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Case Reports
. 2004 Mar;25(3):382-8.

Susac syndrome: report of four cases and review of the literature

Tai Hung Do  1 , Christiane Fisch, François Evoy
Affiliations
Case Reports

Susac syndrome: report of four cases and review of the literature

Tai Hung Do et al. AJNR Am J Neuroradiol. 2004 Mar.

Abstract

Susac syndrome is a rare disease of unknown pathogenesis. It is caused by a microangiopathy affecting the arterioles of the brain, retina, and cochlea, giving the classic clinical triad of subacute encephalopathy, visual loss secondary to retinal branch occlusions, and sensorineural hearing loss. The features of four cases of this syndrome are presented. MR imaging, retinal fluorescein angiography, and audiography findings enable diagnosis. Early therapy may reduce sequelae and improve recovery.

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Figures

F<sc>ig</sc> 1.
Fig 1.
Case 1. A, Axial T2-weighted MR image shows hyperintense lesions in the periventricular white matter and corpus callosum. B, Axial T2-weighted MR image shows a small, hyperintense foci in the left posterior arm of the internal capsule.
F<sc>ig</sc> 2.
Fig 2.
Case 2. A, Axial T1-weighted postcontrast MR image shows leptomeningeal enhancement in the interpeduncular area. B, Axial T2-weighted MR image shows hyperintense lesions in the corpus callosum and periventricular white matter. C, Histopathologic examination shows areas of capillary hyaline thrombi with perivascular inflammatory cells (hematoxylin and eosin stain; magnification ×400). D, Retinal fluorescein angiography shows peripheral retinal arteriolar branch occlusions in the left eye. E, Axial T2-weighted MR image shows bilateral hyperintense lesions in the thalamus and posterior arm of the internal capsule. F, Axial T1-weighted postcontrast MR imaging shows enhancing lesions in the corpus callosum and the posterior arm of the internal capsule.
F<sc>ig</sc> 3.
Fig 3.
Case 3. A, Axial T1-weighted postcontrast MR image shows numerous foci of enhancement in the cerebellum. B, Axial T2-weighted MR image shows small, hyperintense lesions in the right periventricular frontal white matter. C, Audiogram shows moderate to severe sensorineural hearing loss on the left ear.
F<sc>ig</sc> 4.
Fig 4.
Case 4. A, Axial T2-weighted MR image shows hyperintense lesions in the left middle cerebellar peduncle. B, Retinal fluorescein angiogram shows an inflamed left retinal arteriole with a leaking arteriolar wall. C, Axial T2-weighted MR image shows increase number of hyperintense lesions in the cerebellum.
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References

    1. Susac JO, Hardman JM, Selhorst JB. Microangiopathy of the brain and retina. Neurology 1979;29:313–316 - PubMed
    1. Ayache D, Plouin GI, Bakouche P, et al. Microangiopathy of the inner ear, retina, and brain (Susac syndrome): report of a case. Arch Otolaryngol Head Neck Surg 2000;126:82–84 - PubMed
    1. Susac JO. Susac’s syndrome: the triad of microangiopathy of the brain and retina with hearing loss in young women. Neurology 1994;44:591–593 - PubMed
    1. Mass M, Bourdette D, Bernstein W, Hammerstad J. Retinopathy, encephalopathy, deafness associated microangiopathy (the RED M syndrome): three new cases. Neurology 1988;38(Suppl):215
    1. Nicolle MW, Mclachlan RS. Microangiopathy with retinopathy, encephalopathy, and deafness (RED-M) and systemic features. Semin Arthritis Rheum 1991;21:123–128 - PubMed

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