{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,21]],"date-time":"2026-03-21T04:19:29Z","timestamp":1774066769990,"version":"3.50.1"},"reference-count":15,"publisher":"MDPI AG","issue":"11","license":[{"start":{"date-parts":[[2022,11,16]],"date-time":"2022-11-16T00:00:00Z","timestamp":1668556800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Alzheimer Forschung Initiative e.V.","award":["EXC2151 - 390873048"],"award-info":[{"award-number":["EXC2151 - 390873048"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>Superficial siderosis of the central nervous system (SS-CNS) is a rare condition characterized by a hemosiderin accumulation along the subpial surfaces and arises from an intermittent chronic bleeding in the subarachnoid space usually as a result of a chronic subarachnoid hemorrhage by trauma, vascular malformations, CNS tumors, or cerebral amyloid angiopathy (CAA). We present a 61-year-old male with a 12-year history of limb weakness, muscle wasting, cramps, clumsiness, progressive unsteady gait, and fine motor impairments. His medical history included the resection of a left parietal meningioma and a myxopapillary ependymoma near the conus terminalis (L3\/4) at the age of 51 years. The clinical examination revealed a motor neuron syndrome with a clear bilateral wasting of the hand muscles, a diffuse atrophy of the shoulder and calf muscles, and a weakness of the arms, fingers, hips, and feet. Deep tendon reflexes were symmetrically briskly hyperactive. Standing and walking were only possible with a support. Magnetic resonance imaging of the entire neuroaxis showed progressive severe cerebral, brainstem, and spinal superficial siderosis in form of extensive hypointensities on T2-weighted gradient-echo images and susceptibility-weighted sequences. Despite a successful neurosurgical removal of the tumors and delaed medical treatment with an iron chelator for one year, we observed no clinical recovery or stability in our patient, making this case unique, and suggesting an irreversible neurodegenerative process. This case reinforces the need of including SS-CNS in the list of amyotrophic lateral sclerosis (ALS)-mimics and demonstrates the fundamental use of a complete neuraxial MRI investigation on evaluating possible ALS cases.<\/jats:p>","DOI":"10.3390\/brainsci12111558","type":"journal-article","created":{"date-parts":[[2022,11,17]],"date-time":"2022-11-17T03:12:20Z","timestamp":1668654740000},"page":"1558","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["CNS Superficial Siderosis Mimicking a Motor Neuron Disease"],"prefix":"10.3390","volume":"12","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1581-474X","authenticated-orcid":false,"given":"Sergio","family":"Castro-Gomez","sequence":"first","affiliation":[{"name":"Department of Neurodegenerative Disease and Geriatric Psychiatry\/Neurology, University Hospital, 53127 Bonn, Germany"},{"name":"Institute of Physiology II, University Hospital Bonn, 53115 Bonn, Germany"},{"name":"Department of Neuroimmunology, Institute of Innate Immunity, University Hospital Bonn, 53127 Bonn, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julius","family":"Binder","sequence":"additional","affiliation":[{"name":"Department of Neurodegenerative Disease and Geriatric Psychiatry\/Neurology, University Hospital, 53127 Bonn, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Arndt-Hendrik","family":"Schievelkamp","sequence":"additional","affiliation":[{"name":"Department of Neuroradiology, University Hospital Bonn, 53127 Bonn, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael Thomas","family":"Heneka","sequence":"additional","affiliation":[{"name":"Luxembourg Centre for Systems Biomedicine, L-4365 Esch-sur-Alzette, Luxembourg"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,16]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1093\/brain\/118.4.1051","article-title":"Superficial Siderosis of the Central Nervous System","volume":"118","author":"Fearnley","year":"1995","journal-title":"Brain"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"1144","DOI":"10.1212\/01.wnl.0000208510.76323.5b","article-title":"Superficial Siderosis","volume":"66","author":"Kumar","year":"2006","journal-title":"Neurology"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"3210","DOI":"10.1161\/STROKEAHA.117.018974","article-title":"Prevalence and Natural History of Superficial Siderosis: A Population-Based Study","volume":"48","author":"Pichler","year":"2017","journal-title":"Stroke"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1034\/j.1600-0404.2003.02001.x","article-title":"Superficial Siderosis of the Central Nervous System: Pathogenetic Heterogeneity and Therapeutic Approaches","volume":"107","author":"Leussink","year":"2003","journal-title":"Acta Neurol Scand"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1992","DOI":"10.1212\/WNL.0b013e318259e272","article-title":"Beyond Superficial Siderosis: Introducing \u201cDuropathies","volume":"78","author":"Kumar","year":"2012","journal-title":"\u201d Neurology"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1080\/21678421.2017.1316408","article-title":"Early Diagnosis of Amyotrophic Lateral Sclerosis Mimic Syndromes: Pros and Cons of Current Clinical Diagnostic Criteria. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration","volume":"18","author":"Pradas","year":"2017","journal-title":"Amyotroph. Lateral Scler. Front. Degener."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1136\/jnnp.72.2.274-a","article-title":"Superficial Siderosis Associated with Anterior Horn Cell Dysfunction","volume":"72","author":"Turner","year":"2002","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1097\/CND.0b013e3181ca3712","article-title":"Superficial Siderosis Mimicking Amyotrophic Lateral Sclerosis","volume":"11","author":"Hoxworth","year":"2010","journal-title":"J. Clin. Neuromuscul. Dis."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1411","DOI":"10.1007\/s00701-010-0653-2","article-title":"Superficial Siderosis of the Central Nervous System: Secondary Progression despite Successful Surgical Treatment, Mimicking Amyotrophic Lateral Sclerosis. Case Report and Review","volume":"152","author":"Payer","year":"2010","journal-title":"Acta Neurochir."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1097\/NRL.0b013e318251e6d6","article-title":"Superficial Siderosis Should Be Included in the Differential Diagnosis of Motor Neuron Disease","volume":"18","author":"Kumar","year":"2012","journal-title":"Neurologist"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1487","DOI":"10.1007\/s13760-020-01489-x","article-title":"Flail Arm Syndrome Mimic Caused by Hemosiderin Deposition in the Anterior Horn","volume":"120","author":"Deguchi","year":"2020","journal-title":"Acta Neurol. Belg."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"187","DOI":"10.1111\/cns.12792","article-title":"Two-Year Observational Study of Deferiprone in Superficial Siderosis","volume":"24","author":"Kessler","year":"2018","journal-title":"CNS Neurosci. Ther."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1007\/s00401-008-0421-z","article-title":"The Pathology of Superficial Siderosis of the Central Nervous System","volume":"116","author":"Koeppen","year":"2008","journal-title":"Acta Neuropathol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1038\/s41577-018-0112-5","article-title":"Microglia Take Centre Stage in Neurodegenerative Disease","volume":"19","author":"Heneka","year":"2019","journal-title":"Nat. Rev. Immunol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1093\/jnen\/61.8.689","article-title":"Tin-Protoporphyrin Prevents Experimental Superficial Siderosis in Rabbits","volume":"61","author":"Koeppen","year":"2002","journal-title":"J. Neuropathol. Exp. Neurol."}],"container-title":["Brain Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2076-3425\/12\/11\/1558\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:19:40Z","timestamp":1760145580000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2076-3425\/12\/11\/1558"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,16]]},"references-count":15,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2022,11]]}},"alternative-id":["brainsci12111558"],"URL":"https:\/\/doi.org\/10.3390\/brainsci12111558","relation":{},"ISSN":["2076-3425"],"issn-type":[{"value":"2076-3425","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,11,16]]}}}