Full sample analysis of cerebral venous sinus thrombosis in ophthalmology inpatients.

PURPOSE: We hope that by analyzing the clinical features of cerebral venous sinus thrombosis (CVST), we can help ophthalmologists reduce misdiagnosis or delayed diagnosis. DESIGN: We evaluated 47 patients with CVST in terms of clinical manifestations. METHODS: All cases were analyzed in terms of risk factors, clinical symptoms, ophthalmic examination, imaging examination and lumbar puncture. RESULTS: The body mass indices (BMIs) of 41 patients (87.2%; 95% CI, 77.7-96.8%) were≥24, which is overweight by Chinese standards. There were 22 patients (46.8%; 95% CI, 32.5-61.1%) with BMIs≥28, who were considered obese. Thirteen were hypertensive (27.7%; 95% CI, 14.9-40.5%). The initial symptoms included blurred vision (23, 48.9%; 95% CI, 34.6-63.2%), amaurosis fugax (13, 27.7%; 95% CI, 14.9-40.5%), headache (11 patients, 23.4%; 95% CI, 11.3-35.5%), dizziness (3, 6.4%; 95% CI, -0.6-13.4%), and bilateral diplopia (3, 6.4%; 95% CI, -0.6-13.4%). There were 9 patients (9, 19.2%; 95% CI, 7.9-30.4%) with blindness, 23 patients (48.9%; 95% CI, 34.6-63.2%) with pupillary abnormalities, and 40 patients (85.1%; 95% CI, 74.9-95.2%) with papilledema. Forty-three of the 45 patients who successfully underwent a routine lumbar puncture showed high intracranial pressure (91.7%; 95.6% CI, 89.6-101.6%). Finally, two cases are reported in greater detail for illustrative purposes. CONCLUSION: The main reasons interfering with the diagnosis of CVST might be its nonspecific ocular symptoms and the physicians' clinical thought process being limited to the scope of common ophthalmological diseases.

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.

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.

Ocular involvement in primary antiphospholipid syndrome: results of an extensive ophthalmological evaluation performed in the APS-Rio cohort.

OBJECTIVE: To study ophthalmological manifestations in a well-characterized primary antiphospholipid syndrome (PAPS) cohort (APS-Rio) and compare them with a healthy control group. METHODS: We examined PAPS patients and controls with an extensive ophthalmological evaluation, which included anamnesis, visual acuity, slit-lamp biomicroscopy, binocular indirect ophthalmoscopy, and retinography of the anterior and posterior segments of the eye. PAPS group also underwent angiography exam and optical coherence tomography using spectral domain technology (SD-OCT). RESULTS: 98 PAPS patients and 102 controls were included. The most common symptom in PAPS was amaurosis fugax (34.7% vs. 6.9%; p = .001). In the multivariate analyses, Raynaud's phenomenon was associated with amaurosis fugax (OR 3.71, CI:1.33-10.32; p = .012), and livedo correlated with hemianopia (OR 6.96, CI:1.11-43.72, p = .038) and diplopia (OR 3.49, CI:1.02-11.53, p = .047). After ophthalmological evaluation, 84 PAPS patients had ocular involvement (1.0% glaucoma, 94.0% posterior findings, 62.7% anterior findings, and 56.6% both posterior and anterior findings). Vascular tortuosity was more frequent in the PAPS group (63.2% vs. 42.2%; p = .002), as well as peripheral tortuosity (29.6% vs. 7.8%; p < .001). After excluding patients with atherosclerotic risk factors, peripheral vascular tortuosity was still statistically associated with PAPS (35.0 vs. 7.8%, p < .001). Triple positivity was more frequent in PAPS patients with peripheral vascular tortuosity than in those without this ocular finding (34.5% vs. 15.9%, p = .041). CONCLUSION: Vasomotor phenomena are importantly related to ocular symptoms in PAPS. Vascular tortuosity was a frequent finding in PAPS patients. Peripheral vascular tortuosity was associated with triple positivity and might be a biomarker of ischemic microvascular retinopathy due to PAPS.

Carotid dissection and central serous chorioretinopathy related to sarcoidosis-antiphospholipid syndrome: a case report.

Sarcoidosis is a chronic multisystemic disease, which can be rarely associated with autoimmune disorders, such as antiphospholipid syndrome (APS). Although amaurosis fugax is an uncommon complication, its presentation can unmask a carotid artery dissection (CAD) in these diseases. In addition, central serous chorioretinopathy (CSC) has been related to vascular disorders too. We presented a case of a Caucasian middle-aged man, who developed CAD symptoms, such as amaurosis fugax in the right eye (RE) and headache. His medical history included arterial hypertension, hypothyroidism, and Lofgren's syndrome. On examination, retinal pigment epithelium (RPE) atrophy and subretinal fluid (SRF) in the macular area of the RE were observed. These findings were confirmed by optical coherence tomography (OCT), which also revealed an increase in choroidal thickness. However, these differed significantly from the contralateral eye. These clinical symptoms and imaging findings suggested a CSC in the RE, but not all clinical processes were justified. Subsequently, a CT angiography was performed and confirmed a significant occlusion in the right internal carotid artery and progressive sharpening of the lumen with an intimal flap due to a carotid dissection. In addition, the laboratory results were compatible with antiphospholipid syndrome (APS). To the authors' knowledge, the patient returned to the ED due to an anterior uveitis and he is currently asymptomatic with Cemidon and Adalimumab treatment. We described for the first time a case of carotid dissection and central serous chorioretinopathy in the context of two autoimmune-based pathologies, such as sarcoidosis and antiphospholipid syndrome. Abbreviations: APS = Antiphospholipid syndrome, BCVA = Best-corrected visual acuity, CAD = Carotid artery dissection, CNV = Choroidal neovascular membrane, CSC = Central serous chorioretinopathy, CT = Computed tomography, ED = Emergency Department, ICAD = Internal carotid artery dissection, LE = Left eye, OCT = Optical coherence tomography, RAPD = Relative afferent pupillary defect, RPE = Retinal pigment epithelium, RE = Right eye, SRF = Subretinal fluid.

[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.

Stroke Risk before and after Central Retinal Artery Occlusion: A Population-based Analysis.

PURPOSE: To determine the risk of stroke, transient ischemic attack (TIA), and transient monocular vision loss (TMVL) before and after a central retinal artery occlusion (CRAO). DESIGN: Population-based, retrospective case series. PARTICIPANTS: Patients diagnosed with a CRAO in Olmsted County, Minnesota, from 1976 to 2016. METHODS: Patients living in Olmsted County with a diagnosis code of CRAO from 1976 to 2016 were reviewed. New CRAOs were confirmed, and stroke, TIA, and TMVL events in the 15 days before and after CRAO were recorded. MAIN OUTCOME MEASURES: Incidence of stroke, TIA, and TMVL events in the 15 days before and after CRAO. RESULTS: Eighty-nine patients with a CRAO were identified, providing an annual incidence of 2.58/100 000 (95% confidence interval [CI], 2.04-3.11). Median age at the time of CRAO was 76 years (range, 46-100 years); 56.2% were male, and 89.9% of the cohort was White. In the 15 days before and after CRAO, there were 2 ischemic strokes (2.2%), 1 hemorrhagic stroke (1.1%), 2 TIAs (2.2%), and 9 TMVL events (10.1%). Starting in 1999, 15 of 45 patients underwent magnetic resonance imaging within 2 months of CRAO. One patient (6.7%) had evidence of asymptomatic diffusion restriction, and 9 patients (60%) had a remote infarct. CONCLUSIONS: This population-based study demonstrated that the risk of symptomatic ischemic stroke is 2.2% in the 15 days before and after a CRAO, which is slightly lower than most studies from tertiary centers. These data should be considered as practice recommendations are developed regarding the urgency of neurovascular workup in patients with acute CRAO.

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.

Transient smartphone blindness. / Transitorisk smarttelefonblindhet.

BACKGROUND: The aetiology of transient monocular vision loss is not necessarily thromboembolic, and careful history-taking is crucial in making the correct diagnosis. CASE PRESENTATION: A previously healthy woman in her thirties was referred to our outpatient stroke clinic on suspicion of recurring amaurosis fugax in her right eye. The ophthalmic and neurologic examinations were normal. A review of the medical history revealed that her symptoms occurred in connection with using her smartphone in bed, typically while lying on her left side with the left eye covered by the pillow. When standing up from the bed again with the lights turned off in the bedroom, she noticed vision loss in her right eye. INTERPRETATION: We concluded that the patient had experienced transient smartphone blindness. This phenomenon can occur when monocularly using a smartphone under scotopic lighting conditions; only the occluded eye adapts to darkness, and the other eye is perceived to be blind if looking away from the bright smartphone screen.