95% CI, 52.9-79.7). This trial found that patients who had CVT anticoagulated with either dabigatran or warfarin had low risk of recurrent VTEs, and the risk of bleeding was similar with both medications, suggesting that both dabigatran and warfarin may be safe and effective for preventing recurrent VTEs in patients with CVT. ClinicalTrials.gov identifier: NCT02913326.

Recurrent Cerebral Venous Thrombosis in the Setting of Ulcerative Colitis (P3.281)

Recurrent Cerebral Venous Thrombosis in the Setting of Ulcerative Colitis (P3.281)

Coexistence of High Fibrinogen and Low High-density Lipoprotein Cholesterol Levels Predicts Recurrent Cerebral Venous Thrombosis.

Background:Cerebral venous thrombosis (CVT) may lead to serious neurological disorders; however, little is known about the risk factors for recurrent CVT. Our aim was to determine the association between elevated fibrinogen and decreased high-density lipoprotein cholesterol (HDL-C) levels with recurrent CVT.Methods:This retrospective cohort study included participants if they had a first episode of objectively defined CVT and were admitted to Xuan Wu Hospital, Capital Medical University from August 2005 to September 2009. Demographic and clinical variables were collected, as well as laboratory parameters, including plasma fibrinogen and HDL-C. Patients with CVT were followed for recurrent symptomatic CVT. Follow-up was through the end of September 2010. Potential predictors of recurrence were analyzed using Cox survival analysis.Results:At the end of the follow-up, 95 patients were eligible for the study. Twelve of 95 patients (12.6%) had recurred CVT. The median time of recurrence was 7 months (range: 1–39 months). Eight of these 12 (66.7%) experienced recurrence within the first 12 months after their initial CVT. The recurrence rate of CVT was 2.76 per 100 patient-years. Multivariate Cox regression analysis demonstrated that the coexistence of high fibrinogen (>4.00 g/L) and low HDL-C (<1.08 mmol/L) levels at baseline was the only independent predictor for recurrent CVT (hazard ratio: 4.69; 95% confidence interval: 1.10–20.11; P < 0.05). Of the twelve patients with recurrent CVT in our study, 7 (58.3%) had high fibrinogen plus low HDL-C levels. All 7 of these patients took warfarin for 3–12 months, and 6 of 7 had recurrent CVT after the discontinuation of anticoagulant treatment.Conclusions:Concomitant high fibrinogen and low HDL-C levels may be associated with recurrence of CVT. The effect of potential risk factors related to atherothrombosis on recurrent CVT should be closely monitored.

Cerebral Venous Thrombosis and Thrombophilia: A Systematic Review and Meta-Analysis

Cerebral venous thrombosis (CVT) is a rare manifestation of venous thromboembolism (VTE) and stroke. The aim of our systematic review was to provide an updated summary of the strength of association between CVT and thrombophilia and to explore the relevance of thrombophilia for recurrence of CVT or other VTE, or other outcome variables. MEDLINE (via PubMed), EMBASE (via Ovid), and CENTRAL were systematically searched, including references of retrieved articles. Cohort studies of ≥ 40 patients and case-control studies comparing the prevalence of thrombophilia in patients with CVT and unrelated controls were eligible. Two reviewers independently selected studies, assessed quality, and extracted data. A meta-analysis was performed for high quality case-control studies with unselected cases and healthy controls. Odds ratios with 95% confidence intervals were calculated and pooled. We included 23 cohort studies and 33 case-control studies. A significant association was demonstrated between CVT and all inherited thrombophilic factors, as well as increased levels of homocysteine. Inconclusive results were found on the relevance of thrombophilia for recurrent CVT or other VTE. Although there is a strong association between CVT and thrombophilia, the clinical relevance of thrombophilia testing in patients with CVT seems limited, similarly to other forms of VTE.

Diagnosis and treatment of cerebral venous thrombosis

Dural sinus or cerebral venous thrombosis (CVT) is a frequently unrecognized cause of stroke affecting predominantly young women. Typical clinical signs include headache, visual problems and seizures. Both computed tomography and magnetic resonance venous angiography are suitable for the detection of CVT, although magnetic resonance angiography is more sensitive to detect small cortical venous thrombosis. Evidence for efficacy of initial treatment with heparin in acute CVT comes from two randomized placebo-controlled studies that together included 79 patients. Although not evidence-based, postacute treatment with oral anticoagulation is recommended for up to 12 months after CVT. Long-term anticoagulation is recommended only in patients suffering from a severe coagulopathy or with recurrent CVT.

Abstract 3758: Long-term Recurrence of Venous Thrombotic Events After Cerebral Vein Thrombosis

Background. It is assumed that cerebral venous thrombosis (CVT) implies a lower risk of death and recurrence at short- and mid-term follow-up; however, few information exist on risk factors and outcome of thrombotic venous recurrence after CVT in the long run. We aimed to describe the 20-year recurrence of intra and extracranial venous thrombotic events after an index CVT in a large cohort. Methods. Among 412 consecutive patients, we analyzed 336 (81.6%) cases (82,7% women, mean age: 30.9 years, range: 14 to 83 years) registered from 1986 to 2010 in a third-level referral center of Mexico City, for whom complete information of recurrent events was available and who survived the acute phase of CVT. Results. In a median follow-up of 28 months (range 2 to 288 months), the recurrence rate of either systemic or intracranial venous thrombotic events was 6.8% (n=23): 2.7% of recurrent CVT, 3% lower-limb thrombosis and 1.1% pulmonary embolism). Recurrent venous events occurred by a median of 5 months (range: 1 to 113 months), with 78% cases recurring in the following year after index CVT. Nonetheless, recurrent CVT occurred within 5 months in 78% cases. None of the recurrent CVT events caused death, but 50% of pulmonary embolisms were mortal. In a Cox proportional hazards model adjusted for multiple confounders, baseline factors associated with an increased risk of recurrent intra or extracranial venous thrombosis were thrombosis of jugular veins [hazard ratio (HR): 5.13, 95% confidence interval (CI): 1.39-18.9], diagnosis of anti-phospholipid syndrome (HR: 2.67, 95% CI: 1.01-7.07), mechanical ventilation (HR: 2.07, 95% CI: 1.08-3.93), and alcoholism (HR: 3.44, 95% CI: 1.12-10.58). Conclusion. Most venous recurrent events occur in the following year after index CVT. Recurrent CVT is not associated with a high mortality, but extracranial venous thromboembolisms can be fatal. This study is the largest to date on this topic.

Recurrent cerebral venous thrombosis revealing paraneoplastic angiitis in Hodgkin’s lymphoma

Recurrent cerebral venous thrombosis (CVT), as a manifestation of paraneoplastic angiitis and revealing of nodular lymphocyte predominant Hodgkin's disease (NLPHD), is an extremely rare condition. We herein report a 55-year-old man who developed recurrent CVT despite efficacious anticoagulant therapy and subsequent stenting of the superior longitudinal sinus. Progressive neurological deterioration ensued and a body scan revealed axillary lymph nodes. Pathological analysis led to a diagnosis of NLPHD. Conventional angiography showed CVT and multiple arterial narrowings. A paraneoplastic primary cerebral angiitis with prominent venous structure involvement was suspected. Immunotherapy using rituximab and steroids provided a dramatic recovery. This case of CVT due to paraneoplastic cerebral angiitis is a rare condition and represents a new, very rare manifestation of nodular lymphocyte predominant Hodgkin's disease.

Contribution of multiple thrombophilic and transient risk factors in the development of cerebral venous thrombosis

Introduction Cerebral venous thrombosis (CVT), deep vein thrombosis (DVT) and/or pulmonary embolism (PE) have been associated with thrombophilic defects. However, in contrast to DVT or PE, CVT is a rare disease. We performed a study to identify differences in thrombotic risk profile, predisposing to CVT rather than DVT or PE, particularly the contribution of oral contraception and 11 thrombophilic defects. Materials and methods A single center case-control study (63 CVT cases and 209 controls with DVT or PE) was performed. Results Of CVT patients, 11% had experienced prior DVT or PE, and none had recurrent CVT at 5 years follow-up. CVT was more frequently observed in females (79% versus 51%, P < 0.001). It was more often secondary (75% versus 50%, P < 0.001), mainly due to the difference in age between both groups. At presentation of CVT and DVT/PE, oral contraceptives were used by 78% and 74% of non-pregnant fertile women ( P = 0.8), respectively. Any thrombophilic defect was demonstrated in 88% of CVT and 75% of DVT/PE patients ( P = 0.22), sex and age matched. Individual and two or more defects were equally distributed among both groups. Conclusions We conclude that a majority of CVT and DVT or PE patients show single or multiple thrombophilic defects. At presentation, oral contraceptive intake was observed more frequently in CVT patients. However, no differences were observed in thrombotic risk profile between both groups of comparable age. Hence, additional unknown risk factors should be considered to explain the different sites of thrombosis in these patients.

Risk of thromboembolism after cerebral venous thrombosis

The outcome of cerebral venous thrombosis (CVT) has been studied infrequently. We assessed the frequency of recurrence of cerebral or systemic thromboembolism and factors influencing recurrence. We performed a retrospective study of consecutive patients with CVT in the period 1985-2002 who were admitted to the University Hospital Gasthuisberg. We performed a chart review and a semi-standardized telephone interview that focused on recurrent CVT or systemic thromboembolism. Fifty-four CVT patients with a mean age of 42 years were followed up for a mean of 3.5 years. Eighty percent were women. Coagulation disorders were found in 17 patients (31%). One patient (1.9%) had recurrent CVT and seven patients (12.9%) suffered systemic thromboembolism after a median of 2.5 months. Patients with recurrent thromboembolism more often had coagulopathies (P = 0.04) or a history of deep venous thrombosis (P = 0.007). Patients with early recurrent venous thromboembolism often were not treated with oral anticoagulants (P < 0.001). It was evident from the above study that a substantial number of patients suffer recurrent thromboembolism after CVT.

Recurrent Cerebral Venous Thrombosis Associated with Elevated Factor VIII

Cerebral venous thrombosis (CVT) rarely recurs, and the factors associated with a recurrence remain unclear. Recently, however, elevated plasma coagulation factor VIII has been considered a factor related to recurrent venous thromboembolism. Here we report a patient who had recurrent CVT associated with significantly elevated levels of factor VIII despite the chronic use of an antiplatelet agent. Factor IX was also elevated in this patient. These findings suggest that elevated factor VIII is a factor underlying the recurrence of CVT, and that prolonged anticoagulation therapy may have to be considered in patients with elevated coagulation factor levels.

Recurrent cerebral venous thrombosis: An Arg359X mutation in the antithrombin gene in a Taiwanese family

Recurrent cerebral venous thrombosis: An Arg359X mutation in the antithrombin gene in a Taiwanese family