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CT and MR imaging of Susac syndrome in a young male presenting with acute disorientation

      Abstract

      Susac syndrome is a rare neurologic disorder first described by Susac et al. in 1979. Clinically, Susac syndrome consists of a triad including encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. Microinfarction is believed to be the basic histologic feature and MR is considered the best imaging modality. This case documents the typical imaging findings of Susac syndrome, as well as the unique MR imaging finding of cranial nerve enhancement.

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      References

        • Susac JO
        • Hardimann JM
        • Selhorst JB
        Micoangiopathy of the brain and retina.
        Neurology. 1979; 29: 313-316
        • Vila N
        • Graus F
        • Blesa R
        • Santamaria J
        • Ribalta T
        • Tolosa E.
        Microangiopathy of the brain and retina (Susac's syndrome): two patients with atypical features.
        Neurology. 1995; 45: 1225-1226
        • Susac JO
        Susac's syndrome.
        AJNR Am J Neuroradiol. 2004; 25: 351-352
        • Susac JO
        • Egan RA
        • Rennebohm RM
        • Lubow M
        Susac's syndrome: 1975–2005 microangiopathy/autoimmune endotheliopathy.
        J Neurol Sci. 2007; 257 ([Epub 2007 Feb 28]): 270-272
        • Do TH
        • Fisch C
        • Evoy F
        Susac syndrome: report of four cases and review of literature.
        AJNR Am J Neuroradiol. 2004; 25: 382-388
      1. Microangiopathy of the brain, retina and inner ear (Susac's Syndrome) in an adolescent female presenting as acute disseminated encephalomyelitis.
        Pediatrics. 2004; 114: 226-281
        • Saenz R
        • Quan AW
        • Magalhaes A
        • Kish K
        MRI of Susac's syndrome.
        AJR Am J Roentgenol. 2005; 184: 1688-1690
        • Susac JO
        • Murtagh FR
        • Egan RA
        • Berger JR
        • Bakshi R
        • Lincoff N
        • Gean AD
        • Galetta SL
        • Fox RJ
        • Costello FE
        • Lee AG
        • Clark J
        • Layzer RB
        • Daroff RB
        MRI findings in Susac's syndrome.
        Neurology. 2003; 61: 1783-1787
        • White ML
        • Yan Z
        • Smoker WR
        Evolution of lesions in Susac syndrome at serial MR imaging with diffusion-weighted imaging and apparent diffusion coefficient values.
        AJNR Am J Neuroradiol. 2004; 25: 706-713
        • Kleffner I
        • Deppe M
        • Mohammadi S
        • Schiffbauer H
        • Stupp N
        • Lohmann H
        • Young P
        • Ringelstein E.B.
        Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome.
        Neurology. 2008; 70: 1867-1869
        • Rennebohm RM
        • Egan RA
        • Susac JO
        Treatment of Susac's syndrome.
        Curr Treat Options Neurol. 2008; 10: 67-74
        • Pawate S
        • Agarwal A
        • Moses H
        • Sriram S
        The spectrum of Susac's syndrome.
        Neurol Sci. 2009; 30 ([Epub 2009 Jan 15]): 59-64