Population-Based Surveillance of Amyotrophic Lateral Sclerosis in New Jersey, 2009-2011

Abstract

Background: Limited epidemiological data exist about amyotrophic lateral sclerosis (ALS) in the United States (US). The Agency for Toxic Substances and Disease Registry maintains the National ALS Registry and funded state and metropolitan surveillance projects to obtain reliable, timely information about ALS in defined geographic areas. Methods: Neurologists submitted case reports for ALS patients under their care between January 1, 2009 and December 31, 2011 who were New Jersey residents. A medical record verification form and electromyogram (EMG) report were requested for a sample of case reports. Incidence rates were standardized to the 2000 US Standard Population. Results: The average crude annual incidence rate was 1.87 per 100,000 person-years, the average age-adjusted annual incidence rate was 1.67 per 100,000 person-years, and the point prevalence rate on December 31, 2011 was 4.40 per 100,000 persons. Average annual incidence rates and point prevalence rates were statistically higher for men compared with women; Whites compared with Blacks/African Americans and Asians; and non-Hispanics compared with Hispanics. Conclusions: The project findings contribute new, population-based, state-specific information to epidemiological data regarding ALS. The findings are generally consistent with previously published surveillance studies conducted in the US and abroad.

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.…”

Effects of Demographic Factors on Survival Time after a Diagnosis of Amyotrophic Lateral Sclerosis

“…The median crude incidence rate among studies conducted around the world was 2.1 per 100,000 person-years (range: 0.3–3.6), and the crude prevalence was 5.4 per 100,000 persons (range: 1.0–11.3) [1] . In New Jersey (N.J., USA), the age-adjusted incidence was 1.67 per 100,000 person-years (2009–2011), and the point prevalence as of December 31, 2011, was 4.40 per 100,000 persons [2] . A higher incidence is associated with being older, male, white, and non-Hispanic [1–5] .…”

“…Estimates of the median survival time from diagnosis range between 18 and 29 months [2, 6–9] and most ALS patients die within two to five years of diagnosis [10] . Older age is associated with shorter survival [7–9, 11–13] , and there are conflicting reports of sex being a prognostic indicator [6, 8, 9, 11] .…”