OBJECTIVES: The aim of this study was to describe healthcare resource use in pALS, overall and across disease stages in France.

METHODS: A retrospective longitudinal study was conducted among newly diagnosed pALS identified between 2012 and 2019 in the French National Health Data System, SNDS. pALS were identified as having at least 2 events (or 1 event in the case of death) within 6 months: reimbursement of riluzole, and/or diagnosis of motor neuron disease (MND, ICD-10 code G12.2). We excluded people who had at least one diagnosis for Parkinson disease, Multiple sclerosis, and other conditions within G12 (spinal muscular atrophy and related syndromes), 6 months after the first ALS event. A symptom- and milestone-based algorithm was used to define progression stages: early, middle, and late. Staging criteria were selected from the 2-year pre-index date (date diagnosis criteria met) until the end of follow-up. Stage onset was determined by the first occurrence of an event defining that stage. Disease progression to a later stage was irreversible.

RESULTS: We identified 18,289 incident cases, among which 11,215, 15,098 and 16,025 had an early-, middle- and late-stage of ALS. Overall, 97.4% of patients had at least one hospitalization (60.9%, 85.6% and 80.8% among patients with early-, middle- and late-stage respectively), 59.6% had admissions primarily related to ALS (26.3%, 39.0% and 33.0%), 67.5% experienced one-day hospitalizations (30.5%, 49.6% and 44.0%) and 25.6% were admitted in an intensive care unit (2.8%, 7.6% and 21.9%). The rate of all-cause hospitalizations was 1.98 per patient-year (PPY) for the overall population, and 1.46, 1.66, and 2.82 PPY for patients in early-, middle-, and late-stages, respectively.

CONCLUSIONS: These real-world findings demonstrate significant healthcare burden related to the management of ALS across all stages of progression and increasing with advanced disease, highlighting the potential value of delaying progression into a more resource-intensive stage.