Skip to main content

Superficial Siderosis

  • Chapter
  • First Online:
Essentials of Cerebellum and Cerebellar Disorders

Abstract

Superficial siderosis (SS) is an uncommon and often underdiagnosed disorder caused by chronic or repeated hemorrhage in the subarachnoid space that deposits hemosiderin in the subpial layers of the central nervous system, leading to impaired nerve function. The progression of SS is insidious, often requiring patients to experience advanced symptoms until a clinician with a high level of suspicion for the condition orders magnetic resonance imaging (MRI) of the brain or spine and diagnoses the condition by confirming the characteristic image findings. Conditions that can cause repeated bleeding episodes into the subarachnoid space are wide-ranging, so the diagnosis of SS in some cases may be the beginning of an arduous journey searching for the ailment that led to SS development. This chapter will review the fundamentals of SS and focus on spinal dural defects, which have recently become a topic of interest as the precipitating condition that brings about SS.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 129.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  • Biffi A, Halpin A, Towfighi A, Gilson A, Busl K, Rost N, Smith EE, Greenberg MS, Rosand J, Viswanathan A (2010) Aspirin and recurrent intracerebral hemorrhage in cerebral amyloid angiopathy. Neurology 75(8):693–698

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Bürk K, Skalej M, Dichgans J (2001) High prevalence of CSF-containing cysts in superficial hemosiderosis of the central nervous system. J Neurol 248(11):1005–1006

    Article  PubMed  Google Scholar 

  • Charidimou A, Peeters A, Fox Z, Gregoire SM, Vandermeeren Y, Laloux P, Jäger HR, Baron JC, Werring DJ (2012) Spectrum of transient focal neurological episodes in cerebral amyloid angiopathy: multicentre magnetic resonance imaging cohort study and meta-analysis. Stroke 43(9):2324–2330

    Article  PubMed  Google Scholar 

  • Charidimou A, Boulouis G, Gurol ME, Ayata C, Bacskai BJ, Frosch MP, Viswanathan A, Greenberg SM (2017) Emerging concepts in sporadic cerebral amyloid angiopathy. Brain 140(7):1829–1850

    Article  PubMed  PubMed Central  Google Scholar 

  • Fearnley JM, Stevens JM, Rudge P (1995) Superficial siderosis of the central nervous system. Brain 118(Pt 4):1051–1066

    Article  PubMed  Google Scholar 

  • Feldman HH, Maia LF, Mackenzie IR, Forster BB, Martzke J, Woolfenden A (2008) Superficial siderosis: a potential diagnostic marker of cerebral amyloid angiopathy in Alzheimer disease. Stroke 39(10):2894–2897

    Article  PubMed  Google Scholar 

  • Fredenburg AM, Sethi RK, Allen DD, Yokel RA (1996) The pharmacokinetics and blood-brain barrier permeation of the chelators 1,2 dimethly-, 1,2 diethyl-, and 1-[ethan-1’ol]-2-methyl-3-hydroxypyridin-4-one in the rat. Toxicology 108(3):191–199

    Article  CAS  PubMed  Google Scholar 

  • Greenberg SM, Vonsattel JP, Stakes JW, Gruber M, Finklestein SP (1993) The clinical spectrum of cerebral amyloid angiopathy: presentations without lobar hemorrhage. Neurology 43(10):2073–2079

    Article  CAS  PubMed  Google Scholar 

  • Gregoire SM, Charidimou A, Gadapa N, Dolan E, Antoun N, Peeters A, Vandermeeren Y, Laloux P, Baron JC, Jäger HR, Werring DJ (2011) Acute ischaemic brain lesions in intracerebral haemorrhage: multicentre cross-sectional magnetic resonance imaging study. Brain 134(Pt 8):2376–2386

    Article  PubMed  Google Scholar 

  • Gurol ME, Becker JA, Fotiadis P, Riley G, Schwab K, Johnson KA, Greenberg SM (2016) Florbetapir-PET to diagnose cerebral amyloid angiopathy: a prospective study. Neurology 87(19):2043–2049

    Article  PubMed  PubMed Central  Google Scholar 

  • Hamill R (1908) Report of a case of melanosis of the brain, cord and meninges. J Nerv Ment Dis 594

    Google Scholar 

  • Horie T, Kajihara N, Matsumae M, Obara M, Hayashi N, Hirayama A, Takizawa K, Takahara T, Yatsushiro S, Kuroda K (2017) Magnetic resonance imaging technique for visualization of irregular cerebrospinal fluid motion in the ventricular system and subarachnoid space. World Neurosurg 97:523–531

    Article  PubMed  Google Scholar 

  • Horie T, Kajihara N, Saito H, Shibukawa S, Obara M, Ogino T, Niwa T, Kuroda K, Matsumae M (2021) Visualization of cerebrospinal fluid motion in the whole brain using three-dimensional dynamic improved motion-sensitized driven-equilibrium steady-state free precession. Magn Reson Med Sci 20(1):112–118

    Article  PubMed  Google Scholar 

  • Hoxworth JM, Trentman TL, Kotsenas AL, Thielen KR, Nelson KD, Dodick DW (2012) The role of digital subtraction myelography in the diagnosis and localization of spontaneous spinal CSF leaks. AJR Am J Roentgenol 199(3):649–653

    Article  PubMed  Google Scholar 

  • Ishibe T, Senzoku F, Kamba Y, Ikeda N, Mikawa Y (2016) Time-spatial labeling inversion pulse magnetic resonance imaging of cystic lesions of the spinal cord. World Neurosurg 88:693.e613–693.e621

    Article  Google Scholar 

  • Katoh H, Shibukawa S, Yamaguchi K, Hiyama A, Horie T, Sato M, Watanabe M (2020) A combination of magnetic resonance imaging techniques to localize the dural defect in a case of superficial siderosis—a case report. Medicines (Basel) 7(6):36

    Article  PubMed  Google Scholar 

  • Kessler RA, Li X, Schwartz K, Huang H, Mealy MA, Levy M (2018) Two-year observational study of deferiprone in superficial siderosis. CNS Neurosci Ther 24(3):187–192

    Article  CAS  PubMed  Google Scholar 

  • Koeppen AH, Hurwitz CG, Dearborn RE, Dickson AC, Borke RC, Chu RC (1992) Experimental superficial siderosis of the central nervous system: biochemical correlates. J Neurol Sci 112(1–2):38–45

    Article  CAS  PubMed  Google Scholar 

  • Kumar N (2012) Beyond superficial siderosis: introducing “duropathies”. Neurology 78(24):1992–1999

    Article  PubMed  Google Scholar 

  • Kumar N, Cohen-Gadol AA, Wright RA, Miller GM, Piepgras DG, Ahlskog JE (2006) Superficial siderosis. Neurology 66(8):1144–1152

    Article  CAS  PubMed  Google Scholar 

  • Kumar N, Lane JI, Piepgras DG (2009) Superficial siderosis: sealing the defect. Neurology 72(7):671–673

    Article  PubMed  Google Scholar 

  • Kumar S, Goddeau RP Jr, Selim MH, Thomas A, Schlaug G, Alhazzani A, Searls DE, Caplan LR (2010) Atraumatic convexal subarachnoid hemorrhage: clinical presentation, imaging patterns, and etiologies. Neurology 74(11):893–899

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Leussink VI, Flachenecker P, Brechtelsbauer D, Bendszus M, Sliwka U, Gold R, Becker G (2003) Superficial siderosis of the central nervous system: pathogenetic heterogeneity and therapeutic approaches. Acta Neurol Scand 107(1):54–61

    Article  CAS  PubMed  Google Scholar 

  • Levy M, Llinas RH (2011) Deferiprone reduces hemosiderin deposits in the brain of a patient with superficial siderosis. AJNR Am J Neuroradiol 32(1):E1–E2

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Levy M, Llinas RH (2012) Update on a patient with superficial siderosis on deferiprone. AJNR Am J Neuroradiol 33(6):E99–E100

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Levy M, Turtzo C, Llinas RH (2007) Superficial siderosis: a case report and review of the literature. Nat Clin Pract Neurol 3(1):54–58; quiz 59

    Article  PubMed  Google Scholar 

  • Linn J, Herms J, Dichgans M, Brückmann H, Fesl G, Freilinger T, Wiesmann M (2008) Subarachnoid hemosiderosis and superficial cortical hemosiderosis in cerebral amyloid angiopathy. AJNR Am J Neuroradiol 29(1):184–186

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Linn J, Halpin A, Demaerel P, Ruhland J, Giese AD, Dichgans M, van Buchem MA, Bruckmann H, Greenberg SM (2010) Prevalence of superficial siderosis in patients with cerebral amyloid angiopathy. Neurology 74(17):1346–1350

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Linn J, Wollenweber FA, Lummel N, Bochmann K, Pfefferkorn T, Gschwendtner A, Bruckmann H, Dichgans M, Opherk C (2013) Superficial siderosis is a warning sign for future intracranial hemorrhage. J Neurol 260(1):176–181

    Article  PubMed  Google Scholar 

  • Nose Y, Uwano I, Tateishi U, Sasaki M, Yokota T, Sanjo N (2022) Quantitative clinical and radiological recovery in post-operative patients with superficial siderosis by an iron chelator. J Neurol 269(5):2539–2548

    Article  CAS  PubMed  Google Scholar 

  • Scheid R, Frisch S, Schroeter ML (2009) Superficial siderosis of the central nervous system—treatment with steroids? J Clin Pharm Ther 34(5):603–605

    Article  CAS  PubMed  Google Scholar 

  • Schievink WI, Chu RM, Maya MM, Johnson JP, Cohen HC (2013) Spinal manifestations of spontaneous intracranial hypotension. J Neurosurg Spine 18(1):96–101

    Article  PubMed  Google Scholar 

  • Schievink WI, Maya MM, Moser FG, Prasad RS, Cruz RB, Nuño M, Farb RI (2019) Lateral decubitus digital subtraction myelography to identify spinal CSF-venous fistulas in spontaneous intracranial hypotension. J Neurosurg Spine 1–4

    Google Scholar 

  • Shams S, Martola J, Charidimou A, Cavallin L, Granberg T, Shams M, Forslin Y, Aspelin P, Kristoffersen-Wiberg M, Wahlund LO (2016) Cortical superficial siderosis: prevalence and biomarker profile in a memory clinic population. Neurology 87(11):1110–1117

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Sugimoto T, Kasai Y, Takegami K, Morimoto R, Maeda M, Uchida A (2005) A case of idiopathic spinal cord herniation with duplicated dura mater. J Spinal Disord Tech 18(1):106–111

    Article  PubMed  Google Scholar 

  • Sumi T, Sumi M, Van Cauteren M, Kimura Y, Nakamura T (2007) Parallel imaging technique for the external carotid artery and its branches: comparison of balanced turbo field echo, phase contrast, and time-of-flight sequences. J Magn Reson Imaging 25(5):1028–1034

    Article  PubMed  Google Scholar 

  • Toro J, Díaz C, Reyes S, Jeanneret V, Burbano LE (2014) Superficial siderosis related to a thoracic disc herniation with associated dural injury. CNS Neurosci Ther 20(5):469–472

    Article  PubMed  PubMed Central  Google Scholar 

  • Tsuchiya K, Aoki C, Hachiya J (2004) Evaluation of MR cisternography of the cerebellopontine angle using a balanced fast-field-echo sequence: preliminary findings. Eur Radiol 14(2):239–242

    Article  PubMed  Google Scholar 

  • Uttner I, Tumani H, Arnim C, Brettschneider J (2009) Cognitive impairment in superficial siderosis of the central nervous system: a case report. Cerebellum 8(1):61–63

    Article  PubMed  Google Scholar 

  • Viswanathan A, Greenberg SM (2011) Cerebral amyloid angiopathy in the elderly. Ann Neurol 70(6):871–880

    Article  CAS  PubMed  PubMed Central  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Hiroyuki Katoh .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2023 The Author(s), under exclusive license to Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Katoh, H., Watanabe, M. (2023). Superficial Siderosis. In: Gruol, D.L., Koibuchi, N., Manto, M., Molinari, M., Schmahmann, J.D., Shen, Y. (eds) Essentials of Cerebellum and Cerebellar Disorders. Springer, Cham. https://doi.org/10.1007/978-3-031-15070-8_101

Download citation

Publish with us

Policies and ethics