Amyotrophic lateral sclerosis in an urban setting
Amyotrophic lateral sclerosis in an urban setting
复制标题
城市环境中的肌萎缩侧索硬化症
作者：
C. Johnston;Biba R. Stanton;Biba R. Stanton;M. Turner;R. Gray;Ashley Hay;David Butt;Mary‐Ann Ampong;Christopher Shaw;Christopher Shaw;P. Leigh;P. Leigh;A. Al;A. Al
肌萎缩侧索硬化症是一种神经退行性疾病，主要影响皮层和脊髓运动神经元。之前有几项人群研究报告的发病率为0.44 - 3.2/100，000人-年[1，2]。作为在英格兰东南部建立ALS人口登记的初步步骤，我们确定了1997年1月1日至2004年7月31日期间兰贝斯、南华克和刘易舍姆（LSL）的伦敦自治市的所有流行病例。从多个来源确定了病例，以确保尽可能完整地查明，但重要的是要认识到，这一登记册涵盖的人口相对较少，进出该地区的移民率很高。确定了73名ALS患者，其中56名是新诊断的（23名女性，33名男性）。粗发病率为1.20/100，000人-年，患病率为4.06/100，000（表1）。在英格兰和威尔士，预测的年龄校正发病率为1.66/100，000人-年（95% CI 1.30-2.03），（女性1.34（95% CI 0.95-1.72），男性2.04（95% CI 1.50-2.58））。在75岁时，LSL中ALS的累积终生风险为0.15%（男性）和0.08%（女性）（相比之下，在爱尔兰登记的90岁时，其范围高达0.43%）（表2）。中位发病年龄为63岁（男性59.7岁，女性66.8岁）。60人称自己为白色（83%），11人为黑人（15%），1人为亚洲人（1%），1人下降。相比之下，根据2001年的人口普查，25%的LSL人口是黑人，4%是亚洲人[3]。当事件概率为0.25时，观察到73个事件中有11个或更少事件的二项式检验具有p=0.03，表明ALS在非洲血统的人中可能不太常见。为了标准化ALS的临床定义，世界神经病学联合会（WFN）制定并随后修订了诊断标准。为了检查这对发病率和患病率的影响，我们使用了三种替代病例定义：原始WFN标准（埃尔埃斯科里亚），修订后的WFN标准，以及仅根据原始标准分类为临床可能或明确的病例[4，5]。毫不奇怪，我们发现计算的比率几乎有两倍的变化，当只包括可能和确定的病例时最低，当使用原始标准时最高（表1）。因此，世界范围内发病率的七倍变化可能部分由不同研究的纳入方法解释，即使大多数使用WFN标准。我们的发病率在以前报道的范围内，但在大多数大型研究中处于较低水平，这也可以从终生累积风险率中看出。此外，在意大利和爱尔兰登记册中，风险随着年龄的增长而继续攀升，而在我们的数据中，85岁或以上的人没有看到这一点。这反映了该登记册的规模和LSL的人口结构，例如，只有5%的“老年"居民，而全国平均水平为7.6%。需要确定的病例较少，不确定的可能性更大，即使完全确定，发病率也取决于人口结构[6]。（表2）使用大规模人口登记的比较研究是必要的，以确定全球ALS发病率的真实变化，以确认终身风险是否如爱尔兰和意大利数据所示随着年龄的增长而继续增加，并在更大的样本中探索我们已经确定的非洲血统的可能较低的风险。为此，现在有一封欧洲致编辑的信，J Neurol（2006）253：1642-1643 DOI 10.1007/s 00415 -006-0195-y，这是及时的
Sirs: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease primarily affecting cortical and spinal motor neurons. There have been several previous population studies, reporting incidences between 0.44 and 3.2 per 100,000 person-years [1, 2]. As a preliminary step in establishing a population register for ALS in South-East England, we have identified all prevalent cases in the London Boroughs of Lambeth, Southwark and Lewisham (LSL) between 1 January 1997 and 31 July 2004. Cases were identified from multiple sources to ensure ascertainment was as complete as possible, but it is important to recognise that this register covers a relatively small population, with a high rate of migration in and out of the area. Seventy-three individuals with ALS were identified, of whom 56 were newly diagnosed (23 female, 33 male). Crude incidence was 1.20 per 100,000 person-years and prevalence 4.06 per 100,000 (Table 1). For England and Wales, the projected age-adjusted incidence rate was 1.66 per 100,000 person-years (95% CI 1.30–2.03), (females 1.34 (95% CI 0.95–1.72), males 2.04 (95% CI 1.50–2.58)). The cumulative lifetime risk of ALS in LSL was 0.15% for males and 0.08% for females by age 75, (for comparison, it ranges as high as 0.43% by age 90 in the Irish register) (Table 2). The median age of onset was 63 years (males 59.7, females 66.8). Sixty individuals described themselves as White (83%), 11 Black (15%), one Asian (1%), and one declined. For comparison, 25% of the LSL population is Black and 4% Asian according to the 2001 census[3]. A binomial test for observing 11 or fewer events out of 73 when the event probability is 0.25 has p=0.03, suggesting ALS may be less common in those with African ancestry. To standardize the clinical definition of ALS, the World Federation of Neurology (WFN) has devised and subsequently revised diagnostic criteria. To examine the effect of this on incidence and prevalence rates, we used three alternative case definitions: the original WFN criteria (El Escorial), the revised WFN criteria, and only cases classified as clinically probable or definite by the original criteria [4, 5]. Not surprisingly, we found nearly two-fold variation in the calculated rates, with the lowest when including only probable and definite cases, and the highest when the original criteria were used (Table 1). The sevenfold variation in worldwide incidence may therefore partly be explained by the inclusion methods of different studies, even though most use WFN criteria. Our incidence rates are within previously reported ranges, but are at the lower end for most large studies, as can also be seen from the lifetime cumulative risk rates. In addition, in the Italian and Irish registers, the risk continues to climb with age, whereas in our data, this is not seen for those aged 85 years or over. This reflects the size of this register and the population structure in LSL, for example having only 5% ‘‘elderly’’ residents compared with 7.6% for the national average. There are fewer cases to ascertain, underascertainment is more likely, and even with complete ascertainment, rates depend on population structure [6]. (Table 2) Comparative studies using large scale population registers are necessary to determine the true variation in the worldwide incidence of ALS, to confirm if the lifetime risk continues to increase with age as suggested by the Irish and Italian data, and to explore in a larger sample the possible lower risk for those with African ancestry that we have identified. To this end, it is timely that there is now a European LETTER TO THE EDITORS J Neurol (2006) 253: 1642–1643 DOI 10.1007/s00415-006-0195-y