THEMED ARTICLE y Thrombosis Review

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Diagnosis and treatment of

cerebral venous thrombosis
Expert Rev. Cardiovasc. Ther. 10(12), 1545–1553 (2012)

Christian Weimar*1,2, Dural sinus or cerebral venous thrombosis (CVT) is a frequently unrecognized cause of stroke
Florian Masuhr1,2 and affecting predominantly young women. Typical clinical signs include headache, visual problems
Karim Hajjar1,2 and seizures. Both computed tomography and magnetic resonance venous angiography are
suitable for the detection of CVT, although magnetic resonance angiography is more sensitive
1
Department of Neurology and Stroke
Center, University Duisburg-Essen,
to detect small cortical venous thrombosis. Evidence for efficacy of initial treatment with heparin
Hufelandstr. 55, 45122 Essen, Germany in acute CVT comes from two randomized placebo-controlled studies that together included
2
Department of Neurology, Military 79 patients. Although not evidence-based, postacute treatment with oral anticoagulation is
Hospital Berlin, Scharnhorststr. 13, recommended for up to 12 months after CVT. Long-term anticoagulation is recommended only
10115 Berlin, Germany
*Author for correspondence: in patients suffering from a severe coagulopathy or with recurrent CVT.
Tel.: +49 201 723 6503
Fax: +49 201 723 6948
Keywords: anticoagulation • cerebral hemorrhage • cerebral venous thrombosis • factor Xa inhibitors
christian.weimar@uk-essen.de
• secondary prevention • sinus thrombosis • thrombin inhibitors • venous infarction • warfarin

Epidemiology dysfibrinogenemia, disseminated intravascular

Dural sinus or cerebral venous thrombosis coagulation and heparin-induced thrombocyto-
(CVT) accounts for 0.5–1% of all strokes, penia type 2 [6] . Other common causes include
affecting approximately 3–4 adults/million pregnancy ­(predominantly during the last tri-
and 7 children/million [1,2] . According to the mester or postpartum), malignancies (carcinoma,
International Study on Cerebral Vein and lymphoma, carcinoid and ­leukemia), hematolog-
Dural Sinus Thrombosis, 78% of cases occur in ical ­disorders (polycythemia, sickle cell disease,
patients <50 years of age [3] . During pregnancy, paroxysmal nocturnal hemoglobinuria, immune-
CVT occurs with a frequency of 12/100,000 mediated hemolytic diseases and thrombo-
deliveries. cytemia), ­collagenosis (systemic lupus erythe-
matodes and Sjögren’s syndrome) and vasculitis
Etiology (Behçet’s ­disease, Wegener’s ­granulomatosis and
There are multiple predisposing factors of CVT ­sarcoidosis) [7] .
that do not differ from those causing extra­ Several cases of CVT have been reported after
cerebral venous thrombosis except for a number a lumbar puncture presenting with increased
of local causes. No cause of CVT can be found headache in the supine position [1,3] . Other rare
in approximately 15% of cases, although follow- causes of CVT include intracranial hypo­tension,
up in these patients may detect an underlying cerebral concussion, neurosurgical interventions,
disease up to several months later [4] . Oral hor- obstructive hydrocephalus, impaired venous
monal contraception remains the sole predispos- drainage (central venous catheter, dural arte-
ing factor in approximately 10% but is more fre- riovenous malformation, strangulation), medi-
quently found in combination with coagulation cation poisoning (chemotherapeutics, steroids,
disorders. Coagulation disorders are common erythropoietin, androgens, drugs, vitamin A
causes of CVT, in particular, the Factor V Leiden overdose), metabolic disorders (diabetes, thyro­
mutation with APC resistance that accounts for toxicosis, uremia, nephrotic syndrome), gastro­
10–25% of cases [5] . Further coagulopathies intestinal disorders (liver cirrhosis, chronic
potentially associated with CVT include pro- inflammatory bowel disease) and heart disease
thrombin mutation G 20210 A, antithrombin III (heart failure, cardiomyopathy). Septic CVT
deficiency, protein C and protein S deficiency, can be associated with localized infections, such
antiphospholipid antibody syndrome, plas- as mastoiditis, otitis media, sinusitis, tonsillitis,
minogen deficiency, hyperhomocysteinemia, retropharyngeal abscess with thrombosis of

www.expert-reviews.com 10.1586/ERC.12.126 © 2012 Expert Reviews Ltd ISSN 1477-9072 1545

 Review Weimar, Masuhr & Hajjar

adjacent jugular vein, oral or cerebral abscesses and meningitis. neurological deficits. Papilloedema can be found in approximately
Potential generalized infectious causes include bacterial septi­ 40% of cases, mainly in patients with chronic course or delayed
cemia, endocarditis, hepatitis, encephalitis, measles, tuberculosis, diagnosis. Initial presentation with focal or generalized epilep-
typhus, malaria and aspergillosis. tic seizures occurs in 30–40% of cases. Rare symptoms include
thunderclap headache, subarachnoid hemorrhage, cranial nerve
Clinical presentation palsy, transient ischemic attacks, migraine with aura, psychiatric
Because ways of venous drainage are numerous and reversal of disturbances and tinnitus.
venous flow is possible, thrombosis of a sinus does not necessarily Neurological findings and type of focal seizures are deter-
cause clinical symptoms. Even thrombosis of a major sinus may mined by the localization of CVT and the associated lesions.
result in minor symptoms of isolated intracranial hypertension The common thrombosis of the superior sagittal sinus (60% of
only, such as papilledema and increased opening CSF pressure cases) causes headache, papilloedema, seizures, motor deficits and
[8] . Occlusion of a dural sinus or vein increases venous pressure so impaired consciousness, whereas thrombosis of the cavernosus
that the arterial/venous pressure differential may be inadequate or sinus is associated with ocular nerve palsies and ipsilateral ocu-
marginal in supplying brain tissue drained by the occluded vein. lar affection (chemosis, proptosis, papilloedema). Patients with
This can be local and not cause increased intracranial pressure isolated thrombosis of the lateral sinus present mostly as isolated
(ICP). Consequently, cerebral blood flow drops, giving rise to intracranial hypertension or aphasia if the left transverse sinus
a cytotoxic edema, leading to venous infarction and congestive is occluded. Occlusion of the deep cerebral venous system more
hemorrhage with focal neurological symptoms [1] . CVT can pre- likely causes coma, motor deficits or aphasia. Bilateral venous con-
sent with a wide clinical spectrum mimicking several other disor- gestion and/or ischemia of the thalamic region causes decreased
ders. One-third of CVT patients show an acute symptom onset, alertness as a major finding and presumably causes minor transient
another third present with a subacute beginning (<1 month) and cognitive impairment rather than a severe amnestic syndrome.
the remaining patients have chronic complaints. Headache is the
initial symptom in more than 70% and the predominant symp- Diagnosis
tom in up to 90% [4] . It may be the only symptom or can be Although highly sensitive for detecting even small associated
associated with other common symptoms such as nausea/vomit- hemorrhages (Figure 1) , plain computed tomography (CT) imag-
ing, seizures, reduced consciousness or confusional state and focal ing is not suitable to rule out CVT. Contrast-enhanced imaging
can show indirect signs of a thrombus in a large sinus (‘empty
triangle sign’). A thrombosed cortical vein (‘cord sign’) and the
dense triangle sign indicating a fresh thrombus in the posterior
part of the superior saggital sinus or the sigmoid sinus may be
seen on unenhanced CT (Figure 2) . Imaging of the venous sys-
tem is the key to diagnosis (Figure 3) . Reliable identification of
CVT can be achieved with the enhancement of dynamic venous
CT-angiography using slices of 1–1.5 mm size. With the applica-
tion of multiplanar reconstruction, sensitivity reaches 95% and
specificity 91% compared with digital subtraction angiography
[9] . Like CT imaging, plain MRI is not suitable to definitely rule
out CVT. Instead, the modality of choice is combined MRI and
contrast-enhanced magnetic resonance (MR) venous angiography
(Figure 4) [10] . Although interpretation of MR angiography is more
complex, it allows more advanced conclusions and is superior
in the detection of cortical venous thrombosis compared with
CT angiography. Because the MRI signal changes over time,
different MRI sequences and slice orientations should be com-
bined. Thrombosis of the sinus does not induce signal loss in
the axial and sagittal T1- and T2-sequences but ideally may
appear hyperintense because of its content of methemoglobin.
Depending on the structure and localization of the thrombus,
T1- or T2*-weighted sequences and susceptibility weighted imag-
ing are highly sensitive for direct detection of the thrombus [11,12] .
Thus, besides the thrombosed sinus, even single thrombosed veins
can be detected as hypointense structures [13] . However, these
must be differentiated from localized subarachnoid or subpial
Figure 1. Plain computed tomography scan of hemorrhagic hemorrhages, which may also appear as hypointense on T2*-
venous infarction due to thrombosis of the vein of Labbé. weighted imaging. Subarachnoid hemorrhage localized along a

1546 Expert Rev. Cardiovasc. Ther. 10(12), (2012)

 Diagnosis & treatment of cerebral venous thrombosis Review

sulcus may also be indicative of a single cortical vein thrombosis

[14] . Following contrast enhancement, contrast is spared in the
thrombosed sinus on MRI, like on CT. In a thrombus with a high
methemoglobin level, there may be a hyperintense signal in the
time-of-flight angiography that should not be confounded with a
flow signal. Presence of normal structures in the intracranial dural
sinuses (arachnoid granulations, intrasinus fibrotic bands) should
not be misinterpreted as CVT, which may result in unnecessary
anticoagulation [15,16] . Also, a hypoplastic left lateral sinus is com-
mon and must be differentiated from CVT. With CT and MRI
techniques constantly evolving, conventional digital subtraction
angiography is rarely indicated any more for the detection of CVT
and should only be considered for interventional purposes or in
case CT or MR venography is not available.
Acute CVT presenting with neurological deficits is highly
associated (97%) with d -dimer levels of >500 ng/ml [17] . On
the other hand, d -dimer levels <500 ng/ml do not rule out
CVT, especially when presenting with isolated headache only.
Therefore, although normal d-dimer levels make the presence of
CVT unlikely, exclusion of CVT should not be based on d-dimer
measurement only (Figure 5) .

Treatment
Like in ischemic stroke, treatment of acute CVT requires cardio-
pulmonary monitoring and stroke unit therapy to detect clinical
deterioration and manage complications in time. In line with the Figure 2. Plain computed tomography scan of a dense
therapeutical guidelines for peripheral deep venous thrombosis sigmoid sinus.
(DVT), anticoagulation is also the treatment of choice for CVT.
Initial anticoagulation with heparin is thought to stop the pro-
gression of thrombosis, to reduce the risk of pulmonary embolism
and to prevent re-occlusion of those vessels that have already been
recanalized by internally produced fibrinolytics. Confirmed CVT
should therefore be treated with heparin even in the presence of
an associated intracranial hemorrhage.
Septic or infectious CVT are treated with antibiotics according
to the underlying infectious agent and focus. In certain cases, sur-
gical treatment can be necessary to control the focus of infection.
Despite anti-infective treatment, mortality is higher compared
with noninfectious CVT and treatment with anticoagulation is
justified even in the absence of evidence from controlled trials.

Heparin
The current guidelines for the diagnosis and treatment of CVT
recently published by the American Heart and Stroke Association
as well as the European Federation of Neurological Societies
provide recommendations on anticoagulation for CVT [7,18] .
Evidence for efficacy of treatment with heparin in acute CVT
comes from two randomized placebo-controlled studies that
together included 79 patients. Einhäupl et al. compared dose-
adapted partial thromboplastin time-controlled intravenous
treatment with unfractionated heparin (UFH) versus placebo
in 20 patients with CVT: in the heparin group, eight patients
showed complete restitution, and all patients survived with no
further bleeding complications observed, whereas in the placebo Figure 3. Venous computed tomographic angiography of
group, only one patient showed full recovery, three patients died thrombosis of the right transverse sinus.

www.expert-reviews.com 1547
 Review Weimar, Masuhr & Hajjar

The risk of heparin-induced thrombocytopenia type 2 is

s­ ignificantly lower with LMWH compared with UFH treatment.
Complications including thrombocytopenia and associated vessel
occlusion are uncommon before day 5 and after day 14 of treat-
ment with either LMWH or UFH. Therefore, monitoring of
platelet count is advisable over 2 weeks if treatment lasts 5 days
or longer. Pregnancy is not a contraindication for treatment with
LMWH or UFH, but peri- and post-partal attenuation of anti-
coagulative treatment should be considered individually as risks
of both bleeding and thromboembolism are increased during the
peripartal phase.

Thrombolysis
There are no randomized controlled studies to prove efficacy and
safety of locally applied thrombolysis in CVT [23] . Noncontrolled
case reports have shown high rates of recanalization over long
time intervals – often days – following locally applied urokinase
or rt-PA alone or in combination with thrombectomy, but bleed-
ing complications were more frequent [18] . Particularly, patients
with large space-occupying hemorrhagic infarcts do not benefit
from thrombolytic therapy because an increase in hemorrhage
size accelerates the imminent herniation [24] . Nevertheless, in
patients with thrombosis of the inner cranial veins or extensive
CVT without associated hemorrhage, locally applied thrombo­
lysis may be an option and should be considered as an individual
Figure 4. Contrast-enhanced T1-weighted magnetic treatment attempt after failure of conventional heparin treatment.
resonance image of thrombosis of the right sigmoid sinus. Probably the major indication for local thrombolysis is multiple
sinus occlusions including the jugular veins with no egress of
and two patients developed new intracerebral hemorrhages [19] . blood because these patients have very poor outcomes and mark-
de Bruijn et al. investigated efficacy and safety of treatment edly increased intracranial pressure (ICP). A systematic review
with nadroparin 2 × 90 mg/kg/days for 3 weeks in subcutaneous that included 169 patients treated with local thrombolysis showed
application versus placebo in 59 CVT patients [20] . There was a a possible benefit in patients with severe CVT [25] . Technically,
nonsignificant trend in favor of treatment with low-molecular- thrombolysis is slowly administered using an intra-arterially posi-
weight heparin (LMWH), and neither new ICH nor an increase tioned microcatheter and may be combined with transvenous
in ICH size was observed. thrombectomy if necessary [24,26] . However, the optimal modali-
A meta-analysis of both studies showed a 54% relative risk ties for thrombolytic treatment (dosage, ways of application,
reduction in mortality and severe disability with heparin treat- access and use of heparin) have not been identified yet. Likewise,
ment [21] . Although this meta-analysis was not significant either, mechanical thrombectomy is only supported by case reports but
results of both studies indicate treatment with heparin for CVT may be considered if clinical deterioration occurs despite the use
to be safe and to reduce the risk of unfavorable progression. of anticoagulation [7] . By contrast, intravenous thrombolysis is
It is not known whether intravenous dose-adapted treatment not recommended for the treatment of CVT.
with UFH and weight-adapted treatment with LMWH are
equivalent. A recent nonrandomized prospective observational Elevated ICP
study in patients with CVT found treatment with LMWH to be Although elevated ICP can be found in up to 50% of patients
more effective and associated with fewer bleeding complications with CVT, most patients do not require specific ICP-lowering
[22] . Particularly, patients with hemorrhagic infarctions seemed to treatment. Increased ICP can be managed best with sufficient
benefit from treatment with LMWH. For reasons of practicabil- anticoagulation by optimizing the venous drainage that results
ity, routine application of LMWH treatment for CVT might be in a reduction of ICP. In patients with symptomatic intracranial
preferable because intravenous access or regular blood checks are hypertension and imminent loss of vision, repeated lumbar punc-
not required. The European guidelines for treatment of CVT rec- tures may be needed to drain CSF before anticoagulation can
ommend the use of weight-adapted LMWH [18] . Because coagu- be started. If clinical symptoms progress despite repeated CSF
lation normalizes within 1–2 h after termination of intravenous drainage, permanent CSF drainage may be necessary.
heparinization, UFH may be beneficial for the treatment of criti- Specific ICP-lowering treatment is indicated in fewer than 20%
cally ill patients (ICU patients) or in case surgical interventions of all patients. General rules of ICP-lowering treatment should
are imminent. be applied (upper body elevation, hyperventilation, intravenous

1548 Expert Rev. Cardiovasc. Ther. 10(12), (2012)

 Clinical pathway – Cerebral venous thrombosis

Clinical findings First CVT and Oral

Headache

www.expert-reviews.com
Increased anticoag-
Papilledema intracranial Osmotherapy No major ulation for
Seizures pressure coagulation 3–6 months
Reduced consciousness disorder† (INR 2-3)
Confusional state Intravenous
Risk factors Plain CT: unfractionated
Local or heparin (including
Initial diagnosis generalized in hemorrhagic
CT/MRI with venous cerebral edema transformation):
angiography (CTA/MRA) Atypical
Adjusted dose
D-dimers hemorrhage
Dense triangle sign to increase
Conventional angiography initial aPTT x
in unclear cases only Cord sign
1.5–2 or
Local thrombo-
Etiologic work-up Contrast-enhanced Low-molecular-
lyses (urokina-
History of oral hormonal CT: weight heparin Recurrent CVT
se, rt-PA; After 10–14
contraception, pregnancy Empty triangle sign in full or
Progression particularly in days Long-term
Coagulation disorders anticoagulant despite Major oral
CT-venous multiple sinus
aPTT dose coagulation
angiography: sufficient occlusions anticoagulation
INR Control of disorder†
anticoagulation including the or (INR 2-3)
Thrombin time Venous flow thrombocytes
jugular veins or
Fibrinogen disruption every other day Predisposing
Thrombocytes inner cranial veins
disease
Factor-V Leiden mutation MRI: Antiepileptic
Anti-phospholipid-antibodies MRA: venous flow treatment after
Prothrombin mutation disruption first seizure,
G 20210A Visualization of the exceptionally as
Antithrombin-III thrombus (Met-Hb) primary prevention
Protein-C and -S
Homocystein
Factor VIII
Vasculitis screening
Predisposing diseases/
infections

Figure 5. Clinical pathway for diagnosis and treatment.

†
Antiphospholipid syndrome, homozygous prothrombin G20210A mutation, homozygous Factor V Leiden mutation, Protein-C or -S, or antithrombin deficiency, combined
thrombophilias.
Diagnosis & treatment of cerebral venous thrombosis

aPTT: Activated partial thromboplastin time; CVT: Cerebral venous thrombosis; INR: International normalized ratio.
Reproduced with permission from [41] .
Review

1549
 Review Weimar, Masuhr & Hajjar

administration of osmotherapeutic agents). However, these pro- Prophylaxis with weight-adapted LMWH should also be given
cedures have limited duration of action and are of little effect. in risk situations (e.g., immobilization >4 days, steroid therapy,
One case report showed favorable outcome in six of eight patients flight >4 h), as well as in infants and adolescents with a history
after hemicraniectomy despite clinical and radiological findings of CVT [33] .
of herniation [27] . In particular, patients presenting with large There is little knowledge about recurrent CVT during a new
hemorrhagic infarctions and imminent lateral herniation should pregnancy. One retrospective study found no recurrence of CVT
be offered prompt surgical decompression without removal of during 22 subsequent pregnancies [34] . Results from several case
the hematoma or infarcted area [18] . Anticoagulation should reports also indicate that the risk of recurrence is not increased
be continued within 12–24 h after surgical treatment. Volume [35,36] . Likewise, the risk for a repeat DVT during a new pregnancy
restrictions should be avoided. Because of its prothrombotic is low after a pregnancy-associated peripheral DVT, particularly
activity and no proven benefit, treatment with steroids is not if no inherited or acquired thrombophilia is present. Women
recommended. with either prior extracerebral or CVT who are pregnant or plan
to conceive should be tested for thrombophilia that may help
Antiepileptic medication to reduce the individual risk of recurrence during subsequent
Approximately 40–50 % of patients with CVT develop epileptic pregnancies. Counselling about symptoms suggestive of CVT
seizures leading to acute or persisting clinical deterioration [28,29] . and neurological surveillance during a subsequent pregnancy is
Particularly, patients presenting with a combination of cortical recommended. Anticoagulation with weight-adapted LMWH for
thrombosis, motor deficits and hemorrhagic infarctions are prone 6 weeks after delivery is recommended in patients with a history of
to develop seizures and even epileptic status. In this case, immedi- CVT that is in line with recommendations from the guidelines for
ate antiepileptic treatment is indicated preferably via intravenous prevention of DVT, as the postpartum period carries the greatest
application. Patients presenting with early seizures and hemor- day by day risk of developing DVT and CVT.
rhagic infarctions are at highest risk of developing symptomatic
epilepsy and may benefit from long-term antiepileptic treatment Expert commentary
(12 months). In all other patients, antiepileptic treatment should With the help of MR and CT angiography, CVT is increasingly
be terminated after a seizure-free period of 3–6 months. In con- diagnosed in oligosymptomatic patients, that is, with headache
trast to the high incidence of seizure activity during the acute or papilledema only [37] . Therefore and presumably due to anti-
phase, the risk to develop residual epilepsy following CVT is coagulation in the acute phase, the rate of death or dependancy
relatively small. In the absence of evidence to support preventive has declined from approximately 50% 30 years ago to approxi-
antiepileptic treatment in CVT, prophylactic treatment can be mately 15% in recent case series [2] . Heparin treatment remains
considered individually [29] . the baseline therapy including in those patients with concomitant
intracerebral hemorrhage. Endovascular treatment has only been
Oral anticoagulation evaluated in small case series and should be restricted to those
There is no clear evidence on the optimal duration of long-term patients with clinical deterioration and radiological progression of
anticoagulation. MR-based follow-up studies indicate that reca- cerebral venous congestion. Oral anticoagulation with vitamin K
nalization occurs within 3 months irrespective of continuation antagonists is not indicated beyond 12 months except in patients
of oral anticoagulation [30] . However, the recanalization rate has with severe coagulopathies or recurrent CVT. Whether the new
not been found to correlate with the risk of CVT recurrence. oral anticoagulants provide an alternative in the postacute and
Despite the lack of evidence, several guidelines recommend possibly the acute phase is still unknown.
an average duration of 3–12 months for treatment with dose-
adapted vitamin K antagonists, whereas long-term anticoagula- Five-year view
tion beyond 12 months is advised only in patients with severe The most promising new therapeutic options are direct factor Xa
coagulopathies or recurrent CVT [7,31] . In addition, children inhibitors and thrombin inhibitors. Rivaroxaban is currently the
and adolescents below the age of 18 years who have heterozygote only newer anticoagulant that has been approved for the treat-
prothrombin G20210A mutation may benefit from long-term ment of DVT, whereas both dabigatran and rivaroxaban have
­a nticoagulation [32] . been approved for the long-term prevention of recurrent VTE.
To date, there is not enough clinical experience and evidence In patients with acute DVT without pulmonary embolism (PE),
for the use of new anticoagulants (dabigatran, rivaroxaban and rivaroxaban was noninferior for prevention of recurrent VTE to
apixaban) for the treatment of CVT, although rivaroxaban has the standard of care (enoxaparin plus a vitamin K antagonist),
been approved for the treatment of acute DVT. and extended-duration rivaroxaban had superior efficacy to pla-
cebo in patients with confirmed DVT or PE who had received
Secondary prevention in patients at risk 6–12 months of prior VTE treatment [38] . Likewise, dabigatran
Recommendations for CVT prevention in adults are in accord- had similar efficacy compared with warfarin in the prevention of
ance with the current guidelines for the prevention of DVT in recurrent events after acute VTE [39] .
patients at high risk of venous thromboembolism (VTE) – that Current guidelines recommend anticoagulation with rivaroxa-
is, treatment with anticoagulation in risk situations. ban or dabigatran as a second-line therapy for extended treatment

1550 Expert Rev. Cardiovasc. Ther. 10(12), (2012)

 Diagnosis & treatment of cerebral venous thrombosis Review

after the acute phase of DVT or PE [40] . Although clinical expe- Boehringer Ingelheim, Bristol-Myers Squibb, CoAxia, D-Pharm,
rience in patients with CVT is still lacking, the known advan- Daiichi Asubio, GlaxoSmithKline, Lundbeck, Medtronic, MindFrame,
tages over vitamin K antagonists will probably propagate the Neurobiological Technologies, Novartis, Novo-Nordisk and Sanofi-Aventis.
use of the new oral anticoagulants in patients with CVT. In C Weimar has no ownership interest and does not own stocks of any phar-
addition, improved endovascular techniques for thrombectomy maceutical company exceeding €10,000. F Masuhr received honoraria
are increasingly applied to the cerebral venous system and may for oral presentations from: Bayer Pharma, Boehringer Ingelheim, Daiichi
become a proven alternative in patients with progressive throm- Sankyo and Sanofi-Aventis. F Masuhr has no ownership interest and does
bosis despite anticoagulation or in case of contraindications for not own stocks of any pharmaceutical company exceeding €10,000. The
anticoagulation. authors have no other relevant affiliations or financial involvement with
any organization or entity with a financial interest in or financial conflict
Financial & competing interests disclosure with the subject matter or materials discussed in the manuscript apart
C Weimar has received honoraria for participation in clinical t­ rials, con- from those disclosed.
tribution to advisory boards or oral presentations from: Bayer Pharma, No writing assistance was utilized in the production of this manuscript.

Key issues
• Headache is the initial symptom in more than 70% and the predominant symptom in up to 90% of patients with cerebral venous
thrombosis (CVT).
• Initial presentation with focal or generalized epileptic seizures can be found in 30–40% of cases.
• There are no pathognomonic symptoms for CVT. Therefore, immediate cerebral imaging including angiography is required if CVT is
suspected.
• Both CT and MR venous angiography are suitable for the detection of CVT, while MR angiography is more sensitive to detect small
cortical venous thrombosis.
• Although normal d-dimer levels make the presence of CVT unlikely, exclusion of CVT should not be based on d-dimer measurement
only.
• Confirmed CVT should be treated with intravenous or low-molecular-weight heparin even in the presence of intracranial hemorrhage.
• Immediate anticoagulation is thought to stop the progression of thrombosis and prevent vessels from closing up again that already
have been recanalized by internally produced fibrinolytics.
• Following the acute phase, treatment with oral anticoagulation is recommended for up to 12 months after CVT.
• Long-term anticoagulation is recommended only in patients suffering from a severe coagulopathy or recurrent CVT.
• To this day, there is not enough clinical experience nor evidence for the use of new anticoagulants (dabigatran, rivaroxaban and
apixaban) for the treatment of CVT.

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 Diagnosis & treatment of cerebral venous thrombosis Review

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