Connective tissue and vascular phenotype in patients with cervical artery dissection

Background: Clinical observations and electron microscopic investigation of skin biopsies demonstrated connective tissue abnormalities in a sizeable proportion of patients with spontaneous cervical artery dissection (sCAD), suggesting an unknown connective tissue disorder as a risk factor for sCAD. Objective: To evaluate in a case-control setting if patients with sCAD exhibit clinical signs indicative of a connective tissue disorder or show a vascular phenotype. Methods: We investigated 43 consecutive patients with sCAD and 43 consecutive patients of similar age with ischemic stroke of other etiology. All patients underwent standardized MRI of the head and neck. The clinical investigation contained 25 items characteristic for connective tissue diseases such as hyperextensible skin, articular hypermobility, capillary fragility, and facial stigmata. A sum score counting all positive items was calculated. Additionally, the diameter of the common carotid artery (CCA) and vertebral artery (VA) and heart valve pathologies were assessed. Results: Connective tissue sum scores did not differ between the sCAD group (mean 2.37 +/- 2.1, median 2) and the control group (mean 1.95 +/- 1.9, median 2, p = 0.34). One sCAD patient had osteogenesis imperfecta (2.3%) and exhibited the highest sum score of 8. The diameter of the CCA and VA and the prevalence of heart valve pathologies did not show any significant differences between groups. Conclusion: The connective tissue and vascular phenotype did not differ significantly between patients with spontaneous cervical artery dissection (sCAD) and control subjects with ischemic stroke of other etiology. These findings argue against a clinically apparent connective tissue disorder underlying sCAD. The prevalence of known connective tissue diseases in sCAD patients is low.