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

Abstract

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.

Prevalence of Amyotrophic Lateral Sclerosis — United States, 2012–2013

“…Because ALS is predominantly a disease that affects whites, these states and metro areas were selected for their over-representation of minority populations of blacks, Asians, and Hispanics. Incidence rates ranged from 1.1 to 2.1 per 100,000 personyears in individual states and metro areas (39,41,(49)(50)(51) and the incidence rate was 1.5 person-years for all states and metro areas combined (52). Incidence rates by race and ethnicity were calculated for all states and metro areas combined and showed that the incidence rate in whites was higher than the incidence rates for blacks and Asians.…”

“…Third, the calculation of ALS incidence with Registry data is not possible at this time because the date of diagnosis is not captured through the large administrative database approach, and cases without a date of diagnosis comprise 68% of cases in the Registry. However, through a separate Registry project, incidence has been calculated and the findings published for ALS incidence in smaller defined geographic areas of the United States (39,41,(49)(50)(51)(52). Finally, the Registry has been officially active since October 2009 and is still being enhanced.…”

Amyotrophic lateral sclerosis among patients with a Medicare Advantage prescription drug plan; prevalence, survival and patient characteristics

“…To aid in validating National ALS registry findings, local surveillance projects were conducted in three states comprising eight metropolitan areas and the results were reported in 2015, as well as in subsequent studies using the same data (12)(13)(14)(15). These studies described ALS incidence and prevalence across the United States, but were limited to specific populations within certain geographies, and used only three years of data collection (2009-2011).…”

CDC Grand Rounds: National Amyotrophic Lateral Sclerosis (ALS) Registry Impact, Challenges, and Future Directions

“…Proposed reasons for the increase in prevalence includes comprehensive health care that allows patients to live longer, and large ALS clinics that provide patients with neurologic and nursing care, dietary support, and physical therapy care in one setting ( 13 ). However, not all ALS patients have access to large multidisciplinary ALS clinics, and those living in rural areas still tend to see their local primary care physician or neurologist ( 13 , 14 ).…”