Cerebral Venous ThrombosisBack to top
(Stroke. 2011 Feb 3.)
Headache, generally indicative of an increase in intracranial pressure, is the most common symptom in CVT and was present in nearly 90% of patients in the ISCVT.10 Similar headache frequency has been reported in other populations studied.63 The headache of CVT is typically described as diffuse and often progresses in severity over days to weeks. A minority of patients may present with thunderclap headache, suggestive of subarachnoid hemorrhage, and a migrainous type of headache has been described.64 Isolated headache without focal neurological findings or papilledema occurs in up to 25% of patients with CVT and presents a significant diagnostic challenge.65 CVT is an important diagnostic consideration in patients with headache and papilledema or diplopia (caused by sixth nerve palsy) even without other neurological focal signs suggestive of idiopathic intracranial hypertension. When focal brain injury occurs because of venous ischemia or hemorrhage, neurological signs and symptoms referable to the affected region are often present; most common are hemiparesis and aphasia, but other cortical signs and sensory symptoms may occur. Psychosis, in conjunction with focal neurological signs, has also been reported.66 Clinical manifestations of CVT may also depend on the location of the thrombosis (Figure 2). The superior sagittal sinus is most commonly involved, which may lead to headache, increased intracranial pressure, and papilledema.67 A motor deficit, sometimes with seizures, can also occur. Scalp edema and dilated scalp veins may be seen on examination.68 For lateral sinus thromboses, symptoms related to an underlying condition (middle ear infection) may be noted, including constitutional symptoms, fever, and ear discharge. Pain in the ear or mastoid region and headache are typical. On examination, increased intracranial pressure and distention of the scalp veins may be noted. Hemianopia, contralateral weakness, and aphasia may sometimes be seen owing to cortical involvement.69 Approximately 16% of patients with CVT have thrombosis of the deep cerebral venous system (internal cerebral vein, vein of Galen, and straight sinus), which can lead to thalamic or basal ganglial infarction. Most patients present with rapid neurological deterioration. CVT may be confused with other medical conditions. 7075 Cortical vein thrombosis is also uncommon, and specific clinical syndromes related to the larger cortical veins are rarely seen (eg, temporal lobe hemorrhage associated with vein of Labbe´ thrombosis).76
A normal D-dimer level according to a sensitiveimmunoassay or rapid enzyme-linked immunosorbentassay (ELISA) may be considered to help identify patients with low probability of CVT82,83(Class IIb; Level of Evidence B). If there is a strongclinical suspicion of CVT, a normal D-dimer levelshould not preclude further evaluation.
In patients with lobar ICH of otherwise unclearorigin or with cerebral infarction that crosses typicalarterial boundaries, imaging of the cerebral venoussystem should be performed (Class I; Level of EvidenceC).
Recommendations1. In patients with the clinical features of idiopathic intracranial hypertension, imaging of the cerebralvenous system is recommended to exclude CVT(Class I; Level of Evidence C).2. In patients with headache associated with atypicalfeatures, imaging of the cerebral venous system isreasonable to exclude CVT (Class IIa; Level of Evidence C).
may be on a continuum with IIH (pseudotumor cerebrii)
do not diagnose IIH without MRI/MRV to rule out CVT
OCPS may be a risk b/c hypercoag state
any hypercoag can lead to CVT
CVT may present with a sudden-onset headache, often associated with nausea and vomiting, clinically mimicking SAH.
CTs may be misinterpreted, and the failure to measure cerebrospinal fluid (CSF) pressure when performing an LP (which should be high in CVT) may add to the diagnostic lag.
CVT is an especially feared complication of pregnancy and the postpartum period
Treatment modalities for CVT include anticoagulation, thrombolytics, and careful observation. Antibiotics may be given if an infectious triggering event is suspected. Although anticoagulation may prevent further thrombus formation, it could theoretically cause further intracerebral hemorrhage, leading some to advocate heparin only when patients deteriorate despite symptomatic treatment.37 However, several trials support the safety and efficacy of IV heparin, even in patients with preexisting hemorrhage.180-183 The use of low molecular weight heparin results in a slightly more favorable outcome than unfractionated heparin.
The symptoms associated with CVT are quite varied. This variability stems from differences in thrombus location and acuity of thrombus formation. Headache is the primary feature of CVT in 74% to 90% of affected patients. Papilledema is noted in 45% of cases. Lethargy, decreased level of consciousness, or mental status changes may be seen. Seizures are seen in 50% of patients in the acute phase.
CT is ok for screening but need MRI/MRV for true diagnosis
Heparinize and admit
Clinical manifestations consist of headache, vomiting, focal or generalized seizure, confusion, blurred vision, focal neurologic deficits, and altered consciousness. The headache frequently precedes other symptoms, is diffuse, and often severe. The severity of symptoms correlates with the degree of thrombosis and the vessel involved. The diagnosis is confirmed by magnetic resonance imaging. 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 the use of anticoagulants [ 13]. Local thrombolysis may be indicated in rare cases unresponsive to adequate anticoagulation.Pregnancy related neuro emergencies: Seizures, ischemic stroke, cerebral vasospasm, intracranial hemorrhage, cerebral venous thrombosis, hypertensive encephalopathy, pituitary apoplexy Cerebral venous thrombosis Cerebral venous thrombosis (CVT) is an uncommon but serious disorder. Headache is the most common clinical manifestation, occurring in 80 to 88 percent [ 79,80]. The headache frequently precedes other symptoms, is typically diffuse, and often severe. Headache onset may be “thunderclap,” acute, or progressive [ 81]. Rarely, headache may be the only symptom of CVT. Other clinical manifestations can include papilledema, visual loss, focal or generalized seizures, focal neurologic deficits, confusion, altered consciousness, and coma [ 80]. The severity of symptoms correlates with the degree of thrombosis and the vessel involved. The diagnosis is confirmed by CT venography, MRI combined with MR venography, conventional angiography, surgery, or autopsy. Many cases have been linked to inherited and acquired thrombophilias, pregnancy, puerperium, infection, and malignancy [ 80]. 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 anticoagulation. Local thrombolysis may be indicated in rare cases unresponsive to adequate anticoagulation (UpToDate) pregnant-headache seizures consider CVT
chemosis, proptosis, opthalmoplegia.
Usually c CN III, IV, V, and V defects
Can be caused by tumor, fungal or bacterial infection, or vascular malformations
dural sinus in pregnant and postpartum (up to 3 months)
screen with ct, confirm with MRI.
triad of dural sinus thrombosis? is headache, papilledema, and high CSF opening pressure. MRI with magnetic resonance venography is considered the gold standard for diagnosis (Am J EM, 2/07, pg. 218).
(From Neurocrit Care Volume 11, Number 3 / December, 2009, 330-337)
Presentation The most common presenting symptom was headache, which was noted in 50/61 (82%) (Fig. 1). Headache generally progressed over days to weeks, but sudden, severe thunderclap headaches were noted in 4/61 patients (7%). Sudden severe headache did not appear to correlate with SAH, as only 1/4 of the patients with thunderclap headache had SAH. Nausea and vomiting occurred in 22/61 (36%). Neurologic deficits included focal motor or sensory deficits in 32/61 (52%), visual deficits in 17/61 (28%), and aphasia in 13/61 (21%). Overall, focal neurologic deficits were found in 44/61 (72%). Seizures occurred in 19/61 (31%), including 2/61 (3%) presenting with status epilepticus. On admission to our hospital, altered mental status was identified in 21/61 (34%), consisting of confusion in 17/61 (28%) and somnolence or coma in 4/61 (7%). Pulsatile tinnitus, a common symptom of DAVF, was a presenting symptom in only 6/61 (10%). Fig. 1 Presenting CVT symptoms/signs with and without associated ICH. Bar graphshows the number of CVT patients presenting with specific neurological symptoms and signs
Cerebral venous thrombosis patients with IPH had more severe neurological deficits on presentation than those without IPH (Fig. 1). In particular, patients with associated IPH were more likely to have depressed mental status (56% vs. 15%, P = 0.001), aphasia (41% vs. 6%, P = 0.001), seizures (44% vs. 21%, P = 0.046), and focal neurological deficits (67% vs. 41%, P = 0.048). Overall, an unfavorable admission mRS (36) was found in 88% of patients presenting with CVT and IPH, but in only 34% of patients presenting with CVT and no IPH (Fig. 3).
Risk Factors Risk factors for CVT were very common; 51/61 (84%) had at least one risk factor, 16/61 (26%) had two or more risk factors, and only 10/61 (16%) had none. The most common risk factor in women (who outnumber men in our series 2:1) was hormonal; 68% (27/40) of all women were either peripartum or actively taking either oral contraceptives or hormonal replacement therapy (Table 2). Other common acquired risk factors included underlying malignancy (13%) and infection (16%). Forty-eight patients were tested for an inherited or acquired hypercoagulable state, with 40 being evaluated for Factor V Leiden, methylenetetrahydrofolate reductase mutation (MTHFR), prothrombin 20210, protein C activity, protein S activity, antithrombin III activity, and antiphospholipid antibodies. This work-up, excluding MTHFR heterozygotes, was positive in 29% of patients (MTHFR homozygotes in 13%, prothrombin 20210 mutation in 10%, Factor V Leiden in 7% and antithrombin III, protein C and protein S deficiencies each seen in approximately 2%). Finally, traditional vascular risk factors were relatively uncommon: hypertension was present in 10/61 (16%), diabetes mellitus in 4/61 (7%), dyslipidemia in 2/61 (3%), and smoking in 5/61 (8%). None had a history of coronary artery disease.Table 2 Acquired risk factors for cerebral venous thrombosis
HRT or OCP
History of DVT
* % of women; HRT hormone replacement therapy; OCP oral contraceptive therapy; DVTdeep vein thrombosis
Cerebral Venous Thrombosis – When to Suspect it in the ED
CVT is a difficult diagnosis to suspect in the ED: Headache is the most common presenting symptom, and focal neurological signs will prompt evaluation for stroke. Since a negative head CT does not rule out CVT, when does the EP consider ordering an MRV – a test rarely ordered in the ED – which is the diagnostic study of choice?
- Several features distinguish CVT from other mechanisms of cerebrovascular disease. Given the anatomy of cerebral venous drainage, bilateral brain involvement is not infrequent. Bilateral motor signs, including paraparesis, may be present due to sagittal sinus thrombosis and bihemispheric injury. Patients with CVT often present with slowly progressive symptoms.
- An ischemic lesion on CT that crosses usual arterial boundaries (particularly with a hemorrhagic component) or in close proximity to a venous sinus is suggestive of CVT.
- CVT is an important diagnostic consideration in patients with headache and papilledema or diplopia (caused by 6th nerve palsy) even without other signs suggestive of idiopathic intracranial hypertension.
- 30% – 40% of patients with CVT present with ICH. Features suggestive of CVT as a cause of ICH include prodromal headache (which is highly unusual with other causes of ICH), bilateral parenchymal abnormalities, and clinical evidence of a hypercoagulable state. In patients with lobar ICH of otherwise unclear origin or with cerebral infarction that crosses typical arterial boundaries, imaging of the cerebral venous system should be performed.
(1) Sharma KM, et al.Â J Emerg Med 2014 Oct 15. [Epub ahead of print]
(2) Saposnik G, et al.Â StrokeÂ 2011;42: 1158-1192.