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Superficial Siderosis
Kevin R. Moore, MD; Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          • Diagnostic Checklist

            TERMINOLOGY

            • Abbreviations

              • Superficial siderosis (SS)
            • Synonyms

              • CNS siderosis, hemosiderosis
            • Definitions

              • Recurrent subarachnoid hemorrhage (multiple etiologies) causing hemosiderin deposition on cord and nerve surface

            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

                                      Selected References

                                      1. Charidimou A et al: APOE and cortical superficial siderosis in CAA: meta-analysis and potential mechanisms. Neurology. 93(4):e358-71, 2019
                                      2. Wollenweber FA et al: Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. Neurology. 92(8):e792-801, 2019
                                      3. Takai K et al: Superficial siderosis of the central nervous system associated with intraspinal hemorrhage from ventral thoracic epidural veins and a ventral spinal CSF leak: case report. J Neurosurg Spine. 26(6):751-3, 2017
                                      4. Wilson D et al: Infratentorial superficial siderosis: classification, diagnostic criteria, and rational investigation pathway. Ann Neurol. 81(3):333-43, 2017
                                      5. Pikis S et al: Superficial siderosis of the central nervous system secondary to spinal ependymoma. J Clin Neurosci. 21(11):2017-9, 2014
                                      6. De Reuck J et al: Superficial siderosis of the central nervous system: a post-mortem 7.0-tesla magnetic resonance imaging study with neuropathological correlates. Cerebrovasc Dis. 36(5-6):412-7, 2013
                                      7. Kumar N: Neuroimaging in superficial siderosis: an in-depth look. AJNR Am J Neuroradiol. 31(1):5-14, 2010
                                      8. Messori A et al: The importance of suspecting superficial siderosis of the central nervous system in clinical practice. J Neurol Neurosurg Psychiatry. 75(2):188-90, 2004
                                      9. Anderson NE: Late complications in childhood central nervous system tumour survivors. Curr Opin Neurol. 16(6):677-83, 2003
                                      10. Yoshida S et al: Superficial siderosis from spinal teratoma. Lancet. 360(9345):1539, 2002
                                      11. Polidori MC et al: Superficial siderosis of the central nervous system: a 70-year-old man with ataxia, depression and visual deficits. Gerontology. 47(2):93-5, 2001
                                      12. Bostantjopoulou S et al: Superficial CNS siderosis and spinal pilocytic astrocytoma. Neurology. 55(3):450, 2000
                                      13. Padberg M et al: Cerebral siderosis: deafness by a spinal tumor. J Neurol. 247(6):473, 2000
                                      14. Manfredi M et al: Superficial siderosis of the central nervous system and anticoagulant therapy: a case report. Ital J Neurol Sci. 20(4):247-9, 1999
                                      15. Pelak VS et al: Evidence for preganglionic pupillary involvement in superficial siderosis. Neurology. 53(5):1130-2, 1999
                                      16. Lemmerling M et al: Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss. Neuroradiology. 40(5):312-4, 1998
                                      17. Schievink WI et al: Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation. Case report. J Neurosurg. 89(6):1029-31, 1998
                                      18. Lai MT et al: Superficial siderosis of the central nervous system: a case with an unruptured intracranial aneurysm. J Laryngol Otol. 109(6):549-52, 1995
                                      19. Pyhtinen J et al: Superficial siderosis in the central nervous system. Neuroradiology. 37(2):127-8, 1995
                                      20. Bonito V et al: Superficial siderosis of the central nervous system after brachial plexus injury. Case report. J Neurosurg. 80(5):931-4, 1994
                                      21. Pribitkin EA et al: Superficial siderosis of the central nervous system: an underdiagnosed cause of sensorineural hearing loss and ataxia. Am J Otol. 15(3):415-8, 1994
                                      22. River Y et al: Superficial hemosiderosis of the central nervous system. Mov Disord. 9(5):559-62, 1994
                                      23. Bracchi M et al: Superficial siderosis of the CNS: MR diagnosis and clinical findings. AJNR Am J Neuroradiol. 14(1):227-36, 1993
                                      24. Grunshaw ND et al: Superficial siderosis of the central nervous system--diagnosis by magnetic resonance imaging. Clin Radiol. 48(3):186-8, 1993
                                      25. Kumar A et al: Posterior fossa surgery: an unusual cause of superficial siderosis. Neurosurgery. 32(3):455-7; discussion 457, 1993
                                      26. Parnes SM et al: Superficial siderosis of the central nervous system: a neglected cause of sensorineural hearing loss. Otolaryngol Head Neck Surg. 107(1):69-77, 1992
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Terminology

                                        • Imaging

                                          • Top Differential Diagnoses

                                            • Clinical Issues

                                              • Diagnostic Checklist

                                                TERMINOLOGY

                                                • Abbreviations

                                                  • Superficial siderosis (SS)
                                                • Synonyms

                                                  • CNS siderosis, hemosiderosis
                                                • Definitions

                                                  • Recurrent subarachnoid hemorrhage (multiple etiologies) causing hemosiderin deposition on cord and nerve surface

                                                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

                                                                          Selected References

                                                                          1. Charidimou A et al: APOE and cortical superficial siderosis in CAA: meta-analysis and potential mechanisms. Neurology. 93(4):e358-71, 2019
                                                                          2. Wollenweber FA et al: Prognostic relevance of cortical superficial siderosis in cerebral amyloid angiopathy. Neurology. 92(8):e792-801, 2019
                                                                          3. Takai K et al: Superficial siderosis of the central nervous system associated with intraspinal hemorrhage from ventral thoracic epidural veins and a ventral spinal CSF leak: case report. J Neurosurg Spine. 26(6):751-3, 2017
                                                                          4. Wilson D et al: Infratentorial superficial siderosis: classification, diagnostic criteria, and rational investigation pathway. Ann Neurol. 81(3):333-43, 2017
                                                                          5. Pikis S et al: Superficial siderosis of the central nervous system secondary to spinal ependymoma. J Clin Neurosci. 21(11):2017-9, 2014
                                                                          6. De Reuck J et al: Superficial siderosis of the central nervous system: a post-mortem 7.0-tesla magnetic resonance imaging study with neuropathological correlates. Cerebrovasc Dis. 36(5-6):412-7, 2013
                                                                          7. Kumar N: Neuroimaging in superficial siderosis: an in-depth look. AJNR Am J Neuroradiol. 31(1):5-14, 2010
                                                                          8. Messori A et al: The importance of suspecting superficial siderosis of the central nervous system in clinical practice. J Neurol Neurosurg Psychiatry. 75(2):188-90, 2004
                                                                          9. Anderson NE: Late complications in childhood central nervous system tumour survivors. Curr Opin Neurol. 16(6):677-83, 2003
                                                                          10. Yoshida S et al: Superficial siderosis from spinal teratoma. Lancet. 360(9345):1539, 2002
                                                                          11. Polidori MC et al: Superficial siderosis of the central nervous system: a 70-year-old man with ataxia, depression and visual deficits. Gerontology. 47(2):93-5, 2001
                                                                          12. Bostantjopoulou S et al: Superficial CNS siderosis and spinal pilocytic astrocytoma. Neurology. 55(3):450, 2000
                                                                          13. Padberg M et al: Cerebral siderosis: deafness by a spinal tumor. J Neurol. 247(6):473, 2000
                                                                          14. Manfredi M et al: Superficial siderosis of the central nervous system and anticoagulant therapy: a case report. Ital J Neurol Sci. 20(4):247-9, 1999
                                                                          15. Pelak VS et al: Evidence for preganglionic pupillary involvement in superficial siderosis. Neurology. 53(5):1130-2, 1999
                                                                          16. Lemmerling M et al: Secondary superficial siderosis of the central nervous system in a patient presenting with sensorineural hearing loss. Neuroradiology. 40(5):312-4, 1998
                                                                          17. Schievink WI et al: Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation. Case report. J Neurosurg. 89(6):1029-31, 1998
                                                                          18. Lai MT et al: Superficial siderosis of the central nervous system: a case with an unruptured intracranial aneurysm. J Laryngol Otol. 109(6):549-52, 1995
                                                                          19. Pyhtinen J et al: Superficial siderosis in the central nervous system. Neuroradiology. 37(2):127-8, 1995
                                                                          20. Bonito V et al: Superficial siderosis of the central nervous system after brachial plexus injury. Case report. J Neurosurg. 80(5):931-4, 1994
                                                                          21. Pribitkin EA et al: Superficial siderosis of the central nervous system: an underdiagnosed cause of sensorineural hearing loss and ataxia. Am J Otol. 15(3):415-8, 1994
                                                                          22. River Y et al: Superficial hemosiderosis of the central nervous system. Mov Disord. 9(5):559-62, 1994
                                                                          23. Bracchi M et al: Superficial siderosis of the CNS: MR diagnosis and clinical findings. AJNR Am J Neuroradiol. 14(1):227-36, 1993
                                                                          24. Grunshaw ND et al: Superficial siderosis of the central nervous system--diagnosis by magnetic resonance imaging. Clin Radiol. 48(3):186-8, 1993
                                                                          25. Kumar A et al: Posterior fossa surgery: an unusual cause of superficial siderosis. Neurosurgery. 32(3):455-7; discussion 457, 1993
                                                                          26. Parnes SM et al: Superficial siderosis of the central nervous system: a neglected cause of sensorineural hearing loss. Otolaryngol Head Neck Surg. 107(1):69-77, 1992