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Diffuse proliferative cerebral arterial disorders: similar appearances, different diagnosis. J. Barnwell, H. Alvarez, M. Castillo Division of Neuroradiology.

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Presentation on theme: "Diffuse proliferative cerebral arterial disorders: similar appearances, different diagnosis. J. Barnwell, H. Alvarez, M. Castillo Division of Neuroradiology."— Presentation transcript:

1 Diffuse proliferative cerebral arterial disorders: similar appearances, different diagnosis. J. Barnwell, H. Alvarez, M. Castillo Division of Neuroradiology UNC, Chapel Hill

2 Diffuse Proliferative cerebral arterial disorders. Types Cerebral Proliferative Angiopathy Moyamoya Hemorrhagic Angiopathy

3 Outline of Presentation Epidemiology Epidemiology Clinical Presentations Clinical Presentations Pathology, differences between disorders Pathology, differences between disorders Imaging findings: catheter angiography, CT & MRI findings Imaging findings: catheter angiography, CT & MRI findings Treatment Treatment Conclusions Conclusions References References

4 Epidemiology Cerebral Proliferative Angiopathy Moyamoya Rare subset of AVM’s  3.4% of AVMs Females, 2:1 Age at Dx:  Mean: 22 yrs, median: 17.5 yrs  Range Rare Condition  Japan, 3:100,000  Europe, 3:1,000,000  US, 8.6:10,000,000 Asian Americans > African Americans > Caucasians > Hispanics Females, 2:1 Age at Dx:  2 peaks: 5 yrs & 40’s

5 Clinical Features Cerebral Proliferative Angiopathy Moyamoya  Seizure: 45%  Headache: 41%  Focal deficits: 16%  Hemorrhages (12%):  33% single -- 67% recurrent Prognosis: poor  Infarction: 50-75%  TIA: 50-75%  Seizures, headaches  Hemorrhages  Rare: choreiform, cognitive or psychiatric changes Prognosis: variable If a patient with suspect CPA presents with HEMORRHAGE consider HEMORRHAGIC ANGIOPATHY

6 More facts about Moyamoya 10-20% associated with sickle cell disease, NF-1, Down Syndrome, previous cranial irradiation 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, hyperthyroidism <10% associated with congenital cardiac anomalies, renal-artery stenosis, giant cervicofacial hemangiomas, hyperthyroidism Genetic component: Genetic component: 10% of Japanese & 6% of US pts have a 1 st degree relative 10% of Japanese & 6% of US pts have a 1 st degree relative Associated w/abnormalities in chromosomes 3,6,8, & 17 Associated w/abnormalities in chromosomes 3,6,8, & 17 None of these associations are seen with CPA None of these associations are seen with CPA

7 Pathology Features Cerebral Proliferative Angiopathy Moyamoya  Altered internal elastic lamina & smooth muscle cells  Collagenous thickening of veins  Intermingled normal neural tissue  Smooth muscle hyperplasia  Irregular elastic lamina  No inflamacion

8 CT Features Cerebral Proliferative Angiopathy Moyamoya  Areas of dense contrast enhancement which may be focal, lobar or hemispheric  Collateral deep perforators & pial vessels (Ivy sign)  Cortical Infarcts  Calcium in old infarcts  Hemorrhage  Cerebellum always nl Hemorrhage: Consider Hemorrhagic Angiopathy

9 HemorrhagicAngiopathy:CT Hemorrhagic Angiopathy: CT 3 pts with Hemorrhagic Angiopathy show intraparenchymal bleeds. Hemorrhages are much less common in CPA.

10 Angiography Features (1) Cerebral Proliferative Angiopathy Moyamoya  Intermingled nl brain parenchyma  No dominant feeders  Fast capillary transit  Transdural blood supply  Late stenosis (ICA, M1-2, A1-2): 39%  Aneurysms (12%)  Mildly enlarged draining veins  Dilated perforating arteries  Generally bilateral  Spares posterior circulation arteries  Early stenosis of ICA, M1 & A1  Aneurysms

11 Angiography Features (2) Cerebral Proliferative Angiopathy Hemorrhagic Angiopathy  Intermingled nl brain parenchyma  No dominant feeders  Fast capillary transit  Transdural blood supply  Late stenosis  Aneurysms (12%)  Blush may be focal, lobar or hemispheric  Low incidence of bleeds  Intermingled nl brain parenchyma  No dominant feeders  Fast capillary transit  No transdural blood supply  No stenoses  No aneurysms  Small pseudo-tumoral blush; usually subcortical  High incidence of bleeds

12 Cerebral Proliferative Angiopathy: Angiography Arterial stenosesLack of dominant feedersFast capillary transit

13 Intermingled normal brain parenchyma Transdural blood supply

14 Cerebral Proliferative Angiopathy: Angiography 3 frontal angiographic views show arterial proliferation without A-V shunting & filling of multiple moderate dilated veins.

15 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. Cerebral Proliferative Angiopathy: Angiography

16 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. Cerebral Proliferative Angiopathy: Angiography

17 Hemorrhagic Angiopathy: Angiography Angiography demonstrates nl sized arterial feeders with a pseudo tumoral blush & no venous shunting.

18 Early arterial phase (left) & late arterial phase (right) demonstrates nl size arterial feeders & slightly early draining veins. Hemorrhagic Angiopathy: Angiography

19 Moyamoya: 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.

20 MR T2WIs & lateral angiogram show focal CPA in the right frontal lobe. Cerebral Proliferative Angiopathy : MR & Angiography

21 Cerebral 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.

22 Cerebral 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.

23 Cerebral Proliferative Angiopathy: MR MR T1WIs (left, center) &T2WI show CPA in left hemisphere including basal ganglia.

24 Cerebral Proliferative Angiopathy: MR Perfusion MTT, rCBF & rCBV are increased due to capillary & venous ectasia. In classic brain AVMs MTT is decreased due to rapid shunting. CBV CBF MTT

25 Cerebral Proliferative Angiopathy: MR Perfusion T2 image shows diffuse CPA & gliosis, source MRA image confirms presence of vessels & Gd perfusion rCBF map shows high perfusion.

26 Cerebral Proliferative Angiopathy: MR Perfusion Lasjaunias P. et al. Cerebral proliferative angiopathy, clinical and angiographic description of an entity different from cerebral AVMs. Stroke 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 the disease.

27 HemorrhagicAngiopathy: MR Hemorrhagic Angiopathy: MR 2 pts presenting with intraparenchymal hemorrhages. (Left) T1WI non- contrast, (Middle) FLAIR, (Right) T2WI.

28 Moyamoya: MR Flow Voids in basal ganglia Leptomeningeal enhancement (leptomeningeal blood vessel engorgement: Ivy sign).

29 Moyamoya: MR Different patients: FLAIR shows watershed chronic infarcts (far left) & acute parietal infarct (ctr left). T2WI shows left intraventricular acute hemorrhage (ctr right). T2* shows right temporal acute bleed (far right).

30 Moyamoya: Vascular MR Different 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.

31 Treatment 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:  Synangiogenesis or calvarial burr holes  Bypass ECA to ischemic zone is feasible

32 Hemorrhagic Angiopathy: Response to Radiation therapy 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

33 Conclusions Both cerebral proliferative angiopathy & Moyamoya are arterial proliferative conditions leading to stenoses in proximal vessels. Both cerebral proliferative angiopathy & Moyamoya are arterial proliferative conditions leading to stenoses in proximal vessels. Both are ischemic arterial conditions. Both are ischemic arterial conditions. Proliferative angiopathy and hemorrhagic angiopathy have to be considered as a group of disorders different from classical brain AVMs. Proliferative angiopathy and hemorrhagic angiopathy have to be considered as a group of disorders different from classical brain AVMs.

34 Conclusions Treatment of Moyamoya aims to an improvement in arterial supply by direct (bypass) or indirect ( revascularization techniques. Treatment 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. 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. Hemorrhagic angiopathy has a rapid response to the radiotherapy.

35 References Scott R. et al. Moyamoya Disease and Moyamoya Syndrome. NEJM 2009;360: Scott 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:E3a 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:E3a Lasjaunias P. et al. Cerebral Proliferative Angiopathy, Clinical and Angiographic Description of an Entity Different From Cerebral AVMs. Stroke Mar: 1-8. Lasjaunias 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 York Lasjaunias P, Ter Brugge K.G., Berenstein A. Surgical Neuroangiography. Volume 3: Clinical and Interventioal Aspects in Children. Springer. 2006:


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