Amyotrophic lateral sclerosis & frontotemporal degeneration · 2026
Four decades of ALS care: a retrospective study of epidemiology, clinical course and changes in management.
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Bjørnstadjordet Mats, Kvernmo Helene Ballo, Bråthen Geir, Simpson Melanie Rae, Sando Sigrid Botne, Hagen Knut, Devik Kristina, Wergeland Tore
Аннотация
BACKGROUND: Several interventions have been introduced for amyotrophic lateral sclerosis (ALS) in recent decades, and population-level studies investigating their use and impact are needed. This study describes the epidemiology, disease trajectory, and changes in clinical management of ALS in a county of Norway over a 38-year period.
METHODS: We conducted a retrospective chart review of all ALS cases diagnosed between 1986 and 2024 in Trøndelag county, Norway. Data were extracted from medical records using a standardized electronic case report form. Patients were stratified by time of diagnosis into four groups.
RESULTS: A total of 429 patients were included (56% male). Median age at symptom onset was 68 years. The age-standardized incidence of ALS was 3.32 per 100,000 person-years (95%CI 2.90-3.74) and increased over time (p = 0.002). Bulbar onset occurred in 38% of cases. Median diagnostic delay was 13 months (95%CI 12-14), without significant improvement over time. Median survival was 28 months (95%CI 26-31) from symptom onset, shorter among bulbar-onset patients. Use of riluzole, percutaneous endoscopic gastrostomy, and noninvasive ventilation (NIV) increased over the study period, whereas median survival remained stable. Emergency initiation of ventilation occurred in 25% (NIV n = 41/167) and 89% (invasive ventilation n = 16/18) of cases in which these treatment modalities were used.
CONCLUSION: This comprehensive regional study reveals a rising incidence of ALS in Trøndelag, with increased adoption of supportive interventions over time.
METHODS: We conducted a retrospective chart review of all ALS cases diagnosed between 1986 and 2024 in Trøndelag county, Norway. Data were extracted from medical records using a standardized electronic case report form. Patients were stratified by time of diagnosis into four groups.
RESULTS: A total of 429 patients were included (56% male). Median age at symptom onset was 68 years. The age-standardized incidence of ALS was 3.32 per 100,000 person-years (95%CI 2.90-3.74) and increased over time (p = 0.002). Bulbar onset occurred in 38% of cases. Median diagnostic delay was 13 months (95%CI 12-14), without significant improvement over time. Median survival was 28 months (95%CI 26-31) from symptom onset, shorter among bulbar-onset patients. Use of riluzole, percutaneous endoscopic gastrostomy, and noninvasive ventilation (NIV) increased over the study period, whereas median survival remained stable. Emergency initiation of ventilation occurred in 25% (NIV n = 41/167) and 89% (invasive ventilation n = 16/18) of cases in which these treatment modalities were used.
CONCLUSION: This comprehensive regional study reveals a rising incidence of ALS in Trøndelag, with increased adoption of supportive interventions over time.