雑誌文献を検索します。書籍を検索する際には「書籍検索」を選択してください。

検索

書誌情報 詳細検索 by 医中誌

Japanese

Creutzfeldt-Jakob Disease Aki SATO 1 1Department of Neurology, Niigata City General Hospital Keyword: プリオン病 , 皮質リボン状高信号病変 , プリオン蛋白遺伝子V180I変異 , prion disease , cortical ribboning , V180I pp.407-412
Published Date 2021/3/10
DOI https://doi.org/10.11477/mf.1436204406
  • Abstract
  • Look Inside
  • Reference

 Sporadic Creutzfeldt-Jacob disease(sCJD)is a prion disease presenting with subacute or rapidly progressive dementia with a poor prognosis. Asymmetrical cortical lesions with thalamic involvement are found in sCJD cases, which is similar distribution to status epileptics, but the lesions are not observed in the limbic systems, and they rarely occur in the precentral gyrus. Characteristically, hyperintense abnormal findings are more prominent on DWI than on FLAIR and T2WI.

 19.9% of CJD is genetic CJD(gCJD), and CJD with a mutation of codon 180 from valine to isoleucine(V180I)accounts for 40% of gCJD in Japan. Patients with this type of gCJD rarely have a family history because of the low penetration rate. The age of onset is usually later, and its clinical symptoms deteriorate more slowly than sCJD. DWI shows abnormal cortical hyperintense signals(cortical ribboning).


Copyright © 2021, Igaku-Shoin Ltd. All rights reserved.

基本情報

電子版ISSN 1882-1251 印刷版ISSN 0301-2603 医学書院

関連文献

もっと見る

文献を共有