title: Long-term clinical and radiological evolution in one case of Susac's syndrome.
abstract: Susac's syndrome is a rare idiopathic microangiopathy affecting the precapillary arterioles of the brain, retina and cochlea leading to the clinical triad of encephalopathy, retinopathy and hearing loss. The objective of this study is to describe a new case of Susac's syndrome reactivated after a 12-year period with a good response to immunosuppressive therapy. The patient was a 32-year-old woman, complaining of diplopia, right blurred vision, progressive gait disturbance, tinnitus, attention deficit, and slight memory loss. The patient was diagnosed as having Susac's syndrome and discharged with steroid therapy. After a 12-year period of clinical stability she had a relapse. Immunosuppressive therapy resulted in significant clinical and radiological improvement. Early clinical identification of Susac's syndrome is crucial for the initiation of immunosuppressive therapy and differential diagnosis. In our case, the combined use of corticosteroids and azathioprine was key in the relapse management.
authors.affiliation: Department of Neurology, Girona Biomedical Research Institute (IDIBGI), Dr. Josep Trueta University Hospital, Avda. França s/n, 17007, Girona, Spain.
authors.forename: René
authors.initials: R
authors.lastname: Robles-Cedeño
authors.forename: Lluís
authors.initials: L
authors.lastname: Ramió-Torrentà
authors.forename: Gemma
authors.initials: G
authors.lastname: Laguillo
authors.forename: Jordi
authors.initials: J
authors.lastname: Gich
authors.forename: Mar
authors.initials: M
authors.lastname: Castellanos
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000639:trends*; D011859:Radiography; D055955:Susac Syndrome / Q000000981:diagnostic imaging* / Q000628:therapy*; D013997:Time Factors; D016896:Treatment Outcome