Wirtz Paul W, Nijnuis Marianne G, Sotodeh Mohammad, Willems Luc N A, Brahim Joey J, Putter Hein, Wintzen Axel R, Verschuuren Jan J
Department of Neurology, J3R-166, Leiden University Medical Centre, P. O. Box 9600, The Netherlands.
J Neurol. 2003 Jun;250(6):698-701. doi: 10.1007/s00415-003-1063-7.
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.
我们在荷兰一个明确界定的地区,研究了重症肌无力(MG)和兰伯特-伊顿肌无力综合征(LEMS)的流行病学情况,以及它们与小细胞肺癌(SCLC)和胸腺瘤的关联。对1990年1月1日至1999年12月31日期间居住在南荷兰省北部地区、人口达170万的所有MG、LEMS、胸腺瘤或SCLC患者的现有数据进行了评估。共识别出202例MG患者(20例伴有胸腺瘤)和10例LEMS患者(7例伴有SCLC)。LEMS的患病率(2.32×10⁻⁶)比MG(106.1×10⁻⁶)低46倍,而LEMS的年发病率(0.48×10⁻⁶)比MG(6.48×10⁻⁶)低14倍,这反映了伴有SCLC的LEMS患者生存率较低。1593例患者被诊断为SCLC,其中7例(0.44%)出现LEMS。伴有LEMS的SCLC患者确诊时的平均年龄显著更低(p = 0.006)。32例患者被诊断为胸腺瘤,其中10例MG患者(31%)诊断胸腺瘤时的年龄比无MG的患者小(p = 0.27)。本研究证实了其他人所报道的过去几十年中MG患病率的上升,并强调了LEMS相对罕见。我们的SCLC患者中LEMS的发生率低于先前研究报道。在患有SCLC或胸腺瘤的患者中,伴有相关肌无力综合征的患者肿瘤确诊时年龄更小。