Characterization of Amyotrophic Lateral Sclerosis (ALS) Seen in the Medical University of South Carolina ALS Multidisciplinary Clinic (P1.342)

2018 
Objective: To investigate any differences in the clinical presentation and course of ALS in different races, and any disparity in the utilisation of treatment. Background: Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease characterized by its progressive nature, limited understanding of its pathophysiology and lack of present cure. The diagnosis of ALS is complicated by diverse presentation, heterogeneity in the anatomic location (bulbar, cervical, thoracic and lumbar), and differences in progression and survival rates. ALS is also known to present differently in African Americans and Caucasian cohorts, though the findings were not universally reproducible. Design/Methods We obtained IRB approval for a retrospective analysis of patients with the diagnosis of ALS seen in our clinic from 2007–2016. Cases meeting the Awaji criteria of clinically-possible, -probable, or -definite ALS were included. Data analysed included demographic information (age, gender, race/ethnicity); clinical presentation (age at symptom onset, site of onset - limb vs. bulbar, time from onset to first clinic visit, time from onset to diagnosis, Awaji diagnostic category, weight/BMI); clinical course (the first and last ALS Functional Rating Scale-Revised, ALSFRS-R scores, and forced vital capacities, FVC); medical comorbidities; family history of neurodegenerative diseases (ALS, Parkinson’s disease or dementia); and use of therapies (riluzole, non-invasive ventilation, gastronomy). Results: Differences were observed in the presentation and severity of ALS between Caucasian and African American cohorts. Conclusions: Our cohort has a higher representation of African Americans, consistent with the census data in the state of South Carolina compared to the nation (27% vs 13%, 2015). The differences seen in the presentation and severity of disease for the different races need confirmation. Future studies should also focus on disparities in the utilization of therapies to improve outcome. Disclosure: Dr. Rodriguez has nothing to disclose. Dr. Chopade has nothing to disclose. Dr. Chen has nothing to disclose.
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