Genotypes and phenotypes of motor neuron disease: an update of the genetic landscape in Scotland.

BACKGROUND: Using the Clinical Audit Research and Evaluation of Motor Neuron Disease (CARE-MND) database and the Scottish Regenerative Neurology Tissue Bank, we aimed to outline the genetic epidemiology and phenotypes of an incident cohort of people with MND (pwMND) to gain a realistic impression of the genetic landscape and genotype-phenotype associations. METHODS: Phenotypic markers were identified from the CARE-MND platform. Sequence analysis of 48 genes was undertaken. Variants were classified using a structured evidence-based approach. Samples were also tested for C9orf72 hexanucleotide expansions using repeat-prime PCR methodology. RESULTS: 339 pwMND donated a DNA sample: 44 (13.0%) fulfilled criteria for having a pathogenic variant/repeat expansion, 53.5% of those with a family history of MND and 9.3% of those without. The majority (30 (8.8%)) had a pathogenic C9orf72 repeat expansion, including two with intermediate expansions. Having a C9orf72 expansion was associated with a significantly lower Edinburgh Cognitive and Behavioural ALS Screen ALS-Specific score (p = 0.0005). The known pathogenic SOD1 variant p.(Ile114Thr), frequently observed in the Scottish population, was detected in 9 (2.7%) of total cases but in 17.9% of familial cases. Rare variants were detected in FUS and NEK1. One individual carried both a C9orf72 expansion and SOD1 variant. CONCLUSIONS: Our results provide an accurate summary of MND demographics and genetic epidemiology. We recommend early genetic testing of people with cognitive impairment to ensure that C9orf72 carriers are given the best opportunity for informed treatment planning. Scotland is enriched for the SOD1 p.(Ile114Thr) variant and this has significant implications with regards to future genetically-targeted treatments.

Genotype-phenotype characterisation of long survivors with motor neuron disease in Scotland.

BACKGROUND: We investigated the phenotypes and genotypes of a cohort of 'long-surviving' individuals with motor neuron disease (MND) to identify potential targets for prognostication. METHODS: Patients were recruited via the Clinical Audit Research and Evaluation for MND (CARE-MND) platform, which hosts the Scottish MND Register. Long survival was defined as > 8 years from diagnosis. 11 phenotypic variables were analysed. Whole genome sequencing (WGS) was performed and variants within 49 MND-associated genes examined. Each individual was screened for C9orf72 repeat expansions. Data from ancestry-matched Scottish populations (the Lothian Birth Cohorts) were used as controls. RESULTS: 58 long survivors were identified. Median survival from diagnosis was 15.5 years. Long survivors were significantly younger at onset and diagnosis than incident patients and had a significantly longer diagnostic delay. 42% had the MND subtype of primary lateral sclerosis (PLS). WGS was performed in 46 individuals: 14 (30.4%) had a potentially pathogenic variant. 4 carried the known SOD1 p.(Ile114Thr) variant. Significant variants in FIG4, hnRNPA2B1, SETX, SQSTM1, TAF15, and VAPB were detected. 2 individuals had a variant in the SPAST gene suggesting phenotypic overlap with hereditary spastic paraplegia (HSP). No long survivors had pathogenic C9orf72 repeat expansions. CONCLUSIONS: Long survivors are characterised by younger age at onset, increased prevalence of PLS and longer diagnostic delay. Genetic analysis in this cohort has improved our understanding of the phenotypes associated with the SOD1 variant p.(Ile114Thr). Our findings confirm that pathogenic expansion of C9orf72 is likely a poor prognostic marker. Genetic screening using targeted MND and/or HSP panels should be considered in those with long survival, or early-onset slowly progressive disease, to improve diagnostic accuracy and aid prognostication.

Conceptualizing Municipal Elections: The Case of Toronto 2018.

Since Angus Campbell and colleagues first introduced the "levels of conceptualization" (LoC) framework as a measure of political sophistication, a number of scholars have applied the approach to subsequent American national elections. In this study, we present the first application of the LoC framework to a municipal election, and focus upon the 2018 Toronto mayoral race. After describing the method and data we use to adapt the framework to this new context, we replicate previous analyses, and find that LoC is related to local voter turnout and several measures of political sophistication. We then consider the question of whether major candidates were discussed at different LoC, and if their supporters view local politics at different LoC. We conclude by making the case that the LoC framework is helpful for resolving the debate over whether local politics are ideological or managerial in nature.

Improving Hearing Protection Device Noise Attenuation Through Fit-Testing in an Occupational Health Clinic.

BACKGROUND: Occupational noise-induced hearing loss (NIHL) is the second most common medical disability for military veterans and accounts for 24% of all hearing loss in the United States. Hearing loss negatively affects health and billions of dollars are spent annually for direct and indirect medical costs and lost work productivity. Proper hearing protection device (HPD) fit can prevent NIHL. Quantitative fit-testing systems to determine personal attenuation rates (PARs) are available, but not widely utilized. Without quantitative testing, validating appropriate fit and attenuation to a permissible exposure level to prevent NIHL is difficult. METHODS: A quality improvement project measured 100 employees enrolled in a hearing conservation program (HCP) to determine if they obtained a target PAR after inserting the HPDs in their "usual" fashion. Those who did not obtain target PAR (n = 27) received an evidence-based intervention that demonstrated proper HPD fit by a skilled trainer followed by a repeat attempt by the participant to replicate the feel of the demonstrated HPD placement. PAR was subsequently measured to assess for appropriate noise attenuation. FINDINGS: Seventy-one percent achieved target PAR (≥ 20 decibels) at baseline testing. After the intervention, 91 of 100 participants had achieved target PAR, a 20% improvement. CONCLUSIONS/IMPLICATIONS FOR PRACTICE: Findings suggest this evidence-based intervention was an effective strategy for improving HPD fit for employees enrolled in HCPs. This intervention may prevent occupational hearing loss and decrease the health and socioeconomic impacts of NIHL.

Technology clinical trials: Turning innovation into patient benefit.

Health care needs to continuously evolve and innovate to maintain the health of populations. Technology has the potential to enable better patient engagement and ownership, as well as optimise therapeutic interventions and data-science approaches to facilitate improved health care decisions. Yet, to date, technological innovation has not resulted in the rate of change that could have been predicted from other sectors. This article discusses multiple reasons for this and proposes a newly tested and deployed solution: the technology clinical trial. The technology clinical trial methodology has been developed through working directly with patients, clinical and medical devicetrial experts. This approach enables researchers to use the complex environment of health care as an opportunity to transform the pace of innovation and create new care pathways. Instead of testing a single innovation, researchers can 'step back' and systematically review all areas of the patient's journey for potential optimization. Then integrate novel data science, technological advances, process updates, behavioural science, and patient engagement to co-create a streamlined multidisciplinary solution. As a result, this research has the potential for larger advances due to the emergent benefits that can arise when the individual elements work together as a whole. These potential benefits are then robustly tested, characterised and measured in the trial environment to ensure that future application of the innovative pathway is supported by the robust empirical data health care requires.

The brief Dimensional Apathy Scale: A short clinical assessment of apathy.

Objective: Apathy is a prominent syndrome across neurodegenerative diseases. The Dimensional Apathy Scale (DAS) assesses three apathy subtypes-executive, emotional, and initiation-and is sensitive and valid in amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD), and Parkinson's disease. This study describes the development of the brief DAS (b-DAS), which will enable apathy to be swiftly detected in the clinic.Method: 102 ALS and 102 AD patients' previously collected data were used. Mokken analyses were performed on item-level data of each informant/carer-rated DAS subscale (executive, emotional, and initiation) for the initial scale reduction. Item-total correlational analyses against standard apathy (convergent validity criteria) and depression (divergent validity criteria) measures and qualitative examination of items aided final item selection. Receiver operating curve analysis determined optimal cutoffs for the reduced subscales.Results: Mokken analyses suggested unidimensionality of each DAS subscale. Three items were removed that failed to satisfy monotone homogeneity model requirements, three items were removed due to validity criteria not being met, and six items were removed due to a combination of lower item scalability and item-total correlations. Item-theme examination further reduced the b-DAS to nine items, three per subscale, with a supplemental awareness deficit assessment being added. Sensitivity- and specificity-based optimal cutoffs were calculated for each b-DAS subscale.Conclusions: This study presents the b-DAS, an informant/carer-based robust yet short multidimensional apathy instrument with good convergent and divergent validity, with recommended clinical cutoffs. The b-DAS is appropriate for use in the clinic and for research to quickly and comprehensively screen for apathy subtype impairments.

Clinical audit research and evaluation of motor neuron disease (CARE-MND): a national electronic platform for prospective, longitudinal monitoring of MND in Scotland.

Objectives: Launched in 1989, the Scottish Motor Neuron Disease Register (SMNDR) has provided a resource for prospective clinical data collection. However, in 2015 we aimed to evolve a system to allow: i) A patient-centered approach to care based on recognized standards, ii) Harmonized data sharing between Scottish health professionals in "real-time", iii) Regular audit of care to facilitate timely improvements in service delivery, and iv) Patient participation in a diverse range of observational and interventional research studies including clinical trials. Methods: We developed a standardized national electronic data platform-Clinical Audit Research and Evaluation of MND (CARE-MND) which integrates clinical audit and research data fields. Data completion pre- and post-CARE-MND were compared, guided by recently published National Institute for Clinical Excellence (NICE) recommendations. Statistical difference in data capture between time periods was assessed using Z-test of proportions. Results: Data field completion for the historical 2011-2014 period ranged from 4 to 95%; median 50%. CARE-MND capture ranged from 32 to 98%; median 87%. 15/17 fields were significantly more complete post-CARE-MND (p < 0.001). All MND nurse/allied health specialists in Scotland use the CARE-MND platform. Management of MND in Scotland is now coordinated through a standardized template. Conclusions: Through CARE-MND, national audits of MND care and interventions have been possible, leading to protocols for harmonized service provision. Stratification of the MND population is facilitating participation in observational and interventional studies. CARE-MND can act as a template for other neurological disorders.

Changing epidemiology of motor neurone disease in Scotland.

OBJECTIVES: Scotland benefits from an integrated national healthcare team for motor neurone disease (MND) and a tradition of rich clinical data capture using the Scottish MND Register (launched in 1989; one of the first national registers). The Scottish register was re-launched in 2015 as Clinical Audit Research and Evaluation of MND (CARE-MND), an electronic platform for prospective, population-based research. We aimed to determine if incidence of MND is changing over time. METHODS: Capture-recapture methods determined the incidence of MND in 2015-2016. Incidence rates for 2015-2016 and 1989-1998 were direct age and sex standardised to allow time-period comparison. Phenotypic characteristics and socioeconomic status of the cohort are described. RESULTS: Coverage of the CARE-MND platform was 99%. Crude incidence in the 2015-2017 period was 3.83/100,000 person-years (95% CI 3.53-4.14). Direct age-standardised incidence in 2015 was 3.42/100,000 (95% CI 2.99-3.91); in 2016, it was 2.89/100,000 (95% CI 2.50-3.34). The 1989-1998 direct standardised annual incidence estimate was 2.32/100,000 (95% CI 2.26-2.37). 2015-2016 standardised incidence was 66.9% higher than Northern European estimates. Socioeconomic status was not associated with MND. CONCLUSIONS: Our data show a changing landscape of MND in Scotland, with a rise in incidence by 36.0% over a 25-year period. This is likely attributable to ascertainment in the context of improved neurological services in Scotland. Our data suggest that CARE-MND is a reliable national resource and findings can be extrapolated to the other Northern European populations.

ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS.

OBJECTIVE: To elucidate the relationship between disease stage in amyotrophic lateral sclerosis (ALS), as measured with the King's Clinical Staging System, and cognitive and behavioral change, measured with the Edinburgh Cognitive and Behavioural ALS Screen (ECAS). METHODS: A large multicenter observational cohort of 161 cross-sectional patients with ALS and 80 healthy matched controls were recruited across 3 research sites (Dublin, Edinburgh, and London). Participants were administered the ECAS and categorized into independent groups based on their King's clinical disease stage at time of testing. RESULTS: Significant differences were observed between patients and controls on all subtests of the ECAS except for visuospatial functioning. A significant cross-sectional effect was observed across disease stages for ALS-specific functions (executive, language, letter fluency) and ECAS total score but not for ALS-nonspecific functions (memory, visuospatial). Rates of ALS-specific impairment and behavioral change were also related to disease stage. The relationship between cognitive function and disease stage may be due to letter fluency impairment, whereas higher rates of all behavioral domains were seen in later King's stage. The presence of bulbar signs, but not site of onset, was significantly related to ALS-specific, ECAS total, and behavioral scores. CONCLUSION: ALS-specific cognitive deficits and behavioral impairment are more frequent with more severe disease stage. By end-stage disease, only a small percentage of patients are free of neuropsychological impairment. The presence of bulbar symptoms exaggerates the differences observed between disease stages. These findings suggest that cognitive and behavioral change should be incorporated into ALS diagnostic criteria and should be included in future staging systems.

Validation of a Collaboration Readiness Assessment Tool for Use by Supplemental Nutrition Assistance Program-Education (SNAP-Ed) Agencies and Partners.

OBJECTIVE: To evaluate content and face validity of a collaboration readiness assessment tool developed to facilitate collaborative efforts to implement policy, systems, and environment changes in Supplemental Nutrition Assistance Program-Education (SNAP-Ed). METHODS: Evaluation of the validity of the tool involved 2 steps. Step 1 was conducted with 4 subject matter experts to evaluate content validity. Step 2 used an iterative cognitive testing process with 4 rounds and 16 SNAP-Ed staff and community partners to evaluate face validity. RESULTS: Subject matter experts found that survey items appropriately matched the content area indicated and adequately covered collective efficacy, change efficacy, and readiness. Cognitive testing with SNAP-Ed staff and partners informed modifications and resulted in adequate face validity. CONCLUSIONS AND IMPLICATIONS: The ability to measure collaboration readiness will allow agencies and community partners that implement SNAP-Ed to target areas that facilitate collaboration efforts needed for policy, systems, and environment change and collective efficacy. Further cognitive testing of the tool with other populations is needed to ensure its applicability and usefulness. Evaluation of the reliability of the tool with a broad range of SNAP-Ed programs and community agencies is also recommended.

Multidimensional apathy and executive dysfunction in amyotrophic lateral sclerosis.

Apathy and cognitive dysfunction are prominent symptoms of Amyotrophic lateral sclerosis (ALS). More specifically ALS patients show increased Initiation apathy-a lack of motivation for self-generation of thoughts as assessed by the Dimensional Apathy Scale. This study aimed to investigate the cognitive underpinnings of apathy subtypes in ALS. We hypothesized that increased Initiation apathy would be associated deficits on tests of intrinsic response generation, such as verbal fluency. We also explored the relationship of other apathy subtypes to cognitive processes, in particular emotional apathy with emotional and social cognition deficits and executive apathy with planning and goal management deficits. ALS patients, and their carers (N = 30), and healthy matched controls, and their informants (N = 29) were recruited. All participants completed self- and informant/carer-rated Dimensional Apathy Scale, to quantify apathy subtypes (Executive, Emotional and Initiation), along with standard apathy and depression measures. Patients and controls completed the Edinburgh Cognitive and behavioural ALS Screen, and a comprehensive neuropsychological battery including emotional recognition, social cognition, intrinsic response generation tasks (verbal fluency and random number generation) and a new ecologically valid, computerised measure of planning and goal management. The results demonstrated that increased Initiation apathy was the only significantly elevated subtype in ALS (self-rated p < .05, informant/carer-rated p < .01). Initiation apathy was found to be significantly associated with verbal fluency deficit, while Emotional apathy was significantly associated with emotional recognition deficits. No associations were found between apathy subtypes and depression or in controls. This is the first study to show specific associations between apathy subtypes (Emotional and Initiation) and executive and emotional cognitive dysfunction, indicating possible distinct underlying mechanisms to these demotivational symptoms.

Genetic epidemiology of motor neuron disease-associated variants in the Scottish population.

Genetic understanding of motor neuron disease (MND) has evolved greatly in the past 10 years, including the recent identification of association between MND and variants in TBK1 and NEK1. Our aim was to determine the frequency of pathogenic variants in known MND genes and to assess whether variants in TBK1 and NEK1 contribute to the burden of MND in the Scottish population. SOD1, TARDBP, OPTN, TBK1, and NEK1 were sequenced in 441 cases and 400 controls. In addition to 44 cases known to carry a C9orf72 hexanucleotide repeat expansion, we identified 31 cases and 2 controls that carried a loss-of-function or pathogenic variant. Loss-of-function variants were found in TBK1 in 3 cases and no controls and, separately, in NEK1 in 3 cases and no controls. This study provides an accurate description of the genetic epidemiology of MND in Scotland and provides support for the contribution of both TBK1 and NEK1 to MND susceptibility in the Scottish population.

Improved PCR based methods for detecting C9orf72 hexanucleotide repeat expansions.

Due to the GC-rich, repetitive nature of C9orf72 hexanucleotide repeat expansions, PCR based detection methods are challenging. Several limitations of PCR have been reported and overcoming these could help to define the pathogenic range. There is also a need to develop improved repeat-primed PCR assays which allow detection even in the presence of genomic variation around the repeat region. We have optimised PCR conditions for the C9orf72 hexanucleotide repeat expansion, using betaine as a co-solvent and specific cycling conditions, including slow ramping and a high denaturation temperature. We have developed a flanking assay, and repeat-primed PCR assays for both 3' and 5' ends of the repeat expansion, which when used together provide a robust strategy for detecting the presence or absence of expansions greater than ∼100 repeats, even in the presence of genomic variability at the 3' end of the repeat. Using our assays, we have detected repeat expansions in 47/442 Scottish ALS patients. Furthermore, we recommend the combined use of these assays in a clinical diagnostic setting.

Multidimensional apathy in ALS: validation of the Dimensional Apathy Scale.

AIM: Apathy is a prominent symptom of amyotrophic lateral sclerosis (ALS), but measurement is confounded by physical disability. Furthermore, it has been traditionally measured as a unidimensional symptom despite research demonstrating a multifaceted construct. The new Dimensional Apathy Scale (DAS) has been specifically designed for patients with motor disability to measure 3 neurologically based subtypes of apathy: Executive, Emotional and Initiation. We aimed to explore this behavioural symptom by examining the substructure of apathy in ALS and to determine the reliability and validity of the DAS in patients and their carers. METHOD: Patients and carers were recruited through the national Scottish Motor Neurone Disease Register and were asked to complete the DAS, the standardised Apathy Evaluation Scale, and the Geriatric Depression Scale-Short Form. 83 patients with ALS, 75 carers and 83 sex-matched, age-matched and education-matched controls participated. RESULTS: When compared with healthy controls, patients showed a significant increase in apathy on the Initiation subscale, and were significantly less apathetic on the Emotional subscale. Scores on the DAS patient and carer versions did not significantly differ. Internal consistency reliability, convergent and discriminant validity were found to be good for the DAS subscales. There was no association between the DAS and functional disability using the ALS Functional Rating Scale. CONCLUSIONS: Apathy in ALS is characterised by a specific profile of increased initiation apathy and reduced emotional apathy. The DAS is a reliable and valid measure for the assessment of multidimensional apathy in ALS.

Look local: the value of cancer surveillance and reporting by American Indian clinics.

INTRODUCTION: Cancer incidence and mortality rates for American Indians in the Northern Plains region of the United States are among the highest in the nation. Reliable cancer surveillance data are essential to help reduce this burden; however, racial data in state cancer registries are often misclassified, and cases are often underreported. METHODS: We used a community-based participatory research approach to conduct a retrospective ascertainment of cancer cases in clinic medical records over a 9-year period (1995-2003) and compared the results with the state cancer registry to evaluate missing or racially misclassified cases. Six tribal and/or urban Indian clinics participated in the study. The project team consisted of participating clinics, a state cancer registry, a comprehensive cancer center, an American Indian/Alaska Native Leadership Initiative on Cancer, and a set of diverse organizational partners. Clinic personnel were trained by project staff to accurately identify cancer cases in clinic records. These records were then matched with the state cancer registry to assess misclassification and underreporting. RESULTS: Forty American Indian cases were identified that were either missing or misclassified in the state registry. Adding these cases to the registry increased the number of American Indian cases by 21.3% during the study period (P = .05). CONCLUSIONS: Our results indicate that direct reporting of cancer cases by tribal and urban Indian health clinics to a state cancer registry improved the quality of the data available for cancer surveillance. Higher-quality data can advance the efforts of cancer prevention and control stakeholders to address disparities in Native communities.

Long-term outcome of patients with a negative work-up for asymptomatic microhematuria.

OBJECTIVES: To assess the validity of the American Urological Association guidelines, we investigated 14-year outcomes of men aged > or = 50 years who had hematuria detected in a bladder cancer (BC) screening trial, were thoroughly evaluated, and were not found to have urological cancers. The American Urological Association guidelines for follow-up of adults with asymptomatic microhematuria (MH) who have negative evaluations include repeat urinary cytologies, urinalyses, and office visits for several years, primarily to detect BC (Cohen and Brown, N Engl J Med 348: 2330-2338, 2003; and Grossfeld et al, Urology 57:604-610, 2001). METHODS: Of 1575 screening participants, 258 had MH detected by daily home testing with the Ames hemastix during two 14-day periods. This test has been shown to accurately reflect MH on microscopic urinalysis when each is correctly performed. Any man with at least 1 positive test (> or = "trace") underwent a complete evaluation including microscopic urinalysis, culture, cytology, complete blood count, serum creatinine, coagulation profile, intravenous urography or computed tomography scan, and cystoscopy. BC or other urological tumors was not detected in 234 participants. Using Wisconsin state tumor registry and death certificate data, the outcomes of these men were tracked for 14 years since their last testing. RESULTS: Two of the 234 men (0.85%) developed BC during the 14-year follow-up, at 6.7 and 11.4 years after their negative evaluations; one died of BC 7.6 years after his last screening. During this follow-up, 0.93% of the screenees who tested negatively for hematuria had BC diagnosed, none within a year of their last testing date. CONCLUSIONS: Patients who have negative complete evaluations for asymptomatic MH have little chance of subsequently developing BC. The recommended "appropriate" follow-up for these patients may require reconsideration in light of these data.

Grade and stage at presentation do not predict mortality in patients with bladder cancer who survive their disease.

PURPOSE: Our goal was to determine whether presenting grade and stage of bladder cancer (BC), which directly affect disease-specific survival, also influence time to and cause of non-BC deaths. METHODS: Histology slides of all men who lived in Wisconsin age > or = 50 years diagnosed with BC in 1988 were reviewed centrally, and time and cause of death as reported to the state's tumor registry were recorded. Competing risks analyses based on grade, tumor stage, and age at diagnosis were generated to correlate time and causes of death (BC or non-BC) with tumor histology and age at presentation. RESULTS: Grade-stage categories were assigned to 509 patients with BC as follows: LGN = low grade (grade 1 or 2), nonmuscle invading (stage Ta or T1); HGN = high grade (grade 3 or carcinoma in situ), nonmuscle invading (stage Ta, T1, or TIS); and INV = any grade, muscle invasive (> or = stage T2). Three hundred nine patients (60.7%) were LGN, 80 (15.7%) were HGN, and 120 (23.6%) were INV. Grade-stage category predicted overall (P = .0001) and BC-specific (P < .0001) mortality but not non-BC mortality (P = .72), with hazard ratios of 1.095 (95% CI, 0.783 to 1.531) for HGN versus LGN, 1.137 (95% CI, 0.799 to 1.617) for INV versus LGN, and 1.038 (95% CI, 0.670 to 1.607) for INV versus HGN. Age had a highly significant effect on overall and non-BC deaths (P < .0001) but only marginally predicted BC deaths (P = .054). Time to non-BC death did not differ significantly between grade-stage category (P = .12) or cause of death (P = .81). CONCLUSION: Grade-stage category at diagnosis predicts overall and BC mortality but not mortality from other causes. Thus, particularly for INV disease, because BC represents the major threat to life, aggressive therapies that have been shown to be effective are justified.

Lessons learned from a community-based participatory research project to improve American Indian cancer surveillance.

BACKGROUND: American Indian and Alaska Native cancer incidence data are limited by underreporting and misclassification. These populations also suffer from a history of research abuse. OBJECTIVES: The project's goal was to use community-based participatory research (CBPR) to assess the local burden of cancer in the American Indian communities in Wisconsin and assess the accuracy of Wisconsin American Indian cancer data. METHODS: Thirteen organizations partnered to conduct a retrospective review of American Indian clinics cancer cases. A match of the clinic identified cases with Wisconsin Cancer Reporting System records was then conducted. LESSONS LEARNED: Relationship building, mutual education, and local engagement in data interpretation were significant factors in this project achieving its objectives and laying a foundation for future research partnerships. CONCLUSIONS: This project demonstrates the successful application of CBPR in a complex multisite project with multiple partners using collective resources to address cancer health disparities.

Improving cancer incidence estimates for American Indians in Wisconsin.

OBJECTIVES: The purpose of this study was to improve the measurement of cancer incidence among American Indians in Wisconsin and compare incidence rates with state and national incidence rates. METHODS: The Wisconsin Cancer Reporting System (WCRS) entered into a data linkage project with CDC and the Indian Health Service (IHS) to improve classification of American Indian cancer cases in Wisconsin. WCRS data were linked to IHS patient registration files to identify American Indian cases that were misclassified as a non-Indian race for the years 1998-2002. American Indian age-adjusted rates and rate ratios for major cancer sites were compared before and after the linkage, and with statewide and national rates. RESULTS: The age-adjusted incidence rate for all cancer among American Indians increased from the pre-linkage rate of 386.3 per 100,000 to the post-linkage rate of 471.7 per 100,000, a statistically significant increase. The post-linkage rate was over twice the comparable Surveillance Epidemiology and End Results (SEER) national rate among American Indians at 233.6 per 100,000. Post-linkage American Indian incidence rates for male colorectal and female lung cancers were higher than those for the state average. CONCLUSIONS: In contrast to earlier data, the linkage results show that American Indians had similar cancer incidence compared to the general population in Wisconsin, and over twice as high as national SEER American Indian rates. Post-linkage rates resulted in more accurate site-specific and geographically focused cancer incidence rates to help target the national and state priorities of addressing disparities among American Indians.