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Susac syndrome is a rare disorder caused by autoimmune-mediated occlusions of microvessels in the brain, retina and inner ear. These occlusions lead to a characteristic clinical triad of central nervous system dysfunction, visual disturbances and vestibule-cochlear deficits.
The diagnosis is based on clinical manifestations and complementary studies, which demonstrate the involvement of three systems. There are different treatments that include various immunosuppressive drugs combinations such as corticosteroids, intravenous immunoglobulin, mycophenolate mofetil, among others. We present the case of a year-old woman with left hearing loss, tinnitus and episodes of recurrent vertigo, four weeks after bilateral blurred vision, cerebellar ataxia and encephalopathy.
Magnetic resonance imaging of the brain showed multiple rounded hyperintense lesions in t2 and fluid-attenuated inversion recovery FLAIR , hypointense in t1, at the middle level of the corpus callosum, internal capsule, cerebellum and right middle cerebellar peduncle. The audiometry evidenced bilateral perceptual hearing loss, predominantly in the left ear. Angiography by optical coherence tomography showed obstruction in the deep layer retina arteries.
It is important to know the clinical triad characteristic and the complementary studies necessary to arrive at the diagnosis, since immunosuppressive treatment can often be delayed.
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Marrodan M, et al. Mult Scler Relat Disord. Epub May 3. PMID: Long-term outcome in Susac syndrome. Aubart-Cohen F, et al. Medicine Baltimore. Do not forget Susac syndrome in patients with unexplained acute confusion. Star M, et al. J Stroke Cerebrovasc Dis. Epub Feb A review and update on the ophthalmic implications of Susac syndrome. Heng LZ, et al. Surv Ophthalmol. Epub Jan PMID: Review. Susac syndrome: a report of four cases and a review of the literature.
Neth J Med. Show more similar articles See all similar articles. Publication types Case Reports Actions. Computed Tomography Angiography Actions. Diagnosis, Differential Actions.
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[Susac Syndrome, a Rare Cause of Encephalopathy]