White matter relapsing remitting disease: “Susac’s syndrome”—An underdiagnosed entity

SCHOTTLAENDER, LUCIA V.; CORREALE, JORGE; AMERISO, SEBASTIAN F.; MOSCHINI, JAVIER A.; CRIVELLI, LUCIA; MELI, FRANCISCO; FIOL, MARCELA P.

Multiple Sclerosis and Related Disorders

Elsevier

Año: 2013 vol. 2 p. 349 - 354

2211-0348

Susac´s syndrome is a treatable microangiopathy of unknown etiology affecting arterioles of the brain, retina, and cochlea. The typical clinical manifestation is the triad of encephalopathy, visual loss, and sensorineural hearing loss. One or more of these features may not be present at onset and therefore Susac´s syndrome´s diagnosis may be difficult. We describe the clinical presentation, diagnostic tests, and treatment of three cases diagnosed and treated at our institution.