Wyburn-Mason syndrome

The Wyburn-Mason syndrome (WMS), also known as Bonnet-Dechaume-Blanc syndrome, is a rare phacomatosis with ipsilateral retinal, cerebral, and, less frequently, facial angiomas. WMS presents a high risk of subarachnoid hemorrhage (SAH). We discuss the case of a 22-year-old, mentally normal woman with right-sided WMS and unexpected ipsilateral hemiparesis following recurrent SAH. Magnetic resonance studies revealed angiomas in the right orbit, in the posterior cranial fossa, close to right hippocampal formation, and above the basal cistern, supplied by right internal and external carotid arteries. The mechanism of ipsilateral pyramidal deficit in our patient originating from the unaffected hemisphere remains unclear.