Cerebral proliferative angiopathy.

Neurology and Neurosurgery Depar tment of Hospital Municipal Dr. Mário Gatti, Campinas SP, Brasil: Resident of Neurosurgery of Hospital Municipal Dr. Mário Gatti, Campinas SP, Brasil; Resident of Neuropediatry of Hospital das Clínicas, Universidade de São Paulo, São Paulo SP, Brazil; Neurosurgeon of Instituto de Ciencias Neurologicas (ICNE), Hospital Beneficência Portuguesa, São Paulo SP, Brazil and Hospital Municipal Dr. Mario Gatti, Campinas SP, Brasil; Neuroradiologist of Med Imagem, Hospital Beneficência Portuguesa, São Paulo SP, Brazil. Since Cushing and Dandy reported their cases about “monstrous” vascular malformations with high mortality index in 1928, neurosurgery has been dedicating efforts to study such complex pathology among the most important ones in the modern medicine. Cerebral vascular malformation is a relatively rare pathology and its prevalence is difficult to estimate since a few number of affected individuals remain symptomatic. The classification contemplates size, local and venous draining territory. Those malformations are congenital results of an anomalous cerebral development. Physiopathology, angioarchitecture and natural history distinguish many types of malformations such as arteriovenous malformations (AVM), venous angiomas, cavernous malformations, dural arteriovenous fistulas and capillary telangiectasy. The AVMs are dilated tortuous vessel masses characterized by direct connections between arteries and veins (shunts) without a capillary net interposition. A peculiar type of large brain AVM with multiple non dominant arterial feeders to a lobar or hemispheric nidus, relatively small draining veins associated to intermingled normal brain and angiographic evidence of capillary angioectasia and angiogenetic activity determines an atypical entity named cerebral proliferative angiopathy (CPA). Rare vascular pathologies associated with skin and other organ lesions designate neurocutaneous angiomatosis syndrome. Sturge Weber syndrome (SWS) and Rendu-Osler-Weber syndrome (ROWS) are examples of it . We report a female with an atypical diffuse lobar cerebral proliferative angiopathy (CPA).