{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,26]],"date-time":"2026-02-26T14:00:21Z","timestamp":1772114421643,"version":"3.50.1"},"reference-count":10,"publisher":"Springer Science and Business Media LLC","issue":"9","license":[{"start":{"date-parts":[[2022,6,13]],"date-time":"2022-06-13T00:00:00Z","timestamp":1655078400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,6,13]],"date-time":"2022-06-13T00:00:00Z","timestamp":1655078400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100012317","name":"UCLH Biomedical Research Centre","doi-asserted-by":"publisher","award":["BRC-1215-20016-546624"],"award-info":[{"award-number":["BRC-1215-20016-546624"]}],"id":[{"id":"10.13039\/501100012317","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neurol Sci"],"published-print":{"date-parts":[[2022,9]]},"abstract":"<jats:title>Abstract\n<\/jats:title><jats:sec>\n                <jats:title>Background and importance\u00a0\u00a0<\/jats:title>\n                <jats:p>Classical infratentorial superficial siderosis (iSS) is characterised by repeated insidious bleeding into the subarachnoid space, leading to haemosiderin deposition within the subpial layers of the brainstem, cerebellum and spinal cord, sometimes with supratentorial involvement. Although nearly always associated with a dural defect (usually from previous trauma or neurosurgery) there is little evidence to support definitive investigation and management strategies. Here, we present a novel investigation strategy to identify a dural defect and subsequent successful surgical repair with biochemical resolution of subarachnoid bleeding.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Clinical presentation<\/jats:title>\n                <jats:p>A 55-year-old gentleman presented with a 15-year progressive history of sensorineural deafness, followed by a slowly worsening gait ataxia. He had previously sustained cranio-spinal trauma. On examination there were features of myelopathy and ataxia. MRI demonstrated classical iSS, affecting cerebellum and cerebral cortices, with a cervicothoracic epidural CSF collection. Lumbar puncture (LP) revealed elevated ferritin 413\u00a0ng\/mL and red cell count of 4160. Reverse CT myelography, a novel technique involving contrast injection into the collection, delineated a dural defect at the T9\/T10 level that was not present on conventional myelography. Following surgical repair, repeat LP twelve months later demonstrated biochemical improvement (ferritin 18\u00a0ng\/mL, red cells\u2009&lt;\u20091). There was no further neurological deterioration in symptoms during eighteen months follow-up.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusion<\/jats:title>\n                <jats:p>We show the value of a rational targeted investigation pathway in identifying a surgically reparable dural defect underlying classical iSS. We also provide proof of concept that surgical repair can facilitate biochemical resolution of subarachnoid bleeding and might prevent progression of neurological disability.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s10072-022-06181-x","type":"journal-article","created":{"date-parts":[[2022,6,13]],"date-time":"2022-06-13T03:29:52Z","timestamp":1655090992000},"page":"5643-5646","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Targeted detection and repair of a spinal dural defect associated with successful biochemical resolution of subarachnoid bleeding in classical infratentorial superficial siderosis"],"prefix":"10.1007","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0090-3379","authenticated-orcid":false,"given":"Rhannon","family":"Lobo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bilguun","family":"Batbayar","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Natallia","family":"Kharytaniuk","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Cowley","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Parag","family":"Sayal","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5193-8195","authenticated-orcid":false,"given":"Simon","family":"Farmer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2074-1861","authenticated-orcid":false,"given":"David J.","family":"Werring","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,6,13]]},"reference":[{"key":"6181_CR1","doi-asserted-by":"publisher","unstructured":"Fearnley JM, Stevens JM, Rudge P. Review article: superficial siderosis of the central nervous system. Brain. Published online 1995. https:\/\/doi.org\/10.1093\/brain\/118.4.1051","DOI":"10.1093\/brain\/118.4.1051"},{"key":"6181_CR2","doi-asserted-by":"publisher","unstructured":"Wilson D, Chatterjee F, Farmer SF, et al. Infratentorial superficial siderosis: classification, diagnostic criteria, and rational investigation pathway. Ann Neurol. Published online 2017. https:\/\/doi.org\/10.1002\/ana.24850","DOI":"10.1002\/ana.24850"},{"key":"6181_CR3","doi-asserted-by":"publisher","unstructured":"Kumar N, Cohen-Gadol AA, Wright RA, Miller GM, Piepgras DG, Ahlskog JE. Superficial siderosis. Neurology. Published online 2006. https:\/\/doi.org\/10.1212\/01.wnl.0000208510.76323.5b","DOI":"10.1212\/01.wnl.0000208510.76323.5b"},{"key":"6181_CR4","doi-asserted-by":"publisher","unstructured":"Levy M, Turtzo C, Llinas RH. Superficial siderosis: a case report and review of the literature. Nat Clin Pract Neurol. Published online 2007. https:\/\/doi.org\/10.1038\/ncpneuro0356","DOI":"10.1038\/ncpneuro0356"},{"key":"6181_CR5","doi-asserted-by":"publisher","unstructured":"Cummins G, Crundwell G, Baguley D, Lennox G. Treatment of superficial siderosis with iron chelation therapy. BMJ Case Rep. Published online 2013. https:\/\/doi.org\/10.1136\/bcr-2013-009916","DOI":"10.1136\/bcr-2013-009916"},{"key":"6181_CR6","doi-asserted-by":"publisher","unstructured":"Kessler RA, Li X, Schwartz K, Huang H, Mealy MA, Levy M. Two-year observational study of deferiprone in superficial siderosis. CNS Neurosci Ther. Published online 2018. https:\/\/doi.org\/10.1111\/cns.12792","DOI":"10.1111\/cns.12792"},{"key":"6181_CR7","doi-asserted-by":"publisher","unstructured":"Kumar N, Lane JI, Piepgras DG. Superficial siderosis: sealing the defect. Neurology. Published online 2009. https:\/\/doi.org\/10.1212\/01.wnl.0000342457.22536.af","DOI":"10.1212\/01.wnl.0000342457.22536.af"},{"key":"6181_CR8","doi-asserted-by":"publisher","unstructured":"Flores Martin A, Shanmugarajah P, Hoggard N, Hadjivassiliou M. Treatment response of deferiprone in infratentorial superficial siderosis: a systematic review. The Cerebellum. Published online January 6, 2021. https:\/\/doi.org\/10.1007\/s12311-020-01222-7","DOI":"10.1007\/s12311-020-01222-7"},{"key":"6181_CR9","doi-asserted-by":"publisher","unstructured":"Boncoraglio GB, Ballabio E, Erbetta A, Prada F, Savoiardo M, Parati EA. Superficial siderosis due to dural defect with thoracic spinal cord herniation. J Neurol Sci. Published online 2012. https:\/\/doi.org\/10.1016\/j.jns.2011.07.034","DOI":"10.1016\/j.jns.2011.07.034"},{"key":"6181_CR10","doi-asserted-by":"publisher","unstructured":"Egawa S, Yoshii T, Sakaki K, et al. Dural closure for the treatment of superficial siderosis: report of 2 cases. J Neurosurg Spine. Published online 2013. https:\/\/doi.org\/10.3171\/2013.1.SPINE12649","DOI":"10.3171\/2013.1.SPINE12649"}],"container-title":["Neurological Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10072-022-06181-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10072-022-06181-x\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10072-022-06181-x.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,8,17]],"date-time":"2022-08-17T04:12:17Z","timestamp":1660709537000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10072-022-06181-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,6,13]]},"references-count":10,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2022,9]]}},"alternative-id":["6181"],"URL":"https:\/\/doi.org\/10.1007\/s10072-022-06181-x","relation":{},"ISSN":["1590-1874","1590-3478"],"issn-type":[{"value":"1590-1874","type":"print"},{"value":"1590-3478","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,6,13]]},"assertion":[{"value":"18 April 2022","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"26 May 2022","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 June 2022","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Formal written consent was sought from the patient prior to the write-up and submission of the manuscript.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval"}},{"value":"The authors declare no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"Informed consent was sought and obtained from the patient prior to write-up and submission of the\u00a0manuscript.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Informed consent"}},{"value":"The views expressed are those of the authors and not necessarily those of the NHS, the NIHR or the Department of Health.","order":5,"name":"Ethics","group":{"name":"EthicsHeading","label":"Disclaimer"}}]}}