Superficial siderosis of the central nervous system

A 37-year follow-up of a case and review of the literature
  • Ines Stevens
  • Dirk Petersen
  • Wolfgang Grodd
  • Michael Poremba
  • Johannes Dichgans
Original Articles

Summary

The course of a patient suffering from superficial siderosis of the central nervous system for 37 years is presented and diagnostic and therapeutic approaches are evaluated. The syndrome is clinically defined by slowly progressing deafness, cerebellar ataxia, myelopathy and neuropsychological deficits in combination with recurrent xanthochromia of the cerebrospinal fluid with siderophages. The diagnosis may be confirmed by computed tomography, which shows degeneration of the cerebellar vermis, and by magnetic resonance imaging, demonstrating iron deposits on the surface of brain, brain stem and spinal cord. Therapy should seek to identify and remove the source of bleeding, since pharmacotherapy with iron-depleting drugs is of limited effectiveness.

Key words

Superficial siderosis Chronic subarachnoid haemorrhage Triethylenetetramine Magnetic resonance imaging 

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Copyright information

© Springer-Verlag 1991

Authors and Affiliations

  • Ines Stevens
    • 1
  • Dirk Petersen
    • 2
  • Wolfgang Grodd
    • 2
  • Michael Poremba
    • 1
  • Johannes Dichgans
    • 1
  1. 1.Department of NeurologyUniversity of TübingenTübingenGermany
  2. 2.Department of NeuroradiologyUniversity of TübingenTübingenGermany

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