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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 [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]

Growing evidence has suggested that hyperhomocysteinemia (HHcy) is a risk factor for cerebral infarction. However, the effect of HHcy on postoperative cerebral ischemia is still unclear. We aim to investigate the relationship between HHcy and postoperative ischemia of adult patients with moyamoya disease (MMD). A total of 138 adult patients with MMD were prospectively recruited from July 1 to December 31, 2019. After excluding 14 patients accepting conservative therapy, all 124 patients who underwent surgical treatment were enrolled. Patients were grouped according to postoperative ischemia and HHcy presentation, respectively. Clinical data and laboratory examinations were compared by statistical analyses. Potential risk factors were evaluated by univariate and multivariate logistic regression analysis. Comparing to the normal, patients with postoperative ischemia were higher in serum homocysteine (Hcy) level (P = 0.039) and HHcy ratio (P = 0.035). Furthermore, HHcy was more common in males (P = 0.007) than females. Logistic analysis results showed that HHcy (OR 5.234, 95% CI 1.127–24.315; P = 0.035) was an independent risk factor. HHcy was significantly associated with postoperative ischemia in MMD patients. Our study found that HHcy was correlated to the risk of postoperative ischemia. HHcy can be used as an indicator and a potential therapeutic target for postoperative ischemia in adult patients with MMD. URL: http://www.chictr.org. Unique identifier: ChiCTR2000031412. [ABSTRACT FROM AUTHOR]

Direct bypass surgery improves blood flow in patients with moyamoya disease (MMD) as well as causes local flow relocation and disturbance. This study aimed to describe the characteristics and changes of quantitative blood flow in MMD patients. Sixty-four consecutive MMD patients (67 hemispheres) who underwent STA-MCA anastomosis between August 2013 and September 2017 were included in this study. Intraoperative blood flow of superficial temporal artery and middle cerebral artery was measured with a noninvasive, ultrasound flowprobe, before and after the bypass. All data were collected prospectively. Before the bypass, 67 hemispheres were measured and 45 hemispheres acquired post-bypass measurements. Thirty-three (49.3%) had anterograde flow and 34 (50.7%) had retrograde. Retrograde blood flow was significantly higher than anterograde blood flow in MCA-M4 section (3.71 ± 2.78 ml/min vs 2.39 ± 1.71 ml/min, P = 0.023). Thirty-eight (56.7%) patients had local cerebrovascular reactivity, whereas 29 (43.3%) lost it. After bypass, blood flow was changed into three patterns: anterograde flow (1, 2.2%), retrograde flow (3, 6.7%), and bi-direction flow (41, 91.1%). Flow of M4 was significantly improved in both proximal (13.64 ± 9.16 vs 3.28 ± 2.57, P < 0.001) and distal (7.17 ± 7.21 vs 3.28 ± 2.57, P = 0.002) sides to the bypass. Postoperative infarctions occurred in 4 (8.9%) patients. All postoperative infarctions (n = 4) happened in those patients who did not have change of flow direction in M4 section distal to the bypass (P = 0.040). Direct bypass surgery improves local blood flow after bypass surgery in both sides of the anastomosis. Flow direction was usually changed after the surgery into a bi-direction pattern: anterograde flow in M4 distal to the bypass, and retrograde flow in M4 proximal to the bypass. Proximal side acquired around 5.4 times of flow than the distal side. Patients with change of flow direction in distal M4 were related with lower risks of postoperative infarctions. [ABSTRACT FROM AUTHOR]

Background: Cerebral hyperperfusion syndrome (CHS) is a common complication after direct bypass surgery in patients with Moyamoya disease (MMD). Since preventive measures may be inadequate, we assessed whether the blood flow difference between the superficial temporal artery (STA) and recipient vessels (△BF) and the direct perfusion range (DPR) are related to CHS. Methods: We measured blood flow in the STA and recipient blood vessels before bypass surgery by transit-time probe to calculate △BF. Perfusion changes around the anastomosis before and after bypass were analyzed with FLOW800 to obtain DPR. Multiple factors, such as △BF, DPR, and postoperative CHS, were analyzed using binary logistic regression. Results: Forty-one patients with MMD who underwent direct bypass surgery were included in the study. Postoperative CHS symptoms occurred in 13/41 patients. △BF and DPR significantly differed between the CHS and non-CHS groups. The optimal receiver operating characteristic (ROC) curve cut-off value was 31.4 ml/min for ΔBF, and the area under the ROC curve (AUC) was 0.695 (sensitivity 0.846, specificity 0.500). The optimal cut-off value was 3.5 cm for DPR, and the AUC was 0.702 (sensitivity 0.615, specificity 0.750). Conclusion: Postoperative CHS is caused by multiple factors. △BF is a risk factor for CHS while DPR is a protective factor against CHS. [ABSTRACT FROM AUTHOR]

The corresponding hemodynamic changes of the internal carotid artery (ICA) after the revascularization surgery for moyamoya disease (MMD) remain unclear. The aim of this study was to analyze the hemodynamic changes of the ipsilateral ICA after the combined direct and indirect extracranial-intracranial (EC-IC) bypass. MMD patients undergoing combined EC-IC bypass were retrospectively reviewed. The mean transit time (MTT) of ICA was evaluated by color-coding angiography before revascularization and at follow-up. The MTT defined as the blood transit time between the end of cervical portion (C1) and the C7 segment of ICA. The clinical prognosis was assessed with Matsushima grading system, moyamoya vessel reduction system, and modified Rankin Scale (mRS). The correlation between hemodynamic parameter and prognosis was analyzed. Subgroup analysis was conducted between different presentations and different ages. Fifty-one patients were identified and the mean imaging follow-up interval was 5.5 months. The ICA-MTT was increased after the combined revascularization (P < 0.001) compared with contralateral ICA. Faster preoperative ICA-MTT was significantly associated with improved mRS in the ischemic group (P = 0.05). The increased ICA-MTT was significantly associated with favorable neoangiogenesis (P = 0.04), moyamoya vessel reduction (> 50%) (P = 0.023), and improved mRS score (P = 0.008). In subgroup analysis, the correlation in the ischemic subgroup and adult subgroup remained significant. In this cohort, the ICA-MTT increased after the combined EC-IC bypass, and there was a positive correlation between the increased blood transit time and favorable outcomes. Color-coding DSA proved to be useful as a quantitative and serial method to monitor postoperative courses after revascularization in MMD. [ABSTRACT FROM AUTHOR]

Few studies focused on the intraoperative blood pressure in Moyamoya disease (MMD) patients. We aimed to clarify whether or not it relates to early cerebral infarction after revascularization. We reviewed a retrospective cohort of Moyamoya disease from 2011 to 2018 in Beijing Tiantan Hospital, and patients with radiologically confirmed early postoperative infarction were included in the analysis. Controls were matched based on age, sex, and revascularization modality at a ratio of 1:5. Perioperative clinical factors and intraoperative blood pressure data were collected and analyzed. A total of 52 patients out of 1497 revascularization surgeries (3.5%) who experienced CT or MRI confirmed early postoperatively cerebral infarction, aged 38.46 ± 11.70; 26 were male (50.0%). Average real variability (ARV)-systolic blood pressure (SBP) (OR 3.29, p = 0.003), ARV-diastolic blood pressure (DBP) (OR 4.10, p = 0.005), ARV-mean arterial pressure (MAP) (OR 4.08, p = 0.004), and the maximum drops of DBP (OR 1.08, p = 0.003) and MAP (OR 1.06, p = 0.004) were associated with early postoperative infarction. In patients who experienced massive cerebral infarction, the maximum drops of DBP (OR 1.11, p = 0.004) and MAP (OR 1.11, p = 0.003) are independent risk factors, whereas ARVs of SBP (OR 3.90, p < 0.001), DBP (OR 4.69, p = 0.008), and MAP (OR 4.72, p = 0.003) are significantly associated with regional infarction. High variance of intraoperative blood pressure and drastic blood pressure decline are independent risk factors for postoperative infarction in MMD patients who underwent revascularization surgery. Maintaining stable intraoperative blood pressure is suggested to prevent early postoperative cerebral infarction in MMD patients. [ABSTRACT FROM AUTHOR]

Objective: Indirect bypass surgery, which induces spontaneous neoangiogenesis in ischemic brain tissue and improves cerebral blood flow, is an effective treatment for moyamoya disease (MMD). The time course of neoangiogenesis development has not yet been investigated. This study aimed to determine the critical period of neovascularization after indirect bypass in MMD patients. Methods: Patients with MMD who underwent indirect bypass surgery at Peking University International Hospital between January 2015 and October 2017 were retrospectively reviewed. Surgically treated hemispheres with short-term (3–6 months) and long-term (1 year) follow-up digital subtraction angiography (DSA) were included. The effects of revascularization were evaluated on lateral and anteroposterior views using angiography and compared between two follow-ups of the same hemisphere. Results: This study included 25 hemispheres from 24 MMD patients (mean age: 22.48 ± 14.83 years), among whom 13 were pediatric patients and 12 were adults. Qualitative measurements including the Matsushima scale and coverage of neoangiogenesis on anteroposterior views were not significantly different between the short term and long term (P = 0.083 and P = 0.157, respectively). Quantitative measurements including the greatest width and height of the area covered by neovascularization on lateral views of DSA and the greatest depth of neovascularization penetration on anteroposterior views (P = 0.488, 0.298 and 0.527, respectively) were also not significantly different. The mean count of newly formed veins was more at long-term than short-term follow-up (5.5 ± 2.5 vs. 5.1 ± 2.4, P = 0.005). Subgroup analysis of pediatric patients and adults yielded the same results as in the whole series. Conclusion: After indirect bypass surgery, the major time window of arterial neoangiogenesis development was within 6 months after surgery. The general effect of revascularization was very similar in the short and long term; therefore, a follow-up angiography scheduled at 6 months after surgery is recommended. Growth of veins might continue after 6 months. [ABSTRACT FROM AUTHOR]

Background: This study aimed to elucidate the clinical features, surgical treatment and long-term outcomes of adolescent patients with moyamoya disease. Methods: We reviewed consecutive patients with moyamoya vasculopathy who were admitted to our hospital from 2009 to 2015 to identify adolescent patients with moyamoya disease. Clinical features, surgical treatment and long-term outcomes were analyzed. Follow-up was performed by face-to-face or structured telephone interviews. Outcome measures were future stroke events. We performed univariate and multivariate time-to-event analyses to identify risk factors associated with future stroke events. Results: A total of 95 adolescent patients with moyamoya disease (age at onset, 13.1 ± 2.3 years) were included in this study. During follow-up, 12 patients (12.6%) had stroke events. We found that the patients who underwent direct/combined bypass had a significantly lower risk of future strokes [hazard ratio (HR), 0.16; 95% confidence interval (CI), 0.03-0.74; P = 0.019] compared to patients who underwent indirect bypass. Conclusions: Our results demonstrate that direct/combined bypass can be more effective in preventing future strokes than indirect bypass in adolescent patients with moyamoya disease. [ABSTRACT FROM AUTHOR]