Epidemiological analysis of Lambert-Eaton myasthenic syndrome in Türkiye: insights from a nationwide electronic health database

IntroductionLambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction, with limited large-scale epidemiological data. In this study, we aimed to determine the epidemiological profile of LEMS in Türkiye, and to assess associated malignancies, mortality, and...

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Main Authors: Berin Inan, Bilgin Ozturk, Naim Ata, Suayip Birinci, Erdal Eroglu, Omer Karadas, Ersin Tan, Zeki Odabasi
Format: Article
Language:English
Published: Frontiers Media S.A. 2025-08-01
Series:Frontiers in Neurology
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Online Access:https://www.frontiersin.org/articles/10.3389/fneur.2025.1667540/full
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author Berin Inan
Bilgin Ozturk
Naim Ata
Suayip Birinci
Erdal Eroglu
Omer Karadas
Ersin Tan
Zeki Odabasi
author_facet Berin Inan
Bilgin Ozturk
Naim Ata
Suayip Birinci
Erdal Eroglu
Omer Karadas
Ersin Tan
Zeki Odabasi
author_sort Berin Inan
collection DOAJ
description IntroductionLambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction, with limited large-scale epidemiological data. In this study, we aimed to determine the epidemiological profile of LEMS in Türkiye, and to assess associated malignancies, mortality, and prescription rates of pyridostigmine and amifampridine.MethodsWe identified LEMS cases through a retrospective review of clinical records for individuals with a G73.1 code entry in the national healthcare database between 2015 and 2024. Confirmed cases were classified as autoimmune (A-LEMS) or paraneoplastic (P-LEMS). Demographic, clinical, and prescription data were analyzed, and incidence and prevalence rates were calculated using official census data.ResultsA total of 159 LEMS cases were confirmed. The median age at diagnosis was 60 years, and 55.3% of the patients were female. P-LEMS accounted for 59.7% of cases, with small cell lung cancer (SCLC) present in 55.8% of these. Annual incidence of LEMS ranged from 0.09 to 0.30 per million, and the overall 2024 prevalence was 1.11 per million. A-LEMS had a higher prevalence than P-LEMS in 2024, likely due to its lower mortality (23.4% vs. 58.9%). P-LEMS was more common in older males and predominantly associated with SCLC. Pyridostigmine was prescribed to 65.4% of patients, and amifampridine to 24.5%, with both treatments more frequently used in A-LEMS.DiscussionThis is the first nationwide epidemiological study of LEMS in Türkiye, revealing lower incidence and prevalence rates than in other countries. This study provides valuable large-scale epidemiological data, enriching the global understanding of this rare disorder.
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spelling doaj-art-2816f49d2e3a46c2bc83811375c3cb2b2025-08-25T05:25:33ZengFrontiers Media S.A.Frontiers in Neurology1664-22952025-08-011610.3389/fneur.2025.16675401667540Epidemiological analysis of Lambert-Eaton myasthenic syndrome in Türkiye: insights from a nationwide electronic health databaseBerin Inan0Bilgin Ozturk1Naim Ata2Suayip Birinci3Erdal Eroglu4Omer Karadas5Ersin Tan6Zeki Odabasi7Department of Neurology, Gulhane Medical Faculty, University of Health Sciences, Ankara, TürkiyeDepartment of Neurology, Gulhane Medical Faculty, University of Health Sciences, Ankara, TürkiyeGeneral Directorate of the Health Information Systems, Republic of Türkiye Ministry of Health, Ankara, TürkiyeRepublic of Türkiye Ministry of Health, Ankara, TürkiyeDepartment of Neurology, Gulhane Medical Faculty, University of Health Sciences, Ankara, TürkiyeDepartment of Neurology, Gulhane Medical Faculty, University of Health Sciences, Ankara, TürkiyeDepartment of Neurology, Faculty of Medicine, Hacettepe University, Ankara, TürkiyeDepartment of Neurology, Gulhane Medical Faculty, University of Health Sciences, Ankara, TürkiyeIntroductionLambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder of the neuromuscular junction, with limited large-scale epidemiological data. In this study, we aimed to determine the epidemiological profile of LEMS in Türkiye, and to assess associated malignancies, mortality, and prescription rates of pyridostigmine and amifampridine.MethodsWe identified LEMS cases through a retrospective review of clinical records for individuals with a G73.1 code entry in the national healthcare database between 2015 and 2024. Confirmed cases were classified as autoimmune (A-LEMS) or paraneoplastic (P-LEMS). Demographic, clinical, and prescription data were analyzed, and incidence and prevalence rates were calculated using official census data.ResultsA total of 159 LEMS cases were confirmed. The median age at diagnosis was 60 years, and 55.3% of the patients were female. P-LEMS accounted for 59.7% of cases, with small cell lung cancer (SCLC) present in 55.8% of these. Annual incidence of LEMS ranged from 0.09 to 0.30 per million, and the overall 2024 prevalence was 1.11 per million. A-LEMS had a higher prevalence than P-LEMS in 2024, likely due to its lower mortality (23.4% vs. 58.9%). P-LEMS was more common in older males and predominantly associated with SCLC. Pyridostigmine was prescribed to 65.4% of patients, and amifampridine to 24.5%, with both treatments more frequently used in A-LEMS.DiscussionThis is the first nationwide epidemiological study of LEMS in Türkiye, revealing lower incidence and prevalence rates than in other countries. This study provides valuable large-scale epidemiological data, enriching the global understanding of this rare disorder.https://www.frontiersin.org/articles/10.3389/fneur.2025.1667540/fullLambert-Eaton myasthenic syndromeepidemiologyincidenceprevalenceautoimmuneparaneoplastic
spellingShingle Berin Inan
Bilgin Ozturk
Naim Ata
Suayip Birinci
Erdal Eroglu
Omer Karadas
Ersin Tan
Zeki Odabasi
Epidemiological analysis of Lambert-Eaton myasthenic syndrome in Türkiye: insights from a nationwide electronic health database
Frontiers in Neurology
Lambert-Eaton myasthenic syndrome
epidemiology
incidence
prevalence
autoimmune
paraneoplastic
title Epidemiological analysis of Lambert-Eaton myasthenic syndrome in Türkiye: insights from a nationwide electronic health database
title_full Epidemiological analysis of Lambert-Eaton myasthenic syndrome in Türkiye: insights from a nationwide electronic health database
title_fullStr Epidemiological analysis of Lambert-Eaton myasthenic syndrome in Türkiye: insights from a nationwide electronic health database
title_full_unstemmed Epidemiological analysis of Lambert-Eaton myasthenic syndrome in Türkiye: insights from a nationwide electronic health database
title_short Epidemiological analysis of Lambert-Eaton myasthenic syndrome in Türkiye: insights from a nationwide electronic health database
title_sort epidemiological analysis of lambert eaton myasthenic syndrome in turkiye insights from a nationwide electronic health database
topic Lambert-Eaton myasthenic syndrome
epidemiology
incidence
prevalence
autoimmune
paraneoplastic
url https://www.frontiersin.org/articles/10.3389/fneur.2025.1667540/full
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