Cerebral venous thrombosis: Etiology, clinical

features, and diagnosis

Authors:
José M Ferro, MD, PhD
Patrícia Canhão, MD, PhD
Section Editor:
Scott E Kasner, MD
Deputy Editor:
John F Dashe, MD, PhD
Contributor Disclosures
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Feb 2019. | This topic last updated: Aug 31, 2018.

INTRODUCTION Cerebral vein and dural sinus thrombosis (CVT) is less

common than most other types of stroke but can be more challenging to diagnose. Due
to the widespread use of MRI and rising clinical awareness, CVT is recognized with
increasing frequency. In addition, it is now known to have a more varied clinical
spectrum than previously realized. Because of its myriad causes and presentations,
CVT is a disease that may be encountered not only by neurologists and neurosurgeons,
but also by emergency clinicians, internists, oncologists, hematologists, obstetricians,
pediatricians, and family practitioners.

This topic will review the epidemiology, pathogenesis, clinical features, and diagnosis of
CVT. Prognosis and treatment are discussed separately. (See "Cerebral venous
thrombosis: Treatment and prognosis".)

CVT in newborns is also reviewed elsewhere. (See "Stroke in the newborn:

Classification, manifestations, and diagnosis", section on 'Cerebral sinovenous
thrombosis'.)

EPIDEMIOLOGY The available data suggest that CVT is uncommon [1]. The

annual incidence ranges from 0.22 to 1.57 per 100,000 [2-4], and is more common in
women than men, with a female to male ratio of 3:1 [5,6]. The imbalance may be due to
the increased risk of CVT associated with pregnancy and puerperium and with oral
contraceptives [7]. (See 'Acquired thrombophilia' below.)

In adults, CVT affects patients who are younger on average than those with arterial
types of stroke. In the International Study on Cerebral Vein and Dural Sinus Thrombosis
(ISCVT), the median age of patients with CVT was 37 years [5], and only 8 percent of
the patients were older than 65 [8]. Compared with men, women were significantly
younger (median age 34 years, versus 42 years for men) [6].

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There is a low risk of recurrent CVT and venous thromboembolism after a first CVT, as
reviewed separately. (See "Cerebral venous thrombosis: Treatment and prognosis",
section on 'Recurrence'.)

PATHOGENESIS The pathogenesis of CVT remains incompletely

understood because of the high variability in the anatomy of the venous system, and the
paucity of experiments in animal models of CVT. However, there are at least two
different mechanisms that may contribute to the clinical features of CVT (figure 1) [9]:
●Thrombosis of cerebral veins or dural sinus obstructs blood drainage from brain
tissue, leading to cerebral parenchymal lesions (eg, stroke) or dysfunction, and to
increased venous and capillary pressure with disruption of the blood-brain barrier.
●Occlusion of dural sinus resulting in decreased cerebrospinal fluid (CSF)
absorption and elevated intracranial pressure.

Obstruction of the venous structures (figure 2) results in increased venous pressure,

decreased capillary perfusion pressure, and increased cerebral blood volume. Dilatation
of cerebral veins and recruitment of collateral pathways play an important role in the
early phases of CVT and may initially compensate for changes in pressure.

The increase in venous and capillary pressure leads to blood-brain barrier disruption,
causing vasogenic edema, with leakage of blood plasma into the interstitial space. As
intravenous pressure continues to increase, localized cerebral edema and venous
hemorrhage may occur due to venous or capillary rupture. The increased intravenous
pressure may lead to an increase in intravascular pressure and a lowering of cerebral
perfusion pressure, resulting in decreased cerebral blood flow (CBF) and failure of
energy metabolism. In turn, this allows intracellular entry of water from failure of
the Na+/K+ ATPase pump, and consequent cytotoxic edema [10]. Venous infarction
and hemorrhage may be confluent (ie, venous hemorrhagic infarction).

Advances in understanding the pathophysiology of venous occlusion have been aided

by the use of MRI methods, mainly diffusion-weighted MRI and perfusion-weighted MRI
[11-14]. These techniques have demonstrated the coexistence of both cytotoxic and
vasogenic edema in patients with CVT [11,13-15].

The other effect of venous thrombosis is impairment of CSF absorption. Normally, CSF
absorption occurs in the arachnoid granulations, which drain CSF into the superior
sagittal sinus. Thrombosis of the dural sinuses leads to increased venous pressure,
impaired CSF absorption, and consequently elevated intracranial pressure. Elevated
intracranial pressure is more frequent if superior sagittal sinus thrombosis is present,
but it may also occur with thrombosis of the jugular sinus or the lateral sinus. Note that
the lateral sinus consists of two segments; the proximal segment is termed the
transverse sinus, and the distal segment is termed the sigmoid sinus (figure 2).

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 RISK FACTORS AND CAUSES Many conditions are associated with

CVT. The major risk factors for CVT in adults can be grouped as transient or permanent
(table 1). The most frequent risk factors for CVT are [1,5]:
●Prothrombotic conditions, either genetic or acquired
●Oral contraceptives
●Pregnancy and the puerperium
●Malignancy
●Infection
●Head injury and mechanical precipitants

In more than 85 percent of adult patients, at least one risk factor for CVT can be
identified, most often a prothrombotic condition [5]. In the Canadian pediatric ischemic
stroke registry, a risk factor was identified in 98 percent of the children [16]. A
prothrombotic state was found in 41 percent. In infants older than four weeks of age and
in children, head and neck disorders, mostly infections and chronic systemic diseases
(eg, connective tissue disease, hematologic disorder, and cancer) were common. The
most common risk factors in those ≥65 years old are genetic or acquired thrombophilia,
malignancy, and hematologic disorders such as polycythemia [8,17].

Acquired thrombophilia — The most common acquired thrombophilias are pregnancy

and the puerperium, the use of oral contraceptives, and malignancy [18,19].
(See "Cerebrovascular disorders complicating pregnancy" and "Risks and side effects
associated with combined estrogen-progestin oral contraceptives" and "Risk and
prevention of venous thromboembolism in adults with cancer".)

In the prospective International Study on Cerebral Vein and Dural Sinus Thrombosis
(ISCVT) cohort of 624 adults with CVT, women comprised 75 percent [6]. Furthermore,
a gender-specific risk factor (ie, oral contraceptives, pregnancy, puerperium, and
hormone replacement therapy) was identified in 65 percent of women. In an earlier
report of the ISCVT cohort, a prothrombotic condition was found in 34 percent of all
patients, and a genetic prothrombotic condition was found in 22 percent of all patients
[5].

The most frequent risk factor for CVT in young women is the use of oral contraceptives
[20,21]. Furthermore, the risk for CVT in women using oral contraceptives is increased if
they have a prothrombotic defect and if they are obese [21,22].

Genetic thrombophilia — The risk for CVT is influenced by the individual's genetic
background [23]. In the presence of some prothrombotic conditions, patients are at an
increased risk of developing a CVT when exposed to a precipitant such a head trauma,
lumbar puncture, jugular catheter placement, pregnancy, surgery, infection, and drugs.
These prothrombotic conditions include the following:
●Antithrombin deficiency [24,25]
●Protein C deficiency or protein S deficiency [16,26,27]
●Factor V Leiden mutation [20,28,29]

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 ●G20210 A prothrombin gene mutation [20,29-31]
●Hyperhomocysteinemia [32]

In a meta-analysis of case-control studies, with over 200 neonatal and pediatric cases
of sinovenous thrombosis (ie, CVT), and 1200 control subjects, the prevalence of factor
V Leiden (FVL) mutation among cases and controls was 12.8 and 3.6 percent,
respectively, and carriers of the FVL mutation were significantly more likely to develop
CVT (odds ratio [OR] 3.1, 95% CI 1.8-5.5) [33]. Similarly, the prevalence of the
prothrombin gene mutation among cases and controls was 5.2 and 2.5 percent,
respective, and carriers were significantly more likely to develop CVT (OR 3.1, 95% CI
1.4-6.8).

The association of CVT with hyperhomocysteinemia due to gene mutations in

methylene tetrahydrofolate reductase (MTHFR) is controversial [23,34,35].

A 2010 meta-analysis of case-control studies found that the frequency of the MTHFR
677C>T polymorphism in adults was similar for 382 patients with CVT compared with
1217 controls (15.7 versus 14.6 percent; OR 1.12, 95% CI 0.8-1.58), suggesting that
the MTHFR 677C>T polymorphism is not a risk factor for CVT [36]. In contrast, a 2011
meta-analysis, after controlling for heterogeneity among studies, found that the MTHFR
677C>T polymorphism was associated with CVT (OR 2.30, 95% CI 1.20-4.42) [23].

There is no association of CVT with PAI-1 or protein Z polymorphisms.

Other causes — Although infectious causes of CVT were frequently reported in the
past, they are responsible for only 6 to 12 percent of cases in modern-era studies of
adults with CVT [5,18]. Local infections (eg, involving the ears, sinuses, mouth, face, or
neck) are typically responsible, although systemic infection is sometimes the only
cause. Head injury and mechanical precipitants are less common causes of CVT [37-
39].

Inflammatory diseases can also cause CVT, including systemic lupus erythematosus,
Behçet disease, granulomatosis with polyangiitis (Wegener), thromboangiitis obliterans,
inflammatory bowel disease, and sarcoidosis.

As with venous thrombosis in other parts of the body, multiple risk factors may be found
in about half of adult patients with CVT [5]. In light of this, a thorough search for
additional causes should be carried out even when a specific risk factor is identified in a
given patient. (See "Overview of the causes of venous thrombosis".)

Note that some chemotherapeutic agents for the management of associated cancer
have a procoagulant effect and may even cause CVT (eg, L-asparaginase). (See "Drug-
induced thrombosis in patients with malignancy".)

No identified cause — No underlying etiology or risk factor for CVT is found in a

minority of children (≤10 percent) and adults (13 percent) with CVT [8,16,40]. In older
adult CVT patients, the proportion of cases without identified risk factors is higher (37
percent) than it is in adults under age 65 (10 percent) [8].

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 CLINICAL ASPECTS Cerebral vein and dural sinus thrombosis has a highly

variable clinical presentation [41,42]. The onset can be acute, subacute, or chronic.
CVT most often presents with new headache or as a syndrome of isolated intracranial
hypertension. Additional manifestations include focal neurologic deficits,
seizures, and/or encephalopathy.

Symptoms and signs — Symptoms and signs of CVT can be grouped into three major
syndromes:
●Isolated intracranial hypertension syndrome (headache with or without vomiting,
papilledema, and visual problems) [43]
●Focal syndrome (focal deficits, seizures, or both)
●Encephalopathy (multifocal signs, mental status changes, stupor, or coma) [2,41]

Less common presentations include cavernous sinus syndrome, subarachnoid

hemorrhage, and multiple cranial nerve palsies. A case of CVT mimicking a transient
ischemic attack has also been reported [44].

The clinical symptoms and signs in CVT depend upon several factors, including patient
age and sex, the site and number of occluded sinuses and veins, the presence of
parenchymal brain lesions, and the interval from CVT onset to presentation. In children,
signs of diffuse brain injury, coma, and seizures are the main clinical manifestations,
especially in neonates [16]. In older children, the manifestations of CVT resemble those
in adults, with headache and hemiparesis [45]. Women are more likely than men to
have a headache on presentation, and less likely to have a chronic onset of symptoms
[6]. Older adults may also have a distinctive presentation; depressed consciousness
and mental status changes are more common while headaches and isolated intracranial
hypertension are less frequent than in younger patients [8].

Cerebral edema, venous infarction, and hemorrhagic venous infarction are associated
with a more severe syndrome; patients are more likely to be comatose or to have motor
deficits, aphasia, and seizures, and less likely to present with isolated headache.

Headache — Headache is the most frequent symptom of CVT. In the International

Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT) cohort, headache was
present in 89 percent of patients [5]. Headaches associated with CVT are more frequent
in women and young patients than in men or older adults [46]. Headache is usually the
first symptom of CVT, and can be the only symptom [47], or can precede other
symptoms and signs by days or weeks [48].

The features of CVT-related headache are quite variable. Head pain may be localized
or diffuse [48]. Headache caused by intracranial hypertension from CVT is typically
characterized by severe head pain that worsens with Valsalva maneuvers and with
recumbency.

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The site of the headache has no relationship with the localization of the occluded sinus
or the parenchymal lesions [49,50]. Headache onset with CVT is usually gradual,
increasing over several days [7]. However, some patients with CVT have sudden
explosive onset of severe head pain (ie, thunderclap headache) that mimics
subarachnoid hemorrhage [51,52]. (See "Approach to the patient with thunderclap
headache" and "Clinical manifestations and diagnosis of aneurysmal subarachnoid
hemorrhage".)

Headache due to CVT may also resemble migraine with aura [53-55].
(See "Pathophysiology, clinical manifestations, and diagnosis of migraine in
adults" and "Pathophysiology, clinical features, and diagnosis of migraine in children".)

CVT must be included as a possible cause of persisting headache following lumbar

puncture, because lumbar puncture can rarely precipitate a CVT. (See "Post dural
puncture headache".)

Isolated intracranial hypertension syndrome — Isolated intracranial hypertension

syndrome (ie, headache associated with papilledema or visual problems) accounts for a
significant proportion of CVT cases [43]. Visual obscurations may occur, coinciding with
bouts of increased headache intensity.

Isolated intracranial hypertension is more frequent in patients with a chronic

presentation than in those who present acutely [56]. In addition, patients with chronic
course or delayed clinical presentation may show papilledema on funduscopy, a finding
that is less frequent in acute cases. (See "Overview and differential diagnosis of
papilledema".)

Seizures — Focal or generalized seizures, including status epilepticus, are more

frequent in CVT than in other cerebrovascular disorders. In the ISCVT cohort of 624
patients, seizures at presentation occurred in 39 percent, and seizures after the
diagnosis of CVT occurred in 7 percent [57]. In a retrospective cohort of 70 children
(including 25 neonates) with CVT, seizures at presentation occurred in 20 of 45 non-
neonates (44 percent) [58]. Variables associated with seizures include supratentorial
parenchymal brain lesions, sagittal sinus and cortical vein thrombosis, and motor
deficits [57].

Encephalopathy — Severe cases of CVT can cause disturbances of consciousness

and cognitive dysfunction, such as delirium, apathy, a frontal lobe syndrome, multifocal
deficits, or seizures.

Focal syndrome — Weakness with monoparesis or hemiparesis, sometimes bilateral,

is the most frequent focal deficit associated with CVT. In the ISCVT cohort, motor
weakness was present in 37 percent of patients [5]. Aphasia, in particular of the fluent
type, may follow sinus thrombosis, especially when the left lateral sinus is affected.
Sensory deficits and visual field defects are less common.

Isolated sinus and vein thrombosis — Isolated thrombosis of the different sinuses
and veins produces diverse clinical pictures.

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 ●In cavernous sinus thrombosis, ocular signs dominate the clinical picture with
orbital pain, chemosis, proptosis, and oculomotor palsies [59-62].
●Isolated cortical vein occlusion produces motor/sensory deficits and seizures [63-
65].
●With sagittal sinus occlusion, motor deficits, bilateral deficits, and seizures are
frequent, while presentation as an isolated intracranial hypertension syndrome is
infrequent.
●Patients with isolated lateral sinus thrombosis frequently present with isolated
headache or isolated intracranial hypertension [66]. Less often, they may also
present with focal deficits or seizures. Aphasia often follows if the left transverse
sinus is occluded.
●Jugular vein or lateral sinus thrombosis may present as isolated pulsating tinnitus
[67,68].
●Multiple cranial nerve palsies may occur in thrombosis of the lateral sinus [69],
jugular, or posterior fossa veins thrombosis.
●When the deep cerebral venous system (ie, the straight sinus and its branches) is
occluded, the signs and symptoms of CVT are generally severe, with coma or other
alterations in mental status and motor deficits, often bilateral [70-72]. However,
more limited thrombosis of the deep venous system can produce relatively mild
symptoms [73].

Neuroimaging — The neuroimaging features of CVT can include focal areas of edema
or venous infarction, hemorrhagic venous infarction, diffuse brain edema, or (rarely)
isolated subarachnoid hemorrhage [1]. In patients with CVT, the proportion who present
with intracerebral hemorrhage is 30 to 40 percent [74,75]. Small nontraumatic
juxtacortical hemorrhages (image 1), which are located just below the cortex in the
white matter and have a diameter of <2 cm, account for up to one-fourth of intracerebral
hemorrhages in patients with CVT and are associated with superior sagittal sinus
occlusion [76].

In a minority of cases, CT may demonstrate direct signs of CVT, which include the
dense triangle sign, the empty delta sign, and the cord sign, described below
(see 'CT' below). Brain MRI in combination with magnetic resonance (MR) venography
is the most informative technique for demonstrating the presence of dural thrombus,
cortical vein thrombosis, and extent of brain injury. The imaging findings of CVT are
discussed in greater detail below. (See 'Urgent imaging' below.)

DIAGNOSIS In patients with clinically suspected CVT (eg, presenting with new

headache, isolated intracranial hypertension syndrome, focal neurologic deficits,

seizures, and/or encephalopathy), urgent neuroimaging is necessary as the first step in
the diagnostic evaluation. Aside from neuroimaging, there is no simple confirmatory
laboratory test that can confidently rule out CVT in the acute phase of the disease.

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Diagnostic approach — The diagnosis of CVT should be suspected in patients who
present with one or more of the following:
●New onset headache
●Headache with features that differ from the usual pattern (eg, progression or
change in attack frequency, severity, or clinical features) in patients with a previous
primary headache
●Symptoms or signs of intracranial hypertension
●Encephalopathy
●Focal neurologic symptoms and signs, especially those not fitting a specific
vascular distribution or those involving multiple vascular territories
●Seizures

In addition, the diagnosis of CVT should be suspected in patients who have atypical
neuroimaging features on routine CT or MRI at presentation, such as cerebral infarction
that crosses typical arterial boundaries, hemorrhagic infarction, or lobar intracerebral
hemorrhage of otherwise unclear origin [1]. In any of these scenarios, suspicion for CVT
should be particularly high for patients with known risk factors, including prothrombotic
conditions, oral contraceptive use, pregnancy and the puerperium, malignancy,
infection, and head injury, even if the initial neuroimaging study (most often a CT) is
normal.

Urgent imaging — For patients with any presentation raising concern for CVT, we
recommend urgent neuroimaging with brain MRI and magnetic resonance (MR)
venography, or with cranial CT with CT venography if MRI is not an option [1]. The clear
demonstration of absence of flow and intraluminal venous thrombus by CT or MRI is the
most important finding for confirming the diagnosis. However, these findings are not
always evident, and the diagnosis may rest on imaging features demonstrated by MR
venography or CT venography showing only absence of flow in a venous sinus or
cortical vein.

A number of normal anatomic variants may mimic sinus thrombosis, including sinus
atresia, sinus hypoplasia, asymmetric sinus drainage, and normal sinus filling defects
associated with arachnoid granulations or intrasinus septa [1]. For example, a study of
100 subjects (without CVT) with normal brain MRI found artifactual transverse sinus
flow gaps on MR venography (in nondominant or codominant but not in dominant
transverse sinuses) in 31 percent [77]. Another report of 100 subjects without venous
pathology found asymmetric lateral sinuses in 49 percent and partial or total absence of
one lateral sinus in 20 percent [78].

CT — Head CT is normal in up to 30 percent of CVT cases, and most of the findings are
nonspecific [41]. However, CT is often the first investigation to be performed in clinical
practice, and it is useful to rule out other acute or subacute cerebral disorders.

In about one-third of cases, CT demonstrates direct signs of CVT, which are as follows
(image 2) [41,79-81]:

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 ●The dense triangle sign, seen on noncontrast head CT as a hyperdensity with a
triangular or round shape in the posterior part of the superior sagittal sinus caused
by the venous thrombus
●The empty delta sign (also called the empty triangle or negative delta sign), seen
on head CT with contrast as a triangular pattern of contrast enhancement
surrounding a central region lacking contrast enhancement in the posterior part of
the superior sagittal sinus
●The cord sign, usually seen on head CT with contrast as a curvilinear or linear
hyperdensity over the cerebral cortex caused by a thrombosed cortical vein

Indirect signs of CVT on head CT are more frequent. These can include intense
contrast enhancement of falx and tentorium, dilated transcerebral veins, small
ventricles, and parenchyma abnormalities. In addition, associated brain lesions may be
depicted in 60 to 80 percent of patients with CVT. These may be hemorrhagic or
nonhemorrhagic:

●Hemorrhagic lesions include intracerebral hemorrhage, hemorrhagic infarcts, or

rarely (<1 percent) subarachnoid hemorrhage usually limited to the convexity [82-
84].
●Nonhemorrhagic lesions include focal areas of hypodensity caused by edema or
venous infarction, usually not respecting the arterial boundaries, as well as diffuse
brain edema. With serial imaging, some lesions may disappear ("vanishing
infarcts"), and new lesions may appear.

CT venography — Head CT is often normal in patients with CVT, and MRI techniques
for confirming the diagnosis are not readily available in some hospitals and geographic
locations. In this situation, CT venography is a useful alternative to MR venography or
intra-arterial angiography for the diagnosis of CVT, demonstrating filling defects, sinus
wall enhancement, and increased collateral venous drainage [85,86]. When combined
with head CT, it adds considerable information in suspected cases of CVT [87]. The
overall accuracy of head CT combined with CT venography is 90 to 100 percent,
depending on the occlusion site [88]. Guidelines from the American
Heart Association/American Stroke Association (AHA/ASA) published in 2011 consider
CT venography to be at least equivalent to MR venography in the diagnosis of CVT [1].
Compared with digital subtraction intra-arterial angiography, the combination of head
CT and CT venography has a sensitivity and specificity of 95 and 91 percent [87].

CT venography gives a good visualization of the major dural sinuses [89,90], is readily
available, and is quicker than MRI. It can be used for patients who have
contraindications to MRI (eg, pacemaker) [1]. CT venography is often particularly helpful
in subacute or chronic CVT because it can demonstrate heterogeneous density in
thrombosed venous sinuses. However, its use may be limited because of low resolution
of the deep venous system and cortical veins, the risk of contrast reactions, and
radiation exposure [88,91,92].

MRI — MRI using gradient echo T2* susceptibility-weighted sequences in combination

with MR venography is the most sensitive imaging method for demonstrating the

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thrombus and the occluded dural sinus or vein (image 3) [15,91,93-96]. The
characteristics of the MRI signal depend on the age of the thrombus [97,98]:

●In the first five days, the thrombosed sinuses appear isointense on T1-weighted
images and hypointense on T2-weighted images
●Beyond five days, venous thrombus becomes more apparent because signal is
increased on both T1- and T2-weighted images
●After the first month, thrombosed sinuses exhibit a variable pattern of signal, which
may appear isointense

On gradient echo T2*-weighted MRI sequences, the clot can be directly visualized as an
area of hypointensity in the affected cortical vein and/or sinus [65,93,99]. However, a
chronically thrombosed sinus may still demonstrate low signal on these sequences.
Limited data from a series of 28 patients with CVT suggest that the presence of
hyperintensities in the veins or sinuses on diffusion-weighted MRI sequences predicts a
low recanalization rate [100].

Parenchymal brain lesions secondary to venous occlusion, including brain swelling,

edema, or venous infarction, appear as hypointense or isointense on T1-weighted MRI,
and hyperintense on T2-weighted MRI (image 4). Hemorrhagic venous infarcts appear
as hyperintense lesions on both T1 and T2 MRI sequences (image 5).

Agreement between observers for the diagnosis of CVT with MRI varies with the
location of sinus or vein thrombosis. It is good or very good for most of the occluded
sinus and veins; moderate to very good for the left lateral sinus and straight sinus; and
poor to good for the cortical veins [101]. The diagnosis of isolated cortical vein
thrombosis remains difficult to establish with MR venography. Use of T2*-weighted MRI
may enable a diagnosis of isolated cortical vein thrombosis by demonstrating clot as an
area of hypointensity [65,93,99].

MR venography — MR venography, usually performed using the time-of-flight (TOF)

technique, is useful for demonstrating absence of flow in cerebral venous sinuses,
though interpretation can be confounded by normal anatomic variants such as sinus
hypoplasia and asymmetric flow (see 'Diagnosis' above) [1]. Other MR techniques may
be useful to distinguish these variants from venous thrombosis. Contrast-enhanced MR
venography can provide better visualization of cerebral venous channels, and gradient
echo or susceptibility-weighted sequences will show normal signal in a hypoplastic
sinus and abnormally low signal in the presence of thrombus. A chronically thrombosed
hypoplastic sinus will show absence of flow on two-dimensional TOF MR venography
and enhancement on contrast-enhanced MRI and MR venography.

Conventional angiography — Cerebral intraarterial angiography is recommended

mainly when the diagnosis of CVT is uncertain, such as in the rare suspected cases of
isolated cortical vein thrombosis, or when the clinical suspicion for CVT is high but CT
venography or MR venography are inconclusive [1]. Angiography may be helpful for
making this diagnosis by showing the sudden termination of a cortical vein surrounded
by dilated and tortuous collateral "corkscrew veins," or by the filling of a cortical vein that
was not apparent on an earlier angiographic study during the acute phase of CVT.

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Other typical signs of CVT on intraarterial angiography are nonvisualization of all or part
of a venous sinus, delayed venous emptying with pathologically increased collaterals,
and reversal of venous flow.

As with MR and CT venography, conventional cerebral angiography may be limited by

potential pitfalls. Anatomic variations, such as variability of number and location of
cortical veins, hypoplasia of the anterior part of the superior sagittal sinus, duplication of
the superior sagittal sinus, and hypoplasia or aplasia of the transverse sinuses, may
make the diagnosis of CVT by all types of angiography difficult [41]. While the
interobserver agreement for a diagnosis of CVT is not perfect, the combination of
conventional contrast angiography plus brain MRI has a higher interobserver agreement
than angiography alone (94 versus 62 percent) [102].

Laboratory tests — Aside from neuroimaging, there is no simple confirmatory

laboratory test that can confidently rule out CVT in the acute phase of the disease.
Guidelines from the American Heart Association/American Stroke
Association (AHA/ASA)recommend obtaining routine blood studies consisting of a
complete blood count, chemistry panel, prothrombin time, and activated partial
thromboplastin time for patients with suspected CVT [1]. The findings from these tests
may suggest the presence of conditions that contribute to the development of CVT such
as an underlying hypercoagulable state, infection, or inflammatory process. The
guidelines recommend screening for these and other potential prothrombotic conditions
that may predispose to CVT, including use of contraceptives, at the initial clinical
presentation.

The utility of D-dimer testing and lumbar puncture is reviewed in the following sections.

D-dimer — An elevated plasma D-dimer level supports the diagnosis of CVT, but a
normal D-dimer does not exclude the diagnosis in patients with suggestive symptoms
and predisposing factors.

The potential utility of D-dimer for the diagnosis of CVT is illustrated by the following
observations

●A 2012 meta-analysis included 14 studies that evaluated D-dimer in 1134 patients

for the diagnosis of suspected or confirmed CVT [103]. In seven studies that
evaluated patients with suspected CVT, D-dimer was elevated in 145 of 155
patients in whom CVT was confirmed, and was normal in 692 of 771 patients in
whom CVT was ruled out, yielding a sensitivity and specificity of 94 and 90 percent,
respectively. D-dimer performed less well in seven studies that enrolled subjects
with already confirmed CVT; the sensitivity and specificity were 89 and 83 percent,
respectively. The sensitivity of D-dimer for CVT was also lower in patients with
isolated headache as the presenting symptom (82 percent), in those with subacute
or chronic clinical presentations of CVT (83 percent), and in those with a single
affected venous sinus (84 percent).
●In a subsequent study of 233 patients with suspected CVT and symptom onset of
less than seven days, D-dimer demonstrated a sensitivity and specificity of 94 and
98 percent, respectively, for predicting CVT [104].

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Thus, D-dimer measurement may have some value as a diagnostic screening tool for
the assessment of patients with possible CVT. However, a normal D-dimer value cannot
exclude CVT, especially in patients with isolated headache or with thrombosis of a
single sinus. Individual assays used to measure D-dimer vary, but it is reasonable to
use the same threshold levels as used in diagnostic protocols for deep venous
thrombosis (eg, D-dimer >500 ng/mL of fibrinogen equivalent units). (See "Clinical
presentation and diagnosis of the nonpregnant adult with suspected deep vein
thrombosis of the lower extremity", section on 'D-dimer'.)

Lumbar puncture — Lumbar puncture may be useful to exclude meningitis in patients

with CVT who present with isolated intracranial hypertension, a syndrome that may
account for up to 25 percent of all patients with CVT [5]. In addition, lumbar puncture is
valuable in such patients to measure and decrease cerebrospinal fluid pressure when
vision is threatened. However, in the absence of suspicion for meningitis, cerebrospinal
fluid analysis is usually not helpful diagnostically for patients with focal neurologic
findings and neuroimaging confirmation of CVT [1].

The cerebrospinal fluid abnormalities in CVT are nonspecific and may include a
lymphocytic pleocytosis, elevated red blood cell count, and elevated protein; these
abnormalities are present in 30 to 50 percent of patients with CVT [42,43].

Performing a lumbar puncture is not harmful in patients with CVT, as suggested by the
findings of a study that analyzed 624 patients with CVT and identified 224 who had
lumbar puncture [105]. The groups with and without lumbar puncture did not differ on
any of the outcome measures, which were neurologic worsening within 30 days of CVT
onset, acute death, complete recovery at six months, or death or dependency at six
months. Nevertheless, lumbar puncture is contraindicated in patients with large brain
lesions because they have an increased risk of herniation.

Evaluation for thrombophilic state — Searching for a thrombophilic state, either

genetic or acquired, should be done for patients with CVT who have a high pretest
probability of severe thrombophilia, a category that includes those with a
personal and/or family history of venous thrombosis, CVT at a young age, and CVT in
the absence of a transient or permanent risk factor (table 1) [106]. When appropriate,
screening should include:

●Antithrombin
●Protein C
●Protein S
●Factor V Leiden
●Prothrombin G20210A mutation
●Lupus anticoagulant, anticardiolipin, and anti-beta2 glycoprotein-I antibodies

Acute thrombosis can transiently reduce levels of antithrombin, protein C, and protein S,
so the utility of testing for these disorders in the acute phase of CVT is limited. In
practice, it is preferable to test for protein C, protein S, and antithrombin at least two
weeks after oral anticoagulation has been discontinued, since warfarin therapy reduces
measurements of protein C and protein S, and may raise plasma antithrombin

12
concentrations into the normal range in patients with hereditary antithrombin deficiency.
It is possible to test for protein C and protein S levels while receiving heparin therapy,
which does not alter plasma protein C or protein S concentrations. However, testing for
antithrombin should be performed when off heparin, which can lower antithrombin
levels. (See "Antithrombin deficiency" and "Protein C deficiency" and "Protein S
deficiency".)

No underlying etiology or risk factor for CVT is found in approximately 13 percent of

adult patients. However, it is important to continue searching for a cause even after the
acute phase of CVT, as some patients may have a condition such as the
antiphospholipid syndrome, polycythemia, thrombocythemia, malignancy, or
inflammatory bowel disease that is discovered weeks or months after the acute phase.
(See 'Risk factors and causes' above.)

If abnormal results are found in assays for lupus anticoagulant, anticardiolipin, or anti-
beta2 glycoprotein-I antibodies, testing should be repeated at least 12 weeks later, as
the diagnosis of antiphospholipid syndrome requires two positive determinations of
these biomarkers. (See "Diagnosis of antiphospholipid syndrome", section on
'Antiphospholipid antibody testing'.)

An evaluation for paroxysmal nocturnal hemoglobinuria should be pursued if the

complete blood count shows unexplained hemolytic anemia, iron deficiency, or
pancytopenia. (See "Clinical manifestations and diagnosis of paroxysmal nocturnal
hemoglobinuria", section on 'Diagnostic evaluation'.)

In patients older than 40 years without identified etiology, we suggest searching for an
occult malignancy. In patients with sepsis, or with fever and no obvious cause of
infection, we recommend performing a lumbar puncture.

DIFFERENTIAL DIAGNOSIS The clinical presentation of CVT can be

nonspecific (eg, headache, seizure, encephalopathy) and the cause may not be
apparent on initial routine neuroimaging studies.
●For patients presenting with symptoms and signs of isolated intracranial
hypertension syndrome (headache with or without vomiting, papilledema, and
visual problems), the main considerations in the differential are idiopathic
intracranial hypertension (pseudotumor cerebri) and meningitis. Other conditions
associated with elevated intracranial pressure, (eg intracranial mass lesions from
tumor or abscess), are usually apparent on neuroimaging with CT or MRI. If
neuroimaging reveals no structural intracranial lesion responsible for intracranial
hypertension, a lumbar puncture is indicated with measurement of opening
pressure and cerebrospinal fluid for analysis. (See "Idiopathic intracranial
hypertension (pseudotumor cerebri): Clinical features and diagnosis".)
●For patients presenting with a focal neurologic syndrome (eg, focal deficits,
seizures, or both) the differential is broad and includes other vascular etiologies

13
 (eg, intracerebral hemorrhage from a variety of other causes, subdural
hemorrhage, ischemic stroke), infection (eg, meningitis, abscess), and tumor.
●For patients presenting with encephalopathy (eg, multifocal signs, mental status
changes, stupor, or coma) the differential includes infection (eg, bacterial and viral
meningoencephalitis), inflammation (eg, paraneoplastic and autoimmune
encephalitis), demyelination (eg, acute disseminated encephalomyelitis,
neuromyelitis optica spectrum disorders) and toxic and metabolic disturbances.
●For patients presenting with thunderclap headache, which is a rare in CVT the
differential (table 2) includes subarachnoid hemorrhage, other types of intracranial
hemorrhage, reversible cerebral vasoconstriction syndromes (RCVS), cervical
artery dissection, viral and bacterial meningitis, acute complicated sinusitis,
spontaneous intracranial hypotension, ischemic stroke, acute hypertensive crisis,
third ventricular colloid cyst, and pituitary apoplexy. If initial neuroimaging is
nondiagnostic, patients with thunderclap headache should have lumbar puncture
with measurement of opening pressure and cerebrospinal fluid analysis to exclude
subarachnoid hemorrhage and meningitis (algorithm 1). If lumbar puncture is also
nondiagnostic, imaging of the cerebral circulation is necessary, preferably with
magnetic resonance (MR) angiography/venography. (See "Clinical manifestations
and diagnosis of aneurysmal subarachnoid hemorrhage" and "Reversible cerebral
vasoconstriction syndromes" and "Approach to the patient with thunderclap
headache".)
●For women with any of the presentations listed above during pregnancy or
puerperium, additional considerations include preeclampsia and eclampsia
presenting with ischemic stroke, intracerebral hemorrhage, or RCVS.
(See "Cerebrovascular disorders complicating pregnancy".)

SOCIETY GUIDELINE LINKS Links to society and government-sponsored

guidelines from selected countries and regions around the world are provided
separately. (See "Society guideline links: Stroke in adults" and "Society guideline links:
Stroke in children".)

SUMMARY AND RECOMMENDATIONS

●Cerebral venous thrombosis (CVT) is uncommon, with an estimated incidence of

<1.5 per 100,000 annually. Among young adults, CVT is more common in women
than men. The mean age of onset is 39 years old. (See 'Epidemiology' above.)
●The major risk factors for CVT in adults (table 1) are prothrombotic
(hypercoagulable) conditions , oral contraceptives, pregnancy and the puerperium,
malignancy, infection, head injury, and mechanical precipitants. (See 'Risk factors
and causes' above.)
●The clinical presentation of CVT is highly variable. The onset can be acute,
subacute, or chronic. Headache (of gradual, acute, or thunderclap onset) is the
most frequent symptom, occurring in almost 90 percent of patients, and may occur
as part of an isolated intracranial hypertension syndrome, with or without vomiting,
14
 papilledema, and visual problems. In other cases, headache may be accompanied
by focal neurologic deficits, focal or generalized seizures, and encephalopathy with
altered mental status or coma. (See 'Clinical aspects' above.)
●Parenchymal brain lesions, including brain swelling, edema, venous infarction, or
hemorrhagic venous infarction, may occur secondary to venous occlusion.
(See 'Neuroimaging' above.)
●The combination of an abnormal signal in a venous sinus on brain MRI and the
corresponding absence of flow on magnetic resonance (MR) venography confirms
the diagnosis of CVT. However, these findings are not always evident, and the
diagnosis may rest on imaging features showing only absence of flow in a venous
sinus or cortical vein. Other than neuroimaging, there is no simple confirmatory
laboratory test that can confidently rule out CVT in the acute phase of the disease.
(See 'Diagnosis' above.)
●Head CT scan is normal in up to 30 percent of CVT cases, and most of the
findings with CVT are nonspecific. However, in about one-third of patients, CT
demonstrates direct signs of CVT, which are the empty delta sign, the cord sign,
and the dense triangle sign. CT venography is a useful alternative to MR
venography. (See 'CT' above and 'CT venography' above.)
●Brain MRI in combination with MR venography is the most informative technique
for demonstrating the presence of dural thrombus, cortical vein thrombosis, and
extent of brain injury. (See 'MRI' above and 'MR venography' above.)
●Screening for thrombophilia should be done for patients with CVT who have a high
pretest probability for severe thrombophilia, a category that includes those with a
personal and/or family history of venous thrombosis, CVT at a young age, and CVT
in the absence of a transient or permanent risk factor (table 1). (See 'Evaluation for
thrombophilic state'above.)
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