Susac syndrome is a rare autoimmune endotheliopathy caused by a microangiopathic
occlusive arteriolar disease11 . Susac JO, Hardman JM, Selhorst JB. Microangipathy of the brain and
retina. Neurology. 1979;29(3):13-6.
http://dx.doi.org/10.1212/wnl.29.3.313
https://doi.org/10.1212/wnl.29.3.313...
characterized by the triad of encephalopathy, visual loss and sensorineural hearing
loss22 . Saw VP, Canty PA, Green CM, Briggs RJ, Cremer PD, Harrisber B et
al. Susac syndrome: microangiopathy of the retina, cochlea and brain. Clin Exp
Ophtalmol. 2000;28(5):373-81.
http://dx.doi.org/10.1046/j.1442-9071.2000.00345.x
https://doi.org/10.1046/j.1442-9071.2000...
.
A 41-year-old man presented with migraine-like headache, transient visual loss and tinnitus. Brain MRI showed small scattered FLAIR-hyperintense lesions involving the corpus callosum, centrum semiovale and periventricular white matter bilaterally (Figures 1 and 2). Some of them presented restricted diffusion (Figure 3). CSF analysis revealed 6.0 cells/mm33 . Do TH, Fisch C, Evoy F. Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroadiol. 2004;25(3):382-8., a lymphomononuclear pattern, and elevated protein levels with an increase in gamma fraction (18.35%). Angiofluorescein study showed retinian vasculitis (Figure 4). He was treated with immunoglobulin and methylprednisolone pulse33 . Do TH, Fisch C, Evoy F. Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroadiol. 2004;25(3):382-8., presenting good results.
Axial diffusion-weighted image demonstrates restricted diffusion in some of the lesions, characterized by hyperintensity in this sequence.
Sagittal FLAIR image discloses a small hyperintense lesion in the genu of the corpus callosum (arrow).
References
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1Susac JO, Hardman JM, Selhorst JB. Microangipathy of the brain and retina. Neurology. 1979;29(3):13-6. http://dx.doi.org/10.1212/wnl.29.3.313
» https://doi.org/10.1212/wnl.29.3.313 -
2Saw VP, Canty PA, Green CM, Briggs RJ, Cremer PD, Harrisber B et al. Susac syndrome: microangiopathy of the retina, cochlea and brain. Clin Exp Ophtalmol. 2000;28(5):373-81. http://dx.doi.org/10.1046/j.1442-9071.2000.00345.x
» https://doi.org/10.1046/j.1442-9071.2000.00345.x -
3Do TH, Fisch C, Evoy F. Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroadiol. 2004;25(3):382-8.
Publication Dates
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Publication in this collection
Oct 2014
History
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Received
27 May 2014 -
Reviewed
12 June 2014 -
Accepted
02 July 2014