Meningitis and intracranial abscess due to Mycoplasma pneumoniae in a B cell-depleted patient with multiple sclerosis.

Mycoplasma pneumoniae, a frequent respiratory pathogen, can cause neurological disease manifestations. We here present a case of M. pneumoniae as cause of meningitis and occurrence of an intracranial abscess as a complication of mastoiditis with septic cerebral venous sinus thrombosis in a patient with multiple sclerosis on anti-CD20 therapy.

Cerebral venous sinus thrombosis associated with JAK2 V617F mutation-related pre-primary myelofibrosis: a case report and literature review.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a rare but potentially life-threatening subtype of stroke. Prompt and appropriate anticoagulation is crucial for improving the prognosis of CVST and preventing its recurrence. Identifying the underlying cause of CVST is decisive for guiding anticoagulant selection and determining treatment duration. CASE PRESENTATION: A 50-year-old man presented with a 35-day history of headache, nausea, vomiting, and blurred vision. Digital subtraction angiography performed at another facility revealed CVST. A contrast-enhanced black-blood MRI at our center confirmed the diagnosis, which was supported by a high intracranial pressure of 330mmH2O. Laboratory tests showed elevated leukocytes and platelet counts, raising suspicion of an underlying myeloproliferative neoplasms (MPNs). A bone marrow biopsy demonstrated increased megakaryocytes and granulocytes, and genetic testing identified the presence of the Janus kinase 2 V617F (JAK2 V617F) mutation, leading to a diagnosis of pre-primary myelofibrosis (pre-PMF). During hospitalization, anticoagulation with nadroparin calcium and fibrinolytic therapy were initiated. Upon discharge, rivaroxaban and aspirin were prescribed to prevent CVST recurrence and arterial thrombosis. CONCLUSION: This case highlights the importance of recognizing dynamic changes in routine blood tests that may link CVST to underlying hematological disorders. The JAK2 mutation is not only associated with MPNs but also increases the risk of thrombosis, including CVST. Further investigation is warranted to better understand the mechanisms by which JAK2 mutations contribute to thrombosis and to explore the potential benefits of JAK2 inhibitors in reducing this risk.

Pregnancy-related intracranial venous sinus thrombosis secondary to cryptococcal meningoencephalitis: a case report and literature review.

BACKGROUND: Cerebral venous sinus thrombosis (CVST), a serious cerebrovascular and neurological emergency, is common in pregnant individuals and accounts for approximately 0.5-1.0% of all cerebrovascular diseases. However, CVST with cryptococcal meningoencephalitis in immunocompetent pregnant patients is rare. CASE PRESENTATION: A 30-year-old woman who was 33 weeks pregnant presented with recurrent dizziness, headache, and vomiting as the main clinical manifestations, all of which were initially nonspecific. After assessment of the cerebrospinal fluid, skull computerized tomography, magnetic resonance imaging, and other laboratory and imaging examinations, the patient was diagnosed with secondary pregnancy-related CVST with cryptococcal meningoencephalitis. Despite receiving potent anticoagulant and antifungal treatment, the patient's condition deteriorated, and the patient's family opted to cease treatment. CONCLUSIONS: We present a rare case of CVST with cryptococcal meningoencephalitis in an immunocompetent pregnant patient. The difficulty of diagnosing and treating secondary pregnancy-related CVST caused by cryptococcal meningoencephalitis, as well as the great challenges faced at present are highlighted. One crucial lesson from the present case is that when clinical and imaging signs are unusual for CVST during pregnancy, it is essential to account for the possibility of other central nervous system (CNS) diseases, such as CNS infections with Cryptococcus, which may cause CVST.

Cerebral venous sinus thrombosis and SCN1A, a novel association?

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is a rare cause of stroke. Acquired and inherited prothrombotic conditions are the most common risk factors for CVST. Sometimes, an etiology is not found. Wide utilization of next generation sequencing technologies in clinical practice may lead to identification of risk factors other than those classically associated with CVST. METHOD AND RESULTS: This retrospective clinical-laboratory observational study has a reference patient who presented with CVST as an adolescent. Work up for prothrombotic conditions showed high homocysteine level secondary to homozygosity for a common polymorphism, c.677 C > T in the methylenetetrahydrofolate reductase (MTHFR) gene. His older unaffected brother has a similar MTHFR genotype and high homocysteine. The whole exome sequencing revealed a likely pathogenic variant in the sodium voltage gated channel, alpha subunit 1(SCN1A) gene. CONCLUSION: CVST is a multifactorial disease. Prothrombotic conditions are the most common risk factors for CVST. High homocysteine due to the common MTHFR polymorphisms was previously attributed to various thrombotic conditions including CVST. Although high homocysteine due to MTHFR polymorphism may be a contributing factor, additional risk factors such as blood flow abnormalities during SCN1A related seizures may be needed for thrombosis.

Elevated factor XI is associated with increased risk of recurrent cerebral venous sinus thrombosis: a cohort study.

Cerebral venous sinus thrombosis (CVST) has no identified cause in 15% of cases. Elevated factors (F) VIII and FXI have been associated with thromboembolism, but data on CVST are limited. We hypothesized that elevated plasma FVIII and FXI predispose to first and recurrent CVST. In 50 CVST survivors aged < 60 years, following anticoagulant cessation and in 50 controls, we determined plasma FVIII and FXI, along with fibrin clot properties: lysis time, permeability, maximum D-dimer (D-Dmax), and maximum rate of D-dimer increase (D-Drate). We recorded CVST recurrence during a follow-up of 58.5 (55.0-60.0) months. Plasma FVIII was 22.7% higher in CVST than in controls, with elevated FVIII > 150% in 13 (26%) vs. 4 (8%) patients, respectively (p = 0.02). Median FXI tended to be higher in CVST vs. controls (110.5 [99.0-117-0]% vs. 104.5 [97.0-116.0]%, p = 0.07), while FXI > 120% was observed more commonly in the former group (12 [24%] vs. 4 [8%], respectively, p = 0.03). Patients with FVIII > 150% were less likely to achieve complete recanalization compared with the remainder (2 [15.4%] vs. 28 [75.7%], respectively; p < 0.001). Eight patients (16%) experienced CVST recurrence. They had higher baseline FXI, but not FVIII, as compared with the remainder (125.5 [114.5-140.0]% vs. 107.5 [102.0-117.0]%, respectively, p = 0.01). Patients with FXI > 120% were four times more likely to have recurrent CVST (5 [62.5%] vs. 7 [16.7%], respectively; p = 0.01). Plasma FXI > 120% could represent a novel risk factor for first and recurrent CVST. Given advances in anti-FXI agents, CVST might be another indication for this emerging treatment.

Pediatric frequent relapsing nephrotic syndrome with multiple cerebral infarctions accompanied by patent foramen ovale and cerebral venous sinus thrombosis: a case report.

BACKGROUND: Idiopathic nephrotic syndrome (NS) presents as a hypercoagulable state, of which thromboembolism (TE) is a well-known life-threatening complication. Although TE is more likely to occur in venous vessels than arterial vessels, arterial TE is important because it may cause after-effects, including tissue necrosis and cerebral infarction (CI); therefore, prompt diagnosis and appropriate treatment are required. We report a pediatric NS case with multiple CIs. CASE PRESENTATION: A 14-year-7-month-old Japanese girl was diagnosed with frequent relapsing NS, accompanied by headache and disturbance of consciousness during the second relapse. Brain magnetic resonance imaging (MRI) and four-dimensional computed tomography revealed multiple CIs, vasogenic edema, and cerebral venous sinus thrombosis (CVST). The patient had no underlying thrombophilia other than hypercoagulability due to NS and prednisolone (PSL), and no cardiac arrhythmia; however, a right-to-left shunt through the patent foramen ovale (PFO) was observed with the Valsalva maneuver by echocardiography. Therefore, we assumed that a potential cause of multiple CIs might be an embolic stroke, caused by thrombosis formed from a hypercoagulable state due to NS and PSL treatment and reached through PFO. Antiplatelet and anticoagulant therapies were administered for TE. She was treated with PSL and mycophenolate mofetil (MMF) for NS. Rituximab (RTX) was administered to prevent NS relapse after complete remission (CR). She underwent transcatheter PFO closure at age 14 years and 9 months because we considered that the right-to-left shunt through the PFO would be one of the risks for recurrent cerebral embolism when NS relapses. One year after the onset of CIs, an MRI indicated that the CVST had resolved, leaving no neurological sequelae due to CI; therefore, anticoagulant therapy was discontinued. And then she has been in CR for NS with only MMF therapy. CONCLUSIONS: CI is a serious complication in patients with NS. The pathogenesis of multiple CIs is various, including right-to-left shunt through PFO, in addition to the hypercoagulability due to NS. It is important to investigate and manage underlying risks such as PFO, besides preventing the relapses of NS by aggressive treatments using MMF and RTX, in patients with NS.

Dural arteriovenous fistula in the setting of cerebral venous sinus thrombosis and COVID-19 infection.

OBJECTIVE: The aim of this study was to examine the presence of concurrent venous thrombosis and COVID-19 infections in patients with dural arteriovenous fistulas (dAVFs). METHODS: An analysis of all patients diagnosed with dAVF via cerebral angiography by the senior author was conducted, with special attention given to the presence of cerebral venous sinus thrombosis (CVST) and COVID-19 infection. General demographics, clinical presentation, presence of CVST, and COVID-19 infection status were reported. RESULTS: A total of 30 patients with dAVFs were included in this study. Three patients were diagnosed with COVID-19 (10%), with one of these patients developing CVST (33%) at 6 months postinfection. Of the 27 patients not infected with COVID-19, one was diagnosed with a likely chronic CVST at the time of presentation of dAVF (4%). A total of 11 case reports and 3 retrospective studies describing patients diagnosed with CVST at or after diagnosis of dAVFs have been reported in the literature. The incidence of dAVFs in patients with CVST has been reported as 2.4%, and the incidence of dAVF has reportedly increased five- to tenfold since the COVID-19 pandemic. CONCLUSIONS: COVID-19 infections may pose as an emerging risk factor for the development of CVST and subsequent dAVF development. To the authors' knowledge, this study presents the first cases in the literature describing a temporal relationship between COVID-19 and development of a dAVF with CVST. The effect of both COVID-19 and associated vaccines should be further assessed in future studies to examine its impact as an effect modifier on the association of dAVF and CVST.

Anticoagulation strategies in patients with coexisting traumatic intracranial hematomas and cerebral venous sinus thrombosis: an observational cohort study.

PURPOSE: Post-traumatic cerebral venous sinus thrombosis (ptCVT) is a rare but serious complication of traumatic brain injury (TBI). Managing ptCVT is challenging due to the concurrent risk of traumatic intracranial hematoma (ICH) expansion. Limited data exists on the safety and efficacy of anticoagulation therapy (ACT) in these cases. METHODS: This single-center observational cohort study included adult TBI patients with concurrent ICH and ptCVT. Low-molecular-weight heparin (LMWH) or heparin infusion was used to treat all ptCVTs based on institutional protocols. The outcomes of interest were hemorrhagic and thrombotic complications. RESULTS: Out of 1,039 TBI-patients admitted between 2006 and 2020, 32 met the inclusion criteria. The median time from injury to ptCVT diagnosis was 24 h. ACT was initiated at a median of 9 h after ptCVT diagnosis. Patients were administered either heparin infusion (n = 8) or LMWH at dosages ranging from 28 to 72% of the therapeutic level (n = 24). There were no hemorrhagic complications, even in patients receiving LMWH at ≥ 50% of the therapeutic dose. Thrombotic complications occurred in 3 patients (9.4%) - two cases of thrombus progression and one venous infarct. The patients who developed thrombotic complications differed from those who did not by having a 17-h delay in ACT initiation after diagnosis or by receiving an initial LMWH dose at 28% of the therapeutic level. CONCLUSION: LMWH at approximately 50% of the therapeutic level was effective for managing ptCVT associated with TBI in our retrospective dataset, with no risk of hematoma expansion. Prospective trials are warranted to confirm these results.

Outcomes After Decompressive Surgery for Severe Cerebral Venous Sinus Thrombosis Associated or Not Associated with Vaccine-Induced Immune Thrombosis with Thrombocytopenia: A Multicenter Cohort Study.

BACKGROUND: Clinical observations indicated that vaccine-induced immune thrombosis with thrombocytopenia (VITT)-associated cerebral venous sinus thrombosis (CVST) often has a space-occupying effect and thus necessitates decompressive surgery (DS). While comparing with non-VITT CVST, this study explored whether VITT-associated CVST exhibits a more fulminant clinical course, different perioperative and intensive care unit management, and worse long-term outcome. METHODS: This multicenter, retrospective cohort study collected patient data from 12 tertiary centers to address priorly formulated hypotheses concerning the clinical course, the perioperative management with related complications, extracerebral complications, and the functional outcome (modified Rankin Scale) in patients with VITT-associated and non-VITT CVST, both with DS. RESULTS: Both groups, each with 16 patients, were balanced regarding demographics, kind of clinical symptoms, and radiological findings at hospital admission. Severity of neurological symptoms, assessed with the National Institute of Health Stroke Scale, was similar between groups at admission and before surgery, whereas more patients with VITT-associated CVST showed a relevant midline shift (≥ 4 mm) before surgery (100% vs. 68.8%, p = 0.043). Patients with VITT-associated CVST tended to undergo DS early, i.e., ≤ 24 h after hospital admission (p = 0.077). Patients with VITT-associated CVST more frequently received platelet transfusion, tranexamic acid, and fibrinogen perioperatively. The postoperative management was comparable, and complications were evenly distributed. More patients with VITT-associated CVST achieved a favorable outcome (modified Rankin Scale ≤ 3) at 3 months (p = 0.043). CONCLUSIONS: Although the prediction of individual courses remains challenging, DS should be considered early in VITT-associated CVST because an overall favorable outcome appears achievable in these patients.

Superior Sagittal Sinus Thrombectomy in Pediatric Head Injury.

INTRODUCTION: Injury and subsequent thrombosis of the cerebral venous sinuses may be caused by closed head injuries secondary to a variety of different mechanisms. Skull fractures can lacerate or otherwise disrupt adjacent dural sinuses. The sequelae of such injuries may include thrombosis and either partial or total occlusion of the sinus, ultimately resulting in significant venous congestion. Sagittal sinus injury is associated with a more serious outcome due to the obligatory flow into the sinus, especially posterior to the coronal suture. In such cases, venous infarction may be a severe and life-threatening complication of head injury. CASE PRESENTATION: A 2-year-old female presented with a depressed skull fracture near the midline and a thrombus in the sagittal sinus. Anticoagulation, the standard treatment cerebral venous sinus thrombosis (CVST), was contraindicated due to intracranial hemorrhage, so immediate thrombectomy was performed with successful neurologic recovery at 9-month follow-up. To our knowledge, this case is the youngest patient documented to receive mechanical thrombectomy for superior sagittal sinus (SSS) thrombosis due to trauma. CONCLUSION: Closed head injuries in pediatric patients may be associated with CVST, with resulting venous drainage compromise and profound neurologic sequelae. Unlike adult patients with spontaneous CVST in which anticoagulation are the standard of care, pediatric patients experiencing traumatic CVST may have contraindications to anticoagulants. If the patient has a contraindication to anticoagulation such as intracranial bleeding, endovascular mechanical thrombectomy may be an effective intervention when performed by an experienced neurointerventionalist.

Draining Vein Thrombosis of Developmental Venous Anomaly in Sickle Cell Trait Patients: A Case Report and a Literature Review.

INTRODUCTION: Cerebral venous sinus thrombosis (CVST) is a rare but serious condition in both adults and children. Risk factors include thrombophilias, dehydration, and certain inherited conditions like sickle cell trait (SCT). We present a case of CVST in a pediatric patient with SCT to highlight key considerations in diagnosis and management. CASE PRESENTATION: A 14-year-old male with SCT presented with worsening headache and vomiting after prolonged sun exposure and dehydration during athletic camp. Imaging revealed right occipital hemorrhage, hydrocephalus, right CSVT, and bilateral cerebellar developmental venous anomalies. Hypercoagulability testing was normal. Diagnostic evaluation included computed tomography, magnetic resonance imaging, MR venography (MRV), and hypercoagulability testing. The patient was treated with an external ventricular drain, platelet transfusion, and anticoagulation. Management also involved hydration, platelet transfusion, supportive care, and multidisciplinary follow-up. Follow-up MRV showed recanalization. CONCLUSION: This case highlights SCT as a potential CVST risk factor. Timely recognition, evaluation of precipitants like dehydration, supportive care including anticoagulation, and multidisciplinary management are important. An individualized approach is needed to balance thrombosis recurrence and bleeding risks. Patients with SCT require education on risks and prompt evaluation of neurological symptoms to allow early diagnosis and care of CVST.

Prevalence and patterns of cerebral venous sinus thrombosis following vestibular schwannoma surgery: a systematic review and meta-analysis.

PURPOSE: Cerebral venous sinus thrombosis (CVST) is a potentially serious complication following surgical treatment of vestibular schwannoma, a benign tumor originating from Schwann cells of the vestibulocochlear nerve. This study aimed to determine the prevalence of CVST following surgical treatment of vestibular schwannoma and the factors contributing to its occurrence. METHOD: Two independent researchers searched the global databases of PubMed, Web of Science, Scopus, and the Cochrane Library up to September 01, 2023. We employed a random-effects model for data analysis. Heterogeneity was evaluated using the I2 test. To assess the quality of the studies meeting our inclusion criteria, we employed the Joanna Briggs Institute Critical Appraisal Checklist. RESULTS: We included 23 articles in this meta-analysis. The pooled prevalence of CVST after vestibular schwannoma surgery was 6.4% (95%CI 3.4-11.5%). The pooled prevalence of CVST following the retrosigmoid (RS), translabyrinthine (TL), and middle cranial fossa (MCF) approaches was 4.8% (95%CI 2.0-11.0%), 9.6% (95%CI 4.3-20.3%) and 9.9% (95%CI 1.6-42.2%), respectively, revealing a significant difference between the TL and the RS approaches (Odds ratio = 2.10, 95%CI 1.45-3.04, P < 0.001). The sigmoid sinus exhibited the highest post-operative thrombosis rate (7.9%), surpassing the transverse sinus (3.7%) and involvement of both sigmoid and transverse sinuses (1.6%), respectively. No significant associations were found with demographic or surgical factors. CONCLUSION: In the current meta-analysis, we identified a 6.4% CVST prevalence following vestibular schwannoma surgery, with varying rates depending on the surgical approach. No significant associations with patient or surgical factors were found, emphasizing the need for heightened clinical vigilance and further research in this context. TRAIL REGISTRATION: PROSPERO ID: CRD42023453513.

Imaging of cerebrovascular complications from blunt skull base trauma.

Cerebrovascular complications from blunt trauma to the skull base, though rare, can lead to potentially devastating outcomes, emphasizing the importance of timely diagnosis and management. Due to the insidious clinical presentation, subtle nature of imaging findings, and complex anatomy of the skull base, diagnosing cerebrovascular injuries and their complications poses considerable challenges. This article offers a comprehensive review of skull base anatomy and pathophysiology pertinent to recognizing cerebrovascular injuries and their complications, up-to-date screening criteria and imaging techniques for assessing these injuries, and a case-based review of the spectrum of cerebrovascular complications arising from skull base trauma. This review will enhance understanding of cerebrovascular injuries and their complications from blunt skull base trauma to facilitate diagnosis and timely treatment.

External validation of the SI2NCAL2C score for outcomes following cerebral venous thrombosis.

OBJECTIVES: Prognostication for cerebral venous thrombosis (CVT) remains difficult. We sought to validate the SI2NCAL2C score in an international cohort. MATERIALS AND METHODS: The SI2NCAL2C score was originally developed to predict poor outcome (modified Rankin Scale (mRS) 3-6) at 6 months, and mortality at 30 days and 1 year using data from the International CVT Consortium. The SI2NCAL2C score uses 9 variables: the absence of any female-sex-specific risk factors, intracerebral hemorrhage, central nervous system infection, focal neurological deficits, coma, age, lower level of hemoglobin, higher level of glucose, and cancer. The ACTION-CVT study was an international retrospective study that enrolled consecutive patients across 27 centers. The poor outcome score was validated using 90-day mRS due to lack of follow-up at the 6-month time-point in the ACTION-CVT cohort. Model performance was evaluated using the area under the receiver operating characteristic curve (AUC) and calibration plots. Missing data were imputed using the additive regression and predictive mean matching methods. Bootstrapping was performed with 1000 iterations. RESULTS: Mortality data were available for 950 patients and poor outcome data were available for 587 of 1,025 patients enrolled in ACTION-CVT. Compared to the International CVT Consortium, the ACTION-CVT cohort was older, less often female, and with milder clinical presentation. Mortality was 2.5% by 30 days and 6.0% by one year. At 90-days, 16.7% had a poor outcome. The SI2NCAL2C score had an AUC of 0.74 [95% CI 0.69-0.79] for 90-day poor outcome, 0.72 [0.60-0.82] for mortality by 30 days, and 0.82 [0.76-0.88] for mortality by one year. CONCLUSIONS: The SI2NCAL2C score had acceptable to good performance in an international external validation cohort. The SI2NCAL2C score warrants additional validation studies in diverse populations and clinical implementation studies.

Neurologic manifestations of Behçet disease: rheumatology experience.

OBJECTIVE: Neurologic involvement in Behçet disease (BD) is a rare manifestation. Herein, we aimed to evaluate the clinical features and treatment choices of neuro-Behçet (NB) patients. METHODS: There were records of 800 BD patients between 1998 and 2021. Fifty-five of the BD patients had NB and the files of these patients were retrospectively evaluated. Patients were grouped into three subgroups: 22 (40%) had non-parenchymal, 25 (45%) had parenchymal, and 8 (15%) had both parenchymal and non-parenchymal (mixed) involvement. RESULTS: Of the 55 patients, 32 were male. Twenty-six of the NB patients were diagnosed with BD simultaneously. The most common complaint was headache (n = 24, 44%). The most affected site was periventricular white matter (n = 21, 38%). All patients had received corticosteroids. Azathioprine (AZA; n = 39, 71%) was the most common immunosuppressive agent after corticosteroids, followed by cyclophosphamide (n = 16, 29%). CONCLUSION: Neurologic involvement is a rare complication of BD but is related to increased mortality and morbidity. Neurologic manifestations may be the initial symptom of BD, thus leading to diagnosis. Both neurology and rheumatology specialists should be aware of this rare condition.

Neurological autoimmune diseases following vaccinations against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2): A follow-up study.

BACKGROUND AND PURPOSE: Population-based studies suggest severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines may trigger neurological autoimmunity including immune-mediated thrombotic thrombocytopenia. Long-term characterization of cases is warranted to facilitate patient care and inform vaccine-hesitant individuals. METHODS: In this single-center prospective case study with a median follow-up of 387 days long-term clinical, laboratory and imaging characteristics of patients with neurological autoimmunity diagnosed in temporal association (≤6 weeks) with SARS-CoV-2 vaccinations are reported. RESULTS: Follow-up data were available for 20 cases (central nervous system demyelinating diseases n = 8, inflammatory peripheral neuropathies n = 4, vaccine-induced immune thrombotic thrombocytopenia n = 3, myositis n = 2, myasthenia n = 1, limbic encephalitis n = 1, giant cell arteritis n = 1). Following therapy, the overall disability level improved (median modified Rankin Scale at diagnosis 3 vs. 1 at follow-up). The condition of two patients worsened despite immunosuppressants possibly related to their autoimmune diagnoses (limbic encephalitis n = 1, giant cell arteritis n = 1). At 12 months' follow-up, 12 patients achieved complete clinical remissions with partial responses in five and stable disease in one case. Correspondingly, autoimmune antibodies were non-detectable or titers had significantly lowered in all, and repeat imaging revealed radiological responses in most cases. Under vigilant monitoring 15 patients from our cohort underwent additional SARS-CoV-2 vaccinations (BNT162b2 n = 12, mRNA-1273 n = 3). Most patients (n = 11) received different vaccines than prior to diagnosis of neurological autoimmunity. Except for one short-lasting relapse, which responded well to steroids, re-vaccinations were well tolerated. CONCLUSIONS: In this study long-term characteristics of neurological autoimmunity encountered after SARS-CoV-2 vaccinations are defined. Outcome was favorable in most cases. Re-vaccinations were well tolerated and should be considered on an individual risk/benefit analysis.

Association of neutrophil to lymphocyte ratio and D-dimer with functional outcome in patients with cerebral venous sinus thrombosis.

BACKGROUND: Investigations on the risk factors for the prognosis of cerebral venous sinus thrombosis (CVST) are limited. This study aimed to explore whether specific inflammatory factors and coagulation indictors are associated with functional outcome in patients treated for CVST. METHODS: This retrospective study included 137 patients admitted to our hospital between January 2010 and October 2021. The functional outcome was assessed with the modified Rankin Scale (mRS) score at discharge. Patients were divided into two groups, 102 patients with favorable outcomes (mRS 0-1) and 35 patients with poor outcomes (mRS 2-6). The clinical indexes were compared between two groups. Multivariable logistic regression was performed to identify the independent influencing factors for poor outcomes of CVST patients. The prognostic indicators were analyzed using the receiver operating characteristic (ROC) curve. RESULTS: Compared with the favorable outcome group, the incidence of impaired consciousness and brain lesion, the levels of D-dimer, RDW, neutrophil count, neutrophil to lymphocyte ratio (NLR) and red blood cell distribution width to platelet ratio (%) on admission were significantly higher in the poor outcome group, while the level of lymphocyte count was significantly lower. After multivariable logistic regression analysis, baseline D-dimer level (odds ratio (OR), 1.180; 95% confidence interval (CI), 1.019-1.366, P = 0.027) and NLR (OR, 1.903; 95%CI, 1.232-2.938, P = 0.004) were significantly associated with unfavorable outcome at discharge. The ROC curve analysis showed that the areas under the curve of D-dimer, NLR and their combined detection for predicting worse outcome were 0.719, 0.707 and 0.786, respectively. CONCLUSIONS: Elevated D-dimer level and NLR on admission were associated with an increased risk of poor functional outcome in patients with CVST.

Clinical characteristics and outcome of dural arteriovenous fistulas secondary to cerebral venous sinus thrombosis: a primary or secondary event?

BACKGROUND: The Dural Arteriovenous Fistulas (DAVFs) secondary to cerebral venous sinus thrombosis (CVST) are rather rare. The aim of present study is to investigate the clinical and radiological features, and treatment outcome of DAVFS in patients following CVST. METHODS: Data about demographic information, clinical presentations, radiological findings, as well as treatment and outcome of DAVFs sequence to CVST were collected to analysis from January 2013 to September 2020 in this retrospective study. RESULTS: Fifteen patients with DAVFs after CVST were included in the study. The median age was 41 years (range17-76 years). Ten patients (66.67%) were male and 6 patients (33.33%) were female. The median duration of presenting CVST was 182 days (Range 20-365). Mean time from diagnosis of CVST to confirmation of DAVFs was 97 days (range 36-370 days). The most common manifestations of DAVFs following CVST were headache and visual disturbance seen in 7 patients respectively. Five patients had pulsatile tinnitus (%) and 2 had nausea/vomiting. The DAVFs are most frequently located at the transverse/sigmoid sinus (7/15, 46.67%), followed by the superior sagittal the sinus and confluence sinus (6/15, 40.00%) respectively. Angiography of DAVFs revealed Board type I in seven (46.7%) patients, Board type II and III in 4(26.7%) patients, respectively. The Cognard I was noted in seven (46.7%), Cognard IIa and IV in 3 patients, IIb and III in one patient, respectively. The main feeding arteries of DAVFs most commonly originate from the branches of the external carotid artery in 6 (40.0%) patients. The other DAVFs are conjointly supplied by multiple feeders from internal and external carotid artery and vertebral arteries. Fourteen (93.33%) patients were treated with endovascular embolization and none of the patients had permanent deficits during follow-up. CONCLUSION: Intracranial DAVFs following CVST are rare presentations. Most patients have a good outcome after timely interventional therapy. Continued observation and follow-up of (DSA) are important to find DAVFs secondary to CVST.

Identification of LDLR mutation in cerebral venous sinus thrombosis co-existing with dural arteriovenous fistulas: a case report.

BACKGROUND: Cerebral venous sinus thrombosis (CVST) is typically associated with a prothrombotic state of the blood, with its causative factors varying widely. Prior research has not reported the simultaneous occurrence of CVST and dural arteriovenous fistulas (DAVFs) as potentially resulting from genetic mutations. In this case report, we introduce a unique occurrence wherein a patient with a heterozygous mutation of the low-density lipoprotein receptor (LDLR) gene presented with CVST in conjunction with DAVFs. CASE: Presentation: A male patient, aged 51, sought treatment at our facility due to a consistent decline in cognitive functions accompanied by recurrent headaches. Comprehensive evaluations were administered, including neurological examinations, laboratory tests, magnetic resonance imaging, digital subtraction angiography, and whole exome sequencing. Digital subtraction angiography identified DAVFs in the patient's right sigmoid sinus and an occlusion within the left transverse sinus. The whole exome sequencing of blood samples pinpointed a heterozygous mutation in the LDLR gene (NM_000527:exon12:c.C1747T:p.H583Y). Following the confirmed diagnosis of CVST and DAVFs, the patient underwent anticoagulant therapy combined with endovascular procedures - these comprised embolization of the arteriovenous fistula in the right sigmoid sinus and balloon dilation with stent implantation in the left transverse sinus. A six-month follow-up indicated a significant abatement in the patient's symptoms. CONCLUSIONS: This report marks the first documented case of an LDLR gene mutation that could be associated with the onset of CVST and DAVFs. The mutation in the LDLR gene might foster a prothrombotic environment, facilitating the gradual emergence of CVST and the subsequent genesis of DAVFs.

Survival of community-acquired Bacillus cereus sepsis with venous sinus thrombosis in an immunocompetent adult man - a case report and literature review.

BACKGROUND: Bacillus cereus infections in immunocompetent patients are uncommon and mainly observed in fragile patients. It can cause lethal infections with multiple organ dysfunction syndrome (MODS). However, a patient presenting as venous sinus thrombosis and survival without sequela has not been reported. CASE PRESENTATION: A 20-year-old previously healthy male developed gastroenteritis after a meal, followed by fever, convulsions, and severe disturbance of consciousness. The patient had significant leukocytosis with a mildly elevated D-dimer, creatinine level, and respiratory failure. The CT(computed tomography) revealed fatal brain edema and subarachnoid hemorrhage. Previous blood culture in a local hospital revealed B. cereus, which was confirmed by mNGS(metagenomic next-generation sequencing) using blood and urine in our hospital. Accordingly, B. cereus sepsis with MODS were considered. Later, cerebral venous sinus thrombosis was proved. After anti-infection (linezolid 0.6 g, Q12h; and meropenem 1.0 g, Q8h), anti-coagulant (enoxaparin 6000U, Q12h), and other symptomatic treatments, the patient recovered completely without sequela at the 6-month follow-up. CONCLUSIONS: This case suggests that in immunocompetent adults, there is still a risk of infection with B. cereus, causing severe MODS. Special attention should be paid to venous sinus thrombosis and subarachnoid hemorrhage in such cases, while, anti-coagulant is essential therapy.