Cerebral proliferative angiopathy with papilledema

• CPA is a rare vascular malformation with distinct angiographic features.
• Papilledema in CPA has been never reported in the literature so far.
• Our case highlights the need of proper clinical evaluation of the patient.

A young female presented with intermittent blurring of vision and mild to moderate headache for three months. Fundus examination revealed bilateral papilledema with secondary optic atrophy (right more than left). Computed tomography scan of brain showed a diffuse intraparenchymal vascular malformation in right parietooccipital region. Cerebral digital subtraction angiography (DSA) revealed diffuse nidus in right parietooccipital area, supplied by multiple branches of distal middle cerebral artery and anterior cerebral artery. Scattered-puddling appearance of contrast was noted in diffuse nidus and there were no definite arterial feeders. DSA was consistent with the diagnosis of cerebral proliferative angiopathy (CPA). The patient was started on acetazolamide 250 mg twice a day. At six months follow up, she was asymptomatic with resolving papilledema. CPA is a rare vascular malformation, any case report that gives further insight to it will be very useful to the scientific community. Herein, we describe an unusual association of CPA with papilledema.