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Triple and Quadruple Spontaneous Cervical Artery Dissection: Presenting Characteristics and Long-Term Outcome
  1. Marcel Arnold (marcel.arnold{at}insel.ch)
  1. University Hospital of Berne, Inselspital, Switzerland
    1. Gian Marco De Marchis
    1. University Hospital of Berne, Inselspital, Switzerland
      1. Christian Stapf
      1. University Hospital Lariboisière Paris, France
        1. Ralf W Baumgartner
        1. University Hospital of Zurich, Switzerland
          1. Krassen Nedeltchev
          1. University Hospital of Berne, Inselspital, Switzerland
            1. Frédérique Buffon
            1. University Hospital Lariboisière Paris, France
              1. Aekaterini Galimanis
              1. University Hospital of Berne, Inselspital, Switzerland
                1. Heinrich P Mattle
                1. University Hospital of Berne, Inselspital, Switzerland
                  1. Marie-Germaine Bousser
                  1. University Hospital Lariboisière Paris, France

                    Abstract

                    Background: Spontaneous cervicocephalic artery dissection (sCAD) of more than two cervical arteries is rare.

                    Patients and methods: We studied vascular and potential sCAD risk factors, triggering events, clinical and neuroimaging findings, and outcome of patients with multiple sCAD. Patients were drawn from prospective hospital-based sCAD registries.

                    Results: Of 740 consecutive patients with sCAD, 11 (1.5%) had 3 and one 4 (0.1%) sCAD. Eight of these 12 patients were women. One patient had additional dissections of the celiac trunk and hepatic artery. Vascular risk factors included hypertension (n=1), hypercholesterolemia (n=6), current smoking (n=5) and migraine (n=6). No patient had a family history of sCAD, fibromuscular dysplasia (FMD) or connective tissue disease. SCAD was preceded by a minor trauma in 5 and infection in 4 patients. Clinical manifestations included ischemic stroke (n=8), transient ischemic attack (n=3), headache (n=9), neck pain (n=4), Horner syndrome (n=5), pulsatile tinnitus (n=2) and dysgeusia (n=1). Brain MRI revealed ischemic infarcts that affected one vessel territory in 7 and two territories in 2 patients. Three-month outcome was favorable (modified Rankin scale score 0-1) in 10 patients (83%). No new recurrent stroke or sCAD occurred during a mean follow-up of 50+-29 months.

                    Conclusion: Multiple sCAD occurred preferentially in women and caused clinical symptoms and signs mainly in one vascular territory. In none of the patients FMD or another underlying arteriopathy was apparent. The majority of multiple sCAD was preceded by a minor trauma or infection. Clinical outcome was favorable in most patients and long-term prognosis benign. The data suggest that transient vasculopathy may be a major mechanism for multiple sCAD.

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