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Background: The study aimed to investigate the association between nonalcoholic fatty liver disease (NAFLD) and ischemic stroke events after revascularization in patients with Moyamoya disease (MMD)., Methods: This study prospectively enrolled 275 MMD patients from September 2020 to December 2021. Patients with alcoholism and other liver diseases were excluded. NAFLD was confirmed by CT imaging or abdominal ultrasonography. Stroke events and modified Rankin Scale (mRS) scores at the latest follow-up were compared between the two groups., Results: A total of 275 patients were enrolled in the study, among which 65 were diagnosed with NAFLD. Univariate logistic regression analysis showed that NAFLD (P = 0.029) was related to stroke events. Multivariate logistic regression analysis showed that NAFLD is a predictor of postoperative stroke in MMD patients (OR = 27.145, 95% CI = 2.031-362.81, P = 0.013). Kaplan-Meier analysis showed that compared with MMD patients with NAFLD, patients in the control group had a longer stroke-free time (P = 0.004). Univariate Cox analysis showed that NAFLD (P = 0.016) was associated with ischemic stroke during follow-up in patients with MMD. Multivariate Cox analysis showed that NAFLD was an independent risk factor for stroke in patients with MMD (HR = 10.815, 95% CI = 1.259-92.881, P = 0.030). Furthermore, fewer patients in the NAFLD group had good neurologic status (mRS score ≤ 2) than the control group (P = 0.005)., Conclusion: NAFLD was an independent risk factor for stroke in patients with MMD after revascularization and worse neurological function outcomes., (© 2024. The Author(s).)

Background and Purpose: The prognostic nutrition index (PNI) has been associated with the prognosis of various medical disorders. This study aimed to explore the correlation between PNI and the long-term outcomes of adult patients afflicted with moyamoya disease (MMD)., Methods: This prospective study initially employed 138 adult patients diagnosed with MMD. After excluding 15 patients who did not meet the criteria, a total of 123 patients were included. Participants were divided into three groups based on the tertile of change in the PNI score. Statistical analysis compared clinical information and lab tests among the groups. The study was conducted between July 1 and December 31, 2019., Results: After adjusting for multiple variables, patients in the upper two tertiles (tertiles 2-3) exhibited a significantly lower risk of adverse long-term outcomes compared to those in the lowest tertile (tertile 1) (OR, 0.089; 95% CI, 0.009-0.895; P = 0.040). Furthermore, adding PNI tertile to traditional risk factors substantially improved predicting adverse long-term outcomes (net reclassification improvement: 98.03%, P = 0.000; integrated discrimination improvement: 4.65%, P = 0.030). However, there was no statistically significant difference between the first PNI tertile (tertile 1) and the upper two tertiles (tertiles 2-3) in the Kaplan-Meier curve of stroke incidence (log-rank test, P = 0.244)., Conclusions: A higher PNI level was significantly associated with a reduced risk of unfavorable long-term outcomes. Nevertheless, the PNI score did not predict stroke recurrence during extended follow-up. This study provides insights into a potential predictor of adverse long-term outcomes after revascularization in MMD patients., Registration Number: ChiCTR2000031412., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

Purpose: In past pediatric and adult cohort studies of moyamoya disease, the fetal posterior cerebral artery has received less attention. Its relationship with the clinical manifestations and collateral circulation of moyamoya disease or ipsilateral cerebral hemispheres remains unclear., Method: We summarize the clinical features of patients with and without fetal posterior cerebral artery moyamoya disease from consecutive cases.We explored the relationship between fetal posterior cerebral arteries and collateral circulation in the ipsilateral cerebral hemispheres, as well as differences among different subgroups of patients.According to the morphology, the fetal posterior cerebral artery is divided into complete fetal posterior cerebral artery and partial fetal posterior cerebral artery. Clinical features were classified as: infarction,hemorrhage,and non-stroke in unilateral/bilateral cerebral hemispheres. Collateral circulation is divided into extracranial vascular compensation and leptomeningeal collateral circulation. Digital subtraction angiography and CT/MR were used to evaluate the blood flow status and clinical characteristics of patients with moyamoya disease., Result: A total of 960 cerebral hemispheres from 142 pediatric patients and 338 adult patients were included in the study. A total of 273 (56.9%) patients had 399 cerebral hemispheres (41.6%) with fetal posterior cerebral arteries. Adults with fetal posterior cerebral arteries had lower rates of infarction (24.6%vs37.3%, P =0.005) and were less likely to have bilateral stroke (8.4%vs11.5%, P =0.038). Cerebral hemispheres with fetal posterior cerebral artery were more likely to have anterior cerebral artery and middle cerebral artery stenosis and less likely to have occlusion (P =0.002, 0.001), and less likely to involve the posterior circulation (P < 0.001). The cerebral hemispheres of the fetal posterior cerebral artery had higher leptomeningeal collateral circulation scores. There are significant differences in extracranial vascular compensation between cerebral hemispheres with and without fetal posterior cerebral artery. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage (P =0.017)., Conclusions: Our results suggest that fetal posterior cerebral artery is associated with infarct manifestations in pediatric and adult moyamoya disease. In the cerebral hemispheres, the fetal posterior cerebral artery is associated with ipsilateral hemispheric anterior and posterior circulation artery injury, extracranial vascular compensation, leptomeningeal collateral circulation compensation, and infarction. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage., Competing Interests: Declaration of Competing Interest The authors have no conflicts of interest to declare;, (Copyright © 2023. Published by Elsevier Inc.)

Background: Moyamoya disease (MMD) is an uncommon cerebrovascular disease which leads to progressive stenosis and occlusion of the bilateral internal carotid artery and main intracerebral arteries. Concerns are always on how the hemisphere with infarction affects cognitive function, while little attention is paid to the role that the non-infarcted hemisphere plays. Therefore, we aimed to detect cortical indexes, especially cortical complexity in the left or right hemisphere separately in patients with MMD after stroke., Methods: 28 patients with MMD (14 males, 14 females) and 14 healthy controls were included in this study. All participants underwent cognitive tests and magnetic resonance imaging (MRI) scan. The preprocessing of three-dimensional T1 weighted images were performed by standard surface-based morphometry. Surface-based morphometry statistical analysis was carried out with a threshold of False Discovery Rate (FDR) P < 0.05 and fractal dimension (FD) was used to provide a quantitative description of cerebral cortical complexity., Results: Widespread cognitive dysfunctions were found in MMD patient with stroke. Extensive FD reduction in the left hemisphere with right-sided infarction, mainly in the superior temporal, inferior frontal, and insula, while the post central gyrus, superior parietal, and inferior parietal gyrus also showed a wide range of significant differences (FDR corrected P < 0.05). Meanwhile, FD changes in the right hemisphere with left-sided infarction are restricted to the precuneus and cingulate isthmus (FDR corrected P < 0.05)., Conclusions: Extensive cognitive impairment was reconfirmed in Moyamoya disease with stroke, while wild and asymmetrical decrease of cortical complexity is observed on both sides. These differences could be relative to unbalanced cognitive dysfunction, and may be the result of a long-term chronic ischemia and compensatory of the contralateral hemisphere to the infarction., (© 2021. The Author(s).)

Background: Recurrent stroke after surgical revascularization is still a big issue for moyamoya disease (MMD). This study aims to identify predictors for postoperative stroke and unfavorable outcome in ischemic-type MMD., Methods: We identified a consecutive series of patients with ischemic-type MMD who underwent revascularization between January 2005 and December 2012. Predictors for postoperative stroke and functional outcomes were assessed with logistic and Cox regression analysis., Results: A total of 346 patients underwent 437 revascularization procedures and the mean follow-up period was 4.0 years. The incidence of perioperative stroke was 6.9%. Being adult at onset (odds ratio [OR], 5.033; 95% confidence interval [CI], 1.447-17.506; P = 0.011) and posterior cerebral artery (PCA) stenosis (OR, 3.364; 95% CI, 1.588-7.265; P = 0.002) before surgery were predictors of perioperative stroke. The annual subsequent stroke rate beyond 30 days after surgery was 1.2%. Subsequent stroke events tended to occur throughout the first 5 years after surgery in adults, whereas in children they mainly occurred within the first 2 years after surgery. Age at onset (OR, 1.025; 95% CI, 1.003-1.048; P = 0.023), ischemic stroke or transient ischemic attack at presentation (OR, 2.703; 95% CI, 1.062-6.875; P = 0.037), and PCA involvement (OR, 2.664; 95% CI, 1.462-4.854; P = 0.001) were associated with higher risk of overall postoperative stroke. PCA involvement (OR, 2.62; 95% CI, 1.33-5.15; P = 0.005), internal carotid artery supraclinoid segment occlusion (OR, 2.76; 95% CI, 1.27-6.03; P = 0.011), and older age at onset (OR, 1.03; 95% CI, 1.01-1.05; P = 0.033) were predictive of unfavorable outcome., Conclusions: Patients with ischemic-type MMD at an older age and more severe angiopathy might be at higher risk of recurrent stroke and unfavorable outcome after revascularization., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background and Purpose- Evidence on the natural history of hemorrhagic moyamoya disease is still insufficient. We investigated the incidence of recurrent intracranial bleeding, mortality, and risk factors for rebleeding in patients with moyamoya disease. Methods- A total of 128 conservatively managed patients with hemorrhagic presentation and complete follow-up data were included. Recurrent hemorrhages during long-term follow-up were documented. Annual and cumulative incidence rate of bleeding was generated via Kaplan-Meier survival analysis, and risk factors were analyzed using logistic regression analysis. Results- The median follow-up time was 10.1 (1-27) years. During a total of 1300.7 patient-years, 47 (36.7%) patients experienced 59 occurrences of recurrent hemorrhages, rendering an average annual incidence of 4.5%. Among them, 9 patients (19.1%) died from rebleeding and 12 patients sustained severe disability (modified Rankin Scale score of ≥3). The cumulative risk of rebleeding was 7.8% at 5 years, 22.6% at 10 years, and 35.9% at 15 years. Only 4 (3.1%) patients experienced ischemic stroke, yielding an average annual incidence of 0.3%. Multivariate analysis showed that smoking (odds ratio, 4.85; P=0.04) was an independent risk factor of rebleeding. Rebleeding (hazard ratio, 11.04; P=0.02) and hypertension (hazard ratio, 4.16; P=0.04) were associated with increased mortality. Age, type of initial bleeding, digital subtraction angiography staging, family history, and coexisting cerebral aneurysms were not associated with increased risk of rebleeding. Conclusions- Rebleeding events were common and the main cause of death in patients with hemorrhagic moyamoya disease. The risk of rebleeding steadily increased during long-term follow-up. Smoking was a risk factor for rebleeding, and hypertension was associated with increased mortality.

Background: Despite being the most common presentation in children with moyamoya, transient ischemic attack (TIA) in children has rarely been described. The aim of this study is to describe the clinical characteristics of TIAs in children with moyamoya and explore the risk factors for stroke after TIA., Methods: We reviewed 696 consecutive patients with moyamoya vasculopathy (155 pediatric patients and 541 adults) admitted to our hospital from 2009 to 2015 to identify pediatric patients with moyamoya with an initial presentation of TIA. We defined recurrent TIAs that involve more types of symptoms or symptom extensions as symptom progression. The risk factors for subsequent stroke were analyzed using time-to-event analyses., Results: We identified 60 pediatric patients with moyamoya who had presented with TIA (initial presentation age, 10.0 ± 3.5 years). Motor weakness (n = 51 [85%]) was the most common initial presentation. During follow-up, 55 patients (91.7%) had recurrent TIAs and 14 (23.3%) had subsequent strokes. We identified female gender (hazard ratio, 5.08; 95% confidence interval, 1.40-18.47; P = 0.01), Suzuki grade greater than 3 (hazard ratio, 4.01; 95% confidence interval, 1.16-13.82; P = 0.03), and symptom progression (hazard ratio, 5.31; 95% confidence interval, 1.65-17.14; P = 0.01) as independent predictors of future stroke events., Conclusions: Transient ischemic attacks have a relatively high recurrence rate in children with moyamoya and are associated with subsequent stroke. We identified the female sex, Suzuki grade greater than  3, and symptom progression as independent predictors of future strokes., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: Stroke in young adults is uncommon and rarely described. Moyamoya disease is one of the leading causes of stroke in young adults. We aimed to study the prognostic factors for stroke recurrences and functional outcomes in young stroke patients with moyamoya disease after revascularization., Methods: We reviewed 696 consecutive patients with moyamoya disease admitted to our hospital from 2009-2015 and identified patients aged 18-45 years with first-ever stroke. Follow-up was conducted via face-to-face or structured telephone interviews. Outcome measures were recurrent stroke events and unfavorable functional outcomes (modified Rankin Scale >2)., Results: We included 121 young patients with moyamoya disease suffering from stroke (initial presentation age, 35.4 ± 7.5 years). All patients underwent revascularization after the acute phase of initial stroke events as the secondary prevention for recurrences. During follow-up (median, 40 months), 9 patients (7.4%) experienced recurrent strokes and 8 of them (6.6%) suffered unfavorable functional outcomes. In the multivariate analysis, diabetes was an independent predictor for stroke recurrences (hazard ratio 6.76; 95% confidence interval 1.30-35.11; P = 0.02) and was significantly associated with unfavorable functional outcomes (odds ratio 7.87; 95% confidence interval 1.42-38.74; P = 0.01)., Conclusions: We identified diabetes as an independent risk factor for recurrent strokes and unfavorable functional outcomes after revascularization in young stroke patients with moyamoya disease., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Objective: To elucidate the clinical and angiographic features in patients with moyamoya disease (MMD) and the p.R4810K heterozygous variant and present an angiographic grading system to evaluate disease severity., Methods: We retrospectively reviewed 87 patients with MMD and the p.R4810K variant treated at Beijing Tiantan Hospital. Clinical features, stroke subtype, and angiographic characteristics were analyzed., Results: The median age at diagnosis was 25 years (range, 3-59). The ratio of women to men was 1.2:1. The familial occurrence of MMD was 14.9%. The primary symptom at diagnosis was ischemia, hemorrhage, or other in 67, 16, and 4 patients, respectively. Angiographic features correlating with ischemic stroke or stroke, including Suzuki grade, external carotid artery collaterals, leptomeningeal collaterals, and Mugikura grade, were identified. A binary logistic regression model demonstrated a significant correlation of Suzuki grade (P = 0.008) and posterior cerebral artery grade (P = 0.029) with ischemic stroke (142 hemispheres). A modified Suzuki-Mugikura grading system was developed. The areas under the receiver operating characteristic curves used to predict ischemic stroke based on the Suzuki grading, Mugikura grading, and modified Suzuki-Mugikura grading systems were 0.736, 0.69, and 0.741, respectively. Furthermore, the modified Suzuki-Mugikura grades were significantly correlated with infarction in posterior circulation and the number of infarcted regions., Conclusions: The clinical and angiographic features of a Chinese MMD population with the p.R4810K variant were similar to those of a Japanese MMD population; they might be a distinct cerebrovascular disease entity and represent a separate subgroup. A modified Suzuki-Mugikura grading system was valuable for predicting stroke and evaluating disease severity., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Object: The aim of this study was to describe the baseline clinical features and long-term outcomes of patients with moyamoya disease (MMD) based on a 25-year period at a single center in China., Methods:  Data obtained in 528 consecutive patients with MMD treated at the authors' hospital from 1984 to 2010 were reviewed retrospectively. Events of transient ischemic attack, new infarction, and hemorrhage were included. The Kaplan-Meier risk of stroke was calculated., Results:  The mean (±SD) patient age was 26±13 years (range 2-67 years), and the female/male ratio was 0.9:1. There were 332 cases of ischemia and 196 hemorrhages. Adults had a higher rate of bleeding than children (50.7% vs 14.0%, respectively; p<0.001). One hundred twenty-two patients were treated conservatively, and 406 patients underwent revascularization procedures. Of 528 patients, 331 (62.7%) had at least 1 year of follow-up (median 39.5 months) and data from these patients were analyzed. Rebleeding and mortality rates in patients with hemorrhagic MMD (n=104) were higher than in those with ischemic MMD (n=227) (26.9% vs 2.2% [p<0.001] and 4.8% vs 0.4% [p<0.05], respectively). Twenty-five of 60 (41.7%) conservatively treated patients and 8 of 271 (2.9%) surgically treated patients experienced rebleeding events, a difference that was significant in the Kaplan-Meier curve of rebleeding (p<0.01). An improvement in perfusion was found in 164 of 224 (73.2%) surgically treated patients 1 month after discharge. However, there was no significant difference in the rate of ischemic events in the surgical and conservative groups (18.8% and 28.3%, respectively; p=0.09). Among the 104 hemorrhagic cases, rebleeding attacks were observed in 25 patients in the nonsurgical group (n=60) and 3 patients in the surgical group (n=44) (41.7% and 6.8%, respectively; OR 9.7 [95% CI 2.7-35.0]; p<0.01)., Conclusions:  There was no difference in the sex distribution of Chinese patients with MMD. Patients with hemorrhagic MMD had a much higher rate of rebleeding and poorer prognosis than those with the ischemic type. Surgical revascularization procedures can improve cerebral perfusion and have a positive impact in preventing rebleeding in patients with hemorrhagic MMD.

Objective: This study was conducted in order to investigate the association of matrix metalloproteinase (MMP)-9 levels with phenotypes of moyamoya disease (MMD). Methods: This study included plasma samples from 84 MMD patients. The clinical variables of these patients were reviewed from the medical record. The serum concentration of tight junction, adherens junction proteins, and MMPs (MMP-2 and MMP-9) was determined using the ELISA method. Patients with hemorrhagic-onset MMD were compared with those with ischemic-onset MMD. Results: Compared with pediatric patients, the expression of MMP-9 was significantly higher, while the MMP-2 and vascular endothelial-cadherin were lower in adult patients. In adult subgroup analysis, hemorrhagic MMD patients exhibited significantly higher serum concentrations of MMP-9 compared with ischemic MMD patients. The ROC curve identified that a baseline serum MMP-9 level >1,011 ng/ml may be associated with spontaneous hemorrhage in adult MMD patients with 70.37% sensitivity and 71.88% specificity [area under curve (AUC), 0.73; 95% CI 0.597–0.864; P = 0.003]. A late Suzuki stage (>4) (OR 4.565, 95% CI 1.028–20.280, P = 0.046) and serum concentrations of MMP-9 >1,011 ng/ml (OR 7.218, 95% CI 1.826–28.533, P = 0.005) are risk predictors of hemorrhages in MMD patients. Hemorrhagic-type MMD patients had higher serum levels of MMP-9 and BBB permeability compared with ischemic-type MMD patients. Adult MMD patients had higher serum levels of MMP-9 and BBB permeability compared with pediatric patients. Conclusions: MMP-9 might serve as a biomarker for hemorrhage prediction in MMD. Serum MMP-9 level >1,011 ng/ml is an independent risk factor of MMD hemorrhagic strokes. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to compare the postoperative risks and long-term effects between direct bypass surgery (DB) and indirect bypass (IB) surgery for pediatric patients with ischemic-type moyamoya disease (MMD). Method: Pediatric patients (under or equal to 18 years old) who were diagnosed as MMD and given surgical treatments at our center between 2009 and 2015 were retrospectively reviewed from a prospective database. Pediatric hemorrhagic-type MMD patients and those who did not undergo digital subtraction angiography (DSA) were excluded. Patients who underwent DB were matched with patients who underwent IB using 1:1 propensity score matching. Postoperative complications, recurrent ischemic stroke events and modified Rankin Scale (mRS) scores at the last follow-up were compared between the matched pairs. Results: A total of 223 pediatric patients were screened, and 138 patients (DB:34, IB:104) were considered for the propensity score match. Thirty four pairs were obtained. Nine patients had postoperative complications, including 6 (17.6%) in the DB group and 3 (8.8%) in the IB group (P = 0.476). The mean follow-up period was 71.9 ± 22.2 months for the DB group and 60.2 ± 24.3 months for the IB group (P = 0.041). Kaplan-Meier analysis showed a longer stroke-free time in the DB group than in the IB group (P = 0.025). At last follow-up, good neurological status (mRS ≤ 1) was achieved in 32 (94.1%) of the DB group and 34 (100.0%) of the IB group. MRS score at last follow-up were significantly lower than at time of admission (all pts: 1.09 ± 0.45 vs. 0.28 ± 0.51, P < 0.001; DB group: 1.12 ± 0.48 vs. 0.32 ± 0.59, P < 0.001; IB group: 1.06 ± 0.42 vs. 0.24 ± 0.43, P < 0.001). Conclusion: Both techniques were effective in improving the neurological status of pediatric ischemic-type MMD patients, and direct bypass surgery might be more superior in preventing recurrent ischemic strokes in the short-term. [ABSTRACT FROM AUTHOR]

Objective: To estimate the association between p. R4810K variant and clinical outcomes of patients with moyamoya disease (MMD). Methods: The p.R4810K genetic variant was genotyped among 498 Chinese patients with MMD conducted from June 1, 2012, to June 31, 2017. Data was obtained by retrospective chart review, follow-up information and outcome were obtained through clinical visits and telephone. Results: Among 498 patients, 361 (72.5%) were wild-type patients (G/G), 133 (26.7%) heterozygous patients (G/A), and 4 (0.8%) homozygotes (A/A). Compared with GG group, the patients in the G/A+A/A group were younger at diagnosis and had more familial cases, more transient ischemic attack cases, more posterior cerebral artery involved hemispheres, less unilateral lesions. After the median 53 months follow-up, strokes occurred in 9 patients in the G/A+A/A group and in 52 in the G/G group. Multivariate Cox regression analysis showed that the history of hypertension (HR, 2.294; 95% CI, 1.251–4.206; p = 0.007), the presence of TIA (HR, 0.319; 95% CI, 0.120–0.846; p = 0.022), and the Suzuki stage (HR, 1.510; 95% CI, 1.129–2.018; p = 0.005) were associated with recurrent stroke. The p.R4810K (HR, 0.601; 95% CI, 0.292–1.239; p = 0.168) was not associated with recurrent stroke. Multivariate logistic regression analysis showed that recurrent stroke (OR, 5.997; 95% CI, 2.583–13.924; p = 0.000) was the only factor associated with unfavorable neurological status. And the p.R4810K (OR, 0.885; 95% CI, 0.482–1.627; p = 0.695) was not associated with neurological status. Conclusions: Compared to the patients in G/G group, patients in G/A+A/A group exhibited different clinical features, and had a lower rate of recurrent stroke and better clinical outcome after early medical and surgical interventions. Multivariate COX and logistic regression analysis showed that p.R4810Kvariant was not related to either recurrent stroke or neurological status. The p.R4810Kvariant may not be associated with long-term clinical outcome in Chinese patients with MMD. [ABSTRACT FROM AUTHOR]

Objective: This study aims to investigate the clinical features, long-term outcomes, and prognostic predictors of a multicenter cohort of children with moyamoya disease. Methods: A series of 303 consecutive pediatric moyamoya disease (MMD) patients were screened in the present study. The clinical characteristics were retrospectively collected, and long-term outcomes was evaluated. Furthermore, logistic regression analyses were performed to determine the prognostic predictors for the clinical outcome. Results: The mean onset age at diagnosis was 9.4 years old. The gender ratio (girl-to-boy ratio) was 1.1:1.0. Among these 303 patients, 13 patients underwent different surgical modalities in bilateral hemispheres, while eight patients failed to follow-up, and were excluded. Therefore, a total of 282 patients were analyzed. Among these patients, 17 patients underwent combined bypass (CB), 47 patients underwent direct bypass (DB), 150 patients underwent indirect bypass (IB), and 68 patients underwent conservative treatment. Furthermore, recurrent stroke events were observed in 35 patients (12.4%). The Kaplan-Meier analysis demonstrated that there was no significant difference in either ischemia or hemorrhage-free time among the different surgical modalities (P = 0.67 and 0.79, respectively). Furthermore, longer ischemia-free time was observed in the surgical group, when compared to the conservative group (P < 0.01). In addition, 82.7% (177/214) of patients who underwent surgical treatment obtained good outcomes (mRS 0-1), which were significantly higher than the rate of patients who underwent conservative treatment (52.9%, 36/68; P < 0.01). The rate of patients with improved symptoms was also significantly different (93.0 vs. 16.2%, P < 0.01). However, no significant difference was observed in the rate of good outcomes, disability, and improved symptoms among the different surgical modalities. The logistic regression analyses revealed that postoperative ischemic events were the only risk factor associated with unfavorable clinical outcome (OR:3.463; 95% CI:1.436–8.351; P < 0.01). Conclusion: CB, DB, and IB might have similar effects on long-term clinical outcome in pediatric MMD. However, surgical revascularization is superior, when compared to conservative treatment. Furthermore, postoperative ischemic events were confirmed as potential prognostic factors associated with unfavorable clinical outcome. [ABSTRACT FROM AUTHOR]

Objective To investigate the long-term outcomes after conservative and direct surgical treatment for patients with moyamoya disease (MMD) at late Suzuki stage. Methods We retrospectively reviewed 82 patients (164 hemispheres) with MMD at late Suzuki stage at Beijing Tiantan Hospital. Clinical features, radiologic findings, and outcomes were analyzed. Results The mean age at diagnosis was 36.4 ± 11.7 years. The distribution of the initial Suzuki stage of MMD was as follows: stage 4, n = 113; stage 5, n = 45; stage 6, n = 6, posterior cerebral artery involvement was observed in 41 hemispheres (25.0%). The incidence of postoperative stroke (<48 hours) was 6.7%. During the average follow-up of 55.1 ± 16.2 months, including postoperative and follow-up strokes, 7 of 75 conservatively treated hemispheres (9.3%) and 9 of 89 surgically treated patients (10.1%) experienced a stroke event; there was no statistical significance in the Kaplan-Meier curve of stroke incidence between the surgical group and conservative group (log-rank test, P = 0.848). However, the rate of perfusion improvement in direct bypass surgically treated patients was higher than in those conservatively treated patients 3 months after discharge ( P < 0.05). Conclusions Although direct bypass surgery was shown to effectively improve the cerebral perfusion in patients with MMD at late Suzuki stage, compared with conservative treatment, it did not reduce the risk of recurrent stroke. Further study is needed to determine whether direct bypass surgery is effective in patients with MMD at late Suzuki stage. [ABSTRACT FROM AUTHOR]

Objective To investigate long noncoding ribonucleic acid (lncRNA) expression patterns in adult moyamoya disease (MMD) patients and explore their possible roles in the pathophysiology of MMD. Methods A healthy control group ( n = 10) and an MMD group ( n = 15) were evaluated. RNA was extracted from peripheral blood samples and hybridized to microarray to get lncRNA expression profiles. Then predicted lncRNA target genes were identified, and bioinformatics analysis was performed to investigate their molecular functions. Results In the MMD group, 3649 lncRNAs exhibited more than 2-fold expression than their counterparts in the healthy control group; of these, 1494 were upregulated, while 2155 were downregulated. Principal component analysis and Hclust analysis produced completely different clusters between the 2 groups. Gene ontology and KEGG pathway enrichment analysis suggested that the differentially expressed lncRNAs regulate multiple signaling pathways that were related with inflammation and vascular disease, and mitogen-activated protein kinase (MAPK) signaling pathway was the core regulatory pathway. Conclusions Long noncoding RNA expression profiles were quite different between MMD and control groups. Multiple signaling pathways that were closely associated with immune response, vasculogenesis, and smooth muscle contraction were indicated to participate in lncRNAs regulatory mechanism; of these, MAPK signaling pathway, which has been well studied for the treatment of many other cardiovascular diseases, was the core of this regulatory network. Our findings could help further understand the pathophysiology of MMD and provide new potential therapeutic targets. [ABSTRACT FROM AUTHOR]