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Fulminant Susac syndrome—a rare cause of coma: The history of the fatal course in a young man

Susac syndrome is a rare microangiopathy of indeterminate etiology, presumably autoimmune, characterized by a triad of encephalopathy, sensorineural hearing loss, and branch retinal artery occlusions occurring predominantly in women. The onset and progression patterns are multiple, mainly of three m...

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Detalles Bibliográficos
Autores principales: Andour, Hajar, Rostoum, Soufiane, Regragui, Yassine, Fikri, Meriem, Jiddane, Mohamed, Touarsa, Firdaous
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9899952/
https://www.ncbi.nlm.nih.gov/pubmed/36756223
http://dx.doi.org/10.1177/2050313X221149826
Descripción
Sumario:Susac syndrome is a rare microangiopathy of indeterminate etiology, presumably autoimmune, characterized by a triad of encephalopathy, sensorineural hearing loss, and branch retinal artery occlusions occurring predominantly in women. The onset and progression patterns are multiple, mainly of three modes. Fulminant evolution is exceptional, rarely reported across literature. We report through this case a Susac syndrome in a young man in whom evolution was fatal. Magnetic resonance imaging is essential to raise the diagnosis and for follow-up, with almost pathognomonic findings, all the more useful as the clinical triad is usually incomplete and as the encephalopathy is the most limiting of the symptoms.