Biogen Idec, 14 Cambridge Center, Cambridge, MA 02142, USA.
BMC Neurol. 2013 Nov 4;13:160. doi: 10.1186/1471-2377-13-160.
Initial symptoms of amyotrophic lateral sclerosis (ALS) are often subtle and can delay diagnosis. This exploratory analysis was conducted to better characterize the pre-diagnosis pathway undertaken by patients with ALS in the US Centers for Medicare & Medicaid Services Medicare longitudinal claims database.
Quarterly Medicare claims data were analyzed to determine the pre-diagnosis pathway for an ALS patient cohort that included patients aged ≥ 65 years with ≥ 2 ALS claims (International Classification of Diseases, Ninth Revision, Clinical Modification code 335.20) between the first quarter of 2007 and the fourth quarter of 2009, and were enrolled in Medicare ≥ 2 years before the first ALS claim (diagnosis). A cohort of Medicare patients without claims for motor neuron diseases were identified for comparison. A subset of these patients with ≥ 3 years of claims data was included in a time to diagnosis analysis. Data extraction included the most common initial symptoms of ALS, the time from first ALS symptom to diagnosis, and the diagnostic procedures performed before the diagnosis of ALS.
A total of 399 patients met the inclusion criteria and were included in the ALS cohort; 272 patients were included in the time to diagnosis cohort. Before the quarter of diagnosis, symptoms that were more frequently seen in the ALS cohort than the general Medicare cohort included muscle weakness, lack of coordination and speech/swallowing difficulties. Limb-onset ALS (74%) was more common than bulbar-onset ALS (17%). Median time to diagnosis for limb- and bulbar-onset patients was 2.5 years and 1.25 years, respectively. The most common tests conducted before the quarter of diagnosis included sensory and motor nerve conduction tests, imaging studies, and electromyography; however, a substantial number of patients did not receive any nerve conduction testing. Motor nerve conduction testing in patients with bulbar-onset ALS had the largest impact on time to diagnosis.
This analysis describes a diagnostic delay for patients with ALS in the US Medicare population, similar to previous reports. The development of tools and ongoing education that can help to identify patients with ALS earlier in their disease course is needed.
肌萎缩侧索硬化症（ALS）的初始症状通常较为微妙，可能会延迟诊断。本探索性分析旨在更好地描述美国医疗保险和医疗补助服务中心 Medicare 纵向索赔数据库中 ALS 患者的预诊断途径。
分析了季度 Medicare 索赔数据，以确定包括≥65 岁、至少有 2 次肌萎缩侧索硬化症索赔（国际疾病分类，第九修订版，临床修正码 335.20）的 ALS 患者队列的预诊断途径，这些患者在 2007 年第一季度至 2009 年第四季度期间有≥2 次 Medicare 索赔，并且在首次肌萎缩侧索硬化症索赔（诊断）前至少有 2 年的 Medicare 保险。为了比较，还确定了一组没有运动神经元疾病索赔的 Medicare 患者。将这些患者中有≥3 年索赔数据的子集纳入诊断时间分析。数据提取包括 ALS 最常见的初始症状、从首次 ALS 症状到诊断的时间，以及在诊断 ALS 之前进行的诊断程序。
共有 399 名患者符合纳入标准，并被纳入 ALS 队列；272 名患者被纳入诊断时间队列。在诊断季度之前，ALS 队列中比一般 Medicare 队列更常见的症状包括肌肉无力、协调缺失和言语/吞咽困难。肢体起病 ALS（74%）比球部起病 ALS（17%）更为常见。肢体起病和球部起病患者的中位诊断时间分别为 2.5 年和 1.25 年。在诊断季度之前进行的最常见测试包括感觉和运动神经传导测试、影像学研究和肌电图；然而，相当数量的患者未接受任何神经传导测试。球部起病 ALS 患者的运动神经传导测试对诊断时间的影响最大。
这项分析描述了美国 Medicare 人群中 ALS 患者的诊断延迟，与之前的报告相似。需要开发和持续教育工具，以帮助更早地识别疾病过程中的 ALS 患者。
