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学科主题: 临床医学
题名:
Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China
作者: Chen, Lu1; Zhang, Bin1; Chen, Ru2; Tang, Lu1; Liu, Rong1; Yang, Yan1; Yang, Yi1; Liu, Xiaolu1; Ye, Shan1; Zhan, Siyan2; Fan, Dongsheng1
刊名: JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY
发表日期: 2015-10-01
DOI: 10.1136/jnnp-2015-310471
卷: 86, 期:10, 页:1075-1081
收录类别: SCI
文章类型: Review
WOS标题词: Science & Technology
类目[WOS]: Clinical Neurology ; Psychiatry ; Surgery
研究领域[WOS]: Neurosciences & Neurology ; Psychiatry ; Surgery
关键词[WOS]: PROGNOSTIC-FACTORS ; PESTICIDE EXPOSURE ; RISK-FACTOR ; FLAIL ARM ; ALS ; SURVIVAL ; SMOKING ; EPIDEMIOLOGY ; VENTILATION ; ONSET
英文摘要:

Objectives To describe the natural history and clinical features of sporadic amyotrophic lateral sclerosis (ALS) in Chinese patients, and to report data on the prognostic factors for survival.

Methods All patients referred to our ALS centre between 2003 and 2012 were followed up every 3 months. Survival and tracheotomy were predefined as primary outcome measures. Group differences were analysed using parametric and non-parametric tests as appropriate. Survival was analysed using the Kaplan-Meier method and Cox regression analysis.

Results Of the 1624 patients with ALS, 75.1% had limb-onset, 14.0% had bulbar-onset, 7.8% had flail-arm syndrome (FAS), 2.6% had progressive muscular atrophy and 0.5% had primary lateral sclerosis. The male: female ratio was 1.7: 1, and the mean age at onset was 49.8 years. The median diagnostic delay was 14 months, and the median survival time after symptom onset was 71 months. Male gender, older age at symptom onset, lower body mass index, shorter diagnostic delay, bulbaronset ALS phenotype, higher Airlie House category at presentation, rural place of residence, use of traditional Chinese medicine and a history of contact with pesticides were associated with poorer survival, whereas female gender or an FAS phenotype may have a better prognosis.

Conclusions The clinical characteristics and outcomes of Chinese patients with sporadic ALS were different compared with patients from other countries. Compared with other studies, the age at onset of Chinese patients was earlier, the percentage of bulbar-onset ALS was lower and the prognosis was better. This study substantially advances the understanding of the clinical features and epidemiology of this rare disease.

语种: 英语
所属项目编号: 81030019
项目资助者: National Natural Science Foundation of China
WOS记录号: WOS:000361154800006
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/64692
Appears in Collections:北京大学第三临床医学院_神经内科_期刊论文

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作者单位: 1.Peking Univ, Hosp 3, Dept Neurol, Beijing 100191, Peoples R China
2.Peking Univ, Sch Publ Hlth, Dept Epidemiol & Biostat, Hlth Sci Ctr, Beijing 100191, Peoples R China

Recommended Citation:
Chen, Lu,Zhang, Bin,Chen, Ru,et al. Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China[J]. JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY,2015,86(10):1075-1081.
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