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CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2020; 78(12): 817
DOI: 10.1590/0004-282X20200106
IMAGES IN NEUROLOGY

Neuroimaging and ophthalmologic findings in incomplete Susac syndrome

Neuroimagem e achados oftalmológicos na síndrome de Susac incompleta
Renata Cristina MÜLLER 
1   Universidade Federal do Paraná, Hospital de Clínicas, Unidade de Radiologia, Curitiba PR, Brazil.
,
Kenzo HOKAZONO 
2   Universidade Federal do Paraná, Hospital de Clínicas, Unidade de Oftalmologia, Curitiba PR, Brazil.
,
Bernardo Corrêa de Almeida TEIXEIRA 
1   Universidade Federal do Paraná, Hospital de Clínicas, Unidade de Radiologia, Curitiba PR, Brazil.
,
Rosana Hermínia SCOLA 
3   Universidade Federal do Paraná, Hospital de Clínicas, Unidade de Neurologia, Curitiba PR, Brazil.
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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.

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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).
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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 unusual[1],[2]. Incomplete Susac syndrome is defined when only two criteria are present[2].

Author’s Contribution:

The authors declare that they have each made important contributions to the acquisition, analysis, and interpretation of the data. All authors have critically revised the manuscript and have given their approval for the final version to be published.




Publication History

Received: 15 June 2020

Accepted: 22 June 2020

Article published online:
07 June 2023

© 2020. Academia Brasileira de Neurologia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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  • 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
  • 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.20200106202001065223