Our objective was to describe racial and ethnic differences of amyotrophic lateral sclerosis (ALS) in distinct geographic locations around the United States (U.S.). ALS cases for the period 2009–2011 were identified using active case surveillance in three states and eight metropolitan areas. Of the 5883 unique ALS cases identified, 74.8% were white, 9.3% were African-American/black, 3.6% were Asian, 12.0% were an unknown race, and 0.3% were marked as some other race. For ethnicity, 77.5% were defined as non-Hispanic, 10.8% Hispanic, and 11.7% were of unknown ethnicity. The overall crude average annual incidence rate was 1.52 per 100,000 person-years and the rate differed by race and ethnicity. The overall age-adjusted average annual incidence rate was 1.44 per 100,000 person-years and the age-adjusted average incidence rates also differed by race and ethnicity. Racial differences were also found in payer type, time from symptom onset to diagnosis, reported El Escorial criteria, and age at diagnosis. In conclusion, calculated incidence rates demonstrate that ALS occurs less frequently in African-American/blacks and Asians compared to whites, and less frequently in Hispanics compared to non-Hispanics in the U.S. A more precise understanding of racial and ethnic variations in ALS may help to reveal candidates for further studies of disease etiology and disease progression.

Most amyotrophic lateral sclerosis (ALS) cases are considered sporadic, without a known genetic basis, and environmental exposures are thought to play a causal role. To learn more about sporadic ALS etiology, we recruited n = 188 ALS patients from northern New England and Ohio and matched controls 2:1 from the general population of the same regions. Questionnaires evaluated the association between a variety of lifestyle, behavioral (ie, hobbies and activities), and occupational factors and the risk of ALS, including the duration of time between exposure and ALS onset, and exposure frequency. Head trauma was associated with increased ALS risk (adjusted odds ratio [OR] 1.60 95% confidence interval [CI] 1.04‐2.45), with significantly greater effects for injuries occurring 10 or more years prior to symptom onset (P = .037). ALS risk was increased for those reporting severe electrical burns (adjusted OR 2.86, 95% CI 1.37‐6.03), with odds ratios highest for burns after age 30 (OR 3.14), and for burns 10 or more years prior to symptom onset (OR 3.09). Hobbies involving lead were the most strongly associated with ALS risk (adjusted OR 2.92, 95% CI 1.45‐5.91). Exposures to lead 20 or more years prior to diagnosis had larger effect sizes compared to those occurring more recently. Holding a job in mechanics, painting, or construction was associated with ALS. The identification of these specific environmental factors associated with ALS highlight the need for future prospective and laboratory studies to assess causality, biological mechanisms, and find prevention or treatment opportunities.

Background: Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that typically results in death within 2–5 years of initial symptom onset. Multidisciplinary ALS clinics (MDCs) have been established to provide specialty care to people living with the disease. Objective: To estimate the proximity of ALS prevalence cases to the nearest MDC in the US to help evaluate one aspect of access to care. Methods: Using 2013 prevalence data from the National ALS Registry, cases were geocoded by city using geographic information system (GIS) software, along with the locations of all MDCs in operation during 2013. Case-to-MDC proximity was calculated and analyzed by sex, race, and age group. Results: During 2013, there were 72 MDCs in operation in 30 different states. A total of 15,633 ALS cases were geocoded and were distributed throughout all 50 states. Of these, 62.6% were male, 77.9% were white, and 76.2% were 50–79 years old. For overall case-to-MDC proximity, nearly half (44.9%) of all geocoded cases in the US lived >50 miles from an MDC, including approximately a quarter who lived >100 miles from an MDC. There was a statistically significant difference between distance to MDC by race and age group. Conclusions: The high percentage of those living more than 50 miles from the nearest specialized clinic underscores one of the many challenges of ALS. Having better access to care, whether at MDCs or through other modalities, is likely key to increasing survivability and obtaining appropriate end-of-life treatment and support for people with ALS.

Purpose of review The vermiculite ore formerly mined in Libby, Montana, contains asbestiform amphibole fibers of winchite, richterite, and tremolite asbestos. Because of the public health impact of widespread occupational and nonoccupational exposure to amphiboles in Libby vermiculite, numerous related studies have been published in recent years. Here we review current research related to this issue. Recent findings Excess morbidity and mortality classically associated with asbestos exposure have been well documented among persons exposed to Libby vermiculite. Excess morbidity and mortality have likewise been documented among persons with only nonoccupational exposure. A strong exposure–response relationship exists for many malignant and nonmalignant outcomes and the most common outcome, pleural plaques, may occur at low lifetime cumulative exposures. Summary The public health situation related to Libby, Montana, has led to huge investments in public health actions and research. The resulting studies have added much to the body of knowledge concerning health effects of exposures to Libby amphibole fibers specifically and asbestos exposure in general.