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
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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.
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