Susac Syndrome: Description of a Single-Centre Case Series

Sara Beça, Claudia Elera-Fitzcarrald, Albert Saiz, Sara Llufriu, Maria C. Cid, Bernardo Sanchez-Dalmau, Alfredo Adan, Gerard Espinosa

Resultado de la investigación: Contribución a una revistaArtículorevisión exhaustiva

Resumen

This study describes the clinical characteristics, diagnostic results, treatment regimens, and clinical course of a cohort of patients with Susac syndrome (SS). It is a retrospective observational study of all patients with the diagnosis of SS evaluated at the Hospital Clinic (Barcelona, Spain) between March 2006 and November 2020. Nine patients were diagnosed with SS. The median time from the onset of the symptoms to diagnosis was five months (IQR 9.0), and the median follow-up time was 44 months (IQR 63.5). There was no clear predominance of sex, and mean age of symptoms onset was 36 years (range 19–59). Six patients (67%) presented with incomplete classical clinical triad, but this eventually developed in six patients during the disease course. Encephalopathy, focal neurological signs, visual disturbances, and hearing loss were the most frequent manifestations. Brain magnetic resonance imaging showed callosal lesions in all patients. Most were in remission within two years. Only four patients met the proposed criteria for definite SS. When SS is suspected, a detailed diagnostic workup should be performed and repeated over time to identify the clinical manifestations that will lead to a definite diagnosis.

Idioma originalInglés
Número de artículo6549
PublicaciónJournal of Clinical Medicine
Volumen11
N.º21
DOI
EstadoPublicada - nov. 2022
Publicado de forma externa

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