Cerebral proliferative angiopathy

Cerebral proliferative angiopathy (CPA), previously known as diffuse nidus type AVM, is a cerebral vascular malformation separated from classic brain AVM and characterised by the presence of normal brain parenchyma interspersed throughout the tangle of vessels that corresponds to the nidus ^1,2.

Epidemiology

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 brain AVMs ^1,3.

Clinical presentation

Seizures, headaches and neurological symptoms related to cerebral haemorrhage ¹. 

Pathology

• content pending

Radiographic features

Cerebral angiography (DSA) continues to be the gold standard for CPA diagnosis, specially due the dynamic flow evaluation, 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 persisted into the late arterial and early venous phase
• 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 haemorrhage or severe symptoms ³. 

Differential  Diagnosis

Meningothelial hyperplasia (MH).