Ivy Sign Research Articles

8291 Graves’ Disease and Moyamoya Syndrome: A Complex Case of Thyroid Dysfunction and Neurovascular Abnormalities

Abstract Disclosure: A. Jamil: None. Q.V. Luong: None. A. Llorens Bonilla: None. A. Siddiqui: None. Background: Moyamoya disease is a cerebrovascular disease characterized by progressive stenosis and occlusion of cerebral arteries, particularly the internal carotid arteries, proximal anterior and middle cerebral arteries associated with a net-like collateral vessel formation. Moyamoya disease in association with certain systemic conditions (e.g. Graves’ disease, sickle cell anemia, neurofibromatosis type 1, and Down Syndrome) has been referred to as moyamoya syndrome. Graves’ disease is an autoimmune disease in which the development of thyroid-stimulating hormone (TSH) receptor autoantibodies leads to overproduction of thyroid hormones. Although the pathophysiology is unclear, Graves’ disease can adversely impact the clinical course of moyamoya syndrome.Clinical Case: A 26-year-old woman diagnosed with Graves’ disease at the age of 20 years who was in remission presented to the ED with right upper extremity weakness and headache. A stroke work-up was performed, and an initial CT head without contrast showed an acute infarct in the left temporal area. Extensive workup for hypercoagulability and cardiac causes were negative. MRI brain showed multiple infarcts of the left cerebral hemisphere with ivy sign, which are prominent linear or punctate high intensity areas in the subarachnoid space reflecting the development of collaterals that occurs in moyamoya disease. TSH at the time was noted to be <0.01 uIU/mL (range: 0.30-4.20 uIU/mL), indicating a thyrotoxic state. She was discharged on methimazole and antiplatelet therapies. However, the patient continued to have hypertension and headaches which did not resolve with the return to euthyroidism. At age 29, she again developed acute right upper and lower extremity weakness, and consequently presented to the ED. Imaging studies showed multiple punctate infarcts in her bilateral frontal lobes and bilateral ICA stenosis consistent with moyamoya disease. However, her biochemical studies showed euthyroidism. Ultimately, she underwent right frontotemporal craniotomy and direct parietal superficial temporal artery to middle cerebral artery bypass by neurosurgery to decrease her future stroke risk for her moyamoya syndrome, and her Graves’ disease remains in remission.Conclusions: The coexistence of Graves’ disease in moyamoya syndrome may suggest a pathophysiologic connection. It has been suggested that hemodynamic changes in cerebrovascular flow due to thyrotoxicosis might contribute to ischemic neurologic deficits. Surgical intervention for revascularization procedures should be attempted after optimal control of thyrotoxicosis, as this may help to prevent further ischemic events in the future and improve patient outcomes. Presentation: 6/1/2024

MRI-Based Assessment of Risk for Stroke in Moyamoya Angiopathy (MARS-MMA): An MRI-Based Scoring System for the Severity of Moyamoya Angiopathy.

Before revascularization, moyamoya patients require hemodynamic evaluation. In this study, we evaluated the scoring system Prior Infarcts, Reactivity and Angiography in Moyamoya Disease (PIRAMID). We also devised a new scoring system, MRI-Based Assessment of Risk for Stroke in Moyamoya Angiopathy (MARS-MMA), and compared the scoring systems with respect to the capability to predict impaired [15O]water PET cerebral perfusion reserve capacity (CPR). We evaluated 69 MRI, 69 DSA and 38 [15O]water PET data sets. The PIRAMID system was validated by ROC curve analysis with neurological symptomatology as a dependent variable. The components of the MARS-MMA system and their weightings were determined by binary logistic regression analysis. The comparison of PIRAMID and MARS-MMA was performed by ROC curve analysis. The PIRAMID score correlated well with the symptomatology (AUC = 0.784). The MARS-MMA system, including impaired breath-hold-fMRI, the presence of the Ivy sign and arterial wall contrast enhancement, correlated slightly better with CPR impairment than the PIRAMID system (AUC = 0.859 vs. 0.827, Akaike information criterion 140 vs. 146). For simplified clinical use, we determined three MARS-MMA grades without loss of diagnostic performance (AUC = 0.855). The entirely MRI-based MARS-MMA scoring system might be a promising tool to predict the risk of stroke.

Indications for cerebral revascularization for moyamoya syndrome in pediatric sickle cell disease determined by Delphi methodology.

Cerebral revascularization surgery (CRS) has been used to prevent stroke in children with sickle cell disease (SCD) and cerebral vasculopathy (e.g., moyamoya syndrome). While results suggest that it may be an effective treatment, surgical indications have not been well defined. This study sought to determine indications for offering revascularization surgery in centers with established sickle cell programs in the US. Three sequential surveys utilizing the Delphi methodology were administered to neurosurgeons participating in the Stroke in Sickle Cell Revascularization Surgery study. Respondents were presented with clinical scenarios of patients with SCD and varying degrees of ischemic presentation and vasculopathy, and the group's agreement to offer surgical revascularization was measured. Consensus was defined as ≥ 75% similar responses. The response rate to all 3 surveys was 100%. Seventeen neurosurgeons from 16 different centers participated. The presence of moyamoya collaterals (MMCs) and arterial stenosis matching an ischemic distribution yielded the strongest recommendations to offer surgery. There was consensus to offer revascularization in the presence of MMCs and at least 50% arterial stenosis matching an ischemic distribution. In contrast, there was no consensus to offer revascularization with 50%-70% stenosis not matching an ischemic presentation in the absence of MMCs. The presence of the ivy sign in the distribution of the stenotic artery also contributed to the consensus to offer surgery in certain scenarios. There were several clinical scenarios that attained consensus to offer surgery; the strongest was moderate to severe arterial stenosis that matched the distribution of ischemic presentation in the presence of MMCs. Radiological findings of decreased cerebral flow or perfusion also facilitated attaining consensus to offer surgery. The findings of this study reflect expert opinion about questions that deserve prospective clinical research. Determination of indications for CRS can guide clinical practice and aid the design of prospective studies.

Axial Magnetic Resonance Angiography in Evaluating Revascularization after Indirect Bypass Surgery for Moyamoya Axial Magnetic Resonance Angiography after Indirect Bypass

Introduction: At our institution, revascularization after indirect moyamoya surgery is routinely evaluated using magnetic resonance angiography (MRA) rather than catheter angiography. In this paper, we review how revascularization can be visualized on axial MRA and compare its visualization on MRA to that on catheter angiography. We also compare clinical outcomes of patients followed with routine postoperative MRA with outcomes of patients followed with routine catheter angiography. Methods: We retrospectively reviewed the records of all patients treated at our institution who underwent unilateral encephaloduroarteriosynangiosis (EDAS) and/or pial synangiosis between the ages of 1 and 21 years and between December 31, 2003, and May 1, 2021. We included patients who underwent EDAS/pial synangiosis at other hospitals as long as they met all inclusion criteria. Inclusion criteria included having a preoperative MRA within 18 months of surgery and a postoperative MRA 3−30 months after surgery. Clinical outcomes included development of postoperative stroke and transient ischemic attacks (TIAs) and changes in symptoms (improved, unchanged, or worsened), including seizures, balance issues, and headaches. Clinical outcomes were compared between patients who had routine postoperative MRA only versus those who had routine postoperative angiograms, with or without routine MRA. For each surgery, we determined the ratios of the diameters and areas of the donor vessel and the contralateral corresponding vessel, as well as the relative signal intensities of these two vessels, on preoperative and 3- to 30-month postoperative MRA. We did the same for the middle meningeal artery (MMA) ipsilateral to the donor artery and the contralateral MMA. We assessed changes from pre- to post-operation in diameter ratios, area ratios, relative signal intensity, ivy sign, and in brain perfusion on arterial spin labeled (ASL) imaging. MRI and MRA measures of revascularization and flow were compared to Matsushima grades in patients who had postoperative catheter angiograms. Results: Fifty-one operations for 42 unique patients were included. There were no significant differences in the rates of postoperative strokes, postoperative TIAs, changes in symptoms, or new symptoms after surgeries evaluated by routine postoperative MRA versus catheter angiogram (p = 0.282, 1, 0.664, and 0.727, respectively). There were significant associations between greater collateralization on postoperative MRA and greater median increases in preoperative-to-postoperative ratios of donor-vessel-over-contralateral-vessel diameter (p = 0.0461), ipsilateral-MMA-over-contralateral-MMA diameter (p = 0.0135), and the summed donor and ipsilateral MMA diameters over the summed contralateral vessel diameters (p < 0.001). The median increase in the ratio of the donor vessel and contralateral corresponding vessel diameters was significantly higher for Matsushima grade A versus B (p = 0.036). The median increase in the ratio of the sum of donor and ipsilateral MMA diameters over the sum of the contralateral vessel diameters was significantly higher for improved-versus-unchanged perfusion on ASL imaging (p = 0.0074). There was a nonsignificant association between greater postoperative collateralization on MRA and Matsushima grade (p = 0.1160). Conclusion: Cerebral revascularization after EDAS and pial synangiosis can be evaluated on axial MRA by comparing the diameter and/or signal intensity of the donor vessel and corresponding contralateral vessel, as well as the ipsilateral and contralateral MMA, on postoperative-versus-preoperative MRA. The use of routine postoperative MRA rather than catheter angiography does not appear to negatively affect outcomes.

Indirect revascularization for pediatric moyamoya disease.

Indirect revascularization is a common and effective treatment for pediatric moyamoya disease. However, in several cases postoperative angiogenesis is not sufficient. It is not fully understood which factors are involved in the development of postoperative collateral circulation. In this study, the authors aimed to elucidate the factors related to postoperative angiogenesis in indirect revascularization. Among the patients who underwent indirect revascularization for moyamoya disease from January 2015 to December 2022, those whose angiogenesis was evaluated using angiography were included. Age, onset symptoms, comorbidities, preoperative imaging findings, surgical details, perioperative complications, postoperative imaging findings, and modified Rankin Scale (mRS) score at the last outpatient visit were retrospectively examined. Ninety cases (53 patients; 37 bilateral, 16 unilateral) were included. Sixty-eight cases (75.6%) were symptomatic. The mean age at surgery was 7.9 years, and the mean postoperative follow-up duration was 48.5 months. Frontotemporal encephalo-duro-arterio-synangiosis (EDAS) was performed in all cases, and simultaneous frontal encephalo-galeo-synangiosis (EGS) was performed in 34 cases. Postoperative angiography revealed insufficient angiogenesis in 14 of 90 cases (15.6%) after frontotemporal EDAS and in 8 of 34 cases (23.5%) after frontal EGS. A high degree of ivy sign depicted on preoperative MRI was found to be significantly correlated with good angiogenesis after both surgical procedures (p = 0.00030 for EDAS and p = 0.0039 for frontal EGS). In addition, an advanced preoperative Suzuki stage was significantly correlated with good postoperative angiogenesis after EDAS (p = 0.00040). Good angiogenesis was significantly correlated with postoperative improvement of the ivy sign in both procedures (p = 0.0005 in EDAS and p = 0.030 in frontal EGS) as well as correlated with a better mRS score at long-term follow-up after EDAS (p = 0.018). Preoperative ivy sign and Suzuki classification are related to the degree of angiogenesis achieved after indirect revascularization for pediatric moyamoya disease.

Abstract WP127: Diagnostic Value of the Ivy Sign Compared to MR With Cerebrovascular Reactivity in Decompensated Moyamoya Arteriopathy

Introduction: MR cerebrovascular reactivity (CVR) mapping during rapid manipulation of end-tidal pCO2 is used to assess hemodynamic compromise in patients with Moyamoya arteriopathy (MMA). Leptomeningeal ivy sign on MRI FLAIR sequence may indicate regions of decreased perfusion, which would be an inexpensive and easy way to screen for hemodynamic compromise. The purpose of this study was to examine the diagnostic value of the ivy sign compared to MR CVR. Methods: For this retrospective cross-sectional study, we included consecutive patients with uncompensated MMA who were eligible for extracranial-intracranial bypass surgery and completed a preoperative MRI FLAIR with CVR study. Patients with incomplete or artefact degraded imaging were excluded. Two neuroradiologists who were blinded for the CVR result independently assessed the degree of ivy sign on axial 2D FLAIR imaging as grade 0, 1 or 2 in 8 predefined brain regions. Two different readers, blinded for ivy sign, independently assessed the presence or absence of steal in the same regions on MR CVR. Consensus was reached for discordant findings. We assessed interrater agreement using weighted kappa followed by diagnostic accuracy measures for the ivy sign using MR CVR as a reference. Results: We enrolled 73 patients (41/73 (56.1%) female; mean age 43). 47/73 (64%) patients had bilateral MMA with on average 3 brain regions with abnormal CVR. Interrater agreement was good for ivy sign (weighted kappa 0.63 (95%CI 0.54-0.71)) and excellent for CVR (kappa 0.95 (95% CI 0.87-1.00)). For a total of 156/584 (26.7%) brain regions, there was agreement between a positive ivy sign and the presence of steal on CVR and 240/584 (41%) brain regions had no ivy sign and normal CVR. The sensitivity of the ivy sign was 64.5%, specificity 70.2%, positive predictive value 60.5% and negative predictive value 73.6% with an AUC of 0.68 (95% CI 0.64-0.72) Conclusions: In this preselected cohort with high pretest probability for hemodynamic compromise, the diagnostic accuracy of the ivy sign compared to MR CVR was limited and needs to be used with caution to determine haemodynamic status in MMA.

The ivy sign as a radiological marker for follow-up of postoperative cerebral perfusion status in adult moyamoya disease.

Little is known about the relationship between postoperative changes in cerebral perfusion and the ivy sign representing leptomeningeal collateral burden in moyamoya disease (MMD). This study aimed to investigate the usefulness of the ivy sign in evaluating cerebral perfusion status following bypass surgery in patients with adult MMD. Two hundred thirty-three hemispheres in 192 patients with adult MMD undergoing combined bypass between 2010 and 2018 were retrospectively enrolled. The ivy sign was represented as the ivy score on FLAIR MRI in each territory of the anterior, middle, and posterior cerebral arteries. Ivy scores, as well as clinical and hemodynamic states on SPECT, were semiquantitatively compared both preoperatively and at 6 months after surgery. Clinical status improved at 6 months after surgery (p < 0.01). On average, ivy scores in whole and individual territories were decreased at 6 months (all p values < 0.01). Cerebral blood flow (CBF) postoperatively improved in three individual vascular territories (all p values ≤ 0.03) except for the posterior cerebral artery territory (PCAt), and cerebrovascular reserve (CVR) improved in those areas (all p values ≤ 0.04) except for the PCAt. Postoperative changes in ivy scores and CBF were inversely correlated in all territories (p ≤ 0.02), except for the PCAt. Furthermore, changes in ivy scores and CVR were only correlated in the posterior half of the middle cerebral artery territory (p = 0.01). The ivy sign was significantly decreased after bypass surgery, which was well correlated with postoperative hemodynamic improvement in the anterior circulation territories. The ivy sign is believed to be a useful radiological marker for postoperative follow-up of cerebral perfusion status.

Ivy Sign: Usefulness in Diagnosis and Prognosis Prediction of Moyamoya Disease

BackgroundMoyamoya disease can not be found commonly as a rare type compared to other vascular disease, such as aneurysm. However, it can not be ignored for its high fatality and disability rate. In addition, exact pathogenesis study of this disease is still on the way. The ivy sign is always observed in Moyamoya disease (MMD), but the clinical importance of this sign in MMD isn’t clearly known. The main purpose of this research was to specifically investigate the clinical significance. MethodsThe patients with Moyamoya disease were hospitalized from January 2016 to 2020, it’s a retrospective cohort study to gather the baseline clinical and imaging study. In the analysis, univariate and multivariate logistic regression was utilized to testify whether ivy sign was independently associated with MMD characteristics, including cerebrovascular morphology, cerebral hemodynamics, cerebrovascular events and postoperative collateral formation (PCF). Results156 patients were included with 312 hemispheres. AS for the result of multivariate logistic regression analysis, we could discover a fact that ivy sign was tightly connected to the Suzuki stage ≥IV(OR, 1.386; 95%CI, 1.055 - 1.822; p = 0.019), CBF (cerebral blood flow) decreased type (OR, 2.330; 95%CI, 1.733 - 3.133; p = 0.000), age acted as a protective factor for CBF(OR, 0.966; 95%CI, 0.946 - 0.986; p = 0.001), the elder was more likely associated with decreased CBF. ivy sign also played an significant role in Ischemic cerebrovascular events (OR, 5.653; 95%CI, 3.092 - 10.336; p = 0.003), their remarkable connection could be seen on the study. We could also find that ivy was closely connected to the good postoperative collateral formation (PCF) (OR, 2.830; 95%CI, 1.329 - 6.027; p = 0.007), we couldn’t ignore that age was associated with good PCF as well (OR, 0.933; 95%CI, 0.882 - 0.987; p = 0.015). DiscussionWe could be more aware of the connection between ivy sign and Moyamoya disease form this study, to implement on diagnosis, treatment, prognosis more efficiently.

Recurrent Stroke in Young Adult as a Manifestation of Moyamoya Disease: Case Report

Background: Moyamoya disease is a chronic cerebrovascular disorder characterized by progressive narrowing of the intracranial portions of the distal internal carotid artery, the initial proximal components of the middle cerebral artery, anterior cerebral artery, and posterior cerebral artery. Moyamoya disease can manifest as a stroke in young adult. Methods: This report presents a young female patient with Moyamoya disease in Siloam Hospital Denpasar, Bali, Indonesia, diagnosed using MRI and Digital Subtraction Angiography. Case Report: A 29-year-old woman with recurrent stroke, presented with right-side hemiparesis, urinary incontinence, disinhibition syndrome, hypertension, and a history of left hemiparesis from a previous stroke. The MRI brain showed hyperintensity throughout the sulci (ivy sign) in the bilateral frontoparietal with acute infarct in the left frontomedial region, chronic infarct in right frontomedial, occlusion of bilateral carotid arteries (supraclinoid segment), bilateral MCA and ACA. The cerebral DSA study showed stenosis in the right ICA, stenosis in the M1-M2 junction with poor collateral to ACA territory with a puff of smoke appearance, occlusion in the left ICA (supraclinoid segment), and severe stenosis in M1. The patient had undergone direct bypass in the right MCA and right superficial temporal artery. Unfortunately, the bypass did not succeed because of infection of the bypass site and the patient got oral medication with a single antiplatelet (asetosal) and angiotensin receptor blocker (valsartan). Discussion: Moyamoya disease is a rare chronic cerebrovascular disorder that can manifest as an ischaemic stroke in young adult. This patient had a recurrent ischaemic stroke due to Moyamoya disease. According to a global multicenter stroke database, 90% of Moyamoya disease patients initially presented with an ischemic stroke, 7.5% with a transient ischemic attack, and 2.5% with a hemorrhagic stroke. DSA showed that the intracranial collateral system failed to fulfill the demand for brain circulation when the stroke occurred. The patient had failure to grow collateral after direct bypass surgery from the right superficial temporal artery to MCA and single oral antiplatelet as a secondary prevention for ischaemic stroke. Conclusion: Moyamoya disease is a rare disease that needs further studies for its treatment to halt the progression and prevent secondary complications. Young patients with ischaemic stroke needed to be asses with brain MRI, MRA, and DSA to evaluate the etiology of ischaemic stroke. Keywords: Moyamoya disease, Digital Substraction Angiography,ischaemic stroke

Development of Ivy Sign and Infarction in the Lateral Part of the Hemisphere or the Middle Cerebral Artery Territory in Association with Steno-occlusive Involvement of the Posterior Cerebral Artery in Moyamoya Disease.

Development of Ivy Sign and Infarction in the Lateral Part of the Hemisphere or the Middle Cerebral Artery Territory in Association with Steno-occlusive Involvement of the Posterior Cerebral Artery in Moyamoya Disease.

Disappearance of hemichorea as the initial presentation of moyamoya disease after revascularization surgery regardless of residual hypoperfusion evaluated by quantitative N-isopropyl-p-123I-iodoamphetamine SPECT

Background and importanceAlthough hypoperfusion of the basal ganglia or the frontal subcortical matter is suspected, the pathology of chorea in moyamoya disease remains unclarified. Herein, we report a case of moyamoya disease presenting with hemichorea and evaluate pre- and postoperative perfusion using single photon emission computed tomography with N-isopropyl-p-123I-iodoamphetamine (123I-IMP SPECT). Clinical presentationAn 18-year-old woman presented with choreic movement of her left limbs. Magnetic resonance imaging revealed an ivy sign, and 123I-IMP SPECT demonstrated decreased cerebral blood flow (CBF) and cerebral vascular reserve (CVR) values in the right hemisphere. The patient underwent direct and indirect revascularization surgery to improve cerebral hemodynamic impairment. The choreic movements entirely resolved immediately after surgery. Although CBF and CVR values in the ipsilateral hemisphere demonstrated by quantitative SPECT increased, these did not reach the normal values threshold. ConclusionChoreic movement in moyamoya disease may be related to cerebral hemodynamic impairment. Further studies are required to elucidate its pathophysiological mechanisms.

Asymptomatic Moyamoya Disease: An experience from Eastern India (P14-5.011)

<h3>Objective:</h3> <i>This study was undertaken to clarify the clinic-radiological characteristics and prospective follow-up of apparently “asymptomatic” MMA, if it is truly asymptomatic or not.</i> <h3>Background:</h3> <i>“Asymptomatic” Moyamoya Angiopathy (MMA) has been defined as absence of ischemic or hemorrhagic episodes in MMA patients. An ambiguous definition has led to its variable adaptation in the limited studies in the past.</i> <h3>Design/Methods:</h3> <i>Of the 122 angiographically proven MMA in our center over last 6 years, 6 patients had an initial diagnosis of “asymptomatic” MMA following evaluation by atleast one post-graduate doctor. These 6 cases were then further scrutinized by 3 different neurologists for epidemiological, clinical, radiological characteristics and subsequent follow-up. Data were analyzed using descriptive statistics.</i> <h3>Results:</h3> <i>The mean age at diagnosis was 23.7±13.14 years. 3 of the 6 patients had undergone brain imaging for evaluation of non-migraine-like headache, 1 for dizziness, 2 as a part of familial screening for MMA. 4 of the 6 patients had specific triggers for aggravation of symptoms. Brain imaging revealed old vascular insults and ivy sign in 5 of 6 patients(83.3%), cerebral microbleeds in 1 of 6 patients(16.7%), and mean suzuki staging3.6±0.82. 4 of the 6 patients who underwent cerebral perfusion study, all having hypoperfusion. Revascularization surgery was done in 2 of the 6 patients, rest were managed conservatively. None of the patients had any new onset neurological deficit or radiological progression over a mean follow-up period of 22.3±20.22 months.</i> <h3>Conclusions:</h3> <i>Subtle “paroxysmal events” often precipitated by specific triggers might be indicative of transient cerebral hypoperfusion in an apparently “asymptomatic” MMA and are likely indicative of ischemic symptoms, and warrants for a more precise definition to avoid misclassification of “asymptomatic” MMA.</i> <b>Disclosure:</b> Dr. Das has nothing to disclose. Souvik Dubey has nothing to disclose. Dr. Ray has nothing to disclose.

304 Correlation of Preoperative Suzuki Stage, Postoperative Matsushima Grade, and Clinical Outcomes in Surgically Treated Pediatric Moyamoya

INTRODUCTION: Moyamoya disease and syndrome among pediatric patients is often treated with surgical revascularization. There is limited literature regarding associations between preoperative angiography, postoperative angiography, and clinical outcomes. METHODS: We report a case series of consecutive moyamoya disease (MMD) and moyamoya syndrome (MMS) pediatric patients who underwent pial synangiosis. Correlations between preoperative Suzuki stage, Matsushima grade at one year postoperatively, and pediatric modified Rankin score (mRS) were assessed using Speakman’s Rank correlation testing. Factors associated with angiographic and clinical outcomes were analyzed using Fisher’s Exact tests. RESULTS: 40 patients (60 hemispheres) were treated with median follow up 2.29 years. No significant correlation between preoperative Suzuki stage and postoperative Matsushima grade was identified overall. Suzuki stage two hemispheres developed less robust revascularization. Preoperative Suzuki stage was not associated with postoperative disability severity whereas a nonsignificant trend towards improved clinical outcomes was observed with better Matsushima grade. Poor angiographic revascularization was more frequent in MMS patients, Sickle Cell Disease (SCD) patients, patients with preoperative ivy sign, preoperative NIHSS ≥3, and preoperative symptom duration more than 6 months. Postoperative intracranial hemorrhage, reoperation, Suzuki stage ≥5, and postoperative ischemic stroke conferred more unfavorable clinical outcomes whereas Matsushima A and B revascularization and symptom duration &lt;6 months were protective from unfavorable outcomes. CONCLUSIONS: Although Suzuki stage does not significantly correlate with Matsushima grade, the degree of revascularization according to Matsushima grade may be associated with clinical outcomes. Multiple factors associated with degree of revascularization and clinical outcomes are identified.

Development of Ivy Sign and Infarction in the Lateral Part of the Hemisphere or the Middle Cerebral Artery Territory in Association with Steno-occlusive Involvement of the Posterior Cerebral Artery in Moyamoya Disease.

Development of Ivy Sign and Infarction in the Lateral Part of the Hemisphere or the Middle Cerebral Artery Territory in Association with Steno-occlusive Involvement of the Posterior Cerebral Artery in Moyamoya Disease.

Ivy sign: a diagnostic and prognostic biomarker for pediatric moyamoya.

Ivy sign is a radiographic finding on FLAIR MRI sequences and is associated with slow cortical blood flow in moyamoya. Limited data exist on the utility of the ivy sign as a diagnostic and prognostic tool in pediatric patients, particularly outside of Asian populations. The authors aimed to investigate a modified grading scale with which to characterize the prevalence and extent of the ivy sign in children with moyamoya and evaluate its efficacy as a biomarker in predicting postoperative outcomes, including stroke risk. Pre- and postoperative clinical and radiographic data of all pediatric patients (21 years of age or younger) who underwent surgery for moyamoya disease or moyamoya syndrome at two major tertiary referral centers in the US and Israel, between July 2009 and August 2019, were retrospectively reviewed. Ivy sign scores were correlated to Suzuki stage, Matsushima grade, and postoperative stroke rate to quantify the diagnostic and prognostic utility of ivy sign. A total of 171 hemispheres in 107 patients were included. The median age at the time of surgery was 9 years (range 3 months-21 years). The ivy sign was most frequently encountered in association with Suzuki stage III or IV disease in all vascular territories, including the anterior cerebral artery (53.7%), middle cerebral artery (56.3%), and posterior cerebral artery (47.5%) territories. Following surgical revascularization, 85% of hemispheres with Matsushima grade A demonstrated a concomitant, statistically significant reduction in ivy sign scores (OR 5.3, 95% CI 1.4-20.0; p = 0.013). Postoperatively, revascularized hemispheres that exhibited ivy sign score decreases had significantly lower rates of postoperative stroke (3.4%) compared with hemispheres that demonstrated no reversal of the ivy sign (16.1%) (OR 5.5, 95% CI 1.5-21.0; p = 0.008). This is the largest study to date that focuses on the role of the ivy sign in pediatric moyamoya. These data demonstrate that the ivy sign was present in approximately half the pediatric patients with moyamoya with Suzuki stage III or IV disease, when blood flow was most unstable. The authors found that reversal of the ivy sign provided both radiographic and clinical utility as a prognostic biomarker postoperatively, given the statistically significant association with both better Matsushima grades and a fivefold reduction in postoperative stroke rates. These findings can help inform clinical decision-making, and they have particular value in the pediatric population, as the ability to minimize additional radiographic evaluations and tailor radiographic surveillance is requisite.

“Asymptomatic” Moyamoya Angiopathy: Is it Truly Asymptomatic?

IntroductionAn ambiguous definition of “asymptomatic” Moyamoya Angiopathy(aMMA) of absence of ischemic/hemorrhagic episodes in MMA patients, has led to its variable adaptation in the limited past-studies. ObjectiveTo observe the clinic-radiological characteristics and prospective follow-up of apparently “asymptomatic” MMA, and to determine if it is truly asymptomatic or not. Materials and MethodsAn observation, cohort study of 122 angiographically proven MMA over 6 years was undertaken from a single, tertiary-care-center to observe the clinico-radiological characteristics, prospective follow-up of apparently aMMA. Amongst them, 6 had an initial diagnosis of aMMA following evaluation by atleast one post-graduate doctor, which were further scrutinized by 3 different neurologists for epidemiological, clinical, radiological characteristics and subsequent follow-up. Data were analyzed using descriptive statistics. ResultsMean age was 23.7 ± 13.14 years. 3 of 6 underwent brain-imaging for evaluation of non-migraine-like headache, 1 for dizziness, 2 as part of familial screening for MMA. 4 of 6 patients had specific-triggers for aggravation of symptoms. Brain-imaging revealed old vascular insults and ivy sign in 5 of 6 each (83.3%), mean suzuki staging was 3.6±0.82. 4 of 6 underwent cerebral perfusion study, all had hypoperfusion. Revascularization surgery was done in 2 of 6, rest were managed conservatively. None had any new-onset neurological deficit or radiological progression over a mean follow-up period of 22.3 ± 20.22 months. ConclusionsApparently aMMA may not be truly asymptomatic and often have subtle “paroxysmal events” precipitated by specific-triggers, indicative of transient ischemic symptoms. Thus, warrants for a more precise definition to avoid misclassification of aMMA.

The Ivy Sign in Adult MoyaMoya Disease

The Ivy Sign in Adult MoyaMoya Disease

The Ivy Sign in Adult MoyaMoya Disease

The Ivy Sign in Adult MoyaMoya Disease

Abstract TP173: Neuroimaging Biomarkers Of Moyamoya Arteriopathy By Age In A Surgical Cohort

Introduction: Moyamoya arteriopathy (MMA) is a significant risk factor for pediatric stroke. Radiographic signs thought to indicate disease severity guide clinical decision making. We hypothesized that there exists a correlation between age and imaging biomarkers. Methods: We retrospectively identified pediatric MMA patients seen at our center that underwent surgical revascularization. Preoperative MRI/MRA imaging was reviewed by two pediatric neuroradiologists who were blinded to clinical characteristics. The following neuroimaging markers were evaluated by consensus: Suzuki staging, cerebrovascular stenosis score (CVSS), the presence of posterior circulation steno-occlusive lesions, ivy sign, and brush sign. Associations of these features with age were analyzed using simple logistic regression and Spearman correlation analyses. Results: We identified 27 patients undergoing 30 individual revascularization procedures between January 2003 and June 2021. There were 29 images of diagnostic quality for analysis. Median age at imaging was 8.5 years. Younger age was associated with posterior arterial lesions (p=0.028). There was no statistically significant association between age and ivy sign (p=0.205), brush sign (p=0.156), Suzuki stage (p=0.671), or CVSS (0.234). Conclusions: In this cohort of surgical MMA patients, younger children were more likely to have posterior circulation involvement on MRA. While not statistically significant, all analyzed radiographic signs were more prevalent among younger patients. Further studies correlating neuroimaging biomarkers with clinical outcomes in larger cohorts including non-surgical MMA patients are needed.

Idiopathic Moyamoya Disease Presenting as Isolated Hemichorea

AbstractThe study aimed to describe a case of a 7-year-old Caucasian girl who developed isolated chorea in her right upper and lower extremities progressively increasing over 2 years. This study is a case report and conducted at tertiary care center. A 7-year-old Caucasian girl who gradually developed worsening choreiform movements in her right upper and lower extremities over the course of 2 years until medical attention was sought. Literature review of children presenting with chorea as the only manifestation in moyamoya disease. A 7-year-old right-handed Caucasian girl presented with progressively worsening choreiform movements in her right upper and lower extremities affecting her fine motor skills and gait impairment There was no weakness, hyperreflexia, or spasticity on her neurological exam. Neuroimaging studies showed “ivy sign,” asymmetric prominence of vessels within the subarachnoid spaces overlying the left cerebral hemisphere with corresponding serpiginous T2 fluid-attenuated inversion recovery abnormality, but no parenchymal volume loss or diffusion restriction. Magnetic resonance angiography showed focal moderate-to-severe stenosis at the junction of the paraophthalmic and supraclinoid segments of the left internal carotid artery, with poststenotic dilatation. Angiography demonstrated focal stenosis involving the ophthalmic segment of the left internal carotid artery with 50% stenosis. There was compensation through a medium sized left posterior communicating artery as well as posterior cerebral artery to middle cerebral artery and anterior cerebral artery collateralization. Brain single-photon emission computerized tomography scan showed no evidence of perfusion defects in the cerebral hemispheres, basal ganglia, or thalami. She underwent successful revascularization procedure (left pial synangiosis) with resolution of her choreiform movements and normalization of her gait. Our case demonstrates that moyamoya disease should be suspected when evaluating a child with hemichorea and describes resolution of symptoms after revascularization surgery. Neuroimaging and vascular studies should be obtained in children with unilateral movement disorder.