Moya Moya Syndrome With or Withtout Sickle Cell Disease

Overview

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)

Full Title of Study: “Descriptive Bicentric Study of Moya Moya Syndrome and Disease in Sickle Cell Disease Patients and Not Sickle Cell Disease”

Study Type

  • Study Type: Observational
  • Study Design
    • Time Perspective: Retrospective
  • Study Primary Completion Date: December 1, 2022

Clinical Trial Outcome Measures

Primary Measures

  • time from Moya Moya diagnosis to first or recurrent stroke or bad prognosis achivement
    • Time Frame: 1 day
    • 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)

Secondary Measures

  • Time from MM diagnosis to Stroke
    • Time Frame: 1 day
    • Time from MM diagnosis to Stroke
  • poor prognosis or death
    • Time Frame: 1 day
    • poor prognosis or death (mRS> 2) at the last clinical evaluation.

Participating in This Clinical Trial

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

Gender Eligibility: All

Minimum Age: 15 Years

Maximum Age: N/A

Are Healthy Volunteers Accepted: No

Investigator Details

  • Lead Sponsor
    • University Hospital, Montpellier
  • Provider of Information About this Clinical Study
    • Sponsor
  • Overall Official(s)
    • Estelle BRITHMER, Resident, Principal Investigator, University Hospital, Montpellier
  • Overall Contact(s)
    • Nicolas Gaillard, MD, 4 67 33 74 13, n-gaillard@chu-montpellier.fr

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