Vertebral artery dissection as the underlying cause of ventral spinal epidural haematoma.

Spontaneous spine epidural haematoma is a rare occurrence, with an incidence of 0.1/100 000 inhabitants/year. The anterior location of the haematoma is very uncommon since the dural sac is firmly attached to the posterior longitudinal ligament. Vertebral artery dissection as its underlying cause is an exceptionally rare event, with only two documented cases.This article presents the case of young woman who arrived at the emergency room with a spinal ventral epidural haematoma extending from C2 to T10, caused by a non-traumatic dissecting aneurysm of the right vertebral artery at V2-V3 segment. Since the patient was tetraparetic, she underwent emergent laminectomy, and the vertebral artery dissection was subsequently treated endovascularly with stenting.Vertebral artery dissection with subsequent perivascular haemorrhage is a possible cause of spontaneous spine epidural haematoma, particularly when located ventrally in the cervical and/or high thoracic column. Hence the importance of a thorough investigation of the vertebral artery integrity.

Toward Individual Treatment in Cervical Artery Dissection: Subgroup Analysis of the TREAT-CAD Randomized Trial.

OBJECTIVE: Uncertainty remains regarding antithrombotic treatment in cervical artery dissection. This analysis aimed to explore whether certain patient profiles influence the effects of different types of antithrombotic treatment. METHODS: This was a post hoc exploratory analysis based on the per-protocol dataset from TREAT-CAD (NCT02046460), a randomized controlled trial comparing aspirin to anticoagulation in patients with cervical artery dissection. We explored the potential effects of distinct patient profiles on outcomes in participants treated with either aspirin or anticoagulation. Profiles included (1) presenting with ischemia (no/yes), (2) occlusion of the dissected artery (no/yes), (3) early versus delayed treatment start (median), and (4) intracranial extension of the dissection (no/yes). Outcomes included clinical (stroke, major hemorrhage, death) and magnetic resonance imaging outcomes (new ischemic or hemorrhagic brain lesions) and were assessed for each subgroup in separate logistic models without adjustment for multiple testing. RESULTS: All 173 (100%) per-protocol participants were eligible for the analyses. Participants without occlusion had decreased odds of events when treated with anticoagulation (odds ratio [OR] = 0.28, 95% confidence interval [CI] = 0.07-0.86). This effect was more pronounced in participants presenting with cerebral ischemia (n = 118; OR = 0.16, 95% CI = 0.04-0.55). In the latter, those with early treatment (OR = 0.26, 95% CI = 0.07-0.85) or without intracranial extension of the dissection (OR = 0.34, 95% CI = 0.11-0.97) had decreased odds of events when treated with anticoagulation. INTERPRETATION: Anticoagulation might be preferable in patients with cervical artery dissection presenting with ischemia and no occlusion or no intracranial extension of the dissection. These findings need confirmation. ANN NEUROL 2024;95:886-897.

Cervical radiculopathy secondary to vertebral artery dissection: clinical features and outcomes.

PURPOSE: Cervical radiculopathy due to various mechanical causes is commonly seen, however, cervical vertebral artery dissection (cVAD)-related radiculopathy is very rare with poorly characterized clinical outcomes. Thus, we conducted a systematic review of published literature and reported an institutional case to provide a better illustration of this rare entity. METHODS: We systematically reviewed the PubMed literature and queried the clinical database at our center for cVAD-related radiculopathy. We described the baseline characteristics of patients with cVAD-related radiculopathy, the involved segment, diagnostic approach and treatment options. RESULT: 14 previously published studies met the inclusion criteria and along with the case we identified in our center, our study included 17 patients total (median age: 35 years, 9 females). C5 was the most commonly affected root and ipsilateral shoulder/arm pain along with shoulder abduction weakness was most common presentation. Antiplatelet or anticoagulant therapy was the treatment of choice. Most cases managed conservatively (82%). Majority of the cases (92%) had either complete or partial resolution of their symptoms. CONCLUSION: Despite its limitations, this study show that cVAD related radiculopathy is a relatively benign entity with excellent clinical outcomes when managed medically.

Spontaneous intracranial vertebral artery dissections presenting with subarachnoid hemorrhage.

BACKGROUND: Vertebral artery dissection (VAD) is an infrequent source of subarachnoid hemorrhage (SAH), with a high mortality rate, primarily due to the risk of rebleeding both before and after medical intervention. This paper provides a comprehensive analysis of the anatomy, pathophysiology, clinical presentation, treatment strategies, and outcomes of intracranial vertebral artery dissections that result in subarachnoid hemorrhage. METHODS: Comprehensive five-year literature review (2018-2022) and a retrospective analysis of patient records from our institution between 2016 and 2022. We included studies with a minimum of 5 patients. RESULTS: The study incorporated ten series from the literature and 22 cases from CHUM. Key anatomical factors increasing the risk of VAD include the vertebral artery's origin from the aortic arch, asymmetry of the vertebral artery, and its tortuosity. Patients may display specific collagen and genetic abnormalities. The occurrence of VAD appears to be more prevalent in men. Those with a ruptured intracranial VAD typically show prodromal symptoms and present with severe SAH. Rebleeding within the first 24 h is frequent. While standard imaging methods are usually adequate for VAD diagnosis, they may not provide detailed information about the perforator anatomy. Treatment approaches include both deconstructive and reconstructive methods. CONCLUSION: Ruptured VAD is a critical, life-threatening condition. Many patients have a poor neurological status at presentation, and rebleeding prior to treatment is a significant concern. Deconstructive techniques are most effective in preventing rebleeding, whereas the efficacy of reconstructive techniques needs more investigation.

Iatrogenic Vertebral Artery Dissection with Posterior Fossa and Lateral Medullary Stroke After Uncomplicated Cervical Nerve Ablation.

BACKGROUND: A cervical radiofrequency ablation is a procedure that can be performed to treat arthritis-related pain in the neck and upper back. There have been no large studies reporting complications after this procedure. We report a case of a 55-year-old woman with iatrogenic vertebral artery dissection of C3-C4 with segmental occlusion leading to a posterior fossa stroke and lateral medullary stroke after a high-grade cervical nerve ablation. CASE REPORT: A 55-year-old woman presented to the Emergency Department with vomiting, neck pain, temperature changes, dizziness, and dysarthria after undergoing C2-C3, C4-C5 nerve ablation 30 min prior to arrival. The patient was found to have a vertebral artery dissection with posterior fossa and lateral medullary stroke. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Vertebral artery dissection and subsequent stroke should be considered with recent cervical facet joint injections, such as intra-articular facet joint injections, medial branch blocks, or medial branch radiofrequency nerve ablation. The case we report shows devastating outcomes that can result from what many consider a relatively simple procedure.

Epidemiology of Spontaneous Cervical Artery Dissection: Population-Based Study.

BACKGROUND: Cervical artery dissection (CeAD) represents up to 15% to 25% of ischemic strokes in people under the age of 50 years. Noninvasive vessel imaging is increasingly used in clinical practice, but the impact on the frequency of detection of CeAD is unknown. In 2006, the yearly incidence rate of CeAD was estimated at 2.6 per 100 000 person-years, but the current incidence is unknown. METHODS: In this population-based retrospective observational cohort study, we utilized the resources of the Rochester Epidemiology Project to ascertain all adult residents of Olmsted County, MN, diagnosed with internal carotid artery dissection and common carotid artery dissection or vertebral artery dissection from 2002 to 2020. Patients with only intracranial involvement or CeAD following major trauma were excluded. Age-adjusted sex-specific and age- and sex-adjusted incidence rates were estimated using the US White 2010 decennial census, with rates expressed per 100 000 person-years. We assessed longitudinal trends by dividing the data into 5-year time intervals, with the last being a 4-year interval. RESULTS: We identified 123 patients with a diagnosis of CeAD. There were 63 patients with internal carotid artery dissection, 54 with vertebral artery dissection, 2 with concurrent internal carotid artery dissection and vertebral artery dissection, and 4 with common carotid artery dissection. There were 63 (51.2%) female patients and 60 (48.8%) male patients. The average age at diagnosis was 50.2 years (SD, 15.1 [95% CI, 20.1-90.5] years). The incidence rate of spontaneous CeAD encompassing all locations was 4.69 per 100 000 person-years (2.43 for internal carotid artery dissection and 2.01 for vertebral artery dissection). The incidence rate increased from 2.30 per 100 000 person-years from 2002 to 2006 to 8.93 per 100 000 person-years from 2017 to 2020 (P<0.0001). The incidence rate for female patients rose from 0.81 per 100 000 person-years from 2002 to 2006 to 10.17 per 100 000 person-years from 2017 to 2020. CONCLUSIONS: The incidence rate of spontaneous CeAD increased nearly 4-fold over a 19-year period from 2002 to 2020. The incidence rate in women rose over 12-fold. The increase in incidence rates likely reflects the increased use of noninvasive vascular imaging.

Coronavirus Disease 2019 Infection in Cervical Artery Dissections.

OBJECTIVE: Most cervical artery dissection (CeAD) cases are spontaneous or due to minor traumas, and preceding viral infections have been suggested to be a triggering event for CeAD in some. Herein, we analyze the prevalence of coronavirus disease 2019 (COVID-19) in hospitalized patients with CeAD using a national database. METHODS: The National Inpatient Sample was queried from April 2020 to December 2020 for patients with a diagnosis of CeAD using International Classification of Diseases, 10th edition-Clinical Modification codes. Among these, patients with COVID-19 were identified. Multivariable logistic regression was conducted to assess the patient profile of those with COVID-19, in-patient mortality, and home discharge among patients with CeAD. RESULTS: There were 360 (2.32%) hospitalizations involving COVID-19 among 15,500 with CeAD. Concomitant acute ischemic stroke constituted 43.06% of those with a COVID-19 diagnosis, whereas it was 43.73% among those without a COVID-19 diagnosis ( P = 0.902). Home discharges were less common in patients with COVID-19 and CeAD compared to CeAD alone (34.85% vs. 48.63%; P = 0.03), but this was likely due to other factors as multivariate regression analysis did not show an association between COVID-19 and home discharges (odds ratio: 0.69; 95% CI: 0.39 to 1.25; P = 0.22). COVID-19 diagnosis had similar odds of inpatient mortality (odds ratio: 1.11; 95% CI: 0.43 to 2.84; P = 0.84). CONCLUSION: The prevalence of COVID-19 among hospitalized patients with CeAD is low with 2.32% of all CeAD cases. Concomitant COVID infection did not lead to an increased risk of stroke in CeAD. However, potentially worse functional outcomes (fewer home discharges) without an increase in mortality were seen in patients with COVID and CeAD.

Vertebral Artery Dissection in the Setting of Unstable Os Odontoideum: A Case Report.

CASE: A healthy 5-year-old boy presented with a gradual onset of headaches and acute global right-sided weakness over 10 days. The work-up revealed unstable os odontoideum leading to multiple posterior circulation infarcts with vertebral artery dissection. He underwent antiplatelet therapy, cervical collar immobilization, and delayed occiput to C2 posterior spinal fusion and instrumentation with iliac crest autograft. At 2-year follow-up, the patient had a solid fusion mass, appropriate cervical alignment, and was without neurologic sequelae. CONCLUSION: This case adds to a sparse body of literature in the management of vertebral artery dissection with vertebrobasilar insufficiency secondary to unstable os odontoideum.

Imaging of Vertebral Artery Dissection in Children: An Underrecognized Condition with High Risk of Recurrent Stroke.

Vertebral artery dissection (VAD) is a common cause of a rare condition, pediatric posterior circulation arterial ischemic stroke (PCAIS). VAD is clinically important due to the risk of multifocal and continuing infarcts from artery-to-artery thromboembolism, with the potential for occlusion of arteries that perfuse the brainstem. Early diagnosis is important, as recurrent stroke is a common effect of VAD in children. Although the relative efficacies of different treatment regimens for VAD in children remain unsettled, early initiation of treatment can mitigate the risk of delayed stroke. Clinical diagnosis of PCAIS may be delayed due to multiple factors, including nonspecific symptoms and the inability of younger patients to express symptoms. In fact, subacute or chronic infarcts are often present at initial imaging. Although the most common cause of isolated PCAIS is VAD, imaging of the cervical arteries has been historically underused in this setting. Cervical vascular imaging (MR angiography, CT angiography, and digital subtraction angiography) for VAD must be optimized to detect the sometimes subtle findings, which may be identified at initial or follow-up imaging. Osseous variants of the craniocervical junction and upper cervical spine and other extrinsic lesions that may directly injure the vertebral arteries or lead to altered biomechanics have been implicated in some cases. The authors review characteristic imaging features and optimized imaging of VAD and associated PCAIS and related clinical considerations. Identification of VAD has important implications for evaluation, treatment, and imaging follow-up, as this condition may result in progressive arteriopathy and recurrent stroke. © RSNA, 2023 Supplemental material is available for this article. Quiz questions for this article are available through the Online Learning Center.

Risk Profile of Patients with Spontaneous Cervical Artery Dissection.

OBJECTIVE: Epidemiological data to characterize the individual risk profile of patients with spontaneous cervical artery dissection (sCeAD) are rather inconsistent. METHODS AND RESULTS: In the setting of the Italian Project on Stroke in Young Adults Cervical Artery Dissection (IPSYS CeAD), we compared the characteristics of 1,468 patients with sCeAD (mean age = 47.3 ± 11.3 years, men = 56.7%) prospectively recruited at 39 Italian centers with those of 2 control groups, composed of (1) patients whose ischemic stroke was caused by mechanisms other than dissection (non-CeAD IS) selected from the prospective IPSYS registry and Brescia Stroke Registry and (2) stroke-free individuals selected from the staff members of participating hospitals, matched 1:1:1 by sex, age, and race. Compared to stroke-free subjects, patients with sCeAD were more likely to be hypertensive (odds ratio [OR] = 1.65, 95% confidence interval [CI] = 1.37-1.98), to have personal history of migraine with aura (OR = 2.45, 95% CI = 1.74-3.34), without aura (OR = 2.67, 95% CI = 2.15-3.32), and family history of vascular disease in first-degree relatives (OR = 1.69, 95% CI = 1.39-2.05), and less likely to be diabetic (OR = 0.65, 95% CI = 0.47-0.91), hypercholesterolemic (OR = 0.75, 95% CI = 0.62-0.91), and obese (OR = 0.41, 95% CI = 0.31-0.54). Migraine without aura was also associated with sCeAD (OR = 1.81, 95% CI = 1.47-2.22) in comparison with patients with non-CeAD IS. In the subgroup of patients with migraine, patients with sCeAD had higher frequency of migraine attacks and were less likely to take anti-migraine preventive medications, especially beta-blockers, compared with the other groups. INTERPRETATION: The risk of sCeAD is influenced by migraine, especially migraine without aura, more than by other factors, increases with increasing frequency of attacks, and seems to be reduced by migraine preventive medications, namely beta-blockers. ANN NEUROL 2023;94:585-595.

Cerebellar infarction caused by vertebral artery dissection: A case report.

RATIONALE: Vertebral artery dissection is an important cause of posterior circulation ischemic stroke in young and middle-aged people. We reported a young man with cerebellar infarction caused by dissection of the right vertebral artery. PATIENT CONCERNS: A 34-year-old man presented with intermittent dizziness, blurred vision, nausea, and transient tinnitus 10 days before admission. All these symptoms were gradually aggravated and followed by vomiting and unfavorable movement of the right limbs. All these symptoms gradually aggravated. DIAGNOSIS: Neurological examination on admission showed ataxia of the right limbs. Magnetic resonance imaging of the head revealed a right cerebellar infarction. High-resolution vessel wall magnetic resonance imaging showed dissection of the right vertebral artery. Whole-brain CT digital subtraction angiography revealed occlusion of the third segment (V3) of the right vertebral artery. This supports the diagnosis of vertebral artery dissection. INTERVENTIONS: The patient received anticoagulant treatment with warfarin. OUTCOMES: After 2 weeks of treatment, the patient showed remarkably alleviated dizziness and unfavorable movement of the right limbs. After 3 months of treatment, the modified Rankin Scale score was 0. MRI of the head revealed that the original right cerebellar focus was softened, and there were no newly formed infarct foci. LESSONS: When young and middle-aged patients without atherosclerotic risk factors encounter sudden dizziness, tinnitus, and unfavorable limb movement, vertebral artery dissection may be considered. Careful inquiry into the medical history may help make a final diagnosis. Further high-resolution vessel wall magnetic resonance imaging is an effective means to find arterial dissection. Early diagnosis and treatment for vertebral artery dissection has a favorable prognosis.

Long-term outcome of cervical artery dissection.

OBJECTIVE: The aim of the study is to evaluate the natural history of extracranial cervical artery dissection (CAD) including comorbidities, symptoms at presentation, recurrence of symptoms, and long-term outcome following different treatment approaches. METHODS: A retrospective review of patients treated for acute CAD was performed over a 5-year period from January 2017 to April 2022. RESULTS: Thirty-nine patients were included in the study, 25 (64.1%) with acute internal carotid artery dissection and 14 (35.9%) with acute vertebral artery dissection. Thirty-four patients (87.1%) had spontaneous CAD, and five patients (12.8%) had traumatic CAD. The mean age of the cohort was 54.2 years. The mean time from symptom onset to presentation was 4.34 days. The most common symptoms in internal carotid artery dissection were unilateral weakness (44%), headache (44%), slurred speech (36%), facial droop (28%), unilateral paraesthesia (24%), neck pain (12%), visual disturbance (8%), and Horner's syndrome (8%). The most common symptoms in vertebral artery dissection were headache (35.7%), neck pain (35.7%), vertigo (28.57%), ataxia (14.28%), and slurred speech (14.28%). The imaging modalities used for diagnosis included computed tomography angiography (48.7%), magnetic resonance angiography (41%), and duplex ultrasound (10.2%). In patients with carotid artery dissection, 57% had severe stenosis, 24% had moderate stenosis, and 20% had mild stenosis. All patients treated were managed conservatively with either anticoagulation or antiplatelets. Long-term clinical follow-up was available for 33 patients (84.6%). Thirty patients (90.9%) reported complete resolution of symptoms, and three patients (9%) reported persistent symptoms. Anatomic follow-up with imaging was available for 17 patients (43.58%). Thirteen patients (76.47%) had complete resolution of dissection, two patients (11.76%) had partial resolution of dissection, and two patients (11.76%) had persistent dissection. There was one death unrelated to CAD in a multi-trauma patient. There were four early recurrent symptoms in the first 3 to 8 weeks post discharge. The mean follow-up time was 308.27 days. CONCLUSIONS: The majority of CADs can be managed conservatively with good clinical and anatomical outcome and low rates of recurrence.

Internal carotid artery dissection in a patient with hemophilia A: a case report and literature review.

Spontaneous cervical artery dissection (sCeAD) is the most common cause of ischemic stroke at a young age, but its pathogenetic mechanism and risk factors are not fully elucidated. It is reasonable to think that bleeding propensity, vascular risk factors such as hypertension and head or neck trauma, and constitutional weakness of the arterial wall together play a role in the pathogenesis of sCeAD. Hemophilia A is known to be an X-linked condition that leads to spontaneous bleeding in various tissues and organs. To date, a few cases of acute arterial dissection in patients with hemophilia have been reported, but the relationship between these two diseases has not been studied so far. In addition, there are no guidelines indicating the best antithrombotic treatment option in these patients. We report the case of a man with hemophilia A who developed sCeAD and transient oculo-pyramidal syndrome and was treated with acetylsalicylic acid. We also review previous published cases of arterial dissection in patients with hemophilia, discussing the potential pathogenetic mechanism underlying this rare association and potential antithrombotic therapeutic options.

Persistent headache attributed to past cervicocephalic artery dissection: clinical characteristics and contributors to headache persistence.

BACKGROUND: Persistent headache/facial/neck pain attributed to past cervicocephalic arterial dissection is under-documented in literature. Our main goal was to evaluate clinical characteristics and contributors to this persistence. METHODS: A retrospective cohort study which included patients with a radiologically confirmed cervicocephalic arterial dissection (2015-2020) in a Portuguese tertiary hospital. Headache persistence was identified through clinical records. A questionnaire aimed to characterize headache in three moments: previous, persistent, and headache at the time of the interview (on average 2.5 years post-event). RESULTS: Ninety-two patients were identified; 24 (26.1%) had headache persistence ≥3 months, and 20 (22.2%) on average after 2.5 years post-event. There were no differences regarding demographics and vascular risk factors among patients with (n = 22) and without (n = 68) headache persistence. The first group had higher previous headache history (68.2% vs 4.4%, p < 0.001), delay in diagnosis (3.6 vs 1.9 days, p < 0.001), and headache/cervicalgia as the first symptom (81.8% vs 41.2%, p < 0.001). At the time of the interview, 20% still reported daily headache. A logistic regression model depicted headache history (OR = 59.8, p < 0.001), acute headache/cervicalgia (odds ratio, OR = 25.4, p = 0.005), posterior circulation dissection (OR = 7.6, p < 0.001), and less than 4 points by National Institutes of Health Stroke Scale score (OR = 5.0, p = 0.025) as contributors to headache persistence. CONCLUSION: Headache persistence post-cervicocephalic arterial dissection is common, and frequently affects patients daily. As it potentially affects functional outcomes and quality of life, the contributors identified in this study may help clinicians manage patients after the acute event.

A Comparative Study of the Etiology of Intracranial Vertebral Artery Dissection and Carotid Artery Dissection.

BACKGROUND: Both extrinsic (trauma or violent movement) and intrinsic (structural abnormality, atherosclerosis, or hemodynamic instability) factors may result in arterial dissection. The role of these factors in the origin or progression of dissection remains unclear. This study aimed to characterize the clinical features, radiologic features, and outcomes of intracranial vertebral artery dissection compared with carotid artery dissection and to determine the major causative factors. METHODS: Consecutive patients with craniocervical dissection (n=127) were retrospectively analyzed. Patients with intracranial vertebral artery dissection (n=77) and those with carotid artery dissection (n=35) were compared with respect to patient age, sex, cerebrovascular risk factors, laboratory indices, and radiologic features. RESULTS: Intracranial vertebral artery dissection was the most common craniocervical arterial dissection in our cohort (n=77, 60.6%). Body mass index in the intracranial vertebral artery dissection group was significantly greater than that in carotid artery dissection group. Clinical manifestations of intracranial vertebral artery dissection included ischemic stroke (37.7%), dizziness or vertigo (39.0%), and headache or neck pain (44.2%). Two patients had a definite history of trauma. The frontal and lateral tortuosity ratios of the vertebral basilar artery were significantly greater while the vertex angle was smaller in the intracranial vertebral artery dissection group compared with carotid artery dissection group. A positive correlation between the tortuosity ratios and subarachnoid hemorrhage and a significant inverse correlation between the tortuosity ratios and lipid parameters (high-density lipoprotein; apolipoprotein A1) were identified. CONCLUSIONS: Intrinsic causes may play a more important role in the development of intracranial vertebral artery dissection than carotid artery dissection.

Significance of Low Signal in Intracranial Vertebral Artery Wall Observed on Susceptibility-Weighted Angiography.

PURPOSE: We aimed to investigate the clinical significance of the low signal in the intracranial vertebral artery wall observed on susceptibility-weighted angiography. MATERIALS AND METHODS: We retrospectively reviewed susceptibility-weighted angiographies from 200 consecutive patients with acute ischemic stroke in the posterior circulation territory. The presence of eccentric or concentric low signals in the vertebral artery wall was examined and evaluated. The etiology of the low signal was also investigated as much as possible by referring to computed tomography and T1-weighted imaging (T1WI). We also compared its frequency in each stroke subtype. RESULTS: A low signal was observed in 128/200 patients (64%). The low signals (58%) corresponded to vessel wall calcification in 74 of 128 patients and to vessel wall thickening showing intermediate to low (n = 8) or high (n = 16) signals on T1WI in 24 (19%) patients. The low signal did not have vessel wall thickening or calcification in 1 patient, and the cause of the low signal could not be verified in 29 patients. According to stroke subtypes, a low signal was observed in 14/14 (100%) vertebral artery dissections, all of which corresponded to intramural hematoma. A low signal was observed in 51/65 (78%) atherothromboses, which were significantly more frequent than cardioembolism (34/66; 52%) and small-artery disease (18/39; 46%) (p < 0.01). In atherothrombosis, calcification was the most common cause of low signal (n = 32; 63%). CONCLUSION: Low signals on susceptibility-weighted angiography were frequently observed in vertebral artery dissection and atherothrombosis, reflecting intramural hematoma in all of the former and predominantly calcification in the latter.

Bow Hunter's syndrome combined with ipsilateral vertebral artery dissection/pseudoaneurysm: case study and literature review.

BACKGROUND: Bow hunter's syndrome (BHS), also known as rotational vertebral artery occlusion syndrome, is rare. Occasionally, it combines with dissection/pseudoaneurysm of the ipsilateral VA. METHODS: We report a case of BHS combined with ipsilateral VA dissection/pseudoaneurysm and review eight similar cases reported in the literature. Their aetiology, clinical and imaging features, treatment, and prognosis were analysed. RESULTS: Nine patients (seven male, two female; average age 22.0 ± 4.5 years) were enrolled. Visual symptoms comprised the most common clinical finding (66.7%, 7/9). Clinical symptoms were not related to neck rotation in seven patients (77.8%). Eight patients (88.9%) had multiple, scattered, new and old infarctions of the posterior circulation revealed on computed tomography/magnetic resonance imaging (CT/MRI) scans. Dissection/pseudoaneurysm was found in the ipsilateral VA - usually subtle and localised in the atlas, axis, and occipital bone - in all nine patients. Seven patients (66.7%) had special causes for the syndrome (i.e. congenital bone dysplasia). Altogether, 87.5% (7/8) experienced recurrence with cerebral infarction after antithrombotic therapy alone. Aetiologically targeted treatment, including surgical decompression or vertebral fixation, was performed in seven patients (77.8%). CONCLUSION: Young patients presenting with cryptogenic stroke in the posterior circulation and localised, subtle dissection/pseudoaneurysm of the ipsilateral VA around the atlanto-axial joint should undergo carotid ultrasonography with a neck rotation test or dynamic CT angiography/MR angiography/digital subtraction angiography, if necessary, to rule out/diagnose BHS.