TY - JOUR
T1 - Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan
AU - Yoshikawa, Hiroaki
AU - Adachi, Yumi
AU - Nakamura, Yosikazu
AU - Kuriyama, Nagato
AU - Murai, Hiroyuki
AU - Nomura, Yoshiko
AU - Sakai, Yasunari
AU - Iwasa, Kazuo
AU - Furukawa, Yutaka
AU - Kuwabara, Satoshi
AU - Matsui, Makoto
N1 - Funding Information:
This study was supported (in part) by a Health and Labour Sciences Research Grant on Rare and Intractable Diseases (Validation of Evidence-based Diagnosis and Guidelines, and Impact on QOL in Patients with Neuroimmunological Diseases) from the Ministry of Health, Labour and Welfare of Japan (Grant number: 20FC1030).
Publisher Copyright:
© 2022 Author(s) (or their employer(s)).
PY - 2022/9/5
Y1 - 2022/9/5
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85138190891&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85138190891&partnerID=8YFLogxK
U2 - 10.1136/bmjno-2022-000291
DO - 10.1136/bmjno-2022-000291
M3 - Article
AN - SCOPUS:85138190891
SN - 2632-6140
VL - 4
JO - BMJ Neurology Open
JF - BMJ Neurology Open
IS - 2
M1 - e000291
ER -