天津医药

天津医药 ›› 2023, Vol. 51 ›› Issue (7): 681-686.doi: 10.11958/20230447

所属专题： 专题研究

• 专题研究·肌萎缩侧索硬化 • 上一篇    下一篇

肌萎缩侧索硬化患者临床特征分析

刘宇飞(), 朱炬, 刘娜, 任艳苹, 孙晓慧, 田丽(), 张哲成   

  1. 天津市第三中心医院神经内科（邮编300170
  • 收稿日期:2023-03-28 修回日期:2023-04-10 出版日期:2023-07-15 发布日期:2023-06-20
  • 通讯作者: △田丽 E-mail:yayatianli@163.com
  • 作者简介:刘宇飞（1990），女，主治医师，主要从事神经病学方面研究。E-mail：hanqipiaofei@126.com
  • 基金资助:
    天津市卫生健康科技项目(ZC20148)

Clinical characteristics of amyotrophic lateral sclerosis patients

LIU Yufei(), ZHU Ju, LIU Na, REN Yanping, SUN Xiaohui, TIAN Li(), ZHANG Zhecheng   

  1. Department of Neurology, Tianjin Third Central Hospital, Tianjin 300170, China
  • Received:2023-03-28 Revised:2023-04-10 Published:2023-07-15 Online:2023-06-20
  • Contact: △TIAN Li E-mail:yayatianli@163.com

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摘要：

目的 分析肌萎缩侧索硬化（ALS）患者的临床特点和流行病学现状。方法 收集ALS患者110例，男62例，女48例，中位起病年龄60岁。对其基本信息、起病部位、诊断过程、修订版ALS功能评分（ALSFRS-R）、疾病进展速率（ΔFS）、电生理及肺功能检查进行综合分析。每3~6个月通过门诊或电话随访，记录其病情变化、无创正压通气（NIPPV）、胃管、经皮胃造瘘术（PEG）的应用及利鲁唑治疗情况。结果 110例患者中肢体起病者占81.8%（90例）。诊断延迟时间为12（7，20）个月，误诊间期为11（7，17）个月，误诊率为81.3%（74例），误诊较多的为颈椎病及腰椎病。ALSFRS-R评分为39（36，45）分，ΔFS为0.6（0.3，1.0）分/月。球部起病者的确诊及拟诊比例高于肢体起病者（90% vs. 83%，χ2=2.098，P<0.05）。与快速进展者相比，中速及慢速进展者的起病年龄较轻。与慢速进展者相比，快速及中速进展者的诊断延迟时间较短（P<0.01）。81.8%（90例）的患者肌电图提示的下运动神经元损害节段大于等于临床受累节段。首次行肺功能检查提示50%（45例）的患者存在限制性通气功能障碍，用力肺活量（FVC）<70%者占17.8%（16例）。出院后利鲁唑治疗率为85.6%，NIPPV及PEG的应用率偏低。随访过程中死亡50例（45.5%），中位生存时间44.0个月；起病年龄≥60岁者的中位生存时间短于60岁以下者（36个月 vs. 50个月，P<0.05），快速进展者的生存时间短于中速及慢速进展者（27个月 vs. 32个月 vs. 65个月，P<0.01）。结论 对ALS的临床特征进行充分及正确的认识有助于为患者制定合理的诊疗方案，改善预后。

关键词: 肌萎缩侧索硬化, 临床特征, 神经电生理, 生存时间

Abstract:

Objective To analyze the clinical characteristics and epidemiological status of amyotrophic lateral sclerosis (ALS). Methods A total of 110 ALS patients (62 males and 48 females) with a median age of 60 years of onset, were collected. The basic information, site of onset, diagnostic process, modified amyotrophic lateral sclerosis function score (ALSFRS-R), rate of disease progression (ΔFS), electrophysiology and pulmonary function were analyzed comprehensively. The patients were followed up by outpatient or telephone every 3 to 6 months to record changes of their condition, the application of non-invasive positive pressure ventilation (NIPPV), gastric tube, percutaneous gastrostomy (PEG) and the treatment of riluzole. Results Among 110 patients, limb onset accounted for 90 patients (81.8%). The delay diagnosis was 12 (7, 20) months. The misdiagnosis interval was 11 (7, 17) months, and the misdiagnosis rate was 81.3% (74 cases). The most misdiagnosed cases were cervical spondylosis and lumbar spondylosis. The ALSFRS-R score was 39 (36, 45) points, and the ΔFS was 0.6 (0.3, 1.0) points/month. The proportion of confirmed and suspected diagnosis was higher in patients with ball onset than that in patients with limb onset (90% vs. 83%, χ2=2.098, P<0.05). Compared with patients of rapid disease progression, the age of onset was younger in patients with moderate and slow disease progression. Compared with patients of slow disease progression, patients of rapid and moderate disease progression had shorter diagnostic delay. Results of EMG in 90 (81.8%) patients indicated that lower motor neuron lesion segment was greater than or equal to the clinically involved segment. The first pulmonary function examination indicated that 50% (45 cases) patients had restrictive ventilation dysfunction, and the forced vital capacity (FVC)<70% accounted for 17.8% (16 cases). After discharge, the treatment rate of riluzole was 85.6%, and the application rate of NIPPV and PEG was low. During the follow-up, 50 patients (45.5%) died, and the median survival time was 44.0 months. Median survival time was shorter in patients with onset age ≥60 years than that in patients under 60 years (36 months vs. 50 months, P<0.05). Survival time was shorter in patients with rapid disease progression than that in patients with moderate and slow disease progression (27 months vs. 32 months vs. 65 months, P<0.01). Conclusion A full and correct understanding of clinical features of ALS is helpful to make a reasonable diagnosis and treatment plan for patients and improve the prognosis.

Key words: amyotrophic lateral sclerosis, clinical characteristics, neuroelectrophysiological examination, survival

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