Diffuse proliferative cerebral arterial disorders similar.ppt

diffuseproliferativecerebralarterialdisorders similarappearances differentdiagnosis j barnwell h alvarez m castillodivisionofneuroradiologyunc chapelhill diffuseproliferativecerebralarterialdisorders types cerebralproliferativeangiopathymoyamoyahemorrhagicangiopathy outlineofpresentation epidemiologyclinicalpresentationspathology differencesbetweendisordersimagingfindings catheterangiography ct mrifindingstreatmentconclusionsreferences epidemiology clinicalfeatures ifapatientwithsuspectcpapresentswithhemorrhageconsiderhemorrhagicangiopathy morefactsaboutmoyamoya 10 20 associatedwithsicklecelldisease nf 1 downsyndrome previouscranialirradiation 10 associatedwithcongenitalcardiacanomalies renal arterystenosis giantcervicofacialhemangiomas hyperthyroidismgeneticcomponent 10 ofjapanese 6 ofusptshavea1stdegreerelativeassociatedw abnormalitiesinchromosomes3 6 8 17noneoftheseassociationsareseenwithcpa pathologyfeatures ctfeatures hemorrhage considerhemorrhagicangiopathy hemorrhagicangiopathy ct 3ptswithhemorrhagicangiopathyshowintraparenchymalbleeds hemorrhagesaremuchlesscommonincpa angiographyfeatures 1 angiographyfeatures 2 cerebralproliferativeangiopathy angiography arterialstenoses lackofdominantfeeders fastcapillarytransit interminglednormalbrainparenchyma transduralbloodsupply cerebralproliferativeangiopathy angiography 3frontalangiographicviewsshowarterialproliferationwithouta vshunting fillingofmultiplemoderatedilatedveins initiallateralangiogram left showscpa centershowsrevascularizationobtainedviaduralbranchesofthemiddlemeningealarteryafterburrholes follow upangiogram right showsdiminishedcpa cerebralproliferativeangiopathy angiography initiallateralangiogram left showscpa centershowsrevascularizationobtainedviaduralbranchesofthemiddlemeningealarteryafterburrholes follow upangiogram right showsdiminishedcpa cerebralproliferativeangiopathy angiography hemorrhagicangiopathy angiography angiographydemonstratesnlsizedarterialfeederswithapseudotumoralblush novenousshunting earlyarterialphase left latearterialphase right demonstratesnlsizearterialfeeders slightlyearlydrainingveins hemorrhagicangiopathy angiography moyamoya angiography differentstages narrowingofica m1 a1 narrowingoficawith puff of smoke diminishedcorticalflow obliterationofica disappearanceofpuff of smoke furtherreductionofcorticalflow mrt2wis lateralangiogramshowfocalcpaintherightfrontallobe cerebralproliferativeangiopathy mr angiography cerebralproliferativeangiopathy mr sourcemra left showsmultiplehypertrophiedarteries mrafrontalview center showsstenosisofleftmca cpa t2wi right showsabnormalbloodvessels gliosisinlefthemisphere cerebralproliferativeangiopathy mr mristudies differentpts showmultipleflowvoidsont1wi left flair center aftergdtadministration right noteinterminglednormalbraininallpts cerebralproliferativeangiopathy mr mrt1wis left center t2wishowcpainlefthemisphereincludingbasalganglia cerebralproliferativeangiopathy mrperfusion mtt rcbf rcbvareincreasedduetocapillary venousectasia inclassicbrainavmsmttisdecreasedduetorapidshunting cbv cbf mtt cerebralproliferativeangiopathy mrperfusion t2imageshowsdiffusecpa gliosis sourcemraimageconfirmspresenceofvessels gdperfusionrcbfmapshowshighperfusion cerebralproliferativeangiopathy mrperfusion lasjauniasp etal cerebralproliferativeangiopathy clinicalandangiographicdescriptionofanentitydifferentfromcerebralavms stroke 2008mar 1 8 t1wipostgd ttp rcbv rcbfmapsinan11 year oldgirlwithheadachesshowsleftfrontoparietalcpa mridemonstrateincreasecbv cvfindicatinghypervascularizationinlesion decreasedttpinnidusandsurroundingareassuggestingtheischemicnatureofthedisease hemorrhagicangiopathy mr 2ptspresentingwithintraparenchymalhemorrhages left t1winon contrast middle flair right t2wi moyamoya mr flowvoidsinbasalganglia leptomeningealenhancement leptomeningealbloodvesselengorgement ivysign moyamoya mr differentpatients flairshowswatershedchronicinfarcts farleft acuteparietalinfarct ctrleft t2wishowsleftintraventricularacutehemorrhage ctrright t2 showsrighttemporalacutebleed farright moyamoya vascularmr differentpatients mrashowsstenosisofbothmcas largeperforators left centershowsstenosisofleftmca mrperfusion right showslowrcbfindeepregionsofbothhemispheres treatment hemorrhagicangiopathy responsetoradiationtherapy pre postradiationtxangiographyperformedonhemorrhagicangiopathypts preimagesdemonstratepseudotumoralblushattimeofichwithrapidcapillarytransity posttximagesshowexcellentresponsetoirradiation pretreatment posttreatment pretreatment posttreatment conclusions bothcerebralproliferativeangiopathy moyamoyaarearterialproliferativeconditionsleadingtostenosesinproximalvessels bothareischemicarterialconditions proliferativeangiopathyandhemorrhagicangiopathyhavetobeconsideredasagroupofdisordersdifferentfromclassicalbrainavms conclusions treatmentofmoyamoyaaimstoanimprovementinarterialsupplybydirect bypass orindirect synangiogenesisorcalvarialburrholes revascularizationtechniques proliferativeangiopathypts canbecandidatesforarterialrevascularisationtreatments insomeinstancestheycanbenefitfromtargetedembolizations hemorrhagicangiopathyhasarapidresponsetotheradiotherapy references scottr etal moyamoyadiseaseandmoyamoyasyndrome nejm2009 360 1226 37 bacigaluppis dehdashtiar agidr kringst tymianskim mikulisdj neurosurgthecontributionofimagingindiagnosis preoperativeassessment andfollow upofmoyamoyadisease areview neurosurgfocus 2009 26 e3alasjauniasp etal cerebralproliferativeangiopathy clinicalandangiographicdescriptionofanentitydifferentfromcerebra