Cerebral proliferative angiopathy: a review / Angiopatia proliferativa cerebral: uma revisão

Abstract

Introduction: Cerebral Arteriovenous Malformations (AVMs) are vascular lesions defined by arteriovenous shunting flowing through a nidus of coiled and tortuous vascular connections. Cerebral Proliferative Angiopathy (CPA) is a rare type of AVM with pathological, clinical, diagnostic features and treatment differences from a typical AVM. We aimed to summarize important features of CPA already described and identify important gaps of evidence. Methods: Data was selected by a search of PUBMED with the term "cerebral proliferative angiopathy". The articles considered relevant were included in this review. We also screened article references and included them as needed. Discussion: CPA shows a diffuse nidus appearance, presence of transdural supply and stenosis of feeding arteries, without dominant feeders or flow-related aneurysms. There is intermingled brain between the vascular spaces. The usual clinical presentation is seizures, headaches, and progressive neurological deficits. Sound diagnosis can be made based on CT, MRI, Angiography and perfusion techniques, which are essential to treatment choices. Invasive treatment is seldom indicated, in order to preserve the normal brain parenchyma, and clinical control of symptoms, associated with a thorough follow-up, is the usual approach in most reported cases. Conclusion: Still much remains to be learned about CPA. Establishing more precise roles of various radiological assessing methods, how they affect the follow-up of patients, and safety and efficacy profiles of different treatment approaches will provide us the tools to give patients a more solid intervention and clearer follow-up.

Clinico-radiological features of cerebral proliferative angiopathy: A large case series from India

“…3,15 Positron emission computed tomography (PET) and single photon emission computed tomography (SPECT) studies showed the presence of compromised perfusion in the nidus as well as the surrounding areas. 13,14 In our study, one patient died; however, autopsy could not be done. The pathological specimen showed abnormal arteries and veins with altered lamination of the internal elastic lamina and muscle fibres on the arterial site and collageneous thickening of the veins with presence of normal appearing neural tissue intermingled between these vascular channels.…”

“…[4][5][6][7][8][9][10][11][12][13] Liu et al 13 had described 5 cases of CPA from China. A detailed review of case reports and case series with CPA was published by Nunes et al 14 The term 'proliferative' angiopathy, used to describe this rare AVM, is appropriate as there is a strong evidence of new blood vessels formation, which is uncommon in classical AVMs. These new blood vessels supply the nidus as well as the functional brain parenchyma by transdural supply.…”