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Superficial Siderosis, Classical
Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          TERMINOLOGY

          • Abbreviations

            • Superficial siderosis (SS), subarachnoid hemorrhage (SAH)
          • Synonyms

            • Siderosis, CNS siderosis
          • Definitions

            • Chronic, recurrent SAH → hemosiderin deposition on pial surfaces of brain, brainstem, cranial nerves (CNs), spinal cord
            • 2 types of SS
              • Classical SS (primarily affects infratentorial regions, spinal cord)
              • Cortical SS (primarily affects convexity sulci, sparing infratentorial structures)
                • Most often associated with cerebral amyloid angiopathy

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Imaging Recommendations

                  DIFFERENTIAL DIAGNOSIS

                    PATHOLOGY

                    • General Features

                      • Gross Pathologic & Surgical Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    • Image Interpretation Pearls

                                      • Reporting Tips

                                        Selected References

                                        1. Ryu HU et al: Superficial siderosis with rapid progressive cognitive decline. Neurol Sci. ePub, 2020
                                        2. Tsai HH et al: Superficial cerebellar microbleeds and cerebral amyloid angiopathy: a magnetic resonance imaging/positron emission tomography study. Stroke. 51(1):202-8, 2020
                                        3. Wiącek M et al: Superficial siderosis and intracranial hypotension syndrome following brachial plexus avulsion injury. A case of surgical treatment. Clin Neurol Neurosurg. 192:105723, 2020
                                        4. Charidimou A et al: Cortical superficial siderosis and recurrent intracerebral hemorrhage risk in cerebral amyloid angiopathy: large prospective cohort and preliminary meta-analysis. Int J Stroke. 14(7):723-33, 2019
                                        5. Charidimou A et al: Cortical superficial siderosis evolution. Stroke. 50(4):954-62, 2019
                                        6. Richard C et al: Multiple cavernous malformations as a cause for superficial siderosis of the central nervous system. Acta Neurol Belg. 120(3):755-7, 2019
                                        7. Wagner F et al: Superficial siderosis of the central nervous system: neurotological findings related to magnetic resonance imaging. Otol Neurotol. 40(1):31-7, 2019
                                        8. Wollenweber FA et al: Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. Neurology. 92(8):e792-801, 2019
                                        9. Calviere L et al: Patterns of convexal subarachnoid haemorrhage: clinical, radiological and outcome differences between cerebral amyloid angiopathy and other causes. J Neurol. 265(1):204-10, 2018
                                        10. Jetty SN et al: Clinical significance of T2*gradient-recalled echo/susceptibility-weighted Imaging sequences in evaluating superficial siderosis in the setting of intracerebral tumors: pilocytic astrocytoma. J Clin Imaging Sci. 8:36, 2018
                                        11. Wang J et al: Superficial siderosis of the central nervous system: MR findings with susceptibility-weighted imaging. Clin Imaging. 35(3):217-21, 2011
                                        12. Kumar N: Neuroimaging in superficial siderosis: an in-depth look. AJNR Am J Neuroradiol. 31(1):5-14, 2010
                                        13. Koeppen AH et al: The pathology of superficial siderosis of the central nervous system. Acta Neuropathol. 116(4):371-82, 2008
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Superficial siderosis (SS), subarachnoid hemorrhage (SAH)
                                                • Synonyms

                                                  • Siderosis, CNS siderosis
                                                • Definitions

                                                  • Chronic, recurrent SAH → hemosiderin deposition on pial surfaces of brain, brainstem, cranial nerves (CNs), spinal cord
                                                  • 2 types of SS
                                                    • Classical SS (primarily affects infratentorial regions, spinal cord)
                                                    • Cortical SS (primarily affects convexity sulci, sparing infratentorial structures)
                                                      • Most often associated with cerebral amyloid angiopathy

                                                IMAGING

                                                • General Features

                                                  • CT Findings

                                                    • MR Findings

                                                      • Imaging Recommendations

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Gross Pathologic & Surgical Features

                                                              • Microscopic Features

                                                                CLINICAL ISSUES

                                                                • Presentation

                                                                  • Demographics

                                                                    • Natural History & Prognosis

                                                                      • Treatment

                                                                        DIAGNOSTIC CHECKLIST

                                                                        • Consider

                                                                          • Image Interpretation Pearls

                                                                            • Reporting Tips

                                                                              Selected References

                                                                              1. Ryu HU et al: Superficial siderosis with rapid progressive cognitive decline. Neurol Sci. ePub, 2020
                                                                              2. Tsai HH et al: Superficial cerebellar microbleeds and cerebral amyloid angiopathy: a magnetic resonance imaging/positron emission tomography study. Stroke. 51(1):202-8, 2020
                                                                              3. Wiącek M et al: Superficial siderosis and intracranial hypotension syndrome following brachial plexus avulsion injury. A case of surgical treatment. Clin Neurol Neurosurg. 192:105723, 2020
                                                                              4. Charidimou A et al: Cortical superficial siderosis and recurrent intracerebral hemorrhage risk in cerebral amyloid angiopathy: large prospective cohort and preliminary meta-analysis. Int J Stroke. 14(7):723-33, 2019
                                                                              5. Charidimou A et al: Cortical superficial siderosis evolution. Stroke. 50(4):954-62, 2019
                                                                              6. Richard C et al: Multiple cavernous malformations as a cause for superficial siderosis of the central nervous system. Acta Neurol Belg. 120(3):755-7, 2019
                                                                              7. Wagner F et al: Superficial siderosis of the central nervous system: neurotological findings related to magnetic resonance imaging. Otol Neurotol. 40(1):31-7, 2019
                                                                              8. Wollenweber FA et al: Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. Neurology. 92(8):e792-801, 2019
                                                                              9. Calviere L et al: Patterns of convexal subarachnoid haemorrhage: clinical, radiological and outcome differences between cerebral amyloid angiopathy and other causes. J Neurol. 265(1):204-10, 2018
                                                                              10. Jetty SN et al: Clinical significance of T2*gradient-recalled echo/susceptibility-weighted Imaging sequences in evaluating superficial siderosis in the setting of intracerebral tumors: pilocytic astrocytoma. J Clin Imaging Sci. 8:36, 2018
                                                                              11. Wang J et al: Superficial siderosis of the central nervous system: MR findings with susceptibility-weighted imaging. Clin Imaging. 35(3):217-21, 2011
                                                                              12. Kumar N: Neuroimaging in superficial siderosis: an in-depth look. AJNR Am J Neuroradiol. 31(1):5-14, 2010
                                                                              13. Koeppen AH et al: The pathology of superficial siderosis of the central nervous system. Acta Neuropathol. 116(4):371-82, 2008