Inan Berin, Ozturk Bilgin, Ata Naim, Birinci Suayip, Eroglu Erdal, Karadas Omer, Tan Ersin, Odabasi Zeki
Department of Neurology, Gulhane Medical Faculty, University of Health Sciences, Ankara, Türkiye.
General Directorate of the Health Information Systems, Republic of Türkiye Ministry of Health, Ankara, Türkiye.
Front Neurol. 2025 Aug 25;16:1667540. doi: 10.3389/fneur.2025.1667540. eCollection 2025.
Lambert-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.
We 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.
A 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.
This 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.
兰伯特-伊顿肌无力综合征（LEMS）是一种罕见的神经肌肉接头自身免疫性疾病，大规模流行病学数据有限。在本研究中，我们旨在确定土耳其LEMS的流行病学特征，并评估相关恶性肿瘤、死亡率以及吡啶斯的明和氨吡啶的处方率。
我们通过回顾性审查2015年至2024年国家医疗数据库中具有G73.1编码条目的个体的临床记录来识别LEMS病例。确诊病例分为自身免疫性（A-LEMS）或副肿瘤性（P-LEMS）。分析人口统计学、临床和处方数据，并使用官方人口普查数据计算发病率和患病率。
共确诊159例LEMS病例。诊断时的中位年龄为60岁，55.3%的患者为女性。P-LEMS占病例的59.7%，其中55.8%伴有小细胞肺癌（SCLC）。LEMS的年发病率为每百万0.09至0.30，2024年的总体患病率为每百万1.11。2024年A-LEMS的患病率高于P-LEMS，可能是因为其死亡率较低（23.4%对58.9%）。P-LEMS在老年男性中更常见，主要与SCLC相关。65.4%的患者使用了吡啶斯的明，24.5%的患者使用了氨吡啶，两种治疗方法在A-LEMS中使用得更频繁。
这是土耳其首次针对LEMS的全国性流行病学研究，显示出比其他国家更低的发病率和患病率。本研究提供了有价值的大规模流行病学数据，丰富了全球对这种罕见疾病的认识。
