Abstract:

Objective To study the clinical features, auxiliary examination, diagnosis and treatment, and prognosis of cerebral amyloid angiopathy-related inflammation (CAA-ri). Methods and Results Two elderly female cases with CAA-ri during February 2017 to February 2018 in our hospital were reported. Both of them manifested with cognitive impairment, gait abnormality and decreased daily life ability. Lumbar puncture of 2 cases showed the pressure of cerebrospinal fluid (CSF) was normal, white blood cell (WBC) count was generally normal and protein quantification was increased (988 and 975 mg/L). One case presented decreased CSF amyloid-β protein 40 (A β₄₀) and 42 (A β₄₂), and the other one had decreased Aβ₄₂ . One case was apolipoprotein E (ApoE) ε3/ε3, and the other was ε3/ε4. Brain MRI showed long T₁ and long T₂ abnormal signals with flaky fusion, and susceptibility-weighted imaging (SWI) showed multiple cortical and subcortical microhemorrhage. They were diagnosed as CAA-ri, and the symptoms were obviously improved after methylprednisolone implosive sequential therapy. Conclusions In addition to cognitive impairment, gait abnormality can also be the main clinical manifestation of CAA-ri. Typical imaging changes are white matter hyperintensity on T₂WI and FLAIR fusion sequences, with multiple cortical and subcortical hemorrhagic lesions on SWI. Once the typical clinical and imaging changes meet the diagnostic criteria, the diagnosis of CAA-ri can be made without biopsy. Immunotherapy is effective for most patients.

Key words: Cerebral amyloid angiopathy, Inflammation, Amyloid beta-protein, Magnetic resonance imaging

摘要：

目的 总结脑淀粉样血管病相关炎症的临床表现、辅助检查、诊断与治疗及预后特点。方法与结果 选择 2017 年 2 月至 2018 年 2 月共 2 例老年女性脑淀粉样血管病相关炎症患者，均表现为认知功能障碍、步态异常、日常生活活动能力下降；腰椎穿刺脑脊液压力和白细胞计数大致正常，蛋白定量均升高（988 和 975 mg/L），1 例β-淀粉样蛋白42和40（Aβ₄₂ 和 Aβ₄₀）下降、1 例 Aβ₄₂下降；1 例为 ApoE 基因型ε3/ε3型、1例为ε3/ε4型；MRI均可见片状融合的长T₁、长T₂ 异常信号影，磁敏感加权成像（SWI）可见多发皮质和皮质下微出血灶；均诊断为脑淀粉样血管病相关炎症；予甲泼尼龙冲击序贯治疗，症状明显改善。结论 除认知功能障碍外，步态异常也是脑淀粉样血管病相关炎症的主要临床表现，典型影像学改变是 T₂WI和 FLAIR 成像融合的白质异常高信号影，伴 SWI皮质和皮质下多发微出血灶。具有典型临床表现和影像学改变时，明确诊断脑淀粉样血管病相关炎症无须行脑组织活检术，大多数患者免疫调节治疗有效。

关键词: 脑淀粉样血管病, 炎症, 淀粉样β蛋白, 磁共振成像