From the Department of Neurology (M.P., J.D.G., C.N.F.), Emory University School of Medicine (D.B.), Atlanta, GA.
Neurology. 2021 Feb 9;96(6):e840-e844. doi: 10.1212/WNL.0000000000011396. Epub 2020 Dec 28.
To compare characteristics between Black and White patients with amyotrophic lateral sclerosis (ALS) in order to identify disparities and phenotypic variability.
We performed database review for patients seen between 1997 and 2020 at the Emory ALS Center in Atlanta, Georgia. Patients with ALS were included for analyses if race was self-reported as Black or White and symptom onset was prior to January 1, 2017. Variables examined include race, age at onset, diagnostic delay, site of onset, median income, mutation status, feeding tube and tracheostomy status, vital capacity, Amyotrophic Lateral Sclerosis Functional Rating Scale-revised(ALSFRS-R) score, and survival time.
A total of 2,363 patient records were queried, and 1,298 were included in analysis; 203 self-identified as Black and 1,095 as White. Black patients had younger age at symptom onset, lower frequency of mutations, lower median income, longer diagnostic delay, and lower baseline ALSFRS-R and vital capacity compared to White patients. Black patients had a longer median survival than White patients; however, race was not an independent predictor of survival time when controlling for age at symptom onset, bulbar onset, and positivity.
Black patients with ALS had longer median survival compared to White patients, but race was not independently associated with survival after controlling for age, site of onset, and status, factors known to predict prognosis. Black patients with ALS had longer diagnostic delay and lower baseline ventilatory and functional status at first clinic visit compared to White patients, which could be suggestive of barriers to tertiary care. Further studies are needed to identify the underlying causes of ALS racial differences.
比较肌萎缩侧索硬化症（ALS）黑人和白人患者的特征，以确定差异和表型变异性。
我们对 1997 年至 2020 年期间在佐治亚州亚特兰大埃默里 ALS 中心就诊的患者进行了数据库回顾。如果患者的种族自报为黑或白，且症状发作时间早于 2017 年 1 月 1 日，则将其纳入分析。检查的变量包括种族、发病年龄、诊断延迟、发病部位、中位收入、突变状态、喂养管和气管造口状态、肺活量、肌萎缩侧索硬化功能评定量表修订版（ALSFRS-R）评分和生存时间。
共查询了 2363 份患者记录，其中 1298 份被纳入分析；203 份自我认定为黑人，1095 份自我认定为白人。与白人患者相比，黑人患者的症状发作年龄更小，突变频率更低，中位收入更低，诊断延迟时间更长，基线 ALSFRS-R 和肺活量更低。黑人患者的中位生存期长于白人患者；然而，在控制症状发作年龄、延髓发病和突变阳性后，种族并不是生存时间的独立预测因素。
与白人患者相比，黑人肌萎缩侧索硬化症患者的中位生存期更长，但在控制年龄、发病部位和突变状态等已知预测预后的因素后，种族与生存时间无关。与白人患者相比，黑人肌萎缩侧索硬化症患者的诊断延迟时间更长，首次就诊时的基线通气和功能状态更低，这可能表明他们获得三级护理的障碍更大。需要进一步的研究来确定肌萎缩侧索硬化症种族差异的根本原因。
