Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Case Study
  • Published:

Superficial siderosis: a case report and review of the literature

Abstract

Background A 48-year-old man with a past history of a car-accident-induced coma at the age of 3 years and spontaneous bilateral subdural hematomas at the age of 34 years presented with a 2-year history of worsening gait difficulties, clumsiness and dysarthria. CT scans of the brain taken during the 6 months leading up to his presentation had revealed chronic subarachnoid hemorrhage, but angiograms had not been able to identify the source of bleeding.

Investigations Neurological examination, brain MRI scan, lumbar puncture, four-vessel cerebral angiogram and spinal angiogram.

Diagnosis Superficial siderosis; a complete review of the current literature on the condition is provided.

Management Iron chelating agents.

This is a preview of subscription content, access via your institution

Access options

Rent or buy this article

Prices vary by article type

from$1.95

to$39.95

Prices may be subject to local taxes which are calculated during checkout

Figure 1: MRI brain images of the patient showing features characteristic of superficial siderosis.

References

  1. Hamill R (1908) Report of a case of melanosis of the brain, cord and meninges. J Nerv Ment Dis 35: 594

    Article  Google Scholar 

  2. Rosenthal P (1958) Siderosis of the marginal zones of the central nervous system [German]. Dtsch Z Nervenheilkd 178: 431–472

    Article  CAS  PubMed  Google Scholar 

  3. Cammermeyer J (1947) Deposition of iron in paraventricular areas of the human brain in hemochromatosis. J Neuropathol Exp Neurol 6: 111–127

    Article  Google Scholar 

  4. Noetzel H (1940) Diffusion of blood pigment in the inner border area and outer surface of the CNS after subarachnoidal hemorrhage [German]. Arch Psychol Nervenkr 111: 129–138

    Article  Google Scholar 

  5. Koeppen AH and Borke RC (1991) Experimental superficial siderosis of the central nervous system. I: morphological observations. J Neuropathol Exp Neurol 50: 579–594

    Article  CAS  PubMed  Google Scholar 

  6. Koeppen AH et al. (1992) Experimental superficial siderosis of the central nervous system: biochemical correlates. J Neurol Sci 112: 38–45

    Article  CAS  PubMed  Google Scholar 

  7. Koeppen AH et al. (1993) The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol 34: 646–653

    Article  CAS  PubMed  Google Scholar 

  8. Fearnley JM et al. (1995) Superficial siderosis of the central nervous system. Brain 118: 1051–1066

    Article  PubMed  Google Scholar 

  9. Gomori JM et al. (1987) High-field spin-echo MR imaging of superficial and subependymal siderosis secondary to neonatal intraventricular hemorrhage. Neuroradiology 29: 339–342

    Article  CAS  PubMed  Google Scholar 

  10. Offenbacher H et al. (1991) Superficial siderosis of the spinal cord: a rare cause of myelopathy diagnosed by MRI. Neurology 41: 1987–1989

    Article  CAS  PubMed  Google Scholar 

  11. Pinkston JW et al. (1983) Superficial siderosis: a cause of leptomeningeal enhancement on computed tomography. J Comput Assist Tomogr 7: 1073–1076

    Article  CAS  PubMed  Google Scholar 

  12. Kumar N et al. (2006) Superficial siderosis. Neurology 66: 1144–1152

    Article  CAS  PubMed  Google Scholar 

  13. River Y et al. (1994) Superficial hemosiderosis of the central nervous system. Mov Disord 9: 559–562

    Article  CAS  PubMed  Google Scholar 

  14. McCarron MO and Patterson VH (2004) Effects of shunting CSF in superficial siderosis of the CNS. Neurology 62: 1022–1023

    Article  PubMed  Google Scholar 

  15. Angstwurm K et al. (2002) Superficial siderosis of the central nervous system: response to steroid therapy. J Neurol 249: 1223–1225

    Article  CAS  PubMed  Google Scholar 

  16. Koeppen AH and Dickson AC (2002) Tin-protoporphyrin prevents experimental superficial siderosis in rabbits. J Neuropathol Exp Neurol 61: 689–701

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

We would like to thank the following people for translating case reports: Dr Peter Kaplan for French and Italian, Madeleine Rogatinsky for French, Paula Schwarz for Spanish, Dr Kristine Altman for Finnish, Dr Amitabh Gupta for German, Kazumi Schwarz for Japanese, and Dr Amit Hoke for Turkish. Dr Kathleen Burns assisted with the final editing.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Michael Levy.

Ethics declarations

Competing interests

The authors declare no competing financial interests.

Supplementary information

Supplementary Table 1

Details of 270 published cases of superficial siderosis (DOC 618 kb)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Levy, M., Turtzo, C. & Llinas, R. Superficial siderosis: a case report and review of the literature. Nat Rev Neurol 3, 54–58 (2007). https://doi.org/10.1038/ncpneuro0356

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1038/ncpneuro0356

This article is cited by

Search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing