日本兰伯特-伊顿肌无力综合征的全国性调查。

Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan.

作者信息

Yoshikawa Hiroaki, Adachi Yumi, Nakamura Yosikazu, Kuriyama Nagato, Murai Hiroyuki, Nomura Yoshiko, Sakai Yasunari, Iwasa Kazuo, Furukawa Yutaka, Kuwabara Satoshi, Matsui Makoto

机构信息

Health Service Center, Kanazawa University, Kanazawa, Ishikawa, Japan.

Public Health, Jichi Medical University, Shimotsuke, Tochigi, Japan.

出版信息

BMJ Neurol Open. 2022 Sep 5;4(2):e000291. doi: 10.1136/bmjno-2022-000291. eCollection 2022.

DOI:10.1136/bmjno-2022-000291

PMID:36110924

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9445827/

Abstract

BACKGROUND

There was no nationwide epidemiological study of Lambert-Eaton myasthenic syndrome (LEMS) in Japan; therefore, we conducted a nationwide survey.

METHODS

For the first survey, we sent survey sheets to randomly selected medical departments (n=7545) to obtain the number of LEMS who visited medical departments between 1 January 2017 and 31 December 2017. For the second survey, we sent survey sheets to the corresponding medical departments to obtain clinical information on LEMS.

RESULTS

We received 2708 responses (recovery rate: 35.9%) to the first survey. We estimated the number of LEMS as 348 (95% CI 247 to 449). The prevalence was 2.7 (95% CI 1.9 to 3.5) in 1 000 000 population. As a result of the second survey, we obtained 30 case records of 16 men and 14 women. Fourteen patients (46.7%) had a tumour, and 10 out of 14 tumours were small-cell lung carcinoma (71.4%). There was a predominance of men in the LEMS with tumour (paraneoplastic LEMS, P-LEMS) (n=11, 78.6%) and women in the LEMS without tumour (a primary autoimmune form of LEMS, AI-LEMS) (n=11, 68.8%) (p=0.0136). The onset age (mean (SD)) for the P-LEMS was 67.1 (9.0), and that for AI-LEMS was 57.8 (11.2) years old (p=0.0103). The disease duration (median) for P-LEMS was 2 years, and for AI-LEMS was 7.5 years (p=0.0134).

CONCLUSIONS

The prevalence of LEMS in Japan is similar to that in other countries. There are predominances of men in P-LEMS and women in AI-LEMS.

摘要

背景

日本尚未开展全国性的兰伯特-伊顿肌无力综合征（LEMS）流行病学研究；因此，我们进行了一项全国性调查。

方法

在第一次调查中，我们向随机抽取的医学科室（n = 7545）发送调查问卷，以获取2017年1月1日至2017年12月31日期间前往医学科室就诊的LEMS患者数量。在第二次调查中，我们向相应的医学科室发送调查问卷，以获取LEMS患者的临床信息。

结果

第一次调查共收到2708份回复（回收率：35.9%）。我们估计LEMS患者数量为348例（95%置信区间为247至449）。每100万人口中的患病率为2.7（95%置信区间为1.9至3.5）。第二次调查的结果是，我们获得了30例病例记录，其中男性16例，女性14例。14例患者（46.7%）患有肿瘤，14例肿瘤患者中有10例为小细胞肺癌（71.4%）。伴有肿瘤的LEMS（副肿瘤性LEMS，P-LEMS）中男性占优势（n = 11，78.6%），而无肿瘤的LEMS（原发性自身免疫性LEMS，AI-LEMS）中女性占优势（n = 11，68.8%）（p = 0.0136）。P-LEMS的发病年龄（均值（标准差））为67.1（9.0）岁，AI-LEMS为57.8（11.2）岁（p = 0.0103）。P-LEMS的病程（中位数）为2年，AI-LEMS为7.5年（p = 0.0134）。