Cerebral proliferative angiopathy (CPA), previously known as diffuse nidus type AVM, is a cerebral vascular malformation separated from classic brain arteriovenous malformation (AVM) and characterized by the presence of normal brain parenchyma interspersed throughout the tangle of vessels that corresponds to the nidus 1,2.
CPA is more common affecting women, in a ratio of 2:1 2 and is reported as a rare entity, corresponding to 3.4% of all cerebral arteriovenous malformations 1,3.
Seizures, headaches and neurological symptoms related to cerebral hemorrhage 1.
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Cerebral angiography (DSA) continues to be the gold standard for cerebral proliferative angiopathy diagnosis, especially due to its dynamic flow evaluation capability, however, CTA and MRA can also be accurate in making the diagnosis of the other cerebral vascular malformations.
The characteristic features of cerebral proliferative angiopathy are 1-3:
- the absence of early venous drainage, which helps to differentiate CPA from a classical cerebral AVM
- large areas of parenchymal involvement, often an entire lobe or even a hemisphere is affected
- the nidus is fed by multiple arteries (absence of a dominant feeder)
- feeder arteries tend to be of normal size or moderately enlarged
- associated stenosis of feeder arteries is often present
- classical nidus appearance with scattered “puddling” of contrast which persists into the late arterial and early venous phases
- the nidus usually has a fuzzy appearance, it is not well-circumscribed
Treatment and prognosis
The treatment for cerebral proliferative angiopathy carries the risk of damage to the normal brain tissue intermingled in the nidus, and thus it is usually limited to those patients presenting with hemorrhage or severe symptoms 3.
- 1. Lasjaunias PL, Landrieu P, Rodesch G et-al. Cerebral proliferative angiopathy: clinical and angiographic description of an entity different from cerebral AVMs. Stroke. 2008;39 (3): 878-85. doi:10.1161/STROKEAHA.107.493080 - Pubmed citation
- 2. Geibprasert S, Pongpech S, Jiarakongmun P et-al. Radiologic assessment of brain arteriovenous malformations: what clinicians need to know. Radiographics. 30 (2): 483-501. doi:10.1148/rg.302095728 - Pubmed citation
- 3. Maekawa H, Tanaka M, Hadeishi H. Fatal hemorrhage in cerebral proliferative angiopathy. Interv Neuroradiol. 2012;18 (3): 309-13. Free text at pubmed - Pubmed citation