|Year : 2023 | Volume
| Issue : 1 | Page : 14-18
Clinicoetiological profile of cerebral venous sinus thrombosis patients at a tertiary care center
Adnan Firdous Raina, Amit Chandra, Waseem Dar, Hilal Ahmad Ganie, Zubair Kawaja, Maqbool Wani, Ravouf Asimi
Department of Neurology, SKIMS, Srinagar, Jammu and Kashmir, India
|Date of Submission||12-Oct-2022|
|Date of Decision||25-Oct-2022|
|Date of Acceptance||04-Nov-2022|
|Date of Web Publication||17-Jan-2023|
Dr. Adnan Firdous Raina
Department of Neurology, SKIMS, Soura, Srinagar, Jammu and Kashmir
Source of Support: None, Conflict of Interest: None
Background: Cerebral venous sinus thrombosis (CVST) accounts for 10%–20% of strokes in young persons. In India, CVST accounts for around 30% of all strokes. The majority of CVSTs are caused by procoagulant circumstances, with pregnancy and early puerperium being well-established risk factors. The study aimed to look into the clinical profile, radiological characteristics, etiological variables, and outcome of CVST in venous stroke patients admitted to a tertiary care facility. Methods: We included individuals between the ages of 18 and 75 years who had a cerebral venous thrombosis (CVT) diagnosis confirmed by magnetic resonance imaging (MRI) + magnetic resonance venography computed tomography (CT) plus CT venography. The research included all individuals suspected of having CVST with or without particular neurological deficits and a confirmed imaging diagnosis. Patients were removed in situations of ambiguous neuroimaging, arterial strokes, space-occupying lesions, metabolic encephalopathy, and patient reluctance to participate. Results: This study included 82 patients, 21 (25.6%) males and 61 (74.4%) of whom were females. The most common presenting symptom was headache (79.2%), followed by vomiting (54.8%) and abnormal sensorium (35.3%). In this study, 34/82 (41.4%) patients had evident clinical triggers and were classified as induced CVT. Para infectious disease was recognized as a risk factor for CVT in 13/34 (38.2%) patients. A prothrombotic conditions could be established in 48 (58.5%) of these patients. Conclusion: CVST is a treatable and reversible cause of stroke in adolescents. The clinical presentation varies greatly, and symptoms may appear gradually over weeks or months. Although it is still an uncommon cause of headache and stroke, MRI has allowed for early detection.
Keywords: Cerebral venous thrombosis, hemorrhagic infarct, heparin, risk factors, venography
|How to cite this article:|
Raina AF, Chandra A, Dar W, Ganie HA, Kawaja Z, Wani M, Asimi R. Clinicoetiological profile of cerebral venous sinus thrombosis patients at a tertiary care center. Curr Med Issues 2023;21:14-8
|How to cite this URL:|
Raina AF, Chandra A, Dar W, Ganie HA, Kawaja Z, Wani M, Asimi R. Clinicoetiological profile of cerebral venous sinus thrombosis patients at a tertiary care center. Curr Med Issues [serial online] 2023 [cited 2023 Feb 4];21:14-8. Available from: https://www.cmijournal.org/text.asp?2023/21/1/14/367857
| Introduction|| |
Thrombosis of the cerebral veins and dural sinuses is a separate subtype of cerebrovascular illness that can be difficult to diagnose and treat owing to varied and deceptive clinical manifestations. Cerebral venous sinus thrombosis (CVST) contributes to 10%–20% of strokes in young people, which accounts for approximately 30% of all strokes in India. More than 100 CVST causes have been documented in the literature. Despite comprehensive research, no reason is found in 20%–25% of the instances. Procoagulant conditions cause the majority of primary or unprovoked CVST. In India, pregnancy and early puerperium are well-established risk factors for CVST.
In 70%–90% of patients, the most frequent presenting symptom is a headache. In one-third to three-quarters of instances, focal impairments such as hemiparesis, seizures, reduced state of awareness, and papilledema might develop. The symptoms above can be seen in various combinations, ranging from elevated intracranial pressure without localization to profoundly altered sensorium and dense hemiparesis. Neuroimaging is a critical component in the diagnosis of CVST. The current diagnostic tool of choice, with very high sensitivity and specificity, is magnetic resonance imaging (MRI) of the brain in conjunction with magnetic resonance venography (MRV).
Heparin-based antithrombotic medication, oral anticoagulants (OACs), thrombolysis (intravenous/local thrombolysis via selective sinus catheterization), and a combination of thrombolysis and anticoagulation are now available for CVST., Long-term anticoagulant prophylaxis may benefit from screening for an underlying procoagulant disease. Furthermore, symptomatic therapy of intracranial pressure, seizures, headache, vision dysfunction, and etiological treatment is required to address the related disorders.
The outcome of CVST is rather unexpected; it is not uncommon to observe spectacular healing in completely comatose patients and abrupt deterioration in aware people owing to the thrombosis extension. Early detection and treatment of CVST are critical since they can prevent morbidity and save lives.
By performing this study at our tertiary care facility, we tried to bridge gaps in our understanding of CVST in an Indian environment. Its particular goal was to look into the clinical profile, radiological characteristics, etiological variables, and outcome of CVST in venous stroke patients.
| Methods|| |
The study was carried out at a tertiary care multispeciality hospital in the central Kashmir Division. The institutional ethics committee approved the study protocol, and only patients/next of Kin (NOK) who gave written informed consent were included in the study. The study was a prospective, observational study with patients recruited over 3 years from August 2018 to April 2021.
We enrolled patients of either gender between 18 and 75 who had a cerebral venous thrombosis (CVT) diagnosis confirmed by MRI plus MRV computed tomography (CT) plus CT venography. Patients should have attained clinical stabilization after getting the necessary acute CVT therapy.
The research included all patients suspected of having CVST based on a clinical profile of elevated intracranial pressure seizures, with or without specific neurological impairments and a verified imaging diagnosis based on MRI of the brain with MRV. All such patients who were eligible for the trial provided written informed consent. In situations of (i) unclear neuroimaging, (ii) arterial strokes, (iii) space-occupying lesions, (iv) metabolic encephalopathy, and (v) patient unwillingness to participate, and patients were eliminated.
According to a comprehensive pro forma, all CVST cases were subjected to a meticulously detailed history, clinical examination, and laboratory testing. Those who appeared within 48 h were deemed to have an acute onset, those who arrived after 48 h but <1 month were considered to have subacute onset, and those who presented more than 1 month following symptoms were considered to have a chronic onset beginning. Hazardous alcohol use (WHO working definition eight regular average intake of 20–40 g/day for women and 40–60 g/day for males) was also particularly noted in all patients since it has been linked to venous strokes.
All patients received brain MRI and MRV on a 1.5 T MRI machine (Symphony; Siemens; Erlangen, Germany). An axial Fluid Attenuated Inversion Recovery, axial and coronal T2W, T1 three-dimensional (3D) sagittal, diffusion-weighted at B values of 0.500 and 1000 s/Sq mm with a production of apparent diffusion coefficient map, and an axial gradient sequence were all part of the scan procedure. After injecting 20 ml of gadolinium (Magnevist; Bayer Zydus) using a pressure injector at a rate of 4 ml/s and obtaining multiple continuous images of the brain, contrast MRV was performed. The resulting pictures were subtracted from the noncontrast photos. After postprocessing the basic concepts with appropriate software on a computer, a 3D MR venogram was produced. All patients received conventional institutional protocols of anticoagulation (5–7 days) with low-molecular-weight heparin (LMWH) followed by 3–5 days overlap with OACs to achieve an international normalized ratio in the 2–3 range. Antiepileptic medications, osmotic diuretics, and other supportive therapies were administered as needed. All patients were followed up every month for 6 months to determine responsiveness to treatment and clinical outcomes. Only in unprovoked CVST instances 3 months after the ictus was a procoagulant workup performed. Blood samples were obtained 2 weeks after discontinuing OAC and switching to LMWH. A conventional testing panel of factor VIII, protein C a S, antiphospholipid Antibodies (APLAs) panel, and anti-thrombin III were included in the workup. Due to financial restrictions, no genetic testing was performed.
| Results|| |
This study included 82 patients, 21 (25.6%) males, and 61 (74.4%) of whom were females. The most typical age group involved was <30 years, 35 (42.6%) cases, while the mean age at the time of presentation was 41 ± 5 years. The majority of the patients had an acute onset (65.8%), followed by subacute (29.2%) and chronic (4.8%), respectively. [Table 1] shows the clinical characteristics of CVST in the study group. The most common presenting symptom was headache (79.2%), followed by vomiting (54.8%), abnormal sensorium (35.3%), and seizures (32.9%). Limb weakness was observed in (29.2%) of the cases. Pallor was evident in 25.6% of patients at the beginning, while fever was present in 19.5% of cases. The most prevalent neurological symptom was hemiparesis (17.0%), followed by papilledema (13.4%). In 15.8% of the cases, there were cranial nerve palsies. We also did an MRI brain with venography in all patients per specific protocol. We observed that venous infarct with hemorrhagic transformation was a predominant abnormality. However, there were patients with venous infarct 17 (20.7%) and some patients who had only features of raised intracranial pressure (ICP) were on venography confirmed to have sinus venous thrombosis 15 (18.2%) as shown in [Table 2].
|Table 1: Clinical profile of cerebral venous sinus thrombosis in the study group|
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|Table 2: Sinuses involved in magnetic resonance venography in the study group (n=82)|
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[Table 3] shows the MRV-infected sinuses in the study group. MRI Brain with venography showed that among individual sinuses, we had few patients with isolated sinuses involvement, and most of the cases had involvement of a combination of sinuses. We had a higher frequency of participation of transverse and sigmoid sinuses 32 (39.02%), followed by Superior sagittal sinus and transverse sinus in 14 (17.07%) patients.
|Table 3: Sinuses involved in magnetic resonance venography in the study group (n=82)|
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In this study, 34/82 (41.4%) patients had evident clinical triggers and were classified as induced CVT. Parainfectious disease was recognized as a risk factor for CVT in 13/34 (38.2%), high altitude exposures were identified as a risk factor in 8/34 (23.5%), puerperium was identified as a risk factor in 7/34 (20.5%), and oral contraceptive pills (OCP) use was identified as a risk factor in 6/34 (17.6%). A standard procoagulant workup was performed in the other subset of unprovoked CVT 48/82 (58.5%) patients who had no clinical triggers. A prothrombotic condition could be established in 48 (58.5%) of these instances. Elevated factor VIII was found in 29/48 (60.4%), Protein C deficit in 10/48 (20.8%), Protein S deficiency in 6/48 (12.5%), and APLA in 1/48 (2.08%). In nine cases, increased factor VIII and protein C deficit, and both protein C and S deficiencies in four cases, patients exhibited combined prothrombotic conditions. Antithrombin III deficiency was not found in any of the patients. There could have prothrombotic genetic disorders such as Factor V Leiden, which were not evaluated in this study, whereas methylenetetrahydrofolate reductase deficiency was found among 3 (3.65%) patients [Table 4] and [Table 5]. We did neuroimaging in all cases; initially, CT head was performed it suggested abnormalities in 73 points among the study population. Venous infarct with hemorrhagic transformation 34 (41.4%) was the most common abnormality found, followed by indirect signs such as hyperdense sinuses in 22 (26.82%) patients. Other findings included venous infarcts, intracranial hemorrhage, and subarachnoid hemorrhage (SAH) among 23.1%, 23.1%, and 10.9% cases, respectively [Table 6].
|Table 5: Procoagulant conditions identified in unprovoked cerebral venous sinus thrombosis (n=48)|
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| Discussion|| |
Unlike arterial stroke, CVST is more common in young people. Because of the varied and confusing clinical appearance, it is frequently a diagnostic difficulty. Modern neuroimaging tools, diagnostic laboratory investigations, and genetics, on the other hand, are offering additional knowledge about CVST risk factors and clinical characteristics. We compared our CVST experience to previous India and the west studies, emphasizing its different clinical presentations, risk factors, and neuroimaging.
When comparing the age groups involved, 20–40 years was the most common age group in several research, with a mean age of onset of 35 years. The current study found a similar result, with a mean age of onset of 41 ± 5 years. As the study was conducted at a tertiary care center, there was a female preponderance (Male: Female – 1:2.9) (The study population contains predominately females).
Headache was the most prevalent symptom in the current study, accounting for 79.26% of participants. Seizures are significantly more common in CVST than in ischemic stroke. Seizures are sometimes harbinger symptoms in CVT and should raise suspicions about the diagnosis. Seizures occurred in 32.9% of individuals in our study, which is almost comparable to Narayan et al. and most other Western investigations.
Twenty-nine percent of patients had an altered level of consciousness at presentation, which is comparable with Wasay et al. and Ameri et al. The cause of altered sensorium can be attributed to postictal states, raised ICP, aphasia, deep CVT, and delirium due to viral meningoencephalitis. Nineteen percent of patients had fever at the onset which is similar to the findings of Wasay et al. and Anadure et al.
Prothrombotic disorders are the most frequent risk factor found in published literature worldwide for spontaneous CVST. In the international study on cerebral venous thrombosis cohort, 34% of patients exhibited prothrombotic disorders, with 22% having underlying hereditary prothrombotic states. However, previously published research from India lacked information on these prothrombotic factors due to a lack of laboratory data. Recently, Pai et al. and Narayan et al., respectively, found thrombophilia as a risk factor for CVST in 18% and 12.3% of patients. In our study, 48/82 (85.4%) of the unprovoked CVST patients had thrombophilic disorders. Plasma factor VIII was increased in 29/48 (60.41%) of the patients tested. Protein C deficit was found in 10/48 (20.8%) of the individuals tested, whereas protein S deficiency was found in 6/48 (12.5%). All of them are well-known laboratory traits that lead to venous thrombosis. Elevated factor VIII has been identified as an independent risk factor for CVST in Indians. Most CVST cases reported in the literature have a multifactorial etiology, implying that a thorough prothrombotic workup is required. However, this is not feasible in many centers due to financial constraints.
During 8 years, Nagaraja and Sarma reported that 200 of 230 (86%) cases of CVST encountered at a tertiary neurology center were puerperal. Other Indian writers' experiences have been comparable, as Neki discovered that 62% of their CVT cases occurred during the postpartum period. The puerperal group accounts for just 7/34 of the participants (20.5%). The lower incidence in this study is due to enhanced and thorough obstetric care with 100% institutional births that may have reduced puerperal CVT rates.
The reported prevalence of involvement of various sinuses in the international investigation on cerebral vein and dural sinus thrombosis was superior sagittal sinus (62%), transverse sinus (42%), straight sinus (18%), cortical veins (17.1%), and deep veins (17.1%) (10.9%). Combined transverse sinus and sigmoid sinus were most commonly involved in the current study, (32/82), 39.0%, followed by combined superior sagittal sinus and transverse sinus (14/82), 17.0%, transverse sinus alone in (12/82) 14.6%, followed by superior sagittal sinus in (9/82) 10.9%, sigmoid sinus in (6/82) 7.3%, respectively, which differs with reported findings by Bousser and Ferro.
The most prevalent MRI finding was hemorrhagic venous infarction which accounted for 41.4% of all patients. Banakar and Hiregoudar made similar observations in a previous investigation. Neuroimaging features of CVT can include the focal area of edema or venous infarction, hemorrhagic infarction, diffused brain edema, or rarely isolated SAH. In our study, CT brain suggested abnormalities in 85% in the dabigatran group and 92.5% in the warfarin group. It was normal in 9 (10.9%) patients. Provenzale et al. have reported that CT brain can be misinterpreted as normal in up to 40% of patients. Cantú and Barinagarrementeria have described normal CT brain in about 9.47% of patients in his study. Contrast administration is almost always required to increase the reliability of CT in diagnosing CVT. In our study, venous infarct occurred in 26.1% in dabigatran and 20% in the warfarin group. However, venous infarct with hemorrhagic transformation was the most common abnormality recorded in both treatment groups. Direct signs of CVST in CT brain occurred in 12 (14.6%) patients. Our study had a low frequency of direct signs due to decreased number of contrast venography. Intracerebral hemorrhage was present in 23.8% of patients in the dabigatran group and 22.5% in the warfarin group. In minority cases, CT did demonstrate indirect signs of CVT hyperdense sinuses convex SAH.
| Conclusion|| |
CVST is still a rare cause of headache and stroke, but current MRI has enabled early and accurate identification. It is one of the curable and reversible causes of stroke in young people. The clinical presentation is quite variable, and symptoms may develop over weeks or even months.
All authors of this manuscript declare that this scientific study is in compliance with standard reporting guidelines set forth by the EQUATOR Network. The authors ratify that this study required institutional review board/ethics committee review, and hence, prior approval was obtained IRB Min. No. IEC-SKIMS/38/2021/SSB dated 27/07/2018). We also declare that we did not plagiarize the contents of this manuscript and have performed a plagiarism check.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Table 1], [Table 2], [Table 3], [Table 4], [Table 5], [Table 6]