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Cerebral Venous Sinus Disorder

Disease of Intracranial Venous Sinus


Presentation

  • Chronic presentation Could be like a pseudotumor syndrome or headaches of mild severity for months like chronic daily headache. Papilledema is usually present. Cortical vein thrombosis could present with seizures.[drrahulchakor.com]
  • Clinical presentation The overwhelming majority of patients present with headache [5]. The presentation patterns are secondary to the underlying pathology associated with cerebral thrombosis.[nuemblog.com]
  • For clinical manifestations, 56 cases (90.32%) presented headache; 16 cases (25.81%) presented choked papilla, 13 cases (20.97%) presented limited neural function defect, 11 cases (17.74%) presented epileptic seizure and 5 cases (8.06%) presented consciousness[spandidos-publications.com]
  • The majority of patients present under the age of 50 years (80%), with a mean age of 39 years [1,2,6]. Less than 10% of patients present over the age of 60 years [3,6].[epmonthly.com]
  • The majority of patients present under the age of 50 years (80%), with a mean age of 39 years. 1-3,6 Presentation CVT can be a difficult diagnosis to make due to a wide variety of signs and symptoms.[emdocs.net]
Anemia
  • Risk factors for children and infants include: Problems with the way their blood forms clots Sickle cell anemia Chronic hemolytic anemia Beta-thalassemia major Heart disease — either congenital (you're born with it) or acquired (you develop it) Iron deficiency[hopkinsmedicine.org]
  • Causes of on-going or recurrent thrombosis should be identified and treated aggressively, such as iron deficiency anemia, parameningeal infection (mastoiditis), inflammatory bowel disease, nephrotic syndrome, and antiphospholipid antibody syndrome.[clinicaladvisor.com]
  • Risk factors for children and infants include: Problems with the way their blood forms clots Sickle cell anemia Chronic hemolytic anemia Beta-thalassemia major Heart disease. This can be congenital (you're born with it) or acquired (you develop it).[cedars-sinai.edu]
  • ., polycythemia, sickle-cell anemia ) Pregnancy Malignancies Minor head trauma Neurosurgical procedures (e.g., lumbar puncture ) Additional associated systemic conditions Inflammatory bowel disease (e.g., Crohn's disease ) Collagen vascular diseases and[amboss.com]
  • Hyperhomocysteinemia, Cyanotic congenital heart disease) Malignancy CNS Solid tumor outside CNS (Colorectal, Dysgerminoma, Ewing’s sarcoma) Hematological (Hodgkin’s lymphoma, Bone marrow transplant) Hematological condition Polycythemia, Thrombocytosis Anemia[eyewiki.aao.org]
Developmental Delay
  • Complications of venous sinus thrombosis include: Impaired speech Difficulty moving parts of the body Problems with vision Headache Increased fluid pressure inside the skull Pressure on nerves Brain injury Developmental delay Death Can cerebral venous[hopkinsmedicine.org]
  • Complications of CVST include: Speech problems Difficulty moving parts of the body Problems with vision Headache Increased fluid pressure inside the skull Pressure on nerves Brain injury Developmental delay Death Can cerebral venous sinus thrombosis be[cedars-sinai.edu]
  • delay Death Diagnosis Doctors typically take a medical history and do a physical exam.[womenfitness.net]
Infertility
  • […] hemolytic anemia Beta-thalassemia major Heart disease — either congenital (you're born with it) or acquired (you develop it) Iron deficiency Certain infections Dehydration Head injury For newborns, a mother who had certain infections or a history of infertility[hopkinsmedicine.org]
  • Iron deficiency Certain infections Dehydration Head injury For newborns, a mother who had certain infections or a history of infertility Risk factors for adults include: Pregnancy and the first few weeks after delivery Problems with blood clotting; for[cedars-sinai.edu]
Hodgkin Lymphoma
  • ’s lymphoma, Bone marrow transplant) Hematological condition Polycythemia, Thrombocytosis Anemia Paroxysmal nocturnal hemoglobinuria CNS Disorders Dural fistulae Venous anomaly Arteriovenous malformation Inflammatory disease Behçet’s disease Antiphospholipid[eyewiki.aao.org]
Vascular Disease
  • diseases like lupus, granulomatosis with polyangiitis, and Behcet syndrome Obesity Low blood pressure in the brain (intracranial hypotension) Inflammatory bowel disease like Crohn’s disease or ulcerative colitis What are the symptoms of cerebral venous[hopkinsmedicine.org]
  • ., Crohn's disease ) Collagen vascular diseases and blood vessel disorders (e.g., lupus erythematosus, Granulomatosis with polyangiitis, temporal arteritis ) Hyperhomocysteinemia Hematologic conditions (e.g., paroxysmal nocturnal hemoglobinuria, thrombotic[amboss.com]
  • […] cerebral venous sinus thrombosis CSF pressure modified Rankin Scale papilledema NIHSS Additional relevant MeSH terms: Layout table for MeSH terms Thrombosis Papilledema Sinus Thrombosis, Intracranial Intracranial Hypertension Embolism and Thrombosis Vascular[clinicaltrials.gov]
  • diseases such as lupus, granulomatosis with polyangiitis, and Behcet syndrome Obesity Low blood pressure in the brain (intracranial hypotension) Inflammatory bowel disease such as Crohn’s disease or ulcerative colitis What are the symptoms of cerebral[cedars-sinai.edu]
  • Vascular diseases include systemic lupus erythematosus (SLE), Wegener's granulomatosis and Behçet's disease. A survey from Saudi Arabia found Behçet's syndrome to be a common aetiological factor.[patient.info]
Hypotension
  • […] and S deficiency, antithrombin III deficiency, lupus anticoagulant, or factor V Leiden mutation Cancer Collagen vascular diseases like lupus, granulomatosis with polyangiitis, and Behcet syndrome Obesity Low blood pressure in the brain (intracranial hypotension[hopkinsmedicine.org]
  • Intracranial hypotension after a lumbar puncture may cause intracranial veins to dilate, causing reduced blood flow, stasis and subsequent CVST.[nvic.nl]
  • Intracranial hypotension Isolated cortical venous thrombosis has been associated with intracranial hypotension syndrome, but only rarely.[emedicine.medscape.com]
  • S deficiency, antithrombin III deficiency, lupus anticoagulant, or factor V Leiden mutation Cancer Collagen vascular diseases such as lupus, granulomatosis with polyangiitis, and Behcet syndrome Obesity Low blood pressure in the brain (intracranial hypotension[cedars-sinai.edu]
  • […] example, antiphospholipid syndrome, protein C and S deficiency, antithrombin III deficiency, lupus anticoagulant, or factor V Leiden mutation Cancer Collagen vascular diseases like lupus, Wegener’s granulomatosis, and Behcet syndrome Obesity Intracranial hypotension[womenfitness.net]
Heart Disease
  • Risk factors for children and infants include: Problems with the way their blood forms clots Sickle cell anemia Chronic hemolytic anemia Beta-thalassemia major Heart disease — either congenital (you're born with it) or acquired (you develop it) Iron deficiency[hopkinsmedicine.org]
  • disease) Malignancy CNS Solid tumor outside CNS (Colorectal, Dysgerminoma, Ewing’s sarcoma) Hematological (Hodgkin’s lymphoma, Bone marrow transplant) Hematological condition Polycythemia, Thrombocytosis Anemia Paroxysmal nocturnal hemoglobinuria CNS[eyewiki.aao.org]
  • Risk factors for children and infants include: Problems with the way their blood forms clots Sickle cell anemia Chronic hemolytic anemia Beta-thalassemia major Heart disease. This can be congenital (you're born with it) or acquired (you develop it).[cedars-sinai.edu]
  • These are further guided by known acute or chronic conditions such as leukemia, congenital heart disease, or hemoglobinopathy detailed as follows: Prothrombin time/international normalized ratio, partial thromboplastin time in all patients before treatment[clinicaladvisor.com]
  • Maternal preeclampsia/eclampsia is a reported risk factor for neonatal CVT. 253 Neonatal diseases including head and neck infections, meningitis, dehydration secondary to feeding difficulties or gastroenteritis, and congenital heart disease also cause[stroke.ahajournals.org]
Blurred Vision
  • Symptoms of venous sinus thrombosis may include: Weakness Headaches Pain in the sinus area Pain or pressure in the ear Loss of vision, double vision, or blurred vision Swelling around the eyes Tests for diagnosing sinus thrombosis Your doctor also may[upmc.com]
  • Symptoms of the stroke may include headache, blurred vision, fainting, loss of motor control, seizures and possibly a coma.[raredr.com]
  • These are the physical symptoms that may occur: Headache Blurred vision Fainting or loss of consciousness Loss of control over movement in part of the body Seizures Coma Complications of venous sinus thrombosis include: Impaired speech Difficulty moving[womenfitness.net]
Ulcer
  • […] mainly associated during episodes of relapse of ulcerative colitis.[nnjournal.net]
  • […] anticoagulant, or factor V Leiden mutation Cancer Collagen vascular diseases like lupus, granulomatosis with polyangiitis, and Behcet syndrome Obesity Low blood pressure in the brain (intracranial hypotension) Inflammatory bowel disease like Crohn’s disease or ulcerative[hopkinsmedicine.org]
  • Case presentation A 28-year-old Brazilian pardo woman presented to our hospital with abrupt bilateral vision loss associated with recurrent aphthous oral ulcers 6 months before visual symptom onset.[jmedicalcasereports.biomedcentral.com]
  • Cerebral venous thrombosis in ulcerative colitis. Neurol India 2002; 50:215-217. 21. Terazzi E, Mittino D, Rudà R, Cerrato P, Monaco F, Sciolla R, Grasso E, Leone MA, Cerebral Venous Thrombosis Group.[dusunenadamdergisi.org]
  • […] factor V Leiden mutation Cancer Collagen vascular diseases such as lupus, granulomatosis with polyangiitis, and Behcet syndrome Obesity Low blood pressure in the brain (intracranial hypotension) Inflammatory bowel disease such as Crohn’s disease or ulcerative[cedars-sinai.edu]
Seizure
  • If a seizure does not occur, seizure prophylaxis is not necessary. 1,2,11,39 Further testing for underlying etiology is recommended, including thrombophilia. 1,2,3,6 Follow up imaging at 3-6 months is required to assess for recanalization of the affected[emdocs.net]
  • Patients started on anticoagulants had reduced occurrence of headache, seizures, focal deficits and intracranial hemorrhage [ 6 , 7 ].[omicsonline.org]
  • Seizures, including focal, generalized, and status epilepticus, are seen in 30–40% of patients [7,11]. As seizures are rare in strokes, CVT should be considered in any patient with a focal neurologic deficit and seizure [1,2].[epmonthly.com]
  • Patients with infection, seizure or ICH have a greater risk of death.[nature.com]

Workup

  • Article Access Statistics Viewed 2094 Printed 45 Emailed 1 PDF Downloaded 71 Comments 2 New Developments in the Pathophysiology, Workup, and Diagnosis of Dural Venous Sinus Thrombosis (DVST) and a Systematic Review of Endovascular Treatments Sanjay Konakondla[neurologyindia.com]
  • An extensive workup should be done to exclude systemic vasculitis such as Behçet’s disease.[jmedicalcasereports.biomedcentral.com]
  • (See Workup.) Left lateral sinus thrombosis demonstrated on magnetic resonance venography (MRV). This 42-year-old woman presented with sudden onset of headache. Physical examination revealed no neurologic abnormalities.[emedicine.medscape.com]
  • Subsequent workup led to an angiogram, where sagittal sinus thrombosis with extensive trans-cerebral and trans-osseous emissary vein collateral channels was seen. In retrospect, these findings were present on the patient’s earlier contrast MRI.[neuroangio.org]
Erythrocytosis
  • Erythrocytosis associated with carboxyhemoglobinemia in smokers. South Med J 1980;73:137-139. 4. Ferro JM, CanhĂŁo P, Stam J, Bousser MG, Barinagarrementeria F; ISCVT Investigators.[j-stroke.org]
Colitis
  • […] mainly associated during episodes of relapse of ulcerative colitis.[nnjournal.net]
  • […] factor V Leiden mutation Cancer Collagen vascular diseases like lupus, granulomatosis with polyangiitis, and Behcet syndrome Obesity Low blood pressure in the brain (intracranial hypotension) Inflammatory bowel disease like Crohn’s disease or ulcerative colitis[hopkinsmedicine.org]
  • Cerebral venous thrombosis in ulcerative colitis. Neurol India 2002; 50:215-217. 21. Terazzi E, Mittino D, Rudà R, Cerrato P, Monaco F, Sciolla R, Grasso E, Leone MA, Cerebral Venous Thrombosis Group.[dusunenadamdergisi.org]
  • Leiden mutation Cancer Collagen vascular diseases such as lupus, granulomatosis with polyangiitis, and Behcet syndrome Obesity Low blood pressure in the brain (intracranial hypotension) Inflammatory bowel disease such as Crohn’s disease or ulcerative colitis[cedars-sinai.edu]
  • Crohn's disease and ulcerative colitis increase the risk, as does the use of steroids to treat them.[patient.info]
Brain Edema
  • 1 近畿大学 医学部放射線診断学教室 キーワード: 基底核疾患, 硬膜, MRI, 視床疾患, 静脈洞血栓症, 頭痛, 造影剤, 脳浮腫, 大脳出血, 頭部CT Keyword: Brain Edema, Basal Ganglia Diseases, Contrast Media, Dura Mater, Cerebral Hemorrhage, Headache, Magnetic Resonance Imaging, Sinus Thrombosis, Intracranial, Thalamic[webview.isho.jp]
  • Furthermore, CVST induces brain edema, congestion and hemorrhagic cerebral infarction in the drainage area.[spandidos-publications.com]
  • Two different mechanisms have to be identified; however, they are interrelated in many cases. [4] The occlusion of a cerebral vein leads to localized brain edema and a so-called venous infarction.[annalsofian.org]
  • Severe brain edema caused by a meningioma obstructing cerebral venous outflow and treated with venous sinus stenting. Case report. J Neurosurg. 2008; 108 :372-6 6. Ahmed RM, Wilkinson M, Parker GD, Thurtell MJ, Macdonald J, McCluskey PJ. et al.[medsci.org]
  • The main cause of acute death with CVT is transtentorial herniation secondary to a large hemorrhagic lesion, 5 followed by herniation due to multiple lesions or to diffuse brain edema.[stroke.ahajournals.org]

Treatment

  • Treatment should begin immediately and must be done in a hospital.[hopkinsmedicine.org]
  • Treatment Anticoagulation, the administration of a blood thinning drug is the first line of treatment and has been shown to be very effective.[radiology.ucla.edu]
  • , was regarded as the treatment standard. 2015 Elder [ 18 ] Venous sinus stenting is a valuable treatment for fulminant idiopathic intracranial hypertension A good treatment effect for explosive IIH with auxiliary cerebrospinal fluid drainage and diversion[medsci.org]
  • Ongoing controversies regarding etiology, diagnosis, treatment Treatment of neonates with AC is controversial.[clinicaladvisor.com]
  • These tests will include: CT scan MRI scan Angiography Sinus Thrombosis Treatment Removing clots Clot removal using catheters and other specialized equipment, antibiotics, and anticoagulation drugs are the main methods used for the treatment of venous[upmc.com]

Prognosis

  • Table 4: Predictors influencing the prognosis of pregnancy-related CVT patients.[nature.com]
  • & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.[dovemed.com]
  • Complications include seizures, hydrocephalus, intracranial hypertension and neurological deterioration. [ 10 ] Prognosis Long-term follow-up suggests a generally good prognosis but there may be residual pyramidal symptoms, epilepsy, visual field defects[patient.info]
  • […] are associated with a poorer prognosis.[pmj.bmj.com]

Etiology

  • Prior to the advent and widespread use of antibiotics in developed nations, CVT was largely due to infectious etiologies such as mastoiditis, orbital cellulitis, and facial cellulitis[2]. Risk Factors Table 1.[eyewiki.aao.org]
  • It is caused by a wide range of etiologies, polycythemia is one among them. Polycythemia is a myeloproliferative disorder that is caused by a variety of etiologies [ 2 ].[j-stroke.org]
  • In 7 patients, the etiology remained unknown. The most common etiologic factor for women was the use of OCPs (n 7). OCPs’ users comprised the largest group (n 7) in women. We could not find the etiology in four of the men.[dusunenadamdergisi.org]
  • Supportive / Symptomatic treatment with IV fluids, anticonvulsants, and lowering of raised intracranial pressure, antibiotics for infective etiology. The duration of anticoagulation depends on the risk factor for thrombosis.[drrahulchakor.com]
  • Etiology Noninfectious Oral contraceptives, pregnancy, and postpartum period Hypercoagulable states Blot clotting disorders (e.g., factor V Leiden, protein C and S deficiencies, antiphospholipid syndrome ) Hematologic diseases (e.g., polycythemia, sickle-cell[amboss.com]

Epidemiology

  • Epidemiology References: [1] [2] Epidemiological data refers to the US, unless otherwise specified.[amboss.com]
  • Please refer to the article on cerebral venous thrombosis for a broad discussion on epidemiology and risk factors. Presentation is variable and can range from asymptomatic to coma and death.[radiopaedia.org]
  • Thromboembolism and oral contraceptives: an epidemiologic case-control study. Am J Epidemiol. 1969;90:365-80. Vessey MP, Doll R. Investigation of relation between use of oral contraceptives and thromboembolic disease. A further report.[ijrcog.org]
  • Epidemiology Intracranial venous thrombosis appears to have become more common in recent years. However, this has occurred as modern imaging techniques have improved diagnosis.[patient.info]
  • Epidemiology and risk factors for thromboembolic complications of childhood nephrotic syndrome: a Midwest Pediatric Nephrology Consortium (MWPNC) study. J Pediatr, 155 (2009), pp. 105-110 [5] M. Shoroff, G. de Veber.[revistanefrologia.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology of Cerebral Venous Thrombosis - An overview Abstract Cerebral venous sinus thrombosis is a disorder with a unique pathophysiology which needs to be described.A Medline search of all articles detailing pathophysiology of CVST was done,[jpma.org.pk]
  • Article Access Statistics Viewed 2094 Printed 45 Emailed 1 PDF Downloaded 71 Comments 2 New Developments in the Pathophysiology, Workup, and Diagnosis of Dural Venous Sinus Thrombosis (DVST) and a Systematic Review of Endovascular Treatments Sanjay Konakondla[neurologyindia.com]
  • ., after sinusitis ) Through facial infections Meningitis References: [3] [4] [5] Pathophysiology Clinical features Headache (acute, subacute, or chronic) Nausea, vomiting, vision impairment ; ), bilateral papilledema on ophthalmoscopy Cranial nerve symptoms[amboss.com]
  • […] polyangiitis (formerly known as Wegener’s granulomatosis) Churg-Strauss syndrome Thromboangiitis obliterans Inflammatory bowel disease Sarcoidosis Other disorders Thyroid disease (thyroiditis, hyperthyroidism, hypothyroidism) General Pathology Add text here Pathophysiology[eyewiki.aao.org]
  • […] straight sinus, and then to the internal jugular vein (IJV) Superficial venous system has numerous anastomses and predominantly drain the SSS and the LS Deep white matter and basal ganglia are drained by the deep venous system toward the vein of Galen Pathophysiology[lifeinthefastlane.com]

Prevention

  • This prevents blood from draining out of the brain. As a result, blood cells may break and leak blood into the brain tissues, forming a hemorrhage. This chain of events is part of a stroke that can occur in adults and children.[hopkinsmedicine.org]
  • These medications are given in order to prevent the enlargement of the blood clot inside the affected dural sinus and to prevent the formation of new blood clots which could lead to new and possibly worse strokes.[verywell.com]
  • […] is poor with and the condition is known to have a high mortality rate Please find comprehensive information on Cavernous Sinus Thrombosis regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention[dovemed.com]
  • Prevention You can do a lot to prevent stroke by leading a heart healthy lifestyle: Eat a low-fat diet including lots of fruits and vegetables. Get daily exercise. Avoid cigarette smoke. Control chronic health conditions, such as diabetes.[womenfitness.net]
  • You can do a lot to prevent stroke by leading a heart healthy lifestyle: Eat a low-fat diet, including lots of fruits and vegetables. Get daily exercise. Stay away from cigarette smoke. Control chronic health conditions such as diabetes.[cedars-sinai.edu]

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