Moyamoya Research Articles

How Revascularization Affects Choroid Plexus in Patients with Moyamoya Disease? [ISMRM Travel Award記録

How Revascularization Affects Choroid Plexus in Patients with Moyamoya Disease? [ISMRM Travel Award記録

Hybrid Multi-Delay PCASL of Time-Encoded and Variable-TR Schemes for the Assessment of Cerebral Perfusion in Moyamoya Disease [Presidential Award Proceedings

Hybrid Multi-Delay PCASL of Time-Encoded and Variable-TR Schemes for the Assessment of Cerebral Perfusion in Moyamoya Disease [Presidential Award Proceedings

System Inflammation Response Index and Serum Glucose-Potassium Ratio as Prognostic Indicators for Hemorrhagic Moyamoya Disease

Hemorrhagic moyamoya disease (HMMD) can result in poor outcomes. Serum biomarkers may play a significant role in predicting HMMD outcomes. This study retrospectively analyzed the correlation between serum biomarkers at the time of admission and outcomes for patients with HMMD. We evaluated 270 patients with HMMD admitted to Yijishan Hopital of Wannan Medical College between July 2017 and April 2023. The patients were categorized into 2 groups according to their modified Rankin Scale scores at 3months after discharge. Univariate and multivariate analyses were used to identify the associations between biomarkers and clinical outcomes. Receiver operating characteristic curves were obtained to investigate the potential of the biomarkers for predicting prognosis. Of the 270 patients analyzed, 96 (35.6%) had unfavorable outcomes. The potential predictors were identified using the univariate analysis. The subsequent multivariate logistic regression analysis showed that the systemic inflammatory response index (SIRI) (odds ratio 0.86, 95% confidence interval 0.75-0.98, P=0.028] and serum glucose to potassium ratio (GPR) (odds ratio 0.53, 95% confidence interval 0.38-0.76, P<0.001) were independent risk factors of poor outcomes. The receiver operating characteristic analysis indicated that patients with a higher SIRI (≥2.12) and GPR (≥1.75) levels were more likely to have unfavorable outcomes. An elevated GPR and SIRI at admission were associated with a poor clinical outcome at the 3-month follow-up for patients with HMMD. Therefore, these biomarkers could be considered in future management decisions for these patients.

Clinical Course, Therapy, and Long-Term Outcomes of Children With Moyamoya Disease and Posterior Cerebral Artery Involvement.

Posterior cerebral artery involvement (PCAi) has been identified as an important factor related to poor prognosis in moyamoya disease (MMD). This study summarized the characteristics of children with MMD and PCAi, clarified the clinical course, identified prognostic predictors, and investigated the long-term effect of encephaloduroarteriosynangiosis for posterior circulation (EDAS-p). We retrospectively reviewed all our pediatric MMD cases with follow-up angiograms from November 2003 to December 2016. PCAi was classified as early-onset at initial diagnosis and delayed-onset after anterior circulation revascularization. Multivariable data including clinical features, radiographic findings, and surgical outcomes were analyzed. Among 570 children with MMD, 246 (43.2%) had PCAi, with 176 (30.9%) classified as early-onset PCAi. During a median follow-up period of 10 years, 17.8% (70/394) of patients without initial PCAi developed delayed-onset PCAi. The median time to detection of a new PCA lesion was 15.5 (range 7-110) months from initial diagnosis, with a median age of 10.5 (3-22). Younger age at onset, familial occurrence, advanced Suzuki stages, and preoperative infarctions were predictors of delayed-onset PCAi. EDAS-p was performed on 294 hemispheres of 195 patients with PCAi. Stroke-free survival was significantly higher in the EDAS-p group than in the non-EDAS-p group (99.0% vs 90.2%; p < 0.001 [Breslow test]; p = 0.001 [log-rank test]; median follow-up: 101 months). PCAi is not uncommon in children with MMD, underscoring the need for long-term close clinical monitoring, especially in patients with high-risk factors for PCA progression. EDAS-p may be a safe and effective procedure for preventing subsequent stroke in children with MMD and PCAi.

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.

Scalp Reconstruction With Free Tissue Transfer Using Descending Branch of Lateral Circumflex Femoral Vessels as Interposition Graft.

Despite the rich blood supply to the scalp, postoperative skin necrosis at the surgical site sometimes occurs, and unresolved defects often present a significant challenge for the surgeon. When planning a free flap for scalp reconstruction, distant anastomoses may be necessary if local recipient vessels are unavailable due to previous surgery. This study presents the authors' treatment strategy, which includes extending the pedicle length with arteriovenous bundle interposition grafts. Two patients who experienced surgical wound necrosis after bypass surgery for moyamoya disease underwent reconstruction for skin and soft tissue coverage. To evaluate the altered anatomical structures after the previous surgery, three-dimensional computed tomography angiography was performed, and preoperative photos were taken to accurately measure the expected defect. The surgical strategy was designed to accommodate their unique conditions, utilizing an arteriovenous interposition graft, which included 2 stages of microvascular anastomoses, for pedicle lengthening. The procedure successfully bridged the gap between distant recipient vessels and the extensive defect, and the wounds healed without major complications such as venous congestion or total flap loss. Both patients were satisfied with the surgical outcomes. Arteriovenous bundle interposition grafts can be a useful option for patients with complex scalp defects who lack local recipient vessels. The use of an anterolateral thigh flap with artery-to-artery anastomosis, utilizing the lateral circumflex femoral artery from the opposite side in this study, offers many advantages compared with traditional pedicle lengthening tools utilizing vein-to-artery anastomosis.

Superficial Temporal Artery Size Changes After Encephaloduroarteriosynangiosis for the Treatment of Moyamoya Disease

Surgery is the mainstay of stroke prevention in patients with symptomatic moyamoya disease (MMD). We present the results of a single-center retrospective study of indirect revascularization surgery for adult MMD, emphasizing angiographic outcomes, including dilation of the superficial temporal artery and formation of new collaterals. A prospectively maintained database of procedures performed for MMD was reviewed. Adult patients treated with indirect revascularization and with long-term angiographic follow-up were included. Preoperative 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. 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 for treatment. A follow-up angiogram was performed at a median of 13.8months postoperatively, showing superficial temporal artery (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 3mm; 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. A significant increase in STA diameter on follow-up angiography after encephaloduroarteriosynangiosis 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.

Prevalence and Clinical Characteristics of Heterozygous RNF213 p.Arg4810Lys Variant Carriers Diagnosed With Chronic Thromboembolic Pulmonary Hypertension.

Ring finger protein 213 (RNF213) p.Arg4810Lys is a susceptibility gene for moyamoya disease, peripheral pulmonary artery stenosis (PPS), and other vascular diseases and thrombosis. We investigated the prevalence and clinical characteristics of RNF213 variant carriers diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH). We retrospectively analyzed the prevalence of the RNF213 p.Arg4810Lys variant in patients diagnosed with CTEPH (n=112) and PPS (n=10). Clinical and angiographic characteristics were evaluated between RNF213 variant carriers diagnosed with CTEPH and noncarriers with CTEPH and homozygous variant carriers with PPS. Eight heterozygous RNF213 p.Arg4810Lys variant carriers (7.1%) were identified among patients diagnosed with CTEPH, while 5 patients with PPS (50%) carried the homozygous variant. The clinical characteristics of heterozygous variant carriers with CTEPH were not remarkably different from those of noncarriers with CTEPH. All heterozygous variant carriers with CTEPH showed webs/bands lesions at the segmental/subsegmental level, with 75% showing distal tortuous vessels. None of the heterozygous variant carriers with CTEPH exhibited the string-of-beads pattern or elongated stenosis. Homozygous variant carriers with PPS showed the string-of-beads pattern, elongated stenosis, and distal tortuous vessels without webs/bands lesions. A subset of patients diagnosed with CTEPH (7.1%) carried the heterozygous RNF213 p.Arg4810Lys variant. Clinical and angiographic characteristics of heterozygous variant carriers were not remarkably different from those of noncarriers of CTEPH. However, both heterozygous variant carriers with CTEPH and homozygous variant carriers with PPS showed tortuous vessels on angiography.

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

ObjectiveRecurrent 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). MethodsThis 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. ResultsA 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. ConclusionAfter indirect revascularization, surgeries in patients with symptomatic MMD, FV of ipsilateral ECA at 3 mo helps clinicians to identify patients at risk of PIE.

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

In adults, moyamoya disease (MMD) often presents with slight neurocognitive impairment, which may result from frontal lobe hemodynamic insufficiency. 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. 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). 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.

Flow capacity of a superficial temporal artery as a donor in a consecutive series of 100 patients with superficial temporal artery-middle cerebral artery bypass.

A superficial temporal artery-middle cerebral artery (STA-MCA) bypass is classically considered a low-flow bypass. It is known that the flow in the flow augmentation STA-MCA bypass is influenced by flow demand of the revascularized territory and can reach significantly higher values. The authors report their intraoperative flow measurement data in a consecutive series of 100 STA-MCA bypasses performed at their institution. Moreover, in a subanalysis, they show the postoperative bypass flow measured with quantitative MR angiography (qMRA) noninvasive optimal vessel analysis (NOVA). Between January 2013 and October 2023, 100 patients with acute, subacute, or chronic large-vessel occlusion (LVO) or moyamoya disease underwent a flow augmentation STA-MCA bypass revascularization at the authors' department with intraoperative bypass flow measurement. Patients with atherosclerotic LVO who underwent bypass surgery within a 6-week period following the onset of ischemic stroke symptoms were categorized into the acute bypass group, encompassing both acute and subacute LVO cases. Conversely, those who underwent bypass surgery > 6 weeks after the last occurrence of ischemic stroke were classified as the chronic group. Since May 2019, a consecutive subgroup of 37 patients received a postoperative (before discharge) bypass flow measurement with the qMRA-NOVA imaging tool. The mean ± SD intraoperative bypass flow in this consecutive series of 100 STA-MCA bypasses was 53.5 ± 28.8 ml/min (range 14-145 ml/min). In the subanalysis, there was no difference in the intraoperative flow capacity between the acute and chronic groups and between the moyamoya and acute groups. Patients in the moyamoya group showed a significantly higher flow rate in the STA-MCA bypass compared with the chronic group (63.0 ± 30.2 ml/min vs 48.4 ± 26.5 ml/min, p = 0.03). In a consecutive subanalysis of 37 STA-MCA bypass cases, postoperative flow measurements were also performed using qMRA-NOVA, showing a significant increase in the flow of STA-MCA bypasses after surgery compared with intraoperative flow measurements (mean intraoperative bypass flow rate vs qMRA-NOVA postoperative bypass flow rate: 73.4 ± 29.9 ml/min vs 111.3 ± 51.4 ml/min, p = 0.005). Using intraoperative and postoperative quantitative flow measurements of the STA, the data confirm that the flow in the flow augmentation STA-MCA bypass is influenced by the flow demand of the revascularized territory and can reach high values if needed. Moreover, the significant flow increase in the postoperative flow measurement using qMRA-NOVA demonstrates that the bypass can increase its flow over time.

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

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. In this retrospective study, we conducted genetic tests for Ring Finger Protein 213 (RNF213) 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 RNF213 and exhibited definite features of MMD on HR-MRI. Among 306 patients who underwent RNF213 tests for the evaluation of MMD, 56 showed positive RNF213, 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). Concentric wall enhancement was a significant predictor of acute cerebral infarction in patients with MMD.

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

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. 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. 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. 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.

Iodine-123-Iomazenil Single-Photon Emission Computed Tomography Revealed Recovery of Neuronal Viability in Association With Improvement of Cognitive Dysfunction After Revascularization in Moyamoya Disease.

Recent studies indicate that 123I-iomazenil (123I-IMZ) single-photon emission computed tomography (SPECT) can demonstrate neuronal viability. Although cognitive dysfunction has been recognized as an important issue in adult patients with moyamoya disease (MMD), no standard neuroradiological methods to define such conditions have been established. We examined the relationship between cognitive function and 123I-IMZ SPECT before and after revascularization in patients with MMD. The study participants were 16 adult patients with MMD whose cerebrovascular reactivities were decreased only on the surgical sides of combined revascularization. Cognitive function was examined using the Mini-Mental State Examination (MMSE; cutoff: 27) and the Frontal Assessment Battery (FAB; cutoff: 16) before and at 3 to 6 months after surgery. 123I-iodoamphetamine (123I-IMP) SPECT with acetazolamide challenge and 123I-IMZ SPECT were performed concurrently while evaluating cognitive function. The radioreactivities of 123I-IMZ SPECT in regions with decreased cerebrovascular reactivities on 123I-IMP SPECT were investigated using affected-to-contralateral side asymmetry ratio (IMZ-ACR). Twelve patients showed normal cognitive function (MMSE: 29.8 ± 0.4, FAB: 18 ± 0) before surgery. No evident laterality of 123I-IMZ uptake was seen (IMZ-ACR: 0.98 ± 0.04). Neither cognitive function nor 123I-IMZ SPECT worsened after surgery (MMSE: 29.8 ± 0.3, FAB: 18 ± 0, IMZ-ACR: 1.00 ± 0.04). By contrast, 4 patients presented cognitive dysfunction (MMSE: 24.3 ± 3.9, FAB: 14.8 ± 2.7) before revascularization. Preoperative imaging of these patients showed decreased 123I-IMZ uptake, and their IMZ-ACRs (0.83 ± 0.08) were significantly lower than those of the normal group. After revascularization, cognitive functions and 123I-IMZ uptake tended to ameliorate (MMSE: 27.5 ± 1.7, FAB: 16.3 ± 2.2, IMZ-ACR: 0.94 ± 0.09). Preoperative cognitive function was associated with 123I-IMZ uptake in adult patients with MMD. After revascularization, cognitive function could be recovered in the viable areas of the brain, which is consistent with 123I-IMZ SPECT findings.

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

ObjectiveDespite 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. MethodsWe 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. ResultsCTA 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). ConclusionThis 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.

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

Background and objectivesSTA-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.MethodsThis 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.ResultsEight 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.ConclusionOur 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.

A rare cranial disease: Prolactinoma-associated moyamoya syndrome

A rare cranial disease: Prolactinoma-associated moyamoya syndrome

Multi-parameter MRI-Based Machine Learning Model to Evaluate the Efficacy of STA-MCA Bypass Surgery for Moyamoya Disease: A Pilot Study.

Superficial temporal artery-middle cerebral artery (STA-MCA) bypass surgery represents the primary treatment for Moyamoya disease (MMD), with its efficacy contingent upon collateral vessel development. This study aimed to develop and validate a machine learning (ML) model for the non-invasive assessment of STA-MCA bypass surgery efficacy in MMD. This study enrolled 118 MMD patients undergoing STA-MCA bypass surgery. Clinical features were screened to construct a clinical model. MRI features were extracted from the middle cerebral artery supply area using 3D Slicer and employed to build five ML models using logistic regression algorithm. The combined model was developed by integrating the radiomics score (Rad-score) with the clinical features. Model performance validation was conducted using ROC curves. Platelet count (PLT) was identified as a significant clinical feature for constructing the clinical model. A total of 3404 features (851 × 4) were extracted, and 15 optimal features were selected from each MRI sequence as predictive factors. Multivariable logistic regression identified PLT and Rad-score as independent parameters used for constructing the combined model. In the testing set, the AUC of the T1WI ML model [0.84 (95% CI, 0.70-0.97)] was higher than that of the clinical model [0.66 (95% CI, 0.46-0.86)] and the combined model [0.80 (95% CI, 0.66-0.95)]. The T1WI ML model can be used to assess the postoperative efficacy of STA-MCA bypass surgery for MMD.

FOXM1 c.1205C > A mutation is associated with unilateral Moyamoya disease and inhibits angiogenesis in human brain endothelial cells.

Unilateral moyamoya disease (MMD) represents a distinct subtype characterised by occlusive changes in the circle of Willis and abnormal vascular network formation. However, the aetiology and pathogenesis of unilateral MMD remain unclear. In this study, genetic screening of a family with unilateral MMD using whole-genome sequencing helped identify the c.1205C > A variant of FOXM1, which encodes the transcription factor FOXM1 and plays a crucial role in angiogenesis and cell proliferation, as a susceptibility gene mutation. We demonstrated that this mutation significantly attenuated the proangiogenic effects of FOXM1 in human brain endothelial cells, leading to reduced proliferation, migration, and tube formation. Furthermore, FOXM1 c.1205C > A results in increased apoptosis of human brain endothelial cells, mediated by the downregulation of the transcription of the apoptosis-inhibiting protein BCL2. These results suggest a potential role for the FOXM1 c.1205C > A mutation in the pathogenesis of unilateral MMD and may contribute to the understanding and treatment of this condition.

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.