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Diffuse cauda equina enhancement in a middle aged male with Susac syndrome and symptomatic cauda equina syndrome

  • Andrew M. Allmendinger
    Correspondence
    Corresponding author. Tel.: +1 617 732 7260; fax: +1 617 254 5151.
    Affiliations
    Harvard Medical School, Brigham and Women's Hospital, Department of Neuroradiology, 15 Francis St., Boston, MA 02115, United States
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  • Author Footnotes
    1 Tel.: +1 617 732 7260; fax: +1 617 254 5151.
    Narayan Viswanadhan
    Footnotes
    1 Tel.: +1 617 732 7260; fax: +1 617 254 5151.
    Affiliations
    Harvard Medical School, Brigham and Women's Hospital, Department of Neuroradiology, 15 Francis St., Boston, MA 02115, United States
    Search for articles by this author
  • Author Footnotes
    1 Tel.: +1 617 732 7260; fax: +1 617 254 5151.
    Roman A. Klufas
    Footnotes
    1 Tel.: +1 617 732 7260; fax: +1 617 254 5151.
    Affiliations
    Harvard Medical School, Brigham and Women's Hospital, Department of Neuroradiology, 15 Francis St., Boston, MA 02115, United States
    Search for articles by this author
  • Author Footnotes
    1 Tel.: +1 617 732 7260; fax: +1 617 254 5151.
    Liangge Hsu
    Footnotes
    1 Tel.: +1 617 732 7260; fax: +1 617 254 5151.
    Affiliations
    Harvard Medical School, Brigham and Women's Hospital, Department of Neuroradiology, 15 Francis St., Boston, MA 02115, United States
    Search for articles by this author
  • Author Footnotes
    1 Tel.: +1 617 732 7260; fax: +1 617 254 5151.

      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 occlusions and sensorineural hearing loss. All three components of the triad usually do not present at the same time, thus delaying time to diagnosis. MRI studies often show characteristic punched out lesions of the central fibers of the corpus callosum. Intracranial leptomeningeal enhancement may be seen, however, cauda equina involvement has not been described to our knowledge. We present a case of Susac syndrome in a middle-aged male with symptoms of cauda equina syndrome, and spinal MRI showing diffuse enhancement of the nerve roots of the cauda equina.

      Abbreviations:

      ADEM (acute disseminated encephalomyelitis), BRAOs (branch retinal artery occlusions), CT (computed tomography), CMV (Cytomegalovirus), HSV (herpes simplex virus), IVIG (intravenous immunoglobulin), MRI (magnetic resonance imaging), MPRAGE (Magnetization Prepared Rapid Gradient Echo), MS (multiple sclerosis), PET-CT (Positron Emission Tomography-CT), VZV (varicella zoster virus), WBC (white blood cell)

      Keywords

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      References

        • Susac J.O.
        • Hardimann J.M.
        • Selhorst J.B.
        Microangiopathy 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 J.O.
        • Egan R.A.
        • Rennebohm R.M.
        • Lubow M.
        Susac's syndrome: 1975–2005 microangiopathy/autoimmune endotheliopathy.
        J Neurol Sci. 2007; 257 ([15]): 270-272
        • Kleffner I.
        • Duning T.
        • Lohmann H.
        • Deppe M.
        • Basel T.
        • Promesberger J.
        • et al.
        A brief review of Susac syndrome.
        J Neurol Sci. 2012; 1–2 ([15]): 35-40
        • Ozturk A.
        • Degirmenci Y.
        • Tunc M.
        • Kececi H.
        Susac's syndrome: a case of simultaneous development of all three components of the triad.
        J Neurol Sci. 2013; 324: 187-189
        • Susac J.O.
        Susac's syndrome.
        AJNR Am J Neuroradiol. 2004; 25: 351-352
        • Do T.H.
        • 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 A.W.
        • Magalhaes A.
        • Kish K.
        MRI of Susac's syndrome.
        AJR Am J Roentgenol. 2005; 184: 1688-1690
        • Susac J.O.
        • Murtagh F.R.
        • Egan R.A.
        • Berger J.R.
        • Bakshi R.
        • Lincoff N.
        • et al.
        MRI findings in Susac's syndrome.
        Neurology. 2003; 61: 1783-1787
        • White M.L.
        • Yan Z.
        • Smoker W.R.
        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
        • Rennebohm R.
        • Susac J.O.
        • Egan R.A.
        • Daroff R.B.
        Susac's syndrome—update.
        J Neurol Sci. 2010; 299: 86-91
        • Kleffner I.
        • Deppe M.
        • Mohammadi S.
        • Schwindt W.
        • Sommer J.
        • Young P.
        • et al.
        Neuroimaging in Susac's syndrome; focus on DTI.
        J Neurol Sci. 2010; 299: 92-96
        • 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
        • Allmendinger A.M.
        • Spektor V.
        • Destian S.
        CT and MR imaging of Susac syndrome in a young male presenting with disorientation.
        Clin Imaging. 2010; 34: 138-142
        • Rennebohm R.M.
        • Egan R.A.
        • Susac J.O.
        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. Feb. 2009; 30: 59-64