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(Stroke. 2008;39:878.)
© 2008 American Heart Association, Inc.

Original Contributions

Cerebral Proliferative Angiopathy

Clinical and Angiographic Description of an Entity Different From Cerebral AVMs

Pierre L. Lasjaunias, MD, PhD; Pierre Landrieu, MD, PhD; Georges Rodesch, MD, PhD; Hortensia Alvarez, MD; Augustin Ozanne, MD; Staffan Holmin, MD, PhD; Wen-Yuan Zhao, MD; Sasikhan Geibprasert, MD; Dennis Ducreux, MD Timo Krings, MD, PhD

From the Service de Neuroradiologie Diagnostique et Thérapeutique (P.L.L., G.R., H.A., A.O., S.H., W.Z., S.G., D.D., T.K.), Hôpital de Bicetre, Le Kremlin-Bicetre, Paris, France; the Service Neuropediatrie (P.L.), Hôpital de Bicetre, Le Kremlin-Bicetre, Paris, France; the Department of Clinical Neuroscience (S.H.), Section for Neuroradiology, Karolinska Institute, Stockholm, Sweden; and the Department of Neuroradiology (T.K.), University Hospital of the Technical University, Aachen, Germany.

Correspondence to Prof Dr. P.L. Lasjaunias, Service de Neuroradiologie Diagnostique et Thérapeutique, Hôpital de Bicêtre, 78, rue du Général Leclerc, 94275 Le Kremlin-Bicêtre, France. E-mail pierre.lasjaunias{at}bct.ap-hop-paris.fr

Background and Purpose— The purpose of this article is to describe "cerebral proliferative angiopathy" (CPA) as a clinical entity, which may be regarded as separate from "classical" brain AVMs in angioarchitecture, natural history, clinical presentation, and, therefore, treatment and which can be discerned from other cerebral AVMs by characteristic imaging features.

Methods— In a prospectively entered databank encompassing 1434 patients with brain AVMs, a subgroup of 49 patients harboring specific angiographic characteristics were identified. Their charts and imaging films were retrospectively reviewed.

Results— We found a preponderance of CPA in young (mean age: 22) females (67%). Clinical symptoms were seizures, disabling headaches, and stroke-like symptoms; hemorrhagic presentations were exceptional. On cross-sectional imaging, CPA demonstrated as a diffuse network of densely enhancing vascular spaces with intermingled normal brain parenchyma. The discrepancy between the large size of the nidus and the small shunting volume, the absence of flow-related aneurysms, the presence of diffuse angiogenesis (eg, transdural supply, progressive arterial occlusion), and the small calibre of a multitude of feeding arteries and draining veins were the angiographic hallmarks of this disease.

Conclusion— The diffuse angiogenetic activity is presumably related to reduced perinidal perfusion and subsequent chronic cortical ischemia. Natural history demonstrates a low risk of hemorrhage. CPA may be regarded as a separate clinical entity different to "classical" cerebral AVMs, because normal brain is interspersed with the abnormal vascular channels increasing the risk of neurological deficit in aggressive treatments, which in the light of the natural history does not seem to be indicated.

Key Words: arteriovenous malformation • ischemia • angiogenesis • embolization • angiopathy

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