Findings from the New Jersey amyotrophic lateral sclerosis (ALS) surveillance project

Background: Limited epidemiological data exist about Amyotrophic Lateral Sclerosis (ALS), a rare, difficult to diagnose, neurological disease with no known cause or cure. The Agency for Toxic Substances and Disease Registry maintains the National ALS Registry and funds state and metropolitan surveillance projects to obtain reliable, timely information about ALS in defined geographic areas. This abstract summarizes the findings of the New Jersey (NJ) project. Methods: Neurologists (n= 679) were asked if they diagnose or treat NJ residents with ALS, and asked to submit case reports for those patients under their care from January 1, 2009 through December 31, 2011. A medical record verification form and electromyogram (EMG) report were requested for a sample of case reports. Compensation was offered, and no patients were contacted. Results: Twenty-five percent (n=168) of neurologists reported that they had diagnosed or treated ALS patients, and 90% of these provided case reports. Two non neurologists also reported cases. Of 965 collected case reports, 766 unique cases were retained. Ninety-six percent of requested verifications were collected and classified as ALS by a consulting neurologist. Prevalence rates were highest among non-Hispanics, Whites, and men. The annual incidence rates for 2009, 2010 and 2011 were 1.77, 1.98 and 1.84 per 100,000 persons, respectively. Conclusions: A small proportion of neurologists diagnose or treat NJ residents with ALS. With adequate planning, provision of compensation, and implementation of quality assurance procedures, we successfully collected data on NJ ALS cases. Our results are consistent with published rate estimates and contribute new information to epidemiological data regarding ALS.