1.01.05 2022:06:26 23:10:13.572 moyamoya 419487 38 1 3 3 NCT05160454 16NC24 Great Ormond Street Hospital for Children NHS Foundation Trust OTHER NIRS to Evaluate Haemodynamic Reserve in Paediatric Moyamoya NIRS to Evaluate Haemodynamic Reserve in Paediatric Moyamoya NIRS moyamoya November 2021 Suspended COVID19 No February 14, 2018 Actual June 30, 2022 Anticipated June 30, 2022 Anticipated November 8, 2019 December 2, 2021 December 16, 2021 Actual December 2, 2021 December 16, 2021 Actual Sponsor Great Ormond Street Hospital for Children NHS Foundation Trust OTHER No No No Near infrared spectroscpy is a validated method of evaluating cerebral blood flow. The aim in this pilot study to explore its use in children with moyamoya disease. Moyamoya disease is a cerebrovascular condition in which there is chronic brain hypoperfusion. As surgery can possibly augment brain blood flow, a method for evalutaing cerebrovascualr reserve would be helpful in selecting patients for intervention. Near infrared spectroscopy (NIRS) is a validated non-invasive method of evaluating cerebral blood flow. We aim to investigate the feasibility and tolerability of using NIRS haemodynamic reserve in children with moyamoya and controls by evaluating cerebral blood flow in the baseline state and after breath-holding. Feasibility will be assessed by how many successful studies are carried out. Tolerability will be assessed by asking children some simple questions about their experience. Moyamoya Interventional Not Applicable Non-Randomized Single Group Assignment patients and controls Other None (Open Label) 20 Anticipated patient Active Comparator child with moyamoya Device: near infrared spectroscopy control Active Comparator unaffected control Device: near infrared spectroscopy Device near infrared spectroscopy evaluate cerebral blood flow with and without breatholding control patient Number of NIRS studies that can be completed in moyamoya patients how many studies can be completed 2years Patient questionnaire to evaluate children's experience of NIRS in moyamoya ask children re their experience 2years Inclusion Criteria: Patients with moyamoya and unaffected healthy controls - Exclusion Criteria: age <6 - Accepts Healthy Volunteers All 6 Years 18 Years Child Adult Great Ormond Street Hospital for Children NHS Foundation Trust London WC1N 3JH United Kingdom No June 27, 2022 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 M11184 Moyamoya Disease Moyamoya high M4746 Carotid Artery Diseases low M4962 Cerebrovascular Disorders low M4356 Brain Diseases low M4894 Central Nervous System Diseases low M4940 Cerebral Arterial Diseases low M21674 Intracranial Arterial Diseases low M3617 Arterial Occlusive Diseases low M16552 Vascular Diseases low T3892 Moyamoya Disease Moyamoya high BC10 Nervous System Diseases BC14 Heart and Blood Diseases All All Conditions Rare Rare Diseases NCT05050344 RECHMPL21_0343 University Hospital, Montpellier OTHER Moya Moya Syndrome With or Withtout Sickle Cell Disease Descriptive Bicentric Study of Moya Moya Syndrome and Disease in Sickle Cell Disease Patients and Not Sickle Cell Disease BMM-ScD June 2021 Recruiting No May 1, 2021 Actual December 1, 2022 Anticipated December 1, 2022 Anticipated June 10, 2021 September 17, 2021 September 20, 2021 Actual September 18, 2021 September 24, 2021 Actual Sponsor University Hospital, Montpellier OTHER No No No Moya Moya disease or syndrome ar characterized by a progressive or occlusion of the intracranial carotid arteries and their mainproximal branches, followed by the development of fragile neovessels at the base of the skull, leding to a high risk of both ischemic and hemorragic stroke over time. Moya Moya syndrome are associated to a variety of disease, which main frequent is sickle cell disease (SCD). Among patients with SCD who had suffered from at least one ischemic stroke, the prevalence of moya moya syndrome was estimated up to 43%. In general, therapeutic strategies in Moya Moya to prevent first ever ou recurrent stroke can be divided into conservative medical treatment and surgical revascularisation (direct bypass, indirect bypass or combined bypass). The aim of this study is to compare prognosis of patients with Moya Moya syndrome associated with sickle cell disease or not. The investigators retrospectiveluy analysed medical chart from 2010 to 2021 of patients with Moya Moya disease or syndrome at two French university hospitals (including a center of the french West Indies where prevalence of sickle cell disease is high). The diagnosis was based on angiography or MRI records showing uni- or bilateral stenosis of distal intracranial internal carotide arteries or middle cerebral arteries associated wirh classic collateral network. Main endpoint will be comparison of a composite outcome defined as time from Moya Moya diagnosis to first or recurrent stroke or bad prognosis achivement (defined by modified Rankin score >2) Moya Moya Disease Moya Moya syndrome Moya Moya Observational No Cohort Retrospective 50 Anticipated time from Moya Moya diagnosis to first or recurrent stroke or bad prognosis achivement composite endpoint of outcome defined as time from Moya Moya diagnosis to first or recurrent stroke or bad prognosis achivement (defined by modified Rankin score >2) 1 day Time from MM diagnosis to Stroke Time from MM diagnosis to Stroke 1 day poor prognosis or death poor prognosis or death (mRS> 2) at the last clinical evaluation. 1 day Inclusion criteria: age >=15 patients at diagnosis of Moya Moya disease and syndrome Exclusion criteria: misclassified patients clinical diagnosis of MM not confirmed by angiography or MRI No All 15 Years Child Adult Older Adult patients >=15 years of age at diagnosis of Moya Moya disease and syndrome from january 2010 to february 2021 at Montpellier and Martinique University Hospital Non-Probability Sample Nicolas Gaillard, MD Contact 4 67 33 74 13 33 n-gaillard@chu-montpellier.fr Estelle BRITHMER, Resident University Hospital, Montpellier Principal Investigator Uhmontpellier Recruiting Montpellier 34295 France Nicolas Gaillard, MD Contact 4 67 33 74 13 33 n-gaillard@chu-montpellier.fr Estelle Brithmer Contact e-Brithmer@chu-montpellier.fr Undecided NC June 27, 2022 D000009072 Moyamoya Disease D000000755 Anemia, Sickle Cell D000000745 Anemia, Hemolytic, Congenital D000000743 Anemia, Hemolytic D000000740 Anemia D000006402 Hematologic Diseases D000006453 Hemoglobinopathies D000030342 Genetic Diseases, Inborn 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 M3237 Anemia, Sickle Cell Sickle Cell Disease high M15507 Syndrome low M11184 Moyamoya Disease Moya Moya high M3222 Anemia low M8699 Hemolysis low M3225 Anemia, Hemolytic low M3227 Anemia, Hemolytic, Congenital low M8642 Hematologic Diseases low M8691 Hemoglobinopathies low M22839 Genetic Diseases, Inborn low M4746 Carotid Artery Diseases low M4962 Cerebrovascular Disorders low M4356 Brain Diseases low M4894 Central Nervous System Diseases low M4940 Cerebral Arterial Diseases low M21674 Intracranial Arterial Diseases low M3617 Arterial Occlusive Diseases low M16552 Vascular Diseases low T5229 Sickle Cell Anemia Sickle Cell Disease high T3892 Moyamoya Disease Moya Moya high BC15 Blood and Lymph Conditions BC16 Diseases and Abnormalities at or Before Birth All All Conditions BC23 Symptoms and General Pathology BC10 Nervous System Diseases BC14 Heart and Blood Diseases Rare Rare Diseases 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 Unknown status 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 June 27, 2022 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 M11184 Moyamoya Disease Moyamoya Disease high M4746 Carotid Artery Diseases low M4962 Cerebrovascular Disorders low M4356 Brain Diseases low M4894 Central Nervous System Diseases low M4940 Cerebral Arterial Diseases low M21674 Intracranial Arterial Diseases low M3617 Arterial Occlusive Diseases low M16552 Vascular Diseases low T3892 Moyamoya Disease Moyamoya Disease high BC10 Nervous System Diseases BC14 Heart and Blood Diseases All All Conditions Rare Rare Diseases