Características clínicas del síndrome de Susac

Luisa Paz Sendín, Raúl González Torres, Tania Arrieta Hernández, Angel Miquel Santos Martinez

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Resumen

Introducción: El síndrome de Susac es una microangiopatía autoinmune caracterizado por encefalopatía, oclusión de las ramas de la arteria retiniana y perdida de la audición.
Objetivo: Recopilar casos, las teorías establecidas y obtener el conocimiento actualizado sobre el síndrome de Susac, su diagnóstico y tratamiento.
Métodos: Se revisaron entre 2017 y 2024 sobre encefalopatía, enfermedades de la retina, enfermedades vasculares, pérdida auditiva neurosensorial y síndrome de Susac, en idioma inglés y español; se incluyeron revisiones sistemáticas, reportes de caso y metaanálisis, a partir de las bases de datos Scopus, SciELO, PubMed, Cochrane y Lilacs.
Desarrollo: Hasta el año 2021 se reportan 450 casos de síndrome de Susac, con un estimado de 0,148 casos por 100 000 habitantes y predominio femenino. La cefalea y la encefalopatía son las manifestaciones iniciales más frecuentes. La evaluación oftalmológica es importante porque contribuye al diagnóstico, la resonancia magnética muestra en la parte central del cuerpo calloso, lesiones hiperintensas en T2, multifocales, redondas como bolas de nieve. Sus principales diagnósticos diferenciales son migraña y esclerosis múltiple.
Conclusiones: El inicio del síndrome de Susac, con encefalopatía, variabilidad de presentación y amplio diagnóstico diferencial, hace más compleja su identificación. La etiopatogenia no está totalmente dilucidada. La presencia de hiperfluorescencia en las arterias retinianas y las lesiones hiperintensas del cuerpo calloso lo confirman, aun sin la presencia de la triada imagenológica o la clínica. El diagnóstico temprano y el tratamiento adecuado favorecen el pronóstico.

Palabras clave

encefalopatía; enfermedades de la retina; enfermedades vasculares; pérdida auditiva neurosensorial; síndrome de Susac.

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