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Evaluation of plasma D-dimer plus fibrinogen in predicting acute CVST. Int J Stroke 2014; 9:166. 126. Canh o P, Abreu LF, Ferro JM, et al. Safety of lumbar puncture in patients with cerebral venous thrombosis. Eur J Neurol 2013; 20:1075. Topic 1103 Version 40.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 27/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate GRAPHICS Mechanisms of cerebral venous thrombosis CSF: cerebrospinal fluid. Graphic 80911 Version 3.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 28/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Major cerebral veins and sinuses Graphic 72961 Version 2.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 29/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Systemic and local conditions increasing the risk of cerebral venous thrombosis Transient risk factors Infection Central nervous system Ear, sinus, mouth, face, and neck Systemic infectious disease Pregnancy and puerperium Dehydration Mechanical precipitants Head injury Lumbar puncture Neurosurgical procedures Jugular catheter occlusion Drugs Oral contraceptives Hormone replacement therapy Androgens Asparaginase Tamoxifen Glucocorticoids Permanent risk factors Inflammatory diseases Systemic lupus erythematosus Beh et disease Granulomatosis with polyangiitis Thromboangiitis obliterans Inflammatory bowel disease Sarcoidosis Malignancy Central nervous system https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 30/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Solid tumour outside central nervous system Hematologic Hematologic condition Prothrombotic states, genetic or acquired Protein C deficiency Protein S deficiency Antithrombin deficiency Factor V Leiden mutation G20210A prothrombin gene mutation Antiphospholipid syndrome Myeloproliferative neoplasms Nephrotic syndrome Paroxysmal nocturnal hemoglobinuria Hyperhomocysteinemia Polycythemia, thrombocythemia Severe anemia, including paroxysmal nocturnal hemoglobinuria Central nervous system disorders Dural fistulae Other disorders Congenital heart disease Thyroid disease Graphic 65303 Version 10.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 31/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Small nontraumatic juxtacortical hemorrhage associated with cerebral venous thrombosis A noncontrast head CT scan shows a small juxtacortical hemorrhage, which is a characteristic feature of CVT, particularly with thrombosis of the superior sagittal sinus. CT: computed tomography; CVT: cerebral venous thrombosis. Patr cia Canh o, MD, PhD. Graphic 115378 Version 1.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 32/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Cerebral venous thrombosis on CT imaging (A) Axial head CT image shows hyperdensity in superior sagittal sinus (thick arrow) and adjacent superficial co vein (arrow) consistent with "cord" sign. (B) Coronal head CT image shows dense superior sagittal sinus (thick arrow) consistent with "dense triangle" (C) Sagittal image on CT venography shows long-filling defect (arrows) in superior sagittal sinus. (D) Coronal image on CT venography and magnified inset show "empty-delta" sign (circle). CT: computed tomography. Courtesy of Glenn A Tung, MD, FACR. https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 33/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Graphic 138275 Version 1.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 34/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Signs of cerebral venous thrombosis on head CT scan (A) Noncontrast head CT shows a hyperdense thrombosed cortical vein (arrow). (B) Noncontrast head CT shows a hyperdensity in the torcula (small arrowhead) and the straight sinus (large arrowhead), a direct sign of dural sinus thrombosis (the dense triangle sign). (C) Head CT shows non-filling of the confluent sinus after contrast injection (the empty delta sign). CT: computed tomography. Graphic 59194 Version 4.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 35/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Intracerebral hemorrhage due to cerebral sinus thrombosis Noncontrast head CT showing left frontal ICH (arrow) and vasogenic edema (arrowhead) (A). Mid-sagittal plane on CT angiography (B) and MRV (C) showing filling defect (thick arrows) in superior sagittal sinus (B). Subsequent brain MRI with FLAIR (D) and T2* gradient recall echo (E) imaging showing larger ICH (arrows) and associated edema (arrowheads). ICH: intracerebral hemorrhage; CT: computed tomography; MRV: magnetic resonance venography; MRI: magnetic resonance imaging; FLAIR: fluid-attenuated inversion recovery. Courtesy of Glenn A Tung, MD, FACR. Graphic 132274 Version 1.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 36/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Cerebral venous thrombosis on magnetic resonance imaging (A) Axial post-contrast T1-weighted MRI sequence shows filling defect consistent with acute thrombus in superior sagittal sinus (thick arrow) and superior cerebral veins (arrows). (B) Time-of-flight sequence on MR venogram shows absent venous flow in superior sagittal sinus (arrows). (C) Axial susceptibility-weighted MRI sequence shows thick and hypointense thrombus in superior sagittal sinus (thick arrows) and superior cerebral veins (arrows). (D) Follow-up axial susceptibility-weighted MRI sequence 10 months later shows venous recanalization. MRI: magnetic resonance imaging; MR: magnetic resonance. https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 37/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Courtesy of Glenn A Tung, MD, FACR. Graphic 138276 Version 1.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 38/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Parenchymal brain lesions associated with cerebral venous thrombosis (A) Bilateral thalamic edema in a case of deep cerebral venous system thrombosis, identified in a T2-weighted FLAIR MRI. (B) T2-weighted FLAIR MRI (coronal view) showing left temporal hemorrhagic infarct due to a thrombosis of left lateral sinus (arrow). FLAIR: fluid-attenuated inversion recovery; MRI: magnetic resonance imaging. Graphic 82376 Version 4.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 39/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Etiologies of thunderclap headache Most common causes of thunderclap headache: Subarachnoid hemorrhage Reversible cerebral vasoconstriction syndromes (RCVS) Conditions that less commonly cause thunderclap headache: Cerebral infection (eg, meningitis, acute complicated sinusitis) Cerebral venous thrombosis Cervical artery dissection Spontaneous intracranial hypotension Acute hypertensive crisis Posterior reversible leukoencephalopathy syndrome (PRES) Intracerebral hemorrhage Ischemic stroke Conditions that uncommonly or rarely cause thunderclap headache: Pituitary apoplexy Colloid cyst of the third ventricle Aortic arch dissection Aqueductal stenosis Brain tumor Giant cell arteritis Pheochromocytoma Pneumocephalus Retroclival hematoma Spinal epidural hematoma Varicella zoster virus vasculopathy Vogt-Koyanagi-Harada syndrome Disputed causes of thunderclap headache: Sentinel headache (unruptured intracranial aneurysm)* Primary thunderclap headache Sentinel headache due to an unruptured intracranial aneurysm is a possible cause of thunderclap headache, but supporting data are weak. https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 40/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate There is controversy as to whether thunderclap headache can occur as a benign and potentially recurrent headache disorder in the absence of underlying organic intracranial pathology. Graphic 81710 Version 8.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 41/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Evaluation of thunderclap headache (TCH) https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 42/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 43/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate The evaluation of patients with TCH involves assessing for acute subarachnoid hemorrhage as well as other intracranial and cerebrovascular conditions that may present with these symptoms. TCH: thunderclap headache; CT: computed tomography; SAH: subarachnoid hemorrhage; CTA: computed tomography angiography; MRA: magnetic resonance angiography; RCVS: reversible cerebral vasoconstriction syndrome; ICH: intracerebral hemorrhage; SDH: subdural hematoma; MRV: magnetic resonance venography; CVT: cerebral venous thrombosis; CSF: cerebrospinal fluid; MRI: magnetic resonance imaging; RPLS: reversible posterior leukoencephalopathy syndrome. The sensitivity of head CT to detect SAH is reduced when imaging is obtained >6 hours from onset of TCH, when volume of bleeding is small, and with spinal sources of SAH. In addition to SAH, other causes of TCH that may be identified on neuroimaging include ICH, SDH, ischemic stroke, CVT, spontaneous intracranial hypotension, RPLS, and tumors. Vascular imaging in RCVS may be normal within the first two weeks after symptom onset. Evaluation to exclude other entities and/or repeat imaging may be warranted. Refer to UpToDate topic for additional details. Contraindications to lumbar puncture include cerebral edema/mass effect on head CT, coagulopathy, or suspected epidural spinal abscess. When lumbar pucture is contraindicated, neuroimaging with brain MRI as well as MRA and MRV of the head may be performed as the next step. Refer to UpToDate topics for additional information. If MRI is unavailable or contraindicated, head CT with contrast as well as CTA head/neck and CTV of the head may be performed as alternatives. Graphic 114084 Version 2.0 https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 44/45 7/6/23, 12:26 PM Cerebral venous thrombosis: Etiology, clinical features, and diagnosis - UpToDate Contributor Disclosures Jos M Ferro, MD, PhD Grant/Research/Clinical Trial Support: Bayer [Stroke]; Daiichi-Sankyo [Stroke]. All of the relevant financial relationships listed have been mitigated. Patr cia Canh o, MD, PhD No relevant financial relationship(s) with ineligible companies to disclose. Scott E Kasner, MD Grant/Research/Clinical Trial Support: Bayer [Stroke]; Bristol Meyers Squibb [Stroke]; Medtronic [Stroke]; WL Gore and Associates [Stroke]. Consultant/Advisory Boards: Abbvie [Stroke]; AstraZeneca [Stroke]; BMS [Stroke]; Diamedica [Stroke]; Medtronic [Stroke]. All of the relevant financial relationships listed have been mitigated. Glenn A Tung, MD, FACR No relevant financial relationship(s) with ineligible companies to disclose. Richard P Goddeau, Jr, DO, FAHA No relevant financial relationship(s) with ineligible companies to disclose. Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence. Conflict of interest policy https://www.uptodate.com/contents/cerebral-venous-thrombosis-etiology-clinical-features-and-diagnosis/print 45/45