Cerebral Proliferative Angiopathy : Clinical and Angiographic Description of an Entity Different From Cerebral AVMs

• Published in: Stroke (1970) Vol. 39; no. 3; pp. 878 - 885
• Main Authors: LASJAUNIAS, Pierre L, LANDRIEU, Pierre, RODESCH, Georges, ALVAREZ, Hortensia, OZANNE, Augustin, HOLMIN, Staffan, ZHAO, Wen-Yuan, GEIBPRASERT, Sasikhan, DUCREUX, Dennis, KRINGS, Timo
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 01.03.2008
• Subjects: Adolescent; Adult; Aged; Biological and medical sciences; Central Nervous System Diseases - etiology; Cerebral Angiography; Child; Databases, Factual; Female; Headache - etiology; Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy; Humans; Intracranial Arteriovenous Malformations - classification; Intracranial Arteriovenous Malformations - complications; Intracranial Arteriovenous Malformations - diagnosis; Intracranial Arteriovenous Malformations - therapy; Magnetic Resonance Imaging; Male; Medical sciences; Medicin och hälsovetenskap; Middle Aged; Neovascularization, Pathologic - etiology; Nervous system (semeiology, syndromes); Neurology; Retrospective Studies; Tomography, X-Ray Computed; Vascular diseases and vascular malformations of the nervous system; Stroke; Nervous system diseases; Angiography; Cardiovascular disease; Congenital disease; Cerebral disorder; Encephalon; Vascular disease; angiopathy; Angiogenesis; Ischemia; Central nervous system disease; Embolization; Arteriovenous malformation; Cerebrovascular disease
• ISSN: 0039-2499; 1524-4628; 1524-4628
• DOI: 10.1161/STROKEAHA.107.493080

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. 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. 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. 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.