Amyotrophic lateral sclerosis surveillance in Baltimore and Philadelphia

Abstract

IntroductionLimited epidemiological data on amyotrophic lateral sclerosis (ALS) exist in defined geographic areas in the United States.MethodsNeurologists 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.ResultsThe 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.ConclusionsThe 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.

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

“…The age groups 60-69 years and 70-79 years are the most common ages of diagnosis and those aged 18-39 years are the least. This finding is consistent with reported literature in the United States and abroad (1,6,(36)(37)(38)(39)(40)(41)(42)(43).…”

“…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, a Possible Sequela of Chronic Myeloid Leukemia

“…Etiology is deemed multifactorial with both genetic and environmental factors thought to be playing a role [5]. The incidence rates for ALS in Europe and North America range between 1.5 and 2.7 per 100,000 person-years while prevalence rates range between 2.7 and 7.4 per 100,000 person-years [8,9,10,11]. In the Unites States, rates of ALS are higher among the non-Hispanic white population compared with non-Hispanic blacks, Hispanics, and other racial/ethnic groups [11,12].…”

Evaluation of the Completeness of ALS Case Ascertainment in the US National ALS Registry: Application of the Capture-Recapture Method

“…The self-report option was designed to improve capture for people with private health insurance, given that ALS is frequently diagnosed between the ages of 40 and 70 when people are still empoyed. With the goal of evaluating the completeness of case ascertainment using these methods, the Registry conducted a series of independent studies between 2009 and 2011 in eight US states and three metropolitan regions [9][10][11][12][13][14]. A subsequent report by the Registry determined that 54% of ALS patients identified by active case-finding in those studies were able to be ascertained using registry methodology [15].…”