First Author: N.Munoz sanz SPAIN
Co Author(s): 0 0 0 0 0 0 0 0 0
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To report a single case of a rare autoimmune mediated disease, the Susac Syndrome.
Retinal Department, Ophthalmology Service, Fundación Jímenez Díaz, Madrid, Spain.
A case report
37 years old male patient, previously healthy, who presented with complaints of headache, memory loss, vertigo and one episode of hearing loss in the last 6 months. MRI shows multiple small focal lesions in infra and supratentorial white matter and corpus callosum. He also complained of some unspecified visual symptoms. Ophthalmic examination: visual acuity was 20/50 ae 20/30 in his right eye (amblyopic) and 20/20 in his left eye, with normal intraocular pressure and anterior segment. Fundus examination revealed some terminal artery occlusion in the posterior pole and also occlusion of small peripheral arterioles (ghost vessels) with white wall vessel plaques. On fluorescein angiography: small peripheral areas of peripheral branch artery occlusion and wall hyperflourescence bilaterally, without ischemia. For the last month the patient has remained asymptomatic, so no treatment has been initiated.
Susac syndrome is a rare multisystemic autoimmune disease that affects mainly young female adults, with a rate female/male ratio of 3:1. The pathogenesis of the microvessel occlusions seems to be mediated by autoimmune response, with unknown antigen so far. The classic triad of encephalopathy, branch retinal artery occlusions and hearing loss is characteristic. MRI helps to the differential diagnosis with others inflammatory diseases such as multiple sclerosis. Due to its rarity, treatment is empiric and should be individualized, but high-dose corticosteroids and immunosuppressive agents are the most used.