Population-Based Estimates for the Prevalence of Multiple Sclerosis in the United States by Race, Ethnicity, Age, Sex, and Geographic Region.

Importance: Racial, ethnic, and geographic differences in multiple sclerosis (MS) are important factors to assess when determining the disease burden and allocating health care resources. Objective: To calculate the US prevalence of MS in Hispanic, non-Hispanic Black (hereafter referred to as Black), and non-Hispanic White individuals (hereafter referred to as White) stratified by age, sex, and region. Design, Setting, and Participants: A validated algorithm was applied to private, military, and public (Medicaid and Medicare) administrative health claims data sets to identify adult cases of MS between 2008 and 2010. Data analysis took place between 2019 and 2022. The 3-year cumulative prevalence overall was determined in each data set and stratified by age, sex, race, ethnicity, and geography. The insurance pools included 96 million persons from 2008 to 2010. Insurance and stratum-specific estimates were applied to the 2010 US Census data and the findings combined to calculate the 2010 prevalence of MS cumulated over 10 years. No exclusions were made if a person met the algorithm criteria. Main Outcomes and Measurements: Prevalence of MS per 100 000 US adults stratified by demographic group and geography. The 95% CIs were approximated using a binomial distribution. Results: A total of 744 781 persons 18 years and older were identified with MS with 564 426 cases (76%) in females and 180 355 (24%) in males. The median age group was 45 to 54 years, which included 229 216 individuals (31%), with 101 271 aged 18 to 24 years (14%), 158 997 aged 35 to 44 years (21%), 186 758 aged 55 to 64 years (25%), and 68 539 individuals (9%) who were 65 years or older. White individuals were the largest group, comprising 577 725 cases (77%), with 80 276 Black individuals (10%), 53 456 Hispanic individuals (7%), and 33 324 individuals (4%) in the non-Hispanic other category. The estimated 2010 prevalence of MS per 100 000 US adults cumulated over 10 years was 161.2 (95% CI, 159.8-162.5) for Hispanic individuals (regardless of race), 298.4 (95% CI, 296.4-300.5) for Black individuals, 374.8 (95% CI, 373.8-375.8) for White individuals, and 197.7 (95% CI, 195.6-199.9) for individuals from non-Hispanic other racial and ethnic groups. During the same time period, the female to male ratio was 2.9 overall. Age stratification in each of the racial and ethnic groups revealed the highest prevalence of MS in the 45- to 64-year-old age group, regardless of racial and ethnic classification. With each degree of latitude, MS prevalence increased by 16.3 cases per 100 000 (95% CI, 12.7-19.8; P < .001) in the unadjusted prevalence estimates, and 11.7 cases per 100 000 (95% CI, 7.4-16.1; P < .001) in the direct adjusted estimates. The association of latitude with prevalence was strongest in women, Black individuals, and older individuals. Conclusions and Relevance: This study found that White individuals had the highest MS prevalence followed by Black individuals, individuals from other non-Hispanic racial and ethnic groups, and Hispanic individuals. Inconsistent racial and ethnic classifications created heterogeneity within groups. In the United States, MS affects diverse racial and ethnic groups. Prevalence of MS increases significantly and nonuniformly with latitude in the United States, even when adjusted for race, ethnicity, age, and sex. These findings are important for clinicians, researchers, and policy makers.

Prevalence of amyotrophic lateral sclerosis in the United States using established and novel methodologies, 2017.

Objective:To estimate the prevalence of amyotrophic lateral sclerosis (ALS) in the United States for 2017 using data from the National ALS Registry (Registry) as well as capture-recapture methodology to account for under-ascertainment. Established in 2010, the Registry collects and examines data on ALS patients in the US to better describe the epidemiology of ALS (i.e. risk factor exposures, demographics).Methods: The Registry compiled data from national administrative databases (from the Centers for Medicare and Medicaid Services, the Veterans Health Administration, and the Veterans Benefits Administration) and a voluntary enrollment data through a web portal (www.cdc.gov/als). To estimate the number of missing cases, capture-recapture methodology was utilized.Results: The Registry conservatively identified 17,800 adult persons (lower-bound estimate) who met the Registry definition of ALS for an age-adjusted prevalence of 5.5 per 100,000 US population. Using capture-recapture methodology, we obtained a "mean case count" of 24,821 ALS cases (prevalence of 7.7 per 100,000 U.S. population) and estimated the upper-bound estimate to be 31,843 cases (prevalence of 9.9 per 100,000 U.S. population). The pattern of patient characteristics (e.g. age, sex, and race/ethnicity) remained unchanged from previous Registry reports. Overall, ALS was most common among whites, males, and persons aged 60-69 years. The age groups with the lowest number of cases were persons aged 18-39 years. Males had a higher prevalence than females overall and across all data sources.Conclusions: Existing Registry methodology, along with capture-recapture methodology, are being used to better describe the epidemiology and demographics of ALS in the US.

Cardiovascular Diseases, Medications, and ALS: A Population-Based Case-Control Study.

INTRODUCTION: We investigated the associations between antecedent all-cause CVD diagnoses, cause-specific CVD diagnosis, and CVD medication prescriptions with the risk of developing amyotrophic lateral sclerosis (ALS). MATERIALS AND METHODS: We conducted a population-based case-control study of U.S. Medicare enrollees from 2006 to 2013. The final sample included 3,714 incident ALS cases and 18,570 controls (matched on age, sex, enrollment length, and county). Information was collected from Medicare Parts A, B, and D administrative claims data on hypertension, ischemic heart disease, heart failure, acute myocardial infarction, atrial fibrillation, prescriptions of angiotensin-converting enzyme inhibitors, angiotensin II receptors blockers, calcium channel blockers, beta blockers, and antiarrhythmics. Associations were evaluated using conditional logistic regression adjusting for age, sex, race/ethnicity, geographical location, alcohol and tobacco use, and socioeconomic status. RESULTS: The odds ratio (OR) for having one or more ICD-9 codes for any cardiovascular disease diagnosis at least 24 months prior to the date of ALS diagnosis was 0.85 (95% confidence interval [CI]: 0.78-0.92). Cardiovascular conditions that were inversely associated with ALS included heart failure (OR = 0.79; 95% CI 0.70-0.89), atrial fibrillation (OR = 0.81; 95% CI 0.77-0.92), and hypertension (OR = 0.91; 95% CI 0.84-0.98). Exposures to several classes of cardiovascular medications were inversely associated with ALS risk even after adjusting for confounding by indication, including ACE inhibitors (OR = 0.84, 95% CI 0.77-0.91), calcium channel blockers (OR = 0.64, 95% CI 0.59-0.70), and beta blockers (OR = 0.76, 95% CI 0.71-0.83). DISCUSSION/CONCLUSION: These findings merit additional research, including animal studies and pilot clinical trials, to further evaluate and evidence the effects of ACEIs, CCBs, and BBs on the risk of developing and clinical expression of ALS.

Prevalence of amyotrophic lateral sclerosis (ALS), United States, 2016.

Objective: To estimate the prevalence of amyotrophic lateral sclerosis (ALS) in the United States for 2016 using data from the National ALS Registry (Registry). Established in 2009, the Registry collects data on ALS patients in the U.S. to better describe the epidemiology of ALS, examine risk factors such as environmental and occupational exposures, and characterize the demographics of those living with the disease. Methods: To identify adult prevalent cases of ALS, the Registry compiles data from three national administrative databases (maintained by the Centers for Medicare and Medicaid Services, the Veterans Health Administration, and the Veterans Benefits Administration). To ascertain cases not necessarily included in these databases and to better understand risk-factors associated with ALS and disease progression, the Registry also includes data collected from patients who voluntarily enroll via a web portal to complete online surveys. Results: In 2016, the Registry conservatively identified 16,424 adult persons who met the Registry definition of ALS for an age-adjusted prevalence rate of 5.2 per 100,000 U.S. population. The pattern of patient characteristics (e.g., age, sex, and race/ethnicity) has not changed from previous Registry reports. Overall, ALS was more common among whites, males, and persons aged 60-69 years. The age groups with the lowest number of ALS cases were persons aged 18-39 years. Males had a higher prevalence rate of ALS than females overall and across all data sources. Conclusions: Data collected by the National ALS Registry are being used to better describe the epidemiology and demographics of ALS in the U.S.

Evaluation of the Completeness of ALS Case Ascertainment in the US National ALS Registry: Application of the Capture-Recapture Method.

INTRODUCTION: The Centers for Disease Control and Prevention (CDC) National Amyotrophic Lateral Sclerosis (ALS) Registry is the first national registry for a chronic neurologic disease in the USA and uses a combination of case-finding methods including administrative healthcare data and patient self-registration. METHODS: We applied capture-recapture methodology to estimate the completeness of the Registry for ascertaining patients with ALS for the first full year and the fourth year of the Registry (2011, 2014). The Registry uses the combination of two national administrative claims databases (Medicare and Veterans Affairs) with a self-register option at the registry portal. We conducted descriptive analyses of the demographic and clinical characteristics of the ALS cases identified by each of the sources and estimated the completeness of case ascertainment for each of the three ALS Registry sources individually, pairwise, and in all combinations. RESULTS: Case-finding completeness was 54% in 2011 and improved to 56% in 2014. A smaller proportion of ALS patients under age 65 were ascertained than those 65 or older, and ascertainment was also lower for nonwhite than white patients. The uncorrected ALS prevalence was 4.3/100,000 in 2011 (in 2014, 5.0/100,000), but after correction for underascertainment, annual prevalence in 2011 was 7.9/100,000 (95% CI: 7.6-8.2) (in 2014 was 8.9/100,000 [95% CI: 8.7-9.2]). DISCUSSION/CONCLUSION: Our findings indicate that administrative healthcare databases are a very efficient method for identifying the majority of ALS prevalent cases in the National ALS Registry and that the inclusion of a web registry portal for patients to self-register is important to ensure a more representative population for estimating ALS prevalence. Nonetheless, more than 40% of ALS cases were not ascertained by the Registry, with individuals younger than age 65 and people of color underrepresented. Recommendations are provided for additional methods that can be considered to improve the completeness of case ascertainment.

The prevalence of MS in the United States: A population-based estimate using health claims data.

OBJECTIVE: To generate a national multiple sclerosis (MS) prevalence estimate for the United States by applying a validated algorithm to multiple administrative health claims (AHC) datasets. METHODS: A validated algorithm was applied to private, military, and public AHC datasets to identify adult cases of MS between 2008 and 2010. In each dataset, we determined the 3-year cumulative prevalence overall and stratified by age, sex, and census region. We applied insurance-specific and stratum-specific estimates to the 2010 US Census data and pooled the findings to calculate the 2010 prevalence of MS in the United States cumulated over 3 years. We also estimated the 2010 prevalence cumulated over 10 years using 2 models and extrapolated our estimate to 2017. RESULTS: The estimated 2010 prevalence of MS in the US adult population cumulated over 10 years was 309.2 per 100,000 (95% confidence interval [CI] 308.1-310.1), representing 727,344 cases. During the same time period, the MS prevalence was 450.1 per 100,000 (95% CI 448.1-451.6) for women and 159.7 (95% CI 158.7-160.6) for men (female:male ratio 2.8). The estimated 2010 prevalence of MS was highest in the 55- to 64-year age group. A US north-south decreasing prevalence gradient was identified. The estimated MS prevalence is also presented for 2017. CONCLUSION: The estimated US national MS prevalence for 2010 is the highest reported to date and provides evidence that the north-south gradient persists. Our rigorous algorithm-based approach to estimating prevalence is efficient and has the potential to be used for other chronic neurologic conditions.

Estimation of the Prevalence of Amyotrophic Lateral Sclerosis in the United States Using National Administrative Healthcare Data from 2002 to 2004 and Capture-Recapture Methodology.

BACKGROUND: National administrative healthcare data may be used as a case-finding method for prevalence studies of chronic disease in the United States, but the completeness of ascertainment likely varies depending on the disease under study. METHODS: We used 3 case-finding sources (Medicare, Medicaid, and Veterans Administration data) to estimate the prevalence of amyotrophic lateral sclerosis (ALS) in the United States for 2002-2004, and applied the capture-recapture methodology to estimate the degree of under-ascertainment when relying solely on these sources for case identification. RESULTS: Case-finding completeness was 76% overall and did not vary by race, but was lower for males (77%) than for females (88%), and lower for patients under age 65 (66%) than patients over age 65 (79%). The uncorrected ALS prevalence ratio was 2.8/100,000 in 2002, 3.3/100,000 in 2003, and 3.7/100,000 in 2004. After correcting for under-ascertainment, the annual prevalence increased by approximately 1 per 100,000 to 3.7/100,000 in 2002 (95% CI 3.66-3.80), 4.4/100,000 in 2003 (95% CI 4.34-4.50), and 4.8/100,000 in 2004 (95% CI 4.76-4.91). CONCLUSIONS: Federal healthcare claims databases ascertained are a very efficient method for identifying the majority of ALS-prevalent cases in the National ALS Registry, and may be enhanced by having patients self-register through the registry web portal.

A high-density genome-wide association screen of sporadic ALS in US veterans.

Following reports of an increased incidence of amyotrophic lateral sclerosis (ALS) in U.S. veterans, we have conducted a high-density genome-wide association study (GWAS) of ALS outcome and survival time in a sample of U.S. veterans. We tested ∼1.3 million single nucleotide polymorphisms (SNPs) for association with ALS outcome in 442 incident Caucasian veteran cases diagnosed with definite or probable ALS and 348 Caucasian veteran controls. To increase power, we also included genotypes from 5909 publicly-available non-veteran controls in the analysis. In the survival analysis, we tested for association between SNPs and post-diagnosis survival time in 639 Caucasian veteran cases with definite or probable ALS. After this discovery phase, we performed follow-up genotyping of 299 SNPs in an independent replication sample of Caucasian veterans and non-veterans (ALS outcome: 183 cases and 961 controls; survival: 118 cases). Although no SNPs reached genome-wide significance in the discovery phase for either phenotype, three SNPs were statistically significant in the replication analysis of ALS outcome: rs6080539 (177 kb from PCSK2), rs7000234 (4 kb from ZNF704), and rs3113494 (13 kb from LOC100506746). Two SNPs located in genes that were implicated by previous GWA studies of ALS were marginally significant in the pooled analysis of discovery and replication samples: rs17174381 in DPP6 (p = 4.4×10(-4)) and rs6985069 near ELP3 (p = 4.8×10(-4)). Our results underscore the difficulty of identifying and convincingly replicating genetic associations with a rare and genetically heterogeneous disorder such as ALS, and suggest that common SNPs are unlikely to account for a substantial proportion of patients affected by this devastating disorder.

Rural HIV-infected women's access to medical care: ongoing needs in California.

HIV-infected women living in rural areas often have considerably less access to care than their urban and suburban counterparts. In much of the USA, little is known about HIV care among rural populations. This study elucidated barriers to care for rural women in California. Methods included retrospective structured interviews conducted with 64 women living in rural areas and receiving HIV care at 11 California healthcare facilities. Facilities were randomly sampled and all HIV-infected female patients seeking care at those facilities during a specified time period were eligible. The most commonly cited barriers to accessing care included physical health problems that prevented travel to care (32.8%), lack of transportation (31.2%), and lack of ability to navigate the healthcare system (25.0%). Being divorced/separated/widowed (compared to being either married or single) was associated with reporting physical health as a barrier to care (p=0.03); being unemployed (p=0.003) or having to travel 31-90 minutes (p=0.007, compared to less than 31 or greater than 90) were both associated with transportation as a barrier; and speaking English rather than Spanish was associated with reporting "difficulty navigating the system" (p=0.04). Twenty-nine women (45.3%) reported difficulty in traveling to appointments. Overall, 24 (37.5%) women missed an HIV medical appointment in the previous 12-month period, primarily due to their physical health and transportation limitations. Physical health and transportation problems were both the major barriers to accessing health services and the primary reasons for missing HIV care appointments among this population of HIV-infected women living in rural areas. Providing transportation programs and/or mobile clinics, as well as providing support for patients with physical limitations, may be essential to improving access to HIV care in rural areas.

Attenuation of social class and reproductive risk factor associations for Hodgkin lymphoma due to selection bias in controls.

OBJECTIVE: Hodgkin lymphoma (HL) risk has been linked with higher social class and lower parity, but our prior population-based case-control study in adult women had unexpected null findings for these variables. Because subject participation was 87% for cases but 65% for random digit-dialing (RDD) controls, we examined representativeness of our controls and the impact of detected bias on prior results. METHODS: Using data from RDD enumeration, abbreviated interviews with nonparticipating controls, and the US census, we compared participating and nonparticipating RDD controls across several age groups and then recomputed odds ratios for risk factor associations adjusted for bias. RESULTS: The 325 RDD control participants were younger, more likely to be white, better educated, and of lower birth order and lower parity than the nonparticipants. Adjustment of odds ratios for bias strengthened previously null findings for education and for parity, breast-feeding and miscarriages in young adult women; these latter changes eliminated previously apparent age modification of risks. CONCLUSIONS: Selection bias in female RDD controls resulted from differential participation by socioeconomic factors, varied with age, and produced underestimations of several associations in young women, including reproductive factors. Thus, our prior conclusions of etiologic irrelevance for some study variables may have been inaccurate.

Differences in treatment patterns for localized breast carcinoma among Asian/Pacific islander women.

BACKGROUND: Many studies have examined racial/ethnic differences in treatment for localized breast carcinoma, but to the authors' knowledge few have included Asian/Pacific Islander (API) women. METHODS: The population-based study included API and non-Hispanic white women diagnosed with localized invasive breast carcinoma in the Greater San Francisco Bay Area during 1994 (n = 1772). Multiple logistic regression was used to assess the association between race/ethnicity and type of surgery, radiation therapy following breast-conserving surgery (BCS), and hormone therapy for estrogen receptor-positive tumors while adjusting for demographic, medical, and census block-group socioeconomic characteristics. RESULTS: API women were significantly more likely to undergo mastectomies than white women (58% vs. 42%). This difference remained for Chinese and Filipino women after multivariate adjustment (odds ratio vs. whites [OR] = 2.4, 95% confidence interval [95% CI] = 1.4-4.2; OR [95%CI] = 1.8[1.0-3.1], respectively). Chinese women were also more likely than white women to not receive adjuvant therapy, be it radiation after BCS or hormone therapy for estrogen receptor-positive disease. Other API women did not differ from white women in adjuvant therapy use. CONCLUSIONS: This population-based study identified differences in treatment for localized breast carcinoma by race/ethnicity that were not explained by differences in demographic, medical, or socioeconomic characteristics. These results underscore the importance of looking at treatment patterns separately for API subgroups and support the need for research into cultural differences that may influence breast carcinoma treatment choices.

Increase in breast cancer incidence in middle-aged women during the 1990s.

PURPOSE: The San Francisco Bay Area has a history of high breast cancer incidence rates relative to the rest of the United States. For Marin County, where Bay Area rates are highest and, moreover, have continued to increase over time, age- and tumor-specific incidence trends were compared with the rest of the region. METHODS: The study included all white women diagnosed with invasive breast cancer in 1988 to 1997 in the five-county Bay Area (N = 19807). Annual age-specific incidence rates and estimated annual percent changes (EAPCs) were calculated for women ages less than 45, 45 to 64, and greater than or equal to age 65. RESULTS: Women aged 45 to 64 from Marin County experienced a marked increase in breast cancer rates between 1991 and 1997 (EAPC = 8%, p = 0.02), regardless of disease stage or tumor histology. For the youngest and oldest women, no rate differences were observed by region or over time. CONCLUSIONS: This regional difference in trend by age did not appear to be due to screening mammography or environmental exposures. Cohort exposures to breast cancer risk factors, such as oral contraceptive and/or hormone replacement therapy use, may have contributed to these rate increases. Although the reasons remain unclear, the finding may signal a rising risk of breast cancer in this demographic group.