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 2018. | This topic last updated: Jan 09, 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 mean age of patients with CVT was 39 years [5], and only 8 percent of the patients were
older than 65 [8].
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 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, mild parenchymal changes, severe 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].

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).
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 estrogen-progestin 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]. Compared with
men, women were significantly younger (mean age 34 years, versus 42 years for men).
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]
●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]. Head injury and mechanical precipitants are less common causes of CVT [37-
39].

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".)
No identified cause — No underlying etiology or risk factor for CVT is found in
approximately 13 percent of adult patients. 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
[8].

CLINICAL ASPECTS — Cerebral vein and dural sinus thrombosis has a highly variable
clinical presentation [40,41]. 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 in three major
syndromes:

●Isolated intracranial hypertension syndrome (headache with or without vomiting,

papilledema, and visual problems) [42]
●Focal syndrome (focal deficits, seizures, or both)
●Encephalopathy (multifocal signs, mental status changes, stupor, or coma) [2,40]

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 [43].

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 [44]. 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; vigilance and mental problems 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 [45]. Headache is usually the first symptom of
CVT, and can be the only symptom [46], or can precede other symptoms and signs by days
or weeks [47].

The features of CVT-related headache are quite variable. Head pain is more often localized
than diffuse [47]. However, the site of the headache has no relationship with the localization
of the occluded sinus or the parenchymal lesions [48,49]. 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 [50,51]. (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 [52-54].
(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-lumbar
puncture headache".)

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

in CVT than in other stroke types. 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 [55]. In a retrospective cohort of 70 children (including 25 neonates) with CVT,
seizures at presentation occurred in 20 of 45 non-neonates (44 percent) [56]. Variables
associated with seizures include supratentorial parenchymal brain lesions, sagittal sinus
and cortical vein thrombosis, and motor deficits [55].

Isolated intracranial hypertension syndrome — Isolated intracranial hypertension

syndrome (ie, headache associated with papilledema or visual problems) accounts for a
significant proportion of CVT cases [42]. Headache caused by intracranial hypertension
from CVT is typically characterized by severe, dull, generalized head pain that worsens with
Valsalva maneuvers and with recumbency. 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 [57]. 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".)
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.

●In cavernous sinus thrombosis, ocular signs dominate the clinical picture with orbital
pain, chemosis, proptosis, and oculomotor palsies [58-61].
●Isolated cortical vein occlusion produces motor/sensory deficits and seizures [62-64].
●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 [65]. 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
[66,67].
●Multiple cranial nerve palsies may occur in thrombosis of the lateral sinus [68],
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, mental
problems, and motor deficits, often bilateral [69-71]. However, more limited thrombosis
of the deep venous system can produce relatively mild symptoms [72].

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 [73,74]. 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-half of intracerebral hemorrhages in
patients with CVT and are associated with superior sagittal sinus occlusion [75].
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.

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 [76]. 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 [64,77,78].

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 [79,80]. When combined with head
CT, it adds considerable information in suspected cases of CVT [81]. The overall accuracy
of head CT combined with CT venography is 90 to 100 percent, depending on the occlusion
site [82]. 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 [81].

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

MR venography is the most sensitive imaging method for demonstrating the thrombus and
the occluded dural sinus or vein (image 2) [15,77,83-86]. The characteristics of the MRI
signal depend on the age of the thrombus [87,88]:

●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 [64,77,78]. 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
[89].

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 3). Hemorrhagic venous infarcts appear as
hyperintense lesions on both MRI sequences (image 4).

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' below) [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.

CT — Head CT is normal in up to 30 percent of CVT cases, and most of the findings are
nonspecific [40]. 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 5) [40,90-92]:

●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 [93-95].
●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 — CT venography gives a good visualization of the major dural sinuses

[96,97], 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
[82,83,98].

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.

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.
For patients with any presentation raising concern for CVT, we recommend urgent
neuroimaging with brain MRI and magnetic resonance (MR) venography (or 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.

In such cases, 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 [99]. 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 [100].

Aside from neuroimaging, there is no simple confirmatory laboratory test that can
confidently rule out CVT in the acute phase of the disease. 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. Lumbar puncture may be
useful to exclude meningitis in patients with CVT who present with isolated intracranial
hypertension if neuroimaging is unrevealing.

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. 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 [40]. 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) [101].

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 [102]. 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 [103].

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 [41,42].

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 [104]. 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) [105]. 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 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 (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, pituitary apoplexy, and unruptured intracranial aneurysms. 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, 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.)
●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.)
●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.)
●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.)
Cerebral venous thrombosis: Treatment and prognosis
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: Mar 2018. | This topic last updated: Jan 19, 2018.

INTRODUCTION — Cerebral venous thrombosis (CVT) is an uncommon but serious

disorder. Clinical manifestations can include headache, papilledema, visual loss, focal or
generalized seizures, focal neurologic deficits, confusion, altered consciousness, and coma.

Many cases have been linked to inherited and acquired thrombophilias, pregnancy,
puerperium, infection, and malignancy. Infarctions due to CVT are often hemorrhagic and
associated with vasogenic edema. (See "Cerebral venous thrombosis: Etiology, clinical
features, and diagnosis".)

Treatment, which is started as soon as the diagnosis is confirmed, consists of reversing the
underlying cause when known, control of seizures and intracranial hypertension, and
antithrombotic therapy. Anticoagulation is the mainstay of acute and subacute treatment for
CVT.

This topic will review the prognosis and treatment of CVT. Other aspects of this disorder are
discussed separately. (See "Cerebral venous thrombosis: Etiology, clinical features, and
diagnosis".)

ACUTE ANTITHROMBOTIC TREATMENT — While the overall aim of treatment for CVT is

to improve outcome, the immediate goals treatment for CVT are [1-4]:

●To recanalize the occluded sinus/vein

●To prevent the propagation of the thrombus, namely to the bridging cerebral veins
●To treat the underlying prothrombotic state, in order to prevent venous thrombosis in
other parts of the body, particularly pulmonary embolism, and to prevent the recurrence
of CVT
The main treatment option to achieve these goals is anticoagulation, using either heparin or
low molecular weight heparin (LMWH), as discussed in the next section.

Early anticoagulation — Based upon available published data and guidelines, we

recommend anticoagulation with subcutaneous LMWH or intravenous heparin for adults
with symptomatic CVT who have no contraindication. The presence of hemorrhagic venous
infarction is not a contraindication for anticoagulant treatment in CVT. The evidence
summarized below (see 'Efficacy' below) suggests that subcutaneous LMWH is more
effective than unfractionated heparin (UFH), and is at least as safe. Therefore, we prefer
subcutaneous LMWH unless the patient is clinically unstable, or invasive interventions such
as lumbar puncture or surgery are planned, or there is a contraindication to LMWH, such as
renal failure.

Treatment for children during the acute phase of CVT is similar to that for adults, but the
evidence is weaker since there are no randomized controlled trials in this age group.

The duration of antithrombotic treatment is reviewed below. (See 'Long-term

anticoagulation' below.)

Efficacy — Although definitive evidence of effectiveness is lacking, there is a general

consensus that anticoagulation with UFH or LMWH is appropriate treatment for acute CVT
[5]. As an example, more than 80 percent of the patients in the International Study on
Cerebral Vein and Dural Sinus Thrombosis (ISCVT) were treated with anticoagulation [6].

Two randomized controlled trials of anticoagulation in acute CVT (table 1) have been
published [3,7]. Both have methodologic problems, most importantly their modest sample
size.

●The Berlin trial of intravenous heparin versus placebo was stopped prematurely
because of excess mortality in the placebo arm [7]. Patients randomized to the heparin
arm had significantly better outcomes on a nonvalidated composite CVT severity scale
than those in the placebo group. The average length from onset of symptoms to
anticoagulation treatment, four weeks, was exceptionally long [8].
●The Dutch trial of subcutaneous nadroparin versus placebo enrolled 60 patients, but
excluded those who needed lumbar punctures for the relief of increased intracranial
pressure [3]. More patients treated with LMWH followed by oral anticoagulation had a
favorable outcome than controls, but the difference between the groups was not
statistically significant (table 1). Despite randomization, an imbalance at baseline may
have favored the placebo group, as there were more cases with isolated intracranial
hypertension in the placebo group and more patients with infarcts in the nadroparin
group.
A meta-analysis of these two trials found that anticoagulant treatment compared with
placebo was associated with a pooled relative risk of death of 0.33 (95% CI 0.08-1.21) and
a risk of death or dependency of 0.46 (95% CI 0.16-1.31) [9]. While these data suggest that
anticoagulant treatment for CVT may be associated with a reduced risk of death or
dependency, the results did not achieve statistical significance.

Two other trials were performed in India, but they were not included in the meta-analysis
because only abstract information was available, and because the diagnosis of CVT was
confirmed by CT alone [10,11]. These trials included 57 and 40 patients, respectively. Both
favored heparin compared with placebo: 15 percent versus 40 percent fatality in the Maiti
trial [10], and recovery in all patients versus two deaths and one residual hemiparesis in the
Nagajara trial [11].

Limited data suggest that LMWH is more effective than UFH and at least as safe for the
treatment of CVT:

●In an open-label trial, 66 adults with CVT were randomly assigned to treatment with
LMWH or UFH [12]. In-hospital mortality was significantly lower in the LMWH group (0
versus 19 percent). At three months, the proportion of patients with complete recovery
was greater for the LMWH group (88 versus 63 percent), but the difference was not
statistically significant. Small numbers limit the strength of these findings.
●In a nonrandomized case-control study, a greater proportion of adult patients treated
with LMWH (n = 119) compared with UFH (n = 302) were independent at six months
(92 versus 84 percent, adjusted odds ratio 2.4, 95% CI 1.0-5.7) [13]. Treatment with
LMWH was also associated with slightly lower rates of mortality (6 versus 8 percent)
and new intracranial hemorrhage (10 versus 16 percent), but these outcomes were not
statistically significant.
●In a single-center double-blind trial conducted in Iran, 52 cases of CVT were
randomly assigned to treatment with LMWH or UFH. There was no difference between
the treatment groups in neurological deficits, disability, and mortality [14].

Risk of new intracranial hemorrhage — Anticoagulants appear to be safe to use in adult

patients with CVT who have associated intracranial hemorrhage, either intracerebral (such
as hemorrhagic venous infarction) or subarachnoid [15]. In the Berlin and Dutch trials, 34 of
79 patients (43 percent) included in the Berlin and Dutch trials had an intracerebral
hemorrhage at baseline [3,7]. None of the patients randomized to heparin developed a new
intracerebral hemorrhage. In contrast, a new intracerebral hemorrhage developed in three
patients randomized to placebo. Case series have also reported relatively low risks of
intracranial hemorrhage (<5 percent) and systemic hemorrhage (<2 percent), and such
hemorrhages did not influence outcome [16-19]. These findings are in accordance with the
hypothesis that hemorrhage in CVT is caused by the probable mechanism of venous
outflow blockage and very high intradural and intravenous pressure, leading to both rupture
of venules and to hemorrhagic transformation of venous infarctions.

Similarly, observational data from single and multicenter case series suggest that
anticoagulant therapy is safe in children with CVT [20-26].

Endovascular treatment — For adults and children with CVT who develop progressive
neurologic worsening despite adequate anticoagulation with subcutaneous LMWH or
intravenous heparin, endovascular thrombolysis or mechanical thrombectomy at centers
experienced with these methods may be treatment options. However, the limited available
evidence suggests no benefit.

●A randomized controlled trial (TO-ACT) [27], comparing anticoagulation with

endovascular treatment (thrombectomy with or without chemical thrombolysis) in
patients with acute CVT and at least one risk factor for clinical deterioration (coma,
mental status disturbance, CVT involving the deep venous system, intracerebral
hemorrhage) was prematurely stopped for futility in 2017.  
●A 2010 systematic review of 15 studies including 156 patients revealed that despite
endovascular treatment there is a death rate of 9 percent and that local thrombolysis is
complicated by a non-negligible rate of major bleeding (10 percent), including 8 percent
intracranial hemorrhages, 58 percent of which were fatal [28].
●A 2015 systematic review identified 42 studies and 185 patients with CVT who were
treated with mechanical thrombectomy [29]. Many of the patients were severely ill;
pretreatment intracerebral hemorrhage was present in 60 percent, and stupor or coma
in 47 percent. A variety of devices were used, including the AngioJet rheolytic catheter,
balloon angioplasty, stents, and microsnares; concurrent local thrombolysis was used
in 71 percent. Overall, a good outcome was reported for 84 percent of patients;
mortality was 12 percent. New or worsened intracerebral hemorrhage affected 10
percent. A high recanalization rate (95 percent, 21 percent partial) was achieved.
While these results suggest that endovascular treatment may be useful and safe for
CVT, there is concern for the possibility of publication bias and under-reporting of
cases with poor outcome and complications. Furthermore, there may be bias in the
reported outcomes, since treatment and evaluation were not blinded in nearly all the
studies.
●In a Dutch center series of 20 patients with CVT who were treated with endovascular
thrombolysis, 8 patients (40 percent) died or became dependent [30]. We suspect that
the disappointing outcomes seen in this series after endovascular thrombolysis may be
a more accurate reflection of actual outcomes with this technique in clinical practice
when used to treat patients with clinically severe CVT.
Guideline recommendations — Consensus guidelines support the use of anticoagulation
for the acute treatment of CVT in adults and children.

Guidelines adults make the following recommendations for the treatment of acute CVT:

●The 2017 European Stroke Organization guidelines for the diagnosis and treatment of
cerebral venous thrombosis, endorsed by the European Academy of Neurology,
recommend heparin at therapeutic dosage to treat adult patients with acute CVT,
including those with an intracerebral hemorrhage at baseline [31]. The guidelines
suggest using LMWH instead of UFH. No recommendation is made regarding
thrombolysis for acute CVT, except that patients who have a pretreatment low risk of
poor outcome (eg, absence of coma, mental status disturbance, thrombosis of the
deep venous system, intracranial hemorrhage, and malignancy) should not be exposed
to aggressive treatments such as thrombolysis.
●The 2014 American Heart Association/American Stroke
Association (AHA/ASA) guidelines for the prevention of stroke state that
anticoagulation is reasonable for patients with acute CVT, even in selected patients
with intracranial hemorrhage [32]. The 2011 AHA/ASA guidelines for the diagnosis and
management of CVT conclude that initial anticoagulation with adjusted-dose UFH or
weight-based LMWH in full anticoagulant doses is reasonable, followed by vitamin K
antagonists, regardless of the presence of intracerebral hemorrhage [33].
●Guidelines from the American Academy of Chest Physicians (ACCP) issued in 2012
suggest anticoagulation over no anticoagulation during the acute and chronic phases
of CVT [34]. Either dose-adjusted UFH or LMWH can be used as initial treatment, even
in the presence of hemorrhage within a venous infarction. Patients who have stabilized
can be switched to oral anticoagulation, which is generally continued for a period of
three to six months.

Guidelines for acute treatment of CVT in children make similar recommendations based
upon weaker evidence:

●For children with CVT but without significant intracerebral hemorrhage, the ACCP
recommends initial anticoagulation with UFH or LMWH, followed by LMWH or vitamin
K antagonist treatment (ie, warfarin) for a minimum of three months [35].
Anticoagulation for an additional three months is suggested if there is still cerebral
sinovenous occlusion or ongoing symptoms (the latter presumably meaning new
venous infarcts or increased intracranial pressure) after three months of therapy.
●For children with CVT who have significant intracerebral hemorrhage, the ACCP
suggests either initial anticoagulation as for children without hemorrhage, or radiologic
monitoring of the thrombosis at five to seven days and anticoagulation if thrombus
 extension is noted at that time [35]. The ACCP suggests thrombolysis, thrombectomy,
or surgical decompression only in children with severe CVT in whom there is no
improvement with initial anticoagulation therapy.
●Guidelines from the AHA Stroke Council state that it is reasonable to institute either
UFH or LMWH in children with CVT, whether or not there is secondary hemorrhage
[36]. This is followed by warfarintherapy for three to six months.
●The 2011 AHA/ASA guidelines for the diagnosis and management of CVT conclude
that, for children with acute CVT diagnosed beyond the first 28 days of life, it is
reasonable to treat with full-dose LMWH even in the presence of intracranial
hemorrhage, and that it is reasonable to continue LMWH or oral vitamin K antagonists
for three to six months [33]. For neonates with acute CVT, the guidelines state that
treatment with LMWH or UFH may be considered, and that continuation of LMWH for
six weeks to three months may be considered.

OTHER ACUTE MANAGEMENT ISSUES — Major problems that may require intervention

in the acute phase of CVT include elevated intracranial pressure, brain swelling, and
seizures.

Antibiotic treatment is mandatory whenever there is meningitis or other intracranial infection,

or an infection of a neighboring structure, such as otitis or mastoiditis. For associated
inflammatory diseases such as Bechet disease, lupus, or vasculitis, treatment with
glucocorticoids may be necessary.

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".)

Elevated intracranial pressure and herniation — In the acute phase, elevated

intracranial pressure (ICP) may arise from single or multiple large hemorrhagic lesions,
infarcts, or brain edema. Elevated ICP or space-occupying lesions may cause transtentorial
herniation and death.

●General recommendations to control acutely elevated ICP should be followed,

including elevating the head of the bed, intensive care unit admission with sedation,
administering mannitol, hyperventilation to a target PaCO2 of 30 to 35 mmHg, and ICP
monitoring [15,36]. (See "Evaluation and management of elevated intracranial pressure
in adults" and "Elevated intracranial pressure (ICP) in children: Clinical manifestations
and diagnosis".)
●In patients with impending herniation due to unilateral hemispheric lesion,
hemicraniectomy can be life-saving. Retrospective data from a registry, and systematic
 reviews suggest that death can be prevented and a good functional outcome can be
achieved [37,38]. A prospective multicenter registry is ongoing.

There is no good evidence to support ventricular shunting as a treatment for acute

hydrocephalus or impending brain herniation in the acute phase of CVT [39].

In patients with sustained ICP elevation, successful treatment of intracranial hypertension

can prevent visual failure and resolve headache. A prospective study in 59 patients with
CVT presenting with isolated intracranial hypertension noted a complete recover in over 90
percent [40]; patients had a variety of interventions, and most had a therapeutic lumbar
puncture (LP). However, there are no studies specifically evaluating therapeutic LP for
elevated ICP or isolated intracranial hypertension in patients with CVT. Data from the
International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT) study did not
show differences in the outcomes in patients who had diagnostic LP [6]. European
guidelines state that therapeutic LP may be considered in patients with CVT and signs of
intracranial hypertension, because of a potential beneficial effect on visual
loss and/or headache, whenever its safety profile is acceptable [31].

Acetazolamide (500 mg twice daily) or furosemide (for patients who cannot tolerate

acetazolamide) are therapeutic options for patients with increased ICP [33], but efficacy is
unproven. Repeated lumbar punctures, a lumboperitoneal shunt, or fenestration of the optic
nerve sheet are treatment options if severe headaches persist, or if visual acuity is
decreasing, but efficacy is also unproven [33,41].

Although glucocorticoids, in particular intravenous dexamethasone, are prescribed in many

centers, they are not recommended for treating CVT in the absence of an underlying
inflammatory disorder such as Bechet disease or systemic lupus erythematosus [31,42-44].
No randomized clinical trials have been performed to evaluate their efficacy for CVT, but
available evidence suggests they are ineffective. This conclusion is supported by a study
that analyzed data from the observational ISCVT cohort of 642 patients with CVT (including
150 patients treated with glucocorticoids) using three different and complementary case-
control methods [45]. All three analyses failed to demonstrate any benefit of glucocorticoids,
even for patients with parenchymal lesions.

Seizures — For patients with CVT who have both seizures and focal cerebral supratentorial
lesions such as edema or infarction on admission head CT or brain MRI lesions, we
recommend seizure prophylaxis with antiepileptic medication.

In patients with CVT, recurrent seizures are more likely to develop in those who present
with seizures and in those with supratentorial brain lesions (focal edema, or ischemic or
hemorrhagic infarcts) on admission brain imaging [46]. The risk of developing seizures after
CVT diagnosis is very low in patients who do not have these risk factors. (See "Cerebral
venous thrombosis: Etiology, clinical features, and diagnosis", section on 'Seizures'.)

Younger children with CVT may have a higher frequency of seizures than older children or
adults. Consensus guidelines state that continuous electroencephalography may be
considered for children with CVT who are unconscious or mechanically ventilated [36].

Data are limited regarding the effectiveness of seizure prophylaxis with antiepileptic drugs in
patients with CVT [15,47]. In the ISCVT cohort, early seizures (those occurring within two
weeks after CVT diagnosis) were observed in the following patient subgroups, comparing
those not treated with antiepileptic (AED) prophylaxis versus those treated with AED
prophylaxis [46]:

●In patients with no supratentorial lesion and no seizure at presentation, early seizure
occurred in 5 of 197 (2.5 percent) not on AEDs versus 0 of 11 (0 percent) on AEDs
●In those with no supratentorial lesion who presented with seizure, early seizures
occurred in 1 of 14 (7 percent) not on AEDs and 0 of 35 (0 percent) on AEDs
●In patients with a supratentorial lesion but no seizure at presentation, early seizures
occurred in 11 of 134 (8 percent) not on AEDs and 1 of 35 (3 percent) on AEDs (odds
ratio [OR] 0.3, 95% CI 0.04-2.6)
●In patients with a supratentorial lesion who presented with seizure, early seizures
occurred in 24 of 47 (51 percent) not on AEDs and 1 of 148 (<1 percent) on AEDs (OR
0.006, 95% CI 0.001-0.05)

Thus, antiepileptic drug prophylaxis was associated with a reduced risk of early seizures in
patients with CVT. The risk reduction was statistically significant for patients in the highest
risk group (those with a supratentorial lesion and seizure at presentation) [46]. The strength
of this study is limited by its observational and retrospective design, but it represents the
largest experience in the literature.

Based upon these data, we recommend seizure prophylaxis only for patients with both
seizures at presentation and supratentorial lesions such as edema, infarction, or
hemorrhage on admission head CT or brain MRI. Prophylaxis is not clearly required for a
single early symptomatic seizure with CVT in the absence of supratentorial lesion, as there
is often no seizure recurrence. Furthermore, seizure prophylaxis is not recommended for
patients who have focal cerebral lesions without seizures [31,33].

When AED prophylaxis is used, valproate or levetiracetam is preferable

to phenytoin because they have fewer pharmacologic interactions with oral vitamin K
antagonist anticoagulants (eg, warfarin) [48]. General recommendations for the selection
and withdrawal of antiepileptic drugs are discussed separately. (See "Overview of the
management of epilepsy in adults", section on 'Antiseizure drug therapy' and "Seizures and
epilepsy in children: Initial treatment and monitoring", section on 'Selection of an antiseizure
drug'.)

MANAGEMENT AFTER THE ACUTE PHASE — The subacute phase of CVT often

involves decisions regarding the duration of anticoagulation and antiepileptic drug use. In
addition, there may be long-term complications including headaches, visual loss, cognitive
impairment, and psychiatric disturbances.

Long-term anticoagulation — The aim of continuing anticoagulation after the acute phase

is to prevent CVT recurrence, which affects 2 to 7 percent of patients, and to prevent
extracerebral venous thrombosis, which occurs in up to 5 percent of patients with CVT,
mainly from deep venous thrombosis of the limbs or pelvis, and/or pulmonary embolism [6].
(See 'Recurrence' below.)

Selection of anticoagulant — For most adults with CVT, we suggest anticoagulation

with warfarin for a minimum of three months duration after the acute phase, aiming at an
international normalized ratio (INR) target of 2.5 (acceptable range: 2 to 3) [31,35,49].

An alternative preferred by some experts and UpToDate contributors is the use of direct
(non-vitamin K-dependent) oral anticoagulants (ie, direct thrombin and factor Xa inhibitors)
rather than warfarin. However, the authors of this topic note that only small numbers of
patients with CVT have been treated with direct oral anticoagulants and no conclusions can
be drawn about their safety and efficacy for preventing recurrent CVT until ongoing trials are
completed [50]. In accordance with European guidelines, they do not recommend using
direct oral anticoagulants for the prevention of recurrent venous thrombosis after CVT [31].

For patients with malignancy who require long-term anticoagulation and who do not have
severe renal insufficiency (creatinine clearance <30 mL/minute), low molecular weight
heparin (LMWH) is generally preferred rather than oral vitamin K antagonists or direct oral
anticoagulants, but oral anticoagulation is preferred over no therapy. (See "Treatment of
venous thromboembolism in patients with malignancy".)

Patients with severe renal insufficiency should not receive direct oral anticoagulants; vitamin
K antagonists (eg, warfarin) are preferred for oral therapy.

For most patients who require long-term anticoagulation during pregnancy (with the
exception of patients with mechanical heart valves), heparins are safer than other
anticoagulants. Warfarin and direct oral anticoagulants are avoided. (See "Use of
anticoagulants during pregnancy and postpartum".)
Duration of anticoagulation — After the acute phase of CVT, we suggest continuing
anticoagulation for a minimum period of three months. However, there is no definitive
evidence regarding the optimal duration of anticoagulant therapy specifically for reducing
the risk of recurrent CVT [31]. A reasonable approach is to stratify the duration of
anticoagulant therapy according to the individual prothrombotic risk as follows [33,34]:

●For patients with a provoked CVT associated with a transient risk factor (table 2),
anticoagulation is continued for three to six months.
●For patients with an unprovoked CVT, anticoagulation is continued for 6 to 12
months.
●For patients with recurrent CVT, venous thromboembolism after CVT, or a first CVT
with a severe thrombophilia (ie, homozygous prothrombin G20210A mutation,
homozygous factor V Leiden mutation, deficiencies of protein C, protein S, or
antithrombin, combined thrombophilia defects, or antiphospholipid syndrome),
anticoagulation may be continued indefinitely.

Aspirin — Some experts use aspirin for patients who complete a course of anticoagulation

or for patients who cannot tolerate or decline oral anticoagulation, based upon evidence that
aspirin is effective for patients who have completed a conventional course of
anticoagulation after a first unprovoked episode of venous thromboembolism
(see "Rationale and indications for indefinite anticoagulation in patients with venous
thromboembolism", section on 'Aspirin'). However, the use of aspirin or other antiplatelet
agents after CVT has not been evaluated in controlled trials or observational studies, and
current guidelines make no recommendation about using aspirin in this setting [31,33,34].

Seizure prevention — The optimal duration of antiepileptic treatment after CVT is

unknown. However, the risk of epilepsy after CVT ranges from 5 to 11 percent of patients
[6,51,52], and the risk is higher in those with seizures in the acute phase or with
hemorrhagic parenchymal lesions [52]. For patients with CVT and associated parenchymal
brain lesions who present with one or more seizures, antiseizure drugs should be continued
until seizure-free for a defined duration (eg, one year).

General recommendations for the selection and withdrawal of antiepileptic drugs are
discussed separately. (See "Overview of the management of epilepsy in adults", section on
'Antiseizure drug therapy' and "Overview of the management of epilepsy in adults", section
on 'Discontinuing antiseizure drug therapy'and "Seizures and epilepsy in children: Initial
treatment and monitoring", section on 'Selection of an antiseizure drug'.)

Headaches — Headaches severe enough to require bed rest or hospital admission afflict

14 percent of patients with CVT [6]. Repeated brain MRI and magnetic resonance
venography (MRV) are necessary to exclude the rare case of recurrent CVT. MRV may
depict stenosis of a previously occluded sinus [53,54].

Lumbar puncture may be necessary to exclude chronically elevated intracranial pressure

(ICP) if headache persists and brain MRI and MR venography are normal. In such cases,
therapeutic options to treat elevated ICP include acetazolamide, topiramate, repeated
lumbar punctures, placement of a lumbar-peritoneal shunt, or stenting of a sinus stenosis
[55-57].

Visual loss — Severe visual loss due to CVT is fortunately a rare event [58-60].
Nevertheless, elevated intracranial pressure must be rapidly ruled out and managed
accordingly if visual acuity decreases during follow-up and is not explained by ocular
causes. Fenestration of the optic nerve sheath has also been used to relieve pressure and
prevent optic nerve atrophy.

Because of the potential for visual loss caused by severe or long-standing elevation of
intracranial pressure, serial assessment of visual fields and visual acuity is recommended
for children with CVT during follow-up, particularly during the first year [33,36]. It is
reasonable to do the same for adults with visual complaints, chronic headaches, or
papilledema.

Cognitive and psychiatric complications — Despite the apparent general good recovery

in most patients with CVT, approximately one-half of the survivors feel depressed or
anxious [61], and minor cognitive or language deficits may preclude them from resuming
their previous jobs [62]. Patients should be reassured of the very low level of risk of
recurrence of CVT and encouraged to return to previous occupations and hobbies. In some
cases, antidepressants may be necessary.

Subsequent pregnancy — In our view, and in accordance with national guidelines, it is

reasonable to treat with LMWH during pregnancy and puerperium for women with a
previous history of CVT to reduce the risk of recurrent CVT and other venous
thromboembolic events [31,33,63]. In the United States, some clinicians treat with
intravenous unfractionated heparin (UFH) or LMWH during the late third trimester and/or up
to eight weeks postpartum.

Pregnancy and the puerperium are known risk factors for CVT (see "Cerebral venous
thrombosis: Etiology, clinical features, and diagnosis", section on 'Risk factors and causes').
The absolute risk of complications during subsequent pregnancy among women who have
a history of CVT appears to be low, although the relative risks of recurrent CVT and
noncerebral venous thromboembolism are quite elevated compared with the general
population. Supporting evidence comes from a 2016 systematic review of 13 observational
studies evaluating the frequency of CVT or noncerebral venous thromboembolism
associated with pregnancy and the puerperium in women with a history of previous CVT
[64]. The following observations were reported:

●Recurrent CVT occurred in 2 of 217 pregnancies (9 per 1000 pregnancies, 95% CI 3-

33 per 1000), a rate that was more than 80-fold higher than the previously reported
incidence in the general population
●Noncerebral venous thromboembolism occurred in 5 of 186 pregnancies (27 per
1000, 95% CI 12-61 per 1000), a rate that was approximately 16-fold higher than the
incidence previously described in the general population
●Spontaneous abortion occurred in 33 of 186 pregnancies (18 percent, 95% CI 13-24
percent), a rate similar to the estimated rate in the general population
(see "Spontaneous abortion: Risk factors, etiology, clinical manifestations, and
diagnostic evaluation", section on 'Incidence')
●There was no significant difference in the rate of spontaneous abortion for women
treated or not treated with antithrombotic therapy (11 versus 19 percent)

Thus, based upon the available evidence, a history of CVT, including pregnancy- or
puerperium-related CVT, is not a contraindication for future pregnancy.

Women should be advised not to become pregnant while on warfarin because of its

teratogenic effects and increased risk of fetal bleeding. (See "Use of anticoagulants during
pregnancy and postpartum", section on 'Women on warfarin'.)

Oral contraceptives — Because it is a risk factor for CVT, women with prior CVT should
be informed about the risks of combined estrogen-progestin hormonal contraception and
advised against its use [31,36].

PROGNOSIS — Cerebral venous thrombosis (CVT) can result in death or permanent

disability, but usually has a favorable prognosis.

Early deterioration and death — Approximately 5 percent of patients die in the acute

phase of the disorder [65,66]. Most of the early deaths are a consequence of CVT. A
systematic review found that mortality rates among patients with CVT declined since the
1960s; increased detection of less severe cases with advances in neuroimaging along with
improved hospital care may have accounted for some or all of the decreased mortality [67].

In the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT) that
evaluated 624 patients (age >15 years) with CVT, 27 patients (4.3 percent) died during
hospitalization for the acute phase, including 21 patients (3.4 percent) who died within 30
days from symptom onset [65].

Predictors of mortality at 30 days in the ISCVT were as follows [65]:

●Depressed consciousness
●Altered mental status
●Thrombosis of the deep venous system
●Right hemisphere hemorrhage
●Posterior fossa lesions

Early mortality in children with CVT is similar to that in adults. In a European cohort of 396
children with CVT (median age 5.2 years), death in the first two weeks after presentation
occurred in 12 patients (3 percent) [68].

The main cause of acute death with CVT is transtentorial herniation secondary to a large
hemorrhagic lesion [65]. Other causes of early death include herniation due to multiple
lesions or to diffuse brain edema, status epilepticus, medical complications, and pulmonary
embolism [4].

It is important to be able to predict which patients are going to deteriorate after admission,
as this will occur in about 23 percent [69]. Neurologic worsening may occur several days
after admission; symptoms and signs may include depressed consciousness, mental status
disturbance, new seizure, worsening of or a new focal deficit, increase in headache
intensity, or visual loss.

About one-third of patients with neurologic deterioration will show new parenchymal lesions
if neuroimaging is repeated [69]. Patients with depressed consciousness on admission are
more likely to deteriorate. Seizures are more likely to recur in those with seizures at onset
and to occur de novo in patients with parenchymal lesions.

Long-term outcome — Mortality after the acute phase of CVT is predominantly related to

underlying conditions. The main ISCVT report [6] performed a meta-analysis of seven
prospective series, including the large ISCVT itself [6,51,58,70-73], and found that acute
CVT was associated with a 15 percent overall death or dependency rate at the end of
follow-up, which varied from 3 to 78 months.

In the ISCVT at the end of follow-up (median 16 months), death had occurred in 52 of 624
patients (8 percent) [6].

Predictors of poor long-term prognosis in the ISCVT were as follows [6]:

 ●Central nervous system infection
●Any malignancy
●Thrombosis of the deep venous system
●Hemorrhage on head CT or MRI
●Glasgow coma scale score <9 on admission (table 3)
●Mental status abnormality
●Age >37 years
●Male gender

These predictors of poor long-term prognosis were validated in an independent cohort [74].

In the ISCVT, complete recovery at six months was significantly more common for women
than men (81 versus 71 percent), while dependency or death was less likely for women
than men (12 versus 20 percent) [75]. These differences were driven entirely by the more
favorable outcome for the subgroup of women who had gender specific risk factors (mainly
oral contraceptives, pregnancy, or puerperium) for CVT. Women without gender specific
risk factors had outcomes similar to men. (See "Cerebral venous thrombosis: Etiology,
clinical features, and diagnosis", section on 'Epidemiology'.)

Intracerebral hemorrhage present at the time of CVT diagnosis was identified in 245
patients (39 percent) of the ISCVT cohort [76]. In this subgroup with early intracerebral
hemorrhage, predictors of poor prognosis at six months were older age, male gender,
thrombosis of the deep cerebral venous system or of the right lateral sinus, and a motor
deficit [76]. In contrast, several studies have found that a good outcome after CVT is
predicted when intracranial hypertension is the only manifestation of CVT at the time of
diagnosis [65,70,72].

In patients with CVT presenting with isolated intracranial hypertension, a subgroup analysis
of the ISCVT cohort found that poor outcome was associated with a longer diagnostic delay
[77].

In the ISCVT study, complete recovery at last follow-up was noted in 79 percent of the
entire cohort of patients [6].

Recurrence — The risk of recurrent CVT is approximately 2 to 4 percent, while the risk of

recurrent venous thromboembolism in other locations ranges from 4 to 7 percent. These
estimates are illustrated by the following observations:

●In a study that prospectively followed 145 patients with CVT for a median of six years
after discontinuation of anticoagulant therapy, a recurrent CVT developed in five
 patients (3 percent), and other types of venous thromboembolism developed in another
10 patients (6 percent) [78]. The risks of recurrent CVT or other types of venous
thromboembolism were 0.5 and 2.0 per 100 person-years, respectively. The risk of
venous thrombotic events was significantly higher in males and in patients with severe
thrombophilia.
●In the ISCVT study, which evaluated 624 patients with CVT over a median 16 months
of follow-up, 14 (2 percent) had a recurrent CVT and 27 (4 percent) had other
thrombotic events during follow-up [6]. The risks of recurrent CVT or any venous
thrombosis were 1.5 and 4.1 per 100 person-years, respectively [79]. The risk of
venous thrombotic events was significantly higher in males and in patients
with polycythemia/thrombocythemia.
●In a retrospective cohort study of 706 patients with first CVT who were followed for 6
to 297 months (median 40 months), CVT recurred in 31 patients (4 percent) and
venous thromboembolism in a different site occurred in 46 patients (7 percent) [80].
Previous venous thromboembolism was the only factor predicting recurrent events.
●In the European cohort of 396 children with CVT (median age five years) followed for
a median of 36 months, recurrent venous thrombosis occurred in 22 children at a
median of six months, including CVT in 13 children (3 percent) [68]. There were no
recurrences of CVT among children younger than 25 months. Factors independently
associated with recurrent cerebral and systemic venous thrombosis in children were
nonadministration of anticoagulant therapy before relapse (hazard ratio [HR] 11.2, 95%
CI 3.4-37.0), persistent occlusion on repeat venous imaging (HR 4.1, 95% CI 1.1-14.8),
and heterozygosity for the prothrombin (factor II) G20210A mutation (HR 4.3, 95% CI
1.1-16.2) [68].

Recanalization — Although there are few studies, available data suggest that cerebral vein
and sinus recanalization occurs in 40 to 90 percent of patients after CVT, mostly within the
first four months [81]. A systematic review identified only five small studies that evaluated
recanalization after CVT [82]. In the pooled population of these studies, recanalization rates
at three months and one year of follow-up were nearly identical (59 of 70 [84 percent] and
60 of 70 patients [85 percent]). A subsequent retrospective study of 102 patients with first-
ever non-septic CVT treated with anticoagulation for at least 12 months found that the
cumulative proportion of patients with any recanalization by 3, 6, and 9 months was 71, 90,
and 94 percent, respectively [83]. The time for 50 percent of patients to attain any
recanalization was 64 days; the corresponding time for complete recanalization was 169
days.

The likelihood of recanalization appears to vary by location, with the highest rates observed
in deep cerebral veins and cavernous sinuses, and lowest rates observed in the lateral
sinus [81]. In adults, the relationship of recanalization with outcome is unclear and data are
limited. Some studies suggest that recanalization of the occluded sinus is not related with
outcome after CVT [82,84], while others suggest that complete recanalization is associated
with improved functional outcome [83].

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

●For adults with symptomatic cerebral venous thrombosis (CVT), with or without
hemorrhagic venous infarction, we recommend initial anticoagulation therapy with
subcutaneous low molecular weight heparin (LMWH) or intravenous heparin (Grade
1C). For children with CVT, with or without significant secondary hemorrhage, we
suggest initial anticoagulation therapy with subcutaneous LMWH or intravenous
heparin (Grade 2C). (See 'Early anticoagulation' above.)
●Measures to control acutely increased intracranial pressure and impending herniation,
including decompressive surgery, may be required in patients with CVT. (See 'Elevated
intracranial pressure and herniation' above.)
●For patients with CVT who have both seizures at presentation and focal cerebral
supratentorial lesions (eg, edema, infarction, or hemorrhage on admission CT or MRI),
we recommend seizure prophylaxis with antiepileptic medication (Grade 1B).
Prophylaxis is not clearly required for those with a single early symptomatic seizure
due to CVT in the absence of a supratentorial cerebral lesion, as there is often no
seizure recurrence. Furthermore, seizure prophylaxis is not clearly required for patients
who have focal cerebral lesions without seizures. (See 'Seizures' above.)
●After the acute phase of CVT, we suggest anticoagulation with warfarin for a
minimum of three months, aiming at an international normalized ratio (INR) target of
2.5 (acceptable range: 2 to 3) (Grade 2C). An alternative to warfarin preferred by some
experts (but not the authors of this topic) is the use of direct oral anticoagulants (ie,
direct thrombin and factor Xa inhibitors). (See 'Long-term anticoagulation'above
and 'Selection of anticoagulant' above.)
●It is reasonable to stratify the duration of anticoagulation after the acute phase of CVT
according to individual prothrombotic risk, continuing anticoagulation for three to six
months for patients with a provoked CVT associated with a transient risk factor (table
2), and for 6 to 12 months for patients with an unprovoked CVT. Indefinite oral
anticoagulation is reserved for patients with recurrent CVT, extracerebral venous
thromboembolism after CVT or an associated severe thrombophilia. (See 'Duration of
anticoagulation' above.)
 ●Antiepileptic drug management, headaches, visual loss, and cognitive and psychiatric
complications are additional problems that may complicate the subacute phase of CVT.
(See 'Management after the acute phase' above.)
●For pregnant women with a history of CVT, we suggest temporary anticoagulation
with subcutaneous LMWH throughout pregnancy and continuing up to eight weeks
postpartum (Grade 2C). (See 'Subsequent pregnancy' above.)
●For adolescent girls and adult women with a history of CVT, we advise not using
combined oral contraceptives. (See 'Oral contraceptives' above.)
●CVT is associated with a good outcome (complete recovery or minor residual
symptoms or signs) in close to 80 percent of patients. Nevertheless, approximately 5
percent of patients die in the acute phase of the disorder, and longer-term mortality is
nearly 10 percent. The main cause of acute death with CVT is neurologic, most often
from brain herniation. After the acute phase, most deaths are related to underlying
disorders such as cancer. (See 'Prognosis' above.)
●Predictors of poor long-term prognosis include central nervous system infection,
malignancy, deep CVT location, intracranial hemorrhage, Glasgow coma scale score
on admission <9, mental status abnormality, age >37 years, and male sex. Isolated
intracranial hypertension at the time of CVT diagnosis may be a predictor of good
outcome. Recurrent CVT appears to be uncommon, with rates ranging from 2 to 4
percent. (See 'Prognosis' above.)

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