3Outline of Presentation EpidemiologyClinical PresentationsPathology, differences between disordersImaging findings: catheter angiography, CT & MRI findingsTreatmentConclusionsReferences
4Cerebral Proliferative Angiopathy EpidemiologyCerebral Proliferative AngiopathyMoyamoyaRare subset of AVM’s3.4% of AVMsFemales, 2:1Age at Dx:Mean: 22 yrs, median: 17.5 yrsRange 10-65Rare ConditionJapan, 3:100,000Europe, 3:1,000,000US, 8.6:10,000,000Asian Americans > African Americans > Caucasians > Hispanics2 peaks: 5 yrs & 40’sAsian Americans to Caucasians: 4.6African Americans to Caucasians: 2.2Hispanics to Caucasians: 0.5
5Clinical Features Cerebral Proliferative Angiopathy Moyamoya Seizure: 45%Headache: 41%Focal deficits: 16%Hemorrhages (12%):33% single -- 67% recurrentPrognosis: poorInfarction: 50-75%TIA: 50-75%Seizures, headachesHemorrhagesRare: choreiform, cognitive or psychiatric changesPrognosis: variable33% single -- 67% recurrent vs. 4% in typical AVMIf a patient with suspect CPA presents with HEMORRHAGEconsider HEMORRHAGIC ANGIOPATHY
6More facts about Moyamoya 10-20% associated with sickle cell disease, NF-1, Down Syndrome, previous cranial irradiation<10% associated with congenital cardiac anomalies, renal-artery stenosis, giant cervicofacial hemangiomas, hyperthyroidismGenetic component:10% of Japanese & 6% of US pts have a 1st degree relativeAssociated w/abnormalities in chromosomes 3,6,8, & 17None of these associations are seen with CPA
7Cerebral Proliferative Angiopathy Pathology FeaturesCerebral Proliferative AngiopathyMoyamoyaAltered internal elastic lamina & smooth muscle cellsCollagenous thickening of veinsIntermingled normal neural tissueSmooth muscle hyperplasiaIrregular elastic laminaNo inflamacionAltered internal elastic laminareduplicated, interrupted, and/or distortedAltered smooth muscle cellshyperplastic or thinned leading to aneurysmsCollagenous thickening of veinsincreased type IV collagen in the subendotheliumIntermingled normal neural tissue
8CT Features Cerebral Proliferative Angiopathy Moyamoya Hemorrhage: Areas of dense contrast enhancement which may be focal, lobar or hemisphericCollateral deep perforators & pial vessels (Ivy sign)Cortical InfarctsCalcium in old infarctsHemorrhageCerebellum always nlHemorrhage:Consider Hemorrhagic Angiopathy
9Hemorrhagic Angiopathy: CT 5 yoIntracerbral subcortical arteriolesMay rehemorrhage3 pts with Hemorrhagic Angiopathy show intraparenchymal bleeds. Hemorrhages are much less common in CPA.
13Transdural blood supply Intermingled normal brain parenchyma
14Cerebral Proliferative Angiopathy: Angiography 3 frontal angiographic views show arterial proliferation without A-V shunting & filling of multiple moderate dilated veins.
15Cerebral Proliferative Angiopathy: Angiography Initial lateral angiogram (left) shows CPA, center shows revascularization obtained via dural branches of the middle meningeal artery after burr holes, follow-up angiogram (right) shows diminished CPA.
16Cerebral Proliferative Angiopathy: Angiography Initial lateral angiogram (left) shows CPA, center shows revascularization obtained via dural branches of the middle meningeal artery after burr holes, follow-up angiogram (right) shows diminished CPA.
17Hemorrhagic Angiopathy: Angiography ImagingSmall pseudo-tumoral blush, usually in the subcortical white matterNormal sized arterial feedersNormal draining veins or a lack of venous shuntingIntermingled with normal brainRespond well to radiotherapyPaolo Tortori-Donati, Andrea Rossi, C. Raybaud. Pediatric neuroradiology: brain, head , neck, and spineAngiography demonstrates nl sized arterial feeders with a pseudo tumoral blush & no venous shunting.
18Hemorrhagic Angiopathy: Angiography ImagingSmall scattered nidus like lesions in the subcortical/white matter areasArterial feeders are normal in sizeDrains into normal veinsIntermingled with normal brainRespond well to radiotherapyEarly arterial phase (left) & late arterial phase (right) demonstrates nl size arterial feeders & slightly early draining veins.
19Moyamoya: angiography, different stages Narrowing of ICA, M1, A1Narrowing of ICA with“Puff-of-Smoke”,diminished cortical flow.Obliteration of ICA,disappearance of Puff-of-Smoke,further reduction of cortical flow.
20Cerebral Proliferative Angiopathy : MR & Angiography MR T2WIs & lateral angiogram show focal CPA in the right frontal lobe.
21Cerebral Proliferative Angiopathy: MR Source MRA (left) shows multiple hypertrophied arteries, MRA frontal view (center) shows stenosis of left MCA & CPA, T2WI (right) shows abnormal blood vessels & gliosis in left hemisphere.
22Cerebral Proliferative Angiopathy: MR MRI studies (different pts) show multiple flow voids on T1WI (left), FLAIR (center) & after Gdt administration (right). Note intermingled normal brain in all pts.
23Cerebral Proliferative Angiopathy: MR MR T1WIs (left, center) &T2WI show CPA in left hemisphere including basal ganglia.
24Cerebral Proliferative Angiopathy: MR Perfusion CBVCBFMTTMTT, rCBF & rCBV are increased due to capillary & venous ectasia. In classic brain AVMs MTT is decreased due to rapid shunting.
26Cerebral Proliferative Angiopathy: MR Perfusion MRI (T1-weighted postcontrast, time to peak [TTP]), cerebral blood volume (CBV), and cerebral blood flow (CBF) map in a11-year-old girl with headaches demonstrating a large left frontoparietal nidus with brain parenchyma intermingled between the vascularspaces. Perfusion-weighted MRI demonstrates an increase in cerebral blood volume and flow indicating hypervascularization withinthe nidus and decreased times to peak in the area surrounding the nidus testifying for the ischemic nature of the disease.T1WI post Gd, TTP, rCBV & rCBF maps in an 11-year-old girl with headaches shows left frontoparietal CPA. MRI demonstrate increase CBV & CVF indicating hypervascularization in lesion & decreased TTP in nidus and surrounding areas suggesting the ischemic nature of the disease.Lasjaunias P. et al. Cerebral proliferative angiopathy, clinical and angiographic description of an entity different from cerebral AVMs. Stroke Mar: 1-8.
30Moyamoya: Vascular MRDifferent patients: MRA shows stenosis of both MCAs & large perforators (left). Center shows stenosis of left MCA. MR perfusion (right) shows low rCBF in deep regions of both hemispheres.
31Cerebral Proliferative Angiopathy TreatmentCerebral Proliferative AngiopathyMoyamoyaTargeted embolizationIncrease cortical blood supply:Synangiogenesis or calvarial burr holes increase cortical blood supply by recruiting additional dural arteriesAntiplatelet TxCalcium channel blockersSurgery:Synangiogenesis or calvarial burr holesBypass ECA to ischemic zone is feasibleCPAEmbolization: partial and targetedSurgery: non-targetedEmbolization and radiation:Only use if intractable seizures and/or headachesIncrease cortical blood supply:Calvarial burr holes increase cortical blood supply by recruiting additional dural arteriesMoyamoyaAntiplatelet: to prevent emboliCCB (relieve intractable headaches/migraines, also to reduce frequency and severity of TIAs)Surgery:Spares ECA, so bypass ECA to ischemic zone is feasible
32Hemorrhagic Angiopathy: Response to Radiation therapy Unlike proliferative angiopathy, hemorrhagic angiopathy tends to present with recurrent intracerebellar hemorrhages as shown in this adult patient and there is no gender predilection. An angiogram shows an almost tumoral blush without enlarged arterial pedicles or venous drainage. This disease responds favorably to radiation therapy as shown in the angiogram to your right.This slide shows hemorrhage in the right occipital lobe of a 14 year old girl. In the center, an angiogram shows the typical appearance of proliferative angiopathy without dilated arteries or veins. . On the right, the control angiogram after radiation shows disappearance of the lesion.Pre TreatmentPost TreatmentPre TreatmentPost TreatmentPre & Post radiation Tx angiography performed on hemorrhagic angiopathy pts. Pre images demonstrate pseudo tumoral blush at time of ICH with rapid capillary transity. Post Tx images show excellent response to irradiation.
33ConclusionsBoth cerebral proliferative angiopathy & Moyamoya are arterial proliferative conditions leading to stenoses in proximal vessels.Both are ischemic arterial conditions.Proliferative angiopathy and hemorrhagic angiopathy have to be considered as a group of disorders different from classical brain AVMs.
34ConclusionsTreatment of Moyamoya aims to an improvement in arterial supply by direct (bypass) or indirect (synangiogenesis or calvarial burr holes) revascularization techniques.Proliferative angiopathy pts. can be candidates for arterial revascularisation treatments. In some instances they can benefit from targeted embolizations.Hemorrhagic angiopathy has a rapid response to the radiotherapy.
35ReferencesScott R. et al. Moyamoya Disease and Moyamoya Syndrome. NEJM 2009;360:Bacigaluppi S, Dehdashti AR, Agid R, Krings T, Tymianski M, Mikulis DJ.Neurosurg The contribution of imaging in diagnosis, preoperative assessment, and follow-up of moyamoya disease: a review. Neurosurg Focus. 2009; 26:E3aLasjaunias P. et al. Cerebral Proliferative Angiopathy, Clinical and Angiographic Description of an Entity Different From Cerebral AVMs. Stroke Mar: 1-8.Paolo Tortori-Donati, Andrea Rossi, C. Raybaud. Pediatric Neuroradiology: Brain, Head , Neck, and Spine. Springer Berlin Heidelberg New YorkLasjaunias P, Ter Brugge K.G., Berenstein A. Surgical Neuroangiography. Volume 3: Clinical and Interventioal Aspects in Children. Springer. 2006:Zanation 68852