Vessel Wall Enhancement Research Articles

Hypothesizing the interplay of thrombus formation, wall enhancement, and perifocal edema as predictive factors for rupture in brain AVMs

The presence of shear stress within intracranial AVMs (arteriovenous malformations) due to high flow, perinidal angiogenesis, intranidal aneurysms, and biological factors are presumed risk factors for rupture. However, emerging imaging and histological evidence suggests that risk factors for AVM rupture may extend beyond the classical understanding. The presence of perifocal edema at the time of rupture, luminal thrombosis, and vessel wall enhancement on vessel wall MRI elucidate the possibility of an underlying inflammatory process within AVMs, which may predispose them to instability and eventual rupture. We hypothesize that for some AVM ruptures, the occurrence of thrombosis within vascular outpouchings of the AVM initiates a cascade of events, including local hypoxia, inflammation, breakdown of the blood–brain barrier and wall matrix degradation, which is evident from the presence of perifocal edema and vessel wall enhancement observed on imaging. These changes might ultimately raise the risk of AVM rupture and subsequent hemorrhage. Understanding this inflammatory component offers promising insights into AVM pathogenesis and may facilitate the development of preventive strategies to mitigate rupture risk.

Temporal evolution of primary angiitis of the central nervous system (PACNS) on MRI following immunosuppressant treatment

PurposeTo systematically analyse the time course of vessel wall enhancement and associated stenosis in patients with primary angiitis of the central nervous system (PACNS) following immunosuppressive therapy.Material and methodsTwo neuroradiologists retrospectively analysed MRIs of patients with PACNS seen at the Bern University Hospital and the St. Gallen Cantonal Hospital between 2015 and 2020. MRIs were examined for the presence of vessel wall enhancement, length of vessel wall enhancement (mm), circumferential extent of enhancement (degree) and degree of stenosis (%). Descriptive statistics and measurements of interobserver reliability were obtained. To investigate the temporal profiles of the variables following the commencement of immunosuppressant treatment, four series of Bayesian generalised multi-level models were generated.ResultsA total of 23 patients with 43 affected vessels identified from 209 MRI exams were evaluated (mean follow-up: 715 days, standard deviation ± 487 days), leading to a complete dataset of 402 entries. Vessel wall enhancement and circumferential extent of enhancement decreased for approximately 1 year after the initiation of immunosuppressant therapy. Changes were more pronounced in younger patients. Disappearance of vessel wall enhancement (in at least one vessel) was seen in about half of patients after a median of 172 days interquartile range 113–244, minimum 54 days, maximum 627 days.ConclusionsThis study evaluated the typical time course of vessel wall enhancement in patients with PACNS. Our results could be a useful reference for radiologists and clinicians interpreting follow-up imaging in patients with PACNS.Critical relevance statementRoutine clinical exams can be interpreted with more confidence when radiologists are aware of the typical temporal evolution of vessel wall enhancement in patients with primary angiitis of the central nervous system after initiation of immunosuppressive therapy.Key PointsFew data exist for vessel wall imaging of primary angiitis of the central nervous system.Following immunosuppressant therapy, vessel wall enhancement decreases for approximately one year.These results may serve as a reference for radiologists performing follow-up imaging.Graphical

The Contribution of Vessel Wall Magnetic Resonance Imaging to the Diagnosis of Primary and Secondary Central Nervous System Vasculitis.

To describe high-resolution brain vessel wall MRI (VW-MRI) patterns and morphological brain findings in central nervous system (CNS) vasculitis patients. Fourteen patients with confirmed CNS Vasculitis from two tertiary centers underwent VW-MRI using a 3T scanner. The images were reviewed by two neuroradiologists to assess vessel wall enhancement characteristics and locations. Fourteen patients were included (six females; average age 48 ± 19 years). Diagnoses included primary CNS vasculitis (PCNSV) in six patients and secondary CNS vasculitis (SCNSV) in eight, half of which were infection-related. Thirteen patients showed vessel wall enhancement, which was intense in eleven patients (84.6%) and concentric in twelve (92.3%), affecting the anterior circulation in nine patients (69.2%), posterior in two patients (15.4%), and both circulations in two patients (15.4%). The enhancement patterns were similar across different CNS vasculitis types. DWI changes corresponded with areas of vessel wall enhancement in 77% of patients. Conclusions: CNS vasculitis is often associated with intense, concentric vessel wall enhancement in VW-MRI, especially in the anterior circulation. The consistent presence of DWI alterations in affected territories suggests a possible link to microembolization or hypoperfusion. These imaging findings complement parenchymal brain MRI and MRA/DSA data, potentially increasing the possibility of a clinical diagnosis of CNS vasculitis.

Vessel wall imaging in the diagnosis of antiphospholipid syndrome presenting as Moyamoya syndrome-A case report.

Objectives: We describe a case of anti-phospholipid syndrome (APLS) vasculopathy presenting with Moyamoya syndrome (MMS) and show the associated intracranial vessel wall MRI (VWI) findings. Methods: A 37-year-old-woman presented with acute onset dizziness and left-sided weakness. Neurologic exam revealed a left facial droop and left hemiparesis. She underwent a comprehensive laboratory work-up for stroke. Neuroimaging included a CT head, CT angiogram, VWI, and digital subtraction angiography. Results: Work-up revealed a triple-positive APLS antibody profile. CT of the head showed an acute right basal ganglia infarction and right frontal subarachnoid hemorrhage. CT angiogram revealed severe stenosis of the right internal carotid artery terminus in a Moyamoya pattern. Intracranial VWI showed long-segment concentric vessel wall thickening and homogeneous vessel wall enhancement and T2-hyperintense wall edema of the stenotic right ICA terminus, M1 middle cerebral artery, and A1 anterior cerebral artery. She was treated with long-term anticoagulation with warfarin and a right superficial temporal artery to middle cerebral artery bypass. Discussion: We present intracranial VWI features of vessel wall pathology in a patient with primary APLS presenting with MMS.

Histopathological analysis of the wall enhancement of the spinal dural arteriovenous fistulae's draining veins.

The mechanism behind SDAVF is still unclear. We discovered that the vessel wall of the SDAVF-DV occasionally showed enhancement in MRI, and this study assessed the relationship between the enhancement of the draining vein's wall and its histology. For histopathologic analysis, 16 draining vein samples from 16 patients with SDAVF were included, 3 normal arteries and 3 normal veins were chosen as comparison. We assessed the imaging and microscopic characteristics of the draining veins in SDAVF patients. The former included the presence of significant enhancement of the wall of the draining vein in MRI, and the latter included the adherence, aggregation, infiltration of pro-inflammatory factors and inflammatory cells. Immuno-histochemical staining was performed using IL-1β, IL-8, TGF-β as well as MPO and MMP-9, and positive results were counted. Multiple logistic regression analysis was used to determine whether the infiltration of inflammatory cells was connected to vessel wall enhancement in the SDAVF-DV. Infiltration of inflammatory cells was significantly higher in SDAVF-DV compared to normal vessels, 7 out of 16 patients significantly had enhancement of the vessel wall of SDAVF-DV, and logistic regression analysis showed that samples with more infiltration of inflammatory cells were more likely to show enhancement of the SDAVF-DV walls. There was considerable inflammatory cells infiltration in SDAVF-DV, and this may explain why their vessel wall had such a significant enhancement in MRI.

Are hemodynamics responsible for inflammatory changes in venous vessel walls? A quantitative study of wall-enhancing intracranial arteriovenous malformation draining veins.

Signal enhancement of vascular walls on vessel wall MRI might be a biomarker for inflammation. It has been theorized that contrast enhancement on vessel wall imaging (VWI) in draining veins of intracranial arteriovenous malformations (AVMs) may be associated with disease progression and development of venous stenosis. The aim of this study was to investigate the relationship between vessel wall enhancement and hemodynamic stressors along AVM draining veins. Eight AVM patients with 15 draining veins visualized on VWI were included. Based on MR venography data, patient-specific 3D surface models of the venous anatomy distal to the nidus were segmented. The enhanced vascular wall regions were manually extracted and mapped onto the venous surface models after registration of image data. Using image-based blood flow simulations applying patient-specific boundary conditions based on phase-contrast quantitative MR angiography, hemodynamics were investigated in the enhanced vasculature. For the shear-related parameters, time-averaged wall shear stress (TAWSS), oscillatory shear index (OSI), and relative residence time (RRT) were calculated. Velocity, oscillatory velocity index (OVI), and vorticity were extracted for the intraluminal flow-related hemodynamics. Visual observations demonstrated overlap of enhancement with local lower shear stresses resulting from decreased velocities. Thus, higher RRT values were measured in the enhanced areas. Furthermore, nonenhancing draining veins showed on average slightly higher flow velocities and TAWSS. Significant decreases of 55% (p = 0.03) for TAWSS and of 24% (p = 0.03) for vorticity were identified in enhanced areas compared with near distal and proximal domains. Velocity magnitude in the enhanced region showed a nonsignificant decrease of 14% (p = 0.06). Furthermore, increases were present in the OSI (32%, p = 0.3), RRT (25%, p = 0.15), and OVI (26%, p = 0.3) in enhanced vessel sections, although the differences were not significant. This novel multimodal investigation of hemodynamics in AVM draining veins allows for precise prediction of occurring shear- and flow-related phenomena in enhanced vessel walls. These findings may suggest low shear to be a local predisposing factor for venous stenosis in AVMs.

Time-of-flight and black-blood MRI to study intracranial arteries in rats

Intracranial aneurysms (IAs) are usually incidentally discovered by magnetic resonance imaging (MRI). Once discovered, the risk associated with their treatment must be balanced with the risk of an unexpected rupture. Although clinical observations suggest that the detection of contrast agent in the aneurysm wall using a double-inversion recovery black-blood (BB) sequence may point to IA wall instability, the exact meaning of this observation is not understood. Validation of reliable diagnostic markers of IA (in)stability is of utmost importance to deciding whether to treat or not an IA. To longitudinally investigate IA progression and enhance our understanding of this devastating disease, animal models are of great help. The aim of our study was to improve a three-dimensional (3D)-time-of-flight (TOF) sequence and to develop a BB sequence on a standard preclinical 3-T MRI unit to investigate intracranial arterial diseases in rats. We showed that our 3D-TOF sequence allows reliable measurements of intracranial artery diameters, inter-artery distances, and angles between arteries and that our BB sequence enables us to visualize intracranial arteries. We report the first BB-MRI sequence to visualize intracranial arteries in rats using a preclinical 3-T MRI unit. This sequence could be useful for a large community of researchers working on intracranial arterial diseases.Relevance statement We developed a black-blood MRI sequence to study vessel wall enhancement in rats with possible application to understanding IAs instability and finding reliable markers for clinical decision-making.Key points• Reliable markers of aneurysm stability are needed for clinical decision.• Detection of contrast enhancement in the aneurysm wall may be associated with instability.• We developed a black-blood MRI sequence in rats to be used to study vessel wall enhancement of IAs.Graphical

High-resolution MRI vessel wall enhancement in moyamoya disease: risk factors and clinical outcomes.

Intracranial vessel wall enhancement (VWE) on high-resolution magnetic resonance imaging (HRMRI) is associated with the progression and poor prognosis of moyamoya disease (MMD). This study assessed potential risk factors for VWE in MMD. We evaluated MMD patients using HRMRI and traditional angiography examinations. The participants were divided into VWE and non-VWE groups based on HRMRI. Logistic regression was performed to compare the risk factors for VWE in MMD. The incidence of cerebrovascular events of the different subgroups according to risk factors was compared using Kaplan-Meier survival and Cox regression. We included 283 MMD patients, 84 of whom had VWE on HRMRI. The VWE group had higher modified Rankin Scale scores at admission (p = 0.014) and a higher incidence of ischaemia and haemorrhage (p = 0.002) than did the non-VWE group. Risk factors for VWE included the ring finger protein 213 (RNF213) p.R4810K variant (odds ratio [OR] 2.01, 95% confidence interval [CI] 1.08-3.76, p = 0.028), hyperhomocysteinaemia (HHcy) (OR 5.08, 95% CI 2.34-11.05, p < 0.001), and smoking history (OR 3.49, 95% CI 1.08-11.31, p = 0.037). During the follow-up of 63.9 ± 13.2months (median 65months), 18 recurrent stroke events occurred. Cox regression showed that VWE and the RNF213 p.R4810K variant were risk factors for stroke. The RNF213 p.R4810K variant is strongly associated with VWE and poor prognosis in MMD. HHcy and smoking are independent risk factors for VWE. Vessel wall enhancement in moyamoya disease is closely associated with poor prognosis, especially related to the ring finger protein 213 p.R4810K variant, hyperhomocysteinaemia, and smoking, providing crucial risk assessment information for the clinic. • The baseline presence of vessel wall enhancement is significantly associated with poor prognosis in moyamoya disease. • The ring finger protein 213 p.R4810K variant is strongly associated with vessel wall enhancement and poor prognosis in moyamoya disease. • Hyperhomocysteinaemia and smoking are independent risk factors for vessel wall enhancement in moyamoya disease.

Imaging investigation of cervicocranial artery dissection by using high resolution magnetic resonance VWI and MRA: qualitative and quantitative analysis at different stages

BackgroundTo explore the value of magnetic resonance angiography (MRA) and high resolution magnetic resonance vessel wall imaging (HRMR-VWI) in cervicocranial artery dissection (CCAD) for the disease diagnosis, course staging and treatment. On the basis of qualitative evaluation, this study also extract the changes of different stages in vessel wall in different vessel segments to identify imaging indicators for the quantitative evaluation of CCAD.MethodsWe retrospectively enrolled 34 patients with CCAD (38branches) with conventional MRA and HRMR-VWI examinations. Two radiologists independently analyzed imaging features of vessel wall and lumen in the different stages, and the typical sign detection of artery dissection were compared between MRA and HRMR-VWI. Then the parameters of vessel wall was quantitatively evaluated by the post-processing software (Vesselmass, Leiden University Medical Center, Leiden, The Netherlands.ResultsHRMR-VWI revealed typical sign detection of artery dissection in all patients in the acute and subacute stage. Among them, the intimal flap/double lumen sign ditection were more common than the MRA, there was significant difference (P = 0.012). MRA revealed typical sign detection of artery dissection in more than half the patients, and the detection was no significant difference at the chronic stage between MRA and HRMR-VWI (P = 1.000/1.000/0.761). In the acute and subacute stage, the typical sign detection of intramural hematoma and Grade II enhancement revealed by HR-MRI was higher than the observations in the chronic stage (P = 0.000/0.000/0.016), while there was no significant difference by MRA (P = 0.902). The values of wall thickness, relative signal intensity of vessel wall enhancement, relative signal intensity of intramural hematoma (IMH), and percentage of stenosis in CCAD decreased from acute to subacute and then to chronic stages. Each quantitative parameter in patients with CCAD in the early stages (i.e., acute and subacute stages) was significantly different from that in patients with CCAD in the recovered group at chronic stage (P < 0.05). Wall thickness and relative signal intensity of vessel wall enhancement in patients with CCAD in the early stages were not significantly different from those in patients with CCAD in the incompletely recovered group at chronic stage (P > 0.05).ConclusionsAs the only noninvasive imaging technology, HRMR-VWI displays the structure of the vessel wall in vivo, showing not only excellent performance in the early diagnosis of CCAD, but also describing the changes of different stages in the qualitative and quantitative characteristics of vessel wall. It also helps to guide the diseasediagnosis, course staging and treatment of CCAD. Although the diagnostic efficacy of MRA was not as good as HRMR-VWI, it should be the first choice of method for routine examination in evaluating CCAD, especially at the chronic stage of CCAD.

Symptomatic Non-stenotic Atherosclerotic Disease in Small Subcortical Infarcts: A North American Pilot Study.

Recent small subcortical infarcts (SSI) are a common radiographic predecessor to lacunar stroke. SSI is comprised of several pathophysiologic processes such as branch atherosclerotic disease (BAD) and lipohyalinosis, both of which have differing outcomes and natural history. Presently, there is not a proven method to determine whether a SSI is due to BAD or lipohyalinosis in non-stenotic vessels. However, high-resolution vessel wall imaging (HRVWI) has been reported in East Asian cohorts. We aimed to use HRVWI to identify individuals with BAD-related SSI in a North American cohort. We performed a cross-sectional study from the Rhode Island Hospital. All patients had a SSI as defined by consensus criteria. The presence of vessel wall enhancement of parent vessels were reviewed by two authors. Standard descriptive statistical techniques were used. Of 28 patients who underwent HRVWI, 7 met criteria for SSI. The median age was 68years and 3 were female. Parent vessel wall enhancement was present in 2 patients. In our North American cohort, HRVWI was able to dichotomize individuals based on parent vessel wall enhancement suggestive of a BAD-related SSI. Further studies are needed to expand our cohort size and confirm our findings.

Effect of arteriosclerotic intracranial arterial vessel wall enhancement on downstream collateral flow.

The effect of arteriosclerotic intracranial arterial vessel wall enhancement (IAVWE) on downstream collateral flow found in vessel wall imaging (VWI) is not clear. Regardless of the mechanism underlying IAVWE on VWI, damage to the patient's nervous system caused by IAVWE is likely achieved by affecting downstream cerebral blood flow. The present study aimed to investigate the effect of arteriosclerotic IAVWE on downstream collateral flow. The present study recruited 63 consecutive patients at the Second Hospital of Hebei Medical University from January 2021 to November 2021 with underlying atherosclerotic diseases and unilateral middle cerebral artery (MCA) M1-segment stenosis who underwent an magnetic resonance scan within 3 days of symptom onset. The patients were divided into 4 groups according to IAVWE and the stenosis ratio (Group 1, n = 17; Group 2, n = 19; Group 3, n = 13; Group 4, n = 14), and downstream collateral flow was analyzed using three-dimensional pseudocontinuous arterial spin labeling (3D-pCASL) and RAPID software. The National Institutes of Health Stroke Scale (NIHSS) scores of the patients were also recorded. Two-factor multivariate analysis of variance using Pillai's trace was used as the main statistical method. No statistically significant difference was found in baseline demographic characteristics among the groups. IAVWE, but not the stenosis ratio, had a statistically significant significance on the late-arriving retrograde flow proportion (LARFP), hypoperfusion intensity ratio (HIR), and NIHSS scores ( F = 20.941, P <0.001, Pillai's trace statistic = 0.567). The between-subject effects test showed that IAVWE had a significant effect on the three dependent variables: LARFP ( R2 = 0.088, F = 10.899, P = 0.002), HIR ( R2 = 0.234, F = 29.354, P <0.001), and NIHSS ( R2 = 114.339, F = 33.338, P <0.001). Arteriosclerotic IAVWE significantly reduced downstream collateral flow and affected relevant neurological deficits. It was an independent factor affecting downstream collateral flow and NIHSS scores, which should be a focus of future studies. ChiCTR.org.cn, ChiCTR2100053661.

Clinical Problem Solving: A 38-year-Old Woman With Systemic Lupus Erythematosus Presenting With Headache, Nausea, and Vomiting.

A 38-year-old woman with migraine headaches and systemic lupus erythematosus with recent cessation of her immunosuppressive therapy presents with prolonged headache and hypertensive emergency. Her examination is notable for a peripheral right facial palsy and stable malar rash. There are no signs of systemic infection nor systemic symptoms of a lupus flare. Initial CT head reveals bilateral hypodensities in the basal ganglia. Within 8hours of presentation, she develops right hemiplegia and becomes encephalopathic. MRI shows multifocal acute infarcts (most notably in the left basal ganglia), enhancement of the right facial nerve, and multifocal vessel wall enhancement in the anterior and posterior circulation. We discuss the differential diagnosis, comprehensive workup, and subsequent treatment decisions in the management of this immunocompromised patient with encephalopathy, headache, and rapidly progressing focal neurologic deficits.

Diagnostic Value of Vessel Wall Imaging to Determine the Timing of Extracranial‒Intracranial Bypass for Moyamoya Syndrome Associated with Active Sjögren's Syndrome: A Case Report.

Sjögren's syndrome is a chronic autoimmune disorder that predominantly affects exocrine organs. It is characterized by an organ-specific infiltration of lymphocytes. The involvement of the major cerebral arteries in Sjögren's syndrome has rarely been reported. A recent study reported a case of successful extracranial-intracranial (EC-IC) bypass without complications, even in the active inflammatory state, although the optimal timing of such a bypass remains unclear. We here report the case of a 43-year-old woman presenting with acute ischemic stroke due to progressive middle cerebral artery (MCA) occlusion and signs of primary Sjögren's syndrome. During intensive immunosuppressive therapy for active Sjögren's syndrome, the patient was monitored using contrast-enhanced magnetic resonance vessel wall imaging (MR-VWI). A couple of intravenous cyclophosphamide injections combined with a methylprednisolone pulse and antiplatelet therapy resulted in clear resolution of vessel wall enhancement, which suggested remission of inflammatory vasculitis. Nevertheless, she still experienced a transient ischemic attack (TIA) due to decreased regional cerebral blood flow by MCA occlusion, as demonstrated by the conventional time-of-flight MR angiography and single-photon emission computed tomography. Considering the increased risk of further stroke, the decision was made to perform an EC-IC bypass as a treatment for medically uncontrollable hemodynamic impairment. Her postoperative course was uneventful without further repeated TIAs, and continued immunosuppressive therapy for Sjögren's syndrome provided effective management. Our findings emphasize the diagnostic value of contrast-enhanced MR-VWI in monitoring the effect of immunosuppressive therapy for the major cerebral artery vasculitis and in determining the timing of EC-IC bypass as a "rescue" treatment for moyamoya syndrome associated with active Sjögren's syndrome.

Vasculitis in the Central Nervous System: Treatment and Outcomes in a Large Single-Center Cohort (P9-5.014)

<h3>Objective:</h3> To describe the demographics, treatment, and outcomes of patients presenting with vasculitis involving the central nervous system (CNS) at a major referral center covering a five-state catchment area in the Intermountain West. <h3>Background:</h3> CNS vasculitis is inflammation of blood vessel walls in the brain or spine that can be life-threatening and/or result in ischemic strokes. Markers suggestive of CNS inflammation include cerebrospinal fluid (CSF) pleocytosis and/or elevated protein level, and on neuroaxis imaging, the presence of parenchymal, leptomeningeal, or vessel wall enhancement. At the time of symptom onset, the diagnostic evaluation focuses on classifying the size of the involved vessels and determining if the vasculitis is a primary angiitis of the CNS or if it is occurring secondary to another process elsewhere in the body, such as a systemic vasculitis, connective tissue disorder, infection, malignancy, or toxic/drug exposure. <h3>Design/Methods:</h3> A retrospective chart review for patients within the University of Utah Health system queried all International Classification of Diseases (ICD) 9 codes for 437.4 (Cerebral arteritis) and ICD10 codes for I67.7 (Cerebral arteritis, not elsewhere classified) with one encounter with a neurologist. Of the 144 total patients identified between January 2012 to October 2022, we further confirmed the diagnosis based on neurologists’ review of each record and categorized patients further to determine if the CNS presentation of vasculitis was primary, secondary, or indeterminate/uncertain. <h3>Results:</h3> We described the demographic characteristics of vasculitis patients with CNS involvement seen at the University of Utah, as well as presenting symptoms, relevant comorbidities, distinct imaging findings (including a focus on imaging modality), CSF abnormalities, treatments, and outcomes. <h3>Conclusions:</h3> This is a comprehensive characterization of patients with vasculitis involving the CNS in a large area of the Intermountain West. Understanding the differences in these patient characteristics, symptoms, and variability in diagnostic and treatment plans will inform better, more efficient approaches. <b>Disclosure:</b> Dr. Hoshina has nothing to disclose. Alen Delic has nothing to disclose. Mr. Wong has nothing to disclose. Dr. Lyden has nothing to disclose. Dr. Kadish has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. The institution of Dr. Kadish has received research support from Alexion Pharmaceuticals. Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology/AAN Publications. The institution of Dr. Clardy has received research support from Sumaira Foundation for NMO. The institution of Dr. Clardy has received research support from Western Institute for Veteran Research. The institution of Dr. Clardy has received research support from NIH/NINDS. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel and Lodging with U of Iowa. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Speaker Honoraria for Grand Rounds with Barrow Neurological Institute. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Speaker Honoraria for Grand Rounds with Beaumont Health.

Abstract WP101: Vessel Wall Enhancement Is Associated With Symptomatic Adult Moyamoya Disease And Syndrome In An American Population

Introduction: Moyamoya Disease (MMD) and Moyamoya Syndrome (MMS) are chronic progressive cerebrovascular disease characterized by bilateral or unilateral stenotic arteries around the circle of Willis with subsequent development of collateral blood vessels. MMS is associated with conditions such as Down Syndrome (DS) or sickle cell disease (SCD). The presence of vessel wall enhancement (VWE) on high resolution magnetic resonance vessel wall imaging (HR MR VWI) in MMD or MMS is controversial. Recent studies suggest VWE is associated with recent ischemic or hemorrhagic stroke. The significance of VWE in MMD or MMS in non-Asian population has not been well established. Our study aimed to investigate the prevalence of VWE in adult MMD/MMS in the United States and identify its predictive value for stenosis progression. Methods: We retrospectively reviewed patients with MMD or MMS admitted to Clements University Hospital and Parkland Memorial Hospital affiliated with University of Texas Southwestern Medical Center from January 2014 to March 2022. Patients were included if HR MR VWI was performed during workup. Patients were excluded if intracranial atherosclerosis was the suspected stroke etiology from cardiovascular risk factors. The presence of the VWE on HR MR VWI and stenosis grading were evaluated by blinded neuroradiologists and vascular neurologists. Symptomatic disease was defined as ischemic stroke, hemorrhagic stroke or transient ischemic attack within 24 months before HR MR VWI was obtained. Results: A total of 32 patients were included, of which 28 had MMD, 3 had MMS from SCD and 1 had MMS from DS. Mean age at diagnosis was 34 ± 10 years old, and 29 (90.6%) were female. VWE was seen in 22 (68.8%) patients. VWE is more common in symptomatic disease than asymptomatic disease (95% vs 20%). 24 patients had follow up MR angiogram with average time from HR MR VWI of 25.8 ± 16.7 months. Among 12 patients with 16 non-occlusive arteries, stenosis progression was seen in 67% of arteries with VWE compared to 25% of arteries without VWE. Conclusions: In a North American population with MMD or MMS, VWE on HR MR VWI was more common in symptomatic disease. The presence of VWE was associated with increased risk of stenosis progression. More studies are needed to confirm these findings.

Abstract WP96: Quantitative Imaging Markers On Vessel Wall Magnetic Resonance Imaging Correlate With Disease Activity And Treatment Response In Cerebral Vasculopathies

Introduction: Little is known about the utility of vessel wall magnetic resonance imaging (VWMRI) in assessing treatment response in various inflammatory and non-inflammatory intracranial vasculopathies, and if there is any correlation of vessel wall enhancement (VWE) with disease activity. We aimed to quantitatively investigate the differences in VWE patterns on follow-up VWMRI in patients with primary CNS vasculitis (PCNSV), secondary CNS vasculitis (SCNSV) and intracranial atherosclerotic disease (ICAD). Methods: VWMRI scans of patients with a confirmed diagnosis of either PCNSV, SCNSV or ICAD presenting to our tertiary care center between January 2015 and January 2021 were reviewed. Inclusion criteria were age greater than 18 years and at least 2 VWMRI scans available, including one from time of first clinical presentation with active disease. For each patient, 20 arterial segments were evaluated on VWMRI for the presence of VWE and the total number of enhancing arterial segments were reported. Percent concentricity, median number of enhancing arterial segments (MEAS) and percent change in MEAS was calculated and compared across all 3 diagnoses. Results: A total of 28 patients met study inclusion criteria (median age 47, 43% male) and had a diagnosis of either PCNSV (n=6), SCNSV (n=7) or ICAD (n=15). Median time to follow-up VWMRI was 22, 20 &amp; 11 weeks for PCNSV, SCNSV &amp; ICAD respectively. On initial VWMRI, the MEAS was 7 for each PCNSV &amp; SCNSV and 6 for ICAD. Higher percent concentricity of VWE was seen more with PCNSV (92.3%) and SCNSV (71.4%) compared to ICAD (22.2%). All (100%) patients with PCNSV and 2 (33%) patients with SCNSV were treated with immunosuppression whereas 3 (50%) patients with SCNSV were treated with antimicrobials. All patients with PCNSV and SCNSV improved clinically whereas those with ICAD progressed or remained stable. The MEAS on follow up VWMRI decreased with treatment in patients with PCNSV (43% decrease) and SCNSV (14% decrease) but increased in those with ICAD (16% increase) and correlated clinically with disease activity and treatment response. Conclusion: Percent concentricity and MEAS on VWMRI may be useful non-invasive markers to monitor disease activity &amp; treatment response in patients with intracranial vasculopathies.

Neuroinflammatory Disease Responsive to MEK-Inhibitor

Objective Illustrate that some neuroinflammatory diseases may respond best to antiproliferative therapies rather than immunomodulatory therapies. Background Genomics are increasingly employed in the diagnostic armamentarium of refractory neuro-inflammatory diseases. Metagenomic next-generation sequencing is used to detect pathogens and germline genetic testing is used to detect inborn errors of the immune system. Genetic testing of tissue can identify somatic mutations for targeted treatment. MEK-inhibitors are an emerging treatment for RAS/MAPK pathway mutated diseases which include some neuro-inflammatory mimics like neuro-histiocytoses. Design/Methods NA. Results Previously healthy 12-year-old girl presented with 1 month of diplopia and headaches. Her brother has clinically diagnosed NF1 (café-au-lait macules, cutaneous neurofibromas). Exam notable for right third nerve palsy. MRI showed T2/FLAIR hyperintense lesions of the right temporal lobe, basal ganglia, and cervical through thoracic cord, nodular leptomeningeal enhancement along the entire spinal cord, and right middle cerebral artery vessel wall enhancement. CSF: WBC 6/mm3 (62% lymphocytes 37% monocytes), protein 133 mg/dL. She improved with pulse methylprednisolone and maintenance steroids. At 5 months, she developed malignant elevated intracranial pressure with CSF OP &gt;50 cm water, bradycardia, and encephalopathy requiring weekly LPs. Brain biopsy showed astrocytic and microglial activation without significant inflammation. No histiocytes were noted. There was no evidence of neoplasia or infection. She was tried on anakinra. At 6 months, she developed left third nerve palsy and seizures. For weeks, she required daily LPs for intracranial hypertension despite placement of ventriculoperitoneal shunt. Additional treatments included infliximab, steroids, and siltuximab. NGS from brain biopsy identified 2 NF1 mutations (nonsense, splicing). Allele fractions: 6% and 9%. Her mental status and need for frequent LP improved dramatically with trametinib. Conclusions This case illustrates the importance of considering somatic genomic testing of neural tissue even when the neuropathology is not suggestive of a malignancy or histiocytosis as this can inform newer molecularly targeted therapeutic options.

Vessel wall imaging features of Moyamoya disease in a North American population: patterns of negative remodelling, contrast enhancement, wall thickening, and stenosis

BackgroundThis study characterized vessel wall imaging (VWI) features of Moyamoya disease (MMD) in a predominantly adult population at a North American center.MethodsConsecutive patients with VWI were included. Twelve arterial segments were analyzed for wall thickening, degree and pattern of contrast enhancement, and remodeling.ResultsOverall, 286 segments were evaluated in 24 patients (mean age = 36.0 years [range = 1–58]). Of 172 affected segments, 163 (95%) demonstrated negative remodeling. Complete vessel wall obliteration was most frequent in the proximal M1 (17/48, 35%). Affected segments enhanced in 72/172 (42%) (n = 15 for grade II; n = 54 for concentric and n = 18 for eccentric); 20 of 24 (83%) patients had at least one enhancing segment. Both enhancing and non-enhancing segments were present in 19/20 (95%) patients. Vessel wall enhancement was most common in the proximal segments and correlated to the degree of stenosis (p < 0.001), and outer wall diameter (p < 0.001), but not disease duration (p = 0.922) or Suzuki score (p = 0.477). Wall thickening was present in 82/172 (48%) affected segments and was associated with contrast enhancement (p < 0.001), degree of stenosis (p < 0.001), and smaller outer wall diameter (p = 0.004).ConclusionThis study presents VWI findings in North American patients with MMD. Negative remodeling was the most common finding. Most patients had both enhancing and non-enhancing abnormal segments. Vessel wall enhancement was most common in proximal segments, variable in pattern or degree and was correlated to the degree of stenosis and smaller outer wall diameter.

Intraorbital findings in giant cell arteritis on black blood MRI

ObjectiveBlindness is a feared complication of giant cell arteritis (GCA). However, the spectrum of pathologic orbital imaging findings on magnetic resonance imaging (MRI) in GCA is not well understood. In this study, we assess inflammatory changes of intraorbital structures on black blood MRI (BB-MRI) in patients with GCA compared to age-matched controls.MethodsIn this multicenter case-control study, 106 subjects underwent BB-MRI. Fifty-six patients with clinically or histologically diagnosed GCA and 50 age-matched controls without clinical or laboratory evidence of vasculitis were included. All individuals were imaged on a 3-T MR scanner with a post-contrast compressed-sensing (CS) T1-weighted sampling perfection with application-optimized contrasts using different flip angle evolution (SPACE) BB-MRI sequence. Imaging results were correlated with available clinical symptoms.ResultsEighteen of 56 GCA patients (32%) showed inflammatory changes of at least one of the intraorbital structures. The most common finding was enhancement of at least one of the optic nerve sheaths (N = 13, 72%). Vessel wall enhancement of the ophthalmic artery was unilateral in 8 and bilateral in 3 patients. Enhancement of the optic nerve was observed in one patient. There was no significant correlation between imaging features of inflammation and clinically reported orbital symptoms (p = 0.10). None of the age-matched control patients showed any inflammatory changes of intraorbital structures.ConclusionsBB-MRI revealed inflammatory findings in the orbits in up to 32% of patients with GCA. Optic nerve sheath enhancement was the most common intraorbital inflammatory change on BB-MRI. MRI findings were independent of clinically reported orbital symptoms.Key Points• Up to 32% of GCA patients shows signs of inflammation of intraorbital structures on BB-MRI.• Enhancement of the optic nerve sheath is the most common intraorbital finding in GCA patients on BB-MRI.• Features of inflammation of intraorbital structures are independent of clinically reported symptoms.

Vessel wall enhancement as a predictor of arterial stenosis progression and poor outcomes in moyamoya disease.

This study aimed to determine the association between vessel wall enhancement and progression of arterial stenosis and clinical outcomes in patients with moyamoya (MMD) using high-resolution magnetic resonance (HRMR) vessel wall imaging. Consecutive participants diagnosed with MMD were prospectively recruited and underwent HRMR at baseline and during follow-up, which had an interval period of ≥ 6 months and were clinically followed up for ≤ 24 months to record the occurrence of ischemic stroke. The relationship between vessel wall enhancement and arterial stenosis progression and stroke occurrence was evaluated. HRMR vessel wall imaging was used to identify 309 stenotic lesions at the internal carotid artery (ICA) in 170 participants (mean age: 37.7 ± 11.3 years old, male: 44.1%). The baseline presence (adjusted odds ratio [aOR] = 3.57, 95% CI = 1.97-6.44, p < 0.001) and progression (aOR = 2.96, 95% CI = 1.29-6.80, p = 0.010) of vessel wall enhancement and middle cerebral artery (MCA) involvement (aOR = 4.98, 95% CI = 1.50-16.52, p = 0.009) were significantly associated with rapid progression of arterial stenosis. Furthermore, vessel wall enhancement (adjusted HR = 3.59, 95% CI = 1.33-9.70, p = 0.011) and rapid progression of arterial stenosis (adjusted HR = 4.52, 95% CI = 1.48-13.81, p = 0.008) were correlated with future stroke occurrence. The baseline presence of vessel wall enhancement was associated with rapid progression of arterial stenosis and increased risk for stroke in MMD patients. Our findings suggest that vessel wall enhancement may serve as a predictor of disease progression and poor outcomes in MMD. • The baseline presence of vessel wall enhancement was significantly associated with the rapid progression of arterial stenosis. • The baseline presence of vessel wall enhancement and rapid progression of arterial stenosis were both correlated with increased risk for future occurrence of stroke. • Our findings suggest that vessel wall enhancement may serve as a predictor of rapid progression of arterial stenosis and poor outcomes in MMD patients.