Markers of connective tissue dysplasia in cervical artery dissection and its predisposing factors

Abstract

Introduction . C ervical artery dissection (CeAD) is the most frequent cause of ischemic stroke in young adults. Arterial wall dysplasia underlies its weakness and predisposes to dissection. Objective . To assess clinical signs of connective tissue dysplasia (CTD) in patients with CeAD using special criteria of CTD, and to evaluate predisposing factors for the CeAD development. Materials and methods. We examined 80 patients (mean age 38,5±13,5; 49 females) with CeAD, verified by MRI/MRA and20 healthy volunteers . We estimated 48 signs of CTD included in the Villefranche diagnostic criteria for the vascular type of Ehlers – Danlos syndrome, the Ghent criteria for Marfan syndrome, the Beighton criteria of joint hypermobility and some others, as well as history of headache. Each sign was counted as present or absent, yielding the individual and mean CTD group scores. Results. Clinical CTD signs were more frequently detected in patients with CeAD than in controls (mean score 7.9 ± 3.6 vs. 4.6 ± 2.5; p < 0.0039). Significant signs (more than 8 points) were present in 53% of patients. Regression analysis was performed to determine diagnostic-prognostic value of CTD signs. The main diagnostic criteria included history of headache (p=0.022), arterial hypotension (р=0.012), extensive bruising (р=0.011), and widened atrophic scars (р=0.019). The additional diagnostic criteria included translucent skin (р=0.034), high palate (р=0.034), predisposition to constipation (р=0.050), nasal bleeding (р=0.043), and blue sclera (р=0.050). In the presence of the 4 main and 2 additional criteria, the predictive value of dissection according to regression model is 75 – 77% (ROC analysis: AUC 0.90, 95% CI, 0.84–0.96). Most patients 97% had various predisposing factors of CeAD development, either isolated 47% or combined 50% . Conclusions. The presence of the 4 main and 2 additional diagnostic criteria of CTD has a high predictive value of CeAD and can be used as its diagnostic-prognostic criteria. Dissection of the arterial wall with signs of dysplasia is provoked by various additional factors.