Cerebral Venous Thrombosis
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Thrombosis of the venous channels in the brain is an uncommon cause of cerebral infarction relative to arterial disease, but it is an important consideration because of its potential morbidity. (See Prognosis.)
Knowledge of the anatomy of the venous system is essential in evaluating patients with cerebral venous thrombosis (CVT), since symptoms associated with the condition are related to the area of thrombosis. For example, cerebral infarction may occur with cortical vein or sagittal sinus thrombosis secondary to tissue congestion with obstruction. (See Presentation.)
Lateral sinus thrombosis may be associated with headache and a pseudotumor cerebri–like picture. Extension into the jugular bulb may cause jugular foramen syndrome, while cranial nerve palsies may be seen in cavernous sinus thrombosis as a compressive phenomenon. Cerebral hemorrhage also may be a presenting feature in patients with venous sinus thrombosis. (See Presentation.)
Imaging procedures have led to easier recognition of venous sinus thrombosis (see the images below), offering the opportunity for early therapeutic measures. (See Workup.)
The following guidelines for CVT have been provided by the American Heart Association and the American Stroke Association [1] :
In patients with suspected CVT, routine blood studies consisting of a complete blood count, chemistry panel, prothrombin time, and activated partial thromboplastin time should be performed.
Screening for potential prothrombotic conditions that may predispose a person to CVT (eg, use of contraceptives, underlying inflammatory disease, infectious process) is recommended in the initial clinical assessment.
Testing for prothrombotic conditions (including protein C, protein S, or antithrombin deficiency), antiphospholipid syndrome, prothrombin G20210A mutation, and factor V Leiden can be beneficial for the management of patients with CVT. Testing for protein C, protein S, and antithrombin deficiency is generally indicated 2-4 weeks after completion of anticoagulation. There is a very limited value of testing in the acute setting or in patients taking warfarin.
In patients with provoked CVT (associated with a transient risk factor), vitamin K antagonists may be continued for 3-6 months, with a target international normalized ratio of 2.0-3.0.
In patients with unprovoked CVT, vitamin K antagonists may be continued for 6-12 months, with a target international normalized ratio of 2.0-3.0.
For patients with recurrent CVT, venous thromboembolism (VTE) after CVT, or first CVT with severe thrombophilia (ie, homozygous prothrombin G20210A; homozygous factor V Leiden; deficiencies of protein C, protein S, or antithrombin; combined thrombophilia defects; or antiphospholipid syndrome), indefinite anticoagulation may be considered, with a target international normalized ratio of 2.0-3.0.
For women with CVT during pregnancy, low-molecular-weight heparin (LMWH) in full anticoagulant doses should be continued throughout pregnancy, and LMWH or vitamin K antagonist with a target international normalized ratio of 2.0-3.0 should be continued for ≥6 weeks postpartum (for a total minimum duration of therapy of 6 months).
It is reasonable to advise women with a history of CVT that future pregnancy is not contraindicated. Further investigations regarding the underlying cause and a formal consultation with a hematologist or maternal fetal medicine specialist are reasonable.
It is reasonable to treat acute CVT during pregnancy with full-dose LMWH rather than unfractionated heparin.
For women with a history of CVT, prophylaxis with LMWH during future pregnancies and the postpartum period is reasonable.
Many causative conditions have been described in cerebral venous thrombosis (CVT). These may be seen alone or in combination. For example, a prothrombin gene mutation in association with oral contraceptive use raises the odds ratio for developing CVT.
Infection may occur by extension from the paranasal sinuses. These cases also may be associated with subdural empyema. Bacterial meningitis as a coexistent condition should be considered in these cases. Frontal sinuses are the most common source of infection, with spread through the emissary veins between the posterior sinus mucosa and the meninges. Rarely, sphenoid sinusitis may be associated with cavernous sinus thrombosis. Multiple organisms are to be considered, Staphylococcus aureus being the most common. In chronic infections, gram-negative organisms and fungi such as Aspergillus species may be found.
Trauma may also be an etiologic event. Cerebral sinus thrombosis easily may be overlooked in cases of minor head trauma. Neurosurgical procedures such as dural taps and infusions into the internal jugular vein have been implicated as well.
Many medical conditions have been associated with CVT. For example, hypercoagulable states associated with the antiphospholipid syndrome, protein S and C deficiencies, antithrombin III deficiency, lupus anticoagulant, and the Leiden factor V mutation may result in CVT. Antibodies against the fibrinolytic receptor, annexin A2 (titer >3 standard deviations), are significantly associated with CVT. [2] Pregnancy also is associated with a hypercoagulable tendency. Malignancies may be associated with hypercoagulable states as well, and therefore may be risk factors.
Isolated cortical venous thrombosis has been associated with intracranial hypotension syndrome, but only rarely. In a study, Schievink and Maya found that CVT was present in only 3 (2.1%) out of 141 patients with spontaneous intracranial hypotension. [3]
A few cases of CVT have been reported after lumbar puncture (LP), suggesting a causal association. In a study by Canhao et al, LP induced a sustained decrease in mean blood flow velocity (BFV) in the straight sinus (SS), suggesting that the decrease in venous blood flow is a possible mechanism contributing to the occurrence of CVT. In the study, the investigators used transcranial Doppler ultrasonography to register the mean BFV of the SS before, during, and after LP. LP induced a decrease of 47% in mean BFV in the SS, with the mean decrease being significant immediately at the end, 30 min after, and more than 6 hours after LP. [4]
Several medications are reported to increase the risk of CVT, including the following:
Oral contraceptives – Including the third-generation formulations
Corticosteroids
Epsilon-aminocaproic acid
Thalidomide
Tamoxifen
Erythropoietin
Phytoestrogens
L-asparaginase
Heparin – Heparin therapy has been reported to produce thrombotic thrombocytopenia with associated venous sinus thrombosis
Other diseases that have been described as risk factors for CVT include the following:
Inflammatory bowel diseases, such as Crohn disease and ulcerative colitis, are described as risk factors for venous thrombosis [5] ; corticosteroids used in treatment of these conditions may play a causative role
Pregnancy and puerperium are important considerations in women of childbearing age
Hematologic conditions, including paroxysmal nocturnal hemoglobinuria, thrombotic thrombocytopenic purpura, sickle cell disease, and polycythemia, are to be considered
Collagen-vascular diseases, such as systemic lupus erythematosus, Wegener granulomatosis, and Behçet syndrome, have been reported to be associated with CVT
Hyperhomocysteinemia is a strong and independent risk factor for CVT, being present in 27-43% of patients with CVT but in only 8-10% of the general population; whether treatment with folate, pyridoxine, and/or cobalamin reduces the risk of CVT is unclear
Dehydration
Spontaneous intracranial hypotension
High altitude
Hepatic cirrhosis
The incidence of cerebral venous thrombosis (CVT) is difficult to determine, but generally, it is believed to be an uncommon cause of stroke, with the reported ratio of venous to arterial strokes being 1:62.5. In 1973, Towbin reported CVT in 9% of 182 autopsies, [6] while in 1995, Daif reported a frequency in Saudi Arabia of 7 cases per 100,000 hospital patients. [7]
However, with the advent of newer imaging techniques, the reported incidence of CVT is likely to increase as less severe cases are found.
CVT is believed to be more common in women than in men. In a series of 110 cases, Ameri and Bousser found a female-to-male ratio of 1.29:1. [8]
In 1992, Ameri and Bousser reported a uniform age distribution in men with CVT, while 61% of women with CVT were aged 20-35 years. [8] This difference may be related to pregnancy or the use of oral contraceptives. [9]
Smith demonstrated the efficacy of anticoagulant and thrombolytic therapy in patients with cerebral venous thrombosis (CVT). In his study, he compared outcomes of patients who were treated with heparin and local infusion of urokinase (12 patients) with those of patients who received no treatment (21 patients). [10] The results appear in the Table, below.
Table. Patients With Cerebral Venous Thrombosis Treated With Heparin and Local Infusion of Urokinase vs Nontreated Group (Open Table in a new window)
Treated Group, % (n = 12)
Nontreated Group, % (n = 21)
Full recovery
62.5
29
Mild disability
12.5
13
Severe disability
12.5
9.6
Fatal outcome
12.5
48
Herniation attributable to unilateral mass effect is the major cause of death in CVT. In CVT patients with large parenchymal lesions causing herniation, decompressive surgery has been lifesaving and often results in good functional outcome, even in patients with severe clinical conditions. [11]
Mortality in untreated cases of venous thrombosis has been reported to range from 13.8-48%; this high mortality rate may be a reflection of clinical severity at entrance into the study. Between 25% and 30% of patients have full recovery.
In a Portuguese study that prospectively analyzed 91 patients with CVT over a mean 1-year follow-up interval, the majority of patients experienced complete recovery. [12] Of the patients analyzed, 7% died in the acute phase, 1% died during the one year follow-up, 82% recovered completely, and 1% were dependent; 59% developed thrombotic events during the follow-up, 10% had seizures, 11% complained of severe headaches, and 1 patient experienced severe visual loss.
In 2003, Buccino et al found a good overall outcome in their reinvestigation of a series of 34 patients with confirmed CVT. [13] However, 10 patients (30%) had episodic headaches, 3 patients (8.8%) had seizures, 4 patients (11.7%) had pyramidal signs, and 2 (5.9%) had visual deficits. Mild nonfluent aphasia was seen in 3 patients. Working memory deficit and depression of mood were seen in 6 patients (17.6%).
[Guideline] Bushnell C, McCullough LD, Awad IA, Chireau MV, et al. Guidelines for the prevention of stroke in women: a statement for healthcare professionals from the American Heart Association/American Stroke Association. National Guideline Clearinghouse. Available at http://guideline.gov/content.aspx?id=47871&search=cerebral+venous+thrombosis. Accessed: November 21, 2014.
Cesarman-Maus G, Cantú-Brito C, Barinagarrementeria F, Villa R, Reyes E, Sanchez-Guerrero J. Autoantibodies against the fibrinolytic receptor, annexin A2, in cerebral venous thrombosis. Stroke. 2011 Feb. 42(2):501-3. [Medline].
Schievink WI, Maya MM. Cerebral venous thrombosis in spontaneous intracranial hypotension. Headache. 2008 Nov-Dec. 48(10):1511-9. [Medline]. [Full Text].
Canhão P, Batista P, Falcão F. Lumbar puncture and dural sinus thrombosis–a causal or casual association?. Cerebrovasc Dis. 2005. 19(1):53-6. [Medline].
Ennaifer R, Moussa A, Mouelhi L, et al. Cerebral venous sinus thrombosis as presenting feature of ulcerative colitis. Acta Gastroenterol Belg. 2009 Jul-Sep. 72(3):350-3. [Medline].
Towbin A. The syndrome of latent cerebral venous thrombosis: its frequency and relation to age and congestive heart failure. Stroke. 1973 May-Jun. 4(3):419-30. [Medline].
Daif A, Awada A, al-Rajeh S, et al. Cerebral venous thrombosis in adults. A study of 40 cases from Saudi Arabia. Stroke. 1995 Jul. 26(7):1193-5. [Medline].
Ameri A, Bousser MG. Cerebral venous thrombosis. Neurol Clin. 1992 Feb. 10(1):87-111. [Medline].
Galarza M, Gazzeri R. Cerebral venous sinus thrombosis associated with oral contraceptives: the case for neurosurgery. Neurosurg Focus. 2009 Nov. 27(5):E5. [Medline].
Smith AG, Cornblath WT, Deveikis JP. Local thrombolytic therapy in deep cerebral venous thrombosis. Neurology. 1997 Jun. 48(6):1613-9. [Medline].
Ferro JM, Crassard I, Coutinho JM, Canhão P, Barinagarrementeria F, Cucchiara B. Decompressive surgery in cerebrovenous thrombosis: a multicenter registry and a systematic review of individual patient data. Stroke. 2011 Oct. 42(10):2825-31. [Medline].
Ferro JM, Lopes MG, Rosas MJ, et al. Long-Term Prognosis of Cerebral Vein and Dural Sinus Thrombosis. results of the venoport study. Cerebrovasc Dis. 2002. 13(4):272-8. [Medline].
Buccino G, Scoditti U, Patteri I, et al. Neurological and cognitive long-term outcome in patients with cerebral venous sinus thrombosis. Acta Neurol Scand. 2003 May. 107(5):330-5. [Medline].
Flores-Barragan JM, Hernandez-Gonzalez A, Gallardo-Alcaniz MJ, Del Real-Francia MA, Vaamonde-Gamo J. [Clinical and therapeutic heterogeneity of cerebral venous thrombosis: a description of a series of 20 cases.]. Rev Neurol. 2009 Dec 1-15. 49(11):573-6. [Medline].
Oppenheim C, Domigo V, Gauvrit JY, et al. Subarachnoid hemorrhage as the initial presentation of dural sinus thrombosis. AJNR Am J Neuroradiol. 2005 Mar. 26(3):614-7. [Medline].
Farb RI, Vanek I, Scott JN, et al. Idiopathic intracranial hypertension: the prevalence and morphology of sinovenous stenosis. Neurology. 2003 May 13. 60(9):1418-24. [Medline].
Wasay M, Kojan S, Dai AI, Bobustuc G, Sheikh Z. Headache in Cerebral Venous Thrombosis: incidence, pattern and location in 200 consecutive patients. J Headache Pain. 2010 Apr. 11(2):137-9. [Medline].
Tardy B, Tardy-Poncet B, Viallon A, et al. D-dimer levels in patients with suspected acute cerebral venous thrombosis. Am J Med. 2002 Aug 15. 113(3):238-41. [Medline].
Lalive PH, de Moerloose P, Lovblad K, et al. Is measurement of D-dimer useful in the diagnosis of cerebral venous thrombosis?. Neurology. 2003 Oct 28. 61(8):1057-60. [Medline].
Kosinski CM, Mull M, Schwarz M, et al. Do normal D-dimer levels reliably exclude cerebral sinus thrombosis?. Stroke. 2004 Dec. 35(12):2820-5. [Medline].
Ozsvath RR, Casey SO, Lustrin ES, et al. Cerebral venography: comparison of CT and MR projection venography. AJR Am J Roentgenol. 1997 Dec. 169(6):1699-707. [Medline].
Mas JL, Meder JF, Meary E, Bousser MG. Magnetic resonance imaging in lateral sinus hypoplasia and thrombosis. Stroke. 1990 Sep. 21(9):1350-6. [Medline].
Adams WM, Laitt RD, Beards SC, et al. Use of single-slice thick slab phase-contrast angiography for the diagnosis of dural venous sinus thrombosis. Eur Radiol. 1999. 9(8):1614-9. [Medline].
Ayanzen RH, Bird CR, Keller PJ, et al. Cerebral MR venography: normal anatomy and potential diagnostic pitfalls. AJNR Am J Neuroradiol. 2000 Jan. 21(1):74-8. [Medline].
Medel R, Monteith SJ, Crowley RW, Dumont AS. A review of therapeutic strategies for the management of cerebral venous sinus thrombosis. Neurosurg Focus. 2009 Nov. 27(5):E6. [Medline].
Bentley JN, Figueroa RE, Vender JR. From presentation to follow-up: diagnosis and treatment of cerebral venous thrombosis. Neurosurg Focus. 2009 Nov. 27(5):E4. [Medline].
Einhaupl KM, Villringer A, Meister W, et al. Heparin treatment in sinus venous thrombosis. Lancet. 1991 Sep 7. 338(8767):597-600. [Medline].
de Bruijn SF, Stam J, Vandenbroucke JP. Increased risk of cerebral venous sinus thrombosis with third- generation oral contraceptives. Cerebral Venous Sinus Thrombosis Study Group. Lancet. 1998 May 9. 351(9113):1404. [Medline].
Rahman M, Velat GJ, Hoh BL, Mocco J. Direct thrombolysis for cerebral venous sinus thrombosis. Neurosurg Focus. 2009 Nov. 27(5):E7. [Medline].
Ekseth K, Bostrom S, Vegfors M. Reversibility of severe sagittal sinus thrombosis with open surgical thrombectomy combined with local infusion of tissue plasminogen activator: technical case report. Neurosurgery. 1998 Oct. 43(4):960-5. [Medline].
Ferro JM, Bousser MG, Canhão P, Coutinho JM, Crassard I, Dentali F, et al. European Stroke Organization guideline for the diagnosis and treatment of cerebral venous thrombosis – endorsed by the European Academy of Neurology. Eur J Neurol. 2017 Oct. 24 (10):1203-1213. [Medline].
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Treated Group, % (n = 12)
Nontreated Group, % (n = 21)
Full recovery
62.5
29
Mild disability
12.5
13
Severe disability
12.5
9.6
Fatal outcome
12.5
48
W Alvin McElveen, MD Director, Stroke Unit, Lakewood Ranch Medical Center; Neurologist, Manatee Memorial Hospital
W Alvin McElveen, MD is a member of the following medical societies: American Academy of Neurology, Southern Clinical Neurological Society, American Stroke Association, American Medical Association, American Society of Neuroimaging
Disclosure: Nothing to disclose.
Andrew P Keegan, MD Private Practice, Bradenton Neurology, Inc; Consulting Staff, Department of Neurology, Manatee Memorial Hospital, Lakewood Ranch Medical Center, Blake Medical Center
Andrew P Keegan, MD is a member of the following medical societies: American Academy of Neurology, American Medical Association
Disclosure: Nothing to disclose.
Helmi L Lutsep, MD Professor and Vice Chair, Department of Neurology, Oregon Health and Science University School of Medicine; Associate Director, OHSU Stroke Center
Helmi L Lutsep, MD is a member of the following medical societies: American Academy of Neurology, American Stroke Association
Disclosure: Medscape Neurology Editorial Advisory Board for: Stroke Adjudication Committee, CREST2; Executive Committee for the NINDS-funded DEFUSE3 Trial; Physician Advisory Board for Coherex Medical.
Ralph F Gonzalez, MD Private Practice, Bradenton Neurology, Inc; Consulting Staff, Department of Neurology, Blake Hospital, Lakewood Ranch Medical Center, Manatee Memorial Hospital
Ralph F Gonzalez, MD is a member of the following medical societies: American Academy of Neurology and Florida Medical Association
Disclosure: Nothing to disclose.
Howard S Kirshner, MD Professor of Neurology, Psychiatry and Hearing and Speech Sciences, Vice Chairman, Department of Neurology, Vanderbilt University School of Medicine; Director, Vanderbilt Stroke Center; Program Director, Stroke Service, Vanderbilt Stallworth Rehabilitation Hospital; Consulting Staff, Department of Neurology, Nashville Veterans Affairs Medical Center
Howard S Kirshner, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Neurology, American Heart Association, American Medical Association, American Neurological Association, American Society of Neurorehabilitation, National Stroke Association, Phi Beta Kappa, and Tennessee Medical Association
Disclosure: Nothing to disclose.
Norman C Reynolds Jr, MD Neurologist, Veterans Affairs Medical Center of Milwaukee; Clinical Professor, Medical College of Wisconsin
Norman C Reynolds Jr, MD is a member of the following medical societies: American Academy of Neurology, Association of Military Surgeons of the US, Movement Disorders Society, Sigma Xi, and Society for Neuroscience
Disclosure: Nothing to disclose.
Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference
Disclosure: Medscape Salary Employment
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