Application of Amyotrophic Lateral Sclerosis Staging Systems in ALS Patients (P4.460)

Objective: To examine the differences in the clinical characteristics of ALS patients in different racial and gender groups, and to identify differences in disease progression using two proposed ALS staging systems. Background: The prevalence of ALS has been reported to be lower in non-Caucasians compared to Caucasians. This discrepancy is difficult to evaluate due to the limited number of non-Caucasian cases identified. Also, it is unclear whether disease progression as determined by the proposed staging systems (Al Chalabi and ALS-MITOS) in a group of racially diverse patients in the US is similar to patients in Europe. Understanding the differences between these subgroups is important for stratification in clinical trials. Design/Methods: We conducted a retrospective chart review of 139 patients who attended the ALS Clinic at University of Maryland. We reviewed demographics, clinical characteristics, clinical measures (ALSFRS-R, force vital capacity), and the percentage of time patients remained in each stage to compare the disease progression of different racial groups using two staging systems. Results: The average age of onset was 61.6 years in Caucasians and 58.2 years for non-Caucasians. For all patients, those with bulbar onset were older than limb onset patients, and the limb onset patients had slower progression. Caucasians survived an average of 34.4 months after symptom onset, while African Americans and other non-Caucasians survived 44.7 and 37.7 months, respectively. African American females spent a larger fraction of total survival in the earlier stages of both staging systems than Caucasian females. Conclusions: Both clinical staging systems were easily applied to our patient population. Differences in the time spent in each stage between Caucasian and non-Caucasian may represent underlying genetic factors. Although further studies are needed to validate these staging systems, we believe that these scales and others will help to identify characteristics useful for subject stratification in clinical studies. Disclosure: Dr. Lubinski has nothing to disclose. Dr. Diaz-Abad has nothing to disclose. Dr Kwan has nothing to disclose.