Background and Purpose Spontaneous cervical artery dissection (SCAD) is a common cause of stroke in the young; however, its recurrence has been rarely studied. Recurrent SCAD at the level of the previously dissected vessel has been reported in only six patients.
Methods We prospectively evaluated and followed a series of 81 patients seen in our center with carotid (n=66) or vertebral (n=15) artery dissection, in whom the diagnosis was confirmed by angiography, MRI, or both. Repeated clinical and ultrasound examinations were performed in all patients during a mean follow-up of 34 months (range, 12 to 57 months).
Results Three patients died of the consequences of stroke, and four patients were unavailable for follow-up. Three of the remaining 74 patients (4%) had a recurrent carotid dissection while under prophylaxis with aspirin (n=2) or anticoagulation (n=1). None of the patients had a recurrent vertebral dissection. Recurrent carotid dissection occurred 16 months to 4 years after the first episode and presented with acute head and neck pain (n=2) and hemispheric transient ischemic attack (n=1). In one patient recurrence was documented by Doppler and MRI at the level of the first dissection.
Conclusions We confirm that recurrence of SCAD is uncommon and usually represents a benign condition. Delayed recurrence seems to be more common than previously suggested and can occur at the level of the previously dissected vessel.
Spontaneous cervical artery dissection is an increasingly recognized cause of stroke and represents the most common nonatherosclerotic cause of stroke in young adults.1 With the exception of migraine, most affected patients do not have vascular risk factors or identifiable signs of an underlying vasculopathy, and the cause of SCAD usually remains unclear. Among the determined or presumed causes of SCAD, fibromuscular dysplasia, vessel tortuosity, cystic medial necrosis, and Marfan's and Ehlers-Danlos syndromes have been reported.2 3 4 The predominant location of the dissections in the most mobile parts of the carotid arteries and VAs underscores the importance of a concomitant mechanical factor in the pathogenesis of SCAD.4
Recurrence of SCAD has been assessed prospectively only twice before. Schievink et al5 investigated the risk of recurrence of SCAD in 200 patients and found a recurrence in 16 patients (8%) during a mean follow-up of 7.4 years. More recently, Leys et al6 reported a recurrent dissection in 3 (3%) of 105 patients with cervical artery dissection during a mean follow-up of 36 months. In this study both traumatic and nontraumatic (spontaneous) cervical artery dissections were included.
Considering the limited data available in the literature on the prognosis of SCAD, we decided to review our experience in 81 patients who were prospectively evaluated in our center.
Subjects and Methods
Between 1989 and 1994 we evaluated and prospectively followed in our institution 81 patients with SCAD. In all patients diagnosis was established by selective catheter angiography of craniocervical vessels, MRI, or both. Clinical, ultrasound, and neuroradiological characteristics of some of these patients and diagnostic criteria for SCAD have been reported before.7 Patients received heparin during the first 5 to 10 days and were treated afterward with oral anticoagulants. Follow-up consisted of clinical evaluation and extracranial and transcranial B-mode and Doppler ultrasound studies. Clinical and Doppler ultrasound follow-up examinations were performed at 3- to 6-month intervals for at least 1 year after dissection. Patients with residual stenosis were followed until recanalization (stenosis <40%) occurred or Doppler findings had remained stable for at least 6 months. Patients and family physicians were instructed to contact us in case of onset of symptoms suggestive of redissection or new neurological symptoms. Our Neurological Department represents the only referral center for stationary neurological investigations in the canton of Bern (population, over 1 million). Oral anticoagulation was usually maintained for at least 6 months or until recanalization was documented ultrasonographically. Later patients received antiplatelet drugs, usually aspirin at a dose of 300 to 500 mg daily.
Mean±SD duration of follow-up was 33±26 months (range, 12 to 57 months) for patients with ICA dissection, 40±14 months for patients with VA dissection, and 34±24 months for the entire group.
In 66 patients (42 men and 24 women; mean±SD age, 46±9 years), the ICA was affected. In 15 patients (7 men and 8 women; mean±SD age, 44±8 years), the VA was involved. Seven patients presented with a bilateral ICA dissection, and 2 patients had a simultaneous ICA and VA dissection. Overall, there were 73 ICA dissections (41 left, 32 right) and 15 VA dissections (10 left, 5 right). There were no bilateral VA dissections.
Three patients died of the complications of the first acute stroke (mass effect), and 4 patients did not attend scheduled follow-up examinations. Recurrent dissection was documented in 3 of 74 patients and always involved the ICA. Recurrence occurred 16 months to 4 years after the first dissection and affected the contralateral ICA in 2 patients and the ipsilateral ICA in 1 patient. The case histories of these 3 patients are summarized below.
This 47-year-old woman with a history of classic migraine was admitted in August 1991 because of acute, intense, and continuous left-sided temporal and retro-orbital pain and ipsilateral eye drooping. Neurological examination revealed a left Horner's syndrome and hypesthesia in the first division of the left trigeminal nerve. Doppler and duplex ultrasound examinations and angiography (Fig 1⇓) performed 21 and 30 days after onset of symptoms, respectively, were entirely normal. However, cervical MRI documented the presence of a left ICA dissection showing a characteristic crescent intramural T1 and T2 hyperintense signal in the prepetrosal segment of the artery (Fig 2⇓). The small size of the hematoma led to only limited narrowing of the carotid artery lumen and explains the negative angiographic findings. The patient was discharged on aspirin 500 mg daily. She was seen a few months later in our clinic because of a persistent left facial pain that was improved by a tricyclic medication. The Horner's syndrome improved but never resolved completely. In December 1994 the patient underwent mastectomy for breast cancer in situ. On awakening from general anesthesia, she complained of severe and persistent left-sided headache and facial pains, which were felt to be similar to those experienced 3 years before at the time of ICA dissection. The only abnormality on the neurological examination was a left Horner's syndrome, which appeared to be more marked than on the last clinic visit. Extracranial Doppler sonography revealed a stenosis signal with increased blood flow velocities in the retromandibular high-cervical segment of the left ICA. An intimal flap was visualized on B-mode scan (Fig 3⇓). Cervical MRI demonstrated a dissection with subacute intramural hematoma in the prepetrosal segment of the left ICA (Fig 4⇓). MR angiography confirmed the presence of luminal narrowing at this level. The patient was treated initially with intravenous heparin and later with warfarin. No further events have occurred during the last 13 months.
In June 1988, this 41-year-old man was admitted for evaluation of intense left hemifacial pain and transient aphasia. Examination revealed only a left Horner's syndrome. Doppler and duplex ultrasound examinations suggested a left carotid occlusion with normal appearance of the carotid bifurcation on B-mode scan. Angiography confirmed the presence of a left carotid occlusion with flame-shaped appearance, considered to be consistent with ICA dissection. The patient was initially treated with heparin and later with oral anticoagulants. Seven months later, ultrasound studies showed complete recanalization of the previously dissected vessel. The patient remained asymptomatic until February 1993, when he again had a very intense right-sided headache and neck pain. Neurological examination was normal. Ultrasound studies revealed a right high-cervical ICA stenosis, and cervical MRI revealed the recurrence of dissection with a right prepetrosal intramural hemorrhage. Intravenous and later oral anticoagulants were resumed until stenosis had resolved on follow-up ultrasound studies, when antiplatelet agents were started. The patient had no further vascular events during the following 23 months.
In February 1992, this 39-year-old man was admitted because of a progressive left hemispheric stroke. Clinical findings included a severe right hemiparesis and a motor aphasia but no Horner's syndrome. Ultrasound studies suggested the presence of a left ICA occlusion secondary to dissection. ICA dissection was confirmed angiographically and presented as a flame-shaped occlusion of the prepetrosal segment of the left ICA. On cervical MRI the vessel wall was thickened, but there was no evident mural hemorrhage. With anticoagulant treatment the neurological deficits improved, whereas the occlusion of the left ICA persisted on follow-up ultrasound examinations. The patient was readmitted in June 1993 because of right hemispheric TIA while still anticoagulated with warfarin. There was no facial or head pain and no Horner's syndrome. Ultrasound studies documented a new mild stenosis of the right ICA and persistent left ICA occlusion. Cervical MRI was consistent with acute right prepetrosal ICA dissection. During the following 31 months, while on oral anticoagulants for the first 12 months and aspirin 500 mg daily thereafter, the patient had no further vascular events.
This study confirms that recurrence of SCAD occurs only rarely. Despite different methods to assess evolution of SCAD, the rate of 4% found in our study during a mean follow-up of 34 months is similar to the 3% found by Leys et al6 during the same observation time and the 8% reported by Schievink et al5 during a mean follow-up of 7.4 years.
In Schievink's study the recurrence rate of SCAD was higher during the first month and dropped to approximately 1%/y after the first year. However, our observations emphasize the possibility of delayed recurrences, which may occur 9 to 14 years after the first dissection.2 5 It is possible that the pathogenesis of “early” and “late” recurrences differs. A stepwise progression over a few weeks to months has been reported in SCAD as well as in intracranial dissections8 and may be related to a transient arteriopathy (eg, vasculitis5 ). Conversely, late recurrences may imply the presence of a congenital or acquired, persisting intrinsic vessel abnormality.
The rate of recurrence of SCAD is similar for the carotid arteries and VAs. Combining our study as well as the other two prospective studies reported,5 6 we counted a total of 303 initial ICA dissections with 15 recurrences and a total of 108 initial VA dissections with 5 recurrences.
Although recurrences of SCAD with severe neurological outcome have been reported,9 10 in our series recurrent SCAD was a benign condition that presented with head and facial pain, Horner's syndrome, and TIA but no permanent neurological deficits. Similarly, in the series reported by Leys et al,6 recurrence of dissection was revealed by a TIA in one patient and neck pain in the other two. In one patient described by Schievink et al, redissection was asymptomatic and was detected during angiography for follow-up of a dissecting aneurysm.5 The clinical presentations of the remaining 15 patients in this study were not specified. The oligosymptomatic course of dissections and redissections can impair their clinical recognition. Diagnosis of SCAD may occasionally be arduous even by ultrasound and angiography, particularly when a significant vessel narrowing is lacking, as illustrated by our first case report. Cervical MRI may represent the most sensitive diagnostic tool for SCAD, although subintimal dissections causing a (pseudo)occlusion may be difficult to distinguish from an intraluminal thrombus on MRI.
The risk factors for recurrence of SCAD remain essentially unknown. As discussed above, it appears reasonable to postulate that an underlying persisting arteriopathy should predispose to recurrence. Data available on this subject in the literature are contradictory, however, and the rarity of recurrent dissections in all reported series precludes any final statement. In the study of Schievink et al,5 only a younger age correlated with a higher risk for recurrence, and in all our patients recurrent SCAD was idiopathic in nature. Conversely, in the series of Leys et al,6 all recurrences occurred in patients with dissection due to fibromuscular dysplasia and Ehlers-Danlos syndrome. The discrepancy between these reports may be partly related to difficulties in the definition and detection of secondary forms of SCAD. For example, Goldstein et al11 described a patient with recurrent SCAD in whom fibromuscular dysplasia was not evident on angiography but could be diagnosed histopathologically. Ultrastructural connective tissue abnormalities may occur in patients with SCAD but without skin, joint, or skeletal abnormalities on clinical examination.12
Recurrent SCAD at the level of the previously dissected vessel is rare and has been documented before in only six patients. Bogousslavsky et al9 described a patient with recurrent ICA dissection 32 months after the initial event. D'Anglejean Chatillon et al2 observed recurrent bilateral ICA dissection in a woman 14 years after a first left-sided ICA dissection. Goldstein et al11 described a 33-year-old woman with stroke and an ICA pseudoaneurysm, which was resected and found to be associated with areas of both chronic and recent dissection. In the study of Leys et al,6 recurrent ICA dissection occurred in two patients 4 and 24 months after the first event. In the second patient the recurrent dissection followed a chiropractic treatment. A third patient had a recurrent dissection of the VA 10 months after the first dissection. Finally, our patient had a recurrent ICA dissection while awakening from general anesthesia 3 years after the first episode. With the exception of the suggestion of mechanical factors in three of four patients in whom the circumstances of local recurrence were mentioned, no other elements appear to indicate a higher risk for local recurrence of SCAD. The only reason why recurrence should preferentially affect a different vessel is scarring at the level of the first dissection. This phenomenon, however, has never been documented or proven to prevent recurrences.
SCAD predominantly affects younger patients and generally has a good prognosis. Considering the improved diagnostic sensitivity for SCAD, we can expect an increasing number of reports of recurrent SCAD. Prolonged treatment with aspirin or warfarin does not prevent recurrences, as shown in our series, but may limit secondary (embolic) cerebrovascular events. Current knowledge does not allow however recommendations for long-term treatment and prevention of recurrent SCAD.
Based on this study and a review of the literature, we conclude that recurrence of SCAD (1) is uncommon, (2) may occur more than 1 year after the first event, (3) can involve the previously dissected vessel, and (4) often runs an oligosymptomatic or asymptomatic course and may be diagnosed only as a consequence of a high degree of suspicion.
Selected Abbreviations and Acronyms
|ICA||=||internal carotid artery|
|SCAD||=||spontaneous cervical artery dissection|
|TIA||=||transient ischemic attack|
We thank Gordon A. Smith, MD, for reviewing the English used in the manuscript.
- Received February 6, 1996.
- Revision received June 18, 1996.
- Accepted June 18, 1996.
- Copyright © 1996 by American Heart Association
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