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Background : Using two case reports of adult women with moyamoya disease presenting with intracranial hemorrhage from ruptured aneurysms on moyamoya collateral vessels, we aim to demonstrate the potential for effective endovascular treatment navigated by CT angiography, digital subtraction angiography, and flat panel CT. Case 1 Presentation : A 45-year-old female patient with sudden onset of headache, followed by somnolency. CT scan showed a four-ventricle hematocephalus caused by a 27 × 31 × 17 mm hematoma located in the left basal ganglia. Angiography revealed a 3 mm aneurysm on hypertrophic lenticulostriate artery bridging the M1 occlusion. Selective catheterization and distal embolisation with acrylic glue was done. Case 2 Presentation : A 47-year-old woman was admitted for a sudden onset of severe headache, CT scan showed four-ventricle hematocephalus. A 4 mm aneurysm on the collateral vessel-anterior chorioidal artery bridging the closure of the terminal segment of the internal carotid artery was diagnosed as the source of bleeding. Selective catheterization and distal embolisation with acrylic glue was done. Conclusions : Selective embolisation of ruptured aneurysms on moya moya collaterals is a simple, effective, and safe procedure when relevant microcatheters are used with imaging software navigation such as 3D DSA, 3D road map and flat-panel CT.

Background: Moyamoya disease (MMD) is a significant cause of childhood stroke and transient ischemic attacks (TIAs). This study aimed to assess the safety and efficacy of remote ischemic conditioning (RIC) in children with MMD., Methods: In a single-center pilot study, 46 MMD patients aged 4 to 14 years, with no history of reconstructive surgery, were randomly assigned to receive either RIC or sham RIC treatment twice daily for a year. The primary outcome measured was the cumulative incidence of major adverse cerebrovascular events (MACEs). Secondary outcomes included ischemic stroke, recurrent TIA, hemorrhagic stroke, revascularization rates, and clinical improvement assessed using the patient global impression of change (PGIC) scale during follow-up. RIC-related adverse events were also recorded, and cerebral hemodynamics were evaluated using transcranial Doppler., Results: All 46 patients completed the final follow-up (23 each in the RIC and sham RIC groups). No severe adverse events associated with RIC were observed. Kaplan-Meier analysis indicated a significant reduction in MACEs frequency after RIC treatment [log-rank test (Mantel-Cox), P = 0.021]. At 3-year follow-up, two (4.35%) patients had an ischemic stroke, four (8.70%) experienced TIAs, and two (4.35%) underwent revascularization as the qualifying MACEs. The clinical improvement rate in the RIC group was higher than the sham RIC group on the PGIC scale (65.2% vs. 26.1%, P < 0.01). No statistical difference in cerebral hemodynamics post-treatment was observed., Conclusions: RIC is a safe and effective adjunct therapy for asymptomatic children with MMD. This was largely due to the reduced incidence of ischemic cerebrovascular events., (© 2024. The Author(s).)

Background and Objectives: Posterior cerebral artery involvement (PCAi) has been identified as an important factor related to poor prognosis in moyamoya disease (MMD). This study summarized the characteristics of children with MMD and PCAi, clarified the clinical course, identified prognostic predictors, and investigated the long-term effect of encephaloduroarteriosynangiosis for posterior circulation (EDAS-p)., Methods: We retrospectively reviewed all our pediatric MMD cases with follow-up angiograms from November 2003 to December 2016. PCAi was classified as early-onset at initial diagnosis and delayed-onset after anterior circulation revascularization. Multivariable data including clinical features, radiographic findings, and surgical outcomes were analyzed., Results: Among 570 children with MMD, 246 (43.2%) had PCAi, with 176 (30.9%) classified as early-onset PCAi. During a median follow-up period of 10 years, 17.8% (70/394) of patients without initial PCAi developed delayed-onset PCAi. The median time to detection of a new PCA lesion was 15.5 (range 7-110) months from initial diagnosis, with a median age of 10.5 (3-22). Younger age at onset, familial occurrence, advanced Suzuki stages, and preoperative infarctions were predictors of delayed-onset PCAi. EDAS-p was performed on 294 hemispheres of 195 patients with PCAi. Stroke-free survival was significantly higher in the EDAS-p group than in the non-EDAS-p group (99.0% vs 90.2%; p < 0.001 [Breslow test]; p = 0.001 [log-rank test]; median follow-up: 101 months)., Discussion: PCAi is not uncommon in children with MMD, underscoring the need for long-term close clinical monitoring, especially in patients with high-risk factors for PCA progression. EDAS-p may be a safe and effective procedure for preventing subsequent stroke in children with MMD and PCAi.

Background: Moyamoya 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., Methods: We 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., Results: The 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., Conclusions: The 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., Competing Interests: Declaration of competing interest KK has received research funds from AstraZeneca, Eisai Co., Ltd., Kyowa Kirin Co., Ltd., Mitsubishi Corporation, OMRON Corporation, Real World Data Co., Ltd., Sumitomo Pharma Co., Ltd., and Toppan Inc.; consulting fees from Advanced Medical Care Inc., JMDC Inc., LEBER Inc., Ubicom Holdings, Inc., and Shin Nippon Biomedical Laboratories Ltd.; executive compensation from Cancer Intelligence Care Systems, Inc.; honoraria from Kaken Pharmaceutical Co., Ltd., Kyoto University Original Co., Ltd., Pharma Business Academy, Shionogi & Co., Ltd. Toppan Inc.; and held stock in Real World Data Co., Ltd. The other authors report no conflicts., (Copyright © 2024. Published by Elsevier Inc.)

Moyamoya disease (MMD) is characterized by idiopathic, progressive stenosis of the circle of Willis and the terminal portion of the internal carotid arteries with the development of prominent small collateral vessels and a characteristic moyamoya or puff-of-smoke radiographic appearance. The incidence and prevalence of MMD varies by region, age, and sex, with higher rates in Asian and East Asian populations compared to North American or European populations. There is a bimodal distribution of patients diagnosed with MMD. Pediatric patients are more commonly diagnosed within the 1st decade of life, whereas adult patients present in the 5th or 6th decade of life. Overall, there is a nearly 2:1 female-to-male ratio. Ischemic symptoms are the most common presentation in pediatric and adult populations, but adult patients are nearly twice as likely to present with intracranial hemorrhage compared to their pediatric counterparts. Surgical revascularization is indicated in symptomatic cases, and antiplatelet therapy may be a useful adjunct to prevent recurrent symptoms. Direct and combined bypass procedures seem to be more effective in adults, whereas children respond well to indirect bypass. The identification of key genetic, molecular, and environmental factors including RNF213 and GUCY1A3 loss-of-function mutations, angiogenic growth factors, autoantibodies, CNS infections, and radiation exposure suggest multiple pathways for the development of moyamoya arteriopathy. Further research is needed to better understand the heterogeneity of pathogenetic mechanisms that lead to moyamoya and to identify novel therapeutic targets to prevent, stabilize, and treat MMD.

Background: Recent advances are in the genetics, diagnosis, pathophysiology, and management of moyamoya disease (MMD), and moyamoya syndrome (MMS), a term used to describe moyamoya-like vasculopathy associated with various systemic diseases or conditions., Summary: Ring finger protein (RNF213) has been reported to be a susceptibility gene not only for MMD but also for atherosclerotic intracranial arterial stenosis and ischemic stroke attributable to large artery atherosclerosis. The latest guidelines by the Research Committee on MMD of the Japanese Ministry of Health, Labor, and Welfare, removed limitations of the previous definition that required bilateral involvement of the intracranial carotid artery to make the diagnosis, given the increasing evidence of progression to bilateral involvement in unilateral MMD. 3-dimensional constructive interference in steady-state MRI is useful for the differential diagnosis of MMD from atherosclerosis. Recent advances in the pathophysiology of MMD suggest that genetic and environmental factors play important roles in vascular angiogenesis and remodeling via complex mechanisms. The latest Japanese Guidelines and American Scientific Statement described that antiplatelet therapy can be considered reasonable. Endovascular interventional stent placement fails to prevent ischemic events and does not halt MMD progression. In the Japan Adult Moyamoya trial, a randomized controlled trial for bilateral extracranial-intracranial direct bypass versus conservative therapy in patients with MMD, who had intracranial hemorrhage, recurrent bleeding, completed stroke, or crescendo transient ischemic attack was significantly fewer with direct bypass than with conservative care., Key Messages: This review presents updated information on genetics, diagnosis, pathophysiology, and treatment of adult MMD and MMS. Despite recent advances, many mysteries still exist in the etiologies of moyamoya vasculopathy. The diagnostic criteria and treatment guidelines have been updated but not yet been globally established. Ongoing and future studies investigating underlying pathophysiological mechanisms of MMD and MMS may clarify potentially effective medical, surgical, or endovascular treatments., (© 2024 The Author(s). Published by S. Karger AG, Basel.)

Background: Disease-specific interventions for management and health behavior implementation are needed to improve the health and quality of life of adolescents with moyamoya disease., Objective: This study aimed to develop a program for adolescents with moyamoya disease based on the salutogenesis theory, which focuses on the process of enhancing health through successful adaptation to external stressors, and to evaluate its effectiveness., Methods: A randomized controlled trial was performed according to the CONSORT guidelines. This preliminary research and experimental treatment were conducted at a Severance Hospital ward and outpatient clinic among 48 participants randomized into the intervention (seven sessions of salutogenesis program, n = 24) or the control group (one session of one-to-one moyamoya disease education program, n = 24) from September 6, 2018 to January 4, 2019. Changes in the following study outcomes were reported: "knowledge of moyamoya disease," "social support," "sense of coherence," "moyamoya disease health behavior," "stress," "depression," "subjective health status," "frequency of ischemic symptoms," and "quality of life"., Results: The salutogenesis program improved the knowledge and social support of adolescents with illness-related problems and helped them attain healthy behaviors and stress reduction. It was confirmed to be effective in improving their quality of life., Conclusions: The salutogenesis program for adolescents with moyamoya disease effectively improved the generalized resistance resources and sense of coherence in adolescents with moyamoya disease., Trial Registration: Korean Clinical Research Information Service registry, KCT0006869., Competing Interests: The authors have declared that no competing interests exist., (Copyright: © 2023 Yeom, Oh. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.)

Adult moyamoya disease and syndrome are rare disorders with significant morbidity and mortality. A writing group of experts was selected to conduct a literature search, summarize the current knowledge on the topic, and provide a road map for future investigation. The document presents an update in the definitions of moyamoya disease and syndrome, modern methods for diagnosis, and updated information on pathophysiology, epidemiology, and both medical and surgical treatment. Despite recent advancements, there are still many unresolved questions about moyamoya disease and syndrome, including lack of unified diagnostic criteria, reliable biomarkers, better understanding of the underlying pathophysiology, and stronger evidence for treatment guidelines. To advance progress in this area, it is crucial to acknowledge the limitations and weaknesses of current studies and explore new approaches, which are outlined in this scientific statement for future research strategies.

Purpose: Moyamoya disease (MMD) is a rare disease which has a high incidence of onset in adolescence. Disease self-management skills are imperative for adolescents with MMD. This study aimed to describe the systematic development, content, and usability of the Moyamoya Healthy Youth application (app), which was developed to enhance self-management skills for adolescents with MMD., Design and Methods: The theoretical grounding for the app was salutogenic model and the development process of the app was guided by the intervention mapping (IM) protocol. Results of each IM step were applied to the next step leading to the design of the app. Additionally, a pilot test was conducted to determine the usability of the app., Results: Following the salutogenic model, we identified the stressors, behaviors, and resources regarding managing symptoms of MMD by interviewing adolescents with MMD, their parents, and healthcare providers. Based on the findings of the interviews, we determined the program outcomes and performance objectives to improve the self-management of MMD in adolescents. The app was developed by translating the theoretical methods to achieve the performance objectives into practical strategies for delivering the program. A pilot test with eight participants showed satisfaction with the app in terms of its usefulness and ease of use., Conclusion: We delineated the development process of the Moyamoya Healthy Youth. Additionally, we presented the positive outcomes regarding the usability of the app., Practice Implications: The Moyamoya Healthy Youth app could benefit adolescents with MMD, by improving their self-management skills which are crucial for their health., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Inc. All rights reserved.)

The effect and safety of endovascular treatment of basilar tip aneurysms associated with moyamoya disease are unknown. This study was to investigate the safety and effect of endovascular treatment of basilar tip aneurysms associated with moyamoya disease. Patients with moyamoya disease concurrent with basilar tip aneurysms were retrospectively enrolled and treated with endovascular embolization. The clinical and angiographic data were analyzed. Thirty patients with a basilar tip aneurysm were enrolled, including 8 (26.67%) male and 22 (73.33%) female patients aged 38 to 72 years (mean 54.4 ± 8.15). Endovascular treatment was successfully performed in 29 (96.67%) patients but failed in 1 (3.33%). Immediately after embolization, aneurysm occlusion degree was Raymond-Roy grade I in 26 (89.66%), grade II in 2 (6.90%), and grade III in 1 (3.45%). Intraprocedural complications occurred in 2 (10%) patients, including aneurysm rupture in 1 (3.33%), leading to death of the patient, and stent thrombosis in 2 (6.67%) which was successfully treated with thrombolysis. At discharge, good clinical outcome (modified Rankin Scale 0-2) was achieved in 29 (96.67%) and death in 1 (3.03%). Follow-up was performed 6 to 26 months (median 15) in 27 (93.1%) patients. Aneurysm occlusion degree was Raymond-Roy grade I in 21 (77.78%) patients, grade II in 4 (14.81%), and grade III in 2 (7.41%), not significantly (P = .67) different from those immediately after embolization. Aneurysm recurrence was found in 4 patients (14.81%). The clinical outcome was modified Rankin Scale 0 to 2 in all 27 patients, not significantly different from that at discharge. Endovascular embolization can be performed safely and effectively for basilar tip aneurysms associated with moyamoya disease even though more advanced embolization techniques are necessary., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Moyamoya disease (MMD) is a chronic steno-occlusion cerebrovascular disease accompanied by the formation of the abnormal vascular network at the base of the brain. The etiology of MMD is not fully clarified. Lack of pathological specimens hinders the research progress. Induced pluripotent stem cells (iPSC) derived from patients with outstanding differentiation potential and infinite proliferation ability could conquer the problem of insufficient samples. The technology of iPSC holds the promise of clarifying the underlying molecular mechanism in the development of MMD. In this review, we summarized the latest progress and difficulties in the research of mechanism and detailed the application of iPSC in MMD, aiming to provide an outlook of iPSC in molecular mechanism and novel therapies of MMD., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Background: Childhood moyamoya disease (MMD) can lead to progressive and irreversible neurological impairment. Early age at onset is likely associated with a worst prognosis of the disease. The study aims to summarize the clinical characteristics of childhood MMD for supporting the diagnosis and treatment of early MMD., Methods: A retrospective study was conducted on children aged zero to 16 years who were diagnosed with MMD in the Department of Neurology and neurosurgery of our hospital from October 2016 to April 2020. The clinical characteristics of children with MMD were summarized for analysis, and the distribution of sex and initial attack type among different age groups was determined by data comparison., Results: The study surveyed 114 children (male to female sex ratio of 1:1.07) with MMD, and 6.1% of them had family history. The mean age of onset was 7.15 ± 3.30 years, and the peak age of onset was five to eight years. The most common initial attack type was transient ischemic attack (TIA) (62 cases, 54.4%) with limb weakness. The incidence of the initial attack type in the three age groups was varied (P < 0.05). The result of overall prognosis was good in 86 cases (89.6%)., Conclusions: In this study, MMD cases were mainly ischemic type and TIA was the most common initial attack type. Infant group was more prone to have cerebral infarction, whereas preschool and school-age groups tended to have TIA. The treatments and prognosis of the studied MMD cases were achieved with good outcomes., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: De novo cerebral microbleeds (CMBs) on T2*-weighted magnetic resonance imaging (MRI) develop over time in adult moyamoya disease (MMD) and are generally associated with a decline in global cognitive function. The present supplementary analysis of a 5-year prospective cohort aimed to elucidate the incidence of an interval increase in CMBs in adult patients receiving medical management alone for ischemic MMD and its impact on cognitive function., Methods: Sixty-four patients without misery perfusion in the symptomatic cerebral hemispheres at inclusion who did not experience any further ischemic symptoms or new hemorrhagic events during a 5-year follow-up period underwent T2*-weighted MRI and five kinds of neuropsychologic tests at inclusion and the end of the 5-year follow-up., Results: When T2*-weighted MRI was compared between inclusion and the end of the 5-year follow-up, 10 patients (15%) had an interval increase in CMBs in the symptomatic cerebral hemisphere at inclusion. The scores from two kinds of neuropsychologic tests significantly deteriorated at the end of the 5-year follow-up compared with those at inclusion in patients with an interval increase in CMBs, whereas the scores of four kinds of neuropsychologic tests significantly improved at the end of the 5-year follow-up compared with those at inclusion in patients without interval increases in CMBs, asymptomatic ischemic lesions, or angiographic disease progression., Conclusions: The incidence of an interval increase in CMBs was 15% per 5 years in adult patients receiving medical management alone for ischemic MMD, and this increase was associated with a decline in cognitive decline.

Moyamoya disease is a medical condition that shows the typical characteristics like continuous and chronic thickening of the walls and the contraction of the internal carotid artery; as a result, the internal diameter of the artery gets narrowed. There are six phases of the disease ranging from I to VI (moyamoya vessels completely disappear, followed by the complete blockage of the arteries). Surgery is a commonly recommended treatment for the moyamoya disease. Our research study identifies the effect of autologous bone marrow stem cell therapy (ABMSCT) on the levels of inflammatory factors and Conexin43 (Cx43) protein in patients suffering from moyamoya. In our study, we have selected 52 moyamoya patients admitted to our hospital from 30 July 2019 to 10 February 2020. The control group (CG) was treated with superficial temporal artery to a middle cerebral artery (STA-MCA) bypass + encephalo-duro-myosinangiosis (EDMS). The experimental group (Exp. Grp) was treated with ABMSC. The cerebral vascular tissue of the patients was treated with hematoxylin-eosin (HE) staining. Immunohistochemical staining was used to identify the levels of Cx43 protein. The concentrations of vascular endothelial growth factor (VEGF), inflammatory factor interleukin-6 (IL6), interleukin-1 β (IL1 β ), tumor necrosis factor (TNF α ), and anti-inflammatory factor interleukin-1 β (IL1 β ) were determined by enzyme-linked immunosorbent assay (ELISA). We have found that after treatment of the expression of Cx43 protein, the proportions of grade IV (7.7%), grade III (311.5%), and grade II (3.8%) patients in the Exp. Grp were lower than those in the CG. The proportion of grade I patients in the Exp. Grp (77%) was higher than that in the CG (38.5%). After treatment, the inflammatory factors IL6 (0.97 ± 0.82 pg/mL), IL1 β (8.33 ± 1.21 pg/mL), and TNF α (1.73 ± 0.71 pg/mL) in the Exp. Grp were lower than those in the CG. The anti-inflammatory factor IL1 β (15.09 ± 4.72 pg/mL) increased in the Exp. Grp compared with the CG (11.25 ± 3.48 pg/mL) post treatment. Intracranial infection, hydrocephalus, hemiplegia, and transient neurological dysfunction in the Exp. Grp were lower than those in the CG, with statistical differences ( P < 0.05). Our study suggests that the treatment of autologous bone marrow stem cells (ABMSC) was beneficial to balance the inflammatory response of disorders, reduce the damage of vascular tissue in the brain, and regulate tissue repair by co-acting with various inflammatory factors as compared to traditional surgery. We conclude that the involvement of Cx43 in the occurrence and development of moyamoya. We also have found that the risk factors of intracranial infection after ABMSCT were less as compared to those after conventional surgery., Competing Interests: The authors declare that they have no conflicts of interest with respect to this article., (Copyright © 2022 Liming Zhao et al.)

Objectives: Although revascularization surgery is recommended for adult patients with moyamoya disease (MMD) who present with ischemic symptoms due to hemodynamic compromise, the clinical course of such patients who are treated with medical management alone remains unclear. Here, we report outcomes of adult patients with cerebral misery perfusion due to ischemic MMD who received medical management alone., Materials and Methods: We prospectively followed up patients who showed misery perfusion in the symptomatic cerebral hemisphere on 15 O gas positron emission tomography (PET) and received strict medical management alone after refusing revascularization surgery., Results: Of 57 patients who showed symptomatic misery perfusion on 15 O gas PET, three (5%) were included into the present study. Two of these patients suffered further ischemic events at 7 and 8 months after inclusion, after which, their modified Rankin disability scale scores deteriorated. In the remaining patient, fatal intracerebral hemorrhage developed at 10 months after inclusion., Conclusions: These findings suggest that receiving medical management alone is associated with considerably poor outcomes for adult patients with cerebral misery perfusion due to ischemic MMD., Competing Interests: Declaration of Competing Interest Kuniaki Ogasawara declares the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Consigned research fund from Nihon Medi-Physics Co., Ltd., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background and Aims: Patients with Moyamoya disease (MMD) can present with ischaemic or haemorrhagic stroke. There is no good evidence for treatment strategies in MMD-associated acute ischaemic stroke (AIS), particularly for treatments like intravenous thrombolysis (IVT) and endovascular thrombectomy (ET). As the intracranial vessels are friable in MMD, and the risk of bleeding is high, the use of IVT and ET is controversial. To clarify the safety and efficacy of IVT/ET in the treatment of MMD-associated AIS, we performed a systematic review and meta-summary to examine this issue., Methods: A systematic search was performed from four electronic databases: PubMed (MEDLINE), Cochrane Library, EMBASE and Scopus, profiling data from inception till 21 November 2021, as well as, manually on Google Scholar., Results: Ten case reports detailing 10 MMD patients presenting with AIS and undergoing IVT or ET, or both, were included in the analysis. The median National Institute of Health Stroke Scale score at presentation was 10 (Interquartile Range [IQR] = 6.0-16.5). IVT alone was instituted in 6 patients, primary ET was attempted in 2, and 2 had received bridging IVT with ET. Of the 4 patients who underwent ET, 2 patients achieved successful reperfusion (modified Thrombolysis In Cerebral Infarction scale [mTICI] ≥ 2b). In terms of functional outcomes, One patient achieved complete recovery (modified Rankin Scale 0), 4 patients attained improvement in neurological status, and 4 had no improvement, whilst functional outcome was unreported in 1 patient. No patient experienced symptomatic intracranial haemorrhage., Conclusions: In this systematic review and meta-summary, the utility of IVT and ET in MMD-associated AIS appears feasible in selected cases. Further larger cohort studies are required to evaluate these treatment approaches., Highlights: · AIS in MMD was typically managed with bypass surgery but not via thrombolysis or thrombectomy. · In this meta-summary, all patients treated with thrombolysis and/or thrombectomy survived and some experienced symptomatic and/or functional improvement. · Further larger cohort studies are necessary for investigating the role of thrombolysis and/or thrombectomy as treatment of AIS in MMD., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Introduction: An ambiguous definition of "asymptomatic" Moyamoya Angiopathy(aMMA) of absence of ischemic/hemorrhagic episodes in MMA patients, has led to its variable adaptation in the limited past-studies., Objective: To observe the clinic-radiological characteristics and prospective follow-up of apparently "asymptomatic" MMA, and to determine if it is truly asymptomatic or not., Materials and Methods: An observation, cohort study of 122 angiographically proven MMA over 6 years was undertaken from a single, tertiary-care-center to observe the clinico-radiological characteristics, prospective follow-up of apparently aMMA. Amongst them, 6 had an initial diagnosis of aMMA following evaluation by atleast one post-graduate doctor, which were further scrutinized by 3 different neurologists for epidemiological, clinical, radiological characteristics and subsequent follow-up. Data were analyzed using descriptive statistics., Results: Mean age was 23.7 ± 13.14 years. 3 of 6 underwent brain-imaging for evaluation of non-migraine-like headache, 1 for dizziness, 2 as part of familial screening for MMA. 4 of 6 patients had specific-triggers for aggravation of symptoms. Brain-imaging revealed old vascular insults and ivy sign in 5 of 6 each (83.3%), mean suzuki staging was 3.6±0.82. 4 of 6 underwent cerebral perfusion study, all had hypoperfusion. Revascularization surgery was done in 2 of 6, rest were managed conservatively. None had any new-onset neurological deficit or radiological progression over a mean follow-up period of 22.3 ± 20.22 months., Conclusions: Apparently aMMA may not be truly asymptomatic and often have subtle "paroxysmal events" precipitated by specific-triggers, indicative of transient ischemic symptoms. Thus, warrants for a more precise definition to avoid misclassification of aMMA., Competing Interests: Conflicts of Interest The authors have stated explicitly that there are no conflicts of interest in connection with this article., (Copyright © 2022 Elsevier Inc. All rights reserved.)

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., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Introduction: No clear guidelines for treating adult patients with ischemic moyamoya disease (MMD) without cerebral hemodynamic compromise such as misery perfusion have been established. Our previous prospective cohort study of adult patients with MMD without misery perfusion who were treated with medical management alone, including an antiplatelet drug, showed a recurrent ischemic event rate of 3% per 2 years. The present prospective study aimed to elucidate the 5-year clinical, cerebral perfusion, and cognitive outcomes of medical management alone for Japanese adult patients with ischemic MMD without cerebral misery perfusion by following the same patients for another 3 years., Methods: In total, 68 patients without recurrent events at a 2-year follow-up were prospectively followed up for another 3 years. Cerebral blood flow (CBF) in the symptomatic cerebral hemisphere was measured using brain perfusion single-photon emission computed tomography at inclusion and at the end of the subsequent 3-year follow-up. Neuropsychological testing was performed at inclusion and at the end of the initial 2- and subsequent 3-year follow-ups., Results: During the subsequent 3-year follow-up, 2 patients (3%) developed further ischemic events. In patients without further ischemic events, CBF was significantly greater at the end of the subsequent 3-year follow-up than at inclusion (p = 0.0037), and all neuropsychological test scores improved or remained unchanged at the end of initial 2- and subsequent 3-year follow-ups compared with that at inclusion., Conclusion: In adult patients receiving medical management alone for ischemic MMD without cerebral misery perfusion, the incidence of further ischemic events was 6% per 5 years and did not change between the initial 2 years after the last is-chemic event and the subsequent 3 years. In patients without further ischemic events, CBF and cognitive function had not deteriorated at 5 years after the last ischemic event., (© 2021 S. Karger AG, Basel.)

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.

Introduction: Moyamoya disease (MMD) and posterior reversible encephalopathy syndrome (PRES) share similar pathophysiological characteristics of endothelial dysfunction and impaired cerebral autoregulation. However, there have never been any published studies to demonstrate the relationship between these 2 rare diseases., Patient Concerns: A 26-year-old Asian man presented with a throbbing headache, blurred vision, and extremely high blood pressure. We initially suspected acute cerebral infarction based on the cerebral computed tomography, underlying MMD, and prior ischemic stroke. However, the neurological symptoms deteriorated progressively., Diagnosis: Cerebral magnetic resonance imaging indicated the presence of vasogenic edema rather than cerebral infarction., Interventions and Outcomes: An appropriate blood pressure management prevents the patient from disastrous outcomes successfully. Cerebral magnetic resonance imaging at 2 months post treatment disclosed the complete resolution of cerebral edema. The patient's recovery from clinical symptoms and the neuroimaging changes supported the PRES diagnosis., Conclusion: This report suggests that patients with MMD may be susceptible to PRES. It highlights the importance of considering PRES as a differential diagnosis while providing care to MMD patients with concurrent acute neurological symptoms and a prompt intervention contributes to a favorable clinical prognosis., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

The present study reports on the language treatment outcomes from sentence- and story-level linguistic facilitation and its generalization effect on communicative abilities, working memory, and sentence processing in the case of an adult with Moyamoya Disease (MMD). After treatment,the patient's overall performance, including the Aphasia Quotient, and sentence processing ability as measured by language testing, were improved. Furthermore, the treatment effects were generalizable to working memory abilities. Our case study conveys clinically meaningful implications since it is the first report on the effects of language treatment on linguistic and cognitive domains for an individual with MMD-induced agrammatic Broca's aphasia.

Objective: Moyamoya angiopathy (MM) is a chronic, progressive steno-occlusive arteriopathy of the distal internal carotid artery and its proximal branches. MM is recognized as a shared end-pathway common to a broad range of inciting pathologies, suggesting that tailored management is important. Pediatric MM differs from MM in adults. Currently, there are many uncertainties and controversies regarding the diagnosis and management of children with MM. Hence, we conducted an international survey to identify the contemporary management trends followed worldwide., Methods: A survey relating to lifestyle modifications, medical management, diagnosis, surgical management, and follow-up for pediatric MM was circulated across web-based platforms, through various international pediatric neurological and neurosurgical societies. Data collected included geographic region of practice, experience, responses to questions, and comments., Results: One hundred twenty-seven responses were evaluated (104 neurosurgeons and 23 neurologists, from 32 countries, across 6 continents). We found wide variations in the recommendations for management and lifestyle modification, with significant differences between regions of practice. Eighty percent recommend restrictions on physical activity, particularly for symptomatic and non-operated patients. Eighty-four percent prescribe aspirin. Sixty-five percent perform indirect revascularization. Seventy-eight percent recommend performing a staged surgery for bilateral MM. Only 26% perform acetazolamide challenge SPECT to evaluate brain perfusion. Only 15% of responders were from highly experienced centers., Conclusion: This survey reflects the contemporary trends in management of pediatric MM, while highlighting the heterogeneity in the management approach of these patients. There is a need for multicenter, international studies to evaluate the safety, efficacy, and long-term outcome of various aspects of treatment of these patients.

An embryological association between moyamoya disease (MMD) and cardiac manifestations has been proposed. Data up to this point remains anecdotal, and the prevalence of cardiac manifestations in a western MMD population is uncertain. The objective of this study was to determine the prevalence of cardiac manifestations including coronary artery disease (CAD) and congenital cardiac defects in a mostly Caucasian population of MMD patients and review prior reports of such cases. Medical records of MMD patients who presented to our institution between 1990 and 2019 were retrospectively reviewed for the presence of various congenital cardiac malformations and concomitant CAD. The prevalence of congenital cardiac defects and CAD was determined. A literature search for prior cases of MMD with concomitant cardiac manifestations was performed. A total of 181 MMD patients were included in our analysis, 139 (76.8%) of whom were Caucasian. Ten patients had cardiac manifestations (5.5%). There were six total MMD patients with congenital cardiac defects (3.3%). All patients with congenital defects were diagnosed in childhood. The prevalence of congenital defects in MMD was slightly higher than the general population as reported previously (0.8-1.2%). Four MMD patients had CAD (2.2%). The mean age of patients with CAD was 41.0 years (SD = 12.3, range = 33-59) in our series and 33.1 years (SD = 15.0) in a review of prior reports. These mean ages of CAD are in contrast to the 7th and 8th decades of lifein the general population as indicated by prior studies. Our findings support an association between MMD and cardiac manifestations. Further investigation is warranted in order to further characterize this potential relationship and shed light on a possible cardio-cephalic neural crest syndrome.

Internal carotid artery (ICA) agenesis/aplasia is occasionally accompanied with cerebral aneurysms caused by hemodynamic stress. If the aneurysms are located around the circle of Willis, they are managed with clipping or coil embolization. Herein, we report a case of ICA agenesis with perforating artery aneurysms treated successfully with revascularization. Moreover, a literature review of ICA agenesis with cerebral aneurysms was performed to compare with the current case. We conducted a literature review using data from PubMed. A secondary search was also performed by reviewing the references of each article previously searched. In our case, the aneurysms shrank and disappeared after direct and indirect bypass surgeries, and indirect bypass developed as in moyamoya disease (MMD). The epidemiological and clinical features of aneurysms accompanied with ICA agenesis were identified via a literature review. Aneurysms with ICA agenesis categorized as type F based on the Lie classification system, or referred to as rete mirabile, are occasionally located in an untreatable site; hence, they cannot be treated with clipping or coil embolization. Moreover, results showed that previous studies did not use revascularization for the treatment of aneurysm. In conclusion, if an aneurysm with ICA agenesis is difficult to approach directly or via an endovascular procedure, revascularization can be a treatment option.

We conducted a systematic review of the literature to evaluate the efficacy of various treatment modalities for intracranial aneurysms (IA) in patients with moyamoya disease (MMD) based on anatomical location of IA. A comprehensive review of studies documenting single cases or series of MMD patients with concomitant IA was conducted. Aneurysms were classified into two primary anatomical categories: those of the Circle of Willis (CoW) and those of peripheral "moyamoya" collateral vessels. Conservative, endovascular, and open surgical treatment modalities and their outcomes between each anatomical subgroup were descriptively compared. A total of 124 studies consisting of 275 patients with 313 IA were included. Of all IA, 59.6% were located on CoW vessels, 33.7% on peripheral vessels, and 6.7% in "other" locations. Of all CoW IA, 87.2% treated with endovascular techniques had no or minimal deficit at follow-up as compared with 56.7% of those treated with open surgery. Ninety-five percent of patients with peripheral aneurysms treated with endovascular therapy had no or minimal deficit, in contrast to open surgery (69.6%). Of peripheral IA treated conservatively with or without revascularization, 65.7% had spontaneous resolution as compared with 12.0% IA of the CoW. Our results support the use of endovascular techniques for direct treatment of both CoW and peripheral IA. Aneurysms of peripheral vessels respond well to indirect treatment through surgical revascularization as opposed to CoW aneurysms. The quality of evidence is limited due to heterogeneity of included studies and IA management in MMD patients should be considered in a case-specific manne.

Objective: The best management of asymptomatic moyamoya disease (MMD) remains controversial. In this study, the authors aimed to explore an experience for treatment modality for asymptomatic MMD., Methods: The authors retrospectively reviewed a total of 23 patients (age range 30-58 years) with asymptomatic MMD during the past 5 years at their institutions. The patients were divided into 2 groups: The surgical group included 11 patients, and the conservative group included 12 patients. The demographic, radiologic, and clinical findings of the patients were evaluated. At follow-up over 13-65 months, the future clinical and radiologic progression events were evaluated., Results: During the follow-up period, 3 patients suffered from future clinical progression events in the conservative group: 1 experienced stroke, and 2 experienced transient ischemic attack. Among the patients in the surgical group, only 1 experienced transient ischemic attack. Kaplan-Meier analysis showed that patients undergoing surgeries had longer clinical progression-free survival times compared with patients in the conservative group (P = 0.002)., Conclusions: Surgical treatment may be an alternative choice for patients with asymptomatic MMD. However, the best strategy for asymptomatic MMD in order to reduce future cerebrovascular risks still needs to be further explored., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background and Purpose: There has been an increasing prevalence of Moyamoya disorder (MMD) reported from recent US literature. There is a paucity of data available regarding trends of prevalence and epidemiological factors in the United States. To goal of this study was to test the hypotheses that racial-, sex-specific MMD hospitalizations and epidemiological factors have been increasing in the United States over the last decade., Methods: In this retrospective observational study, using the National Inpatient Sample (NIS) database from 2005 to 2016, MMD-related hospitalizations in patients aged ≥18 years were identified. Trends of epidemiological factors were analyzed over time using the linear regression model with the significance of differences in trend over time assessed using the Wald test. Sex- and race-specific burden of MMD were calculated using the annual US Census data. Joinpoint regression model was used to evaluate trends of hospitalizations over time., Results: A total of 24,484 adult hospitalizations were identified from January 2005 to September 2015 after excluding <18 years. Among them, approximately ~90% were aged ≤60 years, and 73.5% were females. The most common vascular and non-vascular presentations were ischemic stroke (17.3%) and seizures (21%), respectively. The trend of antithrombotic therapy has increased, while extracranial-intracranial bypass has remained stagnant. The actual average hospitalizations of MMD was 10.4 cases/ million population/year (range 4.1-17.9) and varied significantly by sex (females 14.7 [range 6.2-23.6] and males 5.9 [range 1.8-11.9]) over the 2005 to 2016 study period. The burden of hospitalizations also differed by race (African Americans 40.6 [range 32.8-63.7], Asians 24.8 [15.4-34.8], Non-Hispanic Whites 8.1 [range 6.4-11.5], and Hispanics 8.4 [2.8-12.8]) over the 2010 to 2016 study period. Joinpoint regression analysis showed an increasing overall MMD trend across the study period (+11.7%; P < 0.001), which was higher in males (+14.5% vs. +10.7%; P < 0.001). The Hispanic group had significantly increased hospitalizations over the years (+20.2%; P < 0.001)., Conclusion: Although overall more prevalent in females, MMD-related hospitalizations are increasing more rapidly in males. Among the racial subpopulations, African Americans had the highest MMD-related hospitalizations, even higher than Asian Americans. MMD-related hospitalizations have increased quicker in Hispanics than in any other racial group., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Hashimoto's disease has reportedly been associated with stroke; however, cerebrovascular morphology and clinical course remain poorly documented. The present study aimed to determine the angiographic features and clinical outcomes of intracranial arterial stenosis (IAS) associated with Hashimoto's disease in a retrospective cohort., Methods: Overall, 107 adult patients with IAS were screened for anti-thyroid antibodies; of these, 26 patients tested positive. The 42 affected hemispheres were classified into subgroups according to the steno-occlusion site and the development of abnormal collateral (moyamoya) vessels. These subgroups were dichotomized into moyamoya vessels positive (MM type) and negative (non-MM type). The initial presentation, IAS progression, and vascular events during the follow-up period were compared., Results: The following sites of stenosis were identified: the bifurcation of the internal carotid artery in 11 (26.2%), M1 or A1 in 29 (69.0%), and more distal (M2-M4/A2-A4) in 2 (4.8%) hemispheres. Further, 17 hemispheres were categorized into the MM type and 25 were classified into the non-MM type. During the follow-up period (mean 2.5 years), IAS progression was identified in 8 (32%) hemispheres of the non-MM type and 0 (0%) hemispheres of the MM type (p = 0.041). Ischemic attacks occurred in 5 (20.0%) hemispheres of the non-MM type (4.6%/year) and 0 hemispheres of the MM type (p = 0.08). Further, 4 (23.5%) hemispheres of the MM type experienced intracerebral hemorrhage, whereas none of the non-MM type hemorrhaged (p = 0.012)., Conclusions: Hashimoto's disease-associated IAS exhibits various angiographic morphologies, resulting in different clinical presentations. Screening for anti-thyroid antibodies and careful management based on vascular morphology appears important in adults with IAS.

Objectives: To explore clinical features and compare of the difference between conservative treatment and surgical treatmentin prognosis of intracranial aneurysms associated with moyamoya disease (MMD)., Methods: 104 patients with MMD a ssociated with intracranial aneurysms diagnosed by digital subtraction angiography (DSA) or computed tomography angiography (CTA) were retrospectively analyzed., Results: All patients of MMD with intracranial aneurysms had 129 aneurysms distributed at different sites. The distribution of the aneurysms was as follows: 28(21.71%) anterior communicating artery aneurysms, 21(16.28%) basilar artery aneurysms, 19(14.73%) middle cerebral artery aneurysms, 17(13.18%) posterior cerebral artery aneurysms, 12(9.30%)internal carotid artery aneurysms, 11(8.53%) posterior communicating artery aneurysms, 10(7.75%) anterior cerebral artery aneurysms, 5(3.88%) anterior choroidal artery aneurysms, 2(1.55%) moyamoya vessel aneurysms, and 4(3.1%) other location aneurysms. 82 cases (78.85%) had one aneurysm, and 21 cases (20.19%) had 2 or more aneurysms. 64 cases were treated surgically, and 40 cases were treated conservatively. 57 of 81 cases (70.37%) had a good outcome, 2 cases (2.47%) were in paralyzed, and 22 cases were died. The mortality rate was (27.16%). Death occurred in 16 (47.06%) of 34 patients with conservative treatment, and 6(12.77%) of 47 patients with surgical treatment. 7 cases (8.64%) had twice cerebral hemorrhage, and one case (1.23%) had third cerebral hemorrhage in the follow-up period. There were 5 deaths, two good outcomes, and one coma among 8 cases experienced re-hemorrhage., Conclusion: Compared with conservative treatment, positive surgical treatment showed clinical significance for preventing cerebral hemorrhage and reducing mortality of MMD with intracranial aneurysms., Abbreviations: MMD: moyamoya disease; DSA: digital subtraction angiography; CTA: computed tomography angiography.

Some of the pediatric moyamoya patients spend their childhood without diagnosed as moyamoya disease (MMD) because of their mild ischemic attacks and emerge again with ischemic or hemorrhagic stroke in their adulthood. This study was aimed to clarify the clinical characteristics of adult moyamoya patients with childhood onset and elucidate the impact of long disease period on their clinical features. Present study included 116 untreated hemispheres of 69 adult patients with MMD. They were divided into two groups: childhood onset group (26 hemispheres of 14 patients) and adult onset group (90 hemispheres of 55 patients). Clinical features were compared between the two groups. The incidence of hemorrhagic stroke was significantly higher in childhood onset group (P = 0.0091). Lenticulostriate and choroidal channels were more developed in childhood onset group (P = 0.044 and P <0.001, respectively). Vault moyamoya was more frequently observed in childhood onset group (P <0.001). The development of surgical collaterals through indirect bypass was more marked in childhood onset group (P = 0.0019). Multivariate analysis revealed that childhood onset and developed choroidal channels were significantly associated with the occurrence of hemorrhagic stroke (OR 4.31 [95% CI 1.21-15.4], P = 0.025 and OR 6.78 [95% CI 1.78-25.8], P = 0.0050, respectively). This study clearly shows that adult moyamoya patients with childhood onset have more developed spontaneous collaterals, which may, in turn, highly causes hemorrhagic stroke. Adult moyamoya patients with "childhood onset" should be recognized as a novel and important concept when elucidating the underlying mechanisms of hemorrhagic stroke in MMD.

Background Purpose: Moyamoya syndrome is the progressive stenosis of intracranial carotids with secondary collateralization. Whole body cryotherapy (WBC) involves external cooling and is used in holistic and sports medicine, its neurologic effects are unknown., Case Report: We report a first case of symptoms of moyamoya syndrome presenting following WBC and diagnosed with classic MRI ( "Brush Sign", "Ivy sign") and digital subtracted angiography., Conclusion: WBC may provoke symptoms of moyamoya syndrome possibly through hyperventilation or vasoconstriction. Practitioners should be aware of possible consequences of WBC in patients with poor cerebrovascular reserve.

Rationale: The incidence of pure arterial malformations is relatively low, and few cases have been reported. Only 2 cases with pure arterial malformation have been reported to receive surgery or endovascular treatment., Patient Concerns: We report 3 cases and review the relevant literatures. The head examinations of the patients suggested the presence of high-density shadows in front of the pons and midbrain, the dilation of the supraclinoid segment of the right internal carotid artery, and moyamoya in the left brain with an aneurysm-like expansion located on the left posterior communicating artery respectively. After admission, head digital subtraction angiography (DSA) was performed., Diagnoses: Digital subtraction angiography (DSA) for these 3 patients showed that the left posterior communicating artery, the supraclinoid segment of the right internal carotid artery, and the left posterior communicating artery appeared dilated, tortuous, and spirally elongated. In addition, the lesions in the latter 2 patients were accompanied with local aneurysmal changes., Interventions: Two patients were given conservative treatment, and another patient was given endovascular treatment. A head DSA was reviewed 6 months after therapy., Outcomes: The prognosis status of the 3 patients was good. Two patients in the conservative treatment group showed no changes in the lesions on head DSA examination. The DSA examination of the third patient indicated that the vascular remodeling of the diseased vessels was good, the blood vessels were unobstructed, and the aneurysms had disappeared., Lessons: Pure arterial malformations mostly occur in young women and may involve any blood vessels in the brain. It can be accompanied with local aneurysms and calcification. The patients are often given conservative treatment but need to be reviewed regularly. However, it is beneficial to give endovascular treatment to the patients with local aneurysms.

Aims: This study investigated the safety and efficacy of remote ischemic conditioning (RIC) on ameliorating the sequelae of ischemic moyamoya disease (iMMD)., Methods: A total of 30 iMMD patients underwent long-term RIC and were followed up at 0.5, 1, and 2 years for clinical outcomes, including frequency of stroke recurrence, Patient Global Impression of Change (PGIC) scale, peak systolic velocities (PSV), and cerebral perfusion., Results: During the whole RIC treatment process, no RIC-related adverse event occurred. Only one of 30 patients suffered a onetime infarction (3.3%), and the ratios of acceptable PGIC were 88.2%, 64.3%, and 92.3% at 0.5, 1, and 2 years follow-up. Kaplan-Meier analysis showed the frequency of stroke recurrence was significantly reduced after RIC (P = .013). The frequency of TIA per week was 1.1 (0.6, 2.8) prior to RIC and 0.1 (0.0, 0.5) post-RIC (P < .01). Compared to baseline, PSV values were significantly reduced after RIC treatment (P = .002 at 0.5, P = .331 at 1, and P = .006 at 2 years). In patients undergoing perfusion studies, 75% obtained improvement on followed-up SPECT and 95% on followed-up PET maps., Conclusions: Remote ischemic conditioning may be beneficial on controlling iMMD-induced ischemic events, relieving symptoms, and improving cerebral perfusion, without incidence of complications in this case series., (© 2019 The Authors. CNS Neuroscience & Therapeutics published by John Wiley & Sons Ltd.)

Background: Over the last decades, significant advancements have been achieved in the treatment of pediatric brain tumors as a result of radiation therapy (RT). With the increasing diffusion of this treatment, iatrogenic damage to cerebrovascular tissues contouring the radiation target volume has become the subject of debate, especially radiation-induced moyamoya syndrome (RIMS)., Methods: A systematic literature search was performed on the association between moyamoya vasculopathy and cranial irradiation in children. Large case series of patients with moyamoya were analyzed and clinicoradiologic data were collected reviewing pediatric patients treated with RT for primary brain tumors at our institution., Results: The risk of developing RIMS is higher in younger children, in patients with optic pathway glioma, and in those receiving higher radiation doses. Headache is the most common presenting symptom and cerebral infarction is frequent. The preferred surgical techniques were pial synangiosis and encephaloduroarteriosynangiosis. In our case series, surgical revascularization led to neovascularization, with clinical improvement or stability in all patients. Medical therapy did not significantly affect the clinical course., Conclusions: Pediatric patients receiving involved field RT for the treatment of brain tumors have an increased risk of developing RIMS. Prompt diagnosis and early surgical revascularization play a pivotal role in decreasing the clinical impact of this complication. The use of new techniques, such as the intensity-modulated RT, and the increasing dose saving for the organs at risk, are essential to prevent iatrogenic vasculopathy. The combination of appropriate medical therapy and surgery will improve patient management and clinical outcome., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Moyamoya syndrome, a progressive, idiopathic stenosis of the internal carotid arteries, results in increased risk for both ischemic and hemorrhagic strokes. Revascularization procedures have been shown in small studies to be both safe and efficacious for these patients; however, randomized controlled trials are lacking. The goal of this systematic review is to organize the literature evaluating surgical intervention versus conservative medical management., Methods: A systematic review was performed including studies with 3 or more participants with moyamoya syndrome in the setting of sickle cell disease and a measured outcome after either medical or surgical intervention. Relevant studies were identified using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses criteria and a set of predetermined key words., Results: Sixty-one articles were identified with 6 articles ultimately included in this review (N = 122). Of the patients, 73 (59.8%) were revascularized surgically (all indirect procedures), whereas 49 (40.2%) remained on chronic transfusion therapy. Of the patients that underwent indirect revascularization surgery, a total of 1 perioperative (1.4%) and 4 postoperative strokes (5.5%) were reported over 44 months (1 stroke per 53.3 patient-years). In comparison, an average of 46.5% of patients who were receiving chronic transfusions had major events (stroke or transient ischemic attack) while undergoing therapy (1 stroke per 13.65 patient-years, P = 0.00215)., Conclusions: We present a large systematic review of the literature regarding outcomes of surgical and medical management for patients with moyamoya syndrome and sickle cell disease. The findings redemonstrate the efficacy and safety of surgical revascularization, and advocate for earlier discussion around surgical intervention., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Due to the lack of animal models and difficulty in obtaining specimens, the study of pathogenesis of moyamoya disease (MMD) almost stagnated. In recent years, endothelial progenitor cells (EPCs) have attracted more and more attention in vascular diseases due to their important role in neovascularization. With the aid of paradigms and methods in cardiovascular diseases research, people began to explore the role of EPCs in the processing of MMD. In the past decade, studies have shown that abnormalities in cell amounts and functions of EPCs were closely related to the vascular pathological changes in MMD. However, the lack of consistent criteria, such as isolation, cultivation, and identification standards, is also blocking the way forward. The goal of this review is to provide an overview of the current situation and controversial issues relevant to studies about EPCs in the pathogenesis and etiology of MMD.

Introduction: Moyamoya is a chronic cerebrovascular condition of unclear etiology characterized by progressive occlusion of 1 or both internal carotid arteries with neovascular collateral formation. With both an idiopathic form (moya-moya disease) and congenital condition-associated form (moyamoya syndrome), it can cause ischemic and hemorrhagic stroke. Recent findings in Kentucky have challenged traditional estimates of its incidence in US populations. Using the Kentucky Appalachian Stroke Registry (KApSR), our aim was to further characterize its incidence as a cause of stroke and to understand the patient population in Appalachia., Methods: A retrospective review of moyamoya patients was performed using the KApSR database. Data collected included demographics, county location, risk factors, comorbidities, and health-care encounters from January 1, 2012, to December 31, 2016., Results: Sixty-seven patients were identified; 36 (53.7%) resided in Appalachian counties. The cohort accounted for 125 of 6,305 stroke admissions, representing an incidence of 1,983 per 100,000 stroke admissions. Patients presented with ischemic strokes rather than hemorrhagic strokes (odds ratio 5.50, 95% CI: 2.74-11.04, p < 0.01). Eleven patients (16.4%) exhibited autoimmune disorders. Compared to the general population with autoimmune disorder prevalence of 4.5%, the presence of autoimmunity within the cohort was significantly higher (p < 0.01). Compared to non-Appalachian patients, Appalachian patients tended to present with lower frequencies of tobacco use (p = 0.08), diabetes mellitus (p = 0.13), and hypertension (p = 0.16)., Conclusions: Moyamoya accounts for a substantial number of stroke admissions in Kentucky; these patients were more likely to develop an ischemic stroke rather than a hemorrhagic stroke. Autoimmune disorders were more prevalent in moyamoya patients than in the general population. The reduced frequency of traditional stroke risk factors within the Appalachian group suggests an etiology distinct to the population., (© 2020 S. Karger AG, Basel.)

Introduction: Moyamoya angiopathy (MMA) is a progressive steno-occlusive disease of the distal internal carotid arteries mainly described in Asia. It induces the development of collateral vascular networks to reduce chronic cerebral hypoperfusion. Symptoms depend on the patient's age in Asia: children are at greater risk of transient or constituted ischemic events, whereas adults are more exposed to hemorrhagic stroke. Data from the literature seem to show that the pattern of MMA in western countries differs from that in Asia., Material and Methods: A retrospective study of patients with MMA was conducted in Bourgogne-Franche-Comté (mid-eastern France). Clinical data (symptoms, risk factors, age at diagnosis, number and timing of recurrences, type of treatment) as well as radiological data (angiographic findings, Suzuki's grade) were analyzed., Results: Seventeen adult patients (9 men, 53%) were followed at the university hospitals of Besançon and Dijon from 2009 to 2016. Fourteen patients (83%) had bilateral disease. The mean age at diagnosis was 49 years (±16), 83% of the patients were Caucasian and 17% originated from Maghreb. Only 17% of the hemispheres had a hemorrhagic form. Ischemic form was more frequent before diagnosis with transient ischemic attack (24% of patients) and stroke (83% of patients). With medical treatment, 9 patients suffered from stroke recurrence (53% of patients) with an average delay of 22.7±34 months. Three patients (18%) had combined surgical management by encephelo-synangiosis and superficial temporal artery-to-middle cerebral artery (STA-MCA) anastomosis, without symptom recurrence after treatment with an average follow up of 14 months., Conclusion: MMA remains a rare cerebrovascular disease in Europe and requires multidisciplinary care. Epidemiological analysis showed differences with the Asian population, especially the predominance of ischemic forms in adults., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background and Purpose: Moyamoya angiopathy (MMA) is a chronic cerebrovascular disorder predominantly starting in childhood or early adulthood and thus affects the whole lifetime. Little is known on MMAs long-term outcomes in European patients. We report long-term follow-up data on Finnish MMA patients., Methods: We included patients from our Helsinki University Hospital MMA database and arranged long-term follow-up visits for all the patients. This follow-up included a review of the medical records accumulated in due time, detailed neurological and neuropsychological evaluation, and outcome measures modified Rankin Scale (mRS) and Barthel Index (BI)., Results: There were 61 MMA patients with a mean follow-up period of 9.5 years (SD 6.7 years; range 1.3-35.4 years; 581 patient-years). Only two patients had died and two-thirds (n = 40, 65.6%) had no new events during the follow-up period. Eight patients (13.1%) had an ischemic and five patients (8.2%) a hemorrhagic stroke during the follow-up. There were no differences between operated (n = 26) and conservatively (n = 35) treated groups regarding recurrent events or the outcome measured with mRS or BI. Finnish MMA patients reported significantly poorer physical and psychological health aspects of QOL when compared to the general Finnish population. Symptoms of low mood were found in 27 (56%) patients., Conclusions: Finnish MMA patients have a benign and stable course with a ~3.5 % annual stroke risk. We found no differences in the clinical outcomes between the operated and conservative groups, however, the psychosocial well-being requires more attention in MMA patients.

Background: Pregnancy is a potential risk factor for stroke in women with Moyamoya disease. However, the rarity of the disease has limited clinical expertise to ensure a healthy pregnancy. The aim of the present study was to explore the possible risk factors for hemorrhagic and ischemic stroke in pregnant women with Moyamoya disease., Methods: A retrospective review of cases in our hospital during a 20-year period and a review of the reported data were conducted to investigate pregnancy-related cerebrovascular events in women with Moyamoya disease., Results: Thirty pregnancies in 20 women with Moyamoya disease were identified in the case review of our hospital. All were previously diagnosed cases, and no stroke had occurred during the study period. In the reported data review, pregnancy-related stroke in women with Moyamoya disease was identified in 54 (44 intracranial hemorrhage and 10 cerebral infarction). Intracranial hemorrhage occurred most commonly during the antepartum period (n = 39; 88.6%), with most events occurring at ≥24 weeks. Of the intracranial hemorrhage cases, 7 (15.9%) were complicated by hypertensive disorders of pregnancy, and 8 patients (18.2%) died of stroke. The onset of cerebral infarction was either in the antepartum (n = 4; 40.0%) or postpartum (n = 6; 60.0%) period. All postpartum cases occurred within 3-7 days after delivery., Conclusion: Pregnancy-related stroke in patients with Moyamoya disease might be susceptible to gestational age. Intracranial hemorrhage is prone to occur during the antepartum period, especially at ≥24 weeks, and cerebral infarction tends to occur postpartum., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Vascular endothelial growth factor-A 165 (VEGF-A 165 ) has been identified as a combination of 2 alternative splice variants: proangiogenic VEGF-A 165 a and antiangiogenic VEGF-A 165 b. Intracranial atherosclerotic disease (ICAD) and moyamoya disease (MMD) are 2 main types of intracranial arterial steno-occlusive disorders with distinct capacities for collateral formation. Recent studies indicate that VEGF-A 165 regulates collateral growth in ischemia. Therefore, we investigated if there is a distinctive composition of VEGF-A 165 isoforms in ICAD and MMD., Methods: Sixty-six ICAD patients, 6 MMD patients, and 5 controls were enrolled in this prospective study. ICAD and MMD patients received intensive medical management upon enrollment. Surgery was offered to 9 ICAD patients who had recurrent ischemic events, 6 MMD patients, and 5 surgical controls without ICAD. VEGF-A 165 a and VEGF-A 165 b plasma levels were measured at baseline, within 1 week after patients having surgery, and at 1, 3, and 6 months after treatment., Results: A significantly higher baseline VEGF-A 165 a/b ratio was observed in MMD compared to ICAD (P = .016). The VEGF-A 165 a/b ratio increased significantly and rapidly after surgical treatment in ICAD (P = .026) more so than in MMD and surgical controls. In patients with ICAD receiving intensive medical management, there was also an elevation of the VEGF-A 165 a/b ratio, but at a slower rate, reaching the peak at 3 months after initiation of treatment (baseline versus 3 months VEGF-A 165 a/b ratio, P = .028)., Conclusions: Our study shows an increased VEGF-A 165 a/b ratio in MMD compared to ICAD, and suggests that both intensive medical management and surgical revascularization elevate the VEGF-A 165 a/b ratio in ICAD patients., (Copyright © 2018. Published by Elsevier Inc.)

Moyamoya is a chronic, progressive steno-occlusive arteriopathy that typically affects the anterior circulation arteries of the circle of Willis. A network of deep thalamoperforating and lenticulostriate collaterals develop to by-pass the occlusion giving rise to the characteristic angiographic "puff of smoke" appearance. Moyamoya confers a lifelong risk of stroke and neurological demise, with peak age of presentation in childhood ranging between five and 10 years. Moyamoya disease refers to patients who do not have a comorbid condition, whereas moyamoya syndrome refers to patients in whom moyamoya occurs in association with an acquired or inherited disorder such as sickle cell disease, neurofibromatosis type-1 or trisomy 21. The incidence of moyamoya disease and moyamoya syndrome demonstrates geographic and ethnic variation, with a predominance of moyamoya disease in East-Asian populations. Antiplatelet therapy and surgical revascularization procedures are the mainstay of management, as there are no available treatments to slow the progression of the arteriopathy. Future research is required to address the major gaps that remain in our understanding of the pathologic basis, optimal timing for surgery, and determinants of outcome in this high-stroke risk condition of childhood., (Copyright © 2018 Elsevier Inc. All rights reserved.)