H01846 | |
H番号 | H01846 |
名称 | 脳表ヘモジデリン沈着症 |
概要 | 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. |
カテゴリ | 神経系疾患 |
ネットワーク | - |
病因遺伝子 | - |
病原体 | - |
環境要因 | - |
発癌物質 | - |
治療薬 | - |
コメント | See also H01185 Cerebral amyloid angiopathy. |
リンク | ICD-11: 8E42 ICD-10: G96.8 MeSH: D006486 |
文献 | PMID:24670310 著者 Tosaka M, Sato K, Amanuma M, Higuchi T, Arai M, Aishima K, Shimizu T, Horiguchi K, Sugawara K, Yoshimoto Y タイトル Superficial siderosis of the central nervous system caused by hemorrhagic intraventricular craniopharyngioma: case report and literature review. 雑誌 Neurol Med Chir (Tokyo) 55:89-94 (2015) DOI:10.2176/nmc.cr.2012-0362 PMID:17205075 著者 Levy M, Turtzo C, Llinas RH タイトル Superficial siderosis: a case report and review of the literature. 雑誌 Nat Clin Pract Neurol 3:54-8; quiz 59 (2007) DOI:10.1038/ncpneuro0356 PMID:26115675 著者 Charidimou A, Linn J, Vernooij MW, Opherk C, Akoudad S, Baron JC, Greenberg SM, Jager HR, Werring DJ タイトル Cortical superficial siderosis: detection and clinical significance in cerebral amyloid angiopathy and related conditions. 雑誌 Brain 138:2126-39 (2015) DOI:10.1093/brain/awv162 |