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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Arq. Neuro-Psiquiatr. vol.72 no.10 São Paulo Oct. 2014

http://dx.doi.org/10.1590/0004-282X20140128 

Images in Neurology

Susac syndrome

Síndrome de Susac

Fernando Freua 1  

Leandro Tavares Lucato 2  

Flávio Villela 3  

Getúlio Daré Rabello 4   5  

1Departamento de Neurogenética, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo SP, Brazil;

2Departamento de Neuroradiologia, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo SP, Brazil;

3Setor de Córnea e Doenças Externas, Departamento de Oftalmologia, Faculdade de Medicina, Universidade de São Paulo, São Paulo SP, Brazil;

4Departamento de Neurologia, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo SP, Brazil;

5Departamento de Neurologia, Hospital Samaritano, Sao Paulo SP, Brazil.

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

. 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 [ Links ]

. 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 [ Links ]

. 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. [ Links ]

Received: May 27, 2014; Revised: June 12, 2014; Accepted: July 02, 2014

Correspondence: Fernando Freua; Rua Mato Grosso, 306 Cj 1601; 01239-040 São Paulo SP, Brasil; E-mail: fernando.freua@gmail.com

Conflict of interest: There is no conflict of interest to declare.

Creative Commons License This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.