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Carotid artery dissection is a significant cause of stroke in young patients.
Arterial dissection of the carotid arteries occurs when a small tear forms in the innermost lining of the arterial wall (known as the tunica intima). Blood can enter into the space between the inner and outer layers of the vessel, causing narrowing (stenosis) or complete occlusion. The stenosis that occurs in the early stages of arterial dissection is a dynamic process and some occlusions can return to stenosis very quickly.[1] When complete occlusion occurs, it may lead to ischemia. Often, even a complete occlusion is totally asymptomatic because bilateral circulation keeps the brain well perfused. However, when blood clots form and break off from the site of the tear, they form emboli, which can travel through the arteries to the brain and block the blood supply to the brain, resulting in an ischemic stroke, otherwise known as an infarction. Blood clots, or emboli, originating from the dissection are thought to be the cause of infarction in the majority of cases of stroke in the presence of carotid artery dissection.[1] Cerebral infarction causes irreversible damage to the brain. In one study of patients with carotid artery dissection, 60% had infarcts documented on neuroimaging.[2]
The cause of internal carotid artery dissection can be broadly categorized into two classes: spontaneous or traumatic.
Once considered uncommon, spontaneous carotid artery dissection is an increasingly recognized cause of stroke that preferentially affects the middle-aged.[3]
The incidence of spontaneous carotid artery dissection is low, and incidence rates for internal carotid artery dissection have been reported to be 2.6 to 2.9 per 100,000.[4]
Observational studies and case reports published since the early 1980s show that patients with spontaneous internal carotid artery dissection may also have hereditary connective tissue disorders and/or a history of stroke in their family. These include Marfan syndrome, vascular Ehlers-Danlos syndrome, autosomal dominant polycystic kidney disease, pseudoxanthoma elasticum, fibromuscular dysplasia, and osteogenesis imperfecta type I.[5]
However, the reports on the prevalence of hereditary connective tissue diseases in people with spontaneous dissections is highly variable, ranging from 0% to 0.6% in one study to 5% to 18% in another study. Nevertheless, although an association with connective tissue disorders does exist, most people with spontaneous arterial dissections do not have associated connective tissue disorders.[6]
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