Susac syndrome

Síndrome de Susac

Susac syndrome is a rare autoimmune endotheliopathy caused by a microangiopathic occlusive arteriolar disease¹ characterized by the triad of encephalopathy, visual loss and sensorineural hearing loss².

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/mm³, 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 pulse³, presenting good results.

Figure 3
Axial diffusion-weighted image demonstrates restricted diffusion in some of the lesions, characterized by hyperintensity in this sequence.

Figure 4
Fluorescein angiograms showing hemorrhages, staining of the vessel walls and leakage.

Figure 1
Sagittal FLAIR image discloses a small hyperintense lesion in the genu of the corpus callosum (arrow).

Figure 2
Axial FLAIR image shows some tiny scattered hyperintense lesions, more evident in the right cerebral periventricular white matter (arrows).

References

• ¹
  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
• ²
  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.1442-9071.2000.00345.x
• ³
  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.

Publication Dates

• Publication in this collection
  Oct 2014

History

• Received
  27 May 2014
• Reviewed
  12 June 2014
• Accepted
  02 July 2014