Diffuse proliferative cerebral
arterial disorders: similar
appearances, different diagnosis.
J. Barnwell, H. Alvarez, M. Castillo
Division of Neuroradiology
UNC, Chapel Hill
Diffuse Proliferative cerebral
arterial disorders.
Types.
1.
Cerebral Proliferative Angiopathy
2. Moyamoya
3. Hemorrhagic Angiopathy
Outline of Presentation
Epidemiology
 Clinical Presentations
 Pathology, differences between disorders
 Imaging findings: catheter angiography, CT &

MRI findings
Treatment
 Conclusions
 References

Epidemiology
Cerebral Proliferative
Angiopathy
Rare subset of AVM’s

3.4% of AVMs
Moyamoya
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:

Mean: 22 yrs, median: 17.5 yrs
 Range 10-65
Females, 2:1
Age at Dx:

2 peaks: 5 yrs & 40’s
Clinical Features
Cerebral Proliferative
Angiopathy
Moyamoya
Infarction: 50-75%
 TIA: 50-75%
 Seizures, headaches
 Hemorrhages
 Rare: choreiform, cognitive or
psychiatric changes

Seizure: 45%
 Headache: 41%
 Focal deficits: 16%
 Hemorrhages (12%):


33% single -- 67% recurrent
Prognosis: poor
Prognosis: variable
If a patient with suspect CPA presents with HEMORRHAGE
consider HEMORRHAGIC ANGIOPATHY
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
Pathology Features
Cerebral Proliferative
Angiopathy



Altered internal elastic
lamina & smooth
muscle cells
Collagenous thickening
of veins
Intermingled normal
neural tissue
Moyamoya

Smooth muscle
hyperplasia

Irregular elastic lamina

No inflamacion
CT Features
Cerebral Proliferative
Angiopathy

Areas of dense contrast
enhancement which may
be focal, lobar or
hemispheric
Moyamoya





Collateral deep perforators
& pial vessels (Ivy sign)
Cortical Infarcts
Calcium in old infarcts
Hemorrhage
Cerebellum always nl
Hemorrhage:
Consider Hemorrhagic Angiopathy
Hemorrhagic Angiopathy: CT
3 pts with Hemorrhagic Angiopathy show intraparenchymal bleeds.
Hemorrhages are much less common in CPA.
Angiography Features (1)
Cerebral Proliferative
Angiopathy







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
Moyamoya





Dilated perforating
arteries
Generally bilateral
Spares posterior
circulation arteries
Early stenosis of ICA,
M1 & A1
Aneurysms
Angiography Features (2)
Cerebral Proliferative
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
Hemorrhagic
Angiopathy








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
Cerebral Proliferative Angiopathy:
Angiography
Arterial stenoses
Lack of dominant feeders
Fast capillary transit
Intermingled normal brain parenchyma
Transdural blood
supply
Cerebral Proliferative Angiopathy:
Angiography
3 frontal angiographic views show arterial proliferation without A-V
shunting & filling of multiple moderate dilated veins.
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.
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.
Hemorrhagic Angiopathy:
Angiography
Angiography demonstrates nl sized arterial feeders with a pseudo tumoral
blush & no venous shunting.
Hemorrhagic Angiopathy:
Angiography
Early arterial phase (left) & late arterial phase (right) demonstrates nl size arterial
feeders & slightly early draining veins.
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.
Cerebral Proliferative Angiopathy :
MR & Angiography
MR T2WIs & lateral angiogram show focal CPA in the right frontal lobe.
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.
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.
Cerebral Proliferative Angiopathy: MR
MR T1WIs (left, center) &T2WI show CPA in left hemisphere
including basal ganglia.
Cerebral Proliferative Angiopathy: MR
Perfusion
CBV
CBF
MTT
MTT, rCBF & rCBV are increased due to capillary & venous ectasia. In
classic brain AVMs MTT is decreased due to rapid shunting.
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.
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.
Hemorrhagic Angiopathy: MR
2 pts presenting with intraparenchymal hemorrhages. (Left) T1WI noncontrast, (Middle) FLAIR, (Right) T2WI.
Moyamoya: MR
Flow Voids in basal ganglia
Leptomeningeal enhancement
(leptomeningeal blood vessel
engorgement: Ivy sign).
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).
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.
Treatment
Cerebral Proliferative
Angiopathy


Targeted embolization
Increase cortical blood
supply:

Moyamoya


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
Hemorrhagic Angiopathy:
Response to Radiation therapy
Pre Treatment
Post Treatment
Pre Treatment
Post 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.
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.

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.
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.