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Cerebral venous thrombosis (CVT) is a multifactorial disease. The idiopathic form represents 12.5% of all CVTs and is diagnosed by excluding known risk factors. As for any form of venous thromboembolism, thrombophilia should be suspected in patients with recurrent CVT or less than 45 years of age or positive family history for venous thrombosis or...
Citations
... Predisposing variants of thrombophilia include antithrombin, protein C and protein S deficiency, prothrombin polymorphism, factor V Leiden mutation, antiphospholipid antibodies, hyperhomocysteinemia, pregnancy, puerperium, surgery, cancer, immobilization, oral contraceptives, and inflammatory bowel disease. Any combination of such factors may increase the risk of IVST [8,9]. ...
Background:
As of 8 April 2021, a total of 2.9 million people have died with or from the coronavirus infection causing COVID-19 (Corona Virus Disease 2019). On 29 January 2021, the European Medicines Agency (EMA) approved a COVID-19 vaccine developed by Oxford University and AstraZeneca (AZD1222, ChAdOx1 nCoV-19, COVID-19 vaccine AstraZeneca, Vaxzevria, Covishield). While the vaccine prevents severe course of and death from COVID-19, the observation of pulmonary, abdominal, and intracranial venous thromboembolic events has raised concerns.
Objective:
To describe the clinical manifestations and the concerning management of patients with cranial venous sinus thrombosis following first exposure to the "COVID-19 vaccine AstraZeneca".
Methods:
Patient files, laboratory findings, and diagnostic imaging results, and endovascular interventions of three concerning patients were evaluated in retrospect.
Results:
Three women with intracranial venous sinus thrombosis after their first vaccination with "COVID-19 vaccine AstraZeneca" were encountered. Patient #1 was 22 years old and developed headaches four days after the vaccination. On day 7, she experienced a generalized epileptic seizure. Patient #2 was 46 years old. She presented with severe headaches, hemianopia to the right, and mild aphasia 13 days after the vaccination. MRI showed a left occipital intracerebral hemorrhage. Patient #3 was 36 years old and presented 17 days after the vaccination with acute somnolence and right-hand hemiparesis. The three patients were diagnosed with extensive venous sinus thrombosis. They were managed by heparinization and endovascular recanalization of their venous sinuses. They shared similar findings: elevated levels of D-dimers, platelet factor 4 antiplatelet antibodies, corona spike protein antibodies, combined with thrombocytopenia. Under treatment with low-molecular-weight heparin, platelet counts normalized within several days.
Conclusion:
Early observations insinuate that the exposure to the "COVID-19 vaccine AstraZeneca" might trigger the expression of antiplatelet antibodies, resulting in a condition with thrombocytopenia and venous thrombotic events (e.g., intracranial venous sinus thrombosis). These patients' treatment should address the thrombo-embolic manifestations, the coagulation disorder, and the underlying immunological phenomena.
... Bu çalışmada trombofili etiyolojili SVT oranı %32,6 olarak tespit edildi. Batı ülkelerinde MTHFR C677T, FV Leiden, FII 20210G/A ve mutasyonlarının tromboembolizm için en yaygın risk faktörleri olduğu bildirilmiştir (21,22). MTHFR enzimi, folat metabolizmasında önemli bir enzimdir. ...
Amaç: Serebral ven trombozu (SVT) nadir bir inme nedeni olup etiyolojisinde birçok faktör yer almaktadır. Olguların en az 1/4'ü trombofiliye bağlıdır. Tromboembolizm için en yaygın risk faktörleri metilen-tetra-hidro-folat redüktaz (MTHFR) C677T, faktör 5 (FV) G1691A (Leiden), faktör 2 (FII) GA20210 ve mutasyonlarıdır. Farklı genetik polimorfizmleri ve yüksek homosistein düzeyleri ile ilişkisi de araştırılmıştır. Bu çalışmada SVT’li olgularda genetik polimorfizm varlığı ve homosistein düzeylerinin SVT etiyolojisindeki rolünün araştırılması amaçlanmıştır.Gereç ve Yöntemler: Ocak 2010-Haziran 2018 yılları arasında merkezimizde geliş tanısı SVT olan hastaların demografik özellikleri, klinik, radyolojik ve laboratuvar verileri geriye dönük olarak incelendi. SVT için etiyolojik risk faktörleri ve bu risk faktörleri içinde genetik polimorfizmin rolü araştırıldı.Bulgular: Çalışmada 92 (73 kadın ve 19 erkek) hasta ve 52 (44 kadın ve 8 erkek) kontrol birey değerlendirildi. SVT’li hastalarda en sık başvuru semptomu baş ağrısı idi. MTHFR, Faktör 13 (F13) V34L, plazminojen aktivatör inhibitörü (PAI) ve β-fibrinojen mutasyonları kontrol grubunda daha yüksek idi. FV Leiden, FII, Glikoprotein 3a mutasyonu ve homosistein düzeyi açısından her iki grup arasında anlamlı istatistiksel fark tespit edilmedi.Sonuç: Bu çalışmada literatür ile uyumlu olan sonuçlar yanında bazı farklı sonuçlarda tespit edilmiştir. MTHFR (C677T, A1289C), FV Leiden, FII G20210, β-fibrinojen 455 G-A, PAI-1 4G/5G polimorfizmleri SVT için risk oluşturmamaktadır. F13 V34L polimorfizminin SVT'ye karşı koruyucu rolü vardır.
... [4] Various identified. [2] FV G1691A (Leiden), FII GA20210, and MTHFR C677T mutations are the most common genetic risk factors for thromboembolism in Western countries [8,9] however, there is rare data about the association of these mutation and CVST in Iran. Therefore, the aim of this study is to evaluate the frequency of common genetic thrombophilic factors in CVST and to offer practical suggestion for doing laboratory investigations in CVST patients. ...
Background:
Factor V G1691A (FV Leiden), FII GA20210, and methylenetetrahydrofolate reductase (MTHFR) C677T mutations are the most common genetic risk factors for thromboembolism in the Western countries. However, there is rare data in Iran about cerebral venous and sinus thrombosis (CVST) patients. The aim of this study was to evaluate the frequency of common genetic thrombophilic factors in CVST patients.
Materials and methods:
Forty consequently CVST patients from two University Hospital in Isfahan University of Medical Sciences aged more than 15 years from January 2009 to January 2011 were recruited. In parallel, 51 healthy subjects with the same age and race from similar population selected as controls. FV Leiden, FII GA20210, MTHFR C677T, and FV Cambridge gene mutations by polymerase chain reaction technique were evaluated in case and control groups.
Results:
FV Leiden, FII GA20210, and FV Cambridge gene mutations had very low prevalence in both case (5%, 2%, 0%) and control (2.5%, 0%, 0%) and were not found any significant difference between groups. MTHFR C677T mutations was in 22 (55%) of patients in case group and 18 (35.5%) of control group (P = 0.09).
Conclusion:
This study showed that the prevalence of FV Leiden, FII GA20210, and FV Cambridge were low. Laboratory investigations of these mutations as a routine test for all patients with CVST may not be cost benefit.
... Antiphospholipid antibody (aPL) and anticardiolipin antibody (aCL) result in an increased tendency to arterial or venous thrombosis (119) as CVST (42,120). aCL antibodies have been reported in 5-22Á6% of CVST patients (4,27,121). Christopher and colleagues studied 31 patients with a documented diagnosis of CVST. ...
Cerebral venous sinus thrombosis is an uncommon disease marked by clotting of blood in cerebral venous, or dural sinuses, and, in rare cases, cortical veins. It is a rare but potentially fatal cause of acute neurological deterioration previously related to otomastoid, orbit, and central face cutaneous infections. After the advent of antibiotics, it is more often related to neoplasm, pregnancy, puerperium, systemic diseases, dehydration, intracranial tumors, oral contraceptives, and coagulopathies are the most common causes, but in 30% of cases no underlying etiology can be identified. It has been found in association with fibrous thyroiditis, jugular thrombosis after catheterization, or idiopathic jugular vein stenosis. Other factors include surgery, head trauma, arterio-venous malformations, infection, paraneoplastic, and autoimmune disease. This article presents a comprehensive review of cerebral venous sinus thrombosis etiologies.
Cerebrovascular diseases are considered in a different way concerning their etiology with regard to arterial and venous occlusion. The role of thrombophilia in this context remains undetermined. For this reason, a case-control study was conducted including a total of 202 patients (154 females, 48 males) aged from 18 to 76 years (mean: 39.8 years) suffering either from cerebral sinus venous thrombosis (n = 101) or from arterial ischemic stroke (n = 101). Study groups were evaluated on the basis of age- and gender-matched pairs. Gene mutations of factor V-1691 (factor V Leiden) and prothrombin-20210 being considered as the most common thrombophilia markers were analyzed in this study. Factor V Leiden-mutations were found in 16.8% of patients with cerebral sinus venous thrombosis (CVT) and in 17.8% of patients with arterial ischemic stroke (AIS), which was significantly more frequent than in controls at a rate of 4.95% (ORs: 3.89 and 4.16). Prothrombin-mutations were significantly more frequent in CVT at a rate of 14.9% versus 2.97% in controls (OR: 5.70). This does not apply for AIS showing a rate of 4.95% prothrombin-mutations. Rates of factor V Leiden-mutations are not different in CVT compared with AIS. In contrast, however, prothrombin-mutations were significantly more frequent in CVT than in AIS with a rate of 14.9% versus 4.95% (OR 3.35). Furthermore, 3 cases with combined heterozygosity of factor V Leiden- and prothrombin-mutation have been identified in CVT, but not in AIS or controls. All of the above mentioned mutations were exclusively heterozygous. We conclude from these data that thrombophilia in terms of factor V Leiden genotype is a risk factor for both CVT and AIS in equal measure. In contrast, prothrombin-20210-mutations were different playing a significant role in the pathogenesis of cerebral sinus vein- thrombosis, but not in arterial ischemic stroke. Also, the combined occurrence of heterozygous prothrombin- and factor V Leiden-mutation clearly favors the emergence of cerebral sinus venous thrombosis. Therefore, in terms of thrombophilia such as investigated in this study, pathogenesis of arterial and venous occlusions in cerebrovascular disease has to be regarded as different.
Background: Cerebral venous thrombosis (CVT) is rare and involves thrombosis of the veins and sinuses of the brain, most commonly the superior sagittal sinus. Approximately 5 CVT cases occur per 1 million persons in western countries. CVT causes 0.5% of strokes. Early diagnosis is crucial to prevent such outcomes as hydrocephalus, intracranial hypertension, and further seizures. Standard medical treatment of CVT consists of low-molecular-weight heparin and endovascular thrombolysis. Small case reports have found that the newer oral anticoagulants can be used for CVT treatment; however, they are associated with increased risk of bleeding and other adverse effects. Review Summary: CVT can be triggered by an imbalance of the body’s homeostasis or reduced action of the intrinsic antithrombotic mechanism. Factors influencing this change include infection, brain tumor, inflammatory conditions, genetic thrombophilias, head trauma that causes intracranial bleeding, and certain medications. CVT may cause brain infarction and increased intracranial pressure. Sometimes, idiopathic intracranial hypertension presents as the only clinical manifestation. Confirmation of the diagnosis typically is through neuroimaging. Current CVT treatment depends on disease extent and severity. Conclusions: CVT is a rare neurological disease with potentially serious implications and high neurological morbidity and mortality rates. Understanding the role of risk factors—such as genetic or acquired thrombophilia, pregnancy, use of oral contraceptives, and hyperhomocysteinemia—in CVT development is important. Although heparin and warfarin have been used for more than 50 years, newer oral anticoagulants (eg, dabigatran, rivaroxaban, apixaban) might offer an alternative to traditional therapy.
The A>G polymorphism at position 19911 of the prothrombin gene is associated with a mildly increased risk of venous thromboembolism, alone or in association with such common thrombophilia mutations as factor V Leiden and prothrombin 20210 GA. Its role in cerebral sinus-venous thrombosis (CSVT) is not known.
The presence of prothrombin 19911 A>G was investigated in a case–control study of 107 patients with cerebral thrombosis and factor V Leiden (n = 25), prothrombin 20210 GA (n = 47), without known thrombophilia (n = 35) and 842 healthy individuals with the corresponding coagulation profile.
Prothrombin 19911 A>G did not increase the risk of CSVT in carriers of factor V Leiden (adjusted odds ratio 1.6, 95%CI 0.6–4.7), prothrombin 20210 GA (odds ratio 1.1, 95%CI 0.6–2.2), nor in patients without known thrombophilia (odds ratio 1.3, 95%CI 0.5–3.1).
Prothrombin 19911 A>G polymorphism does not appear to be a risk factor for CSVT, alone or in association with factor V Leiden or prothrombin 20210GA.
