The epidemiology of myasthenia gravis, Lambert-Eaton myasthenic syndrome and their associated tumours in the northern part of the province of South Holland
The epidemiology of myasthenia gravis, Lambert-Eaton myasthenic syndrome and their associated tumours in the northern part of the province of South Holland
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DOI：
10.1007/s00415-003-1063-7
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发表时间：
2003-06-01
影响因子：
6
通讯作者：
Verschuuren, JJ
中科院分区：
文献类型：
--
作者：
Wirtz, PW;Nijnuis, MG;Verschuuren, JJ
我们在荷兰一个明确的地区研究了重症肌无力（MG）和Lambert-Eaton肌无力综合征（LEMS）的流行病学，以及它们与小细胞肺癌（SCLC）和胸腺瘤的关系。对1990年1月1日至1999年12月31日期间生活在南荷兰北方地区的所有MG、LEMS、胸腺瘤或SCLC患者的现有数据进行了评价，该地区人口为170万。共确定了202例MG患者（20例胸腺瘤）和10例LEMS患者（7例SCLC）。LEMS的患病率（2.32 x 10（-6））比MG（106.1 x 10（-6））低46倍，而LEMS的年发病率（0.48 x 10（-6））比MG（6.48 x 10（-6））低14倍，反映了LEMS伴SCLC患者的生存率低。在1593例患者中诊断出SCLC，其中7例（0.44%）发展为LEMS。诊断为SCLC的平均年龄在伴有LEMS的SCLC患者中显著较低（p = 0.006）。在32名患者中诊断出胸腺瘤，其中10名MG患者（31%）在诊断胸腺瘤时的年龄比无MG的患者更小（p = 0.27）。这项研究证实了过去几十年来MG的患病率不断增加，并强调了LEMS的相对罕见性。在我们的小细胞肺癌患者中LEMS的发生率低于以前的研究报告。在患有小细胞肺癌或胸腺瘤的患者中，患有相关肌无力综合征的患者在年轻时就被诊断出肿瘤。
We studied the epidemiology of myasthenia gravis (MG) and the Lambert-Eaton myasthenic syndrome (LEMS), and their association with small cell lung carcinoma (SCLC) and thymoma, in a well defined region of the Netherlands. Available data on all the patients with MG, LEMS, thymoma or SCLC living between 1 January 1990 and 31 December 1999 in the northern region of South Holland, with a population of 1.7 million inhabitants, were evaluated. A total of 202 patients with MG (20 with thymoma) and ten patients with LEMS (seven with SCLC) were identified. LEMS was 46 times less prevalent (2.32 x 10(-6)) than MG (106.1 x 10(-6)), whereas the annual incidence rate of LEMS was 14 times lower (0.48 x 10(-6)) than of MG (6.48 x 10(-6)), reflecting the poor survival of LEMS patients with SCLC. SCLC was diagnosed in 1593 patients, seven (0.44 %) of whom developed LEMS. Mean age at diagnosis of SCLC was significantly lower in SCLC patients with LEMS (p = 0.006). A thymoma was diagnosed in 32 patients, of whom the ten patients with MG (31 %) had a younger age at diagnosis of thymoma than the patients without MG (p = 0.27). This study confirms the increasing prevalence of MG over the last few decades as reported by others, and underscores the relative rarity of LEMS. The frequency of LEMS in our patients with SCLC was lower than reported in previous studies. In patients with a SCLC or thymoma, the tumour was diagnosed at younger age in those who had the associated myasthenic syndrome.