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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. [ABSTRACT FROM AUTHOR]

Moyamoya disease (MMD) is a chronic and progressive cerebrovascular stenosis or occlusive disease that occurs near Willis blood vessels. The aim of this study was to investigate the mutation of DIAPH1 in Asian population, and to compare the angiographic features of MMD patients with and without the mutation of the DIAPH1 gene. Blood samples of 50 patients with MMD were collected, and DIAPH1 gene mutation was detected. The angiographic involvement of the posterior cerebral artery was compared between the mutant group and the non-mutant group. The independent risk factors of posterior cerebral artery involvement were determined by multivariate logistic regression analysis. DIAPH1 gene mutation was detected in 9 (18%) of 50 patients, including 7 synonymous mutations and 2 missense mutations. However, the incidence of posterior cerebral artery involvement in mutation positive group was very higher than that in mutation negative group (77.8% versus 12%; p = 0.001). There is an association between DIAPH1 mutation and PCA involvement (odds ratio 29.483, 95% confidence interval 3.920–221.736; p = 0.001). DIAPH1 gene mutation is not a major genetic risk gene for Asian patients with moyamoya disease but may play an important role in the involvement of posterior cerebral artery. [ABSTRACT FROM AUTHOR]

Background: To investigate the risk factors for early seizure after revascularization in patients with moyamoya disease (MMD). Methods: A total of 298 patients with MMD diagnosed in our hospital from 2015 to 2018 were analyzed retrospectively. We summarized the characteristics of seizure after revascularization in patients with MMD and analyzed the predictors of early postoperative seizure. Results: We identified 15 patients with MMD who developed seizures within 1 week after revascularization. According to logistic regression analysis, age (OR: 1.04, 95% CI 0.998–1.086; P = 0.060) and infarct side (OR: 1.92, 95% CI 0.856–4.290; P = 0.113) were not significantly associated with incident early seizure. Postoperative infarction (OR: 12.89, 95% CI 4.198–39.525; P = 0.000) and preoperative cerebral infarction (OR: 4.08, 95% CI 1.267–13.119; P = 0.018) were confirmed as risk factors for early seizure. Conclusions: We believe that a history of preoperative infarction and new infarction are independent risk factors of early seizure in patients with MMD after revascularization. [ABSTRACT FROM AUTHOR]

Objective: To investigate the angioarchitectural factors associated with postoperative cerebral infarction in ischemic moyamoya disease. Methods: Data on patients who underwent surgery for ischemic MMD from 1 October 2015 to 31 October 2020, at Peking University International Hospital were collected and retrospectively analyzed. General conditions such as patient sex, age, site of surgery, preoperative manifestations such as TIA attack and old cerebral infarction, and seven angioarchitectural factors of the MMD based on DSA were selected and measured. Statistical analysis was performed by the Pearson chi-square statistic, analysis of variance (ANOVA), and multifactor logistic regression analysis. Results: Age (OR, 0.969; 95%CI, 0.939–1.000; p = 0.049), A1stenosis (OR, 5.843; 95%CI, 1.730–19.732; p = 0.004), M1stenosis (OR, 6.206; 95%CI, 2.079–18.526; p = 0.001), PCA anomalies (OR, 4.367; 95%CI, 1.452–13.129; p = 0.049), Unstable compensation (OR, 5.335; 95%CI, 1.427–19.948; p = 0.013), TIA (OR, 4.264; 95%CI, 1.844–9.863; p = 0.001), Old cerebral infarction (OR, 2.972; 95%CI, 1.194–7.397; p = 0.019). The above seven factors can be used in the regression equation to predict the probability of postoperative cerebral infarction. The prediction accuracy is 90.2%. Conclusions: Age, TIA attack, old cerebral infarction, and five angioarchitectural factors of MMD are strongly associated with postoperative cerebral infarction. Seven factors, including age, TIA attack, old infarction, and four angioarchitectural factors, can be taken to quantify the probability of surgical cerebral infarction in MMD. [ABSTRACT FROM AUTHOR]

Background: Ischemic events are the most common postoperative complication in bypass surgery for moyamoya disease (MMD), but the risk factors for pediatric MMD remain unclear. The goal of the study was to investigate the risk factors for postoperative ischemic complications in pediatric MMD patients.Methods: We retrospectively reviewed a consecutive series of pediatric MMD cases at Beijing Tiantan Hospital, Capital Medical University from June 2010 through June 2019. Preoperative clinical variables and radiographic findings were recorded, and logistic regression analysis was carried out to identify the risk factors for postoperative ischemic events.Results: A total of 533 operations in 336 patients were included in this study. Postoperative complications occurred after 51 operations (9.6%), including 40/447 indirect bypass procedures, 9/70 direct bypass procedures, and 2/16 combined bypass procedures. Postoperative ischemic events were the most common complication and occurred in 30 patients after 31 procedures (8.9% per patient; 5.8% per operation), including 26/447 indirect bypass procedures, 4/70 direct bypass procedures, and 1/16 combined bypass procedures, and the incidence of these events did not differ significantly between indirect and non-indirect bypass (5.8% vs 5.8%; p = 0.999). Multivariate logistic regression analyses revealed that older age at operation (OR 1.129, 95% CI 1.011-1.260, p = 0.032) and posterior cerebral artery involvement (OR 2.587, 95% CI 1.030-6.496, p = 0.043) were significantly associated with postoperative ischemic events.Conclusion: We speculate that older age at operation and posterior cerebral artery involvement are risk factors for postoperative ischemic events in pediatric MMD patients. [ABSTRACT FROM AUTHOR]

Background: The research on postoperative collateral formation for hemorrhagic moyamoya disease (MMD) evaluated by using digital subtraction angiography (DSA) is limited. Our study objective was to investigate the postoperative collateral formation after indirect bypass for hemorrhagic MMD.Methods: All consecutive inpatients with hemorrhagic MMD who received indirect bypass at Beijing Tiantan Hospital, Capital Medical University from January 2010 through December 2018 were screened. The site of the hemorrhage was classified as either anterior or posterior. Postoperative collateral formation was evaluated on lateral views using the Matsushima scale. Univariate and multivariate logistic regression analyses were carried out to determine the factors influencing postoperative collateral formation.Results: Six-four patients (64 hemispheres) were included in this study. After a median 8.5 months DSA follow-up, 14 (21.9%) hemispheres had grade A collateral circulation, 13 (20.3%) had grade B, and 37 (57.8%) had grade C. Twenty-seven (42.2%) hemispheres had good postoperative collateral formation and 37 (57.8%) had poor postoperative collateral formation. The univariate logistic regression analyses showed that age at operation (OR, 0.954; 95% CI, 0.908-1.003; p = 0.066), hemorrhagic site (OR, 4.694; 95% CI, 1.582-13.923; p = 0.005), and PCA involvement (OR, 3.474; 95% CI, 0.922-13.086; p = 0.066) may effect postoperative collateral formation. The multivariate logistic regression analyses showed that only anterior hemorrhage (OR, 5.222; 95% CI, 1.605-16.987; p = 0.006) was significantly related to good postoperative collateral formation.Conclusion: Anterior hemorrhage was significantly related to good postoperative collateral formation after indirect bypass. [ABSTRACT FROM AUTHOR]

Objective: To identify associated risk factors for the angiographic outcomes after direct and combined bypass surgery in moyamoya disease (MMD). Methods: All direct and combined bypass procedures performed from June 2009 to May 2015 were screened in this prospective cohort study. Patients who acquired presurgical and follow-up catheter angiography were included. Bypass patency and postoperative collateral formation were evaluated. Univariate and multivariate logistic regression analyses were performed to determine the influence factors for bypass patency and postoperative collateral formation. Results: In total, 188 consecutive bypass procedures were included. After an 18-month median follow-up, the anastomosis patency rate was 88.3%. Postoperative collateral formation was associated with the patency of the anastomosis (Gamma = 0.891, p < 0.001). Multivariate logistic regression analysis showed that presence of hemorrhage (OR, 0.298; 95% CI, 0.125–0.709; p = 0.006) was associated with obstructed anastomosis. Among the 188 bypass surgeries, 125 (63.2%) hemispheres had good postoperative collateral formation and 85 (36.8%) had poor postoperative collateral formation. Multivariate logistic regression analysis showed that younger age at operation (OR, 2.396; 95% CI, 1.231–4.664; p = 0.010) was associated with good postoperative collateral formation, while the poor postoperative collateral formation was related to presence of hemorrhage (OR, 0.329; 95% CI, 0.143–0.758; p = 0.009) and dilated anterior choroidal artery (OR, 0.472; 95% CI, 0.240–0.929; p = 0.030). Conclusions: This study has demonstrated that presence of hemorrhage predicts lower patency rates. Younger age at operation was associated with good postoperative collateral formation, while the poor postoperative collateral formation was related to presence of hemorrhage and dilated anterior choroidal artery. [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]

Delayed anastomotic occlusion occurred in a considerable proportion of hemorrhagic moyamoya disease (MMD) patients undergoing direct revascularization. This study aimed to investigate the predictors and outcomes of delayed anastomotic occlusion in adult hemorrhagic MMD. The authors retrospectively reviewed 87 adult hemorrhagic MMD patients. Univariate and multivariate logistic regression analyses were performed. After an average of 9.1 ± 6.9 months of angiographic follow-up, the long-term graft patency rates were 79.8%. The occluded group had significantly worse angiogenesis than the non-occluded group (p < 0.001). However, the improvement of dilated anterior choroidal artery–posterior communicating artery was similar (p = 0.090). After an average of 4.0 ± 2.5 years of clinical follow-up, the neurological statues and postoperative annualized rupture risk were similar between the occluded and non-occluded groups (p = 0.750; p = 0.679; respectively). In the multivariate logistic regression analysis, collateral circulation Grade III (OR, 4.772; 95% CI, 1.184–19.230; p = 0.028) and preoperative computed tomography perfusion (CTP) Grade I–II (OR, 4.129; 95% CI, 1.294–13.175; p = 0.017) were independent predictors of delayed anastomotic occlusion. Delayed anastomotic occlusion in adult hemorrhagic MMD might be a benign phenomenon. Good collateral circulation (Grade III) and compensable preoperative intracranial perfusion (CTP Grade I–II) are independent predictors for this phenomenon. Moreover, the delayed anastomotic occlusion has no significant correlations with the long-term angiographic and neurological outcomes, except neoangiogenesis. [ABSTRACT FROM AUTHOR]