The Journal of Emergency Medicine, Vol. 36, No. 2, pp. 132–137, 2009
Copyright © 2009 Elsevier Inc.
Printed in the USA. All rights reserved
0736-4679/09 $–see front matter

doi:10.1016/j.jemermed.2007.02.041

Clinical
Communications: Adults

CEREBRAL VENOUS THROMBOSIS: A CASE REPORT
Nishant Anand, MD,* Carmie Chan, MD,* and N. Ewen Wang, MD†
*Stanford Kaiser Emergency Medicine Residency Program, Stanford, California and †Division of Emergency Medicine,
Stanford University School of Medicine, Palo Alto, California
Reprint Address: Ewen Wang, MD, Division of Emergency Medicine, Stanford University School of Medicine, 701 Welch Road,
Building C, Palo Alto, CA 94304

e Abstract—The incidence of cerebral venous thrombosis
(CVT) is three to four cases per one million in adults and seven
cases per one million among children. We discuss a case of
CVT in a 19-year-old woman with a history of migraine
headaches. A CVT most commonly presents acutely, with a
wide variety of signs and symptoms in young adults and
children. The most common presenting complaint in adults is
severe headache. The most frequently thrombosed sinuses are
the lateral, cavernous, and superior sagittal sinuses. Risk factors include any genetic or acquired prothrombotic conditions, including pregnancy and the peripartum period. Computed tomography may show the classic “delta sign,” although
magnetic resonance imaging with magnetic resonance venography is more sensitive. Treatment is controversial at this
time. Options include fibrinolysis, anticoagulation, mechanical
thrombectomy, or surgery. Despite the lack of agreement on
management of CVT, the prognosis of CVT has improved,
due to an increasing frequency of diagnosis. © 2009 Elsevier
Inc.

tracranial tumor, other hemorrhagic stroke, or cerebral venous thrombosis (CVT) may present with headache. The
annual incidence of CVT is three to four cases per one
million, with 75% of adult patients being women, and up to
seven cases per one million among children. The majority
of adult patients, up to 80%, will have good neurologic
outcome if diagnosed correctly (2). Among childhood
cases of CVT, neonates are the most commonly affected
age group. In contrast to adults, about half of the children
affected suffered neurological impairment or death from
CVT (3). We will discuss a case of cerebral venous
thrombosis and etiologies, diagnostic tools, and therapeutic options.

CASE REPORT
A 19-year-old right-hand-dominant woman was brought
to the ED by her friends for a severe headache that had
acutely become unbearable. In addition, the patient had
woken up feeling weak. When she tried to get out of bed
and walk, she would fall to the left side. She felt that her
left arm and leg were not as strong as usual. She took
Ibuprofen and metoclopramide without relief.
The patient stated that she had been to the ED 2 days
prior with a headache. During that visit, she received
Reglan and morphine, with decrease in the severity but
without complete resolution of the headache. No diagnostics had been performed.

e Keywords—Cerebral venous thrombosis; sinus thrombosis; stroke

INTRODUCTION
Headaches present a diagnostic and therapeutic challenge
for emergency physicians. Headaches are a common Emergency Department (ED) complaint, accounting for 2.6% of
ED visits (1). Although most headaches are benign, many
serious conditions, including subarachnoid hemorrhage, in-

RECEIVED: 7 September 2005; FINAL SUBMISSION RECEIVED: 5 July 2006;
ACCEPTED: 13 November 2006
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Cerebral Venous Thrombosis

The patient had experienced headaches since her return from Mexico 3 weeks prior. This headache was not
like her previous migraines, but it was not the worst
headache of her life. Associated symptoms included photophobia, nausea, occasional non-bloody emesis, and
chills for the last 10 days.
The patient had a past medical history of B-12 deficiency anemia. She had been receiving monthly B-12
injections. The patient had been diagnosed by her primary care physicians with migraine headaches 3 years
prior. She had never previously been to the ED for
headaches, nor had a computed tomography (CT) scan of
the head ever been obtained.
The patient’s medications included the monthly B-12
shots and oral contraceptives. She had been on the same
regimen for the past few years. The patient was not
aware of any cancer history, bleeding problems, or hypercoagulability in her family. She was a college student
and lived in a dormitory. She had not received a meningococcal vaccination. She denied nicotine, alcohol, and
intravenous drug use.
On physical examination, the patient was an ageappropriate woman who appeared uncomfortable. She
was lying in the room with the lights off. The temperature was 37°C (98.6° F) oral, pulse 85 beats/min, blood
pressure 109/61 mm Hg, and respirations 16 breaths/min.
She was alert and oriented times three. The cardiovascular, pulmonary, and abdominal examinations were benign. The pupils were equal and reactive to light bilaterally. The extraocular movements were intact. No
diplopia was reported. Visual fields were grossly normal.
There was weakness with closing the eyes. Smiling revealed a left facial paralysis sparing the forehead. The
tongue was midline. Strength of the left deltoid, bicep,
tricep, hand flexors and extensors, hip flexors, quadriceps, hamstrings, tibialis anterior, and gastrocnemius
were 2/5 compared to 5/5 on the right. Pain sensation
was decreased on the left side of the body. Reflexes were
hyperactive on the left with clonus and upgoing toes.
Gait was not assessed.
Workup in the ED showed a normal complete blood
cell count with a white blood cell count of 5600, hematocrit 28.8, mean corpuscular volume 69.3, platelets 424.
Chemistry panel results were as follows: sodium 138,
potassium 4, chloride 106, bicarbonate 24, BUN 10,
creatinine 0.6, glucose 107, calcium 8.9. Total bilirubin
was 0.5, alkaline phosphatase 44, AST 22, ALT 16.
Computed tomography (CT) scan showed low attenuation of the right basal ganglia, thalamus, internal capsule, and head of caudate with adjacent mass effect, and
asymmetric enlargement of the right lateral ventricle.
There was high attenuation along the straight sinus and
posterior interhemispheric fissure (Figure 1). After consulting with neurosurgery, an emergent magnetic reso-

133

Figure 1. Computed tomography scan showing mild shift (a),
infarcted tissue (b), blood in the ventricle (c), and thrombosis
in the straight sinus (d).

nance imaging (MRI) scan of the head was obtained. The
MRI scan showed thrombosis of the straight sinus, deep
internal cerebral veins, left sigmoid and transverse sinuses. There was infarction of the thalami, caudate and
basal ganglionic nuclei, with mild mass effect (Figures 2, 3).

DISCUSSION
Cerebral venous thrombosis (CVT) presents with a wide
variety of signs and symptoms. Due to the rarity of the
disorder, much of the knowledge about CVT has been
obtained through case reports, retrospective studies, and
a few small prospective studies. The International Study
on Cerebral Vein Thrombosis (ISCVT) is an international, multi-center prospective observational study striving to obtain prospective data on CVT. The ISCVT
collaborators have published four abstracts thus far relating to risk factors, management, and prognosis of
CVT (4). Research on CVT in children is limited. Agerelated differences in hemostatic, vascular, and neurologic systems between children and adults make extrapolation from adult data difficult (3). Despite the paucity
of research, the prognosis of CVT has improved, due
largely to improvements in diagnosis.

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N. Anand et al.

cases per 100,000 deliveries. Reviews of CVT cases
related to pregnancy have found an increased risk late
in pregnancy and immediately after delivery, as well
as an association with delivery by caesarean section,
preeclampsia, and infection (5). The mortality rate of
pregnancy-related CVT is 2–10% less than the risk of
death from CVT associated with other causes (6). Oral
contraceptives also have been associated with an increased risk of CVT, likely related to increased prothrombogenicity. Other prothrombotic risk factors include high homocysteine levels, and low folate and
vitamin B12 levels (7). Any history of previous thrombotic disease, such as venous thrombosis, should also be
considered a risk factor. In one series of 79 patients with
CVT, 11.4% had a history of deep venous thrombosis
(DVT) and 16.5% had a positive family history for
venous thrombosis (8). Any prothrombotic condition is a
risk factor for DVT and CVT; additional risk factors for
DVT also include obesity, surgery, and immobilization.
Researchers suggest that CVT is due purely to circulating coagulation factors in the blood and vascular wall
defects, whereas DVT, which is a more common disorder, is affected by multiple other factors (9).
Figure 2. Magnetic resononance image illustrating infarcted
tissue (arrow).

A CVT most commonly presents with acute onset of
symptoms. The most frequently thrombosed sinuses are
the lateral, cavernous, and superior sagittal sinuses; the
straight sinus and vein of Galen are less frequently involved. The pathogenesis of this disorder can be explained by the effects of occlusion of both the cerebral
veins and the major venous sinuses (2). The cerebral
veins drain into the major sinuses, which in turn drain
into the internal jugular veins. Blockage in any part of
this system will result in higher hydrostatic pressures
being transmitted backwards into the veins. Thrombosis
of the cerebral veins causes localized edema of the brain
and venous infarction, leading to ischemia and cytotoxic
edema from damage to the cellular membrane pumps; in
addition, disruption of the blood-brain barrier causes
vasogenic edema. The developing venous infarcts may
also transform into hemorrhagic infarcts. Occlusion of
the venous sinuses leads to development of intracranial
hypertension by blocking absorption of cerebrospinal
fluid (2).
Risk factors for CVT include any genetic or acquired
prothrombotic conditions, including pregnancy and puerperium. A predisposing risk factor or direct cause can
be identified in the majority of adult and pediatric cases
of CVT. The incidence of peripartum CVT is about 12

Figure 3. Thrombosis of the straight sinus, deep internal
cerebral veins, left sigmoid and transverse sinuses. (a) Illustrates normal flow. (b) Illustrates absence of flow due to
thrombus.

Cerebral Venous Thrombosis

A CVT also may be caused by mechanical factors,
including head trauma, neurosurgical procedures, or injury to sinuses or jugular vein, including venous catheterization. Generally, CVT after trauma has a subacute
course, usually presenting a few days to weeks after the
injury (10). Lumbar punctures may also cause sinus
thrombosis; low cerebrospinal fluid pressure after a lumbar puncture causes downward traction on the cortical
veins and sinuses, possibly leading to thrombosis (2). Infectious etiologies of CVT include extension from osteomyelitis, sinus thrombophlebitis, or complications of otitis
media and mastoiditis. The incidence of infection-related
CVT is higher in children than adults (3). Other risk factors
include polycythemia, cryofibrinogenemia, sickle-cell
anemia, leukemia, disseminated intravascular coagulation, systemic lupus erythematosus, sarcoidosis, inflammatory bowel syndrome, and Behcet syndrome.
The diagnosis of CVT can be challenging. The most
common presenting complaint is severe headache (80 –
90%), which may be acute or subacute, and is more often
localized rather than diffuse and often constant (11–13).
Such headaches may mimic migraines or subarachnoid
hemorrhages. CVT has been reported to present with a
subarachnoid hemorrhage (SAH), although unlike other
types of SAH, the basal cisterns were spared in CVT
cases presenting with SAH (14).
Patients with CVT may also present with focal neurologic deficits. Thrombosis of the superior sagittal sinus
may present with a hemiplegia with subsequent involvement of the other hemisphere, due to development of
cortical lesions on the other side of the sagittal sinus. If
severe, involvement of the superior sagittal sinus may
lead to signs of increased intracranial pressure and coma.
Thrombosis of the cavernous sinus may classically cause
ptosis, proptosis, chemosis, and extraocular dysmotility
due to deficits of cranial nerves III, IV, and VI. Hyperesthesia of the ophthalmic and maxillary divison of the
fifth cranial nerve and a decreased corneal reflex may be
detected. There also may be evidence of dilatated, tortuous retinal veins and papilledema. With thrombosis of
the superior sagittal sinus and transverse sinus, a 6th
nerve palsy may be present. Involvement of the deep
sinuses, including straight sinus and vein of Galen, may
cause thalamic lesions, leading to behavioral changes
such as mutism, delirium, and amnesia. About 23% of
patients with CVT present with isolated intracranial hypertension, causing headache and diplopia due to involvement of the sixth cranial nerve, and papilledema
(15). Left untreated, patients may suffer permanent visual defects or even blindness. About 40% of patients
with CVT present with seizures, which are limited and
focal in 50% of these patients, but may also secondarily

135

generalize into grand mal seizures (2). This percentage is
even higher in neonates, in whom seizures may be the
presenting complaint for CVT.
Children with CVT may present similarly to adults,
with altered level of consciousness, headache complaints, or cranial nerve palsies. For unknown reasons,
focal neurological deficits are less common in children
compared to adults (3). Neonates with CVT often present
with seizures and diffuse neurologic signs.
Given the potential serious complications of a missed
CVT, one must think of the diagnosis in patients who
present with headache and neurologic symptoms, isolated intracranial hypertension, or those with CT evidence of vascular infarcts in non-arterial distributions.
This is especially true in younger patients without the
usual vascular risk factors (2). In these cases, a search for
predisposing risk factors for CVT may be helpful.
A non-contrast head CT scan may be useful for diagnosis, as well as to rule out other cerebral disorders. The
classic finding on non-contrast CT scan is the “delta
sign,” a dense triangle within the superior sagittal sinus
that is created by thrombosis within the sinus. Falsepositive “delta signs” may appear in trauma patients with
adjacent subdural hemorrhages. On contrast-enhanced
CT scan, the “empty delta sign” appears in the superior
sagittal sinus from enhancement of the surrounding dura
around the thrombosed sinus, which is less dense; however, this sign is only seen in up to 30% of cases (Figure 4)
(16). If the CT scan is abnormal but still inconclusive
regarding pathology, imaging modalities that can further
define CVT include MRI and MR venography, which is
the most sensitive test for CVT. The thrombosed sinus
has a hyperintense signal on MRI, and venography
should demonstrate abnormal flow within the sinuses. If
isolated cortical vein thrombosis is suspected (without
thrombosis of the sinus), cerebral angiography should
also be considered. For neonates, transfontanel Doppler
ultrasound may be useful for diagnosis. CT venography
is a new technique that may also aid in viewing the
cerebral venous system. Currently, the false-negative
rate for CT diagnosis is estimated to be about 25% (15).
Treatment for CVT is controversial, and may depend
on neurology or neurosurgical preferences within individual institutions. Anticoagulation with heparin is currently the first-line treatment to stop the thrombotic process. Limited evidence has suggested that, even with
intracerebral hemorrhage overlying venous infarction,
heparin does seem to be safe with no evidence of increased intracerebral bleeding. Treatment with heparin
(conventional unfractionated or low molecular weight)
also has been shown to be associated with a potentially
important reduction in mortality and morbidity, although
the statistics are not significant in a recent review (17).

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N. Anand et al.

Figure 4. Clot in superior sagittal sinus illustrating the “empty delta sign.”

The duration of oral anticoagulation treatment after the
acute CVT has not been systematically studied; most
patients are treated with warfarin for 6 months after the
initial episode, with target INR 2.5. This recommendation follows established guidelines for treating pulmonary embolism. Recurrence of sinus thrombosis occurs in
2% of patients, with 4% of patients experiencing a recurrent thrombotic event in 1 year (2).
Despite a lack of randomized clinical trials, case reports have also reported success with endovascular
thrombolysis, both mechanical and chemical, in patients
who deteriorate despite heparin therapy (18,19). Intraarterial thrombolysis with urokinase and recombinant
tissue plasminogen activator (rt-PTA) have been reported with successful recanalization. Most experts agree
that thrombolysis should be reserved for patients with a
poor prognosis who have not responded to heparin therapy. Platelet glycoprotein IIb/IIIa inhibitors also show
promise in rat studies; however, there have been no
reports of human treatment (20).
Overall, the prognosis for CVT is quite good, especially compared to arterial stroke. Mortality rates are
estimated between 8% and 20%. Poor prognostic factors
include male gender, age over 37 years, low Glasgow
Coma Scale score, mental status disorders, focal neurologic deficits, hemorrhage or “delta sign” on CT scan,
involvement of the deep venous system, central nervous
system infection, central nervous system disorder or cancer as a predisposing condition, or presentation with
seizures (21,22). Predictors of good outcome include
absence of focal deficits, no cancer, and presence of
isolated intracranial hypertension. Unlike patients with
arterial stroke, most patients with CVT regain indepen-

dence with good neurologic and cognitive long-term
outcomes. Most studies estimate that 70 – 86% of treated
patients have no functional disability at 3 years’ followup, despite some residual neurologic symptoms (13,21).
Long-term complications include seizures (10 –16%) and
new thrombotic events (4 –12%) (8,22,23). It is still
unclear whether patients should be placed on long-term
anti-seizure medications. There are limited data on longterm outcomes of children with CVT; in a Canadian
review, 77% of neonates and 52% of non-neonates were
neurologically normal at follow-up (3).
In the case of our patient, neurosurgery was consulted.
They initiated a heparin drip, and given the severity of
her neurologic findings, decided to administer intravenous tPA, and perform a mechanical thrombectomy on
the same day. On day 2, the patient became stuporous
and was intubated. She was taken to the operating room
by neurosurgery for placement of a frontal external ventricular drain. Deep venous system thromboses with increased intracranial pressure, hydrocephalus, and intraventricular hemorrhage were found. On day 4 she was
extubated. A repeat MRI scan showed patent venous
sinuses, infarcted right thalamus, basal gangli, and internal capsule. A few days later, the patient had normal
alertness, with 3/5 strength on the left that was slightly
improved from admission. The sensory deficits were also
improved. The intracranial pressure was ⬍ 10 and she
was transferred out of the Intensive Care Unit. She was
discharged on hospital day 42, after being kept in the
hospital the last few weeks for rehabilitation. She was
seen back in clinic a week later for follow-up and was
found to have 4/5 grip strength in the left hand with no
functional deficits.

Cerebral Venous Thrombosis

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