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脑淀粉样血管病相关炎症的临床、影像及预后分析

李维 周颖 陈婵娟 谷文萍 侯德仁 薛群 谭红

李维, 周颖, 陈婵娟, 谷文萍, 侯德仁, 薛群, 谭红. 脑淀粉样血管病相关炎症的临床、影像及预后分析[J]. 分子影像学杂志, 2023, 46(5): 805-810. doi: 10.12122/j.issn.1674-4500.2023.05.05
引用本文: 李维, 周颖, 陈婵娟, 谷文萍, 侯德仁, 薛群, 谭红. 脑淀粉样血管病相关炎症的临床、影像及预后分析[J]. 分子影像学杂志, 2023, 46(5): 805-810. doi: 10.12122/j.issn.1674-4500.2023.05.05
LI Wei, ZHOU Ying, CHEN Chanjuan, GU Wenping, HOU Deren, XUE Qun, TAN Hong. Clinical, imaging and prognostic analysis of cerebral amyloid angiopathy-related inflammation[J]. Journal of Molecular Imaging, 2023, 46(5): 805-810. doi: 10.12122/j.issn.1674-4500.2023.05.05
Citation: LI Wei, ZHOU Ying, CHEN Chanjuan, GU Wenping, HOU Deren, XUE Qun, TAN Hong. Clinical, imaging and prognostic analysis of cerebral amyloid angiopathy-related inflammation[J]. Journal of Molecular Imaging, 2023, 46(5): 805-810. doi: 10.12122/j.issn.1674-4500.2023.05.05

脑淀粉样血管病相关炎症的临床、影像及预后分析

doi: 10.12122/j.issn.1674-4500.2023.05.05
基金项目: 

长沙市自然科学基金 Kq2202008

湖南省自然科学基金 2023JJ6039

湖南省自然科学基金 2020JJ8101

长沙市科技局 Kq2001004

湖南省重点研发计划 2023sk2019

详细信息
    作者简介:

    李维,硕士,主治医师,E-mail: 14726977743@163.com

    通讯作者:

    薛群,主任医师,教授,博士生导师,E-mail: qxue_sz@163.com

    谭红,主任医师,教授,硕士生导师,E-mail: tanhong1968@qq.com

Clinical, imaging and prognostic analysis of cerebral amyloid angiopathy-related inflammation

  • 摘要:   目的  探讨脑淀粉样血管病相关炎症患者临床表现、影像特点及预后。  方法  回顾性分析我医院收治的7例脑淀粉样血管病相关炎症患者的临床表现、头部MRI检查、血和/或脑脊液检验、药物治疗及预后等临床资料。  结果  临床表现:急性起病5例,亚急性起病2例;4例患者存在局灶性神经功能缺损,5例患者存在急性认知功能减退,3例患者有头痛表现,1例患者有痫性发作;所有患者均无意识障碍。头部MRI检查:7例患者磁敏感加权成像均可见皮层及皮层下大量弥漫分布的脑微出血,5例患者磁敏感加权成像发现皮质表面铁沉积,2例患者存在凸面蛛网膜下腔出血;7例患者T2WI和Flair均可见皮层及皮质下片状非对称性白质高信号,3例患者头部增强扫描见软脑膜强化,5例患者治疗后头部Flair显示皮质下白质异常信号范围缩小。血或脑脊液检验:6例患者行血载脂蛋白E基因筛查,其中ε4/ε4基因型3例,ε2/ε4基因型2例,ε3/ε4基因型1例;4例患者行脑脊液检验,其中3例患者脑脊液蛋白轻度增高,2例患者脑脊液红细胞稍高,2例患者脑脊液Aβ40和Aβ42水平下降。药物治疗:4例患者接受了糖皮质激素治疗,3例仅予以对症支持治疗。预后:4例经糖皮质激素治疗的患者其临床症状及影像均明显好转,1例患者仅予以对症支持治疗临床症状及影像学亦有改善。  结论  脑淀粉样血管病相关炎症患者主要临床特点为局灶性神经功能缺损、认知功能障碍及头痛;磁共振主要特点是皮层及皮质下片状非对称性白质高信号及脑微出血,增强扫描部分患者有软脑膜不同程度强化;糖皮质激素治疗可使多数患者获益,但部分轻症患者亦可出现自发的临床症状和影像学改善。

     

  • 图  1  7例患者头部MRI检查结果

    Figure  1.  MRI findings of the head in 7 patients. A-D: Flair showed bilateral asymmetric patchy high signals in the occipital lobe with extension to the cortex and subcortex (A), SWI showed multiple microhemorrhagic lesions in the occipital lobe bilaterally (B), enhancement scan showed no significant enhancement (C), lesions significantly decreased after treatment with oral hormones compared with before (D); E-H: Flair showed asymmetric patchy high signal in bilateral frontal lobes with extension to cortex and subcortex (E-F), and SWI showed multiple microhemorrhages, iron deposition on cortical surface, and convex subarachnoid hemorrhages at lesions (G-H); I-J: Flair showed asymmetric patchy high signal with cortical and subcortical extension in the temporo-occipital lobe on both sides (I), and SWI showed multiple microhemorrhages at the lesion (J); K-M: Flair showed bilateral asymmetric patchy high signal in the occipital lobe with extension to the cortex and subcortex (K), and SWI showed multiple microhemorrhages and a convex subarachnoid hemorrhage at the lesion (L-M); N-P: Flair showed asymmetric patchy high signal in the bilateral temporo-occipital lobe with extension to the cortex and subcortex (N), SWI showed multiple microhemorrhages at the lesion (O), and the lesion was resorbed even after symptomatic treatment (P); Q-R: Flair showed bilateral, asymmetric, patchy, high signal in the occipital lobe with extension to the cortex and subcortex (Q), and SWI showed multiple microhemorrhages at the lesion (R); S-T: Flair showed asymmetric patchy high signals with cortical and subcortical extension in the frontaland occipital lobes on both sides (S), and SWI showed multiple microhemorrhages at the lesions (T).

    表  1  7例CAA-ri患者的一般资料、临床表现及影像特点

    Table  1.   General information, clinical manifestations and imaging findings in 7 CAA-ri patients.

    Index Patient
    A B C D E F G
    Age (year) 80 70 78 68 72 65 81
    Gender Female Male Male Male Female Male Female
    Hypertension - + + + + + +
    Diabetes - - - - - - +
    Hyperlipidemia + + - + + + -
    History of ICH - - Occipital lobe - - - -
    Form of disease onset Subacute Acute acute Subacute Acute Acute Acute
    Clinical manifestations
      Focal neurological deficits + + + - - - +
      Acute cognitive decline - + + - + + +
      Epilepsy - + - - - - -
      Headache - + - + + - -
      Consciousness disorders - - - - - - -
    APOE genotype / ε4/ε4 ε2/ε4 ε4/ε4 ε4/ε4 ε3/ε4 ε2/ε4
    CMB
       < 10 - - - - - - -
       > 10 + + + + + + +
    cSS + + + + + - -
    cSAH - - + - + - -
    Asymmetric white matter hyperintensity + + + + + + +
    Mainly involved brain lobes Occipital lobe Frontal parietal Temporal occipital Parietal occipital Temporal occipital Occipital lobe Frontal occipital
    Leptomeningeal enhancement - + - + + - -
    -: No;+: Yes;/: Unknown; CAA-ri: Cerebral amyloid angiopathy-related inflammation; APOE: Apolipoprotein E; ICH: Intracerebral hemorrhage; CMB: Cerebral microbleed; cSS: Cortical superficial siderosis; cSAH: Convexal subarachnoid hemorrhage.
    下载: 导出CSV

    表  2  7例CAA-ri患者脑脊液结果

    Table  2.   Cerebrospinal fluid results in 7 CAA-ri patients

    Item Patient
    A B C D E F G
    Pressure (mmH2O) - 150 - 130 136 128 -
    WBC (×106/L) - 2 - 5 4 6 -
    RBC (×106/L) - 2 - 0 22 15 -
    Proteins (mg/L) - 470 - 620 556 730 -
    Glucose (mmol/L) - 3.6 - 4 4.2 3.8 -
    Chloride (mmol/L) - 127 - 122 128 125 -
    Aβ40 (pg/mL) - - - - 36 31 -
    Aβ42 (pg/mL) - - - - 387 412 -
    T-Tau (pg/mL) - - - - 522 530 -
    P-Tau (pg/mL) - - - - 70.2 69.7 -
    Central nerve demyelinating antibodies - Negative - Negative Negative Negative -
    Autoimmune encephalitis antibody - Negative - Negative Negative Negative -
    Virus complete test - Negative - Negative Negative Negative -
    -: Not checked; WBC: White blood cells; RBC: Red blood cells; Aβ: β-amyloid protein; Aβ40 normal reference range was 138-244 pg/mL; Aβ42 normal value was 567-1027 pg/mL; T-Tau: Total Tau protein, normal reference range was 170-512 pg/mL; P-Tau: Phosphorylated Tau protein, normal reference range was 35.84-66.26 pg/mL.
    下载: 导出CSV

    表  3  7例CAA-ri治疗方案、认知评估及影像变化

    Table  3.   Treatment options, cognitive assessment and imaging changes in 7 CAA-ri patients

    Patient Treatment MMSE MOCA CDR MRI review
    Prior treatment After treatment Prior treatment After treatment Prior treatment After treatment
    A Oral prednisone 18 22 14 15 2 1 White matter high signal reduction
    B Symptomatic treatment 15 14 10 10 2 2 White matter high signal as before
    C Oral prednisone 12 17 8 10 3 2 White matter high signal reduction
    D Ⅳ MPL 20 24 15 18 1 0.5 White matter high signal reduction
    E Symptomatic treatment 19 19 15 16 1 1 White matter high signal as before
    F Ⅳ MPL 13 20 7 15 2 1 White matter high signal reduction
    G Symptomatic treatment 16 18 13 14 2 2 White matter high signal reduction
    Ⅳ MPL: Intravenous methylprednisolone.
    下载: 导出CSV
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出版历程
  • 收稿日期:  2023-07-09
  • 网络出版日期:  2023-10-20
  • 刊出日期:  2023-09-20

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