Illness uncertainty, resilience, and perceived social support among patients with moyamoya disease: a cross-sectional study.

Objective: The present study aims to investigate the levels of illness uncertainty in patients with moyamoya disease and to determine the association of socio-demographic characteristics, perceived social support and resilience with illness uncertainty in patients with moyamoya disease. Method: A cross-sectional survey using convenience sampling was conducted in two hospitals in China from August to December 2023. A socio-demographic characteristics questionnaire, the Chinese versions of Mishel's Unsurety in Disease Scale (MUIS), the Chinese version of Connor-Davidson Resilience Scale (CD-RISC), and the Chinese version of Multidimensional Scale of Perceived Social Support (MSPSS) were used to perform this research. The collected data were analyzed using SPSS 24.0 statistical software. The t-test, one-way analysis of variance (ANOVA), pearson correlation analysis and hierarchical regression analysis were used to identify associated factors. Result: A total of 263 patients with moyamoya disease were recruited in this survey. The score of illness uncertainty was at a moderate level of (100.03 ± 18.59). The present study identified a negative correlation between illness uncertainty with resilience perceived social support. Hierarchical regression analysis showed that gender, occupation, education level, resilience and perceived social support were the related factors of illness uncertainty. Conclusion: Patients with moyamoya disease experienced moderate disease uncertainty on average, which was related to gender, occupation, education level, resilience and perceived social support. Future research is needed to better explore the complex relationships between illness uncertainty, resilience, and perceived social support with different types of moyamoya disease using longitudinal research.

Influence of an improvement in frontal lobe hemodynamics on neurocognitive function in adult patients with moyamoya disease.

BACKGROUND: In adults, moyamoya disease (MMD) often presents with slight neurocognitive impairment, which may result from frontal lobe hemodynamic insufficiency. METHODS: In this study, we performed revascularization surgery by superficial temporal artery-anterior cerebral artery (ACA) direct bypass in 20 adults with MMD with poor anterograde ACA flow (Group M). The pre- and postoperative neurocognitive test results of these patients were retrospectively analyzed. The comparative group (Group C) included 23 patients with unruptured aneurysms or brain tumors who underwent craniotomy, as well as the same neurocognitive tests as Group M. We calculated the compositive frontal lobe function index (CFFI) based on the results of seven neurocognitive tests for each patient, and the difference between the pre- and postoperative CFFI values (CFFI Post - Pre) was compared between the two groups. RESULTS: Frontal perfusion improved postoperatively in all patients in Group M. The CFFI Post - Pre was significantly higher in Group M than in Group C (0.23 ± 0.44 vs. - 0.20 ± 0.32; p < 0.001). After adjusting for postoperative age, sex, preoperative non-verbal intelligence quotient, and preoperative period of stress, Group M had a significantly higher CFFI Post - Pre than Group C in the multiple regression analysis (t value = 4.01; p < 0.001). CONCLUSION: Improving frontal lobe hemodynamics might be the key for improving neurocognitive dysfunction in adults with MMD. The surgical indication and method should be considered from the perspective of both stroke prevention and neurocognitive improvement or protection.

Post-infectious Moyamoya Syndrome: A Review of Existing Scientific Literature From 2000 to 2023.

Moyamoya disease (MMD) is considered a primary disorder of an unknown etiology. In contrast, Moyamoya syndrome (MMS) refers to MMD associated with other underlying diseases, such as meningitis in childhood, neurofibromatosis type II, Down syndrome, cranial irradiation, and different types of anemias, particularly hemoglobinopathies. We aimed to provide a comprehensive clinicopathological overview of MMS. All case reports and case series published from 2000 to 2023 pertaining to MMD were included in the study. Case studies, original articles, editorials, letters to editors, and clinical images were excluded. The search was conducted using the Boolean operators ("AND" and "OR") on PubMed and Google Scholar. A total of 13 case reports and one case series study were included. The study suggests infection might be a trigger in susceptible individuals. The autoimmune antibody findings (anti-double stranded DNA IgG) suggest a potential autoimmune component in some cases. There were diverse presentations and outcomes of post-infectious MMS, with a striking predominance of pediatric cases (66.66%) and a possible female predominance. Both computerized tomography (CT) and magnetic resonance imaging (MRI) showed evidence of restricted blood flow. CT showed that stenosis, occlusion, and collateral formation were frequent vascular findings, but often unspecified in severity. Infarction, hypodensities, and hematoma were the most common parenchymal findings (22.22% each). The findings on MRI were stenosis (50%) and collateral formation (44.44%). Infarction was the most common finding (66.66%) in parenchyma. Hydrocephalus, encephalomalacia, and atrophy were less frequent. Lesions were most frequent in the internal carotid artery (66.66%), middle cerebral artery (66.66%), and anterior cerebral artery (50%). Lesions were less frequent in the posterior cerebral, vertebral, and basilar arteries. The frontal lobe (38.89%) and basal ganglia (33.33%) were commonly affected parenchymal regions. The most common risk factor was human immunodeficiency virus (HIV) infection (50%), followed by trisomy 21, cryptococcal, and other types of meningitides. Aspirin (50%) and antiretroviral therapy (38.89%) were the cornerstones of treatment for MMS. This review accentuates the noteworthy obstacles presented by post-infectious MMS, especially its catastrophic effect on children and its correlation with HIV/AIDS. According to our elaborate literature search using PubMed and Google Scholar, this is the first narrative review in the existing scientific literature summarizing the literature on post-infectious MMS.

Contrast enhancement patterns associated with acute stroke in moyamoya disease using MR vessel wall imaging.

BACKGROUND AND PURPOSE: High-resolution magnetic resonance imaging (HR-MRI) can provide valuable insights into the histopathological characteristics of moyamoya disease (MMD). However, the patterns of vessel wall contrast enhancement have not been well established. We aimed to identify the contrast enhancement patterns of the vessel walls associated with acute cerebral infarction using HR-MRI in MMD. METHODS: In this retrospective study, we conducted genetic tests for Ring Finger Protein 213 (RNF 213) and performed HR-MRI on patients suspected of having MMD. We analyzed wall enhancement patterns including concentric, eccentric, or mixed enhancement types, and the occurrence of acute cerebral infarction in patients who simultaneously tested positive for RNF 213 and exhibited definite features of MMD on HR-MRI. RESULTS: Among 306 patients who underwent RNF 213 tests for the evaluation of MMD, 56 showed positive RNF 213, and HR-MRI was performed on 32 of them. Among the patients with acute cerebral infarction, the incidence rate was significantly higher in the group with concentric wall enhancement compared to patients without acute cerebral infarction (73.3% vs. 17.0%, p < .002). Furthermore, the incidence was notably elevated, even in patients with pure concentric wall enhancement (40.0% vs. 5.9%, p = .033). The area under the curve (AUC) for the group with any concentric wall enhancement showed a significant result of .78 (95% confidence interval [CI]: .61-.95, p = .007), whereas the predictive ability for pure concentric wall enhancement did not reach significance (AUC = .67, 95% CI: .48-.86, p = .100). CONCLUSIONS: Concentric wall enhancement was a significant predictor of acute cerebral infarction in patients with MMD.

Magnetic Resonance Angiography Alone Is Insufficient for Diagnosis and Surgical Planning in Children With Moyamoya.

BACKGROUND: The gold standard for evaluation of the severity of moyamoya vasculopathy is the Suzuki grade determined with cerebral catheter angiography (CA). With greater use of magnetic resonance angiography (MRA) it is important to understand if MRA is truly comparable to CA. METHODS: Children with moyamoya were evaluated using the Suzuki score for CA and the modified MRA six-stage Suzuki score to describe the angiographic findings in moyamoya from initial narrowing of the distal internal carotid artery to the "puff of smoke" appearance of the lenticulostriate collaterals and finally to the disappearance of this network of collaterals. Using Cohen kappa we compared Suzuki grade based on CA with MRA in the same patients. RESULTS: A total of 27 children with moyamoya were reviewed. We calculated a weighted Cohen kappa of 0.49 (P < 0.0001), which is a moderate correlation. CONCLUSIONS: We suggest caution in the reliance on MRA for the diagnosis and evaluation of severity of moyamoya in children.

Ultrasonographic Predictors for Post-operative Ischemic Events After Indirect Revascularization Surgeries in Patients with Moyamoya Disease.

OBJECTIVE: Recurrent stroke after revascularization surgeries predicts poor outcome in patients with moyamoya disease (MMD). Early identification of patients with stroke risk paves the way for rescue intervention. This study aimed to investigate the role of ultrasound in identifying patients at risk of post-operative ischemic events (PIEs). METHODS: This prospective study enrolled patients with symptomatic MMD who underwent indirect revascularization surgeries. Ultrasound examinations were performed preoperatively and at 3 mo post-operatively to evaluate the hemodynamic changes in extracranial and intracranial arteries on the operated side. PIE was defined as ischemic stroke or transient ischemic attack in the operated hemisphere within 1 y. The areas under receiver operating characteristic curves were compared between models for prediction of PIE. RESULTS: A total of 56 operated hemispheres from 36 patients (mean age, 23.0 ± 18.5 y) were enrolled in this study, and 27% developed PIE. In multivariate logistic regression models, PIE was associated with lower end-diastolic velocity and flow volume (FV) of the ipsilateral external carotid artery (ECA), and lower FV of ipsilateral superficial temporal artery and occipital artery at 3 mo post-operatively (all p < 0.05). Moreover, the post-operative FV of the ipsilateral ECA was the only one factor that significantly increased the areas under receiver operating characteristic curves from 0.727 to 0.932 when adding to a clinical-angiographic model for prediction of PIE (p = 0.017). This parameter was significantly lower in hemispheres with PIE, both in adult and pediatric patients. CONCLUSION: After indirect revascularization, surgeries in patients with symptomatic MMD, FV of ipsilateral ECA at 3 mo helps clinicians to identify patients at risk of PIE.

Novel analysis of functional relationship linking moyamoya disease to moyamoya syndrome.

Objective: The aim of this study was to elucidate the genetic pathways associated with Moyamoya disease (MMD) and Moyamoya syndrome (MMS), compare the functional activities, and validate relevant related genes in an independent dataset. Methods: We conducted a comprehensive search for genetic studies on MMD and MMS across multiple databases and identified related genes. Gene Ontology (GO) and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway enrichments analyses were performed for these genes. Commonly shared genes were selected for further validation in the independent dataset, GSE189993. The Sangerbox platform was used to perform statistical analysis and visualize the results. P＜0.05 indicated a statistically significant result. Results: We included 52 MMD and 51 MMS-related publications and identified 126 and 51 relevant genes, respectively. GO analysis for MMD showed significant enrichment in cytokine activity, cell membrane receptors, enzyme binding, and immune activity. A broader range of terms was enriched for MMS. KEGG pathway analysis for MMD highlighted immune and cellular activities and pathways related to MMS prominently featured inflammation and metabolic disorders. Notably, nine overlapping genes were identified and validated. The expressions of RNF213, PTPN11, and MTHFR demonstrated significant differences in GSE189993. A combined receiver operating characteristic curve showed high diagnostic accuracy (AUC = 0.918). Conclusions: The findings indicate a close relationship of MMD with immune activity and MMS with inflammation, metabolic processes and other environmental factors in a given genetic background. Differentiating between MMD and MMS can enhance the understanding of their pathophysiology and inform the strategies for their diagnoses and treatment.

Assessing superficial temporal artery-middle cerebral artery anastomosis patency using FLOW 800 hemodynamics.

OBJECTIVE: The objective of this study was to investigate the use of indocyanine green videoangiography with FLOW 800 hemodynamic parameters intraoperatively during superficial temporal artery-middle cerebral artery (STA-MCA) bypass surgery to predict patency prior to anastomosis performance. METHODS: A retrospective and exploratory data analysis was conducted using FLOW 800 software prior to anastomosis to assess four regions of interest (ROIs; proximal and distal recipients and adjacent and remote gyri) for four hemodynamic parameters (speed, delay, rise time, and time to peak). Medical records were used to classify patients into flow and no-flow groups based on immediate or perioperative anastomosis patency. Hemodynamic parameters were compared using univariate and multivariate analyses. Principal component analysis was used to identify high risk of no flow (HRnf) and low risk of no flow (LRnf) groups, correlated with prospective angiographic follow-ups. Machine learning models were fitted to predict patency using FLOW 800 features, and the a posteriori effect of complication risk of those features was computed. RESULTS: A total of 39 cases underwent STA-MCA bypass surgery with complete FLOW 800 data collection. Thirty-five cases demonstrated flow after anastomosis revascularization and were compared with 4 cases with no flow after revascularization. Proximal and distal recipient speeds were significantly different between the no-flow and flow groups (proximal: 238.3 ± 120.8 and 138.5 ± 93.6, respectively [p < 0.001]; distal: 241.0 ± 117.0 and 142.1 ± 103.8, respectively [p < 0.05]). Based on principal component analysis, the HRnf group (n = 10) was characterized by high-flow speed (> 75th percentile) in all ROIs, whereas the LRnf group (n = 10) had contrasting patterns. In prospective long-term follow-up, 6 of 9 cases in the HRnf group, including the original no-flow cases, had no or low flow, whereas 8 of 8 cases in the LRnf group maintained robust flow. Machine learning models predicted patency failure with a mean F1 score of 0.930 and consistently relied on proximal recipient speed as the most important feature. Computation of posterior likelihood showed a 95.29% chance of patients having long-term patency given a lower proximal speed. CONCLUSIONS: These results suggest that a high proximal speed measured in the recipient vessel prior to anastomosis can elevate the risk of perioperative no flow and long-term reduction of flow. With an increased dataset size, continued FLOW 800-based ROI metric analysis could be used to guide intraoperative anastomosis site selection prior to anastomosis and predict patency outcome.

Superficial Temporal Artery Size Changes After Encephaloduroarteriosynangiosis (EDAS) for the Treatment of Moyamoya Disease.

BACKGROUND AND OBJECTIVES: Surgery is the mainstay of stroke prevention in patients with symptomatic Moyamoya disease. We present the results of a single-center retrospective study of indirect revascularization surgery for adult moyamoya disease, emphasizing angiographic outcomes, including dilation of the superficial temporal artery and formation of new collaterals. METHODS: A prospectively maintained database of procedures performed for Moyamoya disease was reviewed. Adult patients treated with indirect revascularization and with long-term angiographic follow-up were included. Pre-operative and postoperative angiographic images and baseline and procedural characteristics were analyzed. A Wilcoxon signed-rank test was used to test the hypothesis that the superficial temporal artery increases in diameter postoperatively. RESULTS: We identified 40 hemispheres in 27 patients, of which 35 had a sufficient angiographic follow-up. Bilateral procedures were performed on 16 patients. Most patients were female (72.5%), with a median age of 43 years-old. The most common clinical presentation was ischemic stroke in 59.3% of cases. All patients underwent an encephaloduroarteriosynangiosis (EDAS) for treatment. A follow-up angiogram was performed at a median of 13.8 months postoperatively, showing STA-derived collaterals in 71.4% and collateral ingrowth via the burr holes in 61.8% of cases. Disease progression was evident in 34.3% of hemispheres. The normalized STA diameter was significantly increased postoperatively (2.4 to 3 mm; p<0.05). A univariate analysis revealed that transdural collaterals and hyperlipidemia may affect collateral ingrowth from the STA, and no other patient or procedure-related factors, including replacement of the bone flap, impacted on this. CONCLUSION: A significant increase in STA diameter on follow-up angiography after EDAS was found, however this was not directly associated with STA collateral development. Rates of postoperative transient ischemic attacks were low, and no patients had a new ischemic or hemorrhagic stroke at last follow up. The presence of transdural collaterals and the absence of hyperlipidemia were associated with STA collateral development on follow-up angiography, but the causality of this finding is unclear.

Comprehensive Analysis and In Vitro Verification of Endothelial-Mesenchymal Transition-Related Genes in Moyamoya Disease.

Moyamoya disease (MMD) is a rare, chronic, and progressive cerebrovascular disorder with unclear underlying causes and mechanisms. Previous studies suggest a potential involvement of endothelial-mesenchymal transition (EndMT) in the pathogenesis of MMD. This study aimed to explore the contribution of EndMT-related genes (ERGs) in MMD. Two datasets, GSE141022 and GSE157628, were integrated as the training set after batch effects removal. Differentially expressed ERGs were identified between MMD and control groups. Functional enrichment analysis and immune infiltration analysis were further performed. LASSO regression was used for hub MMD-related ERG selection. Consensus clustering was used for MMD subtype classification based on these hub MMD-related ERGs. Molecular characteristics between MMD subtypes were analyzed using WGCNA. PPI network was used to illuminate the genetic relationship. The hub MMD-related ERGs were validated in an independent testing set, GSE189993. The nomogram model was constructed and evaluated using ROC curves and calibration plots. Additionally, CCK-8, EdU, wound healing, and western blot were performed to confirm the function of the hub MMD-related ERGs. A total of 107 DE-ERGs were identified. Functional enrichment analysis showed these genes were associated with EndMT and immune response. The infiltrating levels of immune cells were commonly higher in the MMD group. LASSO regression identified 12 hub MMD-related ERGs, leading to the identification of two MMD subtypes. Four ERGs emerged as the final hub MMD-related ERGs after validation in the testing set, including CCL21, CEBPA, KRT18, and TNFRSF11A. The nomogram model exhibited excellent discrimination ability. In vitro experiments showed that CCL21, CEBPA, KRT18, and TNFRSF11A could promote proliferation, migration, and EndMT. This study investigated the potential role of EndMT in MMD and identified four hub MMD-related ERGs, providing potential therapeutic targets for MMD treatment.

Identification of lysosome-related hub genes as potential biomarkers and immune infiltrations of moyamoya disease by multiple bioinformatics methods and machine-learning strategies.

Background: Moyamoya disease (MMD), characterized by chronic cerebrovascular pathology, poses a rare yet significant clinical challenge, associated with elevated rates of mortality and disability. Despite intensive research endeavors, the exact biomarkers driving its pathogenesis remain enigmatic. Methods: The expression patterns of GSE189993 and GSE141022 were retrieved from the Gene Expression Omnibus (GEO) repository to procure differentially expressed genes (DEGs) between samples afflicted with MMD and those under control conditions. The Least Absolute Shrinkage and Selection Operator (LASSO), Support Vector Machine with Recursive Feature Elimination (SVM-RFE), and Random Forest (RF) algorithms were employed for identifying candidate diagnostic genes associated with MMD. Subsequently, these candidate genes underwent validation in an independent cohort (GSE157628). The CMAP database was ultimately employed to forecast drugs pertinent to MMD for clinical translation. Results: A collective of 240 DEGs were discerned. Functional enrichment scrutiny unveiled the enrichment of the cholesterol metabolism pathway, salmonella infection pathway, and allograft rejection pathway within the MMD cohort. EPDR1, DENND3, and NCSTN emerged as discerned diagnostic biomarkers for MMD. The CMAP database was ultimately employed to scrutinize the ten most auspicious pharmaceutical compounds for managing MMD. Finally, after validation through in vitro experiments, EPDR1, DENND3, and NCSTN were identified as the key genes. Conclusion: EPDR1, DENND3, and NCSTN have emerged as potential novel biomarkers for MMD. The involvement of T lymphocytes, neutrophilic granulocytes, dendritic cells, natural killer cells, and plasma cells could be pivotal in the pathogenesis and advancement of MMD.

Asciminib Use Highlighting Underlying Moyamoya Disease: A Case Report.

We highlight here a case of Moyamoya disease (MMD) developed after treatment for chronic myeloid leukemia (CML). Moyamoya, a term meaning "a hazy puff of smoke" in Japanese, denotes a chronic occlusive cerebrovascular condition involving bilateral stenosis or closure of the terminal part of the internal carotid arteries (ICAs) and the proximal sections of the anterior cerebral arteries (ACAs) and middle cerebral arteries (MCAs) resulting in the development of abnormal vascular collaterals. A 40-year-old African-American female with a past medical history of CML presented to the oncology clinic with expressive aphasia. Of note, she was diagnosed with CML eight years ago and was previously treated with dasatinib and nilotinib with only partial remission. She tested positive for the T315I mutation and was initiated on asciminib therapy about a month before her symptoms surfaced. Asciminib, an allosteric inhibitor targeting breakpoint cluster region-abelson murine leukemia 1 (BCR-ABL1) kinase activity, has gained approval for treating patients diagnosed with chronic-phase CML who have not responded to two prior lines of therapy or for those carrying the T315I mutation. During admission, the patient underwent brain magnetic resonance imaging (MRI) and a computed tomography (CT) angiogram of the head showed moderate to severe narrowing at the origins of the bilateral MCA and ACA, concerning Moyamoya syndrome. Although not classically associated with asciminib therapy, we report here a patient with CML who developed expressive aphasia one month after starting the medication. Due to the high index of suspicion, asciminib was discontinued, and the patient was referred for bone marrow transplant evaluation and concurrently started on cytarabine + peginterferon. The patient had improvement in her symptoms of aphasia after the drug was discontinued and returned to her baseline functional status.  No cardiovascular side effects associated with the use of asciminib are currently reported in the literature. However, we have described a case of such an occurrence. Therefore, extra caution should be taken in prescribing asciminib in patients with risk factors or a prior history of stroke.

Approach to adult Moyamoya disease presenting as bilateral anterior circulation infarcts: a case report.

Moyamoya disease (MMD) is a rare progressive condition of unknown etiology that affects the blood vessels in the brain and is characterized by narrowing or stenosis of the distal parts of the internal carotid and major intracranial arteries, leading to the development of fragile collateral supplies. This makes it a significant causative factor for both ischemic strokes as well as intracerebral hemorrhages. As per the recommendations by the Research Committee on Moyamoya disease in 2021, the diagnostic criteria for MMD are essentially based on indicative radiological findings on cerebral angiography or MRI Brain and MRA, after excluding possible differentials. In this case report, we present the case of a 45-year-old Asian lady who presented with sudden-onset headaches and right-sided weakness. Her admission non-contrast CT brain scan was undiagnostic. MRI brain and MRA scans revealed bilateral anterior circulation watershed infarcts of variable age along with supra-clinoid stenosis. She was initially treated with steroids for vasculitis after discussions with a multidisciplinary team involving neurologists, stroke physicians and neuro-radiologists; however, further imaging revealed a diagnosis of MMD, and she was subsequently referred for revascularization surgery. Given the limited number of similarly documented cases, we hope this report will contribute to the knowledge base.

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

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Neurovascular Issues in Antiphospholipid Syndrome: Arterial Vasculopathy from Small to Large Vessels in a Neuroradiological Perspective.

Antiphospholipid syndrome (APS) is an autoimmune prothrombotic condition characterized by venous thromboembolism, arterial thrombosis, and pregnancy morbidity. Among neurological manifestations, arterial thrombosis is only one of the possible associated clinical and neuroradiological features. The aim of this review is to address from a neurovascular point of view the multifaceted range of the arterial side of APS. A modern neurovascular approach was proposed, dividing the CNS involvement on the basis of the size of affected arteries, from large to small arteries, and corresponding clinical and neuroradiological issues. Both large-vessel and small-vessel involvement in APS were detailed, highlighting the limitations of the available literature in the attempt to derive some pathomechanisms. APS is a complex disease, and its neurological involvement appears multifaceted and not yet fully characterized, within and outside the diagnostic criteria. The involvement of intracranial large and small vessels appears poorly characterized, and the overlapping with the previously proposed inflammatory manifestations is consistent.

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.

Evaluation of deep learning algorithms in detecting moyamoya disease: a systematic review and single-arm meta-analysis.

The diagnosis of Moyamoya disease (MMD) relies heavily on imaging, which could benefit from standardized machine learning tools. This study aims to evaluate the diagnostic efficacy of deep learning (DL) algorithms for MMD by analyzing sensitivity, specificity, and the area under the curve (AUC) compared to expert consensus. We conducted a systematic search of PubMed, Embase, and Web of Science for articles published from inception to February 2024. Eligible studies were required to report diagnostic accuracy metrics such as sensitivity, specificity, and AUC, excluding those not in English or using traditional machine learning methods. Seven studies were included, comprising a sample of 4,416 patients, of whom 1,358 had MMD. The pooled sensitivity for common and random effects models was 0.89 (95% CI: 0.85 to 0.92) and 0.92 (95% CI: 0.85 to 0.96), respectively. The pooled specificity was 0.89 (95% CI: 0.86 to 0.91) in the common effects model and 0.91 (95% CI: 0.75 to 0.97) in the random effects model. Two studies reported the AUC alongside their confidence intervals. A meta-analysis synthesizing these findings aggregated a mean AUC of 0.94 (95% CI: 0.92 to 0.96) for common effects and 0.89 (95% CI: 0.76 to 1.02) for random effects models. Deep learning models significantly enhance the diagnosis of MMD by efficiently extracting and identifying complex image patterns with high sensitivity and specificity. Trial registration: CRD42024524998 https://www.crd.york.ac.uk/prospero/displayrecord.php?RecordID=524998.

Analysis of rescue strategies for acute thrombosis during STA-MCA bypass surgery and the literature review.

BACKGROUND AND OBJECTIVES: STA-MCA bypass surgery is mainly used for Moyamoya disease, giant intracranial aneurysms, and resection of intracranial tumors requiring sacrifice of blood vessels. The intraoperative patency of the reconstructive vessels is critical to the efficacy of the procedure. This study aimed to evaluate the efficacy of intra-arterially infused tirofiban for the treatment of acute thrombosis during STA-MCA bypass surgery and countermeasures for acute thrombosis. METHODS: This study involved 209 patients (272 hemispheres) who underwent STA-MCA surgery between November 2020 and December 2023. Intraoperative acute thrombosis occurred in eight patients (3.83%,8 hemispheres). We retrospectively reviewed the clinical and imaging data, surgical procedure, and follow-up outcomes of eight patients. We implemented the different thrombolytic methods to evaluate the optimal thrombosis management during the bypass surgery. After three months, we assessed neurological functions using the modified Rankin Scale (mRS) and conducted a literature review using PubMed. RESULTS: Eight patients (four male patients and four female patients) developed acute thrombosis during the bypass surgery. Of the eight patients, two underwent re-anastomosis after thrombus removal, three received local injections of tirofiban into the anastomosis or the branches of the superficial temporal artery, and three underwent superselective intra-arterial tirofiban infusion using a microcatheter. Thrombosis were resolved, and arteries were recanalized in all patients. The mRS score was 0 in all patients. No major ischemic or hemorrhagic complications occurred. CONCLUSION: Our treatment methods were efficacious in the management of acute thrombosis. Intra-arterial tirofiban administration seems to be a simple and effective treatment option for acute thrombosis during STA-MCA bypass surgery.

Worse outcome in hemorrhagic moyamoya disease of anterior circulation type compared to posterior circulation type.

OBJECTIVE: Despite the known poor outcomes of patients with hemorrhagic moyamoya disease (MMD), previous studies have not explored the entire population of hemorrhagic MMD, often excluding severely impaired patients due to the lack of imaging studies demonstrating cerebral angioarchitecture. Herein, we investigate the prevalence, location of intracerebral hematoma (ICH), and outcomes in patients with hemorrhagic MMD, including severely impaired individuals who underwent emergency computed tomography (CT) angiography (CTA) to identify secondary ICHs. METHODS: We conducted a retrospective analysis of 2092 patients admitted to our hospital within 3 days of ICH onset from January 2010 to December 2022. CTA was performed for all patients with ICH, principally. RESULTS: CTA was performed in 1645 (78.6%) patients. We diagnosed MMD in 40 patients (2.5%), making it the third leading cause of secondary ICH. Twenty patients had anterior-type hematomas, while the remaining twenty had posterior-type hematomas. At 90 days after onset, 19 patients (95%) with anterior-type hematomas had unfavorable outcomes (modified Rankin scale [mRS] scores of 3-6), compared to 11 patients (55%) with posterior-type hematomas. The number of unfavorable outcomes was significantly higher in the anterior-type group compared to the posterior-type group (p = 0.008). CONCLUSION: This comprehensive study highlights that the majority of MMD cases with ICH result in unfavorable outcomes, especially when the ICH is located in the anterior circulation. While recent studies have focused on preventing bleeding from choroidal anastomosis in the posterior circulation, overall outcome improvement of hemorrhagic MMD necessitates a greater emphasis on addressing anterior circulation ICHs.

An Unusual Presentation of Moyamoya Disease in a Pediatric Patient.

Moyamoya disease (MMD) is a chronic cerebro-vasculopathy that is extremely rare in the pediatric population. The main characteristic feature is the progressive stenosis in the internal carotid artery with or without the involvement of its main branches in the circle of Willis leading to ischemic stroke. Patients have clinical manifestations related to cerebral ischemia in the carotid branch territories, such as sensory impairment, hemiparesis, and aphasia/dysarthria. Herein, we report a case of MMD in a six-year-old Emirati female who presented with unusual manifestations of MMD in the form of headache, vomiting, and double vision and was diagnosed with MMD based on a brain MRI with angiography. To our knowledge, this is the first reported case of MMD in the United Arab Emirates.