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Introduction Our research aim was to develop a novel clinimetric scale sensitive enough to detect disease progression in primary lateral sclerosis (PLS). Methods A prototype of the PLS Functional Rating Scale (PLSFRS) was generated. Seventy‐seven participants with PLS were enrolled and evaluated at 21 sites that comprised the PLSFRS study group. Participants were assessed using the PLSFRS, Neuro‐Quality of Life (QoL), Schwab‐England Activities of Daily Living (ADL), and the Clinical Global Impression of Change scales. Participants completed telephone assessments at 12, 24, and 48 weeks after enrollment. Results The PLSFRS demonstrated internal consistency as well as intrarater, interrater, telephone test‐retest reliability, and construct validity. Significant changes in disease progression were detected at 6 and 12 months; changes measured by the PLSFRS vs the ALSFRS‐R were significantly higher. Discussion The PLSFRS is a valid tool to assess the natural history of PLS in a shorter study period.

Objective: To identify occupational risk factors for ALS using well-characterized participants with ALS (P-ALS), sibling controls (S-controls), and matched population controls (P-controls) within the National ALS Registry. We also compared oxidative stress (OS) biomarkers between groups. Methods: P-ALS were recruited over 4 years. Demographic, socioeconomic, and medical data were ascertained from medical records and structured interviews. P-ALS were followed prospectively for 2 years or until death, whichever came sooner. S-controls and age-, sex-, race/ethnicity-, and residential location-matched P-controls were recruited over 3 years. Occupational exposure to lead and agricultural chemicals (ACs) were assigned by an occupational hygienist, blinded to case status. OS biomarkers in urine were measured. Results: P-ALS (mean age 62.8 years; 63% males) resided across the United States. Demographic and socioeconomic variables did not differ among P-ALS, S-controls, and P-controls. P-ALS were more likely to report occupations with exposure to lead (adjusted OR (aOR)¼2.3, 95% CI 1.1, 4.6) and ACs (aOR ¼ 2.4, 95% CI 1.2, 4.6) compared to pooled controls. Among those with occupations with exposure to both lead and ACs, aOR was 7.2 (95% CI 2.0, 26.1). Urinary 8-oxo-dG was significantly elevated among P-ALS (11.07 ± 5.42 ng/mL) compared to S-controls, P-controls, or pooled controls (pooled 7.43 ± 5.42 ng/mL; p < 0.0001) but was not associated with occupational exposure to either lead or ACs. Conclusions: Findings reveal increased risk of ALS diagnosis among those with occupational exposure to lead and ACs and increased OS biomarkers among cases compared to controls. OS may be an important pathogenic mechanism in ALS.

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Objectives: While physical activity is important for health, many women do not meet recommended levels, particularly mothers. The purpose of this study was to assess whether physical activity levels differ by number of children at home in women aged 25–44 in the general US population. Methods: This cross-sectional analysis used 2017 Behavioral Risk Factor Surveillance System data for females aged 25–44 (N = 6266) from California, Colorado, New York, Texas, and Utah. Ordered logistic regression analysis assessed the relationship between physical activity levels and number of children at home while controlling for state and demographic, socioeconomic, and health-related factors. Results: About half of participants reported “inactive” or “insufficiently active” physical activity levels and about two-thirds reported having one or more children at home. The results of adjusted analysis indicated that physical activity level was significantly related to having one child (adjusted odds ratio = 0.75, 95% confidence interval = 0.63, 0.89), two children (adjusted odds ratio = 0.79; 95% confidence interval = 0.67, 0.93), and three or more children (adjusted odds ratio = 0.80, 95% confidence interval = 0.67, 0.94) at home. Conclusion: Overall, physical activity levels were significantly related to presence of children at home for women aged 25–44, but increasing number of children at home did not impact effect size. For women aged 25–44 in a primary care setting, a moderate prevalence of inactive or insufficiently active physical activity may be expected. Providers should address physical activity with all patients in this target population during well-visits, but particularly for women with children at home; educate patients about the health benefits of regular physical activity; and provide resources that will help them integrate physical activity into their daily lifestyles.

Quantitative measures of disease severity are essential outcome measures for clinical trials. The slow progression of disease in primary lateral sclerosis (PLS) requires clinical measures that are sensitive to changes occurring within the time frame of a clinical trial. Proposed clinical outcome measures include the PLS functional rating scale (PLSFRS), burden scores derived from clinical examination findings, and quantitative measures of motor performance. The PLSFRS has good inter-rater reliability and showed greater longitudinal change over 6-and 12-months compared to the revised ALS functional rating scale. Examination-based upper motor neuron burden (UMNB) scales also have good reliability, and longitudinal studies are in process. Quantitative measures of strength, dexterity, gait, and speech have the potential to provide objective and precise measures of clinical change, but have been the least studied in persons with PLS.