Markers of Connective Tissue Dysplasia in Cervical Artery Dissection and Its Predisposing Factors

Abstract

Introduction: Cervical artery dissection (CeAD) is the most frequent cause of ischemic stroke in young adults. Dysplasia of arterial wall 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 and 20 healthy volunteers. We estimated 48 signs of CTD included into 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 ≤ 0.05. 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 and prognostic criterion. Dissection of the arterial wall with signs of dysplasia is provoked by various additional factors.