Cerebral venous thrombosis (CVT) is a rare manifestation of venous thromboembolism (VTE) and stroke. The aim of our systematic review was to provide an updated summary of the strength of association between CVT and thrombophilia and to explore the relevance of thrombophilia for recurrence of CVT or other VTE, or other outcome variables. MEDLINE (via PubMed), EMBASE (via Ovid), and CENTRAL were systematically searched, including references of retrieved articles. Cohort studies of ≥ 40 patients and case-control studies comparing the prevalence of thrombophilia in patients with CVT and unrelated controls were eligible. Two reviewers independently selected studies, assessed quality, and extracted data. A meta-analysis was performed for high quality case-control studies with unselected cases and healthy controls. Odds ratios with 95% confidence intervals were calculated and pooled. We included 23 cohort studies and 33 case-control studies. A significant association was demonstrated between CVT and all inherited thrombophilic factors, as well as increased levels of homocysteine. Inconclusive results were found on the relevance of thrombophilia for recurrent CVT or other VTE. Although there is a strong association between CVT and thrombophilia, the clinical relevance of thrombophilia testing in patients with CVT seems limited, similarly to other forms of VTE.
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