Recurrent Cervical Artery Dissection Research Articles

Recurrence of cervical artery dissection: A systematic review and meta-analysis.

Cervical artery dissection (CAD) involving the carotid or vertebral arteries is an important cause of stroke in younger patients. The purpose of this systematic review is to assess the risk of recurrent CAD. A systematic review and meta-analysis was conducted on studies in which patients experienced radiographically confirmed dissections involving an extracranial segment of the carotid or vertebral artery and in whom CAD recurrence rates were reported. Data were extracted from 29 eligible studies (n = 5898 patients). Analysis of outcomes was performed by pooling incidence rates with random effects models weighting by inverse of variance. The incidence of recurrent CAD was 4% overall (95% confidence interval (CI) = 3-7%), 2% at 1 month (95% CI = 1-5%), and 7% at 1 year in studies with sufficient follow-up (95% CI = 4-13%). The incidence of recurrence associated with ischemic events was 2% (95% CI = 1-3%). We found low rates of recurrent CAD and even lower rates of recurrence associated with ischemia. Further patient-level data and clinical subgroup analyses would improve the ability to provide patient-level risk stratification.

Cervical Artery Dissections: Etiopathogenesis and Management.

Cervical Artery Dissection (CeAD) is a frequent stroke etiology for patients younger than 50 years old. The most common immediate complications related to CeAD are headache and neck pain (65–95%), TIA/ischemic stroke (>50%), and partial Horner’s syndrome (25%). The prevailing hypothesis regarding the pathogenesis of sCeAD is that the underlying constitutional vessel wall weakness of patients with sCeAD is genetically determined and that environmental factors could act as triggers. The stroke prevention treatment of CeAD remains controversial, involving anticoagulation or antiplatelet therapy and potentially emergent stenting and/or thrombectomy or angioplasty for selected cases of carotid artery dissection with occlusion. The treatment of headache associated with CeAD depends on the headache phenotype and comorbidities. Radiographically, more than 75% of CeAD cases present with occlusion or non-occlusive stenosis. Many patients demonstrate partial and complete healing, more commonly in the carotid arteries. One-fifth of the patients develop dissecting pseudoaneurysm, but this is a benign clinical entity with an extremely low rupture and stroke recurrence risk. Good recovery is achieved in many CeAD cases, and mortality remains low. Family history of CeAD, connective tissue disorders like Ehlers-Danlos syndrome type IV, and fibromuscular dysplasia are risk factors for recurrent CeAD, which can occur in 3–9% of the cases. This review serves as a comprehensive, updated overview of CeAD, emphasizing etiopathogenesis and management.

Antithrombotic therapy in the postacute phase of cervical artery dissection: the Italian Project on Stroke in Young Adults Cervical Artery Dissection

ObjectiveTo explore the impact of antithrombotic therapy discontinuation in the postacute phase of cervical artery dissection (CeAD) on the mid-term outcome of these patients.MethodsIn a cohort of consecutive patients with...

Vertebral Artery Tortuosity and Morphometric Characteristics of Patients with Recurrent Cervical Artery Dissection

BackgroundCervical Artery Dissection is an important cause of stroke in the young. Data on incidence and associations of recurrence in patients with cervical artery dissection are lacking. Increased Vertebral Artery Tortuosity Index has been reported in patients with cervical artery dissection and associated with earlier age of arterial dissection in patients with connective tissue disease. ObjectiveTo test the hypothesis that increased vertebral artery tortuosity is associated with recurrent cervical artery dissection. MethodsWe reviewed data from a single-center registry of cervical artery dissection patients enrolled between 2011-2021. CT angiography was reviewed for neck length, vertebral artery dominance, and vertebral artery tortuosity index. Incidence rate of recurrent dissection was calculated using Poisson regression. Differences between groups were analyzed using the Kruskal-Wallis rank sum test and Fisher's exact test. ResultsThe cohort included 155 patients: women (56%), mean (SD) age 42 (±10) years, and 116 single and 39 multiple artery dissections. Eleven (7.1%) had a recurrence with an incidence rate (95% CI) of 1.91 (1.06, 3.44) per 100 person-years. Vertebral artery tortuosity did not differ significantly between single and recurrent groups (median (IQR) 46.81 (40.85, 53.91) vs 44.97 (40.68, 50.62) p = 0.388). Morphometric characteristics of height, neck length, and BMI were not associated with recurrence. There was no difference in vertebral artery tortuosity by dissection location (carotid vs vertebral). ConclusionIn this single center cohort of patients with cervical artery dissection, there was no difference in VTI between single and recurrent groups.

Abstract TP103: Vertebral Artery Tortuosity And Morphometric Characteristics In Recurrent Cervical Artery Dissection

Background: Cervical Artery Dissection (CeAD) is an important cause of stroke in the young. Data on incidence and associations of recurrent CeAD are lacking. Increased Vertebral Artery Tortuosity Index (VTI) has been reported in patients with CeAD and associated with earlier age of arterial dissection in patients with connective tissue disease. We hypothesized that VTI may be associated with recurrent CeAD. Methods: We reviewed data from a large single-center registry of CeAD patients enrolled between 2011-2021. CT angiography (CTA) was reviewed for neck length, vertebral artery length (VAL), linear distance from the vertebral artery origin to the vertebrobasilar junction (VOBJ), and VTI [((VAL/VOBJ)–1)*100]. Recurrence was defined as radiographic diagnosis of a new dissection during a follow-up period between initial CTA imaging and July 1, 2021. Incidence rate of recurrent dissection was calculated using Poisson regression. Differences between groups were analyzed using the Kruskal-Wallis rank sum test and Fisher’s exact test. Results: The cohort included 155 patients: women (56%), mean (SD) age 42 (±10) years, and 116 single (carotid or vertebral) and 39 multiple artery dissections. Eight (5%) had a recurrence with a 10 year incidence rate (95% CI) of 1.23 events (0.53, 2.38) per 100 person-years. The recurrent group had a lower mean VTI than the nonrecurrent group (39.2 vs. 45.8; p = 0.024) and trended towards a lower mean age (34.6 vs. 42.3; p = 0.058). Participants with vertebral artery dissection were younger than those with carotid artery dissection with mean ages of 38.5 and 45.1 respectively (p < 0.001) and did not differ in mean VTI (46.0 vs. 44.9; p = 0.747). Morphometric characteristics of height, neck length, and BMI were not associated with recurrence. Conclusion: In this single center cohort of patients with CeAD, greater VTI was associated with a decreased risk of recurrent dissection. This finding is hypothesis generating as adults with recurrent dissections may have distinct mechanistic or genetic factors contributing to their risk of dissection. This study is limited by relatively small sample size and the retrospective analysis. Further study of VTI and risk of incident CeAD in larger independent cohorts is warranted.

Cervical Artery Dissection in Young Women: Risk of Recurrence During Subsequent Pregnancies.

To assess the risk of recurrence of cervical artery dissection (CAD) during pregnancy and puerperium in women with a history of CAD and then help physicians with providing medical information to women who wish to become pregnant. Women aged 16-45 years who were admitted to our center for a CAD between 2005 and 2017 were identified from the hospital database, and those with spontaneous and symptomatic CAD were included. They were then contacted to answer a questionnaire that was specifically designed in regard to the recurrence of CAD and pregnancies after the primary CAD. Ninety-one patients satisfied our inclusion criteria, and 89 were included in the analysis. During a median follow-up of 7.0 years, 4 women (4.4%) had recurrent CAD, although none during pregnancy or puerperium. Eighteen women (20%) had a total of 20 full-term pregnancies, occurring at least 6 months after CAD. Of these 20 pregnancies, 13 (65%) were vaginal deliveries, and 7 (35%) were cesarean sections. The reason for the absence of pregnancies after the initial CAD was unrelated to the vascular event in 89% of cases, but 8% of the women had been advised by a physician to avoid any future pregnancy or they had been recommended to undergo abortion or sterilization. In this study, there were no CAD recurrences during subsequent pregnancies or postpartum, irrespective of the type of delivery. Thus, pregnancy after a history of CAD appears to be safe.

Recurrence of cervical artery dissection: protocol for a systematic review

IntroductionCervical artery dissection, including carotid and vertebral artery dissection, is an important cause of stroke in the young. Risk of developing cervical artery dissection has been associated with physical activity in various forms and has been presumed to be related to minor trauma and mechanical stretching of the cervical arteries. This systematic review will aim to synthesise data on the risk of recurrent cervical artery dissection after an initial dissection. This information may be applied to further understand the natural history of this disease, and potentially to help direct evidence-based discussions on safe return to activity after dissection.Methods and analysisA broad search of multiple electronic databases (Medline, Embase, Cochrane Central Register of Controlled Trials and Web of Science) will be conducted to identify studies published as of 13 November 2019, examining all-comers with cervical artery dissection observed over time. Studies will be screened by two independent reviewers in a two-level process to determine eligibility for inclusion. Data will be pooled from eligible articles and the main outcome of recurrent cervical artery dissection at 5 years will be determined using quantitative analysis.Ethics and disseminationEthics approval is not necessary as no primary data are being collected. The information will be disseminated in the form of a systematic review article which will be submitted to a peer-reviewed medical journal.PROSPERO registration numberCRD42020166105.

Recurrent versus first cervical artery dissection - a retrospective study of clinical and vascular characteristics.

Most recurrent cervical artery dissection (CeAD) events occur shortly after the acute first CeAD. This study compared the characteristics of recurrent and first CeAD events and searched for associations between subsequent events of an individual person. Cervical artery dissection patients with a new CeAD event occurring during a 3-6month follow-up were retrospectively selected in seven specialized stroke centers. Clinical and vascular characteristics of the initial and the recurrent CeADs were compared. The study sample included 76 patients. Recurrent CeADs were occlusive in one (1.3%) patient, caused cerebral ischaemia in 13 (17.1%) and were asymptomatic in 39 (51.3%) patients, compared to 29 (38.2%) occlusive, 42 (55.3%) ischaemic and no asymptomatic first CeAD events. In 52 (68.4%) patients, recurrent dissections affected both internal carotid arteries or both vertebral arteries, whilst 24 (31.6%) patients had subsequent dissections in both types of artery. Twelve (28.6%) of 42 patients with an ischaemic first dissection had ischaemic symptoms due to the recurrent CeADs, too. However, only one (1.3%) of 34 patients with a non-ischaemic first CeAD suffered ischaemia upon recurrence. Recurrent CeAD typically affects the same site of artery. It causes ischaemic events less often than the first CeAD. The risk that patients who presented with solely non-ischaemic symptoms of a first CeAD will have ischaemic symptoms in the case of a recurrent CeAD seems very small.

Long-term outcome of cervical artery dissection : IPSYS CeAD: study protocol, rationale, and baseline data of an Italian multicenter research collaboration.

Long-term consequences of cervical artery dissection (CeAD), a major cause of ischemic stroke in young people, have been poorly investigated. The Italian Project on Stroke at Young Age - Cervical Artery Dissection (IPSYS CeAD) project is a multicenter, hospital-based, consecutively recruiting, observational, cohort study aimed to address clinically important questions about long-term outcome of CeAD patients, which are not covered by other large-scale registries. Patients with radiologically diagnosed CeAD were consecutively included in the registry. Baseline demographic and clinical variables, as well as information on risk factors, were systematically collected for each eligible patient. Follow-up evaluations were conducted between 3 and 6 months after the initial event (t1) and then annually (t2 at 1 year, t3 at 2 years , and so on), in order to assess outcome events (long-term recurrent CeAD, any fatal/nonfatal ischemic stroke, transient ischemic attack (TIA), or other arterial thrombotic event, and death from any cause). Between 2000 and 2019, data from 1530 patients (age at diagnosis, 47.2 ± 11.5 years; women, 660 [43.1%]) have been collected at 39 Italian neurological centers. Dissection involved a single vessel in 1308 (85.5%) cases and caused brain ischemia in 1303 (85.1%) (190 TIA/1113 ischemic stroke). Longitudinal data are available for 1414 (92.4%) patients (median follow-up time in patients who did not experience recurrent events, 36.0 months [25th to 75th percentile, 63.0]). The collaborative IPSYS CeAD effort will provide novel information on the long-term outcome of CeAD patients. This could allow for tailored treatment approaches based on patients' individual characteristics.

Clinical Usefulness of Serial Duplex Ultrasound in Cervical Artery Dissection Patients

Purpose: To study the clinical usefulness of serial color-coded duplex ultrasound (DUS) examinations in cervical artery dissection (CeAD) patients. Methods: Single-center, CeAD registry-based re-review of serial, routine DUS exams in consecutive CeAD patients treated at the Stroke Center Basel, Switzerland (2009–2015). Two experienced raters reassessed all DUS for the occurrence of new arterial findings during follow-up, that is. (i) recanalization of the dissected artery (if occluded at baseline), (ii) delayed occlusion of a patent dissected artery, and (iii) recurrent CeAD. We studied whether these new arterial findings were associated with clinical symptoms. Results: In 94 CeAD patients (n = 40 female [42.6%], median age 46 years [interquartile range (IQR) 36.2–53]), 506 DUS examinations were reviewed covering a median length of follow-up of 54.1 weeks (IQR 30.5–100.5). In total, 105 dissected arteries were detected, of which 27 (25.7%) were occluded. In 28/94 patients (29.8%), 31 new arterial findings were recorded, which were associated with clinical symptoms in 9/31 (30%) patients. Recanalization of occluded CeAD was observed in 22/27 (81.5%) arteries and occurred in 20/22 arteries within 3 months. In 4/22 patients (18.2%), recanalization was associated with clinical symptoms (ischemic events [n = 2], pure local symptoms [n = 2]). Delayed occlusions were observed in 4/78 (5.1%) dissected arteries patent at baseline. All were clinically asymptomatic and occurred within 14 days from baseline. Recurrent CeAD (all symptomatic) occurred in 5 previously non-dissected arteries. Conclusion: In CeAD patients, follow-up DUS identified new arterial findings, of which several were associated with clinical symptoms: we found that about 1 of 5 recanalizations were associated with clinical symptoms, of whom half were ischemic symptoms. Further, delayed occlusions occurred in patients with no or mild stenosis at baseline and were asymptomatic. This study emphasizes the potential importance of repeated DUS in CeAD particularly in the early phase of up to 4 weeks.

Artery occlusion independently predicts unfavorable outcome in cervical artery dissection.

To assess the impact of dissected artery occlusion (DAO) on functional outcome and complications in patients with cervical artery dissection (CeAD). We analyzed combined individual patient data from 3 multicenter cohorts of consecutive patients with CeAD (the Cervical Artery Dissection and Ischemic Stroke Patients [CADISP]-Plus consortium dataset). Patients with data on DAO and functional outcome were included. We compared patients with DAO to those without DAO. Primary outcome was favorable functional outcome (i.e., modified Rankin Scale [mRS] score 0-1) measured 3-6 months from baseline. Secondary outcomes included delayed cerebral ischemia, major hemorrhage, recurrent CeAD, and death. We performed univariate and multivariable binary logistic regression analyses and calculated odds ratios (OR) with 95% confidence intervals (CI), with adjustment for potential confounders. Of 2,148 patients (median age 45 years [interquartile range (IQR) 38-52], 43.6% women), 728 (33.9%) had DAO. Patients with DAO more frequently presented with cerebral ischemia (84.6% vs 58.5%, p < 0.001). Patients with DAO were less likely to have favorable outcome when compared to patients without DAO (mRS 0-1: 59.6% vs 80.1%, p unadjusted < 0.001). After adjustment for age, sex, and initial stroke severity, DAO was independently associated with less favorable outcome (mRS 0-1: OR 0.65, CI 0.50-0.84, p = 0.001). Delayed cerebral ischemia occurred more frequently in patients with DAO than in patients without DAO (4.5% vs 2.9%, p = 0.059). DAO independently predicts less favorable functional outcome in patients with CeAD. Further research on vessel patency, collateral status and effects of revascularization therapies particularly in patients with DAO is warranted.

Rare genetic variants in patients with cervical artery dissection.

The potential role of genetic alterations in cervical artery dissection (CeAD) pathogenesis is poorly understood. We aimed to identify pathogenic genetic variants associated with cervical artery dissection by using whole exome sequencing. CeAD-patients with either a family history of cervical artery dissection (f-CeAD) or recurrent cervical artery dissection (r-CeAD) from the CeAD-databases of two experienced stroke centres were analysed by whole exome sequencing.Variants with allele frequency <0.05 and classified as pathogenic by predicting algorithms (SIFT or Polyphen-2) or the ClinVar database were explored. First, we analysed a panel of 30 candidate genes associated with arterial dissection (any site) or aneurysm according to the OMIM (online Mendelian Inheritance of Men) database. Second, we performed a genome-wide search for pathogenic variants causing other vascular phenotypes possibly related to cervical artery dissection.Findings were classified as CeAD-causing (pathogenic variants in genes from the arterial dissection or aneurysm panel) or suggestive (pathogenic variants in genes associated with other vascular phenotypes and variants of unknown significance in genes from the arterial dissection or aneurysm panel). All other variants were classified as benign/uncertain. Among 43 CeAD-patients, 28 patients (17 pedigrees) had f-CeAD and 15 had r-CeAD. No CeAD-causing variants were identified in r-CeAD patients. Among f-CeAD-patients, 5/17 pedigrees carried CeAD-causing variants in COL3A1, COL4A1, COL4A3, COL4A4, COL5A1, COL5A2 and FBN1. Suggestive variants in ABCC6, COL3A1, COL5A2, MEF2A, and RNF213 were detected in three pedigrees with f-CeAD and six patients with r-CeAD.Discussion and conclusion: CeAD-causing variants were rare and exclusively found in f-CeAD-patients, suggesting differences between the genetic architectures of f-CeAD and r-CeAD. The identified variants indicate a high genetic heterogeneity of the study sample.

Determinants and outcome of multiple and early recurrent cervical artery dissections.

To assess putative risk factors and outcome of multiple and early recurrent cervical artery dissection (CeAD). We combined data from 2 multicenter cohorts and compared patients with multiple CeAD at initial diagnosis, early recurrent CeAD within 3 to 6 months, and single nonrecurrent CeAD. Putative risk factors, clinical characteristics, functional outcome, and risk of recurrent ischemic events were assessed. Of 1,958 patients with CeAD (mean ± SD age 44.3 ± 10 years, 43.9% women), 1,588 (81.1%) had single nonrecurrent CeAD, 340 (17.4%) had multiple CeAD, and 30 (1.5%) presented with single CeAD at admission and had early recurrent CeAD. Patients with multiple or early recurrent CeAD did not significantly differ with respect to putative risk factors, clinical presentation, and outcome. In multivariable analyses, patients with multiple or early recurrent CeAD more often had recent infection (odds ratio [OR] 1.81, 95% confidence interval [CI] 1.29-2.53), vertebral artery dissection (OR 1.82, 95% CI 1.34-2.46), family history of stroke (OR 1.55, 95% CI 1.06-2.25), cervical pain (OR 1.36, 95% CI 1.01-1.84), and subarachnoid hemorrhage (OR 2.85, 95% CI 1.01-8.04) at initial presentation compared to patients with single nonrecurrent CeAD. Patients with multiple or early recurrent CeAD also had a higher incidence of cerebral ischemia (hazard ratio 2.77, 95% CI 1.49-5.14) at 3 to 6 months but no difference in functional outcome compared to patients with single nonrecurrent CeAD. Patients with multiple and early recurrent CeAD share similar risk factors, clinical characteristics, and functional outcome. Compared to patients with single nonrecurrent CeAD, they are more likely to have recurrent cerebral ischemia at 3 to 6 months, possibly reflecting an underlying transient vasculopathy.

Recurrence of cervical artery dissection: An underestimated risk.

To explore the recurrence of cervical artery dissection (CeAD). A single-center consecutive series of 282 CeAD patients was prospectively recruited during first admission from 1995 to 2012. Patients with a follow-up of at least 1 year (n = 238) were eligible for the current analysis. All patients with clinical symptoms or signs of recurrent CeAD on ultrasound were examined by MRI. Dermal connective tissue morphology was studied in 108 (45.4%) patients. Median follow-up was 52 months (range 12-204 months). In all, 221 (92.8%) patients presented with monophasic CeAD, including 188 (79.0%) patients with a single CeAD event, 11 (4.6%) with simultaneous dissections in multiple cervical arteries, and 22 (9.2%) with subsequent events within a single phase of 4 weeks. Seventeen patients (7.1%) had late (>1 month after the initial event) recurrent CeAD events, including 5 (2.1%) with multiple recurrences. Patients with late recurrences were younger (37.5 ± 6.9 years) than those without (43.8 ± 9.9; p = 0.011). Ischemic stroke occurred in 164 (68.9%) patients at first diagnosis, but only 4 of 46 (8.7%) subsequent events caused stroke (p < 0.0001), while 19 (41.3%) were asymptomatic. Connective tissue abnormalities were found in 54 (56.3%) patients with monophasic and 8 (66.7%) with late recurrent dissections (p = 0.494). Twenty-two (9.2%) patients had new CeAD events within 1 month and 17 (7.1%) later recurrences. The risk for new events was significantly higher (about 60-fold) during the acute phase than during later follow-up. Connective tissue abnormalities were not more frequent in patients with late recurrent events than in those with monophasic CeAD.

Risk of recurrent cervical artery dissection during pregnancy, childbirth and puerperium.

Hormonal and mechanical factors might increase the risk for cervical artery dissection (CAD) during pregnancy and the puerperium. There is uncertainty how to counsel women with a previous CAD regarding the risk of CAD recurrence during pregnancy and the puerperium. In an observational study of four stroke centers, all women aged 16-45years with primary CAD in the previous decade were asked to participate in a standardized assessment on long-term follow-up with a special focus on pregnancies and recurrent CAD. Ninety-two women were identified and 53 of them were included in the analysis (60%). Eleven women declined to participate, 28 were untraceable. The 39 non-participants did not differ from participants regarding key baseline characteristics. Average follow-up time was 72months. Nine women (17%) had recurrent CAD after a median of 14days (range 2days to 117months). Eleven women (20%) had a total of 13 completed pregnancies at a median of 44months (range 12-84months) after index CAD. Two of the pregnant women (18%) had suffered recurrent CAD ≥18months prior to the pregnancy. All 13 pregnancies and puerperia went without recurrent CADs or cerebrovascular events. This includes giving birth by vaginal delivery (n=6) and caesarean section (n=7). None of the five women with typical connective tissue disease became pregnant. Our observation suggests that the risk of recurrent CAD may not be greatly increased with pregnancies starting at least 12months after CAD in women without typical connective tissue disease.

Familial Occurrence of Cervical Artery Dissection – Coincidence or Sign of Familial Predisposition?

Backgroundand Purpose: The etiology of spontaneous cervical artery dissection (CeAD) is poorly understood in most patients. Mild cervical trauma preceding the dissection event is a common finding, but many CeAD occur spontaneously. It is likely that genetic factors may increase the risk for CeAD. However, familial cases are excedingly rare. Familial clustering of CeAD may be accidental or associated with genetic or environmental risk factors shared between affected relatives. In this explorative study, we aim to show that specific risk factors for familial CeAD exist. Methods: Age of onset, sex, affected artery and number of recurrent CeAD were documented for familial patients and compared with published findings from patients with sporadic CeAD. Concordance of age, sex and dissected artery within the families was analyzed by correlation analysis and by analysis of variance or Kruskal-Wallis testing. Results: The study sample consisted of 9 new patients with a family history of CeAD enrolled in the Neurology Department of the University of Heidelberg or referred to Heidelberg from other centers. The study sample also included published findings from another 23 patients, in total 32 patients. The mean age of the patients with familial CeAD at their first dissections was 38.4 ± 13.3 years. Twenty (62.5%) patients were female and 12 patients (37.5%) suffered multiple dissections. Four patients (12.5%) presented with recurrent dissections after >1 year. Patients with a familial history of CeAD were younger (p = 0.023) and presented more often with multiple dissections (p = 0.024) and recurrent dissections (p = 0.018). Age at the first event (correlation analysis p = 0.026; analysis of variance p = 0.029) and site of the dissection (correlation analysis p = 0.032; Kruskal-Wallis test p = 0.018) differed between the families, and there was no concordance of gender of affected family members (correlation analysis p = 0.500; Kruskal-Wallis test p = 0.211). Conclusions: The high prevalence of multiple dissection events and of long-term (>1 year) recurrent dissections in patients with a familial history of CeAD indicates that a specific predisposition for familial CeAD exists. Since age of onset and affected vessel differ between families, the risk profile for familial CeAD is heterogeneous. A large-scale (whole exome) sequencing analysis of 14 patients from 7 of the analyzed families is currently being performed in order to identify causative genetic variants.

A glycine-valine substitution in alpha2 type V procollagen associated with recurrent cervical artery dissection

A glycine-valine substitution in alpha2 type V procollagen associated with recurrent cervical artery dissection

Spontaneous cervical artery dissection

Cervical artery dissection (CAD) is now recognized as an important arterial cause of ischemic stroke in younger patients. Data concerning epidemiology, specific risk factors, and pathogenesis are accumulating but there are still no large-scale, prospective, population-based studies. Long-term follow-up and data estimating risk of recurrent CAD are needed.1–5 In this issue of Neurology , Arnold et al.6 report the results of the largest, multicenter analysis of CAD to date. Sex differences in CAD were correlated with clinical and prognostic variables. Arnold et al. found a preponderance of men; however, women were 5 years younger when CAD occurred and more often had multiple CAD. These findings are consistent with most earlier studies.1–4 In a study of 200 patients, Schievink et al.1 …

Risk of stroke and recurrent dissection after a cervical artery dissection: a multicenter study.

To assess the risk of stroke, TIA, or dissection recurrence after a first event of cervical artery dissection (CAD). The authors undertook a historical cohort study of consecutive patients with a first event of CAD who were admitted in 24 departments of neurology within a period of at least 1 year. Patients were retrospectively selected from a stroke data bank or from the local administrative data bank using the 10th revision of the International Statistical Classification of Diseases. A neurologist and a radiologist reviewed all charts to validate diagnosis and collect data. In 2002, patients were interviewed by phone or during a visit by the local investigators. Four hundred fifty-nine patients (mean age 44.0 +/- 9.7 years) were included in the study. Among the 457 survivors, 25 (5.5%) could not be contacted in 2002 because they had moved. After a mean follow-up of 31 months, four (0.9%) patients presented a recurrent ischemic stroke attributable to either not yet completely recovered initial CAD (n = 2) or a recurrent CAD (n = 2). Eight (1.8%) patients had a TIA without CAD recurrence. Two TIA occurred at the acute stage of CAD. Of the six remaining TIA, only one was associated with chronic arterial stenosis. In addition, two patients had recurrent CAD without stroke, giving a total of four (0.9%) CAD recurrences. Patients with a first event of CAD have a very low risk of ischemic events or dissection recurrences. Ischemic events seem rarely to be in relation with chronic arterial lesions.

Pathogenesis of cervical artery dissections: association with connective tissue abnormalities.

The etiology of spontaneous cervical artery dissection (CAD) is largely unknown. An underlying connective tissue disorder has often been postulated. To further assess the association of CAD with ultrastructural abnormalities of the dermal connective tissue. In a multicenter study, skin biopsies of 65 patients with proven nontraumatic CAD and 10 control subjects were evaluated. The ultrastructural morphology of the dermal connective tissue components was assessed by transmission electron microscopy. Only three patients (5%) had clinical manifestations of skin, joint, or skeletal abnormalities. Ultrastructural aberrations were seen in 36 of 65 patients (55%), consisting of the regular occurrence of composite fibrils within collagen bundles that in some cases resembled the aberrations found in Ehlers-Danlos syndrome type II or III and elastic fiber abnormalities with minicalcifications and fragmentation. A grading scale according to the severity of the findings is introduced. Intraindividual variability over time was excluded by a second biopsy of the skin in eight patients with pronounced aberrations. Recurrent CAD correlated with connective tissue aberrations. In addition, similar connective tissue abnormalities were detected in four first-degree relatives with familial CAD. CAD is associated with ultrastructural connective tissue abnormalities, mostly without other clinical manifestations of a connective tissue disease. A structural defect in the extracellular matrix of the arterial wall leading to a genetic predisposition is suggested. The dermal connective tissue abnormalities detected can serve as a phenotypic marker for further genetic studies in patients with CAD and large families to possibly identify the underlying basic molecular defect(s).