Introduction: Cervical artery dissection (CeAD) is the most frequent cause of ischemic stroke in young adults. It is suggested that connective tissue dysplasia (CTD) underlies in the arterial wall weakness predisposing to dissection. The aim: to elucidate CTD signs that have prognostic and diagnostic significance for CeAD.
Methods: We examined 80 patients (mean age 38,5±13,5; 49 females, 61,2%) with CeAD, verified by MRI/MRA and 20 healthy volunteers (mean age 30,3±6,7; 12 females 60%). We assessed 48 signs selected from criteria of the vascular type of Ehlers-Danlos syndrome, Marfan syndrome, Beighton criteria of joint hypermobility and other. In addition we estimated arterial hypotension (≤100/70 mm Hg), and headache in the past history. Each sign was counted as present or absent, resulting in an individual CTD score. On the basis of linear regression analysis these signs were divided into major and minor signs.
Results: CTD signs were found more frequently in CeAD patients than in the control (mean score 7,9 ± 3,6 vs 4,6 ± 2,5; p<0.0039). The following CTD signs were significantly more often in patients than in control: arterial hypotension (51% vs 20%, Р=0,018), extensive bruising (40% vs 10%, Р=0,016), widened atrophic scars (23% vs 0%, Р=0,02), headache in the past history (60% vs 35%, p=0,027) - major signs; translucent skin (29% vs 5%, Р=0,037), hight palate (20% vs 0%, Р=0,037), nasal bleeding (34% vs 15%, Р=0,044), blue sclera (20% vs 5%, Р=0,05), propensity to constipation (30% vs 0%, Р=0,05) - minor signs. If there were 4 major and 2 small signs logistic regression model gave 75% predictive value of dissection. The reliability of these signs was confirmed by ROC analysis (AUC 0,90 [95% CI 0,84-0,96], sensitivity - 86%, specificity - 85%).
Conclusion: CTD signs are highly prevalent in patients with CeAD that is in accordance with our morphological data that arterial wall dysplasia is a main cause of spontaneous dissection. The presence of 4 major and 2 minor signs may be used to estimate the predisposition to CeAD and in some cases as additional argument at clinical diagnosis.