Treatment Of Moyamoya Disease Research Articles

Incidence, prevalence, and treatment of Moyamoya disease in Japan: A population-based descriptive study

BackgroundMoyamoya disease (MMD) is characterized by progressive stenosis or occlusion of the terminal portions of the bilateral internal carotid arteries. A Japanese survey in 2003 reported an incidence and prevalence of MMD of 0.54 and 6.03 per 100,000 people, respectively, showing an upward trend over previous surveys. An update to these estimates is therefore warranted. Additionally, evidence is lacking on trends in revascularization and antiplatelet therapy in MMD patients. MethodsWe conducted a population-based descriptive study using a Japanese claims database. From fiscal year (FY) 2015 to 2019, we standardized the incidence and prevalence estimates of MMD to the 2015 Japanese census population by age and sex. We also estimated the 1-year cumulative incidence of revascularization among incident MMD patients and the proportion of prevalent MMD patients receiving antiplatelet therapy in each FY. ResultsThe age-standardized male-to-female ratio of both incident and prevalent MMD patients was approximately 1:2. Standardized incidence and prevalence of MMD per 100,000 population increased slightly from 1.8 to 2.4 and 14.7 to 17.6, respectively. The 1-year cumulative incidence of revascularization among incident MMD patients varied between 21.9 % and 28.9 %. Among prevalent MMD patients, 36.6 % to 39.0 % received antiplatelet therapy. ConclusionsThe incidence and prevalence of MMD in Japan from FY 2015 to 2019 were higher than those estimated in 2003. The trends in revascularization and antiplatelet therapy identified in this study will be useful in further improving the quality of MMD clinical practice.

Hemodynamic Effects of Tortuosity and Stenosis in Superficial Temporal Artery-Middle Cerebral Artery Bypass for Moyamoya Disease

BackgroundSuperficial temporal artery (STA)-middle cerebral artery (MCA) bypass surgery has been widely adopted in treating moyamoya disease (MMD). Geometric variations including high tortuosity and stenosis appear in many cases but the hemodynamic effects have not been comprehensively evaluated. AimWe aim to evaluate the hemodynamic effects of bypass geometry variations based on patient-specific data. Methods17 patients with MMD underwent STA-MCA bypass surgery with highly tortuous bypass geometry were included. For each patient, the original 3-dimensional structure of STA-MCA bypass was reconstruct from clinical imaging data. The bypass structure was virtually improved by removing the tortuosity and stenosis. Computational fluid dynamics (CFD) simulation was performed on both bypass structures under identical patient-specific condition. The simulated hemodynamic parameters of the bypass and its distal branches were compared between the original and virtually improved bypass geometries in all cases using paired t-test or Wilcoxon signed-rank test. The changes of hemodynamic parameters were compared between the cases with and without mild-to-moderate stenosis (44.0%-70.3% in diameter) in the bypass using t-test or Mann-Whitney U test. ResultsThe virtual improvement of bypass geometry significantly increased the flow rate of the bypass and its distal branches (p<0.05) and decreased the transcranial flow resistance (p<0.05). The hemodynamic changes in cases with stenosis removal were significantly higher than those without stenosis (p<0.05). ConclusionHigh tortuosity and stenosis can significantly change the hemodynamics of STA-MCA bypass, and the optimization of bypass geometry deserves further consideration.

A Functional Magnetic Resonance Imaging Study Using Dynamic Amplitude of Low-Frequency Fluctuation to Assess Brain Activity in Patients with Moyamoya Disease.

Introduction: The purpose of this study was to monitor and record the dynamic brain activity of patients with moyamoya disease (MMD), as well as to study the relationship between brain abnormalities and presenting clinical features. Methods: A total of 16 patients with MMD (2 males and 14 females) were invited to participate in the study, as were healthy controls (HCs) with the same number and sex ratio. In this study, the dynamic amplitude of low-frequency fluctuation (dALFF) was utilized to assess changes in spontaneous brain activity. Moreover, we also used correlation analysis to study the relationship among the measured mean of dALFF, behavioral performances, and the retinal nerve fiber layer and the Hospital Anxiety and Depression Scale (HADS) score to explore the potential relationship between MMD and anxiety and depression. Results: Our study reveals that in MMD, dALFF levels decreased in the left lingual gyrus, right insula, and occipital lobe. Discussion: In this study, we found and discussed the potential relationship between the abnormal activities in multiple brain regions and related functional network disorders in patients with MMD, as well as the damage to brain regions that process emotion and vision, in the hopes of providing more ideas for the clinical diagnosis and treatment of MMD.

Clinical effect of a modified superficial temporal artery-middle cerebral artery bypass surgery in Moyamoya disease treatment

BackgroundCerebral extracranial-intracranial (EC-IC) revascularization technique (superficial temporal artery-middle cerebral artery (STA-MCA) bypass grafting) has become the preferred surgical method for the treatment of Moyamoya disease (MMD). We attempted to completely free the two branches of the superficial temporal artery without disconnection. Extracranial and intracranial blood flow reconstruction were then modified by selectively performing a direct bypass technique on one branch and a patch fusion technique on the other of the STA based on the blood flow and the vascular diameter of the intracranial surface blood vessels.MethodsA series of modified STA-MCA bypass surgeries performed consecutively between March 2022 and March 2023 were reviewed and compared to conventional combined bypass surgeries performed during the same period. The following information was collected from all enrolled patients: demographic characteristics, clinical symptoms, and preoperative and postoperative imaging, including Suzuki stage and Matsushima grade. The modified Rankin scale (mRS) was used to assess the changes in neurological status before and after surgery.ResultsA total of 41 patients with Moyamoya disease (MMD) who underwent cerebral revascularization were included in this study, of which 30 were conventional revascularization and 11 were modified revascularization. The mean age was 49.91 years, and 18 (43.9%) of the patients were women. The modified group had a lower incidence of cerebral hyperperfusion syndrome (18.2%) than the conventional group (23.3%). After at least 3 months of follow-up, the bypass patency rate remained 100% in the modified group and 93.3% in the conventional group. All patients in the modified group achieved a better Matsushima grade (A + B), with six (54.5%) having an A and five (45.5%) having a B. In contrast, four patients (13.3%) in the conventional group had a Matsushima grade of C. In all, 72.8% of the modified group had postoperative mRS scores of 0 and 1, which was higher than that of the traditional group (63.3%).ConclusionThe improved STA-MCA bypass could provide blood flow to multiple cerebral ischemic areas, reduce excessive blood perfusion, and ensure blood supply to the scalp, with lower complications and better clinical benefits than the traditional combined bypass.

Research progress on moyamoya disease combined with thyroid diseases.

Moyamoya disease (MMD), also known as abnormal cerebral vascular network disease, is characterized by progressive occlusion or stenosis of the internal carotid and cerebral arteries, as well as the formation of an abnormal cerebral vascular network. It can occur anywhere in the world but is most common in China, Japan, and the Republic of Korea. In recent years, there have been increasing reports on the coexistence of thyroid diseases and MMD, but the mechanism of their coexistence is still unclear. For this article, we used keywords such as "moyamoya disease", "thyroid", "Grave disease", "thyrotoxicosis", and "thyroid autoimmune antibodies" to search for 52 articles that met the requirements in medical databases such as PubMed and Web of Science. This article also reviews the research on the role of thyroid hormone, the mechanism of immune antibodies, the possible correlation between thyroid diseases and MMD disease genes, and the treatment methods, and discusses the possible relationship between MMD and thyroid diseases to provide a reference for the pathogenesis and treatment of MMD with thyroid diseases.

Moyamoya – A progressive disorder an update and review

There is stenosis or narrowing of the internal carotid artery in Moyamoya disease, a relatively rare kind of neurological condition. This may result in illnesses like ischemia, haemorrhage, or even a potentially fatal stroke. It is still unclear what this disease's specific pathogenesis is understood. The Moyamoya disease has a total of six stages. Scientists have not yet developed a drug that can fully treat moyamoya disease because it is such a rare disorder. Surgery is the only approach that works here. But once more, this won't help with finishing the course of treatment. It will only aid in slowing the spread of the disease

Single-cell sequencing analysis of peripheral blood in patients with moyamoya disease

BackgroundAt present, the etiology of moyamoya disease is not clear, and it is necessary to explore the mechanism of its occurrence and development. Although some bulk sequencing data have previously revealed transcriptomic changes in Moyamoya disease, single-cell sequencing data has been lacking.MethodsTwo DSA(Digital Subtraction Angiography)-diagnosed patients with moyamoya disease were recruited between January 2021 and December 2021. Their peripheral blood samples were single-cell sequenced. CellRanger(10 x Genomics, version 3.0.1) was used to process the raw data, demultiplex cellular barcodes, map reads to the transcriptome, and dowm-sample reads(as required to generate normalized aggregate data across samples). There were 4 normal control samples, including two normal samples GSM5160432 and GSM5160434 of GSE168732, and two normal samples of GSE155698, namely GSM4710726 and GSM4710727. Weighted co-expression network analysis was used to explore the gene sets associated with moyamoya disease. GO analysis and KEGG analysis were used to explore gene enrichment pathways. Pseudo-time series analysis and cell interaction analysis were used to explore cell differentiation and cell interaction.ResultsFor the first time, we present a peripheral blood single cell sequencing landscape of Moyamoya disease, revealing cellular heterogeneity and gene expression heterogeneity. In addition, by combining with WGCNA analysis in public database and taking intersection, the key genes in moyamoya disease were obtained. namely PTP4A1, SPINT2, CSTB, PLA2G16, GPX1, HN1, LGALS3BP, IFI6, NDRG1, GOLGA2, LGALS3. Moreover, pseudo-time series analysis and cell interaction analysis revealed the differentiation of immune cells and the relationship between immune cells in Moyamoya disease.ConclusionsOur study can provide information for the diagnosis and treatment of moyamoya disease.

Encephalo-Duro-Pericranio-Synangiosis for the Treatment of Moyamoya Disease with Posterior Cerebral Artery Lesions

We describe our experience performing encephalo-duro-pericranio synangiosis for the parieto-occipital region (EDPS-p) as a treatment for moyamoya disease (MMD) with hemodynamic disturbances caused by lesions of the posterior cerebral artery. From 2004 to 2020, 60 hemispheres of 50 patients with MMD (38/50 females, age 1-55 years) underwent EDPS-p as a treatment for hemodynamic disturbances in the parieto-occipital region. A skin incision was made on the parieto-occipital area to avoid the major skin arteries, and the pedicle flap was created by attaching the pericranium to the dura mater under the craniotomy with multiple small incisions. The surgical outcome was assessed on the basis of the following points: perioperative complications, postoperative improvement of clinical symptoms, subsequent novel ischemic events, qualitative assessment of the development of collateral vessels by magnetic resonance arteriography, quantitative assessment of postoperative perfusion improvement based on the mean transit time, and cerebral blood volume on dynamic susceptibility contrast imaging. Perioperative infarction occurred in 7/60 hemispheres (11.7%). The transient ischemic symptoms observed preoperatively disappeared in 39/41 hemispheres (95.1%) during the follow-up period (12-187 months), and none of the patients experienced novel ischemic events. Collateral vessels supplied from the occipital arteries, middle meningeal arteries, and posterior auricular arteries developed postoperatively in 56/60 hemispheres (93.3%). Postoperative mean transit time and cerebral blood volume showed significant improvement in the occipital, parietal, and temporal areas (P < 0.001), as well as the frontal area (P= 0.01). EDPS-p seems to be an effective surgical treatment for patients with MMD who suffer hemodynamic disturbances caused by posterior cerebral artery lesions.

Chinese expert consensus on the treatment of MMD

Moyamoya disease (MMD), also known as spontaneous occlusion of the circle of Willis, is defined by progressive stenosis or occlusion of the internal carotid arteries, and it can progress to the anterior, middle, and posterior cerebral arteries. As these arteries are gradually stenosed, a collateral network of capillaries develops at the base of the brain, producing the characteristic reticulate appearance (“puff of smoke”) on angiography. Therefore, it was named by Suzuki and Takaku in 1969 after the Japanese term “moyamoya” (Suzuki and Takaku, Arch Neurol 20:288–299, 1969). MMD is most common in East Asian countries such as Japan and Korea, and it shows a slight female predominance. MMD is mainly characterized by ischemia and hemorrhage. Hemorrhagic MMD is very rare in children, and most cases occur in adults due to the rupture of the compensatory blood vessels, which often leads to hemorrhagic symptoms (Scott and Smith, N Engl J Med 360:1226–1237, 2009). In recent years, the diagnosis rate has increased with the popularization of imaging techniques. However, the pathogenesis of MMD is still not completely understood, and there is currently no evidence to suggest that drug treatment can delay or even reverse the progression of MMD. The current drug treatment for in MMD only targets its clinical symptoms, including ischemia and hemorrhage. The main choice of treatment for MMD is surgical revascularization. As an increasing number of hospitals have developed surgical treatment for MMD, our compiling group has jointly discussed the formulation of a consensus among Chinese experts on the treatment of MMD.

Abstract TMP71: Hypoperfusion And Thromboembolism Are Codominant Stroke Mechanisms In Adult Moyamoya Disease

Background: Treatment of moyamoya disease (MMD) has predominantly focused on flow augmentation since the mechanism of ischemic stroke has largely been attributed to hemodynamic failure. Recent studies have challenged this notion suggesting that thromboembolism may play a dominant role. The aim of our study was to investigate the mechanisms of ischemic stroke in MMD by assessing the relationship between infarction patterns and Quantitative Magnetic Resonance Angiography (QMRA) flow state. Methods and Results: This is a retrospective study of adult patients with suspected MMD that presented with MRI confirmed acute ischemic stroke predating or following QMRA by a maximum of six months, between 2009 and 2021. Of the 177 consecutive patients with MMD that underwent QMRA, 35 patients met inclusion, consisting of 41 hemispheres. Flow-status was dichotomized into low-flow and normal-flow based on published criteria. Mixed pattern was the most frequent finding (70.7%), followed by embolic (17.1%), perforator (7.3%), and internal borderzone (IBZ) (4.9%). Infarction patterns were then dichotomized into IBZ+ (internal borderzone alone or mixed) and IBZ- (no internal borderzone constituent) based on the constituents of the patterns. Low-flow states were insignificantly more frequent with IBZ+ compared to IBZ- (48.4% vs. 20.0%, p = 0.14). Ipsilateral PCA fractional flow was significantly higher with IBZ+ compared to IBZ- (345.0% vs. 214.7%, p = 0.04). Conclusions: Mixed infarction pattern was the most common pattern of infarction in patients with MMD, implying that at least 2 interrelated stroke mechanisms of hypoperfusion and thromboembolism likely coincide. An association between ICA flow status and the pattern of infarction was not found. Prospective studies evaluating the utility of antithrombotic agents in MMD are needed.

Efficacyassessment of superficial temporal artery-middle cerebral artery bypass surgery in treating moyamoya diseasefrom a hemodynamic perspective: a pilot study using computational modeling and perfusion imaging.

Superficial temporal artery-middle cerebral artery (STA-MCA) bypass is a common surgery in treating moyamoya disease (MMD) with occluded MCA. Computational fluid dynamics (CFD) simulation might provide a simple, non-invasive, and low-cost tool to evaluate the efficacy of STA-MCA surgery. We aim to quantitatively investigate the treatment efficacy of STA-MCA surgery in improving the blood flow of MMD patients using CFD simulation. This retrospective study included 11 MMD patients with occlusion around proximal MCA who underwent STA-MCA bypass surgery. CFD simulation was performed using patient-specific blood pressure and postoperative artery geometry. The volumetric flow rates of STA and the bypass, average flow velocity in the proximal segment of transcranial bypass, transcranial pressure drop, and transcranial flow resistance were measured and compared with a postoperative increment of cerebral blood flow (CBF) in MCA territories derived from perfusion imaging. Per-branch pressure drop from model inlet to bypass branch outlet was calculated. The volumetric flow rates of STA and the bypass were 80.84 ± 14.54mL/min and 46.03 ± 4.21mL/min. Average flow velocity in proximal bypass, transcranial pressure drop, and transcranial flow resistance were 0.19 ± 0.07m/s, 3.72 ± 3.10mmHg, and 6.54 ± 5.65 10-8Pasm-3. Postoperative mean increment of CBF in MCA territories was 16.03 ± 11.72mL·100g-1·min-1. Per-branch pressure drop was 10.96 ± 5.59mmHg and 7.26 ± 4.25mmHg in branches with and without stenosis. CFD simulation results are consistent with CBF observation in verifying the efficacy of STA-MCA bypass, where postoperative stenosis may influence the hemodynamics.

Comprehensive molecular analyses of an autoimmune-related gene predictive model and immune infiltrations using machine learning methods in moyamoya disease.

Background: Growing evidence suggests the links between moyamoya disease (MMD) and autoimmune diseases. However, the molecular mechanism from genetic perspective remains unclear. This study aims to clarify the potential roles of autoimmune-related genes (ARGs) in the pathogenesis of MMD. Methods: Two transcription profiles (GSE157628 and GSE141025) of MMD were downloaded from GEO databases. ARGs were obtained from the Gene and Autoimmune Disease Association Database (GAAD) and DisGeNET databases. Differentially expressed ARGs (DEARGs) were identified using "limma" R packages. GO, KEGG, GSVA, and GSEA analyses were conducted to elucidate the underlying molecular function. There machine learning methods (LASSO logistic regression, random forest (RF), support vector machine-recursive feature elimination (SVM-RFE)) were used to screen out important genes. An artificial neural network was applied to construct an autoimmune-related signature predictive model of MMD. The immune characteristics, including immune cell infiltration, immune responses, and HLA gene expression in MMD, were explored using ssGSEA. The miRNA-gene regulatory network and the potential therapeutic drugs for hub genes were predicted. Results: A total of 260 DEARGs were identified in GSE157628 dataset. These genes were involved in immune-related pathways, infectious diseases, and autoimmune diseases. We identified six diagnostic genes by overlapping the three machine learning algorithms: CD38, PTPN11, NOTCH1, TLR7, KAT2B, and ISG15. A predictive neural network model was constructed based on the six genes and presented with great diagnostic ability with area under the curve (AUC) = 1 in the GSE157628 dataset and further validated by GSE141025 dataset. Immune infiltration analysis showed that the abundance of eosinophils, natural killer T (NKT) cells, Th2 cells were significant different between MMD and controls. The expression levels of HLA-A, HLA-B, HLA-C, HLA-DMA, HLA-DRB6, HLA-F, and HLA-G were significantly upregulated in MMD. Four miRNAs (mir-26a-5p, mir-1343-3p, mir-129-2-3p, and mir-124-3p) were identified because of their interaction at least with four hub DEARGs. Conclusion: Machine learning was used to develop a reliable predictive model for the diagnosis of MMD based on ARGs. The uncovered immune infiltration and gene-miRNA and gene-drugs regulatory network may provide new insight into the pathogenesis and treatment of MMD.

Outcomes after superficial temporal artery-middle cerebral artery anastomosis combined with multiple burr hole surgery and dural inversion synangiosis for moyamoya disease in adults.

Several forms of cerebral revascularization have been carried out to treat moyamoya disease, however, the existing methods are accompanied by a variety of complications. In this study, the authors aimed to evaluate the clinical and angiographic outcomes of a new surgical procedure: superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis combined with multiple burr hole (MBH) surgery and dural inversion synangiosis for the treatment of moyamoya disease in adults. Patients treated for moyamoya disease from August 2019 to July 2021 were retrospectively reviewed. Clinical data, including perioperative complications and follow-up outcomes, were noted. Preoperative and postoperative angiograms were compared, and the diameters of the frontal branch of the superficial temporal artery (F-STA), the deep temporal artery (DTA), the distal superficial temporal artery (STA) before the bifurcation and the middle meningeal artery (MMA) were measured on preoperative and postoperative angiograms. Meanwhile, a Matsushima score was assigned from postoperative angiograms. This study included 66 patients (67 hemispheres). During the follow-up period, a median of 18 (IQR, 13-21) months, no stroke or death occurred in any of the patients. The clinical outcomes were excellent in 27 patients (40.9%), good in 34 patients (51.6%), fair in 4 patients (6.0%), and poor in 1 patient (1.5%); the overall rate of favorable clinical outcomes (excellent and good) was 92.5%. The modified Rankin Scale (mRS) score was significantly improved at follow-up (P < 0.001). There were 41 hemispheres imaged by cerebral angiography after the operation, at a median postoperative interval of 9 (IQR, 8-12) months; among them, 34 (82.9%) hemispheres had Matsushima scores of grade A and grade B. The average postoperative diameters in the STA, DTA and MMA were increased significantly in 41 hemispheres at follow-up (P < 0.001). Sixteen (24.2%) patients suffered from perioperative complications, including focal hyperperfusion syndrome (HS) in 8 (12.2%) patients, cerebral infarction in 3 (4.5%) patients (including one case accompanied by wound infection), cerebral hemorrhage in 2 (3.0%) patients, seizures in 2 (3.0%) patients, and subdural effusion in 1 (1.5%) patient. The procedure of STA-MCA anastomosis combined with MBH surgery and dural inversion synangiosis may be a safe and effective treatment for adult patients with moyamoya disease.

Bloody fluids located between the temporal muscle and targeted cerebral cortex affect the establishment of indirect collaterals in Moyamoya disease with surgical bypass: A case-control study.

Bypass yields favorable outcomes in the treatment of Moyamoya disease (MMD). Bloody fluids accumulate between the targeted cortex and the temporal muscle after surgical bypass. These fluids are handled empirically via subcutaneous tubes or conservative treatments. However, substances located in certain positions may adversely affect the establishment of indirect collaterals (ICs) from muscular grafts. Patients in our hospital from January 2014 to December 2019 were eligible for inclusion. Digital subtraction angiography (DSA) and radiological examinations were used during the perioperative and follow-up periods. Bloody fluid volumes were calculated using computed tomography- (CT-) based 3D Slicer software. The characteristics of bloody fluids, patient demographics, and clinical data were retrospectively analyzed. In total, 110 patients underwent indirect or combined bypass with follow-up DSA. The mean age of the enrolled patients was 42.4 ± 11.8 years. Previous ischemia (p = 0.001), previous hemorrhage (p = 0.013), bloody fluid volume (p = 0.049), and the time of imaging (p = 0.081) were associated with indirect outcomes. Ordinal regression analysis confirmed that good indirect outcomes were associated with previous ischemia (p < 0.001) and a large bloody fluid volume (p = 0.013). Further subgroups based on fluid volume were significantly correlated with IC establishment (p = 0.030). A large bloody fluid volume and previous ischemic history were associated with good indirect outcomes. The presence of bloody fluids may reflect impaired degrees of muscular donors due to bipolar electrocoagulation, thus highlighting the importance of appropriate application of bipolar forceps.

Angiographic Characteristics of Cerebral Perfusion and Hemodynamics of the Bridging Artery After Surgical Treatment of Unilateral Moyamoya Disease.

PurposeTo investigate the characteristics of cerebral perfusion and hemodynamics of bypass grafting in the treatment of moyamoya disease (MMD) using the iFlow color-coded flow map in comparison with magnetic resonance imaging–perfusion-weighted imaging (MRI–PWI) and computational fluid dynamic (CFD) analysis.Materials and MethodsPatients with MMD treated with bypass grafting who had undergone MRI PWI and digital subtraction angiography for iFlow color-coded map was retrospectively enrolled and CFD was performed for calculating the hemodynamic stresses around the bypass grafting.ResultsForty-five patients with unilateral MMD treated with bypass surgery were enrolled. The bypass surgery was successful in all patients, with no severe neurological complications during the periprocedural period. Followed up for 4–12 months (median 5.5), the neurological function was good in all patients. The cerebral blood flow (CBF), cerebral blood volume (CBV), mean transit time (MTT), and time to peak (TTP) were significantly (p < 0.05) improved in the middle cerebral artery distribution area on the surgical side before and after vascular bypass, and the difference of TTP (s) measured from the proximal bifurcation of common carotid artery to the confluence of sinus was also significant (p < 0.05). A significant (p < 0.05) positive correlation existed in the perfusion parameters between the iFlow blood perfusion and the MRI–PWI perfusion, with r-value for TTP of 0.765 (p < 0.01). The iFlow color-coded blood flow map showed warm color changes on the diseased side, similar to those on the contralateral side. In CFD analysis, the hemodynamic stresses were all improved, in and around the bypass grafting and distal vessels, which were beneficial to blood flow entering distal arterial branches.ConclusionThe iFlow color-coded flow map can be used to analyze cerebral perfusion after bypass grafting for MMD, similar to MRI–PWI, and CFD can be used to analyze the hemodynamics after bypass grafting, revealing improved hemodynamics to promote blood flow entering distal arteries.

Clinical analysis of superficial temporal artery ⁃ middle cerebral artery bypass combined with temporoparietal fascia in the treatment of moyamoya disease

Clinical analysis of superficial temporal artery ⁃ middle cerebral artery bypass combined with temporoparietal fascia in the treatment of moyamoya disease

Research advances in the diagnosis and treatment of moyamoya disease: a bibliometric analysis

Moyamoya disease is a rare cerebrovascular condition involving stenotic carotid arteries and the formation of abnormal blood vessels. In this study, we aimed to characterize the key players involved in moyamoya research at the individual and institutional level and to identify the critical publications that have advanced our understanding of this disease. We performed a title-specific search of the Web of Science database using the search term "moyamoya" for publications dating from 1900 to April 2020. The 100 most frequently cited articles were obtained, screened for duplicates, and reviewed by 2 independent reviewers. These 100 articles were cited an average of 150 times each (range, 74 to 1,360 citations per article). Publication dates ranged from 1969 to 2016, with the largest number of publications (n=40) cited between 2000 and 2009. The article with the greatest number of citations (1,360 citations) was "Cerebrovascular 'moyamoya' disease: disease showing abnormal net-like vessels in base of brain," by Suzuki and Takaku, published in the Archives of Neurology, 1969. Stroke published the greatest number of the most frequently cited articles (23 of 100). The institution that contributed the most articles was Tohoku University (16%); the majority of the most frequently cited articles originated in Japan (62%). We identified the 100 most cited articles on moyamoya disease over the past 51 years to recognize significant and impactful works. These results can be used as a guide to evaluate our current understanding of moyamoya disease and to direct future efforts.

Abstract TMP80: Intraoperative Blood Flow Changes Predict Angiographic Outcomes After Direct Cerebral Revascularization For Moyamoya Disease

Introduction: Moyamoya Disease (MMD) is a progressive steno-occlusive vasculopathy of the large intracranial vessels precipitating ischemic and hemorrhagic events. Cerebral revascularization is the mainstay of treatment in MMD. Intraoperative measurements of cerebral blood flow following direct intracranial bypass may be performed to quantitatively assess the anastomosis. Objectives: To correlate intraoperative flow parameters in direct cerebral bypass for moyamoya disease to postoperative angiographic and clinical outcomes. Methods: Consecutive patients undergoing direct superficial temporal artery (STA) to middle cerebral artery (MCA) bypass were retrospectively analyzed. Preoperative and 6-month postoperative patient data was collected including presenting symptoms, MRI and angiographic findings, neurological complications, and functional outcome. Intraoperative cerebral blood flow was measured from the donor and recipient arteries using a micro flow probe pre- and post-anastomosis. Results: A total of 50 direct revascularization procedures in 39 patients were analyzed, of which 40 were in females (80%). The mean flow prior to anastomosis in the M4 MCA was +2.2 ± 6.1 ml/minute and +26.8 ± 19.0 ml/min in the STA. Post-anastomosis, the mean flow was -10.9 ± 14.8 ml/min (towards the Sylvian Fissure) in the proximal M4 and +7.9 ± 9.2 ml/min in the distal M4. At 6-month follow-up angiography, the graft was patent in all cases, with 98% demonstrating direct supply. A component of indirect supply was observed in 56% of grafts. Matsushima grade A, B, and C revascularization were obtained in 64%, 20%, and 16% of cases, respectively. Amongst Matsushima grade A patients, low flow was observed in the M4 (mean 1.7 ± 4.8 ml/min) prior to bypass, which became retrograde following bypass, measuring -13.5 ± 16.0 ml/min. Lower post bypass flow was observed in grade B and C patients, with -6.8 ± 13.1 ml/min and -5.6 ± 10.3 ml/min, respectively. Postoperative stroke occurred in one patient, who had a Matsushima grade C. Conclusions: Greater intraoperative retrograde flow in the recipient MCA immediately following anastomosis was observed in patients achieving a higher Matsushima grade.

Progression in Moyamoya Disease: Clinical Features, Neuroimaging Evaluation, and Treatment.

Moyamoya disease (MMD) is a chronic cerebrovascular disease characterized by progressive stenosis of the arteries of the circle of Willis, with the formation of collateral vascular network at the base of the brain. Its clinical manifestations are complicated. Numerous studies have attempted to clarify the clinical features of MMD, including its epidemiology, genetic characteristics, and pathophysiology. With the development of neuroimaging techniques, various neuroimaging modalities with different advantages have deepened the understanding of MMD in terms of structural, functional, spatial, and temporal dimensions. At present, the main treatment for MMD focuses on neurological protection, cerebral blood flow reconstruction, and neurological rehabilitation, such as pharmacological treatment, surgical revascularization, and cognitive rehabilitation. In this review, we discuss recent progress in understanding the clinical features, in the neuroimaging evaluation and treatment of MMD.

Insights into potential targeted nonsurgical therapies for the treatment of moyamoya disease.

Since its initial description in 1957 as an idiopathic disease, moyamoya disease has proved challenging to treat. Although the basic pathophysiology of this disease involves narrowing of the terminal carotid artery with compensatory angiogenesis, the molecular and cellular mechanisms underlying these changes are far more complex. In this article, the authors review the literature on the molecular and cellular pathophysiology of moyamoya disease with an emphasis on potential therapeutic targets.