1.01.02 2020:02:20 22:11:59.145 moyamoya 330998 30 1 3 3 NCT03613701 8157113 Affiliated Hospital to Academy of Military Medical Sciences OTHER Relationship Between Endothelial Progenitor Cells and Revascularization Effect of Moyamoya Disease Relationship Between Endothelial Progenitor Cells and Revascularization Effect of Moyamoya Disease REPCREMMD January 2019 Recruiting No September 1, 2017 Actual September 1, 2019 Anticipated December 1, 2020 Anticipated July 29, 2018 August 2, 2018 August 3, 2018 Actual January 29, 2019 January 31, 2019 Actual Sponsor Affiliated Hospital to Academy of Military Medical Sciences OTHER No No Moyamoya disease is a chronic cerebrovascular disease，The typical pathological manifestations are the stenosis or occlusion of the distal internal carotid artery and/or middle cerebral artery, and the proximal anterior cerebral artery. Meanwhile, the abnormal vascular net, which is the smokey vessel, occurs at the bottom of the brain. Currently the pathogenesis of this disease is unknown. Limited studies have reported the expression of endothelial progenitor cells (EPCs) in moyamoya disease, but the results were inconsistent. Some investigators believe that the number of EPCs in peripheral blood of patients with moyamoya disease is increased, while others believe that the number of EPCs in peripheral blood of moyamoya patients is reduced. Therefore, the investigators need to find a more accurate detection method to confirm the growth of EPC in patients with moyamoya disease. At the same time, whether there is endothelial injury in patients with smoke disease, and the expression of endothelial cells (CEC) in patients with smoke disease, there is no research on this aspect at home and abroad. Objective: Detect the expression of endothelial progenitor cells and endothelial cells from peripheral blood of patients with moyamoya disease, and to assess the relationship between clinical characteristics. Design: A single center study, and planned to enroll 120 patients. The present study was to detect the quantities of EPC from peripheral blood in Moyamoya disease by flow cytometry, and to identify the relationship of endothelial progeIlitor cells and effect of the revascularization on Moyamoya disease. The present study also use cerebral ischemia animal model foe intervention experiment, to explore whether EPC can promote vascular remodeling effect of ischemic cerebrovascular disease, and to provide new thought for the treatment of chronic cerebrovascular disorder. Moyamoya Disease Moyamoya disease Endothelial progenitor cells revascularization Observational Yes 6 Months Case-Control Prospective 120 Anticipated Moyamoya disease patients Moyamoya disease patients/Healthy volunteers Expression of endothelial progenitor cells and endothelial cells in peripheral blood Expression of endothelial progenitor cells and endothelial cells in peripheral 2017.9-2018.8 Inclusion Criteria: Whole-brain vessels angiography or magnetic resonance arteriography (MRA) has the following manifestations: stenosis or occlusion of terminal internal carotid artery or the anterior cerebral artery and/or initiating middle cerebral artery; In the arterial phase, the abnormal smokey vascular net near the occlusive or stenosis lesion can be seen. For patients with stable stroke, there was no acute or subacute cerebral infarction or cerebral hemorrhage, and at least 3 months before the last cerebral infarction or cerebral hemorrhage events. Exclusion Criteria: Exclude atherosclerosis, autoimmune diseases, meningitis, intracranial tumors, multiple neurofibromatosis, Down syndrome, craniocerebral trauma, radiation injury, and other underlying diseases that may cause smoke. Acute or subacute cerebral infarction or cerebral hemorrhage were excluded. Accepts Healthy Volunteers All 18 Years 60 Years Adult Health volunteers' inclusion criteria: Age between 18-60; Male or female; Exclusion criteria: Exclude the volunteers with history of cerebrovascular disease and heart disease. Probability Sample Lian Duan, Chief Contact 0086-10-66947156 keyan307@163.com Lian Duan, Chief The 307th Hospital of Military Chinese People's Liberation Army Study Chair The 307th Hospital of Military Chinese People's Liberation Army Recruiting Beijing Beijing 100071 China Lian Duan, Chief Contact 0086-10-66947156 keyan307@163.com No February 21, 2020 D000009072 Moyamoya Disease D000002340 Carotid Artery Diseases D000002561 Cerebrovascular Disorders D000001927 Brain Diseases D000002493 Central Nervous System Diseases D000009422 Nervous System Diseases D000002539 Cerebral Arterial Diseases D000020765 Intracranial Arterial Diseases D000001157 Arterial Occlusive Diseases D000014652 Vascular Diseases D000002318 Cardiovascular Diseases M10615 Moyamoya Disease Moyamoya Disease high M4176 Carotid Artery Diseases low M4393 Cerebrovascular Disorders low M3786 Brain Diseases low M4325 Central Nervous System Diseases low M4371 Cerebral Arterial Diseases low M21105 Intracranial Arterial Diseases low M3046 Arterial Occlusive Diseases low M15983 Vascular Diseases low T3915 Moyamoya Disease Moyamoya Disease high BC10 Nervous System Diseases BC14 Heart and Blood Diseases All All Conditions Rare Rare Diseases NCT02434302 13NR31 Great Ormond Street Hospital for Children NHS Foundation Trust OTHER Characteristics and Outcomes of Childhood Moyamoya in the UK Characteristics and Outcomes of Childhood Moyamoya in the UK April 2015 Unknown status Recruiting No August 2014 August 2016 Anticipated August 2016 Anticipated April 30, 2015 May 4, 2015 May 5, 2015 Estimate May 4, 2015 May 5, 2015 Estimate Sponsor Great Ormond Street Hospital for Children NHS Foundation Trust OTHER No This is a study to ascertain the number of children with moyamoya in the UK, their presenting features, clinical course and outcomes. The study ahs 3 components: Patient identification via the British Paediatric Surveillance Unit, and the 21 collaborating centres Record review/patient interview to determine clinical features & outcomes Centralised review of imaging data to ascertain radiological phenotypes Moyamoya Observational No Cohort Cross-Sectional 150 Anticipated Number of children with moyamoya in UK 2 years clinical features & outcomes of childhood moyamoya 2 years radioplogical features of childhood Moyamoya 2 years Inclusion Criteria: Radiological diagnosis of moyamoya Exclusion Criteria: Refuse consent No All 18 Years Child Adult Children (0-18y) with moyamoya in the UK over study period (2014-2016 August) Non-Probability Sample Vijeya Ganesan, MB ChB MD Contact 02074059200 v.ganesan@ucl.ac.uk vijeya ganesan, MB ChB MD UCL Institute of Child Health Principal Investigator Great Ormond Street Hospital for CHidlren NHS Foundation Trust Recruiting London WC1N 3LU United Kingdom vijeya ganesan, MB ChB MD Contact 02074059200 v.ganesan@ucl.ac.uk February 21, 2020 D000009072 Moyamoya Disease D000002340 Carotid Artery Diseases D000002561 Cerebrovascular Disorders D000001927 Brain Diseases D000002493 Central Nervous System Diseases D000009422 Nervous System Diseases D000002539 Cerebral Arterial Diseases D000020765 Intracranial Arterial Diseases D000001157 Arterial Occlusive Diseases D000014652 Vascular Diseases D000002318 Cardiovascular Diseases M10615 Moyamoya Disease Moyamoya high M4176 Carotid Artery Diseases low M4393 Cerebrovascular Disorders low M3786 Brain Diseases low M4325 Central Nervous System Diseases low M4371 Cerebral Arterial Diseases low M21105 Intracranial Arterial Diseases low M3046 Arterial Occlusive Diseases low M15983 Vascular Diseases low T3915 Moyamoya Disease Moyamoya high BC10 Nervous System Diseases BC14 Heart and Blood Diseases All All Conditions Rare Rare Diseases NCT03627975 Z171100001017144 Affiliated Hospital to Academy of Military Medical Sciences OTHER Effect of Surgical Revascularization on Hemorrhagic Moyamoya Disease Effect of Surgical Revascularization and Conservative Treatment on Hemorrhagic Moyamoya Disease ESRHMMD January 2019 Recruiting No October 1, 2017 Actual October 1, 2020 Anticipated October 1, 2021 Anticipated July 29, 2018 August 8, 2018 August 14, 2018 Actual January 29, 2019 January 31, 2019 Actual Sponsor Affiliated Hospital to Academy of Military Medical Sciences OTHER No No Moyamoya Disease(MMD), also known as spontaneous basilar artery occlusion, is characterized by the gradual thickening of arterial intima at the distal carotid artery and the proximal portion of anterior/middle cerebral artery, the gradual stenosis or occlusion of arterial lumen, and the compensatory expansion of basilar cerebral perforating arteries. Cerebral infarction and cerebral hemorrhage are common clinical symptoms of MMD with high morbidity of disability. For ischemic moyamoya disease, intracranial/extracranial revascularization is the preferred treatment. However, for patients with hemorrhagic moyamoya disease, there is controversy about whether to have surgical treatment, the timing and the method of surgical treatment, and the effect of surgical treatment to prevent rebleeding due to the lack of large sample, multi-center, prospective randomized studies. At present, the studies on the effect of revascularization and conservative treatment on hemorrhagic moyamoya disease are retrospective case analyses without randomized control. The sample size of these studies are small, and the conclusions obtained are inconsistent. Due to the differences in the epidemiology and episode type of moyamoya disease in different countries, there is no prospective, randomized controlled study of blood type moyamoya disease in China to confirm the efficacy of revascularization and lack of uniform norms and standards. Objective: The aim of this study is to perform a prospective, randomized study on hemorrhagic moyamoya disease to confirm the effect of revascularization in China, and to establish specifications and standards to guide the treatment options for hemorrhagic moyamoya disease as well. Design: This study is a single-center study and plan to include 108 patients. According to a random number table, hemorrhagic moyamoya patients will be assigned to three groups: conservative treatment group, direct revascularization group and indirect revascularization group. A prospective, randomized study will be carried out to evaluate the effect of revascularization and conservative treatment on the reduction of rebleeding risk and improvement of ischemia in adult patients with hemorrhagic moyamoya disease. Observation Measures: 1.Rebleeding; 2.Cerebral infarction resulting in severe disability (mRS score≥3); 3.Severe disability or death caused by other reasons; 4. Patients in conservative treatment group need revascularization due to progressive ischemic stroke or progressive Transient ischemic attack(TIA). Moyamoya Disease revascularization hemorrhagic moyamoya disease Interventional Not Applicable Randomized Parallel Assignment Treatment None (Open Label) 108 Anticipated Conservative treatment No Intervention Conservative treatment. The conservative treatment of hemorrhagic moyamoya disease mainly includes the control of hypertension, the prevention and treatment of secondary epilepsy, the control of intracranial hypertension(including the application of mannitol and glycerol fructose, etc.), and the corresponding symptomatic and neurotrophic treatment. Non-specific treatment is mainly deal with intracranial hematoma, including intraventricular drainage, intracranial hematoma evacuation, and ventriculoperitoneal shunt. Indirect vascular reconstruction surgery Experimental Indirect vascular reconstruction surgery. In addition to the pharmacotherapy used in conservative treatment, encephalo-duro-arterio-synangiosis(EDAS) is performed. The surgery is performed according to the procedures described by Matsushima. Procedure: Conservative treatment direct vascular reconstruction surgery Experimental Direct vascular reconstruction surgery. In addition to the pharmacotherapy used in conservative treatment, the superficial temporal artery(STA) and middle cerebral artery(MCA) bypass surgery is performed. The operation is the modified EDAS which basically similar to EDAS, but the surgical incision is as low as possible. And if necessary, the STA may not be preserved. The bone flap should be large enough to select the right recipient blood vessel. Procedure: Conservative treatment Procedure Conservative treatment The conservative treatment of hemorrhagic moyamoya disease mainly includes the control of hypertension, prevention and treatment of secondary epilepsy, the control of intracranial hypertension, and the corresponding symptomatic and neurotrophic treatment. In addition to the pharmacotherapy used in conservative treatment, encephalo-duro-arterio-synangiosis(EDAS) will be performed according to the procedures described by Matsushima. In addition to the pharmacotherapy used in conservative treatment, the superficial temporal artery(STA) and middle cerebral artery(MCA) by pass surgery is performed. The operation is the modified EDAS which basically similar to EDAS, but the surgical incision is as low as possible. And the STA may not be preserved. Indirect vascular reconstruction surgery direct vascular reconstruction surgery indirect revascularization direct revascularization Rebleeding All enrolled patients were followed up regularly by telephone, outpatient and inpatient visits. The observed end-point events of rebleeding Through study completion, an average of 1 year Severe Disability Cerebral infarction resulting in severe disability (mRS score≥3) Through study completion, an average of 1 year Severe Disability or Death Severe disability or death caused by other reasons Through study completion, an average of 1 year Vascular Reconstruction due to Progressive Ischemic Stroke or Progressive TIA Patients in conservative treatment group needs vascular reconstruction due to progressive ischemic stroke or progressive TIA. Through study completion, an average of 1 year Inclusion Criteria: DSA/MRA shows stenosis or occlusion in the distal internal carotid artery or the proximal portion of anterior/middle cerebral artery Abnormal vascular network appeared in the brain Lesions showed bilateral changes Age≥18 years With the onset of cerebral hemorrhage No cerebral infarction or cerebral hemorrhage occurred within the last month At least one month after the acute phase of cerebral hemorrhage or related diseases was treated Exclusion Criteria: Patients with moyamoya syndrome secondary to systemic diseases such as arteriosclerosis, sickle cell anemia, radiation therapy, etc.. Patients with severe mental disorders such as psychosis, liver and kidney dysfunction, poor blood pressure or blood glucose control, severe depression and substance abuse, low IQ, and acute phase of severe stroke with definite limb dysfunction should also be excluded. No All 18 Years Adult Older Adult Lian Duan, Chief Contact 0086-10-66947156 keyan307@163.com Lian Lian, Chief The 307th Hospital of Military Chinese People's Liberation Army Study Chair The 307th Hospital of Military Chinese People's Liberation Army Recruiting Beijing Beijing 100071 China Lian Duan, Chief Contact No February 21, 2020 M9925 Mannitol low M236194 Xylometazoline low M7679 Glycerol low NaAg Natriuretic Agents All All Drugs and Chemicals VaCoAg Vasoconstrictor Agents Resp Respiratory System Agents D000009072 Moyamoya Disease D000002340 Carotid Artery Diseases D000002561 Cerebrovascular Disorders D000001927 Brain Diseases D000002493 Central Nervous System Diseases D000009422 Nervous System Diseases D000002539 Cerebral Arterial Diseases D000020765 Intracranial Arterial Diseases D000001157 Arterial Occlusive Diseases D000014652 Vascular Diseases D000002318 Cardiovascular Diseases M10615 Moyamoya Disease Moyamoya Disease high M4176 Carotid Artery Diseases low M4393 Cerebrovascular Disorders low M3786 Brain Diseases low M4325 Central Nervous System Diseases low M4371 Cerebral Arterial Diseases low M21105 Intracranial Arterial Diseases low M3046 Arterial Occlusive Diseases low M15983 Vascular Diseases low T3915 Moyamoya Disease Moyamoya Disease high BC10 Nervous System Diseases BC14 Heart and Blood Diseases All All Conditions Rare Rare Diseases