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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.

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determined Interesting, and of both therapeutic and diagnostic value, is the relief of pain afforded by corticosteroids in cervical and intracranial dissections, as mentioned above Most neurologists take the approach that warfarin, if used, may be discontinued after several months or a year, when angiography or MRA shows the lumen of the carotid artery to be patent, or at least reduced to no more that 50 percent of the normal diameter, and smooth-walled Vertebral Artery Dissection Dissection of these arteries is less common than dissection of the extracranial carotid artery but is being recognized with increasing frequency It may originate in the neck and extend into the intracranial portion of the vessel or remain isolated to either of these segments In both instances there is a tendency to form pseudoaneurysms, but this is more likely with the intracranial type, and only in the latter is there a risk of rupture through the adventitia, leading to a subarachnoid hemorrhage Rapid and extreme rotational movement of the neck is the most common identi able cause, as in turning the head to back up a car Chiropractic manipulation has been one precipitant Extending the neck to have one s hair washed, and swinging a golf club have also led to dissection Forceful coughing may also cause dissection, as in the carotid vessels There is no female predominance (in contrast to carotid dissection), but the previously cited intrinsic weaknesses of the vascular wall from Ehlers-Danlos disease and bromuscular dysplasia are risk factors The dissection usually originates in the C1-C2 segment of the vessel, where it is mobile but tethered as it leaves the transverse foramen of the axis and turns sharply to enter the cranium The symptoms, mainly vertigo, derive from the lateral medullary syndrome, often with additional features referable to the pons or midbrain, particularly diplopia and dysarthria The clinical manifestations in our experience have uctuated over minutes and hours, quite unlike the usual vertebrobasilar TIA Less common strokes include artery-to-artery embolism to the posterior cerebral territory or, a syndrome that has come to our attention several times in the past few years, a centrally placed infarction of the cervical spinal cord, presumably from occlusion of the anterior spinal artery The diagnosis of vertebral dissection should be suspected if persistent occipitonuchal pain is prominent and follows one of the known precipitants such as chiropractic manipulation of the neck, head trauma, or Valsalva straining or coughing activities but it may otherwise escape detection until the full-blown medullary or cerebellar stroke is established The latter may follow the inciting event by several days or weeks or even longer, obscuring the relationship Axial MRI images, particularly the T1-weighted sequences, show a double lumen in the dissected vessel, and skillful ultrasound investigation documents the same Mokri et al have found that some patients have evidence of spontaneous or traumatic dissection of multiple extracranial vessels; this also occurs as a consequence of dissection of the aortic arch from chest trauma Treatment This has usually been with heparin anticoagulation followed for a period by warfarin, but the precise duration of treatment is dif cult to determine and the same uncertainties as to effectiveness of anticoagulation and of stent placement discussed above in regard to carotid dissection pertain here Whether there is an inordinate risk of subarachnoid hemorrhage with intracranial dissection has not been settled The usual practice is to repeat an imaging or ultrasound study several months after the dissection and discontinue the anticoagulation if the vessel lumen has been reestablished suf ciently to allow good blood ow Pseudoaneu-.

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