Encefalomielitis aguda diseminada. Leucoencefalitis aguda hemorrágica. Otras enfermedades desmielinizantes

Delgado, Francisco; Monteiro, G. Carvalho; Margarit, B. Povedano; Izquierdo, A. Yusta

doi:10.1016/j.med.2019.05.012

Cited by 1 publication

(1 citation statement)

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“…Susac Syndrome (SS) is considered an orphan autoimmune disease that affects the microvasculature of the brain, the retina and the ear [1] [2], secondary to CD8 T-cell mediated inflammatory endotheliopathy. It compromises the main blood-brain barrier functions due to leukocyte and other blood flow restrictions as a result of brain endothelial inflammation and microvascular occlusion [3] [4].…”

Section: Introductionmentioning

confidence: 99%

Comparative Analysis of Post-Rehabilitation Neuropsychological Profile of a Patient with Susac Syndrome—A Case Report

Zúñiga-Márquez¹,

Gutiérrez-Ávila²,

Quintero-Cusguen³

et al. 2021

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Susac Syndrome (SS) is an autoimmune disease characterized by the clinical triad of encephalopathy, hearing loss and retinal arterial occlusions, with prevalent structural changes identified on brain magnetic resonance imaging (white matter, corpus callosum, basal ganglia region and the thalamic region extending to the midbrain) in the majority of cases, which lead to cognitive manifestations of which there is a paucity of descriptions in the literature. The objective of this case study is to compare to post-rehabilitation neurocognitive profile of a 29-year-old woman with SS presenting with compromised intellectual and motor skills and cognitive functions, together with neuropsychiatric symptoms. Better performance was found in the neuropsychological assessment, with changes in the structural cerebral network evidenced on Diffusion Tensor Imaging (DTI) performed following the therapeutic and pharmacological intervention.

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Section: Introductionmentioning

confidence: 99%