脳表ヘモジデリン沈着症のオーバビュー

• 髙尾 昌樹

  国立研究開発法人国立精神・神経医療研究センター 病院 臨床検査部・総合内科

書誌事項

タイトル別名

• Superficial hemosiderosis. An overview.

抄録

Superficial hemosiderosis is a rare neurodegenerative disorders associated with accumulation of hemosiderin on the surface of the brain and spinal cord. The Japanese guideline for the diagnosis and classification of superficial hemosiderosis divided it into three entities, according to the distribution of iron deposition and the clinical symptoms : “classical”, “localized” and “atypical” types. Individuals having classical type are supported by government in terms of medical expenses. Classical type of superficial hemosiderosis shows slowly progressive and irreversible cerebellar ataxia, sensorineural hearing loss, and/or myelopathy. MRI T2*WI or SWI demonstrates characteristic linear low intensity signals along the surface of the brain and spinal cord. In classical cases, it is important to confirm the bleeding lesions including duropathy. However, the appropriate treatment of the lesions does not always produce good clinical recoveries. In individuals of localized type, some of them are associated with cerebral amyloid angiopathy. Pathologically, severe hemosiderin deposits and tissue necrosis are present at the level of cerebellum in classical type. Hemosiderin deposits are also seen at the base of cerebrum, brainstem and spinal cord. The pharmacological treatments including hemostatics, steroids and iron chelators need to be evaluated further.

収録刊行物

• 神経治療学

  神経治療学 38 (2), 80-82, 2021

  日本神経治療学会

キーワード

• superficial siderosis
• superficial hemosiderosis
• MRI
• hemosiderin

詳細情報

• CRID: 1390571106618064512
• NII論文ID: 130008101323
• DOI: 10.15082/jsnt.38.2_80
• ISSN: 21897824
• ISSN: 09168443
• 本文言語コード: ja
• データソース種別:
  • JaLC
  • CiNii Articles
• 抄録ライセンスフラグ: 使用不可