PRES and RCVS: Two Distinct Entities or a Spectrum of the Same Disease?

OBJECTIVES: To report a case of a patient with overlapping posterior reversible encephalopathy syndrome (PRES) and reversible cerebral vasoconstriction syndrome (RCVS), and review the existing literature emphasizing the pathophysiological overlap of these two entities. MATERIALS AND METHODS: We conducted a literature search in electronic database PubMed identifying studies reporting the overlap of PRES and RCVS. RESULTS: PRES and RCVS are two increasingly recognized entities that share similar clinical and imaging features. PRES is characterized by vasogenic edema predominantly in the parieto-occipital regions, associated with acute onset of neurological symptoms including encephalopathy, seizures, headaches, and visual disturbances. RCVS is characterized by reversible segmental and multifocal vasoconstriction of the cerebral arteries and classically presents with thunderclap headache, with or without associated focal neurological deficits and seizures. PRES is frequently associated with uncontrolled hypertension but can also be seen in the setting of renal failure, exposure to cytotoxic agents, or pre-eclampsia. RCVS is often triggered by exposure to vasoactive agents, postpartum state, or immunosuppression. We report a case of a patient presenting with vision changes and hemiparesis, and found to have extensive cytotoxic and vasogenic edema involving the cortex and subcortical white matter on brain imaging. These changes were primarily noted in the parieto-occipital and brainstem regions, along with features of reversible vasculopathy on vascular imaging suggestive of coexisting PRES and RCVS. CONCLUSIONS: PRES and RCVS share precipitating factors, clinical and radiological features, and frequently co-exist, suggesting a common pathophysiological mechanism related to reversible dysregulation of cerebral vasculature, endothelial dysfunction, and breakdown of the blood-brain barrier.

Outcomes of treatment modalities for ruptured intracranial aneurysms based on age - A meta-analysis.

BACKGROUND: Data comparing the effect of age on outcomes of patients who underwent either endovascular coiling (EVC) or neurosurgical clipping (NSC) for ruptured intracranial aneurysms remains limited. OBJECTIVE: To better elucidate the preferred intervention for ruptured aneurysm management by presenting the results of our systematic review of the literature that evaluated the potential advantages of the two interventions between different age groups. METHODS: Systematic review of PubMed and Embase was performed (2002 - June 10, 2019) following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2013 guidelines. Median ages of EVC and NSC cohorts were 54 and 56, respectively. Ages below the median were used in our "younger" cohort; ages above the median were used in our "older" cohort. RESULTS: We reviewed 13 studies on 7,137 patients. In the younger cohort, there were 2840 (EVC: 1412, NSC: 1428) patients. In the older cohort, there were 4297 (EVC: 2552, NSC: 1745) patients. Overall, there was a significant difference in functionality between EVC (77.70%) and NSC (69.23%) (OR=1.69; 95% C.I.: 1.10-2.60, p = 0.0212). In our younger cohort, functionality was significantly different between EVC (77%) and NSC (69%) (OR=1.54; 95% C.I.: 1.29-1.84, p < 0.001). For the older cohort, there was no significant difference in functionality, complications, or efficacy. CONCLUSIONS: We have highlighted the importance of considering age prior to deciding which intervention is most appropriate for ruptured aneurysms, with higher morbidity and mortality with NSC versus EVC in the younger population.

Symptomatic carotid webs and patent foramen ovale: RoPE score in competing stroke etiologies.

BACKGROUND: Patent foramen ovale (PFO)-associated stroke is diagnosed more frequently in young patients with infrequent vascular risk factors and embolic appearing infarcts. The risk of paradoxical embolism (RoPE) score is used to identify PFO-associated stroke. Patients with symptomatic carotid artery web (CaW) share a very similar risk profile and these lesions are frequently overlooked. In this study, we evaluate the RoPE score profile in patients with suspected symptomatic CaW. METHODS: Retrospective analysis of prospectively collected data of patients with symptomatic CaW as the presumed cause of stroke presenting to 2 comprehensive stroke centers from 2014 to 2021. CaW was diagnosed using computed tomography angiography (CTA) of the neck & head. Shunt study was done using a transthoracic, transesophageal, and/or transcranial-Doppler with bubbles. RoPE score ≥7 was considered high. RESULTS: Seventy-five patients had stroke from a symptomatic ipsilateral CaW. Mean age was 49.7±11.2 years and 74.7 % were females. Median RoPE score was 7 [5-8], and 52.0 % had a high RoPE score. PFO was detected in 13.3 % of the patients and 20.5 % within the high RoPE score group. Ten percent of the cases would have been misclassified as PFO-associated strokes based on RoPE score. CONCLUSION: High RoPE scores were observed in the majority of patients with CaW-attributed stroke, and it should not be used to differentiate CaW- versus PFO-associated stroke. Careful extracranial internal carotid artery evaluation for CaW is warranted in cryptogenic strokes, including in PFO positive patients before defining stroke etiology.

Carotid web: an under-recognized and misdiagnosed ischemic stroke etiology.

BACKGROUND: Carotid web (CaW) constitutes a possible cause of ischemic stroke, particularly large vessel occlusion syndromes. We aim to evaluate misdiagnosis rates and diagnosis trends for CaW. METHODS: Based on CT angiography (CTA), we prospectively identified a cohort of patients with symptomatic CaW treated at two comprehensive stroke centers (CSC) from 2014 to 2020 to assess misdiagnosis. Official CTA reports from the CSCs and referring hospitals were then reviewed for mention of CaW. For diagnosis trends, we retrospectively analyzed a CSC electronic medical record, identifying patients with CaW mentioned in an official CTA report from 2011 to 2020. RESULTS: For misdiagnosis, 56 patients with symptomatic CaW were identified in the CSCs; 16 (28%) had bilateral CaW, totaling 72 CaWs. Only one CaW (5.5%) was reported at referring facilities, from 14 patients/18 CaWs imaged with CTA. Conversely, 43 (69%) CaWs were reported from 49 patients/62 CaWs at the CSC (p<0.01). For diagnosis trends, from 2011 to 2020, 242 patients at a CSC accounted for 266 CTA reports mentioning CaW. The majority of these reports (n=206, 77%) were associated with stroke/transient ischemic attack (TIA) ICD-9/ICD-10 codes. The rate of CaW diagnosis adjusted per 1000 patients with stroke/TIA increased over time, 2015 being the most significant point of change ('joinpoint'; p=0.01). The analysis of CaW mentions normalized per 1000 CTA reports also showed increasing rates of diagnosis over time (joinpoint:2014; p<0.02). CONCLUSION: CaW was predominantly identified in patients with strokes/TIAs rather than asymptomatic patients. CaW was commonly overlooked in facilities with lower levels of cerebrovascular certification. Recognition of CaW at a CSC has significantly increased over time, independent of overall imaging and stroke patient volume.

Cardio-Cerebral Infarction, Free-Floating Thrombosis and Hyperperfusion in COVID-19.

Cardio-cerebral infarction, which refers to an acute ischemic stroke (AIS) and acute myocardial infarction (AMI) that occur concurrently, is an uncommon phenomenon with a grave prognosis. Intraluminal carotid thrombus (ICT) is an infrequently encountered cause of ischemic stroke and can be associated with an underlying hypercoagulable state. One severe yet prevalent complication of infection with Coronavirus Disease 2019 (COVID-19) is thrombosis from multi-pathway inflammatory responses. Here, we present a unique case of cardio-cerebral infarction, with a free-floating intraluminal thrombus in the left internal carotid artery, in the setting of recent COVID-19 infection, and with the etiology of both events attributed to a COVID-19 hypercoagulable state. CT perfusion imaging also showed an interesting imaging finding of hyperperfusion, which is believed to be a form of dysfunctional cerebral autoregulation.

Intraluminal carotid thrombosis and acute ischemic stroke associated with COVID-19.

COVID-19 (Coronavirus disease 2019) caused by SARS-CoV-2 has a diverse constellation of neurological manifestations that include encephalopathy, stroke, Guillain-Barré syndrome, myelitis, and encephalitis. Intraluminal carotid thrombi (ILT) are infrequent lesions seen in only 1.6% of patients with acute ischemic stroke. Underlying atherosclerosis is the most common lesion associated with ILT formation. However, with COVID-19, we have encountered ILT in patients without significant atherosclerotic disease. The endothelial inflammation and hypercoagulable state associated with COVID-19 pose a risk of arterial and venous thromboembolism and could have contributed to this presentation although the exact pathophysiology and optimal treatment of ILT in COVID-19 remain elusive. Herein, we present a series of ischemic stroke patients with carotid ILT in the setting of a recent SARS-CoV-2 infection.

Clot composition in retrieved thrombi after mechanical thrombectomy in strokes due to carotid web.

BACKGROUND: The association of carotid webs (CaW) and ischemic stroke is being increasingly recognized. Data on the histologic clot architecture in strokes caused by CaW has not been previously described. Understanding thrombi histopathology may provide insight into the pathophysiology of CaW-related strokes. METHODS: This case series presents three patients with acute ischemic stroke thought to be caused by ipsilateral CaW. Thromboemboli were retrieved from the middle cerebral artery (MCA) by mechanical thrombectomy and histologic analysis was performed. RESULTS: Three patients aged between 41 and 55 years with few to no vascular risk factors presented with symptoms concerning for an acute MCA territory infarction (National Institutes of Health Stroke Scale (NIHSS) range 10-17). Non-contrast computed tomography (CT) Alberta Stroke Program Early CT Score (ASPECTS) range was 7-8 and all patients had hyperdense vessel sign. Initial CT angiogram was concerning for CaW with no superimposed thrombus, later confirmed with conventional angiography. All patients underwent thrombectomy with full reperfusion. Comprehensive stroke workup failed to reveal other etiologies besides ipsilateral CaW. The histopathologic appearance was of typical fresh mixed thrombi. Qualitative thrombus composition analysis of clot from Case #1 yielded 42.5% fibrin, 50.0% red blood cells (RBC), and 7.5% white blood cells (WBC); Case #2 yielded 46.9% fibrin, 43.4% RBC, and 9.7% WBC; and Case #3 yielded 61.5% fibrin, 31.8% RBC, and 6.7% WBC. CONCLUSIONS: The clot composition of large vessel occlusion strokes from CaW is comparable to the histopathology of previously reported clots from other stroke etiologies. Advanced staining techniques may aid in further characterizing the thrombi of this poorly understood condition.