The Superficial Siderosis charity

Superficial siderosis research update

 

 

The rarity of classical infratentorial superficial siderosis means that the research opportunities are few and limited in participant numbers. We are delighted to announce that two teams (from University College London, headed by Professor Werring) and the Perspectum Group) have joined their efforts and are preparing a research project aiming to measure the levels of brain iron using magnetic resonance imaging (M

 

 

 

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Classical infratentorial superficial siderosis results from chronic low-volume continuous or intermittent bleeding into the cerebrospinal fluid (CSF) that bathes the brain and the spinal cord. The iron (in the form of hemosiderin) from the broken-down red cells in the CSF accumulates in the superficial layers of the brain and can sometimes also involve the spinal cord. This iron can be seen on magnetic resonance imaging (MRI) especially the iron-sensitive types of MRI. While the iron can be seen on MRI, researchers and clinicians to date have not been able to reliably measure it on imaging, despite several attempts by different groups around the world.

 

We are delighted to announce that our research team led by Professors Karen Shmueli and David Werring at the Queen Square Institute of Neurology, University College London, and the team at Perspectum, led by Susannah Krzyzanowski, are preparing a joint research project to study the iron levels in the brain, as seen on MRI, using dedicated software tools. We hope to be able to accurately measure the iron so that the technique can be used in other clinical and research centres from around the world. We also want to find out if the iron levels, as seen on MRI, correspond with the clinical picture, that is, whether heavy iron deposits on MRI mean that the individual is likely to be more affected by the infratentorial superficial siderosis.

 

The study is in its preparation stages and we hope to rule it out in the second half of 2024. The study recruitment is limited to UK participants only. If you would like to find out more about it or express your interest in taking part, please do not hesitate to contact the Perspectum team.

Email:

 

If you are interested in reading more about the work of our research team led by Professor Werring at the UCL Queen Square Institute of Neurology please visit our webpages:https://www.ucl.ac.uk/stroke/patients-carers-and-public/infratentorial-superficial-siderosis-central-nervous-system

https://www.ucl.ac.uk/stroke/research

 

You can also email Dr Natallia Kharytaniuk about the research updates from our team: n.kharytaniuk@ucl.ac.uk

 

REFERENCES

Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway. Wilson D, Chatterjee F, Farmer SF, Rudge P, McCarron MO, Cowley P, Werring DJ. Ann Neurol 2017;81(3):333-43. doi: 10.1002/ana.24850 [published Online First: 2016/12/27] Link

Classical infratentorial superficial siderosis of the central nervous system: pathophysiology, clinical features and management. Kharytaniuk N, Cowley P, Sayal P, Eleftheriou P, Farmer SF, Chan E, Bamiou DE, Werring DJ. Pract Neurol. 2022 Jul 11:practneurol-2021-003324. doi: 10.1136/practneurol-2021-003324. Link

Kessler RA, Li X, Schwartz K, Huang H, Mealy MA, Levy M. Two-year observational study of deferiprone in superficial siderosis. CNS Neurosci Ther. 2018 Mar;24(3):187-192. doi: 10.1111/cns.12792. Epub 2017 Dec 28. PMID: 29285884; PMCID: PMC6489821. Link

 

Nose Y, Uwano I, Tateishi U, Sasaki M, Yokota T, Sanjo N. Quantitative clinical and radiological recovery in post-operative patients with superficial siderosis by an iron chelator. J Neurol. 2022 May;269(5):2539-2548. doi: 10.1007/s00415-021-10844-8. Epub 2021 Oct 18. PMID: 34664101. Link