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March 16, 2026Rinsho Shinkeigaku0 citationsOpen Access

Amyloid β-related angiitis (ABRA) with dural contrast enhancement

DIDaichi ImamuraJIJunko IshiiSHSatoshi Hosoki

Key Points

  • To investigate a case of cognitive decline associated with Amyloid β-related angiitis (ABRA) and its treatment.
  • Conducted a brain biopsy for diagnosis after observing atypical dural contrast enhancement.
  • Utilized head MRI to assess brain pathology related to cognitive symptoms.
  • Monitored response to steroid therapy in managing symptoms and imaging findings.
  • Identified ABRA through brain biopsy despite atypical imaging features.
  • Observed significant improvement in cognitive function and imaging characteristics post-steroid therapy.
  • Noted inflammation in the leptomeninges extending towards brain tissue and dura.

Abstract

82歳女性.月単位で増悪していた認知機能低下が急速に進行し,頭部MRIで右頭頂葉軟膜を中心とした病変を認めた.軟膜主体の病変や脳微小出血,脳表ヘモジデローシスなどからアミロイドβ関連血管炎(amyloid β related angiitis,以下ABRAと略記)を疑ったが,硬膜造影効果が非典型であったため,脳生検を施行しABRAの診断に至った.ステロイド加療で症状・画像所見ともに改善した.本疾患の画像経過からは,軟膜にはじまる炎症が脳実質および硬膜の双方向へ進展しており,ABRAの病態に特徴的な経過を考えた.硬膜には血管炎所見は認めず,軟膜の炎症が硬膜に波及する可能性が示唆された.

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Cite This Study

Imamura et al. (2026) studied this question.

synapsesocial.com/papers/69b79dce8166e15b153ab0a7https://doi.org/10.5692/clinicalneurol.cn-002178
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