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  • Classical infratentorial superficial siderosis of the central nervous system: pathophysiology, clinical features and management

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Classical infratentorial superficial siderosis of the central nervous system: pathophysiology, clinical features and management
  1. Natallia Kharytaniuk1,2,3,
  2. Peter Cowley4,
  3. Parag Sayal5,
  4. Perla Eleftheriou6,
  5. Simon F Farmer7,
  6. Edgar Chan8,
  7. Doris-Eva Bamiou1,2,3,
  8. David J Werring9,10
  1. 1 UCL Ear Institute, London, UK
  2. 2 National Institute for Health Research University College London Hospitals Biomedical Research Centre, London, UK
  3. 3 Department of Neuro-otology, Royal ENT and Eastman Dental Hospitals, London, UK
  4. 4 Lysholm Department of Neuroradiology, National Hospital for Neurology and Neurosurgery, London, UK
  5. 5 Victor Horsley Department of Neurosurgery, National Hospital for Neurology and Neurosurgery, London, UK
  6. 6 Red Cell Haematology Department, University College Hospital, London, UK
  7. 7 Department of Neurology, National Hospital for Neurology and Neurosurgery, London, UK
  8. 8 Department of Neuropsychology, National Hospital for Neurology and Neurosurgery, London, UK
  9. 9 Stroke Research Centre, Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London, UK
  10. 10 Comprehensive Stroke Service, National Hospital for Neurology and Neurosurgery, Queen Square, London, UK
  1. Correspondence to Prof David J Werring, Stroke Research Centre, Department of Brain Repair and Rehabilitaiton, UCL Queen Square Institute of Neurology, London, UK; d.werring{at}ucl.ac.uk

Abstract

The term superficial siderosis (SS) is derived from the Greek word ‘sideros’, meaning iron. It includes two subtypes, distinguished by their anatomical distribution, causes and clinical features: ‘classical’ infratentorial SS (iSS, which sometimes also affects supratentorial regions) and cortical SS (cSS, which affects only supratentorial regions). This paper considers iSS, a potentially disabling disorder usually associated with very slow persistent or intermittent subarachnoid bleeding from a dural defect, and characterised by progressive hearing and vestibular impairment, ataxia, myelopathy and cognitive dysfunction. The causal dural defect—most often spinal but sometimes in the posterior fossa—typically follows trauma or neurosurgery occurring decades before diagnosis. Increasing recognition of iSS with paramagnetic-sensitive MRI is leading to an unmet clinical need. Given the diagnostic challenges and complex neurological impairments in iSS, we have developed a multidisciplinary approach involving key teams. We discuss pathophysiology, diagnosis and management of iSS, including a proposed clinical care pathway.

  • SUPERFICIAL SIDEROSIS
  • NEUROSURGERY
  • MRI

Data availability statement

No data are available.

https://doi.org/10.1136/practneurol-2021-003324

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    Data availability statement

    No data are available.

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    Footnotes

    • Contributors DJW had the idea for the article. NK wrote the first draft with extensive editing by DJW. NK and DJW prepared the figures. All authors checked the article for important intellectual content.

    • Funding This work was funded by the NIHR UCLH BRC Deafness and Hearing Problems Theme. NK’s work (doctoral studentship grant BRC-1215-20016-546624) and DEB’s time for this manuscript were funded by the NIHR UCLH BRC Deafness and Hearing Problems Theme. SFF receives funding support from the NIHR UCLH BRC. The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health. Additional support was provided by the Bernice Bibby Research Charity grant (UK Registered Charity Number 1058703).

    • Competing interests None declared.

    • Provenance and peer review Commissioned; externally peer reviewed by Michael Halmagyi, Sydney, Australia.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

    Linked Articles

    • Editors’ commentary
      Highlights from this issue
      Phil E M Smith Geraint N Fuller
      Practical Neurology 2022; 22 261-261 Published Online First: 18 Jul 2022. doi: 10.1136/practneurol-2022-003520

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