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Superfizielle Siderose des ZNS—2 Kasuistiken und eine Übersicht über die Literatur

Superficial siderosis of the CNS—two cases and a review of the literature

  • Ergebnisse und Kasuistik
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Zusammenfassung

Die superfizielle Siderose des ZNS ist ein seltenes Krankheitsbild, bei dem es durch chronisch rezidivierende Subarachnoidalblutungen (SAB) zu oberflächlichen Ablagerungen von Hämosiderin an Großhirn, Kleinhirn und Rückenmark kommt. Bekannte Blutungsquellen sind ZNS-Tumoren, Gefäßmalformationen, Nervenwurzelläsionen und neurochirurgische Eingriffe. Der Blutungsquellennachweis gelingt nur in etwa 50% der Fälle. Klinisch kommt es zu progredientem sensoneuralem Hörverlust, zerebellärer Ataxie und Pyramidenbahnläsionen. Im Liquor können Ferritin und freies Eisen erhöht sein. Die kraniale und spinale Kernspintomographie erlaubt den sensitiven und spezifischen Nachweis der Hämosiderinablagerungen. Einzig die Elimination der Blutungsquelle scheint die Progression der meist irreversiblen Symptomatik verhindern zu können. Daher sollte frühzeitig und gründlich nach der Blutungsquelle gesucht werden. Es werden 2 Kasuistiken vorgestellt und eine Übersicht über die Literatur gegeben.

Summary

Superficial siderosis of the CNS is a rare disease. The superficial deposition of haemosiderin in the cerebrum, cerebellum and spinal cord is due to chronic and recurrent subarachnoidal haemorrhage (SAH). Known sources of bleeding are vascular CNS-tumours, CSF-cavity lesions, vascular malformations, nerve root lesions and neurosurgical interventions. Detection of the source of bleeding is successful in only about 50% of cases. The clinical syndrome is characterized by sensorineural deafness, cerebellar ataxia and pyramidal signs. CSF-investigation might be indicative for SAH, while ferritin and ionic iron can be elevated in the CSF. CT is unspecific and insensitive but MR imaging of the brain and spinal cord is very sensitive and specific. The elimination of the source of bleeding alone might prevent the progression of the disease, therefore, an early and extensive search for this source is highly recommended.

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Literatur

  1. Iwanowski L, Olszewski J (1960) The effects of subarachnoid injections of iron-containing substances on the central nervous system. J Neuropathol Exp Neurol 19: 433–448

    CAS  Google Scholar 

  2. Koeppen AH, Dickson AC, Chu RC, Thach RE. (1993) The pathogenesis of superficial siderosis of the central nervous system. Ann Neurol 34: 646–653

    CAS  PubMed  Google Scholar 

  3. Koeppen AH, Hurwitz CG, Dearborn RE et al. (1992) Experimental superficial siderosis of the central nervous system: biochemical correlates. J Neurol Sci 112: 38–45

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  5. Koeppen AH, Dentinger MP (1988) Brain hemosiderin and superficial siderosis of the central nervous system. J Neuropathol Exp Neurol 47: 249–270

    CAS  PubMed  Google Scholar 

  6. Revesz T, Earl CJ, Barnard RO (1988) Superficial siderosis of the central nervous system presenting with longstanding deafness. J R Soc Med 81: 479–481

    CAS  PubMed  Google Scholar 

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

    Google Scholar 

  8. Tomlinson BE, Walton JN (1964) Superficial haemosiderosis of the central nervous system. J Neurol Neurosurg Psychiatry 27: 332–339

    CAS  Google Scholar 

  9. 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: S51–56

    Google Scholar 

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

    PubMed  Google Scholar 

  11. Schievink WI, Apostolides PJ, Spetzler RF (1998) Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation. Case report. J Neurosurg 89: 1029–1031

    CAS  PubMed  Google Scholar 

  12. Matsumoto S, Kang Y, Sato S et al. (1998) Spinal meningeal melanocytoma presenting with superficial siderosis of the central nervous system. Case report and review of the literature. J Neurosurg 88: 890–894

    CAS  PubMed  Google Scholar 

  13. Haroun RI, Li KW, Rigamonti D (2000) Surgical resection of a cerebral arteriovenous malformation for treatment of superficial siderosis: case report. Surg Neurol 53: 554–558

    Article  CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  15. Bostantjopoulou S, Katsarou Z, Pigadas A, Kazis A (2000) Superficial CNS siderosis and spinal pilocytic astrocytoma. Neurology 55: 450

    CAS  PubMed  Google Scholar 

  16. Straube A, Dudel C, Wekerle G, Klopstock T (2001) Polyradiculopathy in the course of superficial siderosis of the CNS. J Neurol 248: 63–64

    Article  CAS  PubMed  Google Scholar 

  17. Manfredi M, De Togni L, Beltramello A (2000) Superficial siderosis of the central nervous system in a patient with neurofibromatosis type I. Eur Neurol 43: 121–122

    Article  CAS  PubMed  Google Scholar 

  18. Mascalchi M, Salvi F, Pirini MG et al. (1999) Transthyretin amyloidosis and superficial siderosis of the CNS. Neurology 53: 1498–1503

    CAS  PubMed  Google Scholar 

  19. Kato K, Tomura N, Takahashi S et al. (1998) Superficial siderosis appeared in a case of suprasellar embryonal carcinoma. No To Shinkei 50: 936–940

    CAS  PubMed  Google Scholar 

  20. Manfredi M, Magni E, Gandolfini M et al. (1999) Superficial siderosis of the central nervous system and anticoagulant therapy: a case report. Ital J Neurol Sci 20: 247–249

    Article  CAS  PubMed  Google Scholar 

  21. D'Antonio V, Brancatelli G, Banco A, Montalbano E (1999) Superficial siderosis of the central nervous system associated with Escherichia coli meningoencephalitis. A case report. Radiol Med (Torino) 98: 520–522

    Google Scholar 

  22. Kumar A, Aggarwal S, Willinsky R, TerBrugge KG (1993) Posterior fossa surgery: an unusual cause of superficial siderosis. Neurosurgery 32: 455–457

    CAS  PubMed  Google Scholar 

  23. Bracchi M, Savoiardo M, Triulzi F et al. (1993) Superficial siderosis of the CNS: MR diagnosis and clinical findings. Am J Neuroradiol 14: 227–236

    CAS  PubMed  Google Scholar 

  24. Uchino A, Aibe H, Itoh H, Aiko Y, Tanaka M (1997) Superficial siderosis of the central nervous system. Its MRI manifestations. Clin Imaging 21: 241–245

    Article  CAS  PubMed  Google Scholar 

  25. Parnes SM, Weaver SA (1992) Superficial siderosis of the central nervous system: a neglected cause of sensorineural hearing loss. Otolaryngol Head Neck Surg 107: 69–77

    CAS  PubMed  Google Scholar 

  26. River Y, Honigman S, Gomori JM, Reches A (1994) Superficial hemosiderosis of the central nervous system. Mov Disord 9: 559–562

    CAS  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: 57–60

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Abteilung Neurologie der Neurologischen Klinik, Universitätsklinikum Heidelberg, Heidelberg

    A. Hug

  2. Abteilung Neuroradiologie der Neurologischen Klinik, Universitätsklinikum Heidelberg, Heidelberg

    M. Hartmann

  3. Neurologische Klinik, Klinikum Ludwigshafen

    A. Grau

  4. Neurologische Klinik, Universität Heidelberg, Im Neuenheimer Feld 400, 69120 , Heidelberg

    A. Hug

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  1. A. Hug
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  3. A. Grau
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Correspondence to A. Hug.

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Hug, A., Hartmann, M. & Grau, A. Superfizielle Siderose des ZNS—2 Kasuistiken und eine Übersicht über die Literatur. Nervenarzt 75, 584–588 (2004). https://doi.org/10.1007/s00115-003-1559-5

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