PDF(Pubmed)
Abstract：
UNASSIGNED: We sought to characterize the clinical prognostic factors in veterans with amyotrophic lateral sclerosis (ALS) followed in our ALS clinic.
UNASSIGNED: ALS is a rare, progressive neurodegenerative condition associated with decreased survival compared to that in the normal population.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: ALS is a rare, progressive neurodegenerative condition associated with decreased survival compared to that in the normal population.
UNASSIGNED: 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.
UNASSIGNED: 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.
UNASSIGNED: 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.
摘要：
■我们试图在我们的ALS诊所中描述患有肌萎缩性侧索硬化症（ALS）的退伍军人的临床预后因素。
■ALS是一种罕见的，与正常人群相比，进行性神经退行性疾病与生存率降低有关。
■对2010年至2021年在我们的ALS诊所随访的105名诊断为ALS的退伍军人的电子病历进行了审查。从研究方案获得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，初始评估时的年龄，生存（从症状发作到死亡），性别,发病部位(阑尾，球杆,和呼吸)，初始肌萎缩侧索硬化功能相关评分修订（ALSFRS-R），总功能独立性测量(TFIM)得分，初始强迫肺活量(FVC)，和干预措施(利鲁唑，胃造口术,无创通气[NIV]，和气管造口术)。正态分布数据表示为平均值±标准偏差。Fischer对分类数据的分布差异的精确分析。Kaplan-Meier图分析了事件发生的时间。
■症状发作时的平均（SD）年龄为62.0（11.1）岁，诊断年龄为65（11）岁，72%的患者在诊断时超过60岁。症状发作的中位生存时间为4.12(3)年。肢体发作的ALS（阑尾）最常见（52％），其次是球发作的ALS（43％）。平均ALSFRS-R和TFIM评分分别为31(8)和91(25)，分别。家族史（家族性），球杆,诊断时的呼吸道表现与较短的生存时间相关。
■这项研究表明，在患有家族性ALS的退伍军人的临床预后因素中，球杆,和出现时的呼吸发作生存期较短。橙剂的存在,PEG放置，和NIV不影响生存。
■ALS是一种罕见的，与正常人群相比，进行性神经退行性疾病与生存率降低有关。
■对2010年至2021年在我们的ALS诊所随访的105名诊断为ALS的退伍军人的电子病历进行了审查。从研究方案获得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，初始评估时的年龄，生存（从症状发作到死亡），性别,发病部位(阑尾，球杆,和呼吸)，初始肌萎缩侧索硬化功能相关评分修订（ALSFRS-R），总功能独立性测量(TFIM)得分，初始强迫肺活量(FVC)，和干预措施(利鲁唑，胃造口术,无创通气[NIV]，和气管造口术)。正态分布数据表示为平均值±标准偏差。Fischer对分类数据的分布差异的精确分析。Kaplan-Meier图分析了事件发生的时间。
■症状发作时的平均（SD）年龄为62.0（11.1）岁，诊断年龄为65（11）岁，72%的患者在诊断时超过60岁。症状发作的中位生存时间为4.12(3)年。肢体发作的ALS（阑尾）最常见（52％），其次是球发作的ALS（43％）。平均ALSFRS-R和TFIM评分分别为31(8)和91(25)，分别。家族史（家族性），球杆,诊断时的呼吸道表现与较短的生存时间相关。
■这项研究表明，在患有家族性ALS的退伍军人的临床预后因素中，球杆,和出现时的呼吸发作生存期较短。橙剂的存在,PEG放置，和NIV不影响生存。
