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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. Bersano A et al: Heritable and non-heritable uncommon causes of stroke. J Neurol. ePub, 2020
                                      2. Haralur Y et al: Neuroimaging of Multiple Sclerosis Mimics. Neurol Clin. 38(1):149-70, 2020
                                      3. Machado S et al: Cognitive dysfunction and brain atrophy in Susac syndrome. J Neurol. 267(4):994-1003, 2020
                                      4. Ramos-Ruperto L et al: Susac syndrome and pregnancy: a relationship to clarify. About two cases and review of the literature. J Matern Fetal Neonatal Med. 1-6, 2020
                                      5. Agamanolis DP et al: Brain microvascular pathology in Susac syndrome: an electron microscopic study of five cases. Ultrastruct Pathol. 43(6):229-36, 2019
                                      6. Coulette S et al: Diagnosis and prediction of relapses in susac syndrome: a new use for MR postcontrast FLAIR leptomeningeal enhancement. AJNR Am J Neuroradiol. 40(7):1184-90, 2019
                                      7. Egan RA: Diagnostic criteria and treatment algorithm for Susac syndrome. J Neuroophthalmol. 39(1):60-7, 2019
                                      8. Gross CC et al: CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Nat Commun. 10(1):5779, 2019
                                      9. Law LY et al: The spectrum of immune-mediated and inflammatory lesions of the brainstem: clues to diagnosis. Neurology. 93(9):390-405, 2019
                                      10. Onat Demirci N et al: Susac's syndrome as a rare arterial stroke mimic. Neuroradiol J. 32(3):200-2, 2019
                                      11. Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019
                                      12. Marrodan M et al: Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system. J Neurol Sci. 395:29-34, 2018
                                      13. Rennebohm RM et al: Guidelines for treatment of Susac syndrome - an update. Int J Stroke. 1747493017751737, 2018
                                      14. Sarbu N et al: White matter diseases with radiologic-pathologic correlation. Radiographics. 36(5):1426-47, 2016
                                      15. 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
                                      16. Greco A et al: Susac's syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 13(8):814-21, 2014
                                      17. 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
                                      18. Dörr J et al: Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 9(6):307-16, 2013
                                      19. Mateen FJ et al: Susac syndrome: clinical characteristics and treatment in 29 new cases. Eur J Neurol. 19(6):800-11, 2012
                                      20. Bitra RK et al: Review of Susac syndrome. Curr Opin Ophthalmol. 22(6):472-6, 2011
                                      21. Kleffner I et al: Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. Neurology. 70(19 Pt 2):1867-9, 2008
                                      22. Susac JO et al: Susac's syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 257(1-2):270-2, 2007
                                      23. Do TH et al: Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol. 25(3):382-8, 2004
                                      24. 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
                                      25. Xu MS et al: Susac syndrome: serial diffusion-weighted MR imaging. Magn Reson Imaging. 22(9):1295-8, 2004
                                      26. 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. Bersano A et al: Heritable and non-heritable uncommon causes of stroke. J Neurol. ePub, 2020
                                                                          2. Haralur Y et al: Neuroimaging of Multiple Sclerosis Mimics. Neurol Clin. 38(1):149-70, 2020
                                                                          3. Machado S et al: Cognitive dysfunction and brain atrophy in Susac syndrome. J Neurol. 267(4):994-1003, 2020
                                                                          4. Ramos-Ruperto L et al: Susac syndrome and pregnancy: a relationship to clarify. About two cases and review of the literature. J Matern Fetal Neonatal Med. 1-6, 2020
                                                                          5. Agamanolis DP et al: Brain microvascular pathology in Susac syndrome: an electron microscopic study of five cases. Ultrastruct Pathol. 43(6):229-36, 2019
                                                                          6. Coulette S et al: Diagnosis and prediction of relapses in susac syndrome: a new use for MR postcontrast FLAIR leptomeningeal enhancement. AJNR Am J Neuroradiol. 40(7):1184-90, 2019
                                                                          7. Egan RA: Diagnostic criteria and treatment algorithm for Susac syndrome. J Neuroophthalmol. 39(1):60-7, 2019
                                                                          8. Gross CC et al: CD8+ T cell-mediated endotheliopathy is a targetable mechanism of neuro-inflammation in Susac syndrome. Nat Commun. 10(1):5779, 2019
                                                                          9. Law LY et al: The spectrum of immune-mediated and inflammatory lesions of the brainstem: clues to diagnosis. Neurology. 93(9):390-405, 2019
                                                                          10. Onat Demirci N et al: Susac's syndrome as a rare arterial stroke mimic. Neuroradiol J. 32(3):200-2, 2019
                                                                          11. Soun JE et al: Central nervous system vasculopathies. Radiol Clin North Am. 57(6):1117-31, 2019
                                                                          12. Marrodan M et al: Clinical and imaging features distinguishing Susac syndrome from primary angiitis of the central nervous system. J Neurol Sci. 395:29-34, 2018
                                                                          13. Rennebohm RM et al: Guidelines for treatment of Susac syndrome - an update. Int J Stroke. 1747493017751737, 2018
                                                                          14. Sarbu N et al: White matter diseases with radiologic-pathologic correlation. Radiographics. 36(5):1426-47, 2016
                                                                          15. 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
                                                                          16. Greco A et al: Susac's syndrome--pathogenesis, clinical variants and treatment approaches. Autoimmun Rev. 13(8):814-21, 2014
                                                                          17. 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
                                                                          18. Dörr J et al: Characteristics of Susac syndrome: a review of all reported cases. Nat Rev Neurol. 9(6):307-16, 2013
                                                                          19. Mateen FJ et al: Susac syndrome: clinical characteristics and treatment in 29 new cases. Eur J Neurol. 19(6):800-11, 2012
                                                                          20. Bitra RK et al: Review of Susac syndrome. Curr Opin Ophthalmol. 22(6):472-6, 2011
                                                                          21. Kleffner I et al: Diffusion tensor imaging demonstrates fiber impairment in Susac syndrome. Neurology. 70(19 Pt 2):1867-9, 2008
                                                                          22. Susac JO et al: Susac's syndrome: 1975-2005 microangiopathy/autoimmune endotheliopathy. J Neurol Sci. 257(1-2):270-2, 2007
                                                                          23. Do TH et al: Susac syndrome: report of four cases and review of the literature. AJNR Am J Neuroradiol. 25(3):382-8, 2004
                                                                          24. 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
                                                                          25. Xu MS et al: Susac syndrome: serial diffusion-weighted MR imaging. Magn Reson Imaging. 22(9):1295-8, 2004
                                                                          26. Susac JO et al: MRI findings in Susac's syndrome. Neurology. 61(12):1783-7, 2003