Skip to main content
Log in

Superficial siderosis of the central nervous system: secondary progression despite successful surgical treatment, mimicking amyotrophic lateral sclerosis. Case report and review

  • Case Report
  • Published:
Acta Neurochirurgica Aims and scope Submit manuscript

Abstract

Superficial siderosis of the central nervous system is a rare disorder with hemosiderin deposition in the spinal and cranial leptomeninges and subpial layer, mostly from repetitive subarachnoid hemorrhage. Progressive sensorineural deafness, cerebellar ataxia, and pyramidal signs comprise the typical clinical presentation. We describe a 47-year-old patient, who showed initial 2-year improvement after successful occlusion of an intradural bleeding source at T4. Secondary progression of symptoms without further bleedings was noted thereafter, with a clinical picture of amyotrophic lateral sclerosis. This case illustrates that the disease may progress secondarily even without re-bleedings, and that secondary progression might be due to a similar pathomechanism as in amyotrophic lateral sclerosis.

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

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7
Fig. 8
Fig. 9

References

  1. Aquilina K, Kumar R, Lu J, Rawluk D (2005) Superficial siderosis of the central nervous system following cervical nerve root avulsion: the importance of early diagnosis and surgery. Acta Neurochir (Wien) 147(3):291–297

    Article  CAS  Google Scholar 

  2. Bonito V, Agostinis C, Ferraresi S, Defanti CA (1994) Superficial siderosis of the central nervous system after brachial plexus injury. Case report. J Neurosurg 80(5):931–934

    Article  PubMed  CAS  Google Scholar 

  3. Bracchi M, Savoiardo M, Triulzi F, Daniele D, Grisoli M, Bradac GB, Agostinis C, Pelucchetti D, Scotti G (1993) Superficial siderosis of the CNS: MR diagnosis and clinical findings. AJNR 14(1):227–236

    PubMed  CAS  Google Scholar 

  4. Brooks BR, Miller RG, Swash M, Munsat TL (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1(5):293–299

    Article  PubMed  CAS  Google Scholar 

  5. Cohen-Gadol A, Krauss W, Spinner R (2004) Delayed central nervous system superficial siderosis following brachial plexus avulsion injury. Report of three cases. Neurosurg Focus 16(5):E10

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  7. Haroun R, Li K, Rigamonti D (2000) Surgical resection of a cerebral arteriovenous malformation for treatment of superficial siderosis: case report. Surg Neurolo 53(6):554–558

    Article  CAS  Google Scholar 

  8. Hug A, Hartmann M, Grau A (2004) Superficial siderosis of the CNS. 2 cases and a review of the literature. Nervenarzt 75(6):584–588

    Article  PubMed  CAS  Google Scholar 

  9. Koeppen A, Dentiger M (1988) Brain hemosiderin and superficial siderosis of the central nervous system. J Neuropathol Exp Neurol 47(3):249–270

    Article  PubMed  CAS  Google Scholar 

  10. Koeppen A, Borke R (1991) Experimental superficial siderosis of the central nervous system. I. Morphological observations. J Neuropathol Exp Neurol 50(5):579–594

    Article  PubMed  CAS  Google Scholar 

  11. Kole M, Steven D, Kirk A, Lownie S (2004) Superficial siderosis of the central nervous system from a bleeding pseudomeningocele. Case illustration. J Neurosurg 100(4):718

    Article  PubMed  Google Scholar 

  12. Konya D, Peker S, Ozgen S, Kurtkaya O, Necmettin Pamir M (2006) Superficial siderosis due to papillary glioneuronal tumor. J Clin Neurosci 13(9):950–952

    Article  PubMed  Google Scholar 

  13. Lévêque M, Mc Laughlin N, Bojanowski MW (2009) Secondary superficial siderosis of the central nervous system: report of three cases. Neurochirurgie 55(3):315–321

    Article  PubMed  Google Scholar 

  14. 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

    Article  PubMed  Google Scholar 

  15. Li KW, Haroun RI, Clatterbuck RE, Murphy K, Rigamonti D (2001) Superficial siderosis associated with multiple cavernous malformations: report of three cases. Neurosurgery 48(5):1147–1150, discussion 1150–1

    Article  PubMed  CAS  Google Scholar 

  16. Matsumoto S, Kang Y, Sato S, Kawakami Y, Oda Y, Araki M, Kawamura J, Uchida H (1998) Spinal meningeal melanocytoma presenting with superficial siderosis of the central nervous system. Case report and review of the literature. J Neurosurg 88(5):890–894

    Article  PubMed  CAS  Google Scholar 

  17. Messori A, De Bella P, Herber N, Loqullo F, Ruggiero M, Salvolini U (2004) The importance of suspecting superficial siderosis of the central nervous system in clinical practice. J Neurol Neurosurg Psychiatry 75(2):188–190

    Article  PubMed  CAS  Google Scholar 

  18. Miliaras G, Bostantjopoulou S, Argyropoulou M, Kyritsis A, Polyzoidis K (2006) Superficial siderosis of the CNS: report of three cases and review of the literature. Clin Neurol Neurosurg 108(5):499–502, Review

    Article  PubMed  Google Scholar 

  19. Nozaki K, Inomoto T, Takagi Y, Hashimoto N (2003) Spinal intradural extramedullary cavernous angioma. Case report. J Neurosurg 99(3 Suppl):316–319

    PubMed  Google Scholar 

  20. Offenbacher H, Fazekas F, Schmidt R, Kapeller P, Fazekas G (1996) Superficial siderosis of the central nervous system: MRI findings and clinical significance. Neuroradiology 38(Suppl 1):S51–S56

    Article  PubMed  Google Scholar 

  21. Pyhtinen J, Paakko E, Ilkko E (1995) Superficial siderosis in the central nervous system. Neuroradiology 37(2):127–128

    Article  PubMed  CAS  Google Scholar 

  22. River Y, Honigmann S, Gomori J, Reches A (1994) Superficial hemosiderosis of the central nervous system. Mov Disord 9(5):559–562

    Article  PubMed  CAS  Google Scholar 

  23. Roncaroli F, Scheithauer BW, Krauss WE (1999) Hemangioma of spinal nerve root. J Neurosurg 91(2 Suppl):175–180

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  25. Schievink W, Apostolides P, Spetzler R (1998) Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation. Case report. J Neurosurg 89(6):1029–1031

    Article  PubMed  CAS  Google Scholar 

  26. Shih P, Yang BP, Batjer HH, Liu JC (2009) Surgical management of superficial siderosis. Spine J 9(8):e16–e19

    Article  PubMed  Google Scholar 

  27. Stevens I, Petersen D, Grodd W, Poremba M, Dichgans J (1991) Superficial siderosis of the central nervous system. A 37-year follow-up of a case and review of the literature. Eur Arch Psychiatry Clin Neurosci 241(1):57–60

    Article  PubMed  CAS  Google Scholar 

  28. Turner B, Wills AJ (2002) Superficial siderosis associated with anterior horn cell dysfunction. J Neurol Neurosurg Psychiatry 72(2):274–275

    Article  PubMed  CAS  Google Scholar 

Download references

Conflict of interest

There is no conflict of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Michael Payer.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Payer, M., Sottas, C. & Bonvin, C. Superficial siderosis of the central nervous system: secondary progression despite successful surgical treatment, mimicking amyotrophic lateral sclerosis. Case report and review. Acta Neurochir 152, 1411–1416 (2010). https://doi.org/10.1007/s00701-010-0653-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00701-010-0653-2

Keywords

Navigation