Cerebral Venous Thrombosis and Anticardiolipin Antibodies

Abstract

Background and Purpose The association of cerebral venous thrombosis (CVT) with a variety of pathological states is well established. However, there are only rare isolated reports of CVT associated with anticardiolipin antibodies (aCL). Methods To clarify the clinical and neuradiological features as well as outcome of patients with CVT associated with aCL, we reviewed the records of all patients with CVT evaluated at our institution between 1989 and 1996 (retrospective and prospective) and systematically reviewed the pertinent literature. Results We identified 8 aCL+ and 7 aCL− patients with CVT. No patients with lupus anticoagulant (LA) were identified. The mean age was 23±11.01 (range, <1 to 36) years in the aCL+ and 38±9.30 (range, 25 to 54) years in the aCL− patients ( P =.016). Six of 8 aCL+ and 5 of 7 aCL− patients were women. The dural sinuses were involved in all aCL+ and in 6 of 7 aCL− patients, while deep venous system thrombosis occurred in 5 of 8 (63%) aCL+ and 1 of 7 (14%) aCL− patients. In the aCL+ patients CVT was associated with puerperium or oral contraceptive use (n=6) and sickle cell trait (n=1), and in the aCL− patients CVT was associated with systemic lupus erythematosus (n=1), myelodysplastic syndrome (n=1), colonic cancer (n=1), oral contraceptive use or puerperium (n=3), and dehydration (n=1). Seven aCL+ patients received either intrasinus urokinase or intravenous heparin sulfate, and 1 received aspirin. Four aCL+ patients developed new onset or worsening of preexisting migraine, 2 developed recurrent peripheral venous thrombosis, and 1 went on to have intracranial hypertension. Twenty additional patients with CVT associated with antiphospholipid antibodies (aPL) were found reported in the literature. The overall mean age was 36±11.6 (range, 21 to 62) years, and 14 (70%) were women. LA was present in 11 of 18 tested, aCL in 7 (35%), LA and aCL in 1, and the type of aPL was not reported in 3. The mean age for the aCL+ only group was 28 years and for the LA+ (with or without aCL+) was 34 years. Only 1 patient, whose aPL type was not specified, had thrombosis of the deep venous system in addition to involvement of the dural sinuses. Conclusions Our series and review suggest that aCL may be an important factor contributing to development of CVT even in the presence of other potential etiologies or risk factors. Onset of aCL+ CVT occurs at a relative young age and with relatively more extensive superficial and deep cerebral venous system involvement than aCL− CVT.