Triple and quadruple spontaneous cervical artery dissection: presenting characteristics and long-term outcome.
BACKGROUND
Spontaneous cervicocephalic artery dissection (sCAD) of more than two cervical arteries is rare.


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


RESULTS
Of 740 consecutive patients with sCAD, 11 (1.5%) had three, and one had four (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), hypercholesterolaemia (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 five and infection in four patients. Clinical manifestations included ischaemic stroke (n = 8), transient ischaemic 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 ischaemic infarcts that affected one vessel territory in seven and two territories in two patients. The 3-month outcome was favourable (modified Rankin scale score 0-1) in 10 patients (83%). No new recurrent stroke or sCAD occurred during a mean follow-up of 50 (SD 29) months.


CONCLUSIONS
Multiple sCAD occurred preferentially in women and caused clinical symptoms and signs mainly in one vascular territory. In none of the patients was FMD or any other underlying arteriopathy apparent. The majority of multiple sCAD was preceded by a minor trauma or infection. Clinical outcome was favourable in most patients, and long-term prognosis benign. The data suggest that transient vasculopathy may be a major mechanism for multiple sCAD.
Arnold
M
M
Assistance Publique, Hôpitaux de Paris, Department of Neurology, University Hospital Lariboisière, Paris. marcel.arnold@insel.ch
De Marchis
G M
GM
Stapf
C
C
Baumgartner
R W
RW
Nedeltchev
K
K
Buffon
F
F
Galimanis
A
A
Sarikaya
H
H
Mattle
H P
HP
Bousser
M G
MG
D000328:Adult; D001921:Brain / Q000098:blood supply* / Q000473:pathology*; D002545:Brain Ischemia / Q000150:complications / Q000175:diagnosis*; D002560:Cerebrovascular Circulation / Q000502:physiology; D003131:Combined Modality Therapy; D005260:Female; D005343:Fibrinolytic Agents / Q000627:therapeutic use; D006801:Humans; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D008881:Migraine Disorders / Q000175:diagnosis / Q000209:etiology; D011446:Prospective Studies; D012042:Registries; D012307:Risk Factors; D020217:Vertebral Artery Dissection / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D055815:Young Adult
D005343:Fibrinolytic Agents