Restrictive cerebral cortical venopathy: A new clinicopathological entity.

We present a case of a novel restrictive cerebral venopathy in a child, consisting of a bilateral network of small to medium cortical veins without evidence of arteriovenous shunting, absence of the deep venous system, venous ischemia, elevated intracranial pressure, and intracranial calcifications. The condition is unlike other diseases characterized by networks of small veins, including cerebral proliferative angiopathy, Sturge-Weber syndrome, or developmental venous anomaly. While this case may be the result of an anatomic variation leading to the congenital absence of or early occlusion of the deep venous system, the insidious nature over many years argues against this. The absence of large cortical veins suggests a congenital abnormality of the venous structure. The child's presentation with a seizure-like event followed by protracted hemiparesis is consistent with venous ischemia. We propose that this is likely to represent a new clinicopathological entity.