| H01846 | |
| H number | H01846 |
| Name | Superficial siderosis |
| Description | Superficial siderosis is a rare condition caused by hemosiderin deposits in the central nervous system (CNS). Classical type of superficial siderosis primarily affects the infratentorial regions and spinal cord, and typically presents with slowly progressive sensorineural hearing impairment, cerebellar ataxia, and myelopathy. Hemosiderin deposition results from chronic intermittent or continuous minor bleeding into the subarachnoid space. The bleeding is caused by various pathologies, including tumors, meningocele and pseudomeningocele, vascular malformations, and trauma. But only less than 50% of cases have a definite bleeding source identified. A second type of brain siderosis restricted to the part of the cerebral hemispheres, has been identified and referred to as cortical superficial siderosis (CSS). CSS has a different range of potential causes and clinical presentation to classical type, but in older individuals is emerging as a key feature of cerebral amyloid angiopathy. |
| Category | Nervous system disease |
| Network | - |
| Gene | - |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | See also H01185 Cerebral amyloid angiopathy. |
| Other DBs | ICD-11: 8E42 ICD-10: G96.8 MeSH: D006486 |
| Reference | PMID:24670310 AUTHORS Tosaka M, Sato K, Amanuma M, Higuchi T, Arai M, Aishima K, Shimizu T, Horiguchi K, Sugawara K, Yoshimoto Y TITLE Superficial siderosis of the central nervous system caused by hemorrhagic intraventricular craniopharyngioma: case report and literature review. JOURNAL Neurol Med Chir (Tokyo) 55:89-94 (2015) DOI:10.2176/nmc.cr.2012-0362 PMID:17205075 AUTHORS Levy M, Turtzo C, Llinas RH TITLE Superficial siderosis: a case report and review of the literature. JOURNAL Nat Clin Pract Neurol 3:54-8; quiz 59 (2007) DOI:10.1038/ncpneuro0356 PMID:26115675 AUTHORS Charidimou A, Linn J, Vernooij MW, Opherk C, Akoudad S, Baron JC, Greenberg SM, Jager HR, Werring DJ TITLE Cortical superficial siderosis: detection and clinical significance in cerebral amyloid angiopathy and related conditions. JOURNAL Brain 138:2126-39 (2015) DOI:10.1093/brain/awv162 |