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Neuroimaging and ophthalmologic findings in incomplete Susac syndrome

Neuroimagem e achados oftalmológicos na síndrome de Susac incompleta

A 19-year-old woman presented with sudden headache, dysarthria, paresthesia in upper limbs, memory and visual impairment.

Magnetic resonance imaging (MRI) revealed several round lesions in the supra- and infratentorial structures with hyperintensity in fluid-attenuated inversion recovery (FLAIR) and contrast enhancement, predominantly affecting the basal ganglia and central portions of the corpus callosum (Figure 1). Fluorescein angiography revealed multiple retinal branch artery occlusions in both eyes (Figure 2). Audiometry, somatosensory and visual evoked potentials were normal.

Figure 1.
Brain magnetic resonance image (MRI) showing multiple bilateral round lesions with hyperintensity in fluid-attenuated inversion recovery (FLAIR) (a, c) and punctate contrast enhancement affecting the basal ganglia and the cerebellum on axial views (b, d) and typical “snowball” lesions in the central fibers of the corpus callosum on sagittal view (e). Lesions showed no abnormality on diffusion-weighted images (not shown).

Figure 2.
Fundus photograph showing normal optic disk, macula and retinal vascularization in both eyes (a). Fluorescein angiography demonstrating arterial occlusion in retinal equator of both eyes (b).

Susac syndrome is a rare autoimmune endotheliopathy characterized by a clinical triad of encephalopathy, visual disturbance and hearing loss, but complete clinical presentation is unusual11. Monteiro ML, Swanson RA, Coppeto JR, Cuneo RA, DeArmond SJ, Prusiner SB. A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions. Neurology. 1985 Aug;35(8):1113-21. https://doi.org/10.1212/wnl.35.8.1113
https://doi.org/https://doi.org/10.1212/...
,22. Vishnevskia-Dai V, Chapman J, Sheinfeld R, Sharon T, Huna-Baron R, Manor RS, Shoenfeld Y, et al. Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis. Medicine (Baltimore). 2016 Oct;95(43):e5223. https://doi.org/10.1097/md.0000000000005223
https://doi.org/https://doi.org/10.1097/...
. Incomplete Susac syndrome is defined when only two criteria are present22. Vishnevskia-Dai V, Chapman J, Sheinfeld R, Sharon T, Huna-Baron R, Manor RS, Shoenfeld Y, et al. Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis. Medicine (Baltimore). 2016 Oct;95(43):e5223. https://doi.org/10.1097/md.0000000000005223
https://doi.org/https://doi.org/10.1097/...
.

References

  • 1
    Monteiro ML, Swanson RA, Coppeto JR, Cuneo RA, DeArmond SJ, Prusiner SB. A microangiopathic syndrome of encephalopathy, hearing loss, and retinal arteriolar occlusions. Neurology. 1985 Aug;35(8):1113-21. https://doi.org/10.1212/wnl.35.8.1113
    » https://doi.org/https://doi.org/10.1212/wnl.35.8.1113
  • 2
    Vishnevskia-Dai V, Chapman J, Sheinfeld R, Sharon T, Huna-Baron R, Manor RS, Shoenfeld Y, et al. Susac syndrome: clinical characteristics, clinical classification, and long-term prognosis. Medicine (Baltimore). 2016 Oct;95(43):e5223. https://doi.org/10.1097/md.0000000000005223
    » https://doi.org/https://doi.org/10.1097/md.0000000000005223

Publication Dates

  • Publication in this collection
    30 Nov 2020
  • Date of issue
    Dec 2020

History

  • Reviewed
    07 June 2020
  • Received
    15 June 2020
  • Accepted
    22 June 2020
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