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Background: Moyamoya disease (MMD) is characterized by an abundance of moyamoya vessels; however, the precise mechanism driving the spontaneous angiogenesis of these compensatory vessels remains unclear. Previous research has established a link between the stromal cell-derived factor-1 (SDF-1)/ CXC receptor 4 (CXCR4) axis and angiogenesis under hypoxic conditions. Nevertheless, the alterations in this axis within the cerebrospinal fluid, arachnoid membranes and vascular tissue of MMD patients have not been fully investigated., Methods: Our study enrolled 66 adult MMD patients and 61 patients with atherosclerotic vascular disease (ACVD). We investigated the SDF-1 concentration in cerebrospinal fluid (CSF) and CXCR4 expression level on the arachnoid membranes and vascular tissue. We utilized enzyme-linked immunosorbent assay and immunohistochemistr. Additionally, we cultured and stimulated human brain microvascular endothelial cells (HBMECs) and smooth muscle cells (SMCs) under oxygen and glucose deprivation (OGD) conditions followed by reoxygenation, to examine any changes in the SDF-1/CXCR4 axis., Results: The results demonstrated an elevation in the level of SDF-1 in CSF among MMD patients compared to those with ACVD. Moreover, the expression of CXCR4 in arachnoid membranes and vascular tissue showed a similar trend. Furthermore, the content of CXCR4 in HBMECs and SMCs increased with the duration of ischemia and hypoxia. However, it was observed that the expression of CXCR4 decreased at OGD/R 24h compared to OGD 24h. The temporal pattern of SDF-1 expression in HBMECs and SMCs mirrored that of CXCR4 expression., Conclusion: These findings indicate a critical role for the SDF-1/CXCR4 axis in the angiogenesis of moyamoya disease., Competing Interests: Declaration of competing interest The authors have no relevant financial or non-financial interests to disclose., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Moyamoya disease (MMD) is a chronic cerebrovascular disease with unknown etiology. The pathogenesis of vascular changes remains unclear. Ischemic and hemorrhagic adult MMD patients and healthy volunteers were enrolled to collect serum for data-independent acquisition (DIA)-based proteomic analysis and ELISA validation. DIA serum proteomic revealed that apolipoprotein C-I (APOC1), apolipoprotein D (APOD), and apolipoprotein A-IV (APOA4) were decreased. The reductases glutathione S-transferase omega-1 (GSTO1) and peptidyl-prolyl cis-trans isomerase A (PPIA) were upregulated, and ADAMTS-like protein 4 (ADAMTSL4) was downregulated in both ischemic and hemorrhagic MMD. Afamin (AFM) and transforming growth factor-beta-induced protein ig-h3 (TGFBI) increased in ischemic patients but decreased in hemorrhagic patients. Serum ELISA results confirmed that APOA4, APOC1, and APOD were decreased compared to controls. Then, we retrospectively analyzed biochemical indexes of 200 MMD patients. A total of 54 enrolled MMD patients showed decreased total cholesterol (TC) and high-density lipoprotein cholesterol (HDL-c). APOA4, APOC1, and APOD were vital factors in the HDL decrease in MMD patients. Lipoprotein dysfunction in MMD patients is involved in MMD. Intimal thickening by enhanced adhesion, middle layer vascular smooth muscle cell migration, and decreased lipid antioxidant function represented by HDL are potential pathogeneses of vascular changes in MMD., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: To assess the cerebral hemodynamic data associated with transient cerebral hemodynamic disturbance (TCHD), including cerebral hyperperfusion syndrome (CHS) and watershed shift ischemia (WSI), by whole-brain (WB) computed tomography perfusion (CTP) before and after revascularization for ischemic moyamoya disease., Methods: A total of 115 consecutive patients with ischemic moyamoya disease underwent revascularization. All patients underwent WB-CTP 24 hours before operation and on the day of onset of TCHD and 6 months after revascularization. The volumes of delay time (DT) >3 seconds and mismatch and relative cerebral blood flow <30% were calculated in 3 time points., Results: Of the 115 patients, 18 115 had TCHD, comprising 10 with CHS and 8 with WSI. Compared with the brain volume of DT >3 seconds before revascularization, the volume decreased significantly (P < 0.05) on the day of CHS. The volume of mismatch in 3 time points indicated no significant differences (P > 0.05). The volume of relative cerebral blood flow <30% showed obvious differences of significance among 10 patients with CHS (P < 0.05) at 3 time points. In the WSI group, the volume of DT >3 seconds, mismatch, and DT >3 seconds showed significant differences, relatively (P < 0.05), at 3 time points. At the time of onset of TCHD, DT >3 seconds and mismatch in the CHS group were dramatically lower than those in the WSI group (P < 0.05). DT >3 seconds in the no-TCHD group showed significant differences (P < 0.05) at 3 time points., Conclusions: WB-CTP could be used to assess the cerebral hemodynamic characteristics before and after revascularization. DT >3 seconds and mismatch played important roles in evaluating distinct features of TCHD., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background: Recently, several studies have focused on the relationship between blood-brain barrier (BBB) impairment and the etiology of Moyamoya disease (MMD). However, in vivo studies investigating about BBB impairment and cortical perfusion in MMD patients were really rare., Methods: This study included 16 patients diagnosed with MMD and 9 patients with atherosclerotic cerebrovascular disease (ACVD); all of who were treated with superficial temporal artery-middle cerebral artery (STA-MCA) bypass. Cortical perfusion was assessed using intraoperative indocyanine green (ICG) videoangiography by calculating the blood flow index (BFI). In addition, we used sodium fluorescein (NaFl) to evaluate the permeability of BBB in vivo during operation., Results: The results showed that BBB impairment in MMD patients was more significant than that in ACVD patients, whereas, the cortical perfusion was comparable between two groups. BFI was significantly improved after STA-MCA bypass both in the MMD group (post-operation vs pre-operation: 109.2 ± 67.7 vs 64.3 ± 35.0, p = 0.004) and the ACVD group (post-operation vs pre-operation: 137.6 ± 89.6 vs 90.8 ± 58.3, p = 0.015). Moreover, BFI was significantly decreased in the cortex with BBB impairment as compared with that in the cortex with intact BBB (impaired BBB vs intact BBB: 55.7 ± 26.5 vs 87.6 ± 55.1, p = 0.025). Following bypass, the cortical perfusion significantly improved in the area of BBB impairment (post-operation vs pre-operation: 93.8 ± 75.2 vs 55.7 ± 26.5, p = 0.004), which was not observed in the BBB intact area (post-operation vs pre-operation: 92.4 ± 50.4 vs 87.6 ± 55.1, p = 0.58)., Conclusion: In summary, we observed that BBB impairment in MMD patients was more significant than that in ACVD patients. This study also demonstrated for the first time that cortical perfusion was significantly decreased in the cortex with BBB impairment as compared with that in the cortex with intact BBB in MMD patients. We also observed that After STA-MCA bypass, the cortical perfusion was significantly improved in the cortex with BBB impairment. These results may provide a new insight for BBB impairment and cortical perfusion in the etiology of MMD.

Ischemic moyamoya disease (MMD) can be treated with the revascularization of superficial temporal artery to middle cerebral artery (STA-MCA) bypass combined with encephalo-duro-arterio-myo-synangiosis (EDAMS) effectively. The purpose of the present study was to quantify the revascularization of STA-MCA bypass combined with EDAMS via whole-brain computed tomography perfusion (WB-CTP).Seventy-nine consecutive patients with ischemic MMD who admitted to our hospital from August 2012 to October 2018 were carried out STA-MCA bypass combined with EDAMS. WB-CTP was performed at 24 hours prior to operation and 3 months following bypass with a follow-up WB-CTP, respectively. Both automatic analysis of WB-CTP (MIStar, Apollo Medical imaging Technology, Melbourne, Australia) for analyzing values of brain volume in delayed time (DT) >3 seconds and DT > 6 seconds, relative cerebral blood flow (γCBF) < 30% and its mismatch ratio or percentage and diffusion-weighted imaging of magnetic resonance imaging in the ischemic penumbra and the infarct core at the 2 time points were studied for verifying the effectiveness of the combined revascularization. Changes in DT values at MCA-terminal territory after revascularization had been investigated. The dynamic data were with reference to the individual cerebellar arteries.All patients with ischemic MMD underwent STA-MCA bypass combined with EDAMS successfully. The preoperative brain volume in DT > 3 seconds in MCA-terminal territory was significantly larger than that of postoperative one (P < .05) in the ischemic penumbra in ischemic MMD. The mismatch ratio in brain volume of 24 hours prior to revascularization in MCA-terminal territory was significantly lower than that of 3 months (P < .05) following combined revascularization. The percentage of mismatch in brain volume of 24 hours prior to revascularization vs that of 3 months and the value of γCBF < 30% were similar to the above mismatch ratio (P < .05). The ratio of postoperative brain volume in DT > 3 seconds vs DT > 6 seconds indicated no significant differences compared with that of preoperative one (P > .05).The WB-CTP can be regarded as a choice for quantifying the combined revascularization in the ischemic penumbra and the infarct core in ischemic MMD. As proposed methods, brain volume in DT > 3 seconds, the value of γCBF < 30% and mismatch ratio in brain volume in MCA-terminal territory should be paid more attention in assessing the validity of STA-MCA bypass combined with EDAMS in ischemic MMD.

Background: Extracranial internal carotid artery (EICA) aneurysm is regarded as a rare lesion. What is more, patients can suffer from EICA aneurysms and hemorrhagic moyamoya disease (MMD) simultaneously., Case Description: A 38-year-old man was admitted to our hospital and underwent emergent tracheal intubation for hoarseness and breathing difficulty for 1 day. He had a pulsating mass of 2.5 cm in diameter in the left cervical area. Imaging examinations revealed the left EICA aneurysm and MMD. The patient underwent left superficial temporal artery-to-middle cerebral artery (STA-MCA) bypass and ligation of the left EICA simultaneously. Computed tomography revealed hemorrhagic stroke in the right basal ganglia region 20 days after the bypass. The hematoma disappeared 20 days after the stroke. Right bypass was performed 6 months after the left bypass. The patient was discharged with no neurologic abnormalities 10 days after the right bypass. He lived a normal life, and no neurologic abnormalities were found at the 6-month follow-up. Imaging examinations indicated the efficacy of the bilateral STA-MCA bypass and verified the disappearance of the left EICA aneurysm after the ligation., Conclusions: We present a case of EICA aneurysm and hemorrhagic MMD. Ligation of the left ECA eliminates the EICA aneurysm with no neurologic abnormalities. STA-MCA bypass can be regarded as an effective choice for hemorrhagic MMD. Further studies should be performed to confirm the effectiveness of combined surgical revascularization., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Pure arterial malformations (PAMs) are extremely rare abnormalities defined as dilated, overlapping, and tortuous arteries with a coil-like appearance in the absence of venous components. Over the last half century, only seven published reports have described cases of patients with PAMs who received treatment. Methods: Here, we report two cases of women with PAMs who received surgical treatment, and we present a systematic review of the literature. We searched the PubMed, Embase, Web of Science, and Medline databases (up until October 1, 2021) for relevant publications. We performed independent-sample t -tests and Fisher's exact tests to compare continuous and categorical characteristics among the available cases. Results: Our first patient was a 43-year-old woman with PAM of the left internal carotid artery (ICA), who received an ICA-radial artery (RA)-M2 bypass. Post-operative digital subtraction angiography (DSA) revealed the disappearance of the left ICA PAM without ischemic events during follow-up. The second patient was a 53-year-old woman with PAMs of the right ICA and posterior cerebral artery. The P1 lesion was treated by proximal occlusion combined with a superficial temporal artery-P2 bypass. During the 12-month follow-up period, the size of the PAMs decreased significantly as indicated by the post-operative DSA showing the absence of hemorrhages. Our systematic review, which includes 56 PAMs, shows that the reported PAMs were more common in the anterior circulation (33/56, 58.9%) than in the posterior circulation (11/56, 19.7%). Bilateral PAMs were more likely to affect bilateral anterior cerebral arteries (ACA) (ACAbilateral vs. ACAunilateral: 63.6 vs. 26.2%, p = 0.02). In addition, PAMs involving the anterior circulation were likely to affect multiple arteries (anteriormulti vs. posteriormulti: 30.3 vs. 0%, p = 0.038). Conclusion: We found very few reports on treated PAMs; further studies with large sample sizes and long follow-up periods are required to explore the appropriate treatment strategy for PAMs. [ABSTRACT FROM AUTHOR]

Objective: This study compared the clinical features and hemodynamic characteristics of patients in different Suzuki stages of ischemic moyamoya disease (iMMD) before and after treatment with extracranial-intracranial (EC-IC) bypass surgery combined with encephalo-duro-myo-synangiosis and whole-brain computed tomography perfusion (WB-CTP). Methods: A total of 126 patients in different Suzuki stages (II, III, IV, and V) of iMMD who underwent bypass surgery from April 2013 to August 2020 were included in this retrospective study. MIStar automatic analysis of Whole brain CT perfusion imaging software (WB-CTP, Apollo Medical Imaging Technology, Melbourne, Australia) was used. The patients also underwent WB-CTP 1 day before and 1 week and 3 months after the surgery. The relationships between hemodynamic parameters in WB-CTP including delay time (DT) > 3 s, relative cerebral blood flow (rCBF) < 30%, mismatch and mismatch ratio, and clinical outcomes were evaluated for different Suzuki stages, with comparisons between early (II and III) and late (IV and V) stages. Results: Combined bypass surgery was performed in 161 hemispheres of 126 patients with iMMD. Brain volume with DT > 3 s was decreased 1 week (51.5 ± 11.8 ml, P < 0.05) and 3 months (41.5 ± 10.7 ml, P < 0.05) after bypass compared to 1 day before bypass (104.7 ± 15.1 ml) in early-stage patients. In late-stage patients, the volume was increased 1 week after bypass compared to the preoperative value (154.3 ± 14.7 vs. 118.3 ± 19.1 ml, P < 0.05). Preoperative brain volume with rCBF < 30% was lower (9.8 ± 3.9 vs. 33.5 ± 11.0 ml) whereas preoperative mismatch ratio was higher (11.2 ± 2.8 vs. 3.6 ± 1.6) in early-stage as compared to late-stage patients (both P < 0.05). A higher modified Rankin scale score (0–1) was achieved by early-stage patients than by those in the late stage (93.8 vs. 80.4%, P < 0.05) at the 3-month follow-up. Conclusions: WB-CTP is useful for assessing the effectiveness of combined bypass/revascularization in different Suzuki stages of iMMD. Patients in the early stage of disease with higher preoperative brain volume with DT > 3 s and mismatch ratio show greater improvements in hemodynamic parameters and fewer postoperative complications associated with hemodynamic disturbance following bypass than patients in the late stage. Preoperative mismatch ratio can serve as a marker for assessing the status of collateral circulation in different Suzuki stages of iMMD. [ABSTRACT FROM AUTHOR]

Cerebral revascularization is an effective measure for dealing with complicated intracranial aneurysms and ischemic cerebrovascular disease. Intraoperative thrombosis causing bypass occlusion is a severe issue that causes devastating consequences for complications in revascularization. We report our experiences regarding salvage maneuvers for intraoperative thrombosis in cerebral revascularization procedures and discuss the characteristics and culprits. We investigated 720 consecutive patients who underwent cerebral revascularization at First Affiliated Hospital of Soochow University from January 2013 to October 2021, including 688 patients who underwent superficial temporal artery (STA)−middle cerebral artery (MCA) bypass and 32 patients who underwent extracranial artery−radial artery (ECA-RA)−MCA bypass. Forty-one patients experienced intracranial aneurysms, and the remaining 679 patients were involved in moyamoya disease, skull base tumors, intracranial occlusive vascular diseases, or other cerebrovascular diseases. All clinical characteristics, clinical imaging examinations, and neurologic outcomes were studied preoperatively and postoperatively. The patency of bypasses was confirmed by intraoperative Doppler ultrasonography and indocyanine green videoangiography. Seven intraoperative thromboses, which were confirmed by intraoperative Doppler ultrasonography and indocyanine green videoangiography including STA-MCA bypass (n = 5) and ECA-RA-MCA bypass (n = 2) were observed in 720 patients who underwent cerebral revascularization. The anastomotic stoma remained patent in 6 of 7 patients with intraoperative thrombosis after treatment. One case in STA-MCA bypass failed to be salvaged. Of the 4 intraoperative thrombosis in STA-MCA bypass for moyamoya disease cases being successfully saved, 2 were done so by applying absorbable gelatin powder from absorbable gelatin sponge (Gelfoam, Pfizer, New York, New York, USA) around the site of the anastomosis to relieve the downward compression effect of the donor vessel (STA) on the recipient vessel (M4 segment of MCA). One case in ECA-RA-MCA bypass was salvaged by thrombectomy through donor arteriotomy (radial artery) and reanastomosis. The other case was salvaged by complete reanastomosis. All 7 patients who experienced intraoperative thrombosis achieved favorable outcomes at discharge and the 6-month follow-up. Various factors are responsible for intraoperative thrombosis in cerebral revascularization. Relieving the downward compression effect of the donor vessel STA on the recipient vessel M4 segment of MCA by applying Gelfoam around the site of the anastomosis stoma can be recommended to salvage the intraoperative thrombosis in cerebral revascularization. [ABSTRACT FROM AUTHOR]