Susac Syndrome: Expanding the Triad (.pdf)

Susac Syndrome presents with the clinical triad of retinal artery occlusion, sensorineural hearing loss, and encephalopathy and the neuroimaging triad of white matter lesions with callosal involvement, deep grey lesions, and leptomeningeal disease. Since the initial descriptions of both triads, increasing awareness has been made of further clinical manifestations in the retina of Gass plaques caused by local arterial wall damage and fluorescein leakage that occurs in normal appearing retinal arterioles far distant from involved vessels. Many patients appear psychotic when encephalopathic and have skin changes resembling livedo. When bowel and bladder involvement is present in fulminant presentations, cervical cord or cauda equina involvement should be searched for.