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Cerebral venous thrombosis

Key Points

  • Cerebral venous thrombosis (CVT) is an important cause of stroke in young and middle-aged adults, with a sex ratio heavily skewed towards women

  • Manifestations of CVT can be grouped into four distinct clinical syndromes: isolated intracranial hypertension, focal syndrome, diffuse encephalopathy and cavernous sinus syndrome

  • First-line treatment for CVT is heparin, even in the presence of an intracerebral haemorrhage

  • In a trial completed in 2017, endovascular therapy did not improve the clinical outcome of patients with severe CVT

  • Mortality among patients with CVT has declined in the past few decades to 8–10%; although 80% of patients recover without physical disability, many experience residual chronic symptoms

Abstract

Cerebral venous thrombosis (CVT) is an important cause of stroke in young adults. Data from large international registries published in the past two decades have greatly improved our knowledge about the epidemiology, clinical manifestations and prognosis of CVT. The presentation of symptoms is highly variable in this disease, and can range from a patient seen at the clinic with a 1-month history of headache, to a comatose patient admitted to the emergency room. Consequently, the diagnosis of CVT is often delayed or overlooked. A variety of therapies for CVT are available, and each should be used in the appropriate setting, preferably guided by data from randomized trials and well-designed cohort studies. Although deaths from CVT have decreased in the past few decades, mortality remains 5–10%. In this Review, we provide a comprehensive and contemporary overview of CVT in adults, with emphasis on advancements made in the past decade on the epidemiology and treatment of this multifaceted condition.

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Figure 1: Anatomy of the cerebral venous system.
Figure 2: Imaging findings in a patient with cerebral venous thrombosis.
Figure 3: Illustrative brain parenchymal lesions in patients with cerebral venous thrombosis.
Figure 4: Schematic overview of diagnostic and therapeutic steps in CVT.

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S.M.S. and D.A.d.S. researched the data for the article and co-wrote the first draft. J.M.F. and J.M.C. made substantial contribution to discussion of the context an edited the manuscript before submission. J.M.C. supervised the preparation of the manuscript.

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Correspondence to Jonathan M Coutinho.

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Competing interests

J.M.F. has received personal fees from Boehringer Ingelheim and Daiichi Sankyo. J.M.C. has received research grants for CVT from two non-profit organizations: the Dutch Thrombosis Society and the Netherlands Brain Foundation, and is a steering committee member of the RE-SPECT CVT trial, a clinical trial that evaluates the efficacy and safety of dabigatran for the treatment of CVT sponsored by Boehringer Ingelheim.

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Silvis, S., de Sousa, D., Ferro, J. et al. Cerebral venous thrombosis. Nat Rev Neurol 13, 555–565 (2017). https://doi.org/10.1038/nrneurol.2017.104

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