Susac syndrome: the first case report in Peru
Carolina Rivadeneira-Sotelo1, María Meza Vega2, Darwin Segura-Chávez3
1Centro de investigación básica en demencias y enfermedades desmielinizantes del sistema nervioso central, Instituto Nacional de Ciencias Neurológicas, Lima, Perú. Address: Jirón Ancash 1271, Barrios Altos, Distrito de Lima, Lima, Perú.
Medwave
|September 9, 2017
Summary
Susac Syndrome, a rare condition affecting women, involves brain, vision, and hearing issues. Early diagnosis and treatment are crucial to prevent permanent damage.
Area of Science:
- Neurology
- Ophthalmology
- Otorhinolaryngology
Background:
- Susac Syndrome is a rare autoimmune disease.
- It is characterized by encephalopathy, retinal artery occlusion, and hearing loss.
- Onset typically occurs between ages 9-58, predominantly in women.
Observation:
- A 31-year-old woman presented with headache, behavioral changes, and somnolence.
- She developed hearing loss and retinal artery occlusion.
- Brain MRI revealed characteristic lesions in the corpus callosum, periventricular region, and cerebellum.
Findings:
- The case presented the classic triad of Susac Syndrome: encephalopathy, retinal artery occlusion, and sensorineural hearing loss.
- This marks the first reported case in Peru with the complete triad.
- The patient's symptoms and imaging findings were consistent with Susac Syndrome.
Implications:
- Timely diagnosis and treatment are essential to prevent irreversible consequences.
- Even incomplete forms of Susac Syndrome require prompt evaluation.
- This case highlights the importance of recognizing Susac Syndrome in diverse geographical regions.


