Findings from the New Jersey State-Wide Amyotrophic Lateral Sclerosis (ALS) Surveillance Project (P5.080)

OBJECTIVE: To calculate the incidence and prevalence of Amyotrophic Lateral Sclerosis (ALS) in New Jersey (NJ); to describe the demographic characteristics of ALS cases; and to assist the federal Agency for Toxic Substances and Disease Registry (ATSDR) in evaluating the completeness of the National ALS Registry (Registry). BACKGROUND: ATSDR maintains the Registry, which identifies ALS cases using national administrative datasets and patient self-registration through a web portal. This non-traditional case ascertainment requires validation; therefore, ATSDR established surveillance projects in three states and eight metropolitan areas. DESIGN/METHODS: Neurologists were contacted to determine if they diagnosed or cared for ALS patients and were asked to submit one-page case reporting forms for NJ residents with ALS under their care from January 1, 2009 through December 31, 2011. A medical record verification form and a copy of an electromyogram (EMG) report were requested for a sample of cases. Death and hospital data were reviewed to identify and collect additional case reports. RESULTS: Twenty-five percent (168/679) of neurologists diagnosed or cared for ALS patients and 90% (152/168) reported cases. There were 764 reported cases; 91.2% (697/764) were reported during active data collection and 8.7% (67/764) were reported through death and hospital data follow-up. All verified cases were classified as ALS by the consulting neurologist. The average annual age-adjusted incidence rate was 1.67 per 100,000 person-years and the age-adjusted point prevalence rate on December 31, 2011 was 4.40 per 100,000 persons. Incidence and point prevalence rates were higher among males, whites, and non-Hispanics. CONCLUSIONS: Project objectives were met by using active case ascertainment methodology among neurologists,supplemented by a second wave of data collection using death and hospital data. The project findings contribute new, population-based, state-specific epidemiological information regarding ALS. Stratified age-adjusted average annual incidence and point prevalence estimates were generally consistent with literature findings. Study Supported by: McKing Consulting Corporation through a contract funded by the Agency for Toxic Substances and Disease Registry (Contract # GS00F0042P). Disclosure: Ms. Jordan has received personal compensation for activities with McKing Consulting Corp. as an employee. Dr. Fagliano has nothing to disclose. Dr. Lefkowitz has nothing to disclose. Ms. Rechtman has received personal compensation for activities with McKing Consulting Corp. as an employee. Dr. Kayehas received personal compensation for activities with McKing Consulting Corporation as an employee.