Cerebral venous thrombosis in patients with autoimmune disease, hematonosis or coronavirus disease 2019: Many familiar faces and some strangers.

BACKGROUND: Cerebral venous thrombosis, a rare stroke, is characterized by neurological dysfunction caused by bleeding and/or infarction resulting from venous sinus thrombosis, the so-called venous stroke. Current guidelines recommend anticoagulants as first-line therapy in the treatment of venous stroke. With complicated causes of cerebral venous thrombosis, treatment is difficult, especially when combined with autoimmune diseases, blood diseases, and even COVID-19. AIMS: This review summarizes the pathophysiological mechanisms, epidemiology, diagnosis, treatment, and clinical prognosis of cerebral venous thrombosis combined with autoimmune diseases, blood diseases, or infectious diseases such as COVID-19. CONCLUSION: A systematic understanding of particular risk factors that should not be neglected when unconventional cerebral venous thrombosis occurs and for a scientific understanding of pathophysiological mechanisms, clinical diagnosis, and treatment, thus contributing to knowledge on special types of venous stroke.

Decompressive surgery in cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia.

BACKGROUND AND PURPOSE: Cerebral venous sinus thrombosis due to vaccine-induced immune thrombotic thrombocytopenia (CVST-VITT) is an adverse drug reaction occurring after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. CVST-VITT patients often present with large intracerebral haemorrhages and a high proportion undergoes decompressive surgery. Clinical characteristics, therapeutic management and outcomes of CVST-VITT patients who underwent decompressive surgery are described and predictors of in-hospital mortality in these patients are explored. METHODS: Data from an ongoing international registry of patients who developed CVST within 28 days of SARS-CoV-2 vaccination, reported between 29 March 2021 and 10 May 2022, were used. Definite, probable and possible VITT cases, as defined by Pavord et al. (N Engl J Med 2021; 385: 1680-1689), were included. RESULTS: Decompressive surgery was performed in 34/128 (27%) patients with CVST-VITT. In-hospital mortality was 22/34 (65%) in the surgical and 27/94 (29%) in the non-surgical group (p < 0.001). In all surgical cases, the cause of death was brain herniation. The highest mortality rates were found amongst patients with preoperative coma (17/18, 94% vs. 4/14, 29% in the non-comatose; p < 0.001) and bilaterally absent pupillary reflexes (7/7, 100% vs. 6/9, 67% with unilaterally reactive pupil, and 4/11, 36% with bilaterally reactive pupils; p = 0.023). Postoperative imaging revealed worsening of index haemorrhagic lesion in 19 (70%) patients and new haemorrhagic lesions in 16 (59%) patients. At a median follow-up of 6 months, 8/10 of surgical CVST-VITT who survived admission were functionally independent. CONCLUSIONS: Almost two-thirds of surgical CVST-VITT patients died during hospital admission. Preoperative coma and bilateral absence of pupillary responses were associated with higher mortality rates. Survivors often achieved functional independence.

Hem coagulase induced cerebral venous sinus thrombosis in patients with uterine fibroids surgery.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a rare, high-risk, and easily misdiagnosed disease. Currently, there are case reports of hem coagulase-induced thrombotic events, but no reports of CVST being associated with hem coagulase. CASE SUMMARY: A 35-years-old woman presented to the outpatient clinic with a severe headache and sudden memory loss with intravenous hem coagulase for postoperative bleeding after uterine fibroids surgery. Abnormal neurological signs included slowed reactions, poor memory, and decreased numeracy. Magnetic resonance imaging and computed tomography scan showed multiple cerebral infarcts, and the infarct area was non-arterial. Brain magnetic resonance venography showed obstruction of the left sigmoid sinus. High-resolution magnetic resonance imaging of the left sigmoid sinus showed abnormally high signal. The patient was treated with a subcutaneous Low-Molecular-Weight Heparin Sodium injection 0.4 ml, twice a day (7 days), and oral Warfarin Sodium 3 mg, once a day, while monitoring the international normalized ratio, adjust the warfarin sodium dosage according to the international normalized ratio level. One month later, the patient had no neurological symptoms and her cognitive function returned to normal. CONCLUSION: hem coagulase may be a contributing factor to CVST in patients undergoing uterine fibroids surgery and should be administered intravenously with caution.

Cerebral Venous Thrombosis With Topical Estrogen-Progesterone Use in a Young Heterosexual Male: A Case Report.

INTRODUCTION: Our report sheds light on the risk of topical hormonal use in relation to cerebral venous sinus thrombosis. We diagnosed our patient with cerebral venous thrombosis (CVT) using computed tomography venogram, then detailed history and examination were obtained, and thorough blood tests and imaging were done to exclude other causes of CVT like thrombophilias, infections, and malignancies. CASE REPORT: Our patient is a 37-year-old heterosexual male, presented with headache only. The computed tomography venogram showed extensive CVT in the right internal jugular vein, sigmoid, transverse, and straight cerebral venous; detailed history and investigations suggest that his use of crushed oral contraceptive pills mixed with water topically on the scalp is the most important predisposing factor. This patient was managed with anticoagulants and is being followed in the clinic. CONCLUSION: Oral hormonal use in contraceptives is a known risk factor for CVT. This case sheds light on the importance of topical hormonal use concerning CVT in females and males; it stresses the need for more studies in that area, as it is poorly studied.

Cerebral venous sinus thrombosis after COVID-19 vaccination and congenital deficiency of coagulation factors: Is there a correlation?

In January 2020, SARS-COV-2 infection spread worldwide and was declared "pandemic" by WHO. Because of the high contagiousness of the virus and devastating effects of the epidemic on public health, numerous efforts have been made to develop suitable vaccines to prevent the infection. Among the side effects developed by patients who undergone vaccination, there are common symptoms but also more serious reactions such as the thrombosis syndromes. This paper presents two cases of thrombosis temporally associated with live-vectored Covid vaccination similar to vaccine-induced thrombocytopenia (VITT) in patients with inherited thrombophilia (respectively, the deficiency of protein S and a Factor II mutation). The clinical manifestation caused by VITT is characterized by widespread thrombosis especially affecting intracranial venous sinus, which may cause massive bleeding and intracranial hemorrhage. Although this condition is widely described in literature, there is no evident correlation between this side effect and inherited condition of thrombophilia. The authors suggest that the presence of inherited thrombophilia should be better investigated and, if necessary, screened during the anamnestic data collection before the vaccine administration, leading the healthcare professional to choose the appropriate vaccine to the patient.

Vaccine-induced immune thrombotic thrombocytopenia in a male after Ad26.COV2.S vaccination presenting as cerebral venous sinus thrombosis.

Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a rare and life-threatening complication that can occur after COVID-19 vaccination. After the first reports of VITT and CVST in 2021 after Ad26.COV2.S vaccination, the FDA and CDC recommended an emergency pause on 13 April 2021, and after extensive safety reviews, on 23 April 2021, the CDC's Advisory Committee on Immunization Practices (ACIP) reaffirmed its original recommendation for use of the Ad26.COV2.S vaccination. As of 31 August 2021, in the United States, 54 cases of VITT following Ad26.COV2.S vaccination (37 female, 17 male) have been reported out of 14.1 million total shots given, 29 of which had cerebral venous sinus thrombosis (CVST). With more data, on 16 December 2021, the CDC endorsed the ACIP recommendations for individuals to receive an mRNA COVID-19 vaccine in preference over the Ad26.COV2.S vaccination. We report a rare case of a male with confirmed VITT and CVST following Ad26.COV2.S vaccination.

Thromboembolism after COVID-19 Vaccination: A Systematic Review of Such Events in 286 Patients.

BACKGROUND: Development of vaccines with high efficacy against COVID-19 disease has ushered a new ray of hope in the fight against the pandemic. Thromboembolic events have been reported after administration of vaccines. We aim to systematically review thromboembolic events reported after COVID-19 vaccination. METHODS: The available literature was systematically screened for available data on thromboembolic events after COVID-19 vaccination. Data were extracted from selected studies and analyzed for site of thromboembolism as well as other risk factors. All data were pooled to determine cumulative incidence of thromboembolism at various sites after vaccination. RESULTS: A total of 20 studies were selected for the final analysis. The mean age of the population was 48.5 ± 15.4 years (females - 67.4%). The mean time to event after vaccination was 10.8 ± 7.2 days. Venous thrombosis (74.8%, n = 214/286) was more common than arterial thrombosis (27.9%, n = 80/286). Cerebral sinus thrombosis was the most common manifestation (28.3%, n = 81/286) of venous thrombosis followed by deep vein thrombosis (19.2%, n = 49/254). Myocardial infarction was common (20.1%, n = 55/274) in patients with arterial thrombosis followed by ischemic stroke (8.02%, n = 22/274). Concurrent thrombosis at multiple sites was noted in 15.4% patients. Majority of patients had thrombocytopenia (49%) and antiplatelet factor 4 antibodies (78.6%). Thromboembolic events were mostly reported after the AstraZeneca vaccine (93.7%). Cerebral sinus thrombosis was the most common among thromboembolic events reported after the AstraZeneca vaccine. Among the reported cases, mortality was noted in 29.9% patients. CONCLUSIONS: Thromboembolic events can occur after COVID-19 vaccination, most commonly after the AstraZeneca vaccine. Cerebral sinus thrombosis is the most common manifestation noted in vaccinated individuals.

Venous sinus thrombosis after the first dose of Pfizer BioNTech vaccine.

mRNA vaccines including Pfizer BioNTech and Moderna have categorically been considered safe when it comes to preventing COVID-19. However, there is still a small associated risk of thromboembolic phenomenon including venous sinus thrombosis with it and our case report highlights one.We describe a patient who developed severe progressive headache, tinnitus and visual disturbance symptoms post-Pfizer-SARS-CoV-2 vaccination. His medical history included essential tremors, hypertension, type 2 diabetes mellitus, chronic kidney disease stage 3, anxiety, depression and long-term catheterisation. Systemic examination revealed hypotonia, generalised reduced power and central diplopia along with peripheral visual field defect in the left eye. He was extensively investigated, the COVID-19 PCR test was negative and all routine blood tests were in the normal range except a marginally raised D-dimer of 779 ng/mL. CT head was unremarkable. He was also tested for myasthenia gravis; however, acetylcholine receptors antibodies were negative and nerve conduction studies were normal. Subsequent MRI of the brain with venography confirmed venous sinus thrombosis. A 24-hour Holter monitoring test did not reveal any cardiac rate or rhythm abnormality. He was treated with apixaban as per a neurologist's advice. His clinical condition started to improve and was later discharged from the hospital with an outpatient neurologist clinic follow-up.

Massive cerebral venous sinus thrombosis in vaccine-induced immune thrombotic thrombocytopenia after ChAdOx1 nCoV-19 serum: case report of a successful multidisciplinary approach.

We report a case of massive cerebral venous sinus thrombosis in the contest of vaccine-induced immune thrombotic thrombocytopenia that required the rapid coordination of many specialists from different departments, notably emergency, neurology, neuroradiology, hematology, and neurosurgery. The patient was rapidly treated with steroids, immunoglobulin, and fondaparinux. She underwent within 6 h after hospital admission a mechanical thrombectomy in order to allow flow restoration in cerebral venous systems. Neuroendovascular treatment in cerebral venous thrombosis related to VITT has never been described before. It can represent a complementary tool along with the other therapies and a multidisciplinary approach.

Late-Onset Vaccine-Induced Immune Thombotic Thrombocytopenia (VITT) with Cerebral Venous Sinus Thrombosis.

OBJECTIVES: Vaccine-induced thrombotic thrombocytopenia (VITT) is a rare complication after adenoviral vector vaccination against COVID-19 reported up to 24 days after ChAdOx1 nCOV-19 (AZD1222) vaccination. This report describes a case with a significantly later onset of VITT with cerebral venous sinus thrombosis. CASE DESCRIPTION: We report a 42-year-old woman presenting to the emergency department 53 days after AZD1222 vaccination with sudden onset sensory aphasia and an 18-day history of headache. Cranial computed tomography (CT) showed acute intracranial hemorrhage and CT venogram demonstrated thrombosis of the left vein of Labbé and transverse and sigmoid sinus. D-dimers were elevated and despite a normal platelet count, platelet-activating anti-PF4 antibody testing was positive, confirming the diagnosis of VITT. The patient was treated with intravenous immunoglobulins and argatroban, and was discharged without any neurological deficit on day 12. CONCLUSION: Our report of VITT with symptom onset on day 35 and diagnosis of cerebral sinuous thrombosis on day 53 after AZD1222 vaccination significantly enhances the time window during which VITT may occur.

Cerebral venous sinus thrombosis associated with vaccine-induced thrombotic thrombocytopenia.

Thrombosis-thrombocytopenia syndrome and cerebral venous sinus thrombosis have been rarely reported in patients who have received severe acute respiratory syndrome coronavirus 2 adenoviral vector vaccines. Awareness of this potential adverse effect, recognizing early clinical symptoms and subtle signs of cerebral venous sinus thrombosis on head computed tomography and brain magnetic resonance imaging, appropriate vascular imaging, and unique treatment for this condition is critical. This is a report of a case of vaccine-induced thrombotic thrombocytopenia and associated cerebral venous sinus thrombosis with emphasis on imaging and clinical course.

Cerebral venous sinus thrombosis secondary to ChAdOx-1 nCov-19 vaccine.

Thrombosis and thrombocytopaenia secondary to ChAdOx-1 nCov-19 vaccine is a new phenomenon that usually occurs after the first dose of vaccine. Most of these patients are healthy without any prior history of thromboembolic events or heparin use. Hall marks of this condition include detectable antibodies to platelet factor 4 and thrombosis at atypical sites particularly cerebral veins and sinuses mimicking atypical heparin induced thrombocytopaenia. We describe a case of a patient who was diagnosed with this rare condition and treated successfully.

Cerebral venous sinus thrombosis after ChAdOx1 vaccination: the first case of definite thrombosis with thrombocytopenia syndrome from India.

Cerebral venous sinus thrombosis (CVST) following novel coronavirus-2019 (nCoV-19) vaccination is a rare adverse effect. We report the first case of CVST associated with ChAdOx1 vaccination, with positive anti-platelet factor 4 (PF4) antibodies, from India. A 44-year-old woman developed a thunderclap headache 4 days after the first dose of the adenoviral vector vaccine ChAdOx1 (Covishield). Physical examination was unremarkable barring mild neck stiffness with no focal neurological deficits. MRI identified right transverse sinus thrombosis. Laboratory tests revealed raised D-dimer and thrombocytopenia; anti-PF4 antibodies were subsequently identified, consistent with thrombosis with thrombocytopenia syndrome (TTS). She was treated with non-heparin anticoagulation and intravenous immunoglobulin and made an uneventful recovery. Early recognition of adenoviral vector vaccine-related TTS, which resembles heparin-induced thrombocytopenia syndrome, is important as heparin and heparin analogues are best avoided in the treatment.

Characteristics and Outcomes of Patients With Cerebral Venous Sinus Thrombosis in SARS-CoV-2 Vaccine-Induced Immune Thrombotic Thrombocytopenia.

Importance: Thrombosis with thrombocytopenia syndrome (TTS) has been reported after vaccination with the SARS-CoV-2 vaccines ChAdOx1 nCov-19 (Oxford-AstraZeneca) and Ad26.COV2.S (Janssen/Johnson & Johnson). Objective: To describe the clinical characteristics and outcome of patients with cerebral venous sinus thrombosis (CVST) after SARS-CoV-2 vaccination with and without TTS. Design, Setting, and Participants: This cohort study used data from an international registry of consecutive patients with CVST within 28 days of SARS-CoV-2 vaccination included between March 29 and June 18, 2021, from 81 hospitals in 19 countries. For reference, data from patients with CVST between 2015 and 2018 were derived from an existing international registry. Clinical characteristics and mortality rate were described for adults with (1) CVST in the setting of SARS-CoV-2 vaccine-induced immune thrombotic thrombocytopenia, (2) CVST after SARS-CoV-2 vaccination not fulling criteria for TTS, and (3) CVST unrelated to SARS-CoV-2 vaccination. Exposures: Patients were classified as having TTS if they had new-onset thrombocytopenia without recent exposure to heparin, in accordance with the Brighton Collaboration interim criteria. Main Outcomes and Measures: Clinical characteristics and mortality rate. Results: Of 116 patients with postvaccination CVST, 78 (67.2%) had TTS, of whom 76 had been vaccinated with ChAdOx1 nCov-19; 38 (32.8%) had no indication of TTS. The control group included 207 patients with CVST before the COVID-19 pandemic. A total of 63 of 78 (81%), 30 of 38 (79%), and 145 of 207 (70.0%) patients, respectively, were female, and the mean (SD) age was 45 (14), 55 (20), and 42 (16) years, respectively. Concomitant thromboembolism occurred in 25 of 70 patients (36%) in the TTS group, 2 of 35 (6%) in the no TTS group, and 10 of 206 (4.9%) in the control group, and in-hospital mortality rates were 47% (36 of 76; 95% CI, 37-58), 5% (2 of 37; 95% CI, 1-18), and 3.9% (8 of 207; 95% CI, 2.0-7.4), respectively. The mortality rate was 61% (14 of 23) among patients in the TTS group diagnosed before the condition garnered attention in the scientific community and 42% (22 of 53) among patients diagnosed later. Conclusions and Relevance: In this cohort study of patients with CVST, a distinct clinical profile and high mortality rate was observed in patients meeting criteria for TTS after SARS-CoV-2 vaccination.