價Check for updatesEUROPEANGuidelineSTROKE JOURNALEuropean Stroke JoumalEuropean Stroke Organisation (ESO)2023.Vdl.8(1)55-84European Stroke Organisation 2023Article reuse guidelines:Guidelines on Moyamoya angiopathysagepub.com/journals-permissionsD○L10.1177/23969873221144089Endorsed by Vascular European Referencejournals.sagepub.com/home/esoSSAGENetwork(VASCERN)Anna Bersano,Nadia Khan2.3,Blanca Fuentes,Francesco Acerbi,Isabella Canavero',Elisabeth Tournier-Lasserves,Peter Vajcoczy5,Maria Luisa Zedde7,Salman Hussain,Sabrina Lemeret,Markus Kraemer,10 and Dominique Herve!!AbstractThe European Stroke Organisation (ESO)guidelines on Moyamoya Angiopathy (MMA),developed according to ESOstandard operating procedure and Grading of Recommendations,Assessment,Development and Evaluation (GRADE)methodology,were compiled to assist clinicians in managing patients with MMA in their decision making.A working groupinvolving neurologists,neurosurgeons,a geneticist and methodologists identified nine relevant clinical questions,performedsystematic literature reviews and,whenever possible,meta-analyses.Quality assessment of the available evidence was madewith specific recommendations.In the absence of sufficient evidence to provide recommendations,Expert ConsensusStatements were formulated.Based on low quality evidence from one RCT,we recommend direct bypass surgery in adultpatients with haemorrhagic presentation.For ischaemic adult patients and children,we suggest revascularization surgeryusing direct or combined technique rather than indirect,in the presence of haemodynamic impairment and with an intervalof 6-12weeks between the last cerebrovascular event and surgery.In the absence of robust trial,an Expert Consensuswas reached recommending long-term antiplatelet therapy in non-haemorrhagic MMA,as it may reduce risk of embolicstroke.We also agreed on the utility of performing pre-and post-operative haemodynamic and posterior cerebral arteryassessment.There were insufficient data to recommend systematic variant screening of RNF213 p.R4810K.Additionally,we suggest that long-term MMA neuroimaging follow up may guide therapeutic decision making by assessing the diseaseprogression.We believe that this guideline,which is the first comprehensive European guideline on MMA managementusing GRADE methods will assist clinicians to choose the most effective management strategy for MMA.KeywordsMoyamoya angiopathy,diagnosis,therapy,stroke,guidelines,systematic reviewDate received:29 July 2022;accepted:16 November 2022Cerebrovascular Unit,Fondazione IRCCS Istituto Neurologico Carlo Besta,Milan,ItalyMoyamoya Center,University Children's Hospital Zurich,Switzerland3Moyamoya Center for adults,Department of Neurosurgery.University Tubingen,GermanyDepartment of Neurology and Stroke Center,Hospital La Paz Institute for Health Research-ldiPAZ (La Paz University Hospital-UniversidadAutonoma de Madrid).Madrid,SpainSINSERM UI14I,Hospital Robert Debre,Paris,FranceDepartment of Neurosurgery,Cha