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Susac Syndrome
Karen L. Salzman, MD; Jeffrey S. Anderson, MD, PhD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Synonyms

                • Retinocochleocerebral vasculopathy, formerly known as small infarctions of cochlear, retinal, and encephalic tissue (SICRET syndrome)
              • Definitions

                • Microangiopathy of brain, retina, and cochlea

              IMAGING

              • General Features

                • Imaging Recommendations

                  • CT Findings

                    • MR Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. Filippi CG et al: Lesions of the corpus callosum and other commissural fibers: diffusion tensor studies. Semin Ultrasound CT MR. 35(5):445-58, 2014
                                      2. Greco A et al: Susac's syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 13(8):814-21, 2014
                                      3. Allmendinger AM et al: Diffuse cauda equina enhancement in a middle aged male with Susac syndrome and symptomatic cauda equina syndrome. J Neurol Sci. 333(1-2):25-8, 2013
                                      4. Dörr J et al: Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 9(6):307-16, 2013
                                      5. Mateen FJ et al: Susac syndrome: clinical characteristics and treatment in 29 new cases. Eur J Neurol. 19(6):800-11, 2012
                                      6. Bitra RK et al: Review of Susac syndrome. Curr Opin Ophthalmol. Epub ahead of print, 2011
                                      7. Kleffner I et al: Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. Neurology. 70(19 Pt 2):1867-9, 2008
                                      8. Susac JO et al: Susac's syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 257(1-2):270-2, 2007
                                      9. Do TH et al: Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol. 25(3):382-8, 2004
                                      10. White ML et al: Evolution of lesions in Susac syndrome at serial MR imaging with diffusion-weighted imaging and apparent diffusion coefficient values. AJNR Am J Neuroradiol. 25(5):706-13, 2004
                                      11. Xu MS et al: Susac syndrome: serial diffusion-weighted MR imaging. Magn Reson Imaging. 22(9):1295-8, 2004
                                      12. Susac JO et al: MRI findings in Susac's syndrome. Neurology. 61(12):1783-7, 2003
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Pathology

                                              • Clinical Issues

                                                • Diagnostic Checklist

                                                  TERMINOLOGY

                                                  • Synonyms

                                                    • Retinocochleocerebral vasculopathy, formerly known as small infarctions of cochlear, retinal, and encephalic tissue (SICRET syndrome)
                                                  • Definitions

                                                    • Microangiopathy of brain, retina, and cochlea

                                                  IMAGING

                                                  • General Features

                                                    • Imaging Recommendations

                                                      • CT Findings

                                                        • MR Findings

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • Microscopic Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      DIAGNOSTIC CHECKLIST

                                                                      • Consider

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. Filippi CG et al: Lesions of the corpus callosum and other commissural fibers: diffusion tensor studies. Semin Ultrasound CT MR. 35(5):445-58, 2014
                                                                          2. Greco A et al: Susac's syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 13(8):814-21, 2014
                                                                          3. Allmendinger AM et al: Diffuse cauda equina enhancement in a middle aged male with Susac syndrome and symptomatic cauda equina syndrome. J Neurol Sci. 333(1-2):25-8, 2013
                                                                          4. Dörr J et al: Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 9(6):307-16, 2013
                                                                          5. Mateen FJ et al: Susac syndrome: clinical characteristics and treatment in 29 new cases. Eur J Neurol. 19(6):800-11, 2012
                                                                          6. Bitra RK et al: Review of Susac syndrome. Curr Opin Ophthalmol. Epub ahead of print, 2011
                                                                          7. Kleffner I et al: Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. Neurology. 70(19 Pt 2):1867-9, 2008
                                                                          8. Susac JO et al: Susac's syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 257(1-2):270-2, 2007
                                                                          9. Do TH et al: Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol. 25(3):382-8, 2004
                                                                          10. White ML et al: Evolution of lesions in Susac syndrome at serial MR imaging with diffusion-weighted imaging and apparent diffusion coefficient values. AJNR Am J Neuroradiol. 25(5):706-13, 2004
                                                                          11. Xu MS et al: Susac syndrome: serial diffusion-weighted MR imaging. Magn Reson Imaging. 22(9):1295-8, 2004
                                                                          12. Susac JO et al: MRI findings in Susac's syndrome. Neurology. 61(12):1783-7, 2003