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肌萎缩侧索硬化的临床分型、分期及病情评估

李晓光 刘明生 崔丽英

李晓光, 刘明生, 崔丽英. 肌萎缩侧索硬化的临床分型、分期及病情评估[J]. 协和医学杂志, 2018, 9(1): 69-74. doi: 10.3969/j.issn.1674-9081.2018.01.013
引用本文: 李晓光, 刘明生, 崔丽英. 肌萎缩侧索硬化的临床分型、分期及病情评估[J]. 协和医学杂志, 2018, 9(1): 69-74. doi: 10.3969/j.issn.1674-9081.2018.01.013
Xiao-guang LI, Ming-sheng LIU, Li-ying CUI. Clinical Phenotype Classification, Staging and Prognostic Evaluation of Amyotrophic Lateral Sclerosis[J]. Medical Journal of Peking Union Medical College Hospital, 2018, 9(1): 69-74. doi: 10.3969/j.issn.1674-9081.2018.01.013
Citation: Xiao-guang LI, Ming-sheng LIU, Li-ying CUI. Clinical Phenotype Classification, Staging and Prognostic Evaluation of Amyotrophic Lateral Sclerosis[J]. Medical Journal of Peking Union Medical College Hospital, 2018, 9(1): 69-74. doi: 10.3969/j.issn.1674-9081.2018.01.013

肌萎缩侧索硬化的临床分型、分期及病情评估

doi: 10.3969/j.issn.1674-9081.2018.01.013
详细信息
    通讯作者:

    崔丽英 电话:010-69156371, E-mail:pumchcuily@sina.com

  • 中图分类号: R746.4

Clinical Phenotype Classification, Staging and Prognostic Evaluation of Amyotrophic Lateral Sclerosis

More Information
  • 摘要: 肌萎缩侧索硬化(amyotrophic lateral sclerosis, ALS)是一种进展性神经系统变性疾病。其起病隐袭, 进展缓慢, 因呼吸衰竭死亡, 目前尚无治愈方法。近年来研究表明多种措施可延长患者存活期, 提高患者生活质量。为了科学合理地治疗ALS, 本文就其临床分型、分期、治疗模式及对ALS病情进展的评估和随访方法进行介绍。
  • 表  1  肌萎缩侧索硬化临床表型与存活预测[6]

    临床表型 各型所占比例(%) 发病年龄(岁) 诊断延迟(月) 合并FTD比例(%) 中位存活时间(95% CI,年) 存活率[%(标准误]
    3年 5年 10年
    经典型ALS 30.33 62.8 10.9 4.0 2.6(2.0~3.0) 43.3(0.025) 24.2(0.022) 13.0(0.020)
    延髓型ALS 33.33 68.8 9.8 9.0 2.0(1.9~2.2) 21.9(0.020) 8.9(0.014) 3.4(0.010)
    连枷臂综合征 5.56 62.6 12.8 1.4 4.0(3.3~5.0) 62.9(0.058) 38.5(0.058) 17.4(0.050)
    连枷腿综合征 12.99 65.0 13.1 4.1 3.0(2.5~3.4) 49.1(0.039) 25.1(0.033) 12.8(0.030)
    锥体束征型ALS 9.01 58.3 15.9 2.5 6.3(4.0~8.7) 70.8(0.042) 55.3(0.046) 31.9(0.050)
    呼吸型ALS 1.05 62.2 6.4 - 1.4(1.0~2.1) 22.0(0.100) 6.7(0.064) -
    纯下运动神经元综合征 2.85 56.2 15.5 - 7.3(4.7~10.3) 85.7(0.059) 65.6(0.081) 36.6(0.086)
    纯上运动神经元综合征 3.98 58.9 15.9 3.8 13.1(12.2~15.0) 96.1(0.027) 90.3(0.004) 71.1(0.070)
    平均 64.3 10.8 5.4 2.5(2.3~2.6) 39.9(0.014) 22.1(0.012) 11.4(0.010)
    FTD:额颞叶痴呆
    下载: 导出CSV

    表  2  肌萎缩侧索硬化伦敦分期与生存期

    分期 最近一次记录情况 平均生存时间(月) 5年生存率(%)
    球部发病 肢体发病 球部发病 肢体发病
    1期 出现症状(第一区域受累) - - - -
    2期A 确诊 19 59 20.5 49.9
    2期B 第二区域受累 19 28 17.5 29.0
    3期 第三区域受累 13 13 9.8 12.3
    4期A 需经皮胃肠造瘘 9 6 6.4 4.2
    4期B 需无创通气 3 8 5.9 6.0
    5期 死亡或机械通气 - - - -
    下载: 导出CSV

    表  3  肌萎缩侧索硬化米兰都灵分期评分[8]

    域(项目) 评分及标准 域功能评分
    活动
     行走 4分.正常 0
    3分.行走稍困难 0
    2分.搀扶下可步行 0
    1分.只有行走动作但不能移动 1
    0分.缺乏有目的的腿部活动 1
     自理 4分.正常 0
    3分.可独立完成,但费力或效率低 0
    2分.需间歇协助或用替代方法 0
    1分.需他人协助 1
    0分.完全依赖他人 1
    吞咽 4分.正常饮食 0
    3分.饮食稍有问题,偶有呛咳 0
    2分.饮食习惯变化 0
    1分.需鼻饲辅助 1
    0分.不能经口进食,完全肠外或肠内营养 1
    交流
     言语 4分.正常言语 0
    3分.可察觉言语欠清晰 0
    2分.复述后可理解 0
    1分.加入非语音性交流方式 1
    0分.言语丧失 1
     书写 4分.正常 0
    3分.慢或杂乱,所有字清晰可辨 0
    2分.非所有字均清晰可辨 0
    1分.能握住笔,但不能写字 1
    0分.握不住笔 1
    呼吸
     困难 4分.无 0
    3分.行走时发生 0
    2分.吃饭、洗澡或穿戴时发生 0
    1分.休息时发生,坐或躺时均呼吸困难 1
    0分.显著困难,考虑使用机械通气支持 1
     功能不全 4分.无 0
    3分.间歇使用NIPPV 0
    2分.夜间连续使用NIPPV 0
    1分.日夜连续使用NIPPV 1
    0分.气管切开或插管,侵入性机械通气 1
    下载: 导出CSV
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  • 收稿日期:  2017-07-04
  • 刊出日期:  2018-01-30

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