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Superficial Siderosis, Cortical
Miral D. Jhaveri, MD, MBA; Anne G. Osborn, MD, FACR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Superficial siderosis (SS)
              • Cortical SS (cSS)
            • Synonyms

              • Superficial cortical hemosiderosis
            • Definitions

              • SS
                • Hemosiderin deposition along pial surfaces of brain, cranial nerves, spinal cord
              • 2 types of SS now recognized
                • Classic/infratentorial SS
                  • Primarily posterior fossa, spinal cord
                • cSS
                  • SS limited to cortex over hemisphere convexities

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          CLINICAL ISSUES

                          • Presentation

                            • Natural History & Prognosis

                              DIAGNOSTIC CHECKLIST

                              • Consider

                                • Image Interpretation Pearls

                                  Selected References

                                  1. Pongpitakmetha T et al: Cortical superficial siderosis progression in cerebral amyloid angiopathy: prospective MRI study. Neurology. 94(17):e1853-65, 2020
                                  2. Charidimou A et al: APOE and cortical superficial siderosis in CAA: meta-analysis and potential mechanisms. Neurology. 93(4):e358-71, 2019
                                  3. 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
                                  4. Charidimou A et al: Cortical superficial siderosis evolution. Stroke. 50(4):954-62, 2019
                                  5. Wollenweber FA et al: Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. Neurology. 92(8):e792-e801, 2019
                                  6. Banerjee G et al: Cognitive impairment before intracerebral hemorrhage is associated with cerebral amyloid angiopathy. Stroke. 49(1):40-5, 2018
                                  7. Charidimou A et al: Cortical superficial siderosis and first-ever cerebral hemorrhage in cerebral amyloid angiopathy. Neurology. 88(17):1607-14, 2017
                                  8. Charidimou A et al: Cortical superficial siderosis multifocality in cerebral amyloid angiopathy: a prospective study. Neurology. 89(21):2128-35, 2017
                                  9. Wilson D et al: Convexity subarachnoid haemorrhage has a high risk of intracerebral haemorrhage in suspected cerebral amyloid angiopathy. J Neurol. 264(4):664-73, 2017
                                  10. Charidimou A: Cortical superficial siderosis presumed due to cerebral amyloid angiopathy: minimum standards for rating and reporting. AJNR Am J Neuroradiol. 37(5):E43-4, 2016
                                  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

                                            TERMINOLOGY

                                            • Abbreviations

                                              • Superficial siderosis (SS)
                                              • Cortical SS (cSS)
                                            • Synonyms

                                              • Superficial cortical hemosiderosis
                                            • Definitions

                                              • SS
                                                • Hemosiderin deposition along pial surfaces of brain, cranial nerves, spinal cord
                                              • 2 types of SS now recognized
                                                • Classic/infratentorial SS
                                                  • Primarily posterior fossa, spinal cord
                                                • cSS
                                                  • SS limited to cortex over hemisphere convexities

                                            IMAGING

                                            • General Features

                                              • CT Findings

                                                • MR Findings

                                                  • Imaging Recommendations

                                                    DIFFERENTIAL DIAGNOSIS

                                                      PATHOLOGY

                                                      • General Features

                                                        • Gross Pathologic & Surgical Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Natural History & Prognosis

                                                              DIAGNOSTIC CHECKLIST

                                                              • Consider

                                                                • Image Interpretation Pearls

                                                                  Selected References

                                                                  1. Pongpitakmetha T et al: Cortical superficial siderosis progression in cerebral amyloid angiopathy: prospective MRI study. Neurology. 94(17):e1853-65, 2020
                                                                  2. Charidimou A et al: APOE and cortical superficial siderosis in CAA: meta-analysis and potential mechanisms. Neurology. 93(4):e358-71, 2019
                                                                  3. 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
                                                                  4. Charidimou A et al: Cortical superficial siderosis evolution. Stroke. 50(4):954-62, 2019
                                                                  5. Wollenweber FA et al: Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. Neurology. 92(8):e792-e801, 2019
                                                                  6. Banerjee G et al: Cognitive impairment before intracerebral hemorrhage is associated with cerebral amyloid angiopathy. Stroke. 49(1):40-5, 2018
                                                                  7. Charidimou A et al: Cortical superficial siderosis and first-ever cerebral hemorrhage in cerebral amyloid angiopathy. Neurology. 88(17):1607-14, 2017
                                                                  8. Charidimou A et al: Cortical superficial siderosis multifocality in cerebral amyloid angiopathy: a prospective study. Neurology. 89(21):2128-35, 2017
                                                                  9. Wilson D et al: Convexity subarachnoid haemorrhage has a high risk of intracerebral haemorrhage in suspected cerebral amyloid angiopathy. J Neurol. 264(4):664-73, 2017
                                                                  10. Charidimou A: Cortical superficial siderosis presumed due to cerebral amyloid angiopathy: minimum standards for rating and reporting. AJNR Am J Neuroradiol. 37(5):E43-4, 2016