Abenroth Daniel C, Smith A Gordon, Greenlee John E, Austin Sharon D, Clardy Stacey L
Department of Neurology, Veterans Affairs Medical Center, Salt Lake City, Utah, USA.
Department of Neurology, University of Utah Health, 729 Arapeen Drive, Salt Lake City, Utah, 84108, USA.
Muscle Nerve. 2017 Sep;56(3):421-426. doi: 10.1002/mus.25520. Epub 2017 Mar 17.
One nationwide study (The Netherlands) of Lambert-Eaton myasthenic syndrome (LEMS) has been published. We report LEMS epidemiology and its therapeutic response in the United States Veterans Affairs (VA) population.
Medical records for all active patients (12.5 million) in the VA health system were queried for relevant ICD-9 codes for the period October 1, 1999 to September 30, 2013. Clinical, electrophysiologic, and serologic features were evaluated to confirm diagnosis; epidemiologic and treatment data were collected.
Point prevalence was estimated at 2.6 per 1,000,000 (confirmed cases) and 3.3 per 1,000,000 (combined confirmed and probable cases). Crude prevalence was similarly estimated at 9.2 and 10.9 per 1,000,000 respectively. A total of 18 of 48 (38%) patients received 3,4-diaminopyridine (3,4-DAP); 14 of 18 (78%) improved.
This investigation was a large North American epidemiologic study of LEMS. LEMS prevalence in the national VA population was found to be similar to previously published rates in other large international populations. Most patients experienced improvement with therapy, including a majority with 3,4-DAP. Muscle Nerve 56: 421-426, 2017.
一项关于兰伯特-伊顿肌无力综合征（LEMS）的全国性研究（荷兰）已发表。我们报告了美国退伍军人事务部（VA）人群中LEMS的流行病学及其治疗反应。
查询了VA医疗系统中所有现役患者（1250万）在1999年10月1日至2013年9月30日期间的相关ICD-9编码的医疗记录。对临床、电生理和血清学特征进行评估以确诊；收集了流行病学和治疗数据。
点患病率估计为每100万中有2.6例（确诊病例）和每100万中有3.3例（确诊病例与可能病例合计）。粗患病率估计分别为每100万中有9.2例和10.9例。48例患者中有18例（38%）接受了3,4-二氨基吡啶（3,4-DAP）治疗；18例中有14例（78%）病情改善。
这项调查是一项关于LEMS的大型北美流行病学研究。发现全国VA人群中LEMS的患病率与其他大型国际人群中先前公布的患病率相似。大多数患者经治疗后病情改善，包括大多数接受3,4-DAP治疗的患者。《肌肉与神经》56: 421 - 426, 2017年。
