Comparison of Phenotypic Characteristics and Prognosis Between Black and White Patients in a Tertiary ALS Clinic.

Abstract:

OBJECTIVE:To compare characteristics between Black and White patients with amyotrophic lateral sclerosis (ALS) in order to identify disparities and phenotypic variability. METHODS: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, C9orf72 mutation status, feeding tube and tracheostomy status, vital capacity, Amyotrophic Lateral Sclerosis Functional Rating Scale-revised(ALSFRS-R) score, and survival time. RESULTS: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 C9orf72 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 C9orf72 positivity. CONCLUSIONS: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 C9orf72 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.

journal_name

Neurology

journal_title

Neurology

authors

Brand D,Polak M,Glass JD,Fournier CN

doi

10.1212/WNL.0000000000011396

subject

Has Abstract

pub_date

2020-12-28 00:00:00

eissn

0028-3878

issn

1526-632X

pii

WNL.0000000000011396

pub_type

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