Teaching NeuroImages: CRAO and silent brain infarcts caused by cardiac myxomas in Carney complex

A 14-year-old girl presented with sudden painless left eye vision loss. Fundus photography showed left central retinal artery occlusion (CRAO) (figure, A). Brain MRI revealed subclinical embolic infarcts (figure, B). Chest CT confirmed multicentric cardiac myxomas (figure, C). Gene testing revealed point mutation in the PRKAR1A gene (figure, D). Carney complex (CNC) is a rare autosomal dominant, multiple neoplasia syndrome with cardio-cutaneous manifestations. Multiple cardiac myxomas are unusual and strongly related to CNC. Cerebral emboli occur in 34% of patients with cardiac myxomas.1 The PRKAR1A gene is located on 17q22-24 and mutated in about 40% of patients with CNC.2