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The current clinical picture of cerebral proliferative angiopathy: systematic review

  • Review Article - Vascular Neurosurgery - Other
  • Published:
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Abstract

Background

Cerebral proliferative angiopathy (CPA) is a rare subset of arteriovenous malformations (AVM). It has unique clinical presentation, angiographic characteristics, and pathophysiology which often brings challenges for the treatment. We aimed to define its epidemiology, pathophysiology are unknown, and best management strategies.

Methods

A systematic review was conducted according to the PRISMA guidelines. MEDLINE was searched for articles regarding CPA. Extracted data included epidemiological, clinical, and angiographical characteristics, treatment, and outcomes. Treatment was classified as conservative, radiosurgery, endovascular, decompression, and indirect vascularization. A meta-analytical approach was employed for description of the data as study-size adjusted percentages or weighted means, as appropriate.

Results

Thirty-three studies were analyzed, rendering a total 95 cases—half of which came from a single study. Patients were predominantly young (mean 23 years old) and female (60.0%) presenting with headaches (44.9%), seizures (37.1%), or transient ischemic attacks (33.7%). Hemorrhage was present in 18.0%, but rebleeding rates were as high as 67%. The majority of nidus were > 6 cm (52.5%) with hemispheric extension (73.0%). Capillary angioectatic appearance (85.7%), transdural supply (62.5%), and deep venous drainage (73.0%) were also frequent features. Most patients were treated conservatively (54.4%), followed by endovascular (34.2%). Indirect vascularization and radiosurgery were attempted in five and two patients, respectively. Mean follow-up was 110.8 patient-years. Neurological status improved in 50.7%, remained stable in 40.2%, and worsened in 9.0%.

Conclusions

Conservative and endovascular treatments seem adequate interventions, despite limited evidence. Complementary techniques can be used in patients throughout disease history, according to symptom-based, individualized approach. More studies are required for choosing interventions based on reliable long-term results.

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Authors and Affiliations

  1. Department of Neurological Surgery, School of Medicine, Universidade de Sao Paulo, Rua Dr Eneas de Carvalho Aguiar, 255 – Pinheiros, São Paulo, SP, Brazil

    Vitor Nagai Yamaki, Davi Jorge Fontoura Solla, João Paulo Mota Telles, Saul Almeida da Silva, Manoel Jacobsen Teixeira, Eric Homero Albuquerque Paschoal & Eberval Gadelha Figueiredo

  2. Department of Neurosugery, Hospital Ophir Loyola, Belem, PA, Brazil

    Glaucia Lexy Jong Liem & Eric Homero Albuquerque Paschoal

  3. Department of Radiology, School of Medicine, Universidade de Sao Paulo, Sao Paulo, SP, Brazil

    José Guilherme Mendes Pereira Caldas

  4. Department of Neurosurgery, Universidade Federal do Para, Belem, PA, Brazil

    Eric Homero Albuquerque Paschoal

Authors
  1. Vitor Nagai Yamaki
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  2. Davi Jorge Fontoura Solla
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  3. João Paulo Mota Telles
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  4. Glaucia Lexy Jong Liem
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  5. Saul Almeida da Silva
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  6. José Guilherme Mendes Pereira Caldas
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  7. Manoel Jacobsen Teixeira
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  8. Eric Homero Albuquerque Paschoal
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  9. Eberval Gadelha Figueiredo
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Correspondence to João Paulo Mota Telles or Eric Homero Albuquerque Paschoal.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

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This article is part of the Topical Collection on Vascular Neurosurgery – Other

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Yamaki, V.N., Solla, D.J.F., Telles, J.P.M. et al. The current clinical picture of cerebral proliferative angiopathy: systematic review. Acta Neurochir 162, 1727–1733 (2020). https://doi.org/10.1007/s00701-020-04289-7

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  • DOI: https://doi.org/10.1007/s00701-020-04289-7

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