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脑淀粉样血管病相关炎症6例临床诊治的回顾性分析

Analysis of Diagnosis and Treatment of 6 Cases of Cerebral Amyloid Angiopathy-related Inflammation

  • 摘要: 脑淀粉样血管病相关炎症(cerebral amyloid angiopathy-related inflammation, CAA-ri)是β淀粉样蛋白(Aβ)沉积于脑血管壁引起的炎症反应,是CAA中较为罕见的临床亚型。以头痛、癫痫发作、认知和行为改变等为主要临床表现,但无特异性。头部磁共振(MRI)提示单发或多发白质高信号病灶,磁敏感加权序列(SWI)上存在超过1处皮质或皮质下出血性病灶,包括脑出血、脑微出血、皮质表面铁沉积等。本研究回顾性分析了2017年1月至 2019年9月于我院按照2016年新修订的诊断标准诊治的6例很有可能的CAA-ri病例,发现5例患者在常规T2及FLAIR序列也能检测出微出血和皮质表面铁沉积病灶,提示如果患者病程较长、年龄较大、微出血负荷重者常规核磁检查即能发现病灶,这一特点是诊断CAA-ri的线索,临床医生遇到这一现象应该高度重视,可进一步行SWI检查验证。

     

    Abstract: Cerebral amyloid angiopathy associated with inflammation (CAA-ri) is characterized by an inflammatory response to the vascular deposits of β-amyloid within the brain that is a very rare subtype of cerebral amyloid angiopathy.The most common clinical manifestation of CAA-ri was headache, epilepsy, and cognitive dysfunction. Magnetic resonance imaging (MRI) showed focal or multiple white matter lesions, lobar intracerebral hemorrhage, extensive cortical or subcortical microbleeds. We reported 6 cases of probable CAA-ri diagnosed and treated in our hospital from January 2017 to September 2019 according to the revised diagnostic criteria in 2016.We found that 5 patients also had microbleeds and cortical superficial siderosison T2 and fluid-attenuated inversion recovery (FLAIR), suggesting that if the patients had a long course of disease, older age and heavy microbleeds load, the lesions could be found in the routine MRI, which is a clue for the diagnosis of CAA-ri. Clinicians should attach great importance to this phenomenon, and can further verify by susceptibility weighted imaging (SWI).

     

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