Neurovascular considerations in patients with Down syndrome and moyamoya syndrome.

In this article, we describe a rare and complex case of moyamoya syndrome in a 7-year-old boy with Down syndrome and atlantoaxial subluxation. The patient presented with an ischemic stroke in the left hemisphere and cervical cord compression with increased cord edema. Diagnostic digital subtraction angiography revealed unique patterns of vascular involvement, with retrograde flow through the anterior spinal artery, ascending cervical artery, occipital artery, and multiple leptomeningeal arteries compensating for bilateral vertebral artery occlusion. This case underscores the underreported phenomenon of upward retrograde flow through the anterior spinal artery in bilateral vertebral artery occlusion. We address the rare manifestation of posterior circulation involvement in moyamoya syndrome, highlighting the importance of considering atlantoaxial instability as a contributing factor, as the absence of atlantoaxial stability is a risk factor for vertebral artery dissection. This study contributes valuable insights into the intricate relationship of moyamoya syndrome, Down syndrome, and atlantoaxial instability, urging clinicians to consider multifaceted approaches in diagnosis and treatment. It also emphasizes the potential significance of the anterior spinal artery as a compensatory pathway in complex vascular scenarios.

Vertebral Artery Stenting for Acute Multiple Cerebral Infarctions Caused by Vertebral Artery Dissection After Massage: A Case Report.

Vertebral artery dissection is a rare pathology that can cause ischemic stroke in young people. Cervical massage, especially improper pulling manipulation, is a cause of vertebral artery dissection. We present a case of 32-year-old woman who developed acute multiple posterior circulation ischemic cerebral infarctions as a result of left vertebral artery V4 segment dissection after receiving neck massage. She underwent emergency vertebral artery stent implantation at the site of the dissection. Symptoms were relieved the day after treatment. The patient recovered without adverse complications or endovascular restenosis in the following year.

[Vertebral artery dissection during traumatic injury of the cervical spine, two case reports]. / Nyaki gerinc traumás sérülése során jelentkezo arteria vertebralis dissectio, két esetbemutatás.

If severe cervical spinal cord injury or severe cervical vertebral fracture, subluxation or luxation is confirmed, 20-40% of the cases have vertebral artery dissection or occlusion. These can be asymptomatic, but can cause additional neurological damage in addition to cervical myelon and cervical nerve root symptoms. Vertebral artery dissection can be caused by direct injuries, stab wounds or gunshot wounds. Indirect vertebral artery dissection can occur at the same time as subluxation, luxation, or complex fractures of the cervical vertebra. CTA is the examination procedure of choice. In many cases, digital subtaction angiography examination and, if necessary, neurointerventional treatment must precede open neurosurgery. In our report, in the first patient, complete luxation of the C.VI vertebra caused unilateral vertebral artery 2-segment dissection-occlusion, while in our second patient, a stab injury caused direct vertebral artery compression and dissection. The occlusion of the vertebral artery did not cause neurological symptoms in any of the cases. In both of our cases, parent vessel occlusion was performed at the level of the vertebral artery injury before the neurosurgical operation.

Traumatic vertebral artery dissection associated with cervical neck traction devices.

Cervical arterial dissection (CAD) is a common cause of stroke in young people which can be classified as either spontaneous or traumatic. The primary initial symptoms are headache, neck pain, and dizziness. Recently, a 57-year-old woman experienced a severe headache after using a cervical neck traction device. Radiological examination of the head and neck revealed right vertebral artery dissection, which emphasizes the importance of recognizing that using cervical neck traction devices increases the risk of traumatic vertebral artery dissection.

Ultrasound Criteria for Diagnosing Spontaneous Cervical Artery Dissections. / Ultraschallkriterien zur Diagnose spontaner Halsarterien-Dissektionen.

Spontaneous dissection of brain-supplying cervical arteries, which also includes findings after minor injuries, is one of the main causes of ischaemic strokes in young adults. Strokes due to dissection are usually due to arterio-arterial embolism. They are rarely the first symptom of dissection because an intraluminal thrombus must first develop. Therefore, early diagnosis of dissection can contribute to stroke prevention - through immediate therapy with anticoagulants or antiplatelet drugs. This article describes the diagnostic criteria and typical findings of spontaneous dissection, in which no dissecting membrane is to be expected as in aortic dissection. Traumatic dissections following blunt or penetrating injuries also present with different findings. Examiners should be aware of possible differential diagnoses whose ultrasound image may mimic a dissection. A frequently occurring but avoidable cause of misdiagnosis is idiopathic carotidynia. Ultrasound also enables differentiation between dissection and vasculitis or carotid web and detection of normal variants such as fenestration of the vertebral artery. Further possibilities for misdiagnosis may arise in the presence of a variant of the ascending pharyngeal artery or in extracranial vasospasm. The different imaging techniques for the detection of a dissection are complementary, as false-negative findings occur with all techniques; no method serves as the gold standard. In any case, ultrasound can make an important contribution to the detection of a dissection, and it is worth knowing the diagnostic criteria.

Association between cervical artery dissection and spinal manipulative therapy -a medicare claims analysis.

BACKGROUND: Cervical artery dissection and subsequent ischemic stroke is the most serious safety concern associated with cervical spinal manipulation. METHODS: We evaluated the association between cervical spinal manipulation and cervical artery dissection among older Medicare beneficiaries in the United States. We employed case-control and case-crossover designs in the analysis of claims data for individuals aged 65+, continuously enrolled in Medicare Part A (covering hospitalizations) and Part B (covering outpatient encounters) for at least two consecutive years during 2007-2015. The primary exposure was cervical spinal manipulation; the secondary exposure was a clinical encounter for evaluation and management for neck pain or headache. We created a 3-level categorical variable, (1) any cervical spinal manipulation, 2) evaluation and management but no cervical spinal manipulation and (3) neither cervical spinal manipulation nor evaluation and management. The primary outcomes were occurrence of cervical artery dissection, either (1) vertebral artery dissection or (2) carotid artery dissection. The cases had a new primary diagnosis on at least one inpatient hospital claim or primary/secondary diagnosis for outpatient claims on at least two separate days. Cases were compared to 3 different control groups: (1) matched population controls having at least one claim in the same year as the case; (2) ischemic stroke controls without cervical artery dissection; and (3) case-crossover analysis comparing cases to themselves in the time period 6-7 months prior to their cervical artery dissection. We made each comparison across three different time frames: up to (1) 7 days; (2) 14 days; and (3) 30 days prior to index event. RESULTS: The odds of cervical spinal manipulation versus evaluation and management did not significantly differ between vertebral artery dissection cases and any of the control groups at any of the timepoints (ORs 0.84 to 1.88; p > 0.05). Results for carotid artery dissection cases were similar. CONCLUSION: Among Medicare beneficiaries aged 65 and older who received cervical spinal manipulation, the risk of cervical artery dissection is no greater than that among control groups.

Cervical Artery Dissection in Postpartum Women after Cesarean and Vaginal Delivery.

BACKGROUND AND AIMS: Cervical artery dissection (CAD) is an infrequent but potentially disabling and fatal disease, accounting for up to 25 % of strokes in young adults. Pregnancy-related hormonal changes and increased hemodynamic stress on artery walls during vaginal delivery have been associated to CAD. We aim to describe a series of women presenting CAD during postpartum (PP) after cesarean and vaginal delivery. METHODS: CAD women admitted to one hospital in Santiago, Chile, between July 2018 and October 2020 were included in a prospective cohort. Demographic, clinical and imaging data were registered for the PP group. RESULTS: Sixty-seven women were diagnosed with CAD, from which 10 were PP. Seven women had cesarean section and 3 had vaginal delivery. They presented CAD related symptoms after a median of 10.5 (IQR 5-15) days from delivery. All of them had headache as initial symptom, 9 presented cervical pain and 8 had a family history of stroke. Four patients presented preeclampsia during pregnancy. Acute treatment consisted mostly in antiplatelet agents and analgesics. None of these patients had a CAD related stroke. Demographic, clinical and imaging characteristics of these women with CAD during PP are described. CONCLUSIONS: This case series underpins the importance of clinical suspicion of CAD after delivery, highlighting the fact that CAD is not limited to women with vaginal delivery, thus alternative causes beyond acute hemodynamic stress could be involved. Further research is required to determine genetic components, along with deeper knowledge of modulating factors related to CAD in this setting.

Spontaneous Cervical Artery Dissection in Vascular Ehlers-Danlos Syndrome: A Cohort Study.

BACKGROUND AND PURPOSE: Vascular Ehlers-Danlos syndrome is a rare inherited connective tissue disorder because of pathogenic variants in the COL3A1 gene. Arterial complications can affect all anatomic areas and about 25% involve supra-aortic trunks (SATs) but no systematic assessment of cervical artery lesions has been made. The primary objective was to determine an accurate prevalence of spontaneous SAT lesions in a large series of patients with vascular Ehlers-Danlos syndrome at diagnosis and during follow-up. Secondary objectives were to study their neurological consequences (transient ischemic attack or stroke) and the possible relationships with sex, genotype, ascertainment status. METHODS: A retrospective review of a monocentric cohort of patients with molecularly proven vascular Ehlers-Danlos syndrome followed in a tertiary referral center from 2000 to 2017. RESULTS: One hundred forty-four patients were analyzed, 56.9% (n=82) had SAT lesions: 64.6% females, 74.4% index-case patients. Most lesions were identified in early arterial assessment (48% at first work-up, mean age of 35.7±13.0 years). Cumulative incidence of a first identification of a SAT lesion was 41.7% at 40 years old. On the complete period of survey, 183 SAT lesions (with 132 dissections and 33 aneurysms) were identified, mainly in internal carotid arteries (56.3%) and vertebral arteries (28.9%), more rarely in patients with COL3A1 null mutations (P=0.008). Transient ischemic attack or stroke were reported in n=16 (19.5%) of the 82 patients with SAT lesions without relation with age, sex, treatment, or hypertension. CONCLUSIONS: Cervical artery lesions are frequent and mostly asymptomatic in patients with vascular Ehlers-Danlos syndrome. Local dissections and aneurysms are the most frequent type of lesions, but transient ischemic attack or stroke seem rare.

Delayed vertebral artery dissection after mild trauma in a motor vehicle collision.

Vertebral artery dissection can be insidious and is a leading cause of stroke in young patients, second only behind cardioembolic events [1]. We present the case of a 42-year-old patient who presented to the emergency department with diaphoresis and a complaint of neck pain one month after a low speed motor vehicle collision. The patient was transferred to a stroke center where cerebral angiography showed severe vertebral artery stenosis with likelihood of dissection after a noncontrast CT was negative for hemorrhage. She was definitively treated with antiplatelet therapy and discharged to rehab. By reviewing the most recent literature, we better define this illness. Most commonly, patients with arterial dissection present with head or neck pain, stroke, and Horner syndrome. It is now thought that vertebral artery dissection is a multifactorial disease process where certain intrinsic factors are present in the setting of an exacerbating extrinsic factor such as a low speed car accident, direct trauma, heavy lifting, or a rotational sports injury. And while our patient was treated with antiplatelet therapy and intravascular intervention, vertebral artery dissection is rare and further research is required to better guide treatment as there is no definitive data showing superiority of either anticoagulation or antiplatelet pharmaceutics.

Massive Brainstem and Cerebellum Infarction Due to Traumatic Extracranial Vertebral Artery Dissection in a Motor Traffic Accident: An Autopsy Case Report.

ABSTRACT: Traumatic unilateral vertebral artery (VA) injury can cause focal thrombosis and may block the basilar artery which can further lead to ischemic stroke and massive cerebral infarction and subarachnoid hemorrhage and even rapid death. Here, we present an autopsy case with a traumatic extracranial VA dissection in a motor traffic accident, and the identification of cause of death underwent 2 autopsies. A 62-year-old male pedestrian collided with the right rearview mirror of a car and fell down to the ground. He complained pains in the head and neck. Head computed tomography (CT) showed a right linear temporal bone fracture and a small left temporal subdural hematoma. Neck CT and magnetic resonance imaging (MRI) examination showed left transverse process fracture of the sixth cervical vertebra (C6) and left extracranial VA injury. After 6 days of hospitalization, the left temporal subdural hematoma had been nearly absorbed, and the man was discharged home. On day 15 after the traffic accident, the man suffered sudden unconsciousness accompanied by frequent vomiting at home. The man was taken to hospital, and there were no obvious abnormal findings by head CT examination. However, the man soon fell into a coma state and died 2 days later. The first autopsy was performed 7 days after death and confirmed a left transverse process fracture of C6 and that the deceased died of cerebral infarction and secondary subarachnoid hemorrhage caused by blunt force in the motor traffic accident. In the civil lawsuit, the traffic accident wrecker put forward the objection whether the deceased had fatal diseases. The second autopsy (84 days after the death) findings verified the left extracranial VA injury. Histopathological examination further showed intimal dissection and thrombus formation of the left extracranial VA and secondary basal arterial thromboembolism. Massive infarction of the brainstem and cerebellum was disclosed. Therefore, the deceased died of delayed massive brainstem and cerebellum infarction because of left extracranial VA dissection in the motor traffic accident.

Dual origin of vertebral artery and intimal relationship with spontaneous artery dissection: a case report and systematic review.

Dual origin of the vertebral artery (VA) is an uncommon anatomical variation. We describe a case of duplication origin at right vertebral artery (RVA) found incidentally in one elderly patient on investigation for cerebral aneurysm. Angiogram study showed a pseudoaneurysm at V4 segment of the RVA associated with embryological anatomy. The authors performed a systematic review of the similar cases reported worldwide.

Differential Risk Factors and Outcomes of Ischemic Stroke due to Cervical Artery Dissection in Young Adults.

INTRODUCTION: Cervical artery dissection (CeAD) is a major cause of ischemic stroke in young adults. Our understanding of the specific risk factors and clinical course of CeAD is still evolving. In this study, we evaluated the differential risk factors and outcomes of CeAD-related strokes among young adults. METHODS: The study population consisted of young patients 15-45 years of age consecutively admitted with acute ischemic stroke to our comprehensive stroke center between January 1, 2010, and November 30, 2016. Diagnosis of CeAD was based on clinical and radiological findings. Univariate and multivariable logistic regression analyses were used to assess the risk factors and clinical outcomes associated with CeAD-related strokes. RESULTS: Of the total 333 patients with acute ischemic stroke included in the study (mean ± SD age: 36.4 ± 7.1 years; women 50.8%), CeAD was identified in 79 (23.7%) patients. As compared to stroke due to other etiologies, patients with CeAD were younger in age, more likely to have history of migraine and recent neck manipulation and were less likely to have hypertension, diabetes, and previous history of stroke. Clinical outcomes of CeAD were comparable to strokes due to other etiologies. Within the CeAD group, higher initial stroke severity and history of tobacco use were associated with higher modified Rankin Scale score at follow-up. CONCLUSIONS: While history of migraine and neck manipulation are significantly associated with CeAD, most of the traditional vascular risk factors for stroke are less prevalent in this group when compared to strokes due to other etiologies. For CeAD-related strokes, higher initial stroke severity and history of tobacco use may be associated with higher stroke-related disability, but overall, patients with CeAD have similar outcomes as compared to strokes due to other etiologies.

Imaging features of vertebral artery fenestration.

PURPOSE: Vertebral artery fenestration (VAF) is a rare congenital vascular anomaly which has been associated with intracranial aneurysm. VAF can share some similar imaging features with vertebral artery dissection (VAD), which may confound diagnosis of the latter on CT and MR angiography. The purpose of this investigation is to assess the prevalence of VAF, evaluate its association with other vascular anomalies, and identify imaging features to help in distinguishing VAF and VAD. METHODS: Using keyword search on CTA and MRA head and neck imaging reports from 2010 to 2017, cases of VAF and VAD were retrospectively identified and imaging was reviewed. Imaging features including laterality; vertebral segment; length of affected segment; presence, number, and caliber of lumen(s); and presence of other vascular abnormalities were recorded for all cases and subsequently compared using Pearson's chi-squared test to assess for significant differences between the groups. Patient age, gender, and clinical presentations were also recorded. RESULTS: Of 64,888 CT and MR angiographic examinations performed, VAF was identified in 67 (0.1%) and VAD in 54 (0.1%) patients. Compared with VADs, VAFs were shorter in length (p < 0.001), wider in luminal diameter (p < 0.001), more likely to occur at the V4 segment (p < 0.01), more likely to have two distinct lumens rather than one (p < 0.01), and less likely to present post-trauma (p < 0.01). Coexisting intracranial aneurysms were identified in 9% of patients with VAF. CONCLUSION: VAFs, although rare, can be readily distinguished from VADs on angiographic imaging. Diagnosis of VAF should prompt review for intracranial aneurysm.

Traumatic cervical vertebral artery dissection: A case with cerebral infarct due to newly formed thrombus in the cerebral arteries.

We report a 50-year-old man who developed fatal brainstem infarction five days after traumatic cervical vertebral artery dissection (CVAD). Autopsy revealed multiple fresh infarcts in the territory of the vertebrobasilar system. No thrombus was found in the infarct lesions. The cervical vertebral artery (CVA) showed severe atherosclerotic stenosis extending to the proximal half of the left side, similar stenosis at the origin on the right side, fresh thrombotic occlusion extending to the proximal half of the right side, and multiple dissections in the distal foraminal segments on both sides. In the distal half of the basilar artery (BA) and the origin of the right posterior cerebral artery (PCA), the lumen was extensively filled with fresh thrombus. Although an intricate mixture of white and red thrombi filled the lumen at the origin of the right PCA, the white thrombus gradually appeared at the periphery whereas the red thrombus occupied the central and more proximal part of the BA. We confirm that cerebral infarction associated with CVAD is due not only to emboli originating from the dislodged thrombus at sites of arterial dissection, as reported previously, but also to newly formed thrombus in the cerebral arteries caused by impaired blood flow, as was seen in the present case.

Atlanto-occipital ligament calcification: a novel imaging finding in pediatric rotational vertebral artery occlusion.

We describe a 2-year-old girl with bow hunter syndrome complicated by vertebral artery dissection and multiple ischemic infarcts. Pediatric bow hunter syndrome is a rare and likely under-recognized disorder. Interestingly, our patient had atlanto-occipital ligament calcification on CT scan, an imaging finding that has not been reported in association with bow hunter syndrome and one that might help increase recognition of this dynamic disorder of the posterior circulation.

Anatomical Variations of Vertebrobasilar Artery are Closely Related to the Occurrence of Vertebral Artery Dissection-An MR Angiography Study.

GOAL: Intracranial arterial dissection is a major cause of ischemic stroke and subarachnoid hemorrhage in relatively young patients. We assessed the hypothesis that the tortuosity of the vertebrobasilar artery is associated with the occurrence of vertebral artery (VA) dissection, using MR angiography (MRA). MATERIALS AND METHODS: This study enrolled 43 patients with VA dissection, and 63 age- and sex-matched healthy subjects were used as the controls. MRA was employed to evaluate the presence of dominant VA and the lateral shift of vertebrobasilar junction in both groups. The VA diameters were considered different when the difference was greater than .3 mm. These anatomical variations were divided into 3 types: Type 1 (vertebrobasilar junction within 2 mm from the midline), Type 2 (>2 mm-lateral shift of vertebrobasilar junction to the ipsilateral side of the dominant VA), and Type 3 (>2 mm-lateral shift of vertebrobasilar junction to the contralateral side of the dominant VA). FINDINGS: The presence of dominant VA and the lateral shift of vertebrobasilar junction were more prevalent in patients with VA dissection than in the controls (OR: 3.46, P = .013, and OR: 4.51, P = .001, respectively). The lateral shift of vertebrobasilar junction was classified into Type 1 (n = 6), Type 2 (n = 13), and Type 3 (n = 17) among patients with VA dissection, while into Type 1 (n = 20), Type 2 (n = 8), and Type 3 (n = 7) among the controls. Type 3 predominance was observed in patients with VA dissection (P = .02). CONCLUSIONS: Anatomical variations of the vertebrobasilar artery may play an important role in the occurrence of VA dissection.

"COVID-19 and cervical artery dissection- A causative association?"

COVID-19 is a pandemic disease which predominantly affects the respiratory system, however it also causes multi-organ dysfunction in a subset of patients. There is a growing evidence that it increases the propensity of strokes in younger patients. Besides producing a prothrombotic state, arterial dissection could be one of its many manifestations, increasing the risks of stroke. Herein, we report the first case of spontaneous bilateral vertebral artery dissection in a patient with COVID-19. 39-year female presented with spontaneous bilateral vertebral artery dissections without any instigating traumatic events and no history of connective tissue disorders. Whether this patient's vertebral artery dissections were triggered by exaggerated inflammatory response or arteriopathy secondary to COVID-19 remains speculative. Nonetheless, arterial dissection could be one of it's complications. It is important for the physicians to be aware of different clinical manifestations of COVID-19 as we manage these patients with no historical experience, to provide adequate care.

Vertebral artery dissection caused by atlantoaxial dislocation: a case report and review of literature.

BACKGROUND: Atlantoaxial dislocation (AAD) is the most common craniovertebral junction malformation (CVJm) which are anomalies of the bones and soft tissues surrounding the foramen magnum. It usually leads to neurologic abnormalities because of instability of this mobile area. But vertebral artery dissection (VAD) caused by AAD is uncommon. CASE REPORT: We report a 15-year-old boy who presented with acute onset of bilateral VAD leading to posterior circulation ischemic stroke (PCIS). Computed tomography angiography (CTA) indicated dissection and occlusion of bilateral intracranial vertebral arteries and AAD with os odontoideum. After antithrombotic treatment for 3 months, the patient got complete revascularization and received posterior C1-C2 fusion. DISCUSSION: There have only been tens of cases of PCIS caused by CVJm. We reviewed these relevant literatures and suggested that more attention should be paid to vascular impairment for patients with CVJm.

The distance of cervical vertebral dislocation could be a risk factor for blunt vertebral artery injury after traumatic cervical spine injury.

BACKGROUND: Blunt vertebral artery injury (BVAI) is a well-known potentially fatal complication of cervical spine injury. The condition is reported to be associated with vertebral fractures and cervical hyperextension. However, appropriate patient screening methods remain to be elucidated. This study aimed to identify the risk factors associated with BVAI in patients with cervical spine injury. METHODS: We conducted a retrospective, observational, single-centered study, including 137 patients with cervical spine injury transferred to our center from April 2007 to December 2016. Evaluation for BVAI was available in 62 patients based on magnetic resonance angiography or multi-detector computed tomography angiography. BVAI was classified using the Biffl grade. RESULTS: Among the 62 patients evaluated, 13 (21%) were diagnosed with BVAI. All injuries were classified as Biffl grade 2 (50%) or 4 (50%). Univariate analysis of patients with and without BVAI showed that cervical dislocation (p = 0.041) and low average hemoglobin level (p = 0.032) were associated with BVAI. On multivariate logistic regression analysis, cervical dislocation (odds ratio 1.189; 95% confidence interval 1.011-1.399, p = 0.036) remained a significant predictor of BVAI. Based on receiver operating characteristic (ROC) analysis, a dislocation > 6.7 mm was selected as the optimal cutoff value for prediction of BVAI (sensitivity and specificity, 87.5% and 71.4%, respectively). CONCLUSIONS: BVAI frequently occurred in combination with cervical spine dislocation, and the distance of the cervical dislocation was identified as a useful predictor of BVAI.

A Unique Recurrent Stroke Case due to Bilateral Vertebral Artery Dissection with Familial Hirschsprung Disease.

Vertebral artery (VA) dissection is one major cause of brain infarction in young and middle-aged adults. Risk factors for VA dissection are hypertension, diabetes mellitus, hyperlipidemia, trauma, and genetic factors. A 32-year-old man with familial Hirschsprung disease at the age of 2 presented cerebellar ischemic stroke due to bilateral VA dissections. A stroke recurred within 17 days despite oral dual antiplatelet therapy. Bilateral VA dissections and recurrent dissections are related to genetic mutations associated with connective tissue diseases. A part of familial Hirschsprung disease has genetic factors in common with cerebrovascular disease. There may be a common genetic background between his VA dissection and Hirschsprung disease.