Objective: To summarize the prevalence of ALS in all 50 states and Washington, DC in the United States from 2011 to 2018 using data collected and analyzed by the National ALS Registry. In October 2010, the federal Agency for Toxic Substances and Disease Registry (ATSDR) launched the congressionally mandated Registry to determine the incidence and prevalence of ALS within the USA, characterize the demographics of persons with ALS, and identify the potential risk factors for the disease. This is the first analysis of state-level ALS prevalence estimates. Methods: ALS is not a notifiable disease in the USA, so the Registry uses a two-pronged approach to identify cases. The first approach uses existing national administrative databases (Medicare, Veterans Health Administration, and Veterans Benefits Administration). The second method uses a secure web portal to gather voluntary participant data and identify cases not included in the national administrative databases. Results: State-level age-adjusted average prevalence from 2011-2018 ranged from 2.6 per 100,000 persons (Hawaii) to 7.8 per 100,000 persons (Vermont), with an average of 4.4 per 100,000 persons in the US. New England and Midwest regions had higher prevalence rates than the national average. Conclusions: These findings summarize the prevalence of ALS for all 50 states from 2011 to 2018. This is a continuing effort to identify ALS cases on a national population basis. The establishment of the National ALS Registry has allowed for epidemiological trends of this disease and the assessment of potential risk factors that could cause ALS.

Social determinants of health (SDoH) contribute to disparities in SARS-CoV-2 infection, but their associations with long COVID are unknown. To determine associations between SDoH at the time of SARS-CoV-2 infection and risk for long COVID. Prospective observational cohort study. 33 states plus Washington, DC, and Puerto Rico. Adults (aged ≥18 years) enrolled in RECOVER-Adult (Researching COVID to Enhance Recovery) between October 2021 and November 2023 who were within 30 days of SARS-CoV-2 infection; completed baseline SDoH, comorbidity, and pregnancy questionnaires; and were followed prospectively. Social risk factors from SDoH baseline questionnaires, ZIP code poverty and household crowding measures, and a weighted score of 11 or higher on the Long COVID Research Index 6 months after infection. Among 3787 participants, 418 (11%) developed long COVID. After adjustment for demographic characteristics, pregnancy, disability, comorbidities, SARS-CoV-2 severity, and vaccinations, financial hardship (adjusted marginal risk ratio [ARR], 2.36 [95% CI, 1.97 to 2.91]), food insecurity (ARR, 2.36 [CI, 1.83 to 2.98]), less than a college education (ARR, 1.60 [CI, 1.30 to 1.97]), experiences of medical discrimination (ARR, 2.37 [CI, 1.94 to 2.83]), skipped medical care due to cost (ARR, 2.87 [CI, 2.22 to 3.70]), and lack of social support (ARR, 1.79 [CI, 1.50 to 2.17]) were associated with increased risk for long COVID. Living in ZIP codes with the highest (vs. lowest) household crowding was also associated with greater risk (ARR, 1.36 [CI, 1.05 to 1.71]). Selection bias may influence observed associations and generalizability. Participants with social risk factors at the time of SARS-CoV-2 infection had greater risk for subsequent long COVID than those without. Future studies should determine whether social risk factor interventions mitigate long-term effects of SARS-CoV-2 infection. National Institutes of Health.

Background/Objectives: To examine if head injury (HI) is associated with age at ALS diagnosis in the United States. Methods: In this cross-sectional populationf-based analysis, we identified patients with ALS who were registered from 2015 to 2023 who completed the Registry’s head trauma survey module. The association between HI and age at ALS diagnosis was assessed using multivariate analysis. Results: Of the 3424 respondents, 56.6% had experienced a HI. The adjusted odds ratio (aOR) for an ALS diagnosis before age 60 years for patients with a HI was 1.24 (95% CI, 1.07–1.45). One or two HIs had an aOR of 1.15 (95% CI, 0.97–1.36), and five or more HIs had an aOR of 1.58 (95% CI, 1.19–2.09). HI before age 18 years yielded an aOR of 2.03 (95% CI, 1.53–2.70) as well as HI between the ages of 18 and 30 years (aOR = 1.48, 95% CI: 1.06–2.06)). When narrowing the analysis to patients with HI before age 18 compared with patients with no HI, we found an association with HI that led to an emergency department or hospital visit (aOR = 1.50 (95% CI: 1.21–1.86)). Conclusions: In this cross-sectional analysis of ALS patients, HIs occurring in childhood and early adulthood and the number of HIs increased the odds of being diagnosed before age 60 years. These results suggest that HI continues to be a risk factor for ALS and could be associated with a younger age of diagnosis.

To summarize amyotrophic lateral sclerosis (ALS) incidence in all 50 states and the District of Columbia from 2012 to 2019. In 2010, the Agency for Toxic Substances and Disease Registry (ATSDR) launched the congressionally mandated National ALS Registry (Registry) to determine the incidence and prevalence of ALS within the United States, characterize demographics, and identify potential risk factors. This is the first analysis of state-level ALS incidence estimates for all 50 states and the District of Columbia. ALS is not a notifiable disease in the United States. As such, the Registry identifies cases using existing national administrative databases (Medicare, Veterans Health Administration, and Veterans Benefits Administration), and a secure web portal that identifies cases not included in the national databases. Confirmed and likely cases are deduplicated and counted as incident cases for the first year they are identified using a validated algorithm. Incident counts, incident rates, and rate ratios were then calculated. State-level age-adjusted overall incidence rates for 2012 to 2019 ranged from 0.65 per 100,000 persons (Alaska) to 2.25 per 100,000 persons (Vermont), with an overall incidence of 1.44 per 100,000 persons in the United States. New England and the upper Midwest regions had higher incidence rates than national rates. These findings summarize the incidence of ALS for all 50 states from 2012 to 2019. This is a continuing effort to identify ALS cases nationally. The establishment of the Registry allows for epidemiological analyses of ALS data and the assessment of potential risk factors.

Background: Amyotrophic lateral sclerosis (ALS) is a fatal, progressive neurodegenerative disease that typically results in death within 3–5 years from symptom onset. However, little is known about the environmental exposures, clinical aspects, or social determinants of health factors that may be associated with the disease. Multistep modeling has been previously applied to cancer research, demonstrating a linear relationship between logs of incidence and age. This method may help to understand the mechanisms involved in the development of ALS in the United States (e.g., environmental exposures, genetic mutations). We aim to assess whether ALS is a multistep process among patients enrolled in the largest ALS registry in the world—the United States' National ALS Registry.Methods: Incident ALS cases, defined as confirmed and likely, cases between 2012 and 2019 were obtained from the National ALS Registry. Age‐standardized incidence was calculated for all cases and by sex. The log incidence of ALS was regressed against the log of age (years) at case determination, on average, for each year and by sex.Findings: Between 2012 and 2019, there was a mean of 5253 incident ALS cases (confirmed or likely) per year. We identified a linear relationship between the log of the average incidence and log age overall (r² = 0.99), for men (r² = 0.99), and for women (r² = 0.98). The incidence slope estimates were 4.8 (95% CI: 4.4–5.1) overall, 4.7 (95% CI: 4.4–5.1) for men, and 5.0 (95% CI: 4.5–5.5) for women.Interpretation: The linear relationships observed overall, for men, and for women are consistent with a multi‐step process. The slope estimates, on average, are approximately 5.0, which suggests that the development of ALS is a six‐step process. Further investigation of these steps can elucidate potential risk factors and treatments for ALS.

Key Points: Residential segregation is associated with reduced access to several important steps on the kidney transplant care continuum. Residential segregation affects both Black and White individuals with ESKD seeking lifesaving transplant. Background: Individuals currently living in neighborhoods historically influenced by racial segregation have reduced access to health care. Whether this is true for individuals with ESKD seeking transplant is unknown. Methods: We identified Black or White adults (N=42,401; 18–80 years) with ESKD initiating KRT in three US states (Georgia, North Carolina, South Carolina) between January 2015 and December 2019, with follow-up through 2020, from the United States Renal Data System. Residential segregation was defined using the racial Index of Concentration at the Extremes and classified into tertiles (predominantly Black, mixed, or predominantly White neighborhoods). Primary outcomes were referral within 12 months of KRT initiation (among individuals initiating KRT) and evaluation within 6 months of referral (among all referred individuals), determined by linkage of the United States Renal Data System to the Early Steps to Transplant Access Registry. Secondary outcomes included waitlisting (among evaluated individuals) and living or deceased donor transplant (among waitlisted individuals). The association between residential segregation and each outcome was assessed using multivariable Cox models with robust sandwich variance estimators. Results: In models adjusted for clinical factors, individuals living in predominantly Black or mixed (versus predominantly White) neighborhoods were 8% (adjusted hazard ratio [aHR], 0.92 [0.88 to 0.96]) and 5% (aHR, 0.95 [0.91 to 0.99]) less likely to be referred for a kidney transplant, 18% (aHR, 0.82 [0.76 to 0.90]) and 9% (aHR, 0.91 [0.84 to 0.98]) less likely to be waitlisted among those who started evaluation, and 54% (aHR, 0.46 [0.36 to 0.58]) and 24% (aHR, 0.76 [0.63 to 0.93]) less likely to receive a living donor kidney transplant among those who were waitlisted, respectively. For other transplant steps, associations were nonsignificant. Conclusions: Individuals with ESKD living in historically and currently marginalized communities in the Southeast United States have reduced access to important steps along the transplant care continuum.