Moya Moya Syndrome With or Withtout Sickle Cell Disease (BMM-ScD)

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

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)

Study Overview

• Status: Recruiting
• Conditions: Moya Moya Disease

• Study Type: Observational
• Enrollment (Anticipated): 50

Contacts and Locations

• Study Contact: Name: Nicolas Gaillard, MD; Phone Number: 33 4 67 33 74 13; Email: n-gaillard@chu-montpellier.fr

Study Locations

• France
  • Montpellier, France, 34295
    • Recruiting
    • Uhmontpellier
    • Contact: Nicolas Gaillard, MD; Phone Number: 33 4 67 33 74 13; Email: n-gaillard@chu-montpellier.fr
    • Contact: Estelle Brithmer; Phone Number: e-Brithmer@chu-montpellier.fr

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 15 years and older (Child, Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

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

Description

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

Study Plan

How is the study designed?

What is the study measuring?

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

Collaborators and Investigators

• Sponsor: University Hospital, Montpellier
• Investigators: Principal Investigator: Estelle BRITHMER, Resident, University Hospital, Montpellier

Study record dates

Study Major Dates

• Study Start (Actual): May 1, 2021
• Primary Completion (Anticipated): December 1, 2022
• Study Completion (Anticipated): December 1, 2022

Study Registration Dates

• First Submitted: June 10, 2021
• First Submitted That Met QC Criteria: September 17, 2021
• First Posted (Actual): September 20, 2021

Study Record Updates

• Last Update Posted (Actual): September 24, 2021
• Last Update Submitted That Met QC Criteria: September 18, 2021
• Last Verified: June 1, 2021

More Information

Terms related to this study

• Keywords: Moya Moya syndrome; Moya Moya
• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Cerebrovascular Disorders; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Arterial Occlusive Diseases; Hematologic Diseases; Genetic Diseases, Inborn; Anemia; Carotid Artery Diseases; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Hemoglobinopathies; Cerebral Arterial Diseases; Intracranial Arterial Diseases; Anemia, Sickle Cell; Moyamoya Disease
• Other Study ID Numbers: RECHMPL21_0343

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED
• IPD Plan Description: NC

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No