Susac syndrome
Pronunciation: SOO-sak
Autoimmune microangiopathy affecting brain, retina, and inner ear, causing the classic triad of encephalopathy, branch retinal artery occlusions, and sensorineural hearing loss.
Full Definition
Susac syndrome is a rare autoimmune microangiopathy that affects small arteries in the brain, retina, and inner ear (cochlea). The condition is characterized by a classic triad of symptoms: subacute encephalopathy with cognitive dysfunction, branch retinal artery occlusions leading to visual field defects, and sensorineural hearing loss. The syndrome predominantly affects young women and can be mistaken for multiple sclerosis. Diagnosis requires integration of neurological, ophthalmological, and audiological findings, often supported by characteristic MRI findings including corpus callosum lesions.
Usage
Usage note: Named after Dr. John Susac; always capitalize the surname when used as an eponym.
In Context
- "The patient's combination of cognitive decline, visual field defects, and hearing loss raised suspicion for Susac syndrome." — Clinical assessment
- "Ophthalmological examination revealed branch retinal artery occlusions consistent with Susac syndrome." — Specialist consultation