Moya Moya Syndrome With or Withtout Sickle Cell Disease

Study Overview
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Brief Summary
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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)

Official Title
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Descriptive Bicentric Study of Moya Moya Syndrome and Disease in Sickle Cell Disease Patients and Not Sickle Cell Disease

Conditions
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Moya Moya Disease

Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 15 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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 | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| 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 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Moya Moya syndrome, Moya Moya