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.

Comparison of quantity and quality of muscle as clinical prognostic markers in patients undergoing carotid endarterectomy.

OBJECTIVE: The measurement of muscle area is routinely utilised in determining sarcopaenia in clinical research. However, this simple measure fails to factor in age-related morphometric changes in muscle quality such as myosteatosis. The aims of this study were to: firstly investigate the relationship between the masseter area (quantity) and density (quality), and secondly compare the prognostic clinical relevance of each parameter. METHODS: Cross-sectional CT head scans were reviewed for patients undergoing carotid endarterectomy. The masseter was manually delineated and the total masseter area (TMA) and the total masseter density (TMD) calculated. Measurements of the TMA were standardised against the cranial circumference. Observer variability in measurements were assessed using Bland-Altman plots. The relationship between TMA and TMD were evaluated using Pearson's correlation and linear regression analyses. The prognostic value of TMA and TMD were assessed using receiver operator curves and cox-regression analyses. RESULTS: In total, 149 patients who had undergone routine CT scans prior to a carotid endarterectomy were included in this study. No significant observer variations were observed in measuring the TMA, TMD and cranium circumference. There was a significant positive correlation between standardised TMA and TMD (Pearson's correlation 0.426, p < 0.001, adjusted R-squared 17.6%). The area under the curve for standardised TMA in predicting all-cause mortality at 30 days, 1 year and 4 years were higher when compared to TMD. Standardised TMA was only predictive of post-operative overall all-cause mortality (adjusted hazard ratio 0.38, 95% confidence interval 0.15-0.97, p = 0.043). CONCLUSION: We demonstrate a strong relationship between muscle size and density. However, the utilisation of muscle area is likely to be limited in routine clinical care. ADVANCES IN KNOWLEDGE: Our study supports the utilisation of muscle area in clinical sarcopaenia research. We did not observe any additional prognostic advantage in quantifying muscle density.

Alternating Amaurosis Fugax in Trousseau Syndrome: A Case Report.

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.

Carotid arteritis causing amaurosis fugax and ischaemic cerebrovascular events in neurosarcoidosis.

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.

Successful retinal blood flow augmentation after extracranial-intracranial bypass.

A 61-year-old woman visited us with recent onset right-side weakness. Magnetic resonance imaging showed ischemic changes at the left internal border zone due to occlusive disease affecting the left proximal internal carotid artery. Prompt oral dual antiplatelet therapy and intravenous fluid were administered with subsequent induced hypertension and without reperfusion therapy. Although the hemiparesis was improved, she complained of a new-onset transient left-side monocular visual loss. Fluorescein angiography confirmed delayed perfusion in the left eye. We performed extracranial-intracranial bypass for flow augmentation. After bypass, the amaurosis fugax resolved. Follow-up retinal fluorescein angiography also showed improved retinal perfusion.

An update on the incidence of perioperative outcomes after carotid endarterectomy, stratified by type of preprocedural neurologic symptom.

OBJECTIVE: Perioperative complications after carotid endarterectomy (CEA) have decreased over time. Therefore, we aimed to provide an update on 30-day outcomes after CEA, stratified by type of preprocedural neurologic symptom. METHODS: We included all CEAs from the Targeted Vascular module of the American College of Surgeons National Surgical Quality Improvement Program (ACS-NSQIP, 2011-2015) and stratified patients based on type of preprocedural neurologic symptom, that is, asymptomatic, ocular transient ischemic attack (TIA), hemispheric TIA, and stroke. We compared 30-day outcomes across the groups, with 30-day stroke/death as our primary endpoint. RESULTS: Of 16,739 CEA patients, 9784 were asymptomatic (58%). Among the 6955 symptomatic patients, 1216 (17%) had a preprocedural ocular TIA, 2635 (38%) a preprocedural hemispheric TIA, and 3104 (45%) a preprocedural stroke. Preprocedural stroke patients had higher 30-day stroke/death rates compared with those with a preprocedural hemispheric TIA, or ocular TIA, or asymptomatic patients (5.0% vs 3.3%, 1.9%, and 1.8%, respectively; all P < .001), primarily owing to differences in perioperative 30-day stroke rates, with 4.1% vs 2.5%, 1.4%, and 1.3%, respectively (all P < .001). CONCLUSIONS: Among symptomatic CEA patients, those with a preprocedural stroke had a high perioperative 30-day stroke/death rate, compared with those patients with either a preprocedural hemispheric or ocular TIA. Therefore, the common stratification applied to CEA patients, which groups all symptomatic patients, should be avoided, especially as the relative proportion of symptomatic patients with a preprocedural stroke vs those with a hemispheric or ocular TIA will affect the overall outcome for all symptomatic patients after CEA.

[Correlation analysis on retrobulbar vascular hemodynamic characteristics and carotid artery color Doppler ultrasonographic features of amaurosis fugax].

OBJECTIVE: To investigate the correlation of amaurosis fugax and carotid stenosis as well as posterior ocular blood vessel hemodynamic changes. METHODS: Case series studies. 32 patients (19 men and 13 women whose age were 50 to 80 years old and average age was 64.31 ± 8.15) who were diagnosed as amaurosis fugax with 32 eyes were enrolled from 2011.5-2012.12. During the same period 30 cases without posterior ocular ischemic diseases were collected as the control group. Their ophthalmic artery, central retinal artery and carotid artery were examinated by color Doppler ultrasound, to detect vascular peak systolic velocity, end diastolic velocity, resistance index, the degree of carotid stenosis, carotid artery plaque area and type. All data were analyzed by chi-square test and t-test. RESULTS: PSV (25.95 ± 2.45) cm/s, EDV (6.01 ± 0.87) cm/s of the ophthalmic artery in AmF group were lower compared with the control group [PSV:(27.53 ± 1.41) cm/s, EDV: (6.89 ± 0.56) cm/s]. The differences were statistically significant (t = 3.087, 0.712, P = 0.003, 0.048). There was no statistical difference of RI between AmF and the control group (t = 0.188, P = 0.852) . PSV (7.13 ± 0.96) cm/s, EDV (1.78 ± 0.26) cm/s of the central retinal artery in AmF group were lower compared with the control group[PSV:(8.23 ± 0.92) cm/s, EDV:(2.13 ± 0.29) cm/s]. The differences were statistically significant (t = 4.648, 4.976, P = 0.000, 0.000). There was no statistical difference of RI between AmF and the control group (t = 0.180, P = 0.855). Detection rate of carotid artery moderately and severe stenosis (59.4%) was significantly higher compared with the control group (6.7%) . The difference was statistically significant (χ(2) = 19.205, P = 0.000). CONCLUSIONS: The blood flow velocity of ophthalmic artery and central retinal artery in amaurosis fugax patients reduced significantly, while the resistance was normal. About 60% of the patients had carotid artery moderately and severe stenosis. There was significant correlation between amaurosis fugax and carotid artery stenosis.

A traveling "spot sign" in recurrent amaurosis fugax and central retinal artery occlusion.

Sudden monocular blindness is frequently caused by central retinal artery occlusion (CRAO) from embolic sources. Treatment options are insufficient, and spontaneous prognosis toward visual recovery is poor. In addition to ophthalmologic evaluation, transorbital sonographic assessment of the central retinal artery may help establish early diagnosis by Doppler sonographic proof of occlusion and, in some cases, by B-mode detection of an intra-arterial "spot sign". We report the case of a patient with recurrent amaurosis fugax and subsequent CRAO. Ultrasound examination after 2 incidences of amaurosis fugax demonstrated a patent but stenotic central retinal artery, with stenosis caused by an embolus visualized as a "spot sign". The following day, persisting amaurosis suddenly developed. Sonographic re-evaluation revealed downstream dislodgment of the "spot sign" and complete arterial occlusion. Thrombolytic treatment did not result in clinical improvement. In conclusion, this case report describes a single case of repeated amaurosis fugax and deterioration to CRAO via embolization into the central retinal artery and consecutive downstream dislodgment. It emphasizes that ultrasound may render valuable diagnostic information in patients with acute central retinal artery embolization toward its embolic etiology and its risk of subsequent deterioration.

Recurrent transient monocular blindness from stenotic central retinal artery.

Transient monocular blindness (TMB) is caused by a temporary reduction of blood flow to the retina or optic nerve. Even though embolism from the ipsilateral carotid artery has been considered the main mechanism of TMB, the vascular pathology remains unknown in many patients. A 42-year-old man presented with recurrent transient visual loss in the left eye for 2 months. The attacks tended to develop more frequently when his head was bent down. Fluorescence angiography during an attack revealed hypoperfusion of the left central retinal artery (CRA) and cerebral angiography documented a focal isolated stenosis of the CRA at the origin. Aspirin and nimodipine reduced the attacks markedly. Stenosis of the CRA may be a cause of TMB. Intermittent vasospasm in addition to static hypoperfusion may have caused TMB in our patient with isolated CRA stenosis.

Coagulation activation and ultrasound characteristics in patients with carotid artery disease.

INTRODUCTION: Elevated levels of markers for thrombin activation are associated with plaque echogenicity and degree of stenosis in patients with carotid artery stenosis. The Activated Protein C-Protein C Inhibitor (APC-PCI) complex reflects activation of the Protein C system and is a measure of thrombin generation. The aim of the present study was to examine APC-PCI complex in patients undergoing thrombendartherectomy for carotid artery stenosis, and to relate the findings to clinical characteristics and plaque morphology as determined by ultrasound. MATERIALS AND METHODS: Blood was obtained from 125 patients (39 female, median age 71 years) with carotid artery stenosis admitted from September 2005 to May 2007. The APC-PCI complex was measured using a sandwich immunofluorometric method and compared to an age- and sex-matched healthy control-group. Clinical and demographic characteristics, routine laboratory markers and ultrasound characteristics were analysed using univariate and multivariate analysis. RESULTS: APC-PCI complex concentration was significantly increased in patients with carotid artery stenosis (median 0.21 microg/L; 10th to 90th percentile 0.15-0.36) compared to a healthy control-group (0.19 microg/L; 0.11-0.31; P=.009). There was no significant difference in APC-PCI-values between asymptomatic (n=48) and symptomatic (n=77) patients with carotid artery stenosis (0.22 vs. 0.20 microg/L; p=0.626). Patients with minor stroke (n=31) had a higher median APC-PCI-concentration (0.27 microg/L; 0.15-0.63) than patients with amaurosis fugax (0.19 microg/L; 0.15-0.36) or transient ischemic attack (0.21 microg/L; 0.12-0.36) (p=0.016). No association was found between APC-PCI-values and the degrees of carotid artery stenosis or the time from the latest neurological symptoms to blood sampling. Patients with echolucent plaques had significantly lower APC-PCI concentrations (0.20 microg/L; 0.14-0.35 vs. 0.24 microg/L; 0.15-0.60; p=0.043), according to the Gray-Weale classification. CONCLUSIONS: Patients with carotid artery disease exhibit increased concentrations of APC-PCI compared to a healthy control-group, particularly those patients with echogenic plaques, who have significantly higher APC-PCI levels than patients with echolucent plaques.

Jugular venous reflux affects ocular venous system in transient monocular blindness.

BACKGROUND: The frequency of jugular venous reflux (JVR) is higher in patients with transient monocular blindness (TMB). We hypothesize that JVR influences ocular venous outflow, and resulting disturbances in cerebral and ocular venous circulation might be a cause of TMB. To substantiate this hypothesis, we aimed to demonstrate that: (1) TMB patients have vasculature changes in their retinal venules, and (2) JVR could influence ocular venous outflow, as revealed by dilated retinal venules. METHODS: This study has 2 parts. The case-control study included 31 TMB patients and 31 age/gender-matched normal individuals, who all received fundus photography for retinal venule diameter comparisons. The Valsalva maneuver (VM) experiment included 30 healthy volunteers who received both color Doppler imaging of the internal jugular vein and fundus photography for retinal venule diameter measurement. RESULTS: In the case-control study, TMB patients had a wider retinal venule diameter (184.5 +/- 17.5 vs. 174.3 +/- 16.2 microm, right eye, p = 0.023; 194.20 +/- 24.6 vs. 176.6 +/- 19.5 microm, left eye, p = 0.017), especially TMB patients with JVR. The VM experiments showed that the presence of JVR was associated with a greater increase in retinal venule diameters during VM in the subjects' right eye (14.27 +/- 11.16 vs. 2.75 +/- 3.51%, JVR vs. non-JVR, p = 0.0002) and left eye (10.06 +/- 6.42 vs. 1.80 +/- 2.03%, p = 0.0003). CONCLUSIONS: These findings provide evidence that frequently occurring JVR associated with TMB impedes ocular venous outflow, and the subsequent disturbances in ocular venous circulation may be a cause of TMB.

Do temporal artery duplex ultrasound findings correlate with ophthalmic complications in giant cell arteritis?

OBJECTIVE: Ophthalmic complications are common in acute GCA. Do temporal artery ultrasound and clinical parameters correlate with the occurrence and severity of ophthalmic complications? METHODS: The results of temporal artery ultrasound examinations are compared with the occurrence of anterior ischaemic optic neuropathy (AION), central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO), diplopia and amaurosis fugax in 222 consecutive patients with newly diagnosed, active GCA. RESULTS: Temporal artery ultrasound displayed vasculitic wall swelling (halo), stenoses and/or acute occlusions in 84% (58% in 67 large-vessel GCA patients and 95% in 155 patients without proximal arm vasculitis). Ophthalmic complications occurred in 64 (29%), AION in 30 (14%), CRAO in 7 (3%), BRAO in 2 (1%), amaurosis fugax in 16 (7%) and diplopia in 9 patients (4%). Ophthalmic complications were insignificantly more common if temporal artery ultrasound was positive (31 vs 17%; P = 0.11) as a greater number of patients without arm vasculitis showed eye involvement (34 vs 18%; P = 0.02). The number of pathological temporal artery segments, presence of stenoses or bilateral findings did not correlate with ophthalmic complications. Age >or= 72 yrs at diagnosis correlated with a higher incidence of ophthalmic complications. CONCLUSION: Ophthalmic complications occurred less frequently if proximal arm vasculitis was present. Findings of temporal artery ultrasound did not correlate with eye complications.

Reflux of jugular and retrobulbar venous flow in transient monocular blindness.

OBJECTIVE: Transient monocular blindness (TMB) attacks may occur during straining activities that impede cerebral venous return. Disturbance of cerebral and orbital venous circulation may be involved in TMB. METHODS: Duplex ultrasonography and Doppler-flow measurement of jugular and retrobulbar veins were performed in 134 consecutive patients with TMB and 134 age- and sex-matched control subjects. All recruited patients received thorough examinations to screen for possible underlying causes. RESULTS: Of the 134 patients with TMB, 48 patients had ipsilateral carotid arterial lesion and 7 patients had TMB attack(s) caused by cardiac embolism. Of the remaining 79 patients with undetermined cause, 46 had 3 or more TMB attacks (undetermined-frequent group) and 33 had fewer than 3 attacks. In comparison with the control subjects, the TMB patients had greater frequencies of jugular venous reflux (57 vs 30%; p < 0.0001; odds ratio [OR]: 3.079, 95% confidence intervals [CI]: 1.861-5.096) and flow reversal in the superior ophthalmic vein (RSOV; 37 vs 9%; p < 0.0001; OR: 6.052, CI: 3.040-12.048). The undetermined-frequent group had the greatest frequencies of jugular venous reflux (74%, 34 patients; OR: 6.66, CI: 3.13-14.17) and RSOV (59%, 27 patients; OR: 6.51, CI: 3.12-13.58). Of the 50 patients with RSOV, 47 (94%) had RSOV on the side of the TMB attacks. INTERPRETATION: The increased incidences of jugular and orbital venous reflux in TMB patients suggest that disturbance of cerebral and orbital venous circulation is involved in the pathogenesis of TMB, especially among patients with frequent attacks of undetermined cause.

Solid cerebral microemboli and cerebrovascular symptoms in patients with prosthetic heart valves.

BACKGROUND AND PURPOSE: Although cerebral microemboli are often detected by transcranial Doppler ultrasonography in mechanical heart valve patients, the clinical significance of such microemboli is unclear. The aim of this study was to determine the frequency and composition of cerebral microemboli in a prosthetic heart valve population and to correlate these findings to cerebrovascular symptoms, blood inflammation, and coagulation parameters. METHODS: Seventy-six consecutive patients with a total of 81 prosthetic (54 mechanical, 27 biologic) heart valves were monitored for cerebral microemboli by multifrequency transcranial Doppler ultrasonography 1 year after valve replacement. Cerebrovascular events in the first year were recorded by a neurologist. Inflammation and coagulation markers were measured by immunoassays. RESULTS: Microemboli were detected in mechanical heart valve patients only (28 patients, 56%). Twelve percent were solid, occurring in 17 (34%) of the mechanical heart valve population. The presence of solid cerebral microemboli was the only variable that was associated with cerebrovascular symptoms after a final regression analysis (P=0.026). The plasma monocyte chemotactic protein-1 level was raised in patients with solid microemboli (P=0.014). CONCLUSIONS: Solid cerebral microemboli were detected by multifrequency transcranial Doppler ultrasonography in 35% of a mechanical heart valve population, and the frequency was higher in patients who experienced cerebrovascular events during the first year after valve replacement. The results suggest that the detection of solid cerebral microemboli may be helpful in predicting the risk of ischemic stroke in mechanical heart valve patients.