Introduction Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder with vastly differing survival times. The influence of environmental exposures such as weather conditions on mortality among people living with ALS remain poorly defined. The aim of this study was to evaluate the association between short-term meteorological phenomena and the odds of death among patients with ALS. Methods We conducted a retrospective case-crossover study of 298 deaths among clinically verified ALS patients that occurred within a weather-station catchment area. Daily mean data for temperature, atmospheric pressure, and relative humidity were analyzed. Associations were analyzed by comparing hazard intervals (1–3 days before death) with bidirectional control intervals in generalized linear mixed models. Results are expressed as odds ratios (OR) and 95% confidence intervals (CI). Results Absolute meteorological conditions (temperature, pressure, humidity) showed no significant association with mortality, which was independently supported by seasonal/monthly death count analyses. Short-term temperature rises of 3 °C in the 24 h preceding the day before death were associated with 25% higher odds of death (OR 1.25; 95% CI 1.05–1.49). Exploratory subgroup analyses suggested that this association was most pronounced in female, ventilated, and older patients (≥65 years). Changes in relative humidity and atmospheric pressure were not significantly associated with the odds of death. Conclusion Short-term temperature increases are associated with higher odds of death among people living with ALS, independent of absolute weather conditions or seasonal patterns. These findings support attention of stable ambient temperatures in end-stage care, while further studies of weather-related hazards in ALS are needed.
Maximilian Dimitrov, J. Grosskreutz, Benjamin Ilse et al.· Frontiers in Aging Neuroscie...· 0 citations
Neurofilament light chain (NfL) is an established marker of neuronal injury and disease aggressiveness in amyotrophic lateral sclerosis (ALS). In contrast, the clinical and biological significance of cardiac troponin T (cTnT) in ALS is not fully understood. We aimed to evaluate the relationship between plasma cTnT and disease aggressiveness, progression stage, and clinical phenotype in comparison with NfL. Plasma cTnT and cerebrospinal fluid (CSF) NfL were analysed at diagnosis in a population-based cohort of 526 patients with ALS. Disease aggressiveness was modelled using the D50 framework, which quantifies the time taken to lose 50% of functional capacity (ALSFRS-R) and normalises individual disease trajectories. Biomarker associations with disease aggressiveness, phase, and clinical variables were assessed through group comparisons, logistic and linear regression, and receiver operating characteristic analyses. Neurofilament light chain in CSF was strongly associated with disease aggressiveness, with higher levels in patients with more aggressive disease. In contrast, plasma hs-cTnT did not correlate with D50. Across disease phases, CSF NfL remained relatively stable, whereas plasma hs-cTnT increased with advancing rD50, suggesting accumulation with disease progression. Plasma hs-cTnT levels were higher in patients with spinal compared to bulbar onset. Combined biomarker models improved sensitivity and negative predictive value for identifying less aggressive disease. Plasma hs-cTnT and CSF NfL capture distinct but complementary dimensions of ALS pathology. While NfL reflects disease aggressiveness, hs-cTnT aligns with disease phase and clinical phenotype, supporting its use as a complementary biomarker for ALS characterisation and monitoring.
Julia Sellin, Linn Öijerstedt, Janina von der Gablentz et al.· Journal of Neurology· 0 citations
ABSTRACT Objective Amyotrophic lateral sclerosis (ALS) is a clinically heterogeneous neurodegenerative disease requiring reliable biomarkers to improve patient stratification and trial design. While serum neurofilament light chain (sNfL) reflects neuroaxonal stress and disease aggressiveness, troponin T (TnT) may capture complementary aspects of neuromuscular involvement. We assessed the associations of TnT and sNfL with D50‐derived measures of disease aggressiveness (D50) and disease accumulation (rD50) in ALS. Methods In this retrospective observation, TnT and sNfL levels from ALS patients in two independent German cohorts were analyzed using the D50 disease progression model; discovery cohort (Essen, n = 433) and an independent replication cohort (Bonn, n = 185). Results TnT levels were strongly associated with rD50‐defined disease phases in the discovery cohort (p < 0.001). While not all subgroup‐specific associations were replicated, the overall relationship between TnT and disease accumulation was supported in the independent replication cohort. In contrast, sNfL showed no consistent relationship with rD50‐derived disease phases. sNfL concentrations demonstrated a significant inverse association with D50, supporting a relationship with disease aggressiveness across both cohorts (p < 0.001). Associations between TnT levels and D50‐defined disease aggressiveness were generally weaker and less consistent. Interpretation TnT was associated with measures of disease accumulation (rD50), whereas sNfL was more closely associated with disease aggressiveness (D50). Our results suggest that TnT and sNfL capture different dimensions of disease status within the D50 framework. Further longitudinal studies are needed to determine whether combining these biomarkers improves disease stratification or prognostic assessment in clinical practice and therapeutic trials.
Julia Sellin, Sofia Waldorf, Janina von der Gablentz et al.· Annals of Clinical and Trans...· 0 citations
We use cookies to run the site and, with your consent, for analytics and to show ads.
See our Cookie Policy.
