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It has long been appreciated that the process formerly known as Erdheim s medionecrosis aortica cystica, the main cause of aortic dissection, may extend into the common carotid arteries, occluding them and causing massive infarction of the cerebral hemispheres Examples of such an occurrence were cited by Weisman and Adams in 1944 in their study of the neurology of dissecting aneurysms of the aorta In more recent years, attention has been drawn to the occurrence of spontaneous dissection of the internal carotid artery and the fact that it is an important cause of hemiplegia in young adults Several large series of such cases have been reported in separate studies by Ojemann, Mokri, and Bogousslavsky and their colleagues Traumatic or apparently spontaneous carotid dissection is a not uncommon cause of stroke, particularly in younger individuals Bogousslavsky and colleagues found 30 instances (comprising both genders) in 1200 consecutive patients with a rst stroke (25 percent) It should be suspected in young adult women (typically in their late thirties or early forties), who seem especially susceptible to the condition, either as a spontaneous event or in relation to a whiplash injury, bouts of violent coughing, or direct trauma to the head or neck, which need not be severe eg, being struck in the neck by a golf or tennis ball We have also encountered cases that occurred during pregnancy and immediately after delivery Indeed, it is questionable if many cervical arterial dissections are truly spontaneous, since most can be connected to some strenuous event Three of our patients over the years had a carotid dissection that was manifest as a hemiplegia days after blunt head injury A small number of patients have bromuscular disease, as discussed above; the Ehlers-Danlos and Marfan syndromes, osteogenesis imperfecta, and alpha-1-antitrypsin de ciency are also associated with an increased risk of vascular dissection One of these conditions should be suspected if multiple extracranial vessels are involved in spontaneous dissections (neck and thoracic trauma are more common causes of this con guration) It is of interest that a few patients with carotid dissection have had warning attacks of unilateral cranial or facial pain, followed, within minutes to days, by signs of ischemia in the territory of the internal carotid artery The pain is nonthrobbing and centered most.

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often in and around the eye; less often, it is in the frontal or temporal regions, angle of the mandible, or high anterior neck over the carotid artery Rapid and marked relief of the pain after the administration of corticosteroids is virtually a diagnostic feature (see below) The ischemic manifestations consist of transient attacks in the territory of the internal carotid, followed frequently by the signs of hemispheral stroke, which may evolve smoothly over a period of a few minutes to hours or over several days in a uctuating or stepwise fashion A unilateral Horner syndrome is often present Cervical bruit sometimes audible to the patient, amaurosis fugax, faintness and syncope, and facial numbness are less common symptoms Most of the patients described by Mokri and coworkers presented with one of two distinct syndromes: (1) unilateral headache associated with an ipsilateral Horner syndrome or (2) unilateral headache and delayed focal cerebral ischemic symptoms Some patients have evidence of involvement of the vagus, spinal accessory, or hypoglossal nerve; these nerves lie in close proximity to the carotid artery and are nourished by small branches from it In most cases, dissection of the internal carotid artery can be detected by ultrasound and con rmed by MRI, which shows a double lumen, and by MRA These procedures may obviate the need for arteriography The latter procedure reveals an elongated, irregular, narrow column of dye, usually beginning 15 to 3 cm above the carotid bifurcation and extending to the base of the skull, a picture that Fisher has called the string sign There may be a tapered occlusion or an outpouching at the upper end of the string Less often the dissection is con ned to the midcervical region, and occasionally it extends into the middle cerebral artery or involves the opposite carotid artery or the vertebral and basilar arteries The extensive study by Mokri and colleagues showed that a complete or excellent recovery occurred in 85 percent of patients with the angiographic signs of dissection; mainly, these were patients without stroke The outcome in cases complicated by stroke is far less benign About one-quarter of such patients succumb and one-half of the survivors remain seriously impaired (Bogousslavsky et al) In the remainder, early recanalization of the occluded artery can be observed (as determined by ultrasonography), with good functional recovery Pseudoaneurysms form in a small proportion of patients and generally do not require surgical repair; they also do not preclude cautious anticoagulation The pathogenesis of spontaneous carotid dissection is at present uncertain In most of the recently reported cases, cystic medial necrosis has not been found on microscopic examination of the involved artery In some, there was a disorganization of the media and internal elastic lamina, but the speci city of these changes is in doubt, since Ojemann and colleagues noted similar changes in some of their control cases In a small proportion of cases there are the changes of bromuscular dysplasia, as noted earlier A more thorough study of these vessels in routine autopsy material is needed Treatment The treatment has usually been immediate anticoagulation to prevent embolism using rst heparin, then warfarin but it must be acknowledged that this approach has not been demonstrated to be more successful than careful observation Once a stroke has occurred, even though embolic in most cases, prompt reopening of the artery can at times prove bene cial; this is currently performed by endovascular techniques Despite numerous publications demonstrating the ability of skilled operators to reopen a dissection by endovascular methods, acute intervention has not been studied in a way that allows a judgment regarding its value Whether stent placement is additionally bene cial has also not been.