Isolated cortical vein thrombosis: systematic review of case reports and case series

Background and purpose: Isolated cortical vein thrombosis is a distinct subtype of cerebral venous and sinus thrombosis. Because of the rarity of isolated cortical vein thrombosis, limited knowledge on its clinical and radiological manifestations is available.

Methods: We performed a systematic review of published data. Isolated cortical vein thrombosis had to have been diagnosed by MRI, conventional angiography, computed tomography venography, autopsy, or surgery. Cases with concurrent thrombosis of a cerebral sinus were excluded.

Results: Of 175 potentially relevant studies, 47 were included in the analysis, with a total of 116 patients. All studies were case reports and case series. Mean age was 41 years and 68% were women. The most common symptoms were headache (71%), seizures (58%), and focal neurological deficits (62%). Papilledema was not reported in any patient, and increased cerebrospinal fluid pressure was reported only in 2. Infection (19%), pregnancy or puerperium (35% of women), and oral contraceptive use (21% of women) were the most common risk factors. Most cases (73%) were diagnosed with MRI, but conventional angiography was also performed in 47%. A total of 81% had a parenchymal brain lesion and 80% were treated with anticoagulation. In-hospital mortality was 6%.

Conclusions: Signs of increased intracranial pressure seem to be less common in isolated cortical vein thrombosis compared with cerebral venous and sinus thrombosis. MRI and in some cases conventional angiography are the most frequently used diagnostic modalities and anticoagulation is the most widely used therapy.