2026 American Academy of Neurology Abstract Website

Objective:

To quantify age at onset of bulbar symptoms and loss of bulbar function in patients with Friedreich ataxia (FA) using real-world data from US Medicare claims.

Background:

FA is a rare genetic neurodegenerative disease leading to impaired muscle coordination and systemic complications. Bulbar symptoms (impairments in speech, swallowing, or breathing function) are key progression milestones and represent significant loss of independence for patients with FA. Limited data exist on age at which patients experience such bulbar impairments following entry into Medicare.

Design/Methods:

This retrospective study used de-identified medical claims and vital statistics (2005-2023) for patients with FA entering Medicare due to disability. Patients were stratified by age at disability/Medicare entry: 18-24, 25-34, 35-44, 45-54, and 55-64 years (age ≥65 excluded). Endpoints included time from entry to bulbar impairment (breathing, speech, swallowing) and loss of bulbar function (severe breathing impairment requiring ventilation, severe swallowing impairment requiring tube feeding, or loss of speech function including need for speech generation devices).

Results:

Among 6551 patients with FA, median age at disability/Medicare entry was 27.8 years; 49.3% were women. By age strata, median age at disability/Medicare entry was 20.0, 27.4, 37.2, 47.3, and 57.3 years, respectively. Bulbar impairment occurred in 88.5%, 88.9%, 90.8%, 92.3%, and 92.6% of patients by age strata, respectively. Estimated ages at onset by age strata were 33.8, 41.0, 46.7, 54.6, and 63.2 years for any bulbar impairment, and 40.6, 47.5, 51.2, 59.8, and 67.6 years for loss of bulbar function.

Conclusions:

Claims data show bulbar impairment is a key disease progression milestone in FA, with symptoms emerging ≈8-13 years after Medicare entry and persisting ≈4-7 years before loss of bulbar function. These impairments mark a profound loss of independence and are associated with increased clinical complexity and healthcare resource use. These data provide insights on bulbar symptom progression in later-stage FA patients in Medicare.