Research & Literature

The characteristics of superficial siderosis and convexity subarachnoid hemorrhage and clinical relevance in suspected cerebral amyloid angiopathy.

Ni J, Auriel E, Jindal J, et al.

Cerebrovascular diseases (Basel, Switzerland) 2015; (39(5-6)):278-86 doi:10.1159/000381223.

Cortical superficial siderosis: detection and clinical significance in cerebral amyloid angiopathy and related conditions.

Charidimou A, Linn J, Vernooij MW, et al.

Brain : a journal of neurology 2015; (138(Pt 8)):2126-39 doi:10.1093/brain/awv162.

Diffuse non-aneurysmal SAH in spontaneous intracranial hypotension: Sequela of ventral CSF leak?

Schievink WI, Maya MM

Cephalalgia : an international journal of headache 2016; (36(6)):589-92 doi:10.1177/0333102415604473.

Vestibular end organ injury induced by middle ear treatment with ferric chloride in rats.

Lee JH, Kim MS, Park BR

Human & experimental toxicology 2017; (36(2)):146-159 doi:10.1177/0960327116639365.

Deferiprone-induced agranulocytosis: 20 years of clinical observations.

Tricta F, Uetrecht J, Galanello R, et al.

American journal of hematology 2016; (91(10)):1026-31 doi:10.1002/ajh.24479.

Infratentorial superficial siderosis: Classification, diagnostic criteria, and rational investigation pathway.

Wilson D, Chatterjee F, Farmer SF, et al.

Annals of neurology 2017; (81(3)):333-343 doi:10.1002/ana.24850.

Diagnostic needs for rare diseases and shared prediagnostic phenomena: Results of a German-wide expert Delphi survey.

Blöß S, Klemann C, Rother AK, et al.

PloS one 2017; (12(2)):e0172532 doi:10.1371/journal.pone.0172532.

Superficial siderosis of the central nervous system associated with intraspinal hemorrhage from ventral thoracic epidural veins and a ventral spinal CSF leak: case report.

Takai K, Komori T, Niimura M, Taniguchi M

Journal of neurosurgery. Spine 2017; (26(6)):751-753 doi:10.3171/2016.11.SPINE16488.

A Case of Fatal Agranulocytosis That Developed in a Patient with β-Thalassemia Major Treated with Deferiprone.

Mainou M, Kotsiafti A, Klonizakis P, et al.

Hemoglobin 2016; (40(6)):435-437 doi:10.1080/03630269.2016.1276929.

Isolated spinal cord compression syndrome revealing delayed extensive superficial siderosis of the central nervous system secondary to cervical root avulsion.

Nasri A, Kacem I, Sidhom Y, et al.

The journal of spinal cord medicine 2018; (41(4)):490-495 doi:10.1080/10790268.2017.1329053.

Patterns of convexal subarachnoid haemorrhage: clinical, radiological and outcome differences between cerebral amyloid angiopathy and other causes.

Calviere L, Raposo N, Cuvinciuc V, et al.

Journal of neurology 2018; (265(1)):204-210 doi:10.1007/s00415-017-8693-7.

Two-year observational study of deferiprone in superficial siderosis.

Kessler RA, Li X, Schwartz K, et al.

CNS neuroscience & therapeutics 2018; (24(3)):187-192 doi:10.1111/cns.12792.

Spinal Cord Hemorrhage.

Shaban A, Moritani T, Al Kasab S, et al.

Journal of stroke and cerebrovascular diseases : the official journal of National Stroke Association 2018; (27(6)):1435-1446 doi:10.1016/j.jstrokecerebrovasdis.2018.02.014.

Vestibular Dysfunction in Patients With Superficial Siderosis of the Central Nervous System.

Takeda T, Kawashima Y, Hirai C, et al.

Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 2018; (39(6)):e468-e474 doi:10.1097/MAO.0000000000001844.

Focused Neuro-Otological Review of Superficial Siderosis of the Central Nervous System.

Yoo A, Jou J, Klopfenstein JD, Kattah JC

Frontiers in neurology 2018; (9()):358 doi:10.3389/fneur.2018.00358.

Bilateral Vestibulopathy in Superficial Siderosis.

Lee SY, Lee DH, Bae YJ, et al.

Frontiers in neurology 2018; (9()):422 doi:10.3389/fneur.2018.00422.

Oxidative stress and neurodegeneration: the involvement of iron.

Carocci A, Catalano A, Sinicropi MS, Genchi G

Biometals : an international journal on the role of metal ions in biology, biochemistry, and medicine 2018; (31(5)):715-735 doi:10.1007/s10534-018-0126-2.

[Superficial Siderosis].

Ohira M, Takao M

Brain and nerve = Shinkei kenkyu no shinpo 2018; (70(10)):1107-1113 doi:10.11477/mf.1416201143.

Superficial Siderosis of the Central Nervous System: Neurotological Findings Related to Magnetic Resonance Imaging.

Wagner F, Buchwalder M, Wiest R, et al.

Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 2019; (40(1)):31-37 doi:10.1097/MAO.0000000000002071.

Efficacy and safety of deferiprone for the treatment of superficial siderosis: results from a long-term observational study.

Cossu G, Abbruzzese G, Forni GL, et al.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2019; (40(7)):1357-1361 doi:10.1007/s10072-019-03847-x.

[Superficial siderosis of the central nervous system caused by myxopapillary ependymoma of conus medullaris and cauda equine: a case report and literature review].

Xu L, Hu MJ, Li YY, et al.

Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences 2019; (51(4)):769-774 doi:10.19723/j.issn.1671-167X.2019.04.031.

Lateral decubitus digital subtraction myelography to identify spinal CSF-venous fistulas in spontaneous intracranial hypotension.

Schievink WI, Maya MM, Moser FG, et al.

Journal of neurosurgery. Spine 2019; (31(6)):902-905 doi:10.3171/2019.6.SPINE19487.

Cerebral amyloid angiopathy-related transient focal neurological episodes: A transient ischemic attack mimic with an increased risk of intracranial hemorrhage.

Vales-Montero M, García-Pastor A, Iglesias-Mohedano AM, et al.

Journal of the neurological sciences 2019; (406()):116452 doi:10.1016/j.jns.2019.116452.

Risks associated with oral deferiprone in the treatment of infratentorial superficial siderosis.

Sammaraiee Y, Banerjee G, Farmer S, et al.

Journal of neurology 2020; (267(1)):239-243 doi:10.1007/s00415-019-09577-6.

Neuropathological correlates of cortical superficial siderosis in cerebral amyloid angiopathy.

Charidimou A, Perosa V, Frosch MP, et al.

Brain : a journal of neurology 2020; (143(11)):3343-3351 doi:10.1093/brain/awaa266.

Superficial siderosis of the central nervous system in a patient with asymptomatic sacral paraganglioma as source of chronic bleeding.

Prat-Acín R, Galeano-Senabre I, García-Sánchez D, et al.

Revista de neurologia 2020; (71(7)):253-256 doi:10.33588/rn.7107.2020215.

Spinal dural leaks in patients with infratentorial superficial siderosis of the central nervous system-Refinement of a diagnostic algorithm.

Friedauer L, Rezny-Kasprzak B, Steinmetz H, et al.

European journal of neurology 2022; (29(4)):1136-1144 doi:10.1111/ene.14611.

Outcomes of Cochlear Implantation in Patients with Superficial Siderosis: A Systematic Review and Narrative Synthesis.

Chaudhry A, Chaudhry D, Muzaffar J, et al.

The journal of international advanced otology 2020; (16(3)):443-455 doi:10.5152/iao.2020.9037.

Microsurgical anatomy and treatment of dural defects in spontaneous spinal cerebrospinal fluid leaks.

Matsuhashi A, Takai K, Taniguchi M

Journal of neurosurgery. Spine 2021; (34(3)):522-530 doi:10.3171/2020.6.SPINE20487.

Superficial siderosis of the central nervous system associated with ventral dural defects: bleeding from the epidural venous plexus.

Takai K, Taniguchi M

Journal of neurology 2021; (268(4)):1491-1494 doi:10.1007/s00415-020-10319-2.

Treatment Response of Deferiprone in Infratentorial Superficial Siderosis: a Systematic Review.

Flores Martin A, Shanmugarajah P, Hoggard N, Hadjivassiliou M

Cerebellum (London, England) 2021; (20(3)):454-461 doi:10.1007/s12311-020-01222-7.

Unusual Presentation in Infratentorial Superficial Siderosis: Acute Intracranial Hypertension.

Guimarães Rocha MS, Grangeiro Mirô HS, Manfroi G, et al.

Case reports in neurology 2021; (13(1)):9-16 doi:10.1159/000510847.

Susceptibility-weighted Imaging: Technical Essentials and Clinical Neurologic Applications.

Haller S, Haacke EM, Thurnher MM, Barkhof F

Radiology 2021; (299(1)):3-26 doi:10.1148/radiol.2021203071.

Superficial Siderosis: A Clinical Review.

Kumar N

Annals of neurology 2021; (89(6)):1068-1079 doi:10.1002/ana.26083.

Neuropsychological and neuroimaging characteristics of classical superficial siderosis.

Chan E, Sammaraiee Y, Banerjee G, et al.

Journal of neurology 2021; (268(11)):4238-4247 doi:10.1007/s00415-021-10548-z.

Spontaneous intracranial hypotension: diagnostic and therapeutic workup.

Luetzen N, Dovi-Akue P, Fung C, et al.

Neuroradiology 2021; (63(11)):1765-1772 doi:10.1007/s00234-021-02766-z.

Diskogenic Dural Defect Is the Reason for the Ventral Location of the Epidural Spinal Fluid Collection Seen in Superficial Siderosis.

Kumar N

Neurology. Clinical practice 2021; (11(4)):e508-e515 doi:10.1212/CPJ.0000000000001085.

Transient Focal Neurological Events in Cerebral Amyloid Angiopathy and the Long-term Risk of Intracerebral Hemorrhage and Death: A Systematic Review and Meta-analysis.

Sanchez-Caro JM, de Lorenzo Martínez de Ubago I, de Celis Ruiz E, et al.

JAMA neurology 2022; (79(1)):38-47 doi:10.1001/jamaneurol.2021.3989.

Targeted detection and repair of a spinal dural defect associated with successful biochemical resolution of subarachnoid bleeding in classical infratentorial superficial siderosis.

Lobo R, Batbayar B, Kharytaniuk N, et al.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2022; (43(9)):5643-5646 doi:10.1007/s10072-022-06181-x.

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

Kharytaniuk N, Cowley P, Sayal P, et al.

Practical neurology 2022; doi:10.1136/practneurol-2021-003324.

Health-Related Quality of Life in Adults With Classical Infratentorial Superficial Siderosis: A Cross-sectional Study.

Kharytaniuk N, Mazaheri AA, Pavlou M, et al.

Neurology 2022; (99(19)):e2201-e2211 doi:10.1212/WNL.0000000000201115.

Infratentorial Superficial Siderosis and Spontaneous Intracranial Hypotension.

Schievink WI, Maya MM, Harris J, et al.

Annals of neurology 2023; (93(1)):64-75 doi:10.1002/ana.26521.

Cerebrospinal fluid biomarkers of superficial siderosis in patients with spontaneous intracranial hypotension.

Häni L, Fung C, Jesse CM, et al.

European journal of neurology 2023; (30(1)):235-240 doi:10.1111/ene.15591.

Primary acute convexity subarachnoid hemorrhage in older patients: analysis of baseline, clinical and MRI characteristics including quantitative surface study and topographical probabilistic mapping of convexity subarachnoid hemorrhage.

Autieri V, Gaillard N, Mourand I, et al.

Acta neurologica Belgica 2023; (123(2)):519-527 doi:10.1007/s13760-022-02115-8.

CNS Superficial Siderosis Mimicking a Motor Neuron Disease.

Castro-Gomez S, Binder J, Schievelkamp AH, Heneka MT

Brain sciences 2022; (12(11)) doi:10.3390/brainsci12111558.

Cerebral Superficial Siderosis : Etiology, Neuroradiological Features and Clinical Findings.

Weidauer S, Neuhaus E, Hattingen E

Clinical neuroradiology 2023; (33(2)):293-306 doi:10.1007/s00062-022-01231-5.

Safety and effectiveness of spinal dural defect repair in the management of superficial siderosis: A systematic review and patient-level analysis.

Mariajoseph FP, Castle-Kirszbaum M, Chandra RV, et al.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2023; (109()):44-49 doi:10.1016/j.jocn.2023.01.011.

Anterior horn damage in brachial multisegmental amyotrophy with superficial siderosis and dural tear: an autopsy case report.

Takahashi Y, Kodaira M, Yamada M, et al.

BMC neurology 2023; (23(1)):129 doi:10.1186/s12883-023-03180-z.

Superficial siderosis: comparison of two cases indicates two distinct diagnostic entities. Illustrative cases.

Krishnakumar A, Ghadiyaram A, Patel VC, et al.

Journal of neurosurgery. Case lessons 2023; (5(22)).

Incidence and risk factors associated with seizures in cerebral amyloid angiopathy.

Freund BE, Sanchez-Boluarte SS, Blackmon K, et al.

European journal of neurology 2023; (30(12)):3682-3691 doi:10.1111/ene.15903.

Psychosocial impact at the time of a rare disease diagnosis.

Benito-Lozano J, Arias-Merino G, Gómez-Martínez M, et al.

PloS one 2023; (18(7)):e0288875 doi:10.1371/journal.pone.0288875.

Surgical closure of spinal cerebrospinal fluid leaks improves symptoms in patients with superficial siderosis.

El Rahal A, Haupt B, Fung C, et al.

European journal of neurology 2024; (31(3)):e16122 doi:10.1111/ene.16122.

Infratentorial superficial siderosis: report of six cases and review of the literature.

Deng L, Lin Y, Lin Y, Huang W

Frontiers in neuroscience 2024; (18()):1373358 doi:10.3389/fnins.2024.1373358.

The impact of mental health on health-related quality of life in patients with NF2-related Schwannomatosis.

Freier A, Lawson McLean AC, Loeschner D, et al.

Scientific reports 2024; (14(1)):6934 doi:10.1038/s41598-024-57401-7.

Lumbar Subarachnoid-Peritoneal Shunting Deteriorates Superficial Siderosis Associated with a Dural Defect.

Ando N, Nakazawa Y, Miyata T, et al.

Cureus 2024; (16(2)):e54651 doi:10.7759/cureus.54651.

Spinal subarachnoid hematoma involving a thoracic ventral dural defect: illustrative case.

Tanzawa A, Tateoka T, Yagi T, et al.

Journal of neurosurgery. Case lessons 2024; (8(18)).

Title not available

Cehov B, Gudmundsdottir G, Jensen J, Simonsen CZ

Ugeskrift for laeger 2024; (186(43)) doi:10.61409/V03240230.

Prevalence of infratentorial superficial siderosis in a large general population sample from the UK Biobank.

Kharytaniuk N, Hikmat D, Ozkan H, et al.

Journal of neurology 2025; (272(3)):242 doi:10.1007/s00415-025-12965-w.

Cochlear Implantation: A Treatment Approach of Hearing Loss in Superficial Siderosis.

Liaci E, Maccarrone F, Negri M, et al.

The journal of international advanced otology 2025; (21(3)):1-5 doi:10.5152/iao.2025.241682.

Digital Subtraction Myelography for the Detection of Type 1 Spinal CSF Leaks: Evaluation of Temporal Characteristics and Diagnostic Value.

Lützen N, Volz F, El Rahal A, et al.

AJNR. American journal of neuroradiology 2025; (46(12)):2665-2672 doi:10.3174/ajnr.A8847.

Audiological Progression and Treatment Outcomes in Superficial Siderosis: A 10-Year Retrospective Review of 46 Patients.

Nishio A, Kawashima Y, Takeda T, et al.

Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology 2025; (46(8)):895-902 doi:10.1097/MAO.0000000000004535.

When Blood Leaves a Trace: Diagnosing Superficial Siderosis in a Geriatric Patient.

Viju JG, Krishnan P, M A

Cureus 2025; (17(7)):e89025 doi:10.7759/cureus.89025.

Patient journey with Charcot-Marie-Tooth Disease - A German patient survey study.

Pernice HF, May S, Mühlensiepen F, et al.

Orphanet journal of rare diseases 2026; (21(1)).

Two autopsy cases of superficial siderosis: ultrastructural similarity across traumatic and non-traumatic contexts and forensic uncertainties.

Kaimori R, Sakai K, Hayata R, et al.

Legal medicine (Tokyo, Japan) 2026; (82()):102835 doi:10.1016/j.legalmed.2026.102835.

Clinical retrospective analysis of 14 cases of superficial siderosis of the central nervous system.

Ji Y, Raza HK, Xu B, et al.

BMC neurology 2026; (26(1)).

Prone Digital Subtraction Myelography vs Dynamic CT Myelography for Detecting Definite Type 1 CSF Leak in Spontaneous Intracranial Hypotension.

Ha WS, Joo H, Cho S, et al.

AJNR. American journal of neuroradiology 2026; (47(10)):2665-2673 doi:10.3174/ajnr.A9299.

Spinal CSF-Venous Fistulas as the Potential Cause of Chronic Subarachnoid Hemorrhage: Superficial Siderosis and Adhesive Arachnoiditis.

Schievink WI, Maya MM, Parikh SK, et al.

AJNR. American journal of neuroradiology 2026; (47(6)):1709-1714 doi:10.3174/ajnr.A9179.

Repercussions of Diagnostic Delay in Rare Diseases.

Batista LR, Valente LF, Spina F, et al.

Journal of genetic counseling 2026; (35(4)):e70258 doi:10.1002/jgc4.70258.