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The report explores a case of cerebral sinus venous thrombosis associated with hypereosinophilia, presenting a unique clinical scenario. A 22-year-old male presented with persistent headache for eight days, escalating in intensity, along with projectile vomiting and blurred vision. Despite the absence of typical indicators such as fever or respiratory symptoms, comprehensive evaluations revealed hypereosinophilia in the complete blood count. Imaging studies, including magnetic resonance angiography and venography, confirmed cerebral sinus venous thrombosis. The patient was successfully treated with a multidimensional approach, including anticoagulation therapy, corticosteroids, and supportive measures. This report highlights the concealed nature of hypereosinophilia in the context of cerebral sinus venous thrombosis and underscores the importance of a vigilant diagnostic approach in unravelling this silent association.

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Cerebral sinus venous thrombosis (CSVT) is a rare form of venous thromboembolism. Recurrent transient ischemic attacks were extremely rare clinical manifestation of CSVT. We reported a case of transient ischemic attack as an exceptionally rare form of CSVT. A 29-year-old woman presented with a headache and four episodes of a transient right limb weakness, numbness, and dysphasia lasting approximately 15 mins over the course of 2 weeks. Magnetic resonance venography revealed a superior sagittal sinus thrombosis with numerous prominent enlarged and dilated venous. Magnetic resonance imaging revealed venous infarction of the left frontal lobe. Transient ischemic attack-like episodes stopped with anticoagulation. We suggested that the patients with transient ischemic attack and symptoms of increased intracranial pressure should be carefully investigated in terms of CSVT.

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Acute lymphoblastic leukemia (ALL) is a hematological cancer that can cause ocular tissue involvement. Asparaginase is a chemotherapy regimen that is commonly used in leukemia which could lead to similar ocular manifestations. We report a patient with a history of ALL for seven months on asparaginase therapy and persistent cerebral sinus venous thrombosis (CSVT) with acute venous infarction in the left frontal lobe presented with worsening vision. On examination, he had a visual acuity (VA) of (6/21) in the right eye and (6/60) in the left eye, with a mild left eye abduction limitation. Fundal examination showed bilateral prominent multilayered retinal hemorrhages and papilledema with absence of leukemic infiltration. His chemotherapy regimen was held and a one month follow up was scheduled. Follow up after one month of chemotherapy cessation showed resolution of both VA and fundal exam findings. It is crucial to differentiate between asparaginase toxicity and infiltration of the disease in ALL patients. As this would determine whether the treatment should be continued or suspended.

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There was tremendous increase in the number of cases of mucormycosis among patients affected by coronavirus disease 2019 (COVID-19) during the second wave of pandemic in South Asian countries. This invasive fungal infection primarily affects paranasal sinuses and can have orbito-facial and intracranial extension. We are presenting the radiological findings of invasive mucormycosis with pathological and clinical outcome correlation. It is important for radiologists to have the knowledge of various presentations of this opportunistic infection for early diagnosis and helping clinicians in planning the appropriate line of management. The study also emphasizes on the correlation between the extent of involvement with clinical outcome and we proposed a magnetic resonance imaging (MRI) based scoring system to standardize and prognosticate the patients affected with mucormycosis. MATERIALS AND METHODS: We utilized GE 1.5 tesla, 16-channeled MRI machine for scanning the clinically suspected mucormycosis patients and did plain and contrast study of the paranasal sinuses, orbito-facial study and included brain as and when required. Images were acquired in axial, coronal, and sagittal planes using T1, T2, and fat-saturated short tau inversion recovery sequences (STIR), fat-saturated contrast sequences for better evaluation of the extent of the disease. Diffusion-weighted sequence was also acquired to detect ischemic changes in optic nerve or brain parenchyma. Contrast study was used to detect any major vessel occlusion or cavernous sinus thrombosis in the study population. RESULTS: Total number of cases (n) included in the study were 32. The mean age group was 41-50 years with the median age was 47 years. Out of 32 cases (n=32), in 16 cases (50%) the disease was limited only to the paranasal sinuses and in remaining 16 (50%) cases, disease has spread to other regions such as orbits, facial soft tissues, optic nerve, and brain parenchyma. All the 18 cases with Mild score (MRI ROCM score 1-3) survived and all those with severe score (2 cases) (MRI ROCM score 7-10) did not survive. CONCLUSION: During the second wave of COVID-19 pandemic, we observed a significant rise in acute invasive mucormycosis infection primarily involving the paranasal sinuses and spread to orbito-facial, cerebral parenchyma causing related complications and hence increased morbidity and death. Radiologically, using MRI, it was effectively possible to detect early extrasinonasal spread and other fatal complications thereby guiding the physicians and surgeons in the proper early aggressive management of the disease. Here, we have described the radiological characteristics of paranasal sinus mucormycosis and its spread to other regions. We also proposed an MRI-based Scoring System for standardized assessment of the disease severity. We observed in our study that the extent of disease on MRI is directly correlating with mortality.

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BACKGROUND: Cerebrovascular disorders are an important cause of morbidity and mortality in children. The acute care of a child with an ischemic or hemorrhagic stroke or cerebral sinus venous thrombosis focuses on stabilizing the patient, determining the cause of the insult, and preventing secondary injury. Here, we review the use of both invasive and noninvasive neuromonitoring modalities in the care of pediatric patients with arterial ischemic stroke, nontraumatic intracranial hemorrhage, and cerebral sinus venous thrombosis. METHODS: Narrative review of the literature on neuromonitoring in children with cerebrovascular disorders. RESULTS: Neuroimaging, near-infrared spectroscopy, transcranial Doppler ultrasonography, continuous and quantitative electroencephalography, invasive intracranial pressure monitoring, and multimodal neuromonitoring may augment the acute care of children with cerebrovascular disorders. Neuromonitoring can play an essential role in the early identification of evolving injury in the aftermath of arterial ischemic stroke, intracranial hemorrhage, or sinus venous thrombosis, including recurrent infarction or infarct expansion, new or recurrent hemorrhage, vasospasm and delayed cerebral ischemia, status epilepticus, and intracranial hypertension, among others, and this, is turn, can facilitate real-time adjustments to treatment plans. CONCLUSIONS: Our understanding of pediatric cerebrovascular disorders has increased dramatically over the past several years, in part due to advances in the neuromonitoring modalities that allow us to better understand these conditions. We are now poised, as a field, to take advantage of advances in neuromonitoring capabilities to determine how best to manage and treat acute cerebrovascular disorders in children.

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OBJECTIVES: Janus kinase 2 (JAK2-V617F) mutations can cause thrombocytosis, polycythemia and hyper viscosity leading to cerebral sinus venous thrombosis (CSVT). However, data regarding the characteristics and prevalence of JAK2-V617F mutation in patients with CSVT are currently lacking. We aimed to evaluate the characteristics of CSVT patients that carry the JAK2 mutation. MATERIALS AND METHODS: Data of consecutive patients with CSVT, admitted to three large academic medical centers between 2010 and 2020, were retrospectively studied. Demographics, clinical presentations, radiological and clinical outcome parameters were compared between carriers of the JAK2-V617F mutation and controls. RESULTS: Out of 404 patients diagnosed with CSVT, 26 patients (6.5%) were carriers of the mutation. JAK2 mutation carriers more often had thrombocytosis (54% vs. 1%, p < 0.001). Furthermore, carriers of the JAK2 mutation less often had involvement of the transverse sinus (50% vs. 68%, p = 0.021). Finally, patients with the JAK2 mutation were more prone to have intracerebral hemorrhage (ICH, 31% vs. 17%, p = 0.044), but there was no significant difference between groups in terms of mortality nor functional outcome. CONCLUSIONS: JAK2 mutation is not uncommon in patients with CSVT and should be routinely screened for in this population. CSVT in JAK2 mutation carriers may have a tendency toward involving specific venous sinuses and is associated with a higher rate of ICH but similar overall prognosis.

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Iron deficiency anemia has a high prevalence in children and has repeatedly been implicated as a risk factor for arterial and venous thrombosis. As an effective therapy for iron deficiency anemia is available, understanding the association between this form of anemia and the potentially severe thrombosis phenotype is of major clinical interest. Recent findings shed light on pathophysiology of hypercoagulability resulting from iron-restricted erythropoiesis. Specifically, an animal model of induced iron deficiency allowed identifying multiple mechanisms, by which iron deficiency anemia results in increased thrombus formation and thrombus progression both in arterial and venous thrombosis. These findings complement and support conclusions derived from clinical data. The purpose of this mini review is to summarize current evidence on the association of iron deficiency anemia and thrombosis. We want to increase the awareness of iron deficiency as a risk factor for thrombosis in the pediatric population. We discuss how novel pathophysiological concepts can be translated into the clinical settings and suggest clinical studies on prevention and treatment strategies in high-risk patient groups.

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PURPOSE: To distinguish cerebral venous clots from patent dural sinuses, cortical veins, and calvarium on high-resolution susceptibility-weighted imaging, since there is lack of a well-designed study in the literature. METHODS: A retrospective review of 51 consecutive cases and 27 controls was performed with susceptibility-weighted imaging independently by two investigators. MR venography along with MR sequences other than the susceptibility-weighted imaging served as the reference standard. RESULTS: There were 29 males and 49 females in the age range of 1 month to 70 years (mean 27 ± 19.8 years). Substantial (k 0.84 to 1.0) to good (k 0.60 to 0.75) inter-rater agreement was observed on the magnitude images for the demonstration of clots in all venous segments, excluding left sigmoid sinus, jugular bulb, and internal jugular vein (k 0.43 to 0.56). Comparatively magnitude images demonstrated better sensitivity (0.83 (0.54-1.13) to 1.0), specificity (0.92 (0.85-0.99) to 1.0), and negative predictive value (0.98 (0.94-1.02) to 1.0) for the detection of clots across all venous segments. Magnitude images showed positive predictive value ranging from 0.83 (0.66-1.0) to 1.0 for clots anywhere from the anterior aspect of superior sagittal sinus to bilateral transverse sinuses. For the detection of clots from bilateral sigmoid sinuses to internal jugular veins, magnitude images showed relatively better positive predictive value ranging from 0.57 (0.20-0.94) to 0.75 (0.45-1.06) in comparison to the processed magnitude and minimum intensity pixel images. CONCLUSION: Susceptibility-weighted imaging can successfully distinguish majority of clots from patent dural sinuses, cortical veins, and calvarium with excellent inter-rater agreements.

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In patients with inherited bleeding disorders, thrombus development poses a challenge in balancing the management of thrombosis and bleeding. Pediatric antithrombotic therapy guidelines do not address the treatment of a thrombus in the setting of a bleeding disorder. We present a case series of four children with inherited bleeding disorders presenting with cerebral sinus venous thrombosis and bleeding, in order to summarize the different therapeutic approaches and outcomes of these patients.

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Not required for Clinical Vignette.

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Pediatric glioma treatment can be confounded by eloquent anatomical location and pathologic and genetic characteristics. Current literature suggests that the vascular endothelial growth factor (VEGF) inhibitor bevacizumab has been linked to enhancing disease control; however, its safety and effectiveness are unknown. Bevacizumab has been linked with an increased incidence of intratumoral hemorrhage as well as arterial and venous thromboembolism. A rare adverse effect of chemotherapeutic treatment with bevacizumab is sinus venous thrombosis (SVT), with only a few cases reported to date. This review highlights the pathophysiology of bevacizumab, its rare and life-threatening side effect of SVT, and future recommendations.

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PURPOSE: To report clinical profile, diagnostic challenges, and outcomes in cases of subacute/chronic cerebral sinus venous thrombosis (CSVT) presenting to neuro-ophthalmologists/neurologists. METHODS: This was a multicentric, retrospective, observational study. Records of patients with neuroimaging proven subacute/chronic CSVT seen the from January 1, 2016 to March 31, 2020 were analyzed. Data collected included duration of symptoms, diagnosing physician, ophthalmological vs. focal/generalized neurological symptoms, optic disc examination, perimetry, and neuroimaging findings. Statistical analysis was performed using STATA software. RESULTS: Forty-three patients with subacute (30)/chronic (13) CSVT were identified (32 males, 11 females). Median age was 37 (IQR 27-47) years. The presenting complaints were blurred vision 34 (79%), headaches in 25 (58%), vomiting 12 (28%), and diplopia 11 (26%). Eleven patients had associated sixth cranial nerve palsy. All but two patients had either disc edema/optic atrophy; four had unilateral disc edema at presentation. Ophthalmologists and neurologists diagnosed/suspected CSVT correctly in 13/29 (45%) and 11/14 (78.5%) patients, respectively. Most common initial alternate diagnosis was idiopathic intracranial hypertension in 12 (28%). Female gender, age ≤36, unilateral papilledema, not obtaining venogram at initial workup increased chances of initial alternate diagnosis. Median follow-up duration was 21 days. Average visual function remained stable in majority of patients at last follow-up. In total, 47.6% of patients had best-corrected visual acuity ≥20/30 at the final follow-up. CONCLUSION: In our series, subacute or chronic CSVT presented presented primarily with symptoms of intracranial hypertension. Unilateral papilledema, middle-aged patients, female gender, lack of focal/generalized neurological symptoms created diagnostic dilemma. Visual function remained stable in majority of patients.

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The management of patients with elevated intracranial pressure (ICP) requires a systematic approach. After the failure of tier zero, tier one, and tier two therapies, all potential secondary causes of elevated ICP must be reviewed. Up to 28% of patients with blunt traumatic brain injury (TBI) develop cerebral sinus venous thrombosis (CSVT), among these, patients up to 55% have occlusive thrombi. A literature review revealed a dearth of specific treatment guidelines in this scenario. Here, we present one such case of refractory elevated ICP due to occlusive CSVT secondary to skull fractures. Initial CT venogram (CTV) on admission showed an occlusive CSVT; however, subsequent CTV on the post-trauma day (PTD) 4 and 6 showed non-occlusive thrombi only. The risks of worsening acute TBI-related hemorrhage with systemic anticoagulation versus the benefit of treating an occlusive CSVT are discussed here. In cases of occlusive CSVT with refractory elevated ICP and stable intracranial hemorrhage, the benefit of anticoagulation may outweigh the overall risks of hemorrhage expansion as prolonged uncontrolled ICP elevation is inevitably fatal. In this case, anticoagulation started on PTD 6, led to the resolution of ICP elevation and an excellent outcome for the patient, who was discharged to an acute rehab center, subsequently discharged home with no residual motor deficits, and was able to resume employment. Further prospective trials are necessary to develop guidelines for the management of occlusive CSVT in patients with severe TBI and to determine which patient populations are likely to benefit from early initiation of therapeutic anticoagulation.

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Pediatric acute lymphoblastic leukemia regimens include large L-asparaginase dosages and steroids, which are associated with an increased risk of venous thromboemboli in adolescents and young adults. Herein, we report the case of an 18-year-old male with acute lymphoblastic leukemia, who was treated with the pediatric regimen, in which edoxaban was employed as a prophylaxis against cerebral sinus venous thrombosis. The event happened on day 20 of induction therapy, when brain magnetic resonance imaging demonstrated a cerebral sinus venous thrombosis in the superior sagittal sinus. Anticoagulation therapy was initiated, and the patient's symptoms disappeared 3 days later. The induction therapy was restarted after an interruption of 16 days, and the consolidation therapies, which included L-asparaginase and steroids, were completed. Edoxaban was administered as a prophylaxis during the consolidation therapy. There were no further adverse events. Edoxaban could be an effective prophylaxis for coagulation complications in adolescents and young adults with acute lymphoblastic leukemia.

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OBJECTIVES: Cerebral sinus venous thrombosis (CSVT) is a rare stroke subtype that is more common in women, yet data regarding sex-specific characteristics are sparse. We aimed to study male-specific characteristics among patients with CSVT. MATERIALS & METHODS: Data of consecutive patients with CSVT, admitted to a single medical centre between 2005 and 2020, were retrospectively studied. Demographics, clinical presentations, radiological and outcome parameters were compared between male and female patients. Male patients were further divided into older and younger than 35 years old for additional comparisons. RESULTS: Out of 15,224 patients diagnosed with stroke, 150 patients (1%) presented with CSVT and 47 (31.3%) of them were males. Males had significantly higher rates of previous thrombotic events (22% vs. 7%, p = .009), malignancies (32% vs. 16%, p = .022) and Behcet's disease (22% vs. 2%, p < .001). Additionally, we found that malignancies were significantly more prevalent in older males (48% vs. 17%, p = .022), while Behcet's disease was more often found in younger patients (35% vs. 9%, p = .032). Additional age-related differences in disease characteristics among male patients included a higher frequencies of papilledema (42% vs. 13%, p = .028), and cortical vein thromboses (21% vs. 0% p = .021) observed in the younger men. CONCLUSIONS: There are important differences in risk factors for thrombosis between men and women with CSVT. Behcet's disease is common in younger men, while malignancies are major causes of CSVT in older men.

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OBJECTIVES: To discuss headache secondary to cerebrovascular disease. BACKGROUND: Headache is an important symptom in cerebrovascular diseases. In some conditions, headache is the leading symptom. Migraine is associated with an increased risk of stroke. METHODS: The authors undertook a literature search for the terms "headache" and "cerebrovascular diseases". RESULTS: We report studies on headache in subarachnoidal hemorrhage, intracerebral hemorrhage, ischemic stroke, TIA, basilar artery thrombosis, cervical artery dissection, cerebellar stroke, arteritis and cerebral sinus venous thrombosis. In addition, we discuss migraine and stroke and thunderclap headache. CONCLUSIONS: Headache is a leading symptom in many cerebrovascular diseases. Headache in combination with focal neurological deficits requires immediate diagnosis and treatment.

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Vaccine-induced immune thrombotic thrombocytopenia (VITT) (also termed thrombosis with thrombocytopenia syndrome or vaccine-induced thrombotic thrombocytopenia or vaccine-induced immune thrombocytopenia) is characterized by (i) venous or arterial thrombosis; (ii) mild-to-severe thrombocytopenia; (iii) positive antiplatelet factor 4 (PF4)-polyanion antibodies or anti-PF4-heparin antibodies detected by the HIT (heparin-induced thrombocytopenia) ELISA; (iv) occurring 5-30 days after ChAdOx1 nCoV-19 (AstraZeneca) or Ad26.COV2.S (Johnson & Johnson/Janssen) vaccination. VITT's incidence is 1 per 100 000 vaccinated people irrespective of age and up to 1 in 50 000 for people <50 years of age with the AstraZeneca COVID-19 vaccine. The exact mechanism by which adenovirus-vectored COVID-19 vaccines trigger this syndrome is still unclear, as for the increased risk for acute cerebral sinus venous thrombosis and splanchnic vein thrombosis as compared to other locations of venous thrombotic events. VITT is associated with the detection of anti-PF4 antibodies, unrelated to previous use of heparin therapy. PF4 antibodies are thought to activate platelets via the platelet FcγRIIA receptors leading to further platelet activation that causes thrombosis and thrombocytopenia.

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Megalencephaly-capillary malformation-polymicrogyria (MCAP) syndrome (OMIM #602501) is characterized by megalencephaly, midline capillary malformations, and cortical malformations. This genetic overgrowth syndrome is associated with mosaic gain-of-function pathogenic PIK3CA variants (OMIM #171834).

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To collect and summarize pregnancy outcomes among women with a history of cerebral sinus vein thrombosis (CSVT). A retrospective multicenter case-control study. The study group comprised all women diagnosed with CSVT during 2004-2018 at four university hospitals, and with follow-up data of pregnancy. A control group of women with a singleton pregnancy was established by matching, four-to-one, according to maternal age. The data of 74 pregnancies of 65 women with CSVT were analyzed. The median time-to-pregnancy interval from the CSVT was 4.2 [2.7-6.8] years. Anticoagulation therapy in the form of enoxaparin was administered in 68 (91.9%) pregnancies. Adjunctive low-dose aspirin was used throughout 12 (16.2%) pregnancies. Overall, 54 (73.0%) of the pregnancies ended in live births and 20 (27.0%) in miscarriage. The use of anticoagulation therapy during pregnancy was positively associated with live birth outcome (P < 0.001). Late adverse outcomes were encountered in 19 (25.7%) pregnancies, including the delivery of a small for gestational age infant (n = 12), gestational hypertensive disorders (n = 6) and placental abruption (n = 3). The use of adjunctive aspirin was associated with a lower rate of late adverse pregnancy outcomes (P = 0.03). No recurrent CSVT, thrombosis at other sites, and major bleeding episodes were observed during pregnancy. Live-birth rate was higher (P = 0.007) and the rate of late adverse outcome was lower (P = 0.01) for the control (n = 296) than the study group. Among pregnant women with a prior CSVT, no recurrent thrombosis events were observed during gestation. The use of prophylactic anticoagulation was associated with live birth. The use of adjunctive aspirin should be further studied in this setting, as its utilization correlated with a lower rate of late pregnancy complications.

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