Cerebral proliferative angiopathy

Proliferative
Angiopathy in a pediatric patient. T2-weighted image of the Brain showing multiple dilated tortuous signal void vascular structures affecting the right parieto-occipital region
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Proliferative
Angiopathy in a pediatric patient. T1-weighted post-contrast image of the brain showing the tubular signal void vascular structures being surrounded by bright signal and exerting mass effect on the surrounding parenchyma
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Proliferative
Angiopathy in a pediatric patient. Non-contrast CT of the brain showing subtle abnormality involving the right hemisphere with sulcal effacement and compression of the lateral ventricle
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Cerebral
proliferative angiopathy: A rare clinical entity with peculiar angiographic features: Magnetic resonance imaging brain axial, coronal and sagittal T2-weighted images showing multiple flow voids in left frontal parasagittal region with multiple dilated veins draining into superior sagittal sinus suggestive of arteriovenous malformation
www.ncbi.nlm.nih.gov • CC-by-nc-sa-3.0
Cerebral
proliferative angiopathy: A rare clinical entity with peculiar angiographic features: Cerebral digital subtraction angiography left internal carotid artery injection (a) showing diffuse proliferative angiogenesis with feeders supplied from branches of left anterior cerebral artery and left middle cerebral artery. (b) The venous drainage is through deep venous system and superficial venous system into superior sagittal sinus. (c) Left external carotid artery injection shows no drainage
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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 .

Epidemiology

CPA is more common affecting women, in a ratio of 2:1 and is reported as a rare entity, corresponding to 3.4% of all cerebral arteriovenous malformations .

Clinical presentation

Seizures, headaches and neurological symptoms related to cerebral hemorrhage .

Pathology

  • content pending

Radiographic features

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 :

  • 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 .

Differential diagnosis

Siehe auch:
Assoziationen und Differentialdiagnosen zu zerebrale proliferative Angiopathie:
zerebrale
arteriovenöse Malformation
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