Cerebral proliferative angiopathy
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Cerebral proliferative angiopathy (CPA), previously known as diffuse nidus type AVM, is a cerebral vascular malformation separated from classic brain AVMarteriovenous malformation (AVM) and 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 AVMscerebral arteriovenous malformations 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 CPAcerebral proliferative angiopathy diagnosis, speciallyespecially due theto 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
persistedpersists into the late arterial and early venousphasephases - the nidus usually has a fuzzy appearance, it is not well
circumscribed-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 Diagnosisdiagnosis
-<p><strong>Cerebral proliferative angiopathy (CPA)</strong>, previously known as <strong>diffuse nidus type AVM</strong>, is a <a href="/articles/cerebral-vascular-malformations">cerebral vascular malformation</a> separated from <a href="/articles/cerebral-arteriovenous-malformation">classic brain AVM</a> and characterised by the presence of normal brain parenchyma interspersed throughout the tangle of vessels that corresponds to the nidus <sup>1,2</sup>.</p><h4>Epidemiology</h4><p>CPA is more common affecting women, in a ratio of 2:1 <sup>2 </sup>and is reported as a rare entity, corresponding to 3.4% of all brain AVMs <sup>1,3</sup>.</p><h4>Clinical presentation</h4><p>Seizures, headaches and neurological symptoms related to cerebral haemorrhage <sup>1</sup>. </p><h4>Pathology</h4><ul><li><em>content pending</em></li></ul><h4>Radiographic features</h4><p>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. </p><p>The characteristic features of cerebral proliferative angiopathy are <sup>1-3</sup>:</p><ul>- +<p><strong>Cerebral proliferative angiopathy (CPA)</strong>, previously known as <strong>diffuse nidus type AVM</strong>, is a <a href="/articles/cerebral-vascular-malformations">cerebral vascular malformation</a> separated from <a href="/articles/cerebral-arteriovenous-malformation">classic brain arteriovenous malformation (AVM)</a> and characterised by the presence of normal brain parenchyma interspersed throughout the tangle of vessels that corresponds to the nidus <sup>1,2</sup>.</p><h4>Epidemiology</h4><p>CPA is more common affecting women, in a ratio of 2:1 <sup>2 </sup>and is reported as a rare entity, corresponding to 3.4% of all cerebral arteriovenous malformations <sup>1,3</sup>.</p><h4>Clinical presentation</h4><p>Seizures, headaches and neurological symptoms related to cerebral haemorrhage <sup>1</sup>. </p><h4>Pathology</h4><ul><li><em>content pending</em></li></ul><h4>Radiographic features</h4><p><a title="Cerebral angiography" href="/articles/cerebral-angiography">Cerebral angiography (DSA)</a> 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. </p><p>The characteristic features of cerebral proliferative angiopathy are <sup>1-3</sup>:</p><ul>
-<li>feeder arteries tend to be of normal size or moderately enlarged </li>- +<li>feeder arteries tend to be of normal size or moderately enlarged</li>
-<li>classical nidus appearance with scattered “puddling” of contrast which persisted into the late arterial and early venous phase</li>-<li>the nidus usually has a fuzzy appearance, it is not well circumscribed</li>-</ul><h4>Treatment and prognosis</h4><p>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 <sup>3</sup>. </p><h4>Differential Diagnosis</h4><p><a href="/articles/meningothelial-hyperplasia">Meningothelial hyperplasia (MH).</a></p><p> </p><p> </p>- +<li>classical nidus appearance with scattered “puddling” of contrast which persists into the late arterial and early venous phases</li>
- +<li>the nidus usually has a fuzzy appearance, it is not well-circumscribed</li>
- +</ul><h4>Treatment and prognosis</h4><p>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 <sup>3</sup>. </p><h4>Differential diagnosis</h4><ul><li><a href="/articles/meningothelial-hyperplasia">meningothelial hyperplasia (MH)</a></li></ul>
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