Department of Neurology, Chinese PLA General Hospital, Beijing, China.
Acta Neurol Scand. 2014 Aug;130(2):111-7. doi: 10.1111/ane.12240. Epub 2014 Apr 1.
The epidemiology, diagnosis, and treatment of motor neuron disease (MND) in Chinese patients are ill known.
A registered study of 461 MND patients was conducted across 10 facilities in 7 Chinese cities from February 2009 to March 2010.
Patients were classified as amyotrophic lateral sclerosis (ALS) (84.4%), progressive bulbar palsy (PBP) (4.1%), progressive muscular atrophy (PMA) (10.4%), or primary lateral sclerosis (PLS) (0.9%). MND was predominant in men (men/women; 1.6:1.0). Mean onset age was 52.6 years, with the highest incidence being observed between 51 and 60 years. Notably, 26.0% of MND patients were employed in forestry, fishery, or animal husbandry industries. Ten cases (2.7%) reported family history of MND, and 54.2% exhibited cervical onset. MND was also associated with head/neck trauma. Non-invasive positive pressure ventilation was the most common supportive therapy.
As a novel comprehensive report of a Chinese population, this study reveals that epidemiological characteristics of MND patients were similar to those observed in international populations. MND is age-related, male gender predominant, and may be associated with both environmental and genetic risk factors.
中国人罹患运动神经元病(MND)的流行病学、诊断和治疗情况知之甚少。
2009 年 2 月至 2010 年 3 月,在中国大陆 7 个城市的 10 家医疗中心开展了一项针对 461 例 MND 患者的注册研究。
患者分为肌萎缩侧索硬化症(ALS)(84.4%)、进行性球麻痹(PBP)(4.1%)、进行性肌萎缩(PMA)(10.4%)或原发性侧索硬化症(PLS)(0.9%)。MND 以男性为主(男/女;1.6:1.0)。平均发病年龄为 52.6 岁,高发年龄为 51-60 岁。值得注意的是,26.0%的 MND 患者从事林业、渔业或畜牧业。10 例(2.7%)报告有 MND 家族史,54.2%表现为颈段起病。MND 还与头/颈部创伤相关。非侵入性正压通气是最常见的支持治疗方法。
作为一项针对中国人群的全新综合报告,本研究揭示了中国 MND 患者的流行病学特征与国际人群相似。MND 与年龄相关,以男性为主,可能与环境和遗传危险因素均相关。