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1、MoyamoyaDiseaseContentDefinitionEtiologyPathophysiologyClinicalManifestationDiagnosisTreatmentPrognosisDefinitionMoyamoyaDisease isadiseaseinwhichcertainarteriesinthebrainareconstricted. Bloodflowisblockedbytheconstriction,andalsobybloodclots(thrombosis).A collateralcirculation developsaroundtheblo
2、ckedvesselstocompensatefortheblockage,butthecollateralvesselsaresmall,weak,andpronetohemorrhage, aneurysm andthrombosis. OnconventionalX-rayangiography,thesecollateralvesselshavetheappearanceofa"puffofsmoke"(describedas"もやもや(moyamoya)"inJapanese)EtiologyAbout10%ofcasesofmoyamoyadiseasearefamilial,a
3、ndsomecasesresultfromspecificgeneticmutations.InJapantheoverallincidenceishigher(0.35per100,000).Moyamoyadiseasecanbeeithercongenitaloracquired. Patientswith Downsyndrome, sicklecellanemia, neurofibromatosistype1, congenitalheartdisease,fibromusculardysplasia,activated proteinC resistance,or headtr
4、auma candevelopmoyamoyamalformations. Itismorecommoninwomenthaninmen,althoughaboutathirdofthoseaffectedaremale.PathophysiologyThediseasemoyamoyamakesthebloodleakoutofthearteries,causingpressuretothebrainandsubsequentheadaches.Thepathogenesisofmoyamoyadiseaseisunknown,althoughthegene ringfingerprote
5、in213 (RNF213)hasbeenimplicated.Onceitbegins,thevascularocclusiontendstocontinuedespiteanyknownmedicalmanagement. Insomepeoplethisleadsto transientischemicattacks orrepeatedstrokeswithseverefunctionalimpairmentorevendeath. Inothers,theblockagemaynotcauseanysymptoms.Thediseasecausesconstrictionsprim
6、arilyinthe internalcarotidartery,andoftenextendstothemiddleandanteriorcerebralarteries,branchesoftheinternalcarotidarteryinsidetheskull. Whentheinternalcarotidarterybecomescompletelyblocked,thefinecollateralcirculationthatitsuppliesisobliterated. Patientsoftensurviveonthecollateralcirculationfromth
7、eback(posterior)ofthecircleofWillis,arisingfromthe basilarartery.ClinicalManifestationTheclinicalfeaturesare strokes,recurrent transientischemicattacks (TIAs),sensorimotorparalysis(numbnessandparalysisofthe
