Coma in adult cerebral venous thrombosis: The BEAST study.

BACKGROUND AND PURPOSE: Coma is an independent predictor of poor clinical outcomes in cerebral venous thrombosis (CVT). We aimed to describe the association of age, sex, and radiological characteristics of adult coma patients with CVT. METHODS: We used data from the international, multicentre prospective observational BEAST (Biorepository to Establish the Aetiology of Sinovenous Thrombosis) study. Only positively associated variables with coma with <10% missing data in univariate analysis were considered for the multivariate logistic regression model. RESULTS: Of the 596 adult patients with CVT (75.7% women), 53 (8.9%) patients suffered coma. Despite being a female-predominant disease, the prevalence of coma was higher among men than women (13.1% vs. 7.5%, p = 0.04). Transverse sinus thrombosis was least likely to be associated with coma (23.9% vs. 73.3%, p < 0.001). The prevalence of superior sagittal sinus thrombosis was higher among men than women in the coma sample (73.6% vs. 37.5%, p = 0.01). Men were significantly older than women, with a median (interquartile range) age of 51 (38.5-60) versus 40 (33-47) years in the coma (p = 0.04) and 44.5 (34-58) versus 37 (29-48) years in the non-coma sample (p < 0.001), respectively. Furthermore, an age- and superior sagittal sinus-adjusted multivariate logistic regression model found male sex (odds ratio = 1.8, 95% confidence interval [CI] = 1.0-3.4, p = 0.04) to be an independent predictor of coma in CVT, with an area under the receiver operating characteristic curve of 0.61 (95% CI = 0.52-0.68, p = 0.01). CONCLUSIONS: Although CVT is a female-predominant disease, men were older and nearly twice as likely to suffer from coma than women.

Clinical value of neuroimaging indicators of intracranial hypertension in patients with cerebral venous thrombosis.

PURPOSE: Intracranial hypertension (IH) frequently complicates cerebral venous thrombosis (CVT). Distinct neuroimaging findings are associated with IH, yet their discriminative power, reversibility and factors favoring normalization in prospective CVT patients are unknown. We determined test performance measures of neuroimaging signs in acute CVT patients, their longitudinal change under anticoagulation, association with IH at baseline and with recanalization at follow-up. METHODS: We included 26 consecutive acute CVT patients and 26 healthy controls. Patients were classified as having IH based on CSF pressure > 25 cmH2O and/or papilledema on ophthalmological examination or ocular MRI. We assessed optic nerve sheath diameter (ONSD), optic nerve tortuousity, bulbar flattening, lateral and IVth ventricle size, pituitary configuration at baseline and follow-up, and their association with IH and venous recanalization. RESULTS: 46% of CVT patients had IH. ONSD enlargement > 5.8 mm, optic nerve tortuousity and pituitary grade ≥ III had highest sensitivity, ocular bulb flattening and pituitary grade ≥ III highest specificity for IH. Only ONSD reliably discriminated IH at baseline. Recanalization was significantly associated with regressive ONSD and pituitary grade. Other neuroimaging signs tended to regress with recanalization. After treatment, 184.9 ± 44.7 days after diagnosis, bulbar flattening resolved, whereas compared with controls ONSD enlargement (p < 0.001) and partially empty sella (p = 0.017), among other indicators, persisted. CONCLUSION: ONSD and pituitary grading have a high diagnostic value in diagnosing and monitoring CVT-associated IH. Given their limited sensitivity during early CVT and potentially persistent alterations following IH, neuroimaging indicators can neither replace CSF pressure measurement in diagnosing IH, nor determine the duration of anticoagulation.

Exploring thrombus composition in cerebral venous thrombosis: the first case report with initial insights and implications for treatment advancements.

BACKGROUND: Endovascular thrombectomy (EVT) is a treatment option in patients with a cerebral venous thrombosis (CVT) who deteriorate despite anticoagulant treatment. Assessment of thrombus composition in CVT may provide insights into the pathophysiology of the disease and suggest new therapeutic strategies. CASE REPORT: A 47-year-old woman (smoking habit and estradiol/progesterone-releasing intra-uterine device) diagnosed with massive CVT underwent EVT (complete recanalization via aspiration catheter and stentriever) due to acute-onset left-sided weakness and dysarthria despite 72 h of full-dose subcutaneous low-molecular heparin. Two main reddish clots (maximum diameter 15 mm) were retrieved. Microscopic assessment showed an erythrocyte-rich thrombus (83.9% of entire thrombus surface) with layers of platelets/fibrin (lines of Zahn: 13.9% fibrin and 38.5% platelet [CD61+]). The immunological profile was dominated by neutrophils (30% MPO+), with neutrophil extracellular traps (NETs) in 1.9% of thrombus surface. T- (CD3+), B-lymphocytes (CD20+), and monocytes/macrophages (CD68+) were rather rare (2.2%, 0.7%, and 2.0% respectively). We found no evidence (0.0%) of hemosiderin and endothelial cells (CD34+). Full clinical recovery occurred prior to discharge. CONCLUSION: This is the first case report of a CVT with histologic assessment of the thrombus retrieved via EVT. Evaluating thrombi in CVT can provide key insights into disease pathophysiology and guide treatment advancements.

Challenges in diagnosis and management of cerebral venous thrombosis as underlying cause of lobar intracerebral hemorrhage.

OBJECTIVES: Cerebral venous thrombosis is an uncommon, yet life-threatening condition, affecting mainly young and middle-aged individuals. Moreover, it represents an underrecognised etiology of lobar intracerebral hemorrhage (ICH). The clinical course of CVT is variable in the first days after diagnosis and medical complications including pulmonary embolism (PE) may result in early neurological deterioration and death if left untreated. MATERIALS AND METHODS: Case report. RESULTS: We describe a 46-year-old man with acute left hemiparesis and dysarthria in the context of lobar ICH due to underlying CVT of Trolard vein. Diagnosis was delayed because of misinterpretation of the initial neuroimaging study. Subsequently, the patient rapidly deteriorated and developed submassive PE and left iliofemoral venous thrombosis in the setting of previously undiagnosed hereditary thrombophilia (heterozygous prothrombin gene mutation G2021A). Emergent aspiration thrombectomy was performed resulting in the successful management of PE. A follow-up MRI study confirmed the thrombosed Trolard vein, thus establishing the CVT diagnosis. Anticoagulation treatment was immediately escalated to enoxaparine therapeutic dose resulting in clinical improvement of neurological deficits. CONCLUSIONS: Delayed diagnosis of cerebral venous thrombosis with underlying causes of lobar ICH may result in dire complications. Swift initiation of anticoagulants is paramount even in patients with lobar intracerebral hemorrhage as the initial manifestation of cerebral venous thrombosis.

Outcomes and complications of patients with cerebral venous thrombosis: a retrospective study.

OBJECTIVES: To assess outcomes and complications of patients with cerebral venous thrombosis (CVT). METHODS: This multicenter retrospective study was conducted at 2 health care centers in Saudi Arabia and Oman. Adult patients diagnosed with CVT in radiological imaging between 2006 and 2020 were included. Data were collected from medical records and analyzed using the software IBM® SPSS version 22. Neurological disability occurring after CVT was graded according to the modified Rankin scale (mRS). RESULTS: The study included 103 patients, of which the majority (68%) were female. The mean age was 39.12±12.96 years. Two-thirds of patients received low-molecular-weight heparin (LMWH) in acute treatment, while 76% of discharged patients used warfarin. The majority of patients had no or mild neurological disability during follow-up, and 6 patients had an mRS score ≥3, implying significant neurological disability. There were 55 patients (52.3%) who had complications from CVT, including seizures in 17 (16.5%) patients and one mortality. Follow-up imaging of 55 patients showed complete thrombus resolution in 20 patients (36%). CONCLUSION: Anticoagulation is the mainstay treatment for CVT patients. Approximately half of patients experience complications. Prospective studies are needed to assess the long-term neurological outcomes in such patients.

High-resolution magnetic resonance imaging of acute intracranial artery thrombus.

BACKGROUND AND PURPOSE: The development of high-resolution magnetic resonance imaging (HR-MRI) has enabled submillimeter-level evaluation of intracranial artery plaque and luminal thrombus. We sought to investigate the value of HR-MRI in assessing the pathogenesis of acute intracranial artery thrombus. METHODS: We examined the presence of intracranial thrombus on three-dimensional T1-weighted HR-MRI in acute ischemic stroke patients with intracranial artery occlusion on magnetic resonance angiography. We defined two thrombus-related HR-MRI features (peri-thrombus plaque and distal residual flow beyond the thrombus) and analyzed their association with potential embolic sources. RESULTS: Luminal thrombus and a shrunken artery without luminal thrombus were detected in 162 (96.4%) and six (3.6%) of 168 patients with intracranial artery occlusion, respectively. Among 111 patients with culprit major artery thrombus, peri-thrombus plaques were observed in 46.8% and distal residual flow beyond the thrombus in 64.0%. Patients with peri-thrombus plaque had a higher prevalence of diabetes (44.2% vs. 25.4%; p = 0.037), a lower prevalence of potential sources of cardioembolism (0% vs. 16.9%; p = 0.002), and a nonsignificantly lower prevalence of potential embolic sources from extracranial arteries (9.6% vs. 20.3%; p = 0.186) than those without. Patients with distal residual flow beyond the thrombus had a lower prevalence of potential sources of cardioembolism (1.4% vs. 22.5%; p < 0.001) and smaller infarct volumes (5.0 [1.4-12.7] mL vs. 16.6 [2.4-94.6] mL; p = 0.012) than those without. CONCLUSIONS: Our study showed that HR-MRI helps clarify the pathogenesis of acute intracranial artery thrombus. The presence of peri-thrombus plaque and distal residual flow beyond the thrombus favor the stroke mechanism of atherosclerosis rather than cardioembolism.

Secondary polycythaemia from chronic hypoxia is a risk for cerebral thrombosis: a case report.

BACKGROUND: Secondary polycythemia is considered the usual complication of chronic hypoxia. It can theoretically increase the oxygen-carrying capacity, but this adaptive trait has a deleterious effect because the blood viscosity increases, which can induce significant morbidity and mortality, such as stroke and myocardial infarction. CASE PRESENTATION: A 55-year-old man with a history of a congenitally small main pulmonary artery presented to the emergency department with sustained unsteady walking, dizziness and vertigo. Evaluation revealed elevated hemoglobin and superior posterior circulation cerebral artery thrombosis. The patient was treated with high flux inhalation of oxygen and anti-platelet aggregation. CONCLUSIONS: The involvement of cerebral vessels has rarely been reported in chronic hypoxia cases. The present case is the first case of superior posterior circulation cerebral artery thrombosis due to chronic hypoxia in a patient with a congenitally small main pulmonary artery. This case demonstrates the importance of recognizing some chronic diseases that can lead to hypoxia and secondary polycythemia thereby leading to hypercoagulable state and subsequent thrombosis.

Malnutrition and cerebral intraparenchymal damage in patients with thrombosis of dural sinuses and/or cerebral veins.

BACKGROUNDS: Thrombosis of dural sinuses and/or cerebral veins (CVT) is an uncommon form of cerebrovascular disease. Malnutrition is common in patients with cerebrovascular disease, and early assessment of malnutrition and individualized nutritional treatment have been reported to improve functional outcomes of these patients. As for CVT patients, little is known about whether these patients would suffer from malnutrition. Also, the correlation between malnutrition and cerebral intraparenchymal damage (CID) in CVT patients was rarely studied. METHODS: Patients with CVT were retrospectively included in this observational study. Multivariate logistic regressions were used to investigate the effects of nutritional indexes on the risk of CID. Subsequently, we used the independent risk factors to construct the nomogram model, and the consistency index (C-index), calibration curve and decision curve analysis (DCA) to assess the reliability and applicability of the model. RESULTS: A total of 165 patients were included in the final analysis. Approximately 72.7% of CVT patients were regarded as malnourished by our malnutrition screening tools, and malnutrition is associated with an increased risk of CID. Prognostic Nutritional Index (PNI) (OR = 0.873; CI: 0.791, 0.963, p = 0.007) remained as an independent predictor for CID after adjustment for other risk factors. The nomogram model showed that PNI and gender have a great contribution to prediction. Besides, the nomogram model was consistent with the actual observations of CID risk (C-index = 0.65) and was of clinical significance. CONCLUSIONS: We reported that malnutrition, as indicated by PNI, was associated with a higher incidence of CID in CVT patients. Also, we have constructed a nomogram for predicting the risk of CID in these patients.

Clinical characteristics of cerebral venous sinus thrombosis patients with new-onset of headache.

OBJECTIVE: This study aimed to assess the clinical characteristics of cerebral venous sinus thrombosis (CVT) patients with new-onset headache and to identify the risk factors for headache in this population. METHODS: We retrospectively reviewed the demographic and clinical data of 69 CVT patients recruited between September 2017 and September 2019. Patients were classified into two groups, the headache group and the non-headache group, according to the presence or absence of new-onset headache symptoms at admission. The following characteristics and parameters were measured and analyzed, including gender, age, amount of thromboembolic cerebral venous sinus(ATCVS), and other relevant indicators. RESULTS: The incidence of headache was 75% in this cohort. The proportion of female patients in the headache group was higher than that in the non-headache group. Patients in the headache group were younger than those without headache. CVT patients of headache group showed higher lymphocyte ratio (LR), blood urea nitrogen (BUN), and intracranial pressure (ICP) compared to the non-headache group, whereas mean corpuscular volume (MCV) and levels of protein (cerebrospinal fluid, CSF) and lactic dehydrogenase (LDH) in CSF were lower in headache patients. The data also revealed younger age and the increased level of chloride ion CI-(CSF) were the risk factors for the occurrence of headache in CVT patients. CONCLUSION: Age, LR, MCV, BUN levels, ICP, protein (CSF), and LDH (CSF) in patients with headache were significantly different from those in the non-headache group at admission. Younger age and a level of CI- (CSF) were risk factors for headache in CVT patients. These findings may provide guidance for clinical diagnosis and treatment of CVT.

Cerebral venous thrombosis with hyperhomocysteinemia due to loss of heterozygosity at methylenetetrahydrofolate reductase (MTHFR) locus: a case report.

BACKGROUND: Loss of heterozygosity (LOH) at methylenetetrahydrofolate reductase (MTHFR) locus has been reported in tumor tissue. But the mutation was never reported in cerebral venous thrombosis (CVT) with hyperhomocysteinemia (HHcy) before. CASE PRESENTATION: A 14-year-old girl was admitted with an intermittent headache and nausea for 2 months. The plasma homocysteine level was 77.2 µmol/L. Lumbar puncture revealed an intracranial pressure > 330 mmH2O. Cerebral MRI and MRV revealed superior sagittal sinus thrombosis. Whole-exome sequencing revealed LOH at Chr1:11836597-11,867,232 affects exons 10-21 of C1orf167, the entire MTHFR, and exons 1-2 of the CLCN6 gene. The normal allele was the c.665 C > T/677 C > T variant in MTHFR. The patient was treated with nadroparin for 2 weeks, followed by oral rivaroxaban. Supplemental folate and vitamins B12 and B6 were prescribed. One month later, she had no headache and the intracranial pressure had decreased to 215 mmH2O. MRI showed shrinkage of the thrombosis in the superior sagittal sinus, the degree of stenosis had significantly decreased. CONCLUSIONS: Rare LOH at the MTHFR locus should be analyzed in CVT with HHcy. With anticoagulation treatment, the prognosis was good.

Cerebral venous thrombosis (CVT) complicating tubercular meningitis.

BACKGROUND: Tuberculous meningitis (TBM), complicated with cerebral venous thrombosis (CVT), has been sparsely reported and needs to be investigated further. METHODS: Among those with tuberculous meningitis in Haihe Hospital, Tianjin University, 3 patients with venous sinus thrombosis were identified retrospectively. "Tuberculous meningitis" and "cerebral venous thrombosis" were used as keywords, and the retrieved literature was summarized and analyzed. Our data were combined with previously reported case data to describe this new condition. RESULTS: Among 28 patients with a median onset age of 31 years for TBM, 17 were females. The manifestations were fever, headache, and seizure. Magnetic resonance imaging (MRI) venography showed that the most common site of venous sinus thrombosis involved superior sagittal sinus, left transverse sinus, left sigmoid sinus, cavernous sinus, and straight sinus. The abnormalities found on MRI include hydrocephalus, exudates, hemorrhage, meningeal enhancement, infarction, and tuberculoma. In the acute phase, all patients received standard anti-TB treatment, and 14/28 patients received anticoagulant treatment. The mortality rate of these patients was 17.9%, and 21/28 (75%) became functionally independent. CONCLUSIONS: CVT is one of the rare complications of TMB and must be considered a differential diagnosis in patients with TBM who show poor clinical features and/or develop new neurological signs.

Risk factors for the development of secondary intracranial hypertension in acute cerebral venous thrombosis.

PURPOSE: Intracranial hypertension (IH) can complicate cerebral venous thrombosis (CVT), potentially causing permanent visual loss. Current knowledge on risk factors for the development of IH following CVT is scarce. We applied a compound classifier (CSF opening pressure > 25 cmH2O, papilledema, or optic disc protrusion on MRI) as a surrogate for IH and studied the predictive value of thrombus location, the number of thrombosed segments, and thrombus volume. METHODS: We prospectively included 26 patients with acute CVT and complete MRI data. IH was defined by CSF opening pressure > 25 cmH2O, papilledema, or optic disc protrusion on MRI. Using high-resolution contrast-enhanced venography, we determined the thrombus location, number of thrombosed segments, and thrombus volume. We analyzed their association with IH by logistic regression, their predictive power by the area under the receiver operating characteristic curve, and their association with CSF opening pressure by linear regression. RESULTS: IH occurred in 46% of CVT patients and was associated with higher thrombus volume (AUC 0.759, p = 0.025) and superior sagittal sinus thrombosis both alone (OR 2.086, p = 0.049) and combined with transverse sinus thrombosis (OR 2.014, p = 0.028). Effects in patients presenting CSF opening pressure > 25 cm H2O and the compound classifier were consistent. Thrombus volume > 4 ml was the single most important predictor of higher CSF opening pressure (ß = 0.566, p = 0.035), increasing IH risk. CONCLUSION: Larger thrombus volume, dominant transverse sinus occlusion, and extensive superior sagittal combined with transverse sinus thrombosis were associated with IH. Thrombus volumetry might identify patients at risk for IH and direct further clinical evaluation.

Deep cerebral venous system involvement in patients with cerebral sinus thrombosis. A proposal of neuroradiological score systems useful for clinical assessment.

PURPOSE: To develop a neuroradiological score in patients with deep cerebral venous thrombosis (DCVT), capable of assessing extension of intracranial changes and venous occlusion at diagnosis; to assess the relationship between neuroradiological and clinical features at follow-up. MATERIAL AND METHODS: In 14 patients with DCVT, we developed 2 score systems on non-enhanced and contrast-enhanced CT: Intracranial Imaging Score (IIS) and Venous Occlusion Imaging Score (VOIS). ISS considers parenchymal venous strokes, hemorrhage, mass effect, and hydrocephalus; VOIS evaluates unilateral or bilateral venous occlusion extension. Modified Rankin Scale (mRS) and vessel recanalization status were assessed at follow-up. RESULTS: At diagnosis, higher IIS was related to bilateral venous thrombosis involvement (p 0,02; r:0,60), but parenchymal strokes were not related to venous occlusion extension (unilateral or bilateral) (p > 0,05). Moreover, the symptoms' onset time did not correlate with the severity scores (p > 0,05). At follow-up, 8 out of 14 patients showed good clinical outcomes with complete recanalization and neurological improvement, 1 patient showed a poor neurological outcome, whereas 5 patients died within 1 week. Positive correlations were found between IIS and mRS (p 0,003, r = 0,73), between IIS and vessels' recanalization status (p 0,002, r = 0,75), and between vessels' recanalization status and mRS (p < 0,001, r = 0,98). CONCLUSION: Neuroradiological scores may enhance diagnostic accuracy, and they may have a predictive significance. In patients with DCVT, although intracranial involvement was not influenced by symptoms' onset time or extension of venous occlusion, clinical outcome was related to both intracranial involvement and venous recanalization state. Collateral venous drainage status may counterbalance the thrombotic process improving prognosis.

Fulminant Extra- And Intracranial Arteriovenous Thrombosis And Non-aneurysmal Subarachnoid Hemorrhage Following COVID-19 Infection.

PURPOSE: Coronavirus disease of 2019 (COVID-19) is associated with increased risk of stroke and intracranial hemorrhage. This first report of fulminant panvascular arteriovenous thrombosis with subarachnoid hemorrhage (SAH) in a post-COVID-19 infection is attributed to extensive arteriovenous inflammation leading to arterial rupture following vasculitis. CASE REPORT: We report a rare case of extensive extra- and intra-cranial cerebral arteriovenous thrombosis following COVID-19 infection, presenting as fatal non-aneurysmal subarachnoid hemorrhage. The clinical course, biochemical and radiological evaluation is discussed. The other possible etiological differentials which were analysed and ruled out during case management are also detailed. CONCLUSION: A high degree of suspicion for COVID-19 induced coagulopathy leading to extensive non- aneurysmal, non-hemispheric SAH and malignant intracranial hypertension should be entertained. Our experience and previous reports on non-aneurysmal SAH in such patients show a poor prognosis.

Cerebral Venous Thrombosis in Patients With Heparin-Induced Thrombocytopenia a Systematic Review.

BACKGROUND: Cerebral venous thrombosis (CVT) has recently been reported as a common thrombotic manifestation in association with vaccine-induced thrombotic thrombocytopenia, a syndrome that mimics heparin-induced thrombocytopenia (HIT) and occurs after vaccination with adenovirus-based SARS-CoV-2 vaccines. We aimed to systematically review the incidence, clinical features, and prognosis of CVT occurring in patients with HIT. METHODS: The study protocol was registered with PROSPERO (CRD42021249652). MEDLINE, EMBASE and Cochrane CENTRAL were searched up to June 1, 2021 for HIT case series including >20 patients, or any report of HIT-related CVT. Demographic, neuroradiological, clinical, and mortality data were retrieved. Meta-analysis of proportions with random-effect modeling was used to derive rate of CVT in HIT and in-hospital mortality. Pooled estimates were compared with those for CVT without HIT and HIT without CVT, to determine differences in mortality. RESULTS: From 19073 results, we selected 23 case series of HIT (n=1220) and 27 cases of HIT-related CVT (n=27, 71% female). CVT developed in 1.6% of 1220 patients with HIT (95% CI,1.0%-2.5%, I2=0%). Hemorrhagic brain lesions occurred in 81.8% of cases of HIT-related CVT and other concomitant thrombosis affecting other vascular territory was reported in 47.8% of cases. In-hospital mortality was 33.3%. HIT-related CVT carried a 29% absolute increase in mortality rate compared with historical CVT controls (33.3% versus 4.3%, P<0.001) and a 17.4% excess mortality compared with HIT without CVT (33.3% versus 15.9%, P=0.046). CONCLUSIONS: CVT is a rare thrombotic manifestation in patients with HIT. HIT-related CVT has higher rates of intracerebral hemorrhage and a higher mortality risk, when compared with CVT in historical controls. The recently reported high frequency of CVT in patients with vaccine-induced thrombotic thrombocytopenia was not observed in HIT, suggesting that additional pathophysiological mechanisms besides anti-platelet factor-4 antibodies might be involved in vaccine-induced thrombotic thrombocytopenia-related CVT.

Headache at the chronic stage of cerebral venous thrombosis.

BACKGROUND: Headache is the most frequent symptom of cerebral venous thrombosis (CVT) but there is limited information about the frequency and phenotype of headache, weeks to months after cerebral venous thrombosis (post-cerebral venous thrombosis headache, PCH). OBJECTIVE: To assess the frequency, characteristics and predictors of PCH. METHODS: In this cross-sectional study, the frequency and characteristics of PCH were assessed in cerebral venous thrombosis survivors. Patients were interviewed between six months and five years after the cerebral venous thrombosis diagnosis. Clinical and imaging characteristics at the time of cerebral venous thrombosis diagnosis, as well as history of headache prior to cerebral venous thrombosis were compared in subjects with (GroupPCH) and without PCH (Groupcontrol). RESULTS: Subjects (n = 100; 82% women) were assessed, on average, at 1.1 ± 1.6 years of follow-up. PCH was present in 59% of the patients, phenotypes of tension-type-like headache were present in 31/59 (52.6%) and of migraine-like headache in 16/59 (27.1%). History of primary headache prior to cerebral venous thrombosis was significantly more common (OR: 6.4; 95% CI: 1.7-36.3) in GroupPCH (33.9%) than in Groupcontrol (7.3%). CONCLUSION: PCH was present in more than half of the patients. History of prior headache may be a risk factor for PCH. Prospective studies are required to confirm these findings and determine mechanisms, as well as interventions for prevention and treatment of PCH.

Dural arteriovenous fistulas in cerebral venous thrombosis: Data from the International Cerebral Venous Thrombosis Consortium: Data from the International Cerebral Venous Thrombosis Consortium.

BACKGROUND AND PURPOSE: To explore the prevalence, risk factors, time correlation, characteristics and clinical outcome of dural arteriovenous fistulas (dAVFs) in a cerebral venous thrombosis (CVT) population. METHODS: We included patients from the International CVT Consortium registries. Diagnosis of dAVF was confirmed centrally. We assessed the prevalence and risk factors for dAVF among consecutive CVT patients and investigated its impact on clinical outcome using logistic regression analysis. We defined poor outcome as modified Rankin Scale score 3-6 at last follow-up. RESULTS: dAVF was confirmed in 29/1218 (2.4%) consecutive CVT patients. The median (interquartile range [IQR]) follow-up time was 8 (5-23) months. Patients with dAVF were older (median [IQR] 53 [44-61] vs. 41 [29-53] years; p < 0.001), more frequently male (69% vs. 33%; p < 0.001), more often had chronic clinical CVT onset (>30 days: 39% vs. 7%; p < 0.001) and sigmoid sinus thrombosis (86% vs. 51%; p < 0.001), and less frequently had parenchymal lesions (31% vs. 55%; p = 0.013) at baseline imaging. Clinical outcome at last follow-up did not differ between patients with and without dAVF. Additionally, five patients were confirmed with dAVF from non-consecutive CVT cohorts. Among all patients with CVT and dAVF, 17/34 (50%) had multiple fistulas and 23/34 (68%) had cortical venous drainage. Of 34 patients with dAVF with 36 separate CVT events, 3/36 fistulas (8%) were diagnosed prior to, 20/36 (56%) simultaneously and 13/36 after (36%, median 115 [IQR 38-337] days) diagnosis of CVT. CONCLUSIONS: Dural arteriovenous fistulas occur in at least 2% of CVT patients and are associated with chronic CVT onset, older age and male sex. Most CVT-related dAVFs are detected simultaneously or subsequently to diagnosis of CVT.

Extensive Cerebral Venous Thrombosis Secondary to Recreational Nitrous Oxide Abuse.

Nitrous oxide, colloquially known as "whippets," is a commonly abused inhalant by adolescents and young adults. There are limited data describing the adverse effects of this abuse. We present a 16-year-old girl with no medical history who presented to the emergency department for confusion, hallucinations, weakness, and headaches. Imaging revealed extensive cerebral thrombosis. She had no prior history of venous or arterial thrombosis. Hypercoagulability workup demonstrated an elevated homocysteine level. She was treated with effective anticoagulation and vitamin B12 folate supplementation. To our knowledge, there are a very few cases in the medical literature of cerebral venous thrombosis following the use of nitrous oxide. The pathophysiology of the disorder appears to be linked to the metabolism of vitamin B12 inducing hyperhomocysteinemia and a procoagulant state.

A clinico-radiological study of deep cerebral venous thrombosis.

PURPOSE: Isolated deep cerebral venous thrombosis (DCVT) may have different presentation and outcome compared to DCVT with additional sinus thrombosis. We compare clinico-radiological findings and outcomes of patients with isolated DCVT with those having additional sinus thrombosis. METHODS: Forty-one DCVT patients with or without additional sinus thrombosis were included. Deep CVT was diagnosed if there was thrombosis of straight sinus, vein of Galen, internal cerebral vein, or basal vein of Rosenthal on MR venography (MRV). Isolated DCVT patients were classified as Group A and those with additional sinus thrombosis as Group B. The clinical features, risk factors, MRI findings, and outcomes at 1, 3, and 6 months were compared between Groups A and B. RESULTS: Median age was 28 years, and 22 (54%) were females. Eight (19.5%) patients were in Group A and 33 (80.5%) in Group B. Group B patients had shorter duration of illness (7 vs 30 days; p = 0.01), frequent vomiting [25 (75.7%) vs 2 (25%); p = 0.01], and papilledema [13 (39%) vs 0 (0%); p = 0.04]. Risk factors were comparable. MRI revealed bilateral thalamic [5 (62.5%) vs 6 (18.2%)] and basal ganglia [(4 (50%) vs 6 (18.2%)] lesions more frequently in Group A. At 1 month, 2 (6%) patients died in Group B and none in Group A, and 24 had good outcome (50% Group A and 60.6% in Group B). At 3 months, 30 had good outcome (62.5% Group A and 80.6% in Group B). Improvement after 3 months was negligible. CONCLUSION: Isolated DCVT is rare, and additional sinus thrombosis is associated with more severe illness and death. However, long-term outcomes in the survivors are similar between the two groups.

Cerebral venous sinus thrombosis after adenovirus-vectored COVID-19 vaccination: review of the neurological-neuroradiological procedure.

Cerebral venous and sinus thrombosis (CVST) after adenovirus-vectored COVID-19 ChAdOx1 nCov-19 (Oxford-AstraZeneca) and Ad26.COV2.S (Janssen/Johnson & Johnson) is a rare complication, occurring mainly in individuals under 60 years of age and more frequently in women. It manifests 4-24 days after vaccination. In most cases, antibodies against platelet factor-4/polyanion complexes play a pathogenic role, leading to thrombosis with thrombocytopenia syndrome (TTS) and sometimes a severe clinical or even fatal course. The leading symptom is headache, which usually increases in intensity over a few days. Seizures, visual disturbances, focal neurological symptoms, and signs of increased intracranial pressure are also possible. These symptoms may be combined with clinical signs of disseminated intravascular coagulation such as petechiae or gastrointestinal bleeding. If TTS-CVST is suspected, checking D-dimers, platelet count, and screening for heparin-induced thrombocytopenia (HIT-2) are diagnostically and therapeutically guiding. The imaging method of choice for diagnosis or exclusion of CVST is magnetic resonance imaging (MRI) combined with contrast-enhanced venous MR angiography (MRA). On T2*-weighted or susceptibility weighted MR sequences, the thrombus causes susceptibility artefacts (blooming), that allow for the detection even of isolated cortical vein thromboses. The diagnosis of TTS-CVST can usually be made reliably in synopsis with the clinical and laboratory findings. A close collaboration between neurologists and neuroradiologists is mandatory. TTS-CVST requires specific regimens of anticoagulation and immunomodulation therapy if thrombocytopenia and/or pathogenic antibodies to PF4/polyanion complexes are present. In this review article, the diagnostic and therapeutic steps in cases of suspected TTS associated CSVT are presented.