Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan

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 me...

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Main Authors: Yutaka Furukawa, Satoshi Kuwabara, Nagato Kuriyama, Yoshiko Nomura, Yasunari Sakai, Makoto Matsui, Yosikazu Nakamura, Hiroyuki Murai, Yumi Adachi, Hiroaki Yoshikawa, Kazuo Iwasa
Format: Article
Language:English
Published: BMJ Publishing Group 2022-08-01
Series:BMJ Neurology Open
Online Access:https://neurologyopen.bmj.com/content/4/2/e000291.full
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author Yutaka Furukawa
Satoshi Kuwabara
Nagato Kuriyama
Yoshiko Nomura
Yasunari Sakai
Makoto Matsui
Yosikazu Nakamura
Hiroyuki Murai
Yumi Adachi
Hiroaki Yoshikawa
Kazuo Iwasa
author_facet Yutaka Furukawa
Satoshi Kuwabara
Nagato Kuriyama
Yoshiko Nomura
Yasunari Sakai
Makoto Matsui
Yosikazu Nakamura
Hiroyuki Murai
Yumi Adachi
Hiroaki Yoshikawa
Kazuo Iwasa
author_sort Yutaka Furukawa
collection DOAJ
description 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.
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spelling doaj.art-0cee86ab7a774249a6bd5e99775ad7bc2023-07-21T08:30:07ZengBMJ Publishing GroupBMJ Neurology Open2632-61402022-08-014210.1136/bmjno-2022-000291Nationwide survey of Lambert-Eaton myasthenic syndrome in JapanYutaka Furukawa0Satoshi Kuwabara1Nagato Kuriyama2Yoshiko Nomura3Yasunari Sakai4Makoto Matsui5Yosikazu Nakamura6Hiroyuki Murai7Yumi Adachi8Hiroaki Yoshikawa9Kazuo Iwasa10Department of Neurology and Neurobiology of Aging, Kanazawa University, Kanazawa, Ishikawa, JapanDepartment of Neurology, Graduate School of Medicine, Chiba University, Chiba, JapanDepartment of Social Health Medicine, Shizuoka Graduate University of Public Health, Shizuoka, JapanPediatric Neurology, Yoshiko Nomura Neurological Clinic for Children, Tokyo, JapanDepartment of Pediatrics, Kyushu University, Fukuoka, JapanNeurology, Kanazawa Medical University, Kahoku-gun, Ishikawa, JapanPublic Health, Jichi Medical University, Shimotsuke, Tochigi, JapanDepartment of Neurology, International University of Health and Welfare, Narita, JapanHealth Service Center, Kanazawa University, Kanazawa, Ishikawa, JapanHealth Service Center, Kanazawa University, Kanazawa, Ishikawa, JapanHealth Science, Ishikawa Prefectural Nursing University, Kahoku-gun, Ishikawa, JapanBackground 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.https://neurologyopen.bmj.com/content/4/2/e000291.full
spellingShingle Yutaka Furukawa
Satoshi Kuwabara
Nagato Kuriyama
Yoshiko Nomura
Yasunari Sakai
Makoto Matsui
Yosikazu Nakamura
Hiroyuki Murai
Yumi Adachi
Hiroaki Yoshikawa
Kazuo Iwasa
Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan
BMJ Neurology Open
title Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan
title_full Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan
title_fullStr Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan
title_full_unstemmed Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan
title_short Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan
title_sort nationwide survey of lambert eaton myasthenic syndrome in japan
url https://neurologyopen.bmj.com/content/4/2/e000291.full
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