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Background: The long-term neuro-ophthalmic outcomes after carotid intervention in patients presenting with preoperative visual symptoms vary widely based on the underlying etiology of retinal ischemia and are not well understood., Methods: We performed a retrospective review of consecutive patients presenting with retinal ischemia who subsequently underwent carotid intervention from January 2018 to December 2022. Patients were classified into three groups (group I: amaurosis fugax/vascular transient monocular vision loss, group II: ocular ischemic syndrome [OIS], and group III: central/branch retinal artery occlusion). Clinical details and the nature of visual symptoms were recorded. Outcomes analyzed were ipsilateral symptom recurrence, visual improvement, stroke rate, and survival., Results: A total of 90 patients were included in this study (70 male, aged 75 ± 9 years): 31 patients (34%) in group I (amaurosis fugax), 7 patients (8%) in group II (OIS), and 52 patients (58%) in group III central/branch retinal artery occlusion. Atherosclerotic risk factors were similar across groups with similar preoperative antiplatelet and statin use. Interventions performed were carotid endarterectomy in 64 (71%), transfemoral carotid artery stenting in 21 (23%), transcarotid artery revascularization in 4 (4%), and carotid artery bypass in 1 patient (1%). The median follow-up was 38.5 months (range: 0-207 months). There was no recurrence of transient or permanent retinal ischemic events in any patient in group I. In group II, 5 of 7 patients presenting with transient symptoms of OIS showed resolution of symptoms and ocular signs. Two patients presenting with permanent vision loss in group II had no improvement but no worsening symptoms, and visual decline was reported in two patients in group III. Ipsilateral stroke rate was 2% at 5 years for the entire group. Survival was 93% and 82% at 1 and 5 years, respectively, with no difference between groups (P < .05). There was one postoperative death from ischemic stroke secondary to stent thrombosis within 30 days (group III), with no long-term mortality from cerebrovascular disease in the rest of the cohort., Conclusions: Neuro-ophthalmic outcomes after carotid intervention for visual symptoms are favorable with low symptomatic recurrence after both carotid endarterectomy and carotid artery stenting. Intervention for OIS when detected early (with transient symptoms) is associated with resolution of symptoms and prevention of permanent visual loss., Competing Interests: Disclosures None., (Copyright © 2024 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

A 28-year-old male presented with a sudden, transient visual impairment in the right eye. Brain MRI revealed no obvious ischemic lesions, and he was diagnosed with amaurosis fugax. Physical examination revealed characteristic features of Marfan syndrome, along with a family history of tall stature and valvular heart disease. Further investigation revealed: 1) an ascending aortic aneurysm, 2) a bicuspid aortic valve with fenestration and regurgitation, and 3) a mitral valve prolapse with regurgitation. Genetic testing identified a heterozygous variant c.6905G>A of FBN1, confirming the diagnosis of Marfan syndrome. Bentall operation and mitral valve replacement were performed, resulting in a favorable outcome. Pathological examination showed extensive myxomatous degeneration with thickening of the aortic and mitral valves, suggesting a potential source of embolism.

Objective: Plaque ulceration in carotid artery stenosis is a risk factor for cerebral ischemic events; however, the characteristics that determine plaque vulnerability are not fully understood. We thus assessed the association between plaque ulceration sites and cerebrovascular ischemic attack., Methods: We retrospectively collected the clinical data of 72 consecutive patients diagnosed with carotid artery stenosis with plaque ulcers. After excluding patients with pseudo-occlusion, a history of previous carotid endarterectomy or carotid artery stenting before the ulcer was first discovered, follow-up data of less than 1 month, or carotid endarterectomy or carotid artery stenting performed within 1 month after the ulcer was first discovered, 60 patients were ultimately included. Patients were divided into proximal and distal groups based on the ulcer location relative to the most stenotic point. The primary endpoints were ipsilateral cerebrovascular ischemic events ("ischemic events"), such as amaurosis fugax, transient ischemic attack, or ischemic stroke due to carotid artery stenosis with plaque ulceration. The association between ulcer location and ischemic events was also assessed., Results: In the patients with plaque ulcer, more patients had proximal than distal plaque ulcers (39 vs 21; P = .028). The median follow-up duration was 3.8 years (interquartile range, 1.5-6.2 years). Nineteen patients (32%) experienced ischemic event. Ischemic events occurred more frequently in the distal than in the proximal group (18% vs 59%; P = .005). Kaplan-Meier curves demonstrated a significantly shorter event-free time in the distal group (log-rank P = .021). In univariate analysis, distal ulcer location was associated with ischemic events (odds ratio [OR], 2.94; 95% confidence interval [CI], 1.13-7.65; P = .03). Multivariate analysis using two different models also showed that distal ulcer location was independently associated with ischemic events (Model 1: OR, 3.85; 95% CI, 1.26-11.78; P = .03; Model 2: OR, 4.31; 95% CI, 1.49-12.49; P = .009)., Conclusions: Patients with carotid artery stenosis and plaque ulcers located distal to the most stenotic point are more likely to experience cerebrovascular ischemic attacks. Therefore, carotid plaques with ulcers located distal to the most stenotic point may be a potential indication for surgical treatment., Competing Interests: Disclosures None., (Copyright © 2024 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: To report a rare case of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) presenting with optic neuritis and central retinal vein occlusion (CRVO)., Background: Optic neuritis is the most common presentation of MOGAD. It classically presents as an acute or subacute, unilateral, painful vision loss, with exam showing relative afferent pupillary defect. CRVO can have a similar clinical presentation to optic neuritis, however, CRVO is not usually associated with optic neuritis nor MOGAD., Design/methods: N/A., Results: A 64 year-old man with history of hypertension, diabetes, and ocular migraines presented with 10 days of progressive right eye vision loss and headache. Fundoscopic exam was notable for grade 4 disc edema and significant burden of dot blot hemorrhages in all quadrants of the right eye. Intravenous fluorescein angiography demonstrated delayed arterial and venous filling and transit time consistent with CRVO. MRI brain and orbits showed enhancement of the posterior portion of the right optic nerve extending into the intracanalicular area, and an enhancing lesion involving the right middle cerebellar peduncle and right anterolateral pons. CSF was notable for mildly elevated protein without pleocytosis. Serum laboratory workup was positive for MOG IgG antibody with a titer of 1:100. MRI spine, and CT chest/abdomen/pelvis were nonrevealing. The rest of serum and CSF analysis including comprehensive infectious, inflammatory and autoimmune panels were all unremarkable. The patient was diagnosed with MOGAD, and he was treated with high dose intravenous methylprednisolone and plasmapheresis. Upon follow up in clinic, his vision has improved, and repeat MOG titer was negative. He was started on Rituxan for maintenance treatment., Conclusions: MOGAD optic neuritis can result in CRVO. In the appropriate clinical context, a diagnosis of MOGAD should be considered in patients presenting with CRVO, and the proper neuro-imaging and laboratory testing should be ordered. Disclosure: Dr. Schichtel has nothing to disclose. Dr. Ibrahim has nothing to disclose. Dr. Zhang has nothing to disclose.

Optic perineuritis is the inflammation of the optic nerve sheath. This affliction can lead to visual field impairment and other signs and symptoms related to the orbital space, such as pain, disc edema, ophthalmoplegia, proptosis. However, not all patients present with such suggestive symptoms, requiring a thorough assessment. We report the case of a young male admitted to our hospital for recurrent episodes of monocular blindness. Amaurosis fugax is a well-known presentation of transient ischemic attacks (TIA) and it was ruled out. Gadolinium-enhanced MRI revealed a typical aspect of optic perineuritis. It was mandatory to consider all possible causes of secondary optic perineuritis as they all represent serious clinical conditions, even if the idiopathic form is more frequent. The clinical and paraclinical evaluation of the patient excluded an underlying disease and primary optic perineuritis was diagnosed. Corticosteroid therapy is usually curative and a course of methylprednisolone was initiated for our patient with good outcome. However, response to treatment is not diagnostic as both primary and secondary optic perineuritis are normally responsive, hence thorough differential diagnosis is necessary., (© 2024 Maria Mirabela Manea et al., published by Sciendo.)

Objective: The National Stroke Association and the American Heart Association consider retinal ischemia, as in the case of amaurosis fugax (AF), to be a stroke event. The purpose of this study was to evaluate the risk factors for ischemic cerebral stroke in patients hospitalized for acute AF., Methods: The National Inpatient Sample Database from 2002 to 2014 was used to identify patients 21 years of age and older with a primary admission diagnosis of AF with the ICD-9 code 362.34. Comorbidity measures and in-hospital events were extracted using relevant ICD-9 codes. Statistical analyses were performed using IBM SPSS 25 and R package., Results: A weighted total of 12,142 patients was identified. The most common comorbidities in this cohort with AF included hypertension, dyslipidemia, tobacco use, coronary artery disease (CAD), and diabetes mellitus. Multivariable regression analysis showed comorbidities of hypercoagulable state, systemic vasculitis, CAD, and atherosclerosis to be independent risk factors for ischemic stroke in patients with AF. In contrast, dyslipidemia was associated with a decreased risk. Asian/Pacific Islander race conferred a 5-fold increased risk compared with Whites., Conclusion: Ischemic stroke and myocardial infarction were diagnosed in 0.3%-0.9% of hospitalized acute AF cases. Presence of hypercoagulable state, systemic vasculitis, CAD, and atherosclerosis each individually increased the risk of ischemic stroke by more than 3-fold; patients with these risk factors and acute AF should be closely monitored for developing acute systemic thrombotic events., (Copyright © 2022 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Competing Interests: The authors report no conflicts of interest.

Background: Retinal migraine is a diagnosis of exclusion and is characterized by repeated episodes of transient monocular blindness associated with migraine. We report a case of systemic lupus erythematosus with acute episodes mimicking retinal migraines., Case Report: A 46-year-old woman with a history of migraine with aura since her 20s and Evans syndrome presented with episodic transient monocular blindness. Retinal migraine was considered as the cause, and migraine prophylaxis initially reduced its frequency. After 5 months, the frequency increased, with chilblain-like lupus lesions on her extremities. Laboratory testing revealed lymphopenia and hypocomplementemia, fulfilling the diagnostic criteria for systemic lupus erythematosus, which may have caused Evans syndrome and transient monocular blindness, mimicking retinal migraines. After intravenous methylprednisolone and rituximab therapy, the transient monocular blindness episodes did not recur., Conclusion: Given the clinical presentation, systemic lupus erythematosus should be considered as a cause of transient monocular blindness and should be distinguished from retinal migraine., Competing Interests: Declaration of conflicting interestsTTa is a consultant/advisor and/or serves on an advisory board for Otsuka, Eli Lilly, Amgen, Pfizer and Teijin. TTa received speaker honoraria from Otsuka, Eli Lilly, Daiichi Sankyo, Amgen, Kowa, Kyowa Kirin, Eisai, UCB Japan, Takeda and Santen Pharmaceutical, as well as research funding from Eli Lilly and Tsumura outside the submitted work. JN received honoraria and research scholarships from Amgen and Daiichi Sankyo.

Competing Interests: Competing interests: None declared.

BACKGROUND A persistent primitive hypoglossal artery (PPHA) is a rare congenital anomaly leading to persistent carotid-basilar anastomosis. This is a report of an 83-year-old man with a PPHA presenting with amaurosis fugax of the left eye requiring carotid endarterectomy under regional anesthesia. CASE REPORT An 83-year-old man presented with 2 weeks of intermittent self-resolving visual disturbances, followed by an episode of left eye amaurosis fugax. The patient had been referred to the hospital for further investigation of symptoms 1 day following the amaurosis fugax event. Carotid Doppler ultrasound demonstrated a greater than 90% stenosis of the left internal carotid artery. Computed tomography carotid and Circle of Willis angiography confirmed a mixed, ulcerated plaque and revealed a persistent left hypoglossal artery originating from the left internal carotid artery and continuing as the basilar artery. On day 3 of admission, left carotid endarterectomy was performed under conscious sedation and regional anesthesia to permit continuous monitoring of neurological status and avoid the need for intraoperative shunting. "Permissive hypertension" by targeting a systolic blood pressure of 190 to 200 mmHg was sought for the duration of clamp time. There was no deterioration of neurological function during clamping of the carotid vessels. The patient recovered well and was discharged 2 days after surgery, with no residual neurology. CONCLUSIONS This report has presented a rare case of PPHA to highlight awareness of this congenital vascular anomaly when undertaking carotid endarterectomy.

Objective: Shunt placement during carotid endarterectomy (CEA) has often been advocated to protect the ischemic penumbra in patients with symptomatic carotid stenosis. In the present study, we assessed the effect of shunt placement during CEA on postoperative stroke risk in symptomatic patients., Methods: We queried the American College of Surgeons National Surgical Quality Improvement Program database (2016-2019) for CEA cases with complete CEA procedure-targeted data available. Symptomatic patients were identified as those with a preoperative diagnosis of stroke on presentation (DS), transient ischemic attack, amaurosis fugax, or temporary monocular blindness. The DS patients were further analyzed according to the severity of their stroke using the modified Rankin scale scores. To better assess the effect of shunt placement on the stroke rate, we compared cases of CEA with the patch angioplasty technique stratified by the use of an intraoperative shunt. Patients who had undergone carotid eversion or primary closure were excluded. The baseline demographics and perioperative outcomes were compared using the χ 2 and Mann-Whitney U tests. Multivariate analysis was performed to identify the independent risk factors for postoperative stroke and cranial nerve injury., Results: We identified 4652 cases of CEA with patch angioplasty in symptomatic patients, including 1889 with (40.6%) and 2763 without (59.4%) shunt placement. The distribution of age, race, and sex was similar for both procedures. Compared with patients without a shunt, those with a shunt had significantly higher rates of emergency surgery (9.1% vs 7.0%; P = .010), nonelective surgery (40.3% vs 37.2%; P = .035), general anesthesia (97.0% vs 86.3%; P < .001), and bleeding disorders (27.2% vs 22.7%; P < .001). The 30-day incidence of postoperative stroke was similar between the patients with (3.2%) and without (2.6%) shunt placement (P = .219). Additionally, a subgroup analysis failed to show any benefit from shunt placement on the incidence of postoperative stroke, regardless of the preoperative symptoms or neurologic disability. In contrast, shunt placement was associated with an increased rate of cranial nerve injury (4.1% vs 2.4%; P = .001). Multivariate analysis revealed that nonelective surgery (odds ratio [OR], 1.99; 95% confidence interval [CI], 1.36-2.91; P < .001) and DS (vs transient ischemic attack, amaurosis fugax, or temporary monocular blindness; OR, 1.64; 95% CI, 1.12-2.41; P = .012) were predictive of 30-day postoperative stroke. After adjusting for confounders, shunt placement had no effect on stroke risk at 30 days but remained an independent risk factor for cranial nerve injury (adjusted OR, 1.87; 95% CI, 1.32-2.64; P < .001)., Conclusions: For symptomatic patients undergoing CEA with patch angioplasty, shunt placement was associated with an increased risk of cranial nerve injury without a reduction in postoperative stroke risk., (Copyright © 2022 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Purpose: Report the case of a patient with a history of central retinal artery occlusion in her right eye and amaurosis fugax associated with acute ischemic changes in her left eye related to a prothrombin G20210A gene variant, in which OCT-A was used as a diagnostic and therapeutic tool., Case Presentation: 55-year-old woman with a history of central retinal artery occlusion in her right eye and prothrombin gene G20210A (F2) variant diagnosis. She presented to our consultation with amaurosis fugax in her left eye. As medical history, she had an episode of bilateral posterior scleritis diagnosed asynchronously with the current episode. Vascular, autoimmune, and metabolic prothrombotic diseases were ruled out. OCT-A showed areas suggesting acute ischemia consistent with macular retinopathy in her left eye. Anticoagulant therapy with Apixaban was initiated, considering the risk for her vision. Control OCT-A showed perfusion improvement in the previous site of the occlusive vascular event. We also considered the extent of the inflammatory response due to posterior scleritis as a differential diagnosis. Nevertheless, it is less likely, considering the temporality between scleritis and the retinal-vascular episodes., Conclusions: While the G20210A prothrombin gene (F2) variant is a rare cause of retinal artery occlusion, it is important to consider it a differential diagnosis. Good visual outcomes can be achieved with prompt initiation of antithrombotic treatment. In addition, OCT-A is useful for diagnosing ischemic retinal changes that cannot be observed with other diagnostic methods and monitoring them.

We describe an unusual case of a 73-year-old man with amaurosis fugax. He had repeated transient monocular symptoms, one of which features lighting bolt-shaped glittering in the full visual field of the right eye since medical treatment for hypertension and diabetes mellitus started. A few days later, he felt difficulty in speech as well as sensory and motor disturbance in the left upper extremity, which finally brought him to our hospital. An MR scan unveiled subacute infarctions dotted in the right cerebral hemisphere and severe carotid stenosis on the same side with a delayed distal flow. He was hospitalised with diagnosed an ischemic stroke. Despite being treated with antithrombotic agents, he had suffered similar visual symptoms repeatedly and therefore, carotid artery stenting was performed on Day 16 starting from the onset. The right ophthalmic artery and choroidal crescent became depicted angiographically after our surgery had been completed. His visual disturbance has never appeared since then. Consequently, it is suggested that the optic disorders were attributed to a hemodynamically precarious state in the area of the ophthalmic artery.

Abstract: Transient ischemic attack (TIA) is defined as a transient episode of neurological dysfunction resulting from focal brain, spinal cord, or retinal ischemia, without associated infarction. Consequently, a TIA encompasses amaurosis fugax (AF) that is a term used to denote momentary visual loss from transient retinal ischemia. In this review, we use the word TIA to refer to both cerebral TIAs (occurring in the brain) and AF (occurring in the retina). We summarize the key components of a comprehensive evaluation and management of patients presenting with cerebral and retinal TIA.All TIAs should be treated as medical emergencies, as they may herald permanent disabling visual loss and devastating hemispheric or vertebrobasilar ischemic stroke. Patients with suspected TIA should be expeditiously evaluated in the same manner as those with an acute stroke. This should include a detailed history and examination followed by specific diagnostic studies. Imaging of the brain and extracranial and intracranial blood vessels forms the cornerstone of diagnostic workup of TIA. Cardiac investigations and serum studies to evaluate for etiological risk factors are also recommended.The management of all TIAs, whether cerebral or retinal, is similar and should focus on stroke prevention strategies, which we have categorized into general and specific measures. General measures include the initiation of appropriate antiplatelet therapy, encouraging a healthy lifestyle, and managing traditional risk factors, such as hypertension, dyslipidemia, and diabetes. Specific management measures require the identification of a specific TIA etiology, such as moderate-severe (greater than 50% of stenosis) symptomatic extracranial large vessel or intracranial steno-occlusive atherosclerotic disease, aortic arch atherosclerosis, and atrial fibrillation., Competing Interests: The authors have no conflicts of interest to declare., (Copyright © 2022 Asia-Pacific Academy of Ophthalmology. Published by Wolters Kluwer Health, Inc. on behalf of the Asia-Pacific Academy of Ophthalmology.)

Significance: Protein C deficiency is a thrombophilic condition that increases the risk of venous and arterial thrombi, the latter of which can cause transient monocular vision loss. In cases of recurrent transient monocular vision loss, in which the typical stroke workup has been unrevealing, investigation for hypercoagulable states is warranted., Purpose: This study reports a case of transient monocular vision loss secondary to protein C deficiency in a patient with no known personal or family history of venous thromboembolism and highlights the eye care provider's role in helping with diagnosis of this condition., Case Report: A 59-year-old woman presented with recurrent transient monocular vision loss of the right eye. Her history was remarkable for suffering an ischemic stroke with hemorrhagic conversion shortly after experiencing episodes of transient monocular vision loss. These episodes initially waned but recurred 3 months later. Extensive workup at the time of recurrence of her visual symptoms was unrevealing. Given the timing of her visual symptoms and history of stroke, her presentation was suggestive of transient ischemic attacks. Her previous extensive workup and chronicity of symptoms did not necessitate emergent evaluation. However, additional workup for hypercoagulable conditions was initiated. The testing revealed protein C deficiency, which prompted initiation of oral anticoagulants for stroke prophylaxis., Conclusions: Transient monocular vision loss is a symptom commonly encountered by eye care providers, which necessitates emergent evaluation to reduce stroke risk if the symptom appears vascular in origin. Testing for hypercoagulable conditions is indicated in patients demonstrating recurrent transient monocular vision loss, even if there is no known personal or family history of venous thromboembolism. Eye care providers need to be aware of this association between hypercoagulable conditions and transient vision loss to aid in prompt diagnosis and treatment with the goal of preventing stroke and permanent vision loss., Competing Interests: Conflict of Interest Disclosure: KAM is a consultant and speaker for RVL Pharmaceuticals and Osmotica Pharmaceuticals, which have no relation to this manuscript., (Copyright © 2021 American Academy of Optometry.)

Competing Interests: The authors report no conflicts of interest.

Objective: The risk of ipsilateral neurological recurrence (NR) was assessed in patients awaiting carotid endarterectomy (CEA) due to symptomatic carotid artery stenosis and whether current national guidelines of performing CEA within 14 days are adequate in present day practice., Methods: This was a retrospective multicentre observational cohort study. Patients scheduled for CEA due to symptomatic carotid artery stenosis in a five year period, 1 January 2014 to 31 December 2018, from four centres were included. Data from the Danish Vascular Registry (www.karbase.dk), operative managing systems, and electronic medical records were reviewed., Results: In total, 1 125 patients scheduled for CEA were included and 1 095 (97%) underwent the planned surgery. During a median delay from index event to CEA of 11 days (interquartile range 8-16 days), 40 patients (3.6%; 95% confidence interval [CI] 2.5%-5%) experienced a NR. One third were minor strokes (n = 12, 30%); half were transient ischaemic attacks (TIA) (n = 22, 55%); and amaurosis fugax accounted for 15% (n = 6). Twenty-six (2%) CEA procedures was cancelled, of which one was due to a disabling recurrent ischaemic event (aphasia). There were no deaths or major strokes in the waiting time for CEA. Best medical treatment (BMT) with platelet inhibitory or anticoagulation drugs and a statin was initiated in nearly all patients (98%) at first assessment. The overall 30 day risk of a post-operative major event (death or stroke) was (Kaplan-Meier [KM] estimate) 2.7% (95% CI 1.8-3.8), and not significantly correlated with the timing of surgery. Most (69%) occurred within the first three days. One, two, and three year mortality rate for CEA patients was (KM estimate) 4.8%, 7.8%, and 11.5% respectively., Conclusion: In symptomatic carotid artery stenosis patients awaiting CEA, very few NRs occurred within 14 days. Institution of immediate BMT in specialised TIA/stroke units followed by early, but not necessarily urgent, CEA is a reasonable course of action in patients with high grade symptomatic carotid artery stenosis., (Copyright © 2021 European Society for Vascular Surgery. Published by Elsevier B.V. All rights reserved.)

Objective: Across stroke subtypes, carotid artery stroke carries the highest risk of recurrence. Despite initiation of best medical therapy (BMT), some patients suffer recurrent neurological events before undergoing carotid endarterectomy (CEA). The aim was to identify clinical predictors of early recurrent events in patients with symptomatic carotid stenosis (sCS) awaiting CEA on modern BMT., Methods: The Helsinki Carotid Endarterectomy Study 2 (HeCES2) is a cross sectional, longitudinal, prospective, and consecutive cohort study, which enrolled 500 symptomatic or asymptomatic patients with carotid stenosis scheduled for CEA in a tertiary stroke centre. Symptomatic patients were included for this analysis (n = 324)., Results: Of all 324 patients with sCS, 39 (12%) had a recurrent cerebrovascular event at a median of six days after the index symptom: four had an ischaemic stroke (1.2%), 16 a hemispheric transient ischaemic attack (TIA; 4.9%), and 19 amaurosis fugax (AFX; 5.9%). The recurrence rate was 4.0 % (n = 13) within 48 h and 9.9% (n = 32) within two weeks. None of the patients (n = 108) presenting with ocular symptoms (AFX or retinal artery occlusion) suffered recurrent hemispheric TIA or stroke. In Cox regression analysis, comorbid hypertension (hazard ratio [HR] 6.58, 95% confidence interval [CI] 1.33-32.47), hemispheric TIA as the index symptom (HR 3.42, 95% CI 1.70-6.90), the number of prior attacks (HR 1.12, 95% CI 1.08-1.15), and high low density lipoprotein/high density lipoprotein ratio (HR 1.51, 95% CI 1.09-2.11) were independently associated with an increased risk of recurrent event, while a history of major cardiovascular event (HR 0.33, 95% CI 0.11-0.96) and high serum fibrinogen level (HR 0.59, 95% CI 0.41-0.86) were associated with a decreased risk., Conclusion: More than every tenth patient with sCS experienced an early recurrent cerebrovascular event prior to scheduled CEA, despite optimal medication. However, stroke recurrence was lower than in earlier observational studies, which could be explained by improved care pathways, more aggressive medication, and expedited CEA. All recurrent strokes occurred in patients initially presenting with minor stroke., (Copyright © 2020 European Society for Vascular Surgery. Published by Elsevier B.V. All rights reserved.)

Online supplemental material is available for this article.

Background: The aim of this study was to report the feasibility and outcomes with the endovascular treatment of spontaneous internal carotid artery dissections (ICADs) using a proximal embolic protection device (EPD)., Methods: This is a retrospective analysis of patients treated for spontaneous symptomatic ICAD using a proximal EPD from January 2017 to December 2018. Indication for treatment was the presence of neurologic symptoms. Early outcomes measured included technical success, perioperative mortality, and major cardiovascular or cerebrovascular complications. Late outcomes were recurrent neurologic symptoms, patency, and reinterventions., Results: A total of 4 male patients with ICAD were included. A preoperative cerebral computed tomography positive for cerebral ischemic events was reported in all cases. In 3 patients, the neurologic symptoms consisted of a transient ischemic attack; the remaining patient presented an amaurosis fugax and aphasia. In 2 patients, ICAD was associated with a carotid significant stenosis. In all patients, the reported approach was feasible with no complications and complete anatomic dissection resolution. At a mean follow-up of 18 months, all stents are patent and no restenosis recurrence or complications were registered., Conclusions: The use of proximal EPDs allowed the treatment of ICAD under flow arrest, minimizing the risk of stroke during the endovascular maneuvers. Larger series are required to validate this treatment strategy., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: This study investigates the prognostic significance of pre-operative symptom status and type of symptom in outcomes after carotid endarterectomy (CEA)., Methods: This review was conducted and reported in accordance with the Preferred Reporting Items for Systematic reviews and Meta-analysis (PRISMA) to identify studies reporting peri-operative outcomes of CEA in symptomatic and asymptomatic patients. The last search was conducted in August 2019 and a methodological assessment was performed using the Newcastle Ottawa Scale. A meta-analysis of outcome data using the odds ratio (OR) as the summary statistic was conducted, and the precision of the effect was reported as 95% confidence interval (CI). Fixed effect or random effects models were used to calculate the pooled estimates., Results: Eighteen studies reporting a total of 91 895 patients were included in the meta-analysis. Asymptomatic patients had a lower peri-operative risk of stroke (OR 0.5, 95% CI 0.45-0.54; p < .001) and death (OR 0.66, 95% CI 0.57-0.77; p < .001) than symptomatic patients, but the risk of myocardial infarction was not significantly different (OR 0.98, 95% CI 0.84-1.15; p = .82). Those suffering a pre-procedural stroke had an increased peri-operative risk of stroke and death vs. patients suffering a pre-procedural transient ischaemic attack or amaurosis fugax., Conclusion: Patients undergoing CEA after a stroke have worse peri-operative outcomes in terms of stroke and death. Further research needs to be performed to ascertain the value of this finding in risk stratification systems and to investigate potential aetiological associations between pre-operative symptom status and peri-operative risk following a CEA., (Copyright © 2020 European Society for Vascular Surgery. Published by Elsevier B.V. All rights reserved.)

With recent advances in medical treatments for carotid artery stenosis (CS), indications for carotid surgery should be more carefully considered for asymptomatic CS (ACS). Accurate stratification of ACS should be based on the risk of cerebral infarction, and subgroups of patients more likely to benefit from surgical treatment should be differentiated. Magnetic resonance imaging (MRI) offers a non-invasive, accurate modality for characterizing carotid plaque. Intraplaque hemorrhage (IPH) seems the most promising feature of vulnerable plaque detectable by MRI. Lectin-like oxidized low-density lipoprotein receptor-1 (LOX-1) is a type II membrane protein of the C-type lectin family with an extracellular domain that can be proteolytically cleaved and released as a soluble form (sLOX-1). This sLOX-1 plays a key role in the pathogenesis of atherosclerosis, and elevated sLOX-1 concentrations correlate with thin or ruptured fibrous caps in patients with acute coronary syndrome. This ongoing study aims to clarify the incidence of ischemic stroke in patients with ACS and IPH confirmed by MRI, and to assess whether sLOX-1 could provide a biomarker for risk of future ischemic events. The study population comprises patients with ACS (>60% area stenosis) associated with MRI-diagnosed IPH receiving follow-up under medical treatment. Primary endpoints comprise transient ischemic attack, stroke or amaurosis resulting from concerned CS. Secondary endpoints comprise any stroke or surgical treatment for progressive luminal stenosis. The target number of patients is 120 and the observational period is 36 months. The study results could help identify individuals with ACS who are refractory to medical therapy.

Objective: We sought to analyze the association between last neurologic event and the risk of stroke or death among patients treated with carotid endarterectomy (CEA) or carotid artery stenting (CAS) under routine conditions in Germany., Methods: Secondary data analysis was performed based on the German statutory quality assurance database for carotid procedures. A total of 144,347 patients treated by CEA and 14,794 patients treated by CAS were included in the analysis. Primary outcome was any in-hospital stroke or death. To analyze the association between the last neurologic event and outcome, multilevel multivariable regression analysis was performed., Results: In patients treated by CEA, raw risk for any in-hospital stroke or death was 2.0% (2923/144,347), with a risk of 1.4% in asymptomatic and 3.0% in symptomatic patients. In patients treated by CAS, raw risk for any in-hospital stroke or death was 3.6% (538/14,794), with a risk of 1.7% in asymptomatic and 6.1% in symptomatic patients. Regression analysis revealed that increasing severity of last neurologic event was significantly associated with an increasing risk of any in-hospital stroke or death in patients treated by both CEA and CAS (P < .004). However, the risk of any stroke or death did not significantly differ between asymptomatic patients and patients with amaurosis fugax before CEA or CAS (P = .219 for CEA, P = .124 for CAS)., Conclusions: Increasing severity of last neurologic event is associated with an increasing risk of any in-hospital stroke or death in patients treated by CEA and CAS. The risk of any stroke or death did not differ between asymptomatic patients and patients with amaurosis fugax., (Copyright © 2019 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Introduction: Isolated monocular ischaemic events are thought to be low risk for stroke recurrence. In the presence of carotid stenosis however, the risks should not be treated similarly and surgical intervention should be considered at an early stage. The aim of this study was to determine the vascular risk profile and stroke recurrence in patients with ischaemic monocular visual loss., Methods and Methods: Consecutive records for all patients with monocular ischaemia were reviewed from January 2014 to October 2016. Stroke, transient ischaemic attack or monocular ischaemia recurrence within 90 days were recorded. Carotid stenosis was assessed with duplex ultrasound, computed tomography or magnetic resonance angiography., Results: In total, 400 patients presented with monocular ischaemia; 391 had carotid imaging (97.8%). Causality was symptomatic carotid stenosis ≥ 50% in 53 (13.6%), including carotid stenosis ≥ 70% in 31 (7.9%). Patients with permanent visual loss ( n = 131) were more likely to have significant stenosis compared with patients with transient visual loss ( n = 260), 19.8% compared with 10.4% ( P = 0.012). Recurrent stroke, transient ischaemic attack or monocular ischaemia within 90 days after presentation occurred in three patients (5.7%) in the carotid stenosis group, compared to three (0.9%) who did not have stenosis ( P = 0.035). Age, male sex and hypertension were associated with carotid stenosis but hypercholesterolaemia, diabetes and smoking were not., Conclusions: Carotid stenosis ≥ 50% is present in patients with ocular ischaemia in approximately 20% of those with persistent visual loss and in 10% with transient visual loss. Those with carotid stenosis have a higher risk of stroke recurrence and should be considered urgent surgical intervention as other forms of stroke.

Amaurosis fugax (AmF) is defined as transient monocular visual loss secondary to retinal ischemia. In most patients presenting with AmF, the attack of visual loss occurs in the same eye. A 64-year-old woman experienced transient visual loss in her right eye. Three days after that, an attack happened on the left side. In total, she had 5 episodes of AmF in 2 months. AmF occurred on both sides at different times, and so may be referred to as "Alternating AmF". Diffusion-weighted magnetic resonance imaging showed high-intensity lesions in various parts of brain, and laboratory examination revealed elevated D-dimer and ovarian tumor marker. We suspected Trousseau syndrome and found a giant ovary tumor. After removal of the tumor, no recurrence was observed. When a patient with alternating AmF is encountered, screening for malignancy is essential., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Retinal migraine is an important differential diagnosis of recurrent transient monocular blindness accompanied by headache when other etiologies are excluded. Here, we report a case of orbital vasculitis which initially mimicked retinal migraine., Case Report: A 47-year-old woman had recurrent episodes of fully reversible transient monocular blindness accompanied by ipsilateral headache for 15 months. The patient's neuroimaging and cardiac and ophthalmologic evaluations were normal. With a diagnosis of retinal migraine, her symptoms remitted in response to prophylactic treatment with topiramate and propranolol for 8 months. Three months after discontinuation of medications, transient monocular blindness recurred. High-resolution vessel wall magnetic resonance imaging revealed enhancement of the ipsilateral orbital vessels. Isolated orbital vasculitis was diagnosed. Complete remission of transient monocular blindness was achieved after steroid pulse therapy., Discussion: Isolated orbital vasculitis should be considered in differential diagnosis of recurrent transient monocular blindness and ipsilateral headache. High-resolution vessel wall magnetic resonance imaging can be helpful for the diagnosis.

Purpose: To report a case with monocular transient vision loss (TVL) associated with Hyperhomocysteinemia. Methods: We present a case with persistent TVL attacks and high level of homocysteine. Results: A 32-year-old male had a history of episodes of recurrent monocular TVL. Extensive ophthalmic, systemic and laboratory studies were unremarkable with the exception of high plasma homocysteine level. He never experienced TVL during the 36-month follow-up after starting folate, B12 and B6 except for one episode in which he had discontinued the treatment for three months. Conclusion: This case may suggest hyperhomocysteinemia as one of the underlying causes of recurrent attacks of TVL without any known source of emboli.

Background and Importance: Mesencephalic tectal gliomas represent a subset of midbrain tumors, which are more frequent in children than in adults. They usually become symptomatic when causing hydrocephalus by occluding the aqueduct. Because of their slow progression, due to their benign histology, they are characterized by a relatively good prognosis, although hydrocephalus might jeopardize patients' prognosis. Treatment is usually represented by cerebrospinal fluid diversion associated or not with biopsy., Clinical Presentation: We report 2 illustrative cases of tectal gliomas in adults where endoscopic third ventriculostomy (ETV) and simultaneous endoscopic biopsy were obtained during the same operation by means of a single burr hole with a flexible endoscope., Conclusion: We recommend using this overlooked neurosurgical tool for such cases, since it allows the surgeon to safely perform an ETV, then judge whether biopsy can be done or not, without harming the patient, and possibly achieving an important piece of information (histopathological diagnosis) to manage this subset of oncological patients., (Copyright © 2018 by the Congress of Neurological Surgeons.)

In its normal anatomy, the extracranial internal carotid artery (ICA) does not have branches. The most common cause of an extracranial ICA branch is the ectopic placement of one of the named external carotid artery branches. Other causes of extracranial ICA branches include persistent fetal carotid-vertebrobasilar anastomoses and recannalized intersegmental arteries. In this case, report we describe a 55-year-old male who was found to have an ascending pharyngeal artery (APA) arising from the ICA during neck dissection. The aberrant APA was not identified on pre-operative imaging. The patient underwent a successful carotid endarterectomy (CEA) with preservation of flow through the ascending pharyngeal. We review the literature on the origin of the APA and discuss the clinical implications of extracranial ICA branches.

A 66-year-old woman with chronic myeloid leukaemia in nilotinib-induced remission was diagnosed with amaurosis fugax, caused by carotid stenosis. Serum cholesterol was 316 mg/dL (Low-Density Lipoprotein (LDL) cholesterol 213 mg/dL). Nilotinib was discontinued and replaced by interferon. Antiplatelet therapy and atorvastatin 40 mg/day were prescribed. Muscle pain and elevation of serum creatine kinase (CK) occurred; thus, atorvastatin was replaced by ezetimibe. Afterwards, muscle pain subsided and CK reverted to normal, but 2 years later serum cholesterol was still elevated at 218 mg/dL with LDL cholesterol 126 mg/dL. Simvastatin 5 mg/day was then started, but again muscle pain occurred and CK rose to 267 U/L. Simvastatin was stopped and serum cholesterol climbed to 252 mg/dL. Creatine was prescribed and simvastatin was reintroduced. Two months later, cholesterol was 171 mg/dL, CK was 72 U/L and there was no muscle pain. This case supports the view that creatine may prevent statin-induced myopathy., Competing Interests: Competing interests: MB and EA are founding members of NovaNeuro Srl, an academic spin-off that ideates, produces and commercialises dietary supplements based on creatine., (© BMJ Publishing Group Limited 2018. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: To present and review the vascular consequences of arteritis in neurosarcoidosis., Patient and Methods: neurosarcoidosis is typically an inflammatory disorder of the meninges surrounding the brain and spinal cord. Although inflammation of small and medium sized vessels is seen pathologically and vasculitis is occasionally described, a large intracerebral arteritis has not previously been reported. A few case reports exist, however, which describe the vascular consequences of large vessel compromise in the disorder. We review the literature and present a new case with novel MRI features which imply carotid arteritis., Results: The case presented with a disorder of the carotid artery on one side leading to a series of TIAs. Inflammation of the wall of the carotid artery was seen adjacent to a granulomatous leptomeningitis. The disorder responded to immunosuppressive therapy without recurrence., Conclusions: The imaging features suggest a granulomatous infiltration of the carotid artery wall leading to arteritis followed by disorganisation of the internal elastic lamina and fibrosis. The data provide further insight into the pathogenesis of neurological impairments in neurosarcoidosis. The MRI features of carotid arteritis in neurosarcoidosis have not previously been demonstrated., (Copyright © 2018. Published by Elsevier B.V.)

A 56-year-old woman was admitted to our hospital because of amaurosis fugax. The carotid angiogram showed irregularly stenotic lesions of the left and right internal carotid arteries (ICAs), suggestive of dissection. Follow-up evaluation was performed by transoral carotid ultrasonography (TOCU) with contrast enhancement (CE), which yielded better vessel lumen and intramural hematoma visualization than color Doppler imaging. CE-TOCU is useful for evaluating ICA dissections that extends to the high cervical portion., (© 2017 Wiley Periodicals, Inc.)

Transient monocular blindness is an acute episode of ischemic origin in which one eye has profound visual loss, followed by full recovery within one hour. Transient monocular blindness most often occurs in the setting of retinal ischemia secondary to carotid embolism, but other mechanisms have been reported, including thrombosis (most often in the setting of giant cell arteritis), hemodynamic disorders (secondary to severe carotid stenosis) or vasospasm. Transient monocular blindness is considered a transient ischemic attack originating in the carotid arteries and must benefit from the same management as transient ischemic attack involving the brain, in order to prevent a subsequent stroke., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Transient monocular blindness is an acute episode of ischemic origin in which one eye has profound visual loss, followed by full recovery within one hour. Transient monocular blindness most often occurs in the setting of retinal ischemia secondary to carotid embolism, but other mechanisms have been reported, including thrombosis (most often in the setting of giant cell arteritis), hemodynamic disorders (secondary to severe carotid stenosis), or vasospasm. Transient monocular blindness is considered a transient ischemic attack originating in the carotid arteries, and must be managed the same as transient ischemic attack involving the brain, in order to prevent a subsequent stroke., (Copyright © 2017. Published by Elsevier Masson SAS.)

We report a modified operative technique termed partial eversion carotid endarterectomy (PECE). During a 9-year period (2006-2015), 352 patients underwent PECE. Indications for surgery, intraoperative details, and outcomes were recorded. The initial 185 patients had carotid duplex ultrasound imaging at 6 weeks and then at 6, 12, and 24 months. Subsequent patients had carotid imaging at 4 to 6 weeks. Indications included stroke (76), transient ischemic attack (153), and amaurosis fugax (33); 58 patients were asymptomatic, and 32 patients had surgery before cardiac surgery. Median clamp time was 14 minutes (interquartile range, 11.5-17 minutes). Median total operation time was 41 minutes (interquartile range, 31-72 minutes). Outcomes included four transient ischemic attacks (1.2%), five strokes (1.4%), and two deaths at 30 days (0.5%). No significant cranial nerve injuries or carotid restenosis was detected during follow-up. PECE is technically straightforward, with outcomes comparable to those of current operative techniques. Its advantages included reduced operative and carotid clamping time., (Copyright © 2016 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Current guidelines recommend that carotid endarterectomy (CEA) be performed as early as possible after the neurologic index event in patients with 50% to 99% carotid artery stenosis. However, recent registry data showed that patients treated ≤48 hours had a significantly increased perioperative risk. Therefore, the aim of this single-center study was to determine the effect of the time interval between the neurologic index event and CEA on the periprocedural complication rate at our institution., Methods: Prospectively collected data for 401 CEAs performed between 2004 and 2014 for symptomatic carotid stenosis were analyzed. Patients were divided into four groups according to the interval between the last neurologic event and surgery: group I, 0 to 2 days; group II, 3 to 7 days; group III, 8 to 14 days; and group IV, 15 to 180 days. The primary end point was the combined rate of in-hospital stroke or mortality. Data were analyzed by way of χ 2 tests and multivariable regression analysis., Results: The patients (68% men) had a median age of 70 years (interquartile range, 63-76 years). The index events included transient ischemic attack in 43.4%, amaurosis fugax in 25.4%, and an ipsilateral stroke in 31.2%. CEA was performed using the eversion technique in 61.1% of patients, and 50.1% were treated under locoregional anesthesia. The perioperative combined stroke and mortality rate was 2.5% (10 of 401), representing a perioperative mortality rate of 1.0% and stroke rate of 1.5%. Overall, myocardial infarction, cranial nerve injuries, and postoperative bleeding occurred in 0.7%, 2.2%, and 1.7%, respectively. We detected no significant differences for the combined stroke and mortality rate by time interval: 3% in group I, 3% in group II, 2% in group III, and 2% in group IV. Multivariable regression analysis showed no significant effect of the time interval on the primary end point., Conclusions: The combined mortality and stroke rate was 2.5% and did not differ significantly between the four different time interval groups. CEA was safe in our cohort, even when performed as soon as possible after the index event., (Copyright © 2016 Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.)

Background and Purpose: Symptomatic carotid stenosis (sCS), a common cause of transient ischemic attack (TIA), is correlated with higher stroke risk. We investigated the frequency and associated factors of sCS in patients with TIA and the association between sCS and stroke risk following TIA., Methods: Over a three-year period (2011-2013), 861 consecutive patients with TIA, who were admitted to the Department of Neurology at the University of Lübeck, Germany, were included in a monocenter study and prospectively evaluated. Diagnosis of TIA was in accordance with the tissue-based definition (transient neurological symptoms without evidence of infarction by brain imaging)., Results: Of 827 patients (mean age, 70 ± 13.2 years; 49.7% women), 64 patients (7.7%; 95% confidence interval [CI], 5.9%-9.7%) exhibited sCS and 3 patients (0.3%) showed an occlusion of the corresponding internal carotid artery. Logistic regression revealed that sCS was associated with male sex (odds ratio [OR], 2.7; 95% CI, 1.2-3.6; p = 0.012), amaurosis fugax (OR, 8.1; 95% CI, 3.4-19-4; p < 0.001), unilateral weakness (OR, 3.4; 95% CI, 1.9-6.1; p < 0.001), symptom duration less than 1 h (OR, 2.0; 95% CI, 1.1-3.4; p = 0.019) and previous stroke (OR, 2.7; 95% CI, 1.5-4.7; p = 0.001). During hospitalization (mean, 6.6 days), five patients (0.6%; 95% CI, 0.1%-1.2%) suffered from stroke. The stroke risk was higher in patients with sCS than in those without sCS (6.3% vs. 0.1%; p < 0.001), whereas the recurrent TIA risk (2.6%) did not differ between the groups (4.7% vs. 2.5%; p = 0.29)., Conclusion: SCS appears to be associated with a higher risk of stroke in patients with TIA defined according to the tissue-based definition.