[1]李　娟　章　洁　姜　辉.肌萎缩侧索硬化症存活20年1例报道并文献复习[J].中国临床神经外科杂志,2021,26(03):173-175.[doi:10.13798/j.issn.1009-153X.2021.03.010]
　LI Juan,ZHANG Jie,JIANG Hui.Experience in treating amyotrophic lateral sclerosis surviving for 20 years: one case report and literature review[J].,2021,26(03):173-175.[doi:10.13798/j.issn.1009-153X.2021.03.010]
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肌萎缩侧索硬化症存活20年1例报道并文献复习()
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《中国临床神经外科杂志》[ISSN:1009-153X/CN:42-1603/TN]

卷:
26
期数:
2021年03期
页码:
173-175
栏目:
论著
出版日期:
2021-03-25

文章信息/Info

Title:
Experience in treating amyotrophic lateral sclerosis surviving for 20 years: one case report and literature review
文章编号:
1009-153X（2021）03-0173-03
作者:
李　娟　章　洁　姜　辉
430070 武汉，中国人民解放军中部战区总医院干部病房一科（李　娟、章　洁、姜　辉）
Author(s):
LI Juan;  ZHANG Jie;  JIANG Hui
Department of First Cadre Ward, General Hospital, Central theater Command, PLA, Wuhan 430070, China
关键词:
肌萎缩侧索硬化症; 运动神经元病; 个体化治疗
Keywords:
Amyotrophic lateral sclerosis;  Motor neuron disease;  Individualized treatment
分类号:
R 746.4
DOI:
10.13798/j.issn.1009-153X.2021.03.010
文献标志码:
A
摘要:
目的 总结长期存活的肌萎缩侧索硬化症（ALS）的治疗经验。方法 回顾性分析2001年10月收治1例ALS的临床资料，从发病至今已近20年，结合相关文献，总结治疗经验。结果 病人已存活20年，期间发生肺炎4次、泌尿系统感染5次均治愈，未发生深静脉血栓形成与压疮。目前病人呈醒状昏迷，营养状况良好，全身瘫痪，依赖呼吸机维持呼吸，无呼吸道和泌尿系统感染。结论 ALS发病机制复杂，早期症状不典型，易误诊，目前尚无特异性治疗措施，实施个体化对症支持治疗，提前做好防治影响病人生命主要并发症的医疗护理措施是延长病人生命的关键。做好营养支持、预防深静脉血栓形成及压疮、预防呼吸道和泌尿系统感染等对减少病人各种并发症、延长生命具有重要意义。
Abstract:
Objective To summarize the experience in treating the long-term survival patients with amyotrophic lateral sclerosis (ALS). Methods The clinical data of one patient with ALS who was admitted to our hospital in October 2001 were analyzed retrospectively. The relevant literature was reviewed and the treatment experience was summarized. Results The patient has survived for 20 years since the onset of the disease. During the period, pneumonia occurred in the patient for 4 times and urinary tract infection for 5 times, and all of these infections were cured. No deep vein thrombosis and pressure sores occurred. At present, the patient is in coma vigil with good nutritional status, general paralysis and dependence on ventilator to maintain breathing, and without respiratory and urinary tract infections. Conclusions The pathogenesis of ALS is complex. ALS is easy to misdiagnose due to its early symptoms are not typical. There is no specific treatment measures for ALS so far. The keys to prolong the life time of the patients with ALS are to implement individualized symptomatic support treatment in the later stage, and prevent and treat the main complications affecting the life time of the patients. Nutritional support, prevention of deep venous thrombosis and pressure sores, prevention of respiratory and urinary tract infections are of great significance to reduce various complications and prolong the life time of the patients with ALS.

参考文献/References:

[1] Mitchell JD, Borasio GD. Amyotrophic lateral sclerosis [J].Lancet, 2007, 369(9578): 2031-2041.
[2] 包雅琳，曹秉振，陈　琳，等. 中国肌萎缩侧索硬化诊断和治疗指南[J]. 中华神经外科杂志，2012，45（7）：531-533.
[3] Miller’s RG, Jackson CE, Kasarskis EJ, et al. Practiceparameter update：the care of the patient with amyotrophic lateral sclerosis：multidisciplinary care，symptom manage-ment，and cognitive/behavioral impairment (an evidence-based review) [J]. Neurology, 2009, 73(15): 1227-1233.
[4] 张　艳，陈向荣，彭叶叶，等. 1例存活19年老年肌萎缩侧索硬化症的护理[J]. 中国临床神经外科杂志，2020，25（10）：719-721.
[5] Van Rheenen W, Shatunov A, Dekker AM, et al. Genome-wide association analyses identify new risk variants and the genetic architecture of amyotrophic lateral sclerosis [J]. Nat Genet, 2016, 48(11): 1043-1048.
[6] 陈君逸，刘向一，徐迎胜，等. HSPB1 基因罕见变异可能与肌萎缩侧索硬化发病相关[J]. 南方医科大学学报，2021，41（1）：75-78.
[7] Vendredy L, Adriaenssens E, Timmerman V. Small heat shock proteins in neurodegenerative diseases [J]. Cell Stress Chaperones, 2020, 25(4): 679-699.
[8] Muranova LK, Sudnitsyna MV, Strelkov SV, et al. Mutations in HspB1 and hereditary neuropathies [J]. Cell Stress Chaperones, 2020, 25(4): 655-665.

相似文献/References:

[1]张　艳　陈向荣　彭叶叶　章　洁.1例存活19年老年肌萎缩侧索硬化症的护理[J].中国临床神经外科杂志,2020,(10):719.[doi:10.13798/j.issn.1009-153X.2020.10.022]
[2]李娟,姜辉,章洁,等.个体化老年综合评估技术在1例存活20年的肌萎缩侧索硬化症中的应用[J].中国临床神经外科杂志,2023,28(02):99.[doi:10.13798/j.issn.1009-153X.2023.02.011]
　LI Juan,ZHANG Jie,JIANG Hui,et al.Application of individualized comprehensive geriatric assessment techniques in an elderly patient with amyotrophic lateral sclerosis surviving 20 years[J].,2023,28(03):99.[doi:10.13798/j.issn.1009-153X.2023.02.011]

备注/Memo

备注/Memo:
通讯作者：姜　辉，E-mail：jianghui430070@sina.com
更新日期/Last Update: 2021-03-25