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1、 1.cerebral proliferative angiopathy 2.moyamoya 3.hemorrhagic angiopathy cerebral proliferative angiopathy moyamoya cerebral proliferative angiopathy moyamoya if a patient with suspect cpa presents with hemorrhage consider hemorrhagic angiopathy cerebral proliferative angiopathy moyamoya altered int
2、ernal elastic lamina usually subcortical high incidence of bleeds arterial stenoses lack of dominant feedersfast capillary transit intermingled normal brain parenchyma 3 frontal angiographic views show arterial proliferation without a-v shunting & filling of multiple moderate dilated veins. initial
3、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. cerebral proliferative angiopathy: angiography initial lateral angiogram (left) shows cpa, center shows re
4、vascularization obtained via dural branches of the middle meningeal artery after burr holes, follow-up angiogram (right) shows diminished cpa. cerebral proliferative angiopathy: angiography angiography demonstrates nl sized arterial feeders with a pseudo tumoral blush & no venous shunting. early art
5、erial phase (left) & late arterial phase (right) demonstrates nl size arterial feeders & slightly early draining veins. narrowing of ica, m1, a1 narrowing of ica with “puff-of-smoke”, diminished cortical flow. obliteration of ica, disappearance of puff-of-smoke, further reduction of cortical flow. m
6、r t2wis & lateral angiogram show focal cpa in the right frontal lobe. 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. mri studies (different pts) show multiple
7、 flow voids on t1wi (left), flair (center) & after gdt administration (right). note intermingled normal brain in all pts. mr t1wis (left, center) &t2wi show cpa in left hemisphere including basal ganglia. mtt, rcbf & rcbv are increased due to capillary & venous ectasia. in classic brain avms mtt is
8、decreased due to rapid shunting. cbv cbf mtt t2 image shows diffuse cpa & gliosis, source mra image confirms presence of vessels & gd perfusion rcbf map shows high perfusion. lasjaunias p. et al. cerebral proliferative angiopathy, clinical and angiographic description of an entity different from cer
9、ebral avms. stroke. 2008 mar: 1-8. 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 th
10、e disease. 2 pts presenting with intraparenchymal hemorrhages. (left) t1wi non- contrast, (middle) flair, (right) t2wi. flow voids in basal ganglia leptomeningeal enhancement (leptomeningeal blood vessel engorgement: ivy sign). different patients: flair shows watershed chronic infarcts (far left) &
11、acute parietal infarct (ctr left). t2wi shows left intraventricular acute hemorrhage (ctr right). t2* shows right temporal acute bleed (far right). different patients: mra shows stenosis of both mcas & large perforators (left). center shows stenosis of left mca. mr perfusion (right) shows low rcbf i
12、n deep regions of both hemispheres. cerebral proliferative angiopathy moyamoya targeted embolization increase cortical blood supply: synangiogenesis or calvarial burr holes increase cortical blood supply by recruiting additional dural arteries antiplatelet tx calcium channel blockers surgery: synang
13、iogenesis or calvarial burr holes bypass eca to ischemic zone is feasible pre & 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. pre treatment post treatmentpre treatmentpost treatment synangiogenesis or calvarial burr holes) lpaolo tortori-donati, andrea rossi, c. raybaud. pediatric neuroradiology: brain, head , neck, a
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