Original clinical investigation

Benign intracranial hypertension associated to blood coagulation derangements

Domenico De Lucia¹ , Marisanta Napolitano¹ , Pierpaolo Di Micco^2,3 , Alferio Niglio⁴ , Andrea Fontanella² and Giuseppe Di lorio⁵

¹  Division of Pathology, Second University of Naples, Naples, Italy

²  Internal Medicine Division, Buonconsiglio Fatebenefratelli Hospital of Naples, Naples, Italy

³  Biochemistry and Biotechnology Department and Ceinge Scarl, "Federico II", University of Naples, Naples, Italy

⁴  Division of Internal Medicine Second University of Naples, Naples, Italy

⁵  Center for Migraine; Second University of Naples, Naples, Italy

author email corresponding author email

Thrombosis Journal 2006, 4:21doi:10.1186/1477-9560-4-21

Published:	24 December 2006

Abstract

Background

Benign Intracranial Hypertension (BIH) may be caused, at least in part, by intracranial sinus thrombosis. Thrombosis is normally due to derangements in blood coagulation cascade which may predispose to abnormal clotting activation or deficiency in natural inhibitors' control. The aim of the study is to examine the strength of the association between risk factors for thrombosis and BIH.

Patients and methods

The incidence of prothrombotic abnormalities among a randomly investigated cohort of 17 patients with BIH, was compared with 51 healthy subjects matched for sex, age, body mass index, height and social background.

Results

The number of subjects with protein C deficiency was significantly higher in patients than in controls (3 vs 1, p < .001; Fisher Exact Test). Moderate to high titers of anticardiolipin antibodies (β2-Glycoprotein type I) were found in 8 out of 17 patients.

Increased plasma levels of prothrombin fragment 1+2, fibrinopeptide A (FPA), and PAI-1 were demonstrated in patients group (5.7 ± 1.15 nM vs 0.45 ± 0.35 nM; 8.7 ± 2.5 ng/mL vs 2.2 ± 1.25 ng/mL; 45.7 ± 12.5 ng/mL vs 8.5 ± 6.7 ng/mL, respectively; p < .001; Fisher Exact Test). Gene polymorphisms for factor V Leiden mutation, prothrombin mutation 20210 A/G, MTHFR 677 C/T, PAI-1 4G/5G, ACE I/D were detected in 13 patients.

Discussion

In agreement with other authors our data suggest a state of hypercoagulability in BIH associated with gene polymorphisms. Our findings also showed that mutations in cardiovascular genes significantly discriminate subjects with a BIH history. The association between coagulation and gene derangements, usually regarded to as cryptogenic, may suggest a possible pathogenetic mechanism in BIH. So, a prothrombotic tendency may exist that would, at least in part, explain some cases of BIH.

Although based on a small population, these findings raise the exciting possibility of using these haemostatic factors as markers for selecting high-risk subjects in BIH disease.