Diffuse proliferative cerebral arterial disorders: similar appearances, different diagnosis.

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 Clinical Presentations Pathology, differences between disorders Imaging findings: catheter angiography, CT & MRI findings Treatment Conclusions References

4. Epidemiology

5. Clinical Features 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% associated with congenital cardiac anomalies, renal-artery stenosis, giant cervicofacial hemangiomas, hyperthyroidism Genetic component: 10% of Japanese & 6% of US pts have a 1st degree relative Associated w/abnormalities in chromosomes 3,6,8, & 17 None of these associations are seen with CPA

7. Pathology Features

8. CT Features • Hemorrhage: • Consider Hemorrhagic Angiopathy

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

10. Angiography Features (1)

11. Angiography Features (2)

12. Cerebral Proliferative Angiopathy: Angiography Arterial stenoses Lack of dominant feeders Fast capillary transit

13. Transdural blood supply Intermingled normal brain parenchyma

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

15. Cerebral 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.

16. Cerebral 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.

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

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

19. Moyamoya: angiography, different stages 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.

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

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 CBV CBF MTT, rCBF & rCBV are increased due to capillary & venous ectasia. In classic brain AVMs MTT is decreased due to rapid shunting.

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 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. 2008 Mar: 1-8.

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

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

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

32. Hemorrhagic Angiopathy: Response to Radiation therapy Post Treatment Pre Treatment Post Treatment Pre Treatment 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.

33. Conclusions Both 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.

34. Conclusions 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. Hemorrhagic angiopathy has a rapid response to the radiotherapy.

35. References Scott R. et al. Moyamoya Disease and Moyamoya Syndrome. NEJM 2009;360:1226-37. 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. 2008 Mar: 1-8. Paolo Tortori-Donati, Andrea Rossi, C. Raybaud. Pediatric Neuroradiology: Brain, Head , Neck, and Spine. Springer Berlin Heidelberg New York. 2005. 291-297. Lasjaunias P, Ter Brugge K.G., Berenstein A. Surgical Neuroangiography. Volume 3: Clinical and Interventioal Aspects in Children. Springer. 2006: 35-39.