Elsevier

The Lancet Neurology

Volume 6, Issue 2, February 2007, Pages 162-170
The Lancet Neurology

Review
Cerebral venous thrombosis: an update

https://doi.org/10.1016/S1474-4422(07)70029-7Get rights and content

Summary

Cerebral venous thrombosis (CVT) is a rare type of cerebrovascular disease that can occur at any age, including in neonates, and it accounts for 0·5% of all stroke. The widespread use of neuroimaging now allows for early diagnosis and has completely modified our knowledge on this disorder. CVT is more common than previously thought and it is recognised as a non-septic disorder with a wide spectrum of clinical presentations, numerous causes, and usually a favourable outcome with a low mortality rate. MRI with T1, T2, fluid-attenuated inversion recovery, and T2* sequences combined with magnetic resonance angiography are the best diagnostic methods. D-dimer concentrations are raised in most patients but normal D-dimers do not rule out CVT, particularly in patients who present with isolated headache. Heparin is the first-line treatment, but in a few cases more aggressive treatments, such as local intravenous thrombolysis, mechanical thrombectomy, and decompressive hemicraniectomy, may be required.

Introduction

Cerebral venous thrombosis (CVT)—ie, thrombosis of the intracranial veins and sinuses—is a rare type of cerebrovascular disease that affects about 5 people per million and accounts for 0·5 % of all stroke. CVT was first recognised at the beginning of the 19th century1 and it was long thought to be an infective disorder that commonly affected the superior sagittal sinus and resulted in bilateral or alternating focal deficits, seizures, and coma, which usually led to death. At this time, CVT was commonly diagnosed at autopsy and usually showed haemorrhagic lesions, which, by analogy with arterial stroke, was thought to contraindicate the use of heparin.2, 3 In the past 25 years, the widespread use of neuroimaging has aided early diagnosis of CVT and has thus completely modified the current information we have on this disorder. CVT is now typically recognised as a non-septic disorder with various clinical presentations and a usually favourable outcome, with mortality well below 10%. MRI and magnetic resonance angiography are the best diagnostic methods for diagnosis and heparin is the first-line treatment.4, 5, 6, 7, 8 However, the diagnosis of CVT is still commonly overlooked or delayed because of the remarkable diversity of its clinical symptoms, modes of onset, and neuroimaging signs; furthermore, a cause cannot be found in about 15% of cases, the individual outcome may still be difficult to predict, and the disorder may occasionally worsen despite anticoagulation. CVT thus remains a diagnostic and therapeutic challenge. In recent years, some progress has been made either from individual studies that focused on specific clinical, MRI, genetic, or therapeutic aspects or from the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT)—a multicentre prospective cohort of 624 adult patients.9

Section snippets

Aetiology

Several disorders can cause, or predispose patients to, CVT (figure 1). These disorders include all medical, surgical, and gyneco-obstetric causes of deep vein thrombosis in the legs, genetic and acquired prothrombotic disorders, cancer, haematological diseases, vasculitis and other inflammatory systemic disorders, pregnancy and puerperium, infections, as well as several local causes—such as brain tumours, arteriovenous malformations, head trauma, CNS infections, and infections of the ear,

Clinical aspects

CVT presents with a remarkably wide spectrum of signs and modes of onset, thus mimicking numerous other disorders. The most common symptoms and signs are headache, seizures, focal neurological deficits, altered consciousness, and papilloedema, which can present in isolation or in association with other symptoms. According to the grouping of symptoms and signs, four main patterns have been identified: isolated intracranial hypertension, focal syndrome, cavernous sinus syndrome, and subacute

Neuroimaging of the thrombosed vessel

The diagnosis of CVT is based on neuroimaging but, in contrast to arterial strokes, brain imaging by itself is of little positive value because it usually shows non-specific lesions, such as haemorrhages, infarcts, or oedema in isolation or in combination, and it can be normal in up to 25% of patients. The key to the diagnosis is the imaging of the venous system itself, which may show the occluded vessel or the intravascular thrombus. The current gold standard is the combination of MRI to

Prognosis

A meta-analysis of several recent prospective series, in particular the large ISCVT cohort,9, 55, 56, 57, 58, 59, 60 confidently established the vital and functional prognosis of patients with acute CVT, showing a 15% overall death or dependency rate. Long-term predictors of poor prognosis are CNS infection, any type of cancer, deep venous system thrombosis, intracranial haemorrhage, Glasgow Coma Scale score on admission of greater than nine, mental status disorder, being older than age 37

Acute treatment

Recent guidelines65 have been published for the treatment of CVT, which combines causal treatment to specifically manage the various causes, antithrombotic treatment, and symptomatic treatment—ie, treatment of intracranial hypertension, seizures, headache, and visual failure (panel 2).

The aims of antithrombotic treatment in CVT are to recanalise the occluded sinus or vein, to prevent the propagation of the thrombus, and to treat the underlying prothrombotic state—in order to prevent venous

CVT in neonates

CVT in children has long been recognised3 but it is only recently that large neuroimaging-based series have been reported.79, 80, 81, 82, 83, 84, 85, 86, 87, 88 The disorder affects all age groups with an estimated incidence of 0·67 per 100 000 per year and a preponderance in neonates.79 In older children, CVT shares many similarities with adult CVT except for a slight male preponderance, and an increased chance of infective cause and worse functional prognosis.80, 81 However, in neonates the

CVT in elderly patients

CVT in elderly people has received little attention. In ISCVT, 8·2% of the patients were age 65 years or older.89 They differed in some respects from CVT in younger patients: there was less frequent isolated intracranial hypertension, more frequent depressed consciousness and mental status changes, and carcinoma was more frequent as a cause. Prognosis was worse with 49% of patients dead or dependent at the end of follow-up. CVT should thus be added to the long list of disorders that cause

The near future of clinical research in CVT

There are still many unsolved issues in the pathophysiology, diagnosis, and management of CVT. Because of the small number of cases diagnosed each year, even at reference centres, cooperation between a network of interested centres is essential. The participation of hospitals from developing countries is particularly welcome to enable a global picture of CVT. The identification of new gene polymorphisms in the coagulation and fibrinolytic system, their role in CVT, and their interaction with

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