Transient Monocular Blindness in a Man in His 30s.

This case report discusses a diagnosis of giant ascending aortic aneurysm in a patient who presented with transient monocular blindness and no cardiovascular symptoms.

The crucial role of gadolinium-enhanced MRI in a case of amaurosis fugax - a case report and literature review.

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.

Systemic lupus erythematosus mimicking retinal migraine: a case report.

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.

Case of Amaurosis Fugax in the Setting of a Persistent Primitive Hypoglossal Artery Requiring Carotid Endarterectomy with Regional Anesthesia.

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.

[Unique visual symptom of amaurosis fugax: a case report].

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.

Outcomes and role of shunting during carotid endarterectomy for symptomatic patients.

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 χ2 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.

Central retinal artery occlusion and subsequent amaurosis fugax in the contralateral eye associated with the G20210A prothrombin gene (F2) variant: a case report.

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.

Current Guidelines on Management of Amaurosis Fugax and Transient Ischemic Attacks.

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.

Case Report: Recurrent Transient Monocular Vision Loss Secondary to Protein C Deficiency.

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.

Low Risk of Neurological Recurrence while Awaiting Carotid Endarterectomy: Results From a Danish Multicentre Study.

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.

Predictive Factors for Pre-operative Recurrence of Cerebrovascular Symptoms in Symptomatic Carotid Stenosis.

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.

CT Angiography of Agenesis of the Left Internal Carotid Artery.

Online supplemental material is available for this article.

Editor's Choice - Prognostic Role of Pre-Operative Symptom Status in Carotid Endarterectomy: A Systematic Review and Meta-Analysis.

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.