中国现代神经疾病杂志 ›› 2020, Vol. 20 ›› Issue (9): 760-765. doi: 10.3969/j.issn.1672-6731.2020.09.002

• 专题综述 • 上一篇    下一篇

2 脑淀粉样血管病相关炎症研究进展

张文浩, 刘冉, 黄一宁   

  1. 100034 北京大学第一医院神经内科
  • 收稿日期:2020-09-06 出版日期:2020-09-25 发布日期:2020-09-30
  • 通讯作者: 刘冉,Email:dyyxys@126.com
  • 基金资助:

    国家自然科学基金青年科学基金资助项目(项目编号:81701148)

Research progress of cerebral amyloid angiopathy associated with inflammation

ZHANG Wen-hao, LIU Ran, HUANG Yi-ning   

  1. Department of Neurology, Peking University First Hospital, Beijing 100034, China
  • Received:2020-09-06 Online:2020-09-25 Published:2020-09-30
  • Supported by:

    This study was supported by the National Natural Science Foundation of China for Young Scientists (No. 81701148).

摘要:

脑淀粉样血管病相关炎症(CAA-I)是脑淀粉样血管病的罕见亚型,逐渐受到临床医师的关注。CAA-I组织病理学兼具脑淀粉样血管病和血管炎的特点;临床主要表现为认知功能减退、癫发作、头痛等,具有较大的临床异质性;头部MRI检查是临床诊断及评估疗效的重要手段,可见T2WI和FLAIR成像脑白质高信号病灶和多发脑微出血灶;免疫抑制治疗反应良好,与脑淀粉样血管病有一定差异。本文拟就CAA-I病因和病理生理学机制、病理学特点、临床表现、辅助检查、治疗等方面研究进展进行综述。

关键词: 脑淀粉样血管病, 血管炎, 中枢神经系统, 免疫疗法, 综述

Abstract:

Cerebral amyloid angiopathy associated with inflammation (CAA-I) is a rare subtype of cerebral amyloid angiopathy (CAA), and is increasingly recognized by neurologists. CAA-I is characterized by the pathological hallmarks of both CAA and vasculitis. The main clinical manifestations contain cognitive decline, seizures, headaches and so on, which present great clinical heterogeneity. Cranial MRI plays an important role in the diagnosis and evaluation of CAA-I, by which white matter hyperintensity (WMH) and multiple cerebral microbleeds (CMBs) could be usually found. CAA-I responds well to immunosuppressive therapy. All the characteristics above are different from CAA somehow. This paper aims to review the advances in pathophysiology, pathology, clinical manifestations, laboratory examination and treatment of CAA-I.

Key words: Cerebral amyloid angiopathy, Vasculitis, central nervous system, Immunotheraphy, Review