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Recent Advances for Global Perspectives on Etiology, Pathophysiology, Clinical Presentations, and Management of Moyamoya Disease
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作者 Maiko Charles Mkwambe Dongchi Zhao Youping Deng 《World Journal of Neuroscience》 CAS 2024年第1期6-23,共18页
Moyamoya disease (MMD) is a condition characterized by the gradual narrowing and blockage of blood vessels in the brain, specifically those in the circle of Willis and the arteries that supply it. This results in redu... Moyamoya disease (MMD) is a condition characterized by the gradual narrowing and blockage of blood vessels in the brain, specifically those in the circle of Willis and the arteries that supply it. This results in reduced blood flow and oxygen to the brain, leading to progressive symptoms and potential complications. The underlying pathophysiological mechanism remains elucidated. However, recent studies have highlighted numerous etiologic factors: abnormal immune complex responses, susceptibility genes, branched-chain amino acids, antibodies, heritable diseases, and acquired diseases, which may be the great potential triggers for the development of moyamoya disease. Its clinical presentation has varying degrees from transient asymptomatic events to significant neurological deficits. Moyamoya disease (MMD) shows different patterns in children and adults. Children with MMD are more susceptible to ischemic events due to decreased blood flow to the brain. Conversely, adults with MMD are more prone to hemorrhagic events involving brain bleeding. Children with MMD may experience a range of symptoms including motor impairments, sensory issues, seizures, headaches, dizziness, cognitive delays, or ongoing neurological problems. Although adults may present with similar clinical symptoms as children, they are more prone to experiencing sudden onset intraventricular, subarachnoid, or intracerebral hemorrhages. One of the challenges in moyamoya disease is the potential for misdiagnosis or delayed diagnosis, particularly when physicians fail to consider MMD as a possible cause in stroke patients. This review aims to provide a comprehensive overview of recent global studies on the pathophysiology of MMD, along with advancements in its management. Additionally, the review will delve into various surgical treatment options for MMD, as well as its rare occurrence alongside atrioventricular malformations. Exciting prospects include the use of autologous bone marrow transplant and the potential role of Connexin 43 protein treatment in the development of moyamoya disease. 展开更多
关键词 moyamoya disease (MMD) ETIOLOGY PATHOPHYSIOLOGY Clinical Presentations MANAGEMENT Future Promising Avenues
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Is medical management useful in Moyamoya disease?
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作者 Sombat Muengtaweepongsa Vatcharasorn Panpattanakul 《World Journal of Clinical Cases》 SCIE 2024年第3期466-473,共8页
Moyamoya disease(MMD),characterized by progressive internal carotid artery stenosis and collateral vessel formation,prompts cerebral perfusion complications and is stratified into idiopathic and Moyamoya syndrome subt... Moyamoya disease(MMD),characterized by progressive internal carotid artery stenosis and collateral vessel formation,prompts cerebral perfusion complications and is stratified into idiopathic and Moyamoya syndrome subtypes.A multifa-ceted approach toward MMD management addresses cerebral infarctions through revascularization surgery and adjunctive medical therapy,while also navigating risks such as intracranial hemorrhage and cerebral infarction resulting from arte-rial stenosis and fragile collateral vessels.Addressing antithrombotic management reveals a potential role for treatments like antiplatelet agents and anticoagulants,despite the ambiguous contribution of thrombosis to MMD-related infarctions and the critical balance between preventing ischemic events and averting hemo-rrhagic complications.Transcranial doppler has proven useful in thromboembolic detection,despite persisting challenges concerning the efficacy and safety of an-tithrombotic treatments.Furthermore,antihypertensive interventions aim to ma-nage blood pressure meticulously,especially during intracerebral hemorrhage,with recommendations and protocols varying based on the patient’s hypertension status.Additionally,lipid-lowering therapeutic strategies,particularly employing statins,are appraised for their possible beneficial role in MMD management,even as comprehensive data from disease-specific clinical trials remains elusive.Com-prehensive guidelines and protocols to navigate the multifaceted therapeutic ave-nues for MMD,while maintaining a delicate balance between efficacy and safety,warrant further meticulous research and development.This protocol manuscript seeks to elucidate the various aspects and challenges imbued in managing and navigating through the complex landscape of MMD treatment. 展开更多
关键词 moyamoya disease Cerebral infarction Antithrombotic management Transcranial doppler REVASCULARIZATION Intracerebral hemorrhage Antihypertensive intervention Lipid-lowering therapies
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Analysis of the Effect of the Comprehensive Nursing Model on Patients with Moyamoya Disease Undergoing Intracranial and Extracranial Revascularization Surgery
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作者 Yuanrong Luo 《Journal of Clinical and Nursing Research》 2024年第2期28-32,共5页
Objective:To explore the effect of a comprehensive nursing model on patients with Moyamoya disease who underwent intracranial and extracranial revascularization surgery.Methods:110 cases were divided into control and ... Objective:To explore the effect of a comprehensive nursing model on patients with Moyamoya disease who underwent intracranial and extracranial revascularization surgery.Methods:110 cases were divided into control and observation groups with 55 cases each.The control group received routine perioperative care,and the observation group received perioperative care along with comprehensive nursing care.The two groups’disease cognition levels,anxiety,symptoms,daily living ability scores,and postoperative complication rates were compared.Results:The anxiety score and total postoperative complications of the observation group upon discharge were lower than that of the control group,and the disease cognition level and daily living ability upon discharge were higher than that of the control group(P<0.05).Conclusion:Applying the comprehensive nursing model in conjunction with perioperative care for patients undergoing surgery can effectively improve their anxiety,strengthen activities of daily living,and reduce the risk of postoperative complications. 展开更多
关键词 Comprehensive nursing model moyamoya disease Intracranial and extracranial revascularization surgery Application effect
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Anesthetic and Obstetric Considerations of Moyamoya Disease in Pregnancy after Intracranial Bypass Grafting: A Case Report and Literature Review
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作者 Dennerd Ovando Jr. Arit Ntekim Ming Xiong 《Open Journal of Anesthesiology》 2023年第5期101-107,共7页
Moyamoya disease (MD) is a chronic idiopathic vasculopathy characterized by bilateral stenosis of the distal internal carotid arteries (ICA’s) with subsequent formation of abnormal collateral vessels. The underlying ... Moyamoya disease (MD) is a chronic idiopathic vasculopathy characterized by bilateral stenosis of the distal internal carotid arteries (ICA’s) with subsequent formation of abnormal collateral vessels. The underlying etiology is unknown [1]. Patients with MD are predisposed to intracranial hemorrhage or cerebral ischemia given the fragile nature of the collateral circulation. Although there is no curative treatment for MD, there are surgical palliative procedures that can augment cerebral blood flow to areas of impaired perfusion and circumvent the abnormal collateral circulation. Medical management exists for patients who are not surgical candidates. Because the incidence of MD peaks during the second and third decades of life, it can potentially affect women of childbearing age [2]. However, the optimal anesthetic and obstetric management of the parturient with MD remains controversial. We present a case of a primigravida with a history of MD who underwent intracranial bypass grafting prior to conception and subsequently had a successful cesarean delivery that was complicated by transient ischemic attacks (TIA) in the postpartum period. 展开更多
关键词 moyamoya disease PREGNANCY Transient Ischemic Attack ANESTHESIA
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Enhancement Expression of bFGF in Chinese Patients with Moyamoya Disease 被引量:12
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作者 ZOU DeWei ZHAO JiZong ZHANG Dong WANG Shuo WANG KeDa ZHANG Yan 《Biomedical and Environmental Sciences》 SCIE CAS CSCD 2011年第1期74-80,共7页
Objective To detect the content of the basic fibroblast growth factor in blood samples of patients with Moyamoya disease, and investigate the relationship between Moyamoya disease and the basic fibroblast growth facto... Objective To detect the content of the basic fibroblast growth factor in blood samples of patients with Moyamoya disease, and investigate the relationship between Moyamoya disease and the basic fibroblast growth factor. Methods This tissue microarray study included 24 cases of superficial temporal artery samples, 15 cases of Moyamoya disease, and 9 cases of normal arteries as control, and bFGF immunofluorescence assay was applied to test the samples. The number of positive cells and total cells of the muscular layer and the endothelium layer were counted separately in every picture, the positive rates were calculated, and the experimental data were analyzed statistically. Results The bFGF immunofluorescence staining of smooth muscular layer cells, intima cells and endothelial cells from the moyamoya disease group were obviously stronger than that from the control group (P0.01). Conclusion The enhancement expression of bFGF in the Moyamaya disease group implicates that bFGF plays an important part in the pathogenesis of Moyamoya disease. 展开更多
关键词 moyamoya disease BFGF PATHOGENESIS Superficial temporal artery IMMUNOFLUORESCENCE
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Microstructural damage pattern of vascular cognitive impairment: a comparison between moyamoya disease and cerebrovascular atherosclerotic disease 被引量:9
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作者 Jia-Bin Su Si-Da Xi +7 位作者 Shu-Yi Zhou Xin Zhang Shen-Hong Jiang Bin Xu Liang Chen Yu Lei Chao Gao Yu-Xiang Gu 《Neural Regeneration Research》 SCIE CAS CSCD 2019年第5期858-867,共10页
Moyamoya disease and cerebrovascular atherosclerotic disease are both chronic ischemic diseases with similar presentations of vascular cognitive impairment. The aim of the present study was to investigate the patterns... Moyamoya disease and cerebrovascular atherosclerotic disease are both chronic ischemic diseases with similar presentations of vascular cognitive impairment. The aim of the present study was to investigate the patterns of microstructural damage associated with vascular cognitive impairment in the two diseases. The study recruited 34 patients with moyamoya disease(age 43.9 ± 9.2 years; 20 men and 14 women, 27 patients with cerebrovascular atherosclerotic disease(age: 44.6 ± 7.6 years; 17 men and 10 women), and 31 normal controls(age 43.6 ± 7.3 years; 18 men and 13 women) from Huashan Hospital of Fudan University in China. Cognitive function was assessed using the Mini-Mental State Examination, long-term delayed recall of Auditory Verbal Learning Test, Trail Making Test Part B, and the Symbol Digit Modalities Test. Single-photon emission-computed tomography was used to examine cerebral perfusion. Voxel-based morphometry and tract-based spatial statistics were performed to identify regions of gray matter atrophy and white matter deterioration in patients and normal controls. The results demonstrated that the severity of cognitive impairment was similar between the two diseases in all tested domains. Patients with moyamoya disease and those with cerebrovascular atherosclerotic disease suffered from disturbed supratentorial hemodynamics. Gray matter atrophy in bilateral middle cingulate cortex and parts of the frontal gyrus was prominent in both diseases, but in general, was more severe and more diffuse in those with moyamoya disease. White matter deterioration was significant for both diseases in the genu and body of corpus callosum, in the anterior and superior corona radiation, and in the posterior thalamic radiation, but in moyamoya disease, it was more diffuse and more severe. Vascular cognitive impairment was associated with regional microstructural damage, with a potential link between, gray and white matter damage. Overall, these results provide insight into the pathophysiological nature of vascular cognitive impairment. This study was approved by the Institutional Review Board in Huashan Hospital, China(approval No. 2014-278). This study was registered with ClinicalTrials.gov on December 2, 2014 with the identifier NCT02305407. 展开更多
关键词 nerve REGENERATION vascular cognitive impairment moyamoya disease CEREBROVASCULAR ATHEROSCLEROTIC disease magnetic resonance IMAGING diffusion tensor IMAGING gray matter volume tract-based spatial statistics single-photon emission computed tomography neural REGENERATION
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Recognition of moyamoya disease and its hemorrhagic risk using deep learning algorithms:sourced from retrospective studies 被引量:3
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作者 Yu Lei Xin Zhang +7 位作者 Wei Ni Heng Yang Jia-Bin Su Bin Xu Liang Chen Jin-Hua Yu Yu-Xiang Gu Ying Mao 《Neural Regeneration Research》 SCIE CAS CSCD 2021年第5期830-835,共6页
Although intracranial hemorrhage in moyamoya disease can occur repeatedly,predicting the disease is difficult.Deep learning algorithms developed in recent years provide a new angle for identifying hidden risk factors,... Although intracranial hemorrhage in moyamoya disease can occur repeatedly,predicting the disease is difficult.Deep learning algorithms developed in recent years provide a new angle for identifying hidden risk factors,evaluating the weight of different factors,and quantitatively evaluating the risk of intracranial hemorrhage in moyamoya disease.To investigate whether convolutional neural network algorithms can be used to recognize moyamoya disease and predict hemorrhagic episodes,we retrospectively selected 460 adult unilateral hemispheres with moyamoya vasculopathy as positive samples for diagnosis modeling,including 418 hemispheres with moyamoya disease and 42 hemispheres with moyamoya syndromes.Another 500 hemispheres with normal vessel appearance were selected as negative samples.We used deep residual neural network(ResNet-152)algorithms to extract features from raw data obtained from digital subtraction angiography of the internal carotid artery,then trained and validated the model.The accuracy,sensitivity,and specificity of the model in identifying unilateral moyamoya vasculopathy were 97.64±0.87%,96.55±3.44%,and 98.29±0.98%,respectively.The area under the receiver operating characteristic curve was 0.990.We used a combined multi-view conventional neural network algorithm to integrate age,sex,and hemorrhagic factors with features of the digital subtraction angiography.The accuracy of the model in predicting unilateral hemorrhagic risk was 90.69±1.58%and the sensitivity and specificity were 94.12±2.75%and 89.86±3.64%,respectively.The deep learning algorithms we proposed were valuable and might assist in the automatic diagnosis of moyamoya disease and timely recognition of the risk for re-hemorrhage.This study was approved by the Institutional Review Board of Huashan Hospital,Fudan University,China(approved No.2014-278)on January 12,2015. 展开更多
关键词 brain central nervous system deep learning diagnosis HEMORRHAGE machine learning moyamoya disease moyamoya syndrome prediction REBLEEDING
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Significance of Cyclooxygenase-2 Elevation in Middle Cerebral Artery for Patients with Hemorrhagic Moyamoya Disease 被引量:2
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作者 章剑剑 熊忠伟 +8 位作者 王胜 孙守家 王昊 吴小林 王龙 张华楸 游超 王煜 陈劲草 《Journal of Huazhong University of Science and Technology(Medical Sciences)》 SCIE CAS 2016年第2期181-185,共5页
The etiology and pathogenesis of moyamoya disease(MMD) remain elusive. Some inflammatory proteins, such as cyclooxygenase(COX)-2, are believed to be implicated in the development of MMD. So far, the relationship b... The etiology and pathogenesis of moyamoya disease(MMD) remain elusive. Some inflammatory proteins, such as cyclooxygenase(COX)-2, are believed to be implicated in the development of MMD. So far, the relationship between COX-2 and MMD is poorly understood and reports on the intracranial vessels of MMD patients are scanty. In this study, tiny pieces of middle cerebral artery(MCA) and superficial temporal artery(STA) from 13 MMD patients were surgically harvested. The MCA and STA samples from 5 control patients were also collected by using the same technique. The expression of COX-2 was immunohistochemically detected and the average absorbance(A) of positively-stained areas was measured. High-level COX-2 expression was found in all layers of the MCA samples from all 5 hemorrhagic MMD patients, while positive but weak expression of COX-2 was observed only in the endothelial layer of the MCA samples from most ischemic MMD patients(6/8, 75%). The average A values of COX-2 in the hemorrhagic MMD patients were substantially higher than those in their ischemic counterparts(t=4.632, P=0.001). There was no significant difference in the COX-2 expression among the "gender" groups, or "radiographic grade" groups, or "lesion location" groups(P0.05 for all). The COX-2 expression was detected neither in the MCA samples from the controls nor in all STA specimens. Our results suggested that COX-2 was up-regulated in the MCA of MMD patients, especially in hemorrhagic MMD patients. We are led to speculate that COX-2 may be involved in the pathogenesis of MMD and even contribute to the hemorrhagic stroke of MMD patients. 展开更多
关键词 moyamoya disease middle cerebral artery COX-2 INFLAMMATION HEMORRHAGE
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Idiopathic steno-occlusive disease with bilateral internal carotid artery occlusion:A Case Report
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作者 Sherifa Ahmed Hamed Hosam Abozaid Yousef 《World Journal of Clinical Cases》 SCIE 2023年第13期3076-3085,共10页
BACKGROUND Moyamoya disease(MMD)is a rare cause of acute stroke and transient ischemic attacks in children.We described clinical,diagnostic features and follow-ups of a young child with acute stroke.CASE SUMMARY We re... BACKGROUND Moyamoya disease(MMD)is a rare cause of acute stroke and transient ischemic attacks in children.We described clinical,diagnostic features and follow-ups of a young child with acute stroke.CASE SUMMARY We report a 4-year-old girl with left hemiparesis after an acute ischemic stroke.Her history was also significant for repeated left or right focal motor seizures,generalized tonic-clonic convulsions and transient ischemic attacks.Her magnetic resonance imaging and computed tomography(CT)of the brain and magnetic resonance angiography,CT angiography and venography on the cerebral vessels revealed evidence of bilateral fronto-parietal ischemic infarctions,occlusion of the right and left internal carotid arteries started at its bifurcation and non-visualization of right and left anterior and middle cerebral arteries.There was evidence of progression in angiography manifested as development of collaterals from the basal perforating vessels,increase in the extent of large intracranial arterial stenosis/occlusion and extensive collateral circulation with predominance from the posterior circulation.Physical and neurological evaluation and comprehensive laboratory investigations excluded an obvious comorbid disease or risk factor for the child’s condition.The diagnosis of MMD was highly suggested as a cause of the child’s steno-occlusive condition.She was treated symptomatically with levetiracetam,an antiepileptic medication.Aspirin was prescribed for secondary prevention.Her clinical manifestations were improved during the three years of follow-up.Revascularization surgery was postponed.CONCLUSION Up to our knowledge,this is the first report for MMD in a child in our country.The clinical improvement and the stabilization of the child’s condition over the 3 years of follow-up could be attributed to the rapid and extensive recruitment of collaterals and absence of risk factors or comorbidities.Revascularization surgery is highly recommended. 展开更多
关键词 Steno-occlusive disease moyamoya disease Internal carotid artery Collateral circulation NEOVASCULARIZATION Case report
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Acute recurrent cerebral infarction caused by moyamoya disease complicated with adenomyosis:A case report 被引量:5
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作者 Shao Zhang Li-Ming Zhao +5 位作者 Bing-Qian Xue Hao Liang Gao-Chao Guo Yang Liu Rui-Yu Wu Chao-Yue Li 《World Journal of Clinical Cases》 SCIE 2022年第14期4617-4624,共8页
BACKGROUND Moyamoya disease is essentially an ischemic cerebrovascular disease.Here,we describe a case of acute recurrent cerebral infarction caused by moyamoya disease with concurrent adenomyosis which,to our knowled... BACKGROUND Moyamoya disease is essentially an ischemic cerebrovascular disease.Here,we describe a case of acute recurrent cerebral infarction caused by moyamoya disease with concurrent adenomyosis which,to our knowledge,is the first in the literature.A literature review is also presented.CASE SUMMARY A 38-year-old female presented to the Research and Treatment Center of Moyamoya Disease in our hospital with"left limb weakness"as the main symptom.She was diagnosed with acute cerebral infarction and moyamoya disease through magnetic resonance imaging and digital subtraction angiography.Prior to this,she had experienced a prolonged menstrual period of one-month duration.This was investigated and adenomyosis was diagnosed.After passing the acute cerebral infarction phase,the patient underwent surgery for adenomyosis followed by combined cerebral revascularization.During the postoperative follow-up,improvements of the perfusion imaging stage and modified Rankin Scale were observed.A review of the literature showed only 16 reported cases of gynecological diseases complicated with stroke.The clinical characteristics,pathogenesis,therapeutic effects,and long-term prognosis of these cases have been studied and discussed.CONCLUSION In patients with moyamoya disease,early management of gynecological-related bleeding disorders is essential to prevent the complications of cerebral events. 展开更多
关键词 moyamoya disease Combined cerebral revascularization Acute recurrent cerebral infarction Abnormal uterine bleeding ADENOMYOSIS Case report
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Histopathological Features of Middle Cerebral Artery and Superficial Temporal Artery from Patients with Moyamoya Disease and Enlightenments on Clinical Treatment
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作者 孙守家 章剑剑 +5 位作者 李正伟 熊忠伟 吴小林 王胜 舒凯 陈劲草 《Journal of Huazhong University of Science and Technology(Medical Sciences)》 SCIE CAS 2016年第6期871-875,共5页
The histopathological features of the middle cerebral artery(MCA) and superficial temporal artery(STA) from moyamoya disease(MMD) and their relationships with gender,age,angiography stage were explored.The cause... The histopathological features of the middle cerebral artery(MCA) and superficial temporal artery(STA) from moyamoya disease(MMD) and their relationships with gender,age,angiography stage were explored.The causes and the clinical significance of vasculopathy of STA were also discussed.The clinical data and specimens of MCA and STA from 30 MMD patients were collected.Twelve samples of MCA and STA from non-MMD patients served as control group.Histopathological examination was then performed by measuring the thickness of intima and media,and statistical analysis was conducted.The MCA and STA specimens from MMD group had apparently thicker intima and thinner media than those from the control group.There was no significant pathological difference between the hemorrhage group and non-hemorrhage group,and between the males and females in MMD patients.Neither the age nor the digital subtraction angiography(DSA) stage was correlated with the thickness of intima in MCA and STA.MMD is a systemic vascular disease involving both intracranial and extracranial vessels.Preoperative external carotid arteriography,especially super-selective arteriography of the STA,benefits the selection of donor vessel. 展开更多
关键词 moyamoya disease middle cerebral artery superficial temporal artery VASCULOPATHY
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Clinical manifestations and cerebral angiographic findings of moyamoya disease
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作者 Jian-Feng Han1,Cheng-Tai Wang2,Jian-Bo Yang1,Feng Guo1,Zheng-Yi Li11. Department of Neurology,the First Affiliated Hospital,Medical School of Xi’an Jiaotong University,Xi’an 710061 2. No.2 Department of Neurology,Hospital of Chinese Traditional Medicine of Baoji,Baoji 721000,China. 《Journal of Pharmaceutical Analysis》 SCIE CAS 2010年第2期131-134,共4页
Objective To study the clinical features and angiographic findings of moyamoya disease (MMD) as well as their relationship. Methods A total of 22 MMD patients received routine digital substraction angiography (DSA). T... Objective To study the clinical features and angiographic findings of moyamoya disease (MMD) as well as their relationship. Methods A total of 22 MMD patients received routine digital substraction angiography (DSA). The clinical manifestations and angiographic findings were analyzed. Results Clinical manifestations varied and each patient often had multiple symptoms,including cerebral infarction in 9 patients with an average age of 23.6 (13-39 years) and cerebral hemorrhage in 7 patients with an average age of 31.2 (28-46 years). Angiographic examination found that all the diseased sides showed MMD blood vessels. The patients who received encephalo-myo-arterio-synangiosis (EMAS) had better prognosis than those without receiving the treatment. Conclusion Cerebral infarction is frequent in children and adolescents with MMD,whereas cerebral hemorrhage is common in adults. DSA is a golden criterion for diagnosing MMD. Surgical treatment,EMAS blood supply reconstruction in particular,should be prescribed. 展开更多
关键词 moyamoya disease digital subtraction angiography clinical characteristic
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On-pump coronary artery bypass in moyamoya disease: A case report
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作者 Ismail Haberal Onur Gürer +2 位作者 Deniz Ozsoy Gürkan Cetin Murat Mert 《World Journal of Cardiovascular Diseases》 2013年第3期317-319,共3页
A 54-year-old female with pre-existing idiopathic moyamoya disease developed chest pain with acute myocardial infarction. Coronary angiography detected occluded and stenotic lesions in the coronary arteries. Right cor... A 54-year-old female with pre-existing idiopathic moyamoya disease developed chest pain with acute myocardial infarction. Coronary angiography detected occluded and stenotic lesions in the coronary arteries. Right coronary artery stenosis was treated by balloon angioplasty and stenting. Because of the restenosis, on-pump cardiopulmonary bypass was performed. The operation was uneventful and no perioperative cerebral ischemic episode occurred. The conclusion is that on-pump cardiopulmonary bypass preserving intraoperative hemodynamic parameters at an optimal level is a safe procedure in a patient with moyamoya disease. 展开更多
关键词 CARDIOPULMONARY BYPASS CORONARY Artery disease moyamoya disease
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Involuntary Movements Induced and Remitted by Indirect Bypass Surgery in a Patient with Moyamoya Disease
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作者 Takao Yanagawa Tetsuyoshi Horiuchi +2 位作者 Yosuke Hara Yu Fujii Kazuhiro Hongo 《Open Journal of Modern Neurosurgery》 2012年第4期71-74,共4页
Involuntary movement is an uncommon manifestation of moyamoya disease. We describe a 12-year-old boy who suffered from involuntary movement after the first surgery but it improved dramatically after the second surgery... Involuntary movement is an uncommon manifestation of moyamoya disease. We describe a 12-year-old boy who suffered from involuntary movement after the first surgery but it improved dramatically after the second surgery. Initially, the patient underwent an encephalo-duro-arterio-myosynangiosis on the right side and encephalo-duro-periostealsynangiosis on the bifrontal side, which ameliorated his motor weakness in his left upper extremity. However, involuntary movement appeared in his right upper extremity. Secondly, the patient underwent encephalo-duro-arterio-myo-periostealsynangiosis on the left side. Thereafter, the patient’s involuntary movement completely resolved. The involuntary movement might occur due to the unequal distribution of the blood flow in the basal ganglia. 展开更多
关键词 Involuntary MOVEMENT Indirect BYPASS SURGERY moyamoya disease
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Classification and treatment strategy for Moyamoya disease-related aneurysms
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作者 Yangchun Hu Xiaojian Wang +4 位作者 Chao Li Liang Zhao Jing Luo Lei Ye Baochun Cheng 《Chinese Neurosurgical Journal》 CAS CSCD 2024年第1期1-7,共7页
Background Moyamoya disease(MMD)is a cerebrovascular disorder characterized by progressive unilateral or bilateral stenosis of the distal internal carotid artery.As hemodynamic features in MMD patients alter,the comor... Background Moyamoya disease(MMD)is a cerebrovascular disorder characterized by progressive unilateral or bilateral stenosis of the distal internal carotid artery.As hemodynamic features in MMD patients alter,the comorbidity of intracranial aneurysm(IA)is sometimes observed clinically.We aim to investigate clinical characteristics and therapeutic strategies for the comorbidity of Moyamoya disease with intracranial aneurysms(MMD-IA).Methods A total of 13 MMD-IA patients were recruited in this study and were manifested to be intracranial hemorrhage.We reviewed the surgical technique notes for all patients.Results According to the locations of an aneurysm,MMD-IA could be divided into several categories:(1)MMD-IA at a circle of Willis—aneurysms usually located at the trunk of Willis circle;(2)MMD-IA at collateral anastomosis—aneurysms located at the distal end of collateral anastomosis;and(3)MMA-IA at basal ganglia region.In this report,aneurysms in 10 patients located at Willis circle,2 at the pericallosal artery,and 1 at the basal ganglia region.Among them,endovascular embolism was performed among 5 patients.Aneurysm clipping was conducted among 7 patients.A patient with an aneurysm at the basal ganglia region just accepted revascularization treatment.All the treatments were successful.Follow-up studies,ranging from 6 to 24 months,demonstrated all patients received satisfactory curative effects.Conclusion Diverse clinical presentations could be observed among MMD-IA patients.Individualized neurosurgical treatments should be chosen according to the locations of the aneurysm. 展开更多
关键词 moyamoya disease ANEURYSM CLIPPING REVASCULARIZATION Endovascular embolism
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GM-CSF Promotes the Development of Dysfunctional Vascular Networks in Moyamoya Disease
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作者 Huiqin Li Xiang Cao +8 位作者 Xinya Gu Mengqi Dong Lili Huang Chenglu Mao Shengnan Xia Haiyan Yang Xinyu Bao Yongbo Yang Yun Xu 《Neuroscience Bulletin》 SCIE CAS CSCD 2024年第4期451-465,共15页
Moyamoya disease(MMD)is a chronic occlusive cerebrovascular disease with the development of a network of abnormal vessels.Immune inflammation is associated with the occurrence and development of MMD.However,the mechan... Moyamoya disease(MMD)is a chronic occlusive cerebrovascular disease with the development of a network of abnormal vessels.Immune inflammation is associated with the occurrence and development of MMD.However,the mechanisms underlying the formation of the abnormal vascular network remain unclear.Twenty-eight patients with MMD,26 ischemic stroke patients,and 26 unrelated healthy volunteers were enrolled in this study The data showed that the levels of granulocyte-macrophage colony-stimulating factor(GM-CSF)were higher in MMD patients than in healthy controls(P<0.01),and GM-CSF was mainly from Th1 and Th17 cells in MMD.We found that increased GM-CSF drove monocytes to secrete a series of cytokines associated with angiogenesis,inflammation,and chemotaxis.In summary,our findings demonstrate for the first time the important involvement of GM-CSF in MMD and that GM-CSF is an important factor in the formation of abnormal vascular networks in MMD. 展开更多
关键词 moyamoya disease Vascular endothelial cells GM-CSF VEGF
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烟雾病合并颅内动脉瘤破裂及治疗后卒中的临床分析
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作者 王浩 王晨潮 +3 位作者 李东朋 董阳 李红伟 杨波 《中国微侵袭神经外科杂志》 CAS 2024年第4期221-225,共5页
目的探讨烟雾病(Moyamoya disease,MMD)合并颅内动脉瘤(intracranial aneurysm,IA)破裂及治疗后卒中的影响因素。方法回顾性分析245例MMD合并IA病例资料,根据是否发生IA破裂分为破裂组(n=71)和未破组(n=174)。采用单因素分析和Logistic... 目的探讨烟雾病(Moyamoya disease,MMD)合并颅内动脉瘤(intracranial aneurysm,IA)破裂及治疗后卒中的影响因素。方法回顾性分析245例MMD合并IA病例资料,根据是否发生IA破裂分为破裂组(n=71)和未破组(n=174)。采用单因素分析和Logistic回归分析,归纳IA破裂的危险因素。记录病人随访资料,采用COX回归分析,归纳治疗后卒中的危险因素。结果MMD合并IA破裂发生率为28.98%;年龄>52.5岁(OR=1.997)、收缩压(SBP)>160mmHg(OR=2.042)是MMD合并IA破裂的独立危险因素,而颈内动脉IA不易破裂(OR=0.353)。随访期卒中发生率为6.53%;中大型IA(HR=3.940)、IA破裂史(HR=3.900)是MMD合并IA随访卒中的独立危险因素。结论高龄、高SBP的MMD合并IA破裂风险高;合并有中大型IA及既往IA破裂史的病人,发生卒中风险高。 展开更多
关键词 烟雾病 颅内动脉瘤 破裂 卒中
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血管样本生物信息学分析鉴定烟雾病相关的潜在关键基因
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作者 刘洋 杨俊华 +1 位作者 吴俊 王硕 《中国卒中杂志》 北大核心 2024年第4期431-439,共9页
目的本研究对烟雾病患者血管样本的差异表达基因(differentially expressed genes,DEGs)进行生物信息学鉴定和分析,旨在探讨烟雾病的潜在发病机制。方法本研究以烟雾病和颈内动脉瘤患者大脑血管样本为研究对象。利用R语言线性模型微阵... 目的本研究对烟雾病患者血管样本的差异表达基因(differentially expressed genes,DEGs)进行生物信息学鉴定和分析,旨在探讨烟雾病的潜在发病机制。方法本研究以烟雾病和颈内动脉瘤患者大脑血管样本为研究对象。利用R语言线性模型微阵列数据(linear models for microarray data,limma)分析包对基因表达综合数据库(gene expression omnibus,GEO)中的GSE141025数据集进行分析,该数据集涵盖4例烟雾病患者和4例颈内动脉瘤患者的大脑中动脉和颞浅动脉样本各1个,共计16个样本。选择烟雾病患者的大脑中动脉、颞浅动脉及颈内动脉瘤患者的颞浅动脉共12个样本进行DEGs筛选。通过R语言功能富集分析工具包clusterProfiler,对筛选出的DEGs进行基因本体(gene ontology,GO)富集分析和京都基因与基因组百科全书(Kyoto encyclopedia of genes and genomes,KEGG)通路分析。利用STRING数据库构建蛋白质-蛋白质相互作用(proteinprotein interaction,PPI)网络,并使用网络可视化软件Cytoscape进行蛋白质网络的可视化和枢纽基因筛选。结果本研究在烟雾病患者的大脑中动脉与颞浅动脉样本间鉴定出138个DEGs,包括18个上调基因和120个下调基因。GO富集分析显示,以上DEGs在细胞外基质、受体配体活性和生长因子活性等方面显著富集,可能与烟雾病相关的血管病变和神经保护机制有关。KEGG通路分析提示,DEGs主要在酪氨酸代谢通路中富集。通过PPI网络分析,共筛选出9个枢纽基因,包括骨膜蛋白(periostin,POSTN)、脑源性神经营养因子(brain derived neurotrophic factor,BDNF)、血小板衍生生长因子受体α(platelet derived growth factor receptor alpha,PDGFRA)、Thy-1细胞表面抗原(Thy-1 cell surface antigen,THY1)、ⅩⅤ型胶原蛋白α1链(collagen typeⅩⅤalpha 1 chain,COL15A1)、成纤维细胞生长因子7(fibroblast growth factor 7,FGF7)、光蛋白聚糖(l umi can,LUM)、层粘连蛋白α2亚基(laminin subunit alpha 2,LAMA2)和RELN(reelin)。此外,上调基因delta样典型Notch配体4(delta like canonical Notch ligand 4,DLL4)在本研究中首次被发现可能在烟雾病中扮演重要角色,或与烟雾病的病理性血管生成有关。结论细胞外基质、生长因子及其受体的表达失调等可能参与烟雾病的发病过程。DEGs分析筛选出的枢纽基因(POSTN、BDNF、PDGFRA、THY1、COL15A1、FGF7、LUM、LAMA2、RELN)以及DLL4可能在烟雾病的病理形成过程中发挥作用。 展开更多
关键词 烟雾病 生物信息学分析 基因表达数据 枢纽基因
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双元护理干预模式对烟雾病患者术后心理健康及康复效果的影响
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作者 周红娜 行君 赵黎明 《临床心身疾病杂志》 CAS 2024年第1期143-148,160,共7页
目的探讨双元护理模式对烟雾病患者术后疾病不确定感、心理弹性、自护能力和康复情况的影响。方法将116例烟雾病患者按随机数字表法分为观察组和对照组,各58例。对照组围术期给予常规护理,观察组采用双元护理干预模式,观察住院全程。比... 目的探讨双元护理模式对烟雾病患者术后疾病不确定感、心理弹性、自护能力和康复情况的影响。方法将116例烟雾病患者按随机数字表法分为观察组和对照组,各58例。对照组围术期给予常规护理,观察组采用双元护理干预模式,观察住院全程。比较入院时及出院时两组患者Mishel疾病不确定感量表(MUIS-A)、心理弹性量表(CD-RISC)、自护能力量表(ESCA)评分及术后并发症发生率、康复情况。结果两组患者出院时MUIS-A各维度评分及总分均较入院时降低,且观察组低于对照组(P<0.05或0.01)。两组患者出院时CD-RISC、ESCA各维度评分及总分均较入院时升高,且观察组高于对照组(P<0.05或0.01)。观察组患者术后并发症总发生率低于对照组(P<0.05),术后住院时间少于对照组(P<0.01)。结论双元护理干预模式应用于烟雾病术后护理有利于减轻患者疾病不确定感,提升心理弹性和自护能力,对减少术后并发症的发生,促进患者康复具有积极作用。 展开更多
关键词 烟雾病 双元护理模式 疾病不确定感 心理弹性 自护能力 并发症 康复
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Moyamoya病侧支途径的DSA评价及其与临床的关系 被引量:28
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作者 赵云辉 马著彬 张玉忠 《临床放射学杂志》 CSCD 北大核心 2001年第10期735-739,共5页
目的 应用 DSA分析 Moyamoya病的侧支途径 ,探讨侧支循环建立情况与临床、CT表现之间的关系。材料与方法 回顾分析 30例经脑血管造影确诊的 Moyamoya病患者的血管造影表现、CT结果及临床资料。结果 所有病例均出现颈内动脉或其分支... 目的 应用 DSA分析 Moyamoya病的侧支途径 ,探讨侧支循环建立情况与临床、CT表现之间的关系。材料与方法 回顾分析 30例经脑血管造影确诊的 Moyamoya病患者的血管造影表现、CT结果及临床资料。结果 所有病例均出现颈内动脉或其分支不同程度狭窄或闭塞和颅底异常血管网 (MMD血管 ) ;7例可见后组 MMD血管 ;2 0例由 Willis环提供侧支血流 ;14例眼动脉增粗 ;17例软脑膜吻合支明显增粗 ;1例脉络膜前动脉增粗。 30例中头颅 CT显示有脑梗塞灶 4例 ,正常表现 3例 ,脑出血 2 3例。结论 DSA是诊断 Moyamoya病的金标准 ,对其侧支途径的研究提供了可靠方法。 Moyamoya病的侧支循环建立情况与临床密切相关。明确 Moyamoya病的侧支循环情况 ,可为临床治疗和预后判断提供可靠依据。 展开更多
关键词 侧支途径 moyamoya 数字减影血管造影 CT 诊断 治疗 外科手术
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