Laurie Wagner

Racial and ethnic differences among amyotrophic lateral sclerosis cases in the United States

Abstract Our objective was to describe racial and ethnic differences of amyotrophic lateral sclerosis (ALS) in distinct geographic locations around the United States (U.S.). ALS cases for the period 2009–2011 were identified using active case surveillance in three states and eight metropolitan areas. Of the 5883 unique ALS cases identified, 74.8% were white, 9.3% were African-American/black, 3.6% were Asian, 12.0% were an unknown race, and 0.3% were marked as some other race. For ethnicity, 77.5% were defined as non-Hispanic, 10.8% Hispanic, and 11.7% were of unknown ethnicity. The overall crude average annual incidence rate was 1.52 per 100,000 person-years and the rate differed by race and ethnicity. The overall age-adjusted average annual incidence rate was 1.44 per 100,000 person-years and the age-adjusted average incidence rates also differed by race and ethnicity. Racial differences were also found in payer type, time from symptom onset to diagnosis, reported El Escorial criteria, and age at diagnosis. In conclusion, calculated incidence rates demonstrate that ALS occurs less frequently in African-American/blacks and Asians compared to whites, and less frequently in Hispanics compared to non-Hispanics in the U.S. A more precise understanding of racial and ethnic variations in ALS may help to reveal candidates for further studies of disease etiology and disease progression.

State and metropolitan area-based amyotrophic lateral sclerosis (ALS) surveillance.

Our objective was to develop state and metropolitan area-based surveillance projects to describe the characteristics of those with ALS and to assist with evaluating the completeness of the National ALS Registry. Because the literature suggested that ethnic/racial minorities have lower incidence of ALS, three state and eight metropolitan areas were selected to over-represent ethnic/racial minorities to have a sufficient number of minority patients. Project activities relied on reports from medical providers and medical records abstraction. The project areas represented approximately 27% of the U.S. population. The combined racial and ethnic distribution of these areas is 64.4% white, 16.0% African-American, 6.7% Asian, and 28.3% Hispanic. Most neurologists did not diagnose or provide care for ALS patients. The number of unique patients reported was close to expected (5883 vs. 6673). Age and gender distribution of patients was similar to the literature. The crude average annual incidence rate was 1.52 per 100,000 person-years, CI 1.44–1.61, and the 2009 prevalence rate was 3.84 per 100,000 population, CI 3.70–3.97. In conclusion, this study represents the largest number of clinically diagnosed ALS patients reported by neurologists in the U.S. Comparison of these data with those in the National ALS Registry will help evaluate the completeness of administrative databases.

Amyotrophic lateral sclerosis surveillance in Baltimore and Philadelphia

Introduction: Limited epidemiological data on amyotrophic lateral sclerosis (ALS) exist in defined geographic areas in the United States. Methods: Neurologists submitted case reports for patients under their care between January 1, 2009, and December 31, 2011, who met the El Escorial criteria. Diagnosis was confirmed for a sample of cases by the consulting neurologist. Death certificate data were used for supplemental case identification. Results: The 248 reported cases were most likely to be 50–69 years old, men, white, and non‐Hispanic. The total crude average annual incidence rate was 1.46 per 100,000 person‐years. Conclusions: The reported demographic characteristics were consistent with previously published findings. The crude annual incidence was slightly lower than the expected rate of 1.6 but was within the range reported previously (0.7–2.5). These findings help quantify the burden of ALS in the United States. Muscle Nerve 51: 815–821, 2015

Updated Prevalence and Demographic Characteristics for ALS Cases in Texas, 2009–2011

Objectives Amyotrophic lateral sclerosis (ALS) is a rare motor neuron disease with incidence rates ranging from 1 to 2/100,000 person-years. The Texas Department of State Health Services previously conducted surveillance for ALS in three metropolitan areas of Texas. This project provides an update to this research, while expanding its scope to the entire state. Methods The Texas Department of State Health Services contacted neurologists throughout Texas to determine whether they diagnose or treat patients with ALS. Those neurologists who cared for Texas residents with ALS between 2009 and 2011 were asked to complete a one-page case reporting form for each patient. Results A total of 1422 unique cases were received. The average crude annual incidence rate was 1.30/100,000 person-years and the 2009 period prevalence rate was 2.92/100,000 individuals. Reported cases were most likely to be 60 to 69 years old, non-Hispanic, white, and men. Conclusions This project provides an update to previously published Texas-specific epidemiological data regarding ALS; also, we note that our findings are consistent with previously published studies.

Evaluating the completeness of the national ALS registry, United States

Abstract Our objective was to evaluate the completeness of the United States National ALS Registry (Registry). We compared persons with ALS who were passively identified by the Registry with those actively identified in the State and Metropolitan Area ALS Surveillance project. Cases in the two projects were matched using a combination of identifiers, including, partial social security number, name, date of birth, and sex. The distributions of cases from the two projects that matched/did not match were compared and Chi-square tests conducted to determine statistical significance. There were 5883 ALS cases identified by the surveillance project. Of these, 1116 died before the Registry started, leaving 4767 cases. We matched 2720 cases from the surveillance project to those in the Registry. The cases identified by the surveillance project that did not match cases in the Registry were more likely to be non-white, Hispanic, less than 65 years of age, and from western states. The methods used by the Registry to identify ALS cases, i.e. national administrative data and self-registration, worked well but missed cases. These findings suggest that developing strategies to identify and promote the Registry to those who were more likely to be missing, e.g. non-white and Hispanic, could be beneficial to improving the completeness of the Registry.