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OBJECTIVE: Amyotrophic Lateral Sclerosis (ALS) is a heterogeneous neurodegenerative condition featuring variable degrees of motor and cognitive impairment. We assessed the impact of specific, empirically derived occupational skills and requirements on cognitive and motor functioning in ALS. METHODS: Individuals with ALS (n = 150) were recruited from the University of Pennsylvania's Comprehensive ALS Clinic. The Edinburgh Cognitive and Behavioral ALS Screen (ECAS) measured cognition, and the Penn Upper Motor Neuron (PUMNS) and ALS Functional Rating Scales (ALSFRS-R) measured motor symptoms. We derived 17 factors representing distinct occupational skills and requirements from the Occupational Information Network (O*NET), which were related to cognitive and motor scores using multiple linear regression. RESULTS: Occupational roles involving greater reasoning ability (ß = 2.12, p < .05), social ability (ß = 1.73, p < .05), analytic skills, (ß = 3.12, p < .01) and humanities knowledge (ß = 1.83, p<.01) were associated with better performance on the ECAS, while jobs involving more exposure to environmental hazards (ß=-2.57, p < .01) and technical skills (ß=-2.16, p<.01) were associated with lower ECAS scores. Jobs requiring more precision skills (ß = 1.91, p < .05) were associated with greater motor dysfunction on the PUMNS. CONCLUSIONS: Occupational histories involving more cognitively complex skills and activities were related to preserved cognitive functioning in ALS consistent with the cognitive reserve hypothesis, while jobs with greater exposure to environmental hazards and technical demands were linked to poorer cognitive functioning. Jobs involving more repetitive movements were associated with worse motor functioning, possibly due to overuse. Occupational history provides insight into protective and risk factors for variable degrees of cognitive and motor dysfunction in ALS.
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Importance: Prior research suggests there are racial disparities in the presentation of dementia, but this has not been investigated in the context of frontotemporal dementia (FTD). Objective: To explore racial disparities in dementia severity, functional impairment, and neuropsychiatric symptoms in individuals with a diagnosis of FTD. Design, Setting, and Participants: This exploratory cross-sectional study of National Alzheimer's Coordinating Center (NACC) data collected between June 2005 to August 2021 evaluated Asian, Black, and White individuals with a diagnosis of FTD (behavioral variant FTD or primary progressive aphasia). Excluded were races with limited data, including American Indian or Alaska Native (n = 4), Native Hawaiian or other Pacific Islander (n = 3), other (n = 13), and unknown (n = 24), and participants with symptom duration more than 4 SDs above the mean. Main Outcomes and Measures: Racial differences at initial NACC visit were examined on Clinical Dementia Rating Dementia Staging Instrument plus NACC Frontotemporal Lobar Degeneration Behavior & Language Domains (FTLD-CDR), Functional Assessment Scale, and Neuropsychiatric Inventory using regression models. Matching was also performed to address the imbalance between racial groups. Results: The final sample comprised 2478 individuals, of which 59 (2.4%) were Asian, 63 (2.5%) were Black, and 2356 (95.1%) were White. The mean (SD) age at initial visit was 65.3 (9.4) years and symptom duration at initial visit was 67.5 (35.6) months. Asian and Black individuals were considerably underrepresented, comprising a small percent of the sample. Black individuals had a higher degree of dementia severity on FTLD-CDR (ß = 0.64; SE = 0.24; P = .006) and FTLD-CDR sum of boxes (ß = 1.21; SE = 0.57; P = .03) and greater functional impairment (ß = 3.83; SE = 1.49; P = .01). There were no differences on FTLD-CDR and Functional Assessment Scale between Asian and White individuals. Black individuals were found to exhibit a higher frequency of delusions, agitation, and depression (delusions: odds ratio [OR], 2.18; 95% CI, 1.15-3.93; P = .01; agitation: OR, 1.73; 95% CI, 1.03-2.93; P = .04; depression: OR, 1.75; 95% CI, 1.05-2.92; P = .03). Asian individuals were found to exhibit a higher frequency of apathy (OR, 1.89; 95% CI, 1.09-3.78; P = .03), nighttime behaviors (OR, 1.72; 95% CI, 1.01-2.91; P = .04), and appetite/eating (OR, 1.99; 95% CI, 1.17-3.47; P = .01) compared to White individuals. Conclusions and Relevance: This exploratory study suggests there are racial disparities in dementia severity, functional impairment, and neuropsychiatric symptoms. Future work must address racial disparities and their underlying determinants as well as the lack of representation of racially minoritized individuals in nationally representative dementia registries.