Department of Neurology, Oklahoma City Veterans Affairs Medical Center, Oklahoma City, OK, USA.
Department of Neurology, the Oklahoma University Health Sciences Center, Oklahoma City, OK, USA.
Sci Prog. 2024 Jul-Sep;107(3):368504241262902. doi: 10.1177/00368504241262902.
We sought to characterize the clinical prognostic factors in veterans with amyotrophic lateral sclerosis (ALS) followed in our ALS clinic.
ALS is a rare, progressive neurodegenerative condition associated with decreased survival compared to that in the normal population.
The electronic medical records of 105 veterans diagnosed with ALS who are followed in our ALS clinic between 2010 and 2021 were reviewed. Approval from the institutional review board was obtained from the study protocol. Demographic and clinical variables included age at symptom onset, age at initial evaluation, survival (from symptom onset to death), gender, site of onset (appendicular, bulbar, and respiratory), initial amyotrophic lateral sclerosis functional-related score-revised (ALSFRS-R), total functional independence measure (TFIM) scores, initial forced vital capacity (FVC), and interventions (Riluzole, gastrostomy, noninvasive ventilation [NIV], and tracheostomy). Normally distributed data was expressed as mean ± standard deviation. Fischer's exact analysis of the distribution differences of categorical data. The Kaplan-Meier plot analyzed the time-to-event.
The mean (SD) age at symptom onset was 62.0 (11.1) years, age at diagnosis was 65 (11) years, with 72% of the patients being over 60 years at diagnosis. The median survival time from symptom onset was 4.12 (3) years. Limb-onset ALS (appendicular) was the most frequent (52%) followed by bulbar-onset ALS (43%). The mean ALSFRS-R and TFIM scores were 31 (8) and 91 (25), respectively. Family history (familial), bulbar, and respiratory presentation at diagnosis were associated with shorter survival times.
This study suggests that of the clinical prognostic factors veterans with familial ALS, bulbar, and respiratory onset at presentations had shorter survival. The presence of Agent Orange, PEG placement, and NIV did not affect survival.
我们旨在描述在我们的肌萎缩侧索硬化症 (ALS) 诊所接受治疗的退伍军人中 ALS 的临床预后因素。
与正常人群相比，ALS 是一种罕见的进行性神经退行性疾病，生存时间缩短。
回顾了 2010 年至 2021 年间在我们的 ALS 诊所接受治疗的 105 名退伍军人的电子病历，这些退伍军人被诊断患有 ALS。该研究方案获得了机构审查委员会的批准。人口统计学和临床变量包括症状发作时的年龄、首次评估时的年龄、生存时间（从症状发作到死亡）、性别、发病部位（肢体、延髓和呼吸）、初始肌萎缩侧索硬化功能相关评分修订版（ALSFRS-R）、总功能独立性测量（TFIM）评分、初始用力肺活量（FVC）和干预措施（利鲁唑、胃造口术、无创通气 [NIV] 和气管切开术）。正态分布数据表示为平均值±标准差。使用 Fisher 精确检验分析分类数据的分布差异。Kaplan-Meier 图分析时间事件。
症状发作的平均（SD）年龄为 62.0（11.1）岁，诊断年龄为 65 岁（11 岁），72%的患者在诊断时年龄超过 60 岁。从症状发作到死亡的中位生存时间为 4.12（3）年。肢体起病的 ALS（肢体）最常见（52%），其次是延髓起病的 ALS（43%）。平均 ALSFRS-R 和 TFIM 评分为 31（8）和 91（25）。家族史（家族性）、延髓和呼吸首发与较短的生存时间相关。
本研究表明，在退伍军人中，家族性 ALS、延髓和呼吸首发的临床预后因素与较短的生存时间相关。存在 Agent Orange、PEG 植入物和 NIV 不会影响生存。
