Key result
Early-onset FSHD was associated with more severe muscle weakness compared to age-matched (mean clinical score 11 vs 5, p<0.05) and duration-matched (11 vs 9) classic-onset patients.
Why the study?
The relation between age at onset and disease severity in facioscapulohumeral muscular dystrophy was not well established.
Does early disease onset correlate with increased disease severity and faster progression in patients with facioscapulohumeral muscular dystrophy?
Cross-Sectional (n=83)
Does early disease onset correlate with increased disease severity and faster progression in patients with facioscapulohumeral muscular dystrophy?
Absolute Event Rate: 11% vs 5%
p-value: p=<0.05
Early-onset FSHD is a marker for a more severe disease phenotype with faster progression and more frequent systemic features compared to classic-onset FSHD.
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May warrant closer monitoring in early-onset FSHD; leaves open whether onset age independently drives progression.
Goselink et al. (2018) conducted a cross-sectional in Facioscapulohumeral muscular dystrophy (FSHD) (n=83). Early disease onset vs. Classic disease onset was evaluated on Mean FSHD clinical score (p=<0.05). Early-onset FSHD was associated with more severe muscle weakness compared to age-matched (mean clinical score 11 vs 5, p<0.05) and duration-matched (11 vs 9) classic-onset patients.
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