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Long-term follow-up of respiratory function in facioscapulohumeral muscular dystrophy.
- Source :
-
Journal of Neurology . Jul2022, Vol. 269 Issue 7, p3682-3689. 8p. - Publication Year :
- 2022
-
Abstract
- Objective: To evaluate the 5-year change in respiratory function in patients with facioscapulohumeral muscular dystrophy (FSHD). Methods: Genetically confirmed patients with FSHD aged ≥ 18 years were examined twice over five years. Forced vital capacity (FVC) and forced expiratory volume in 1 s (FEV1) were measured using hand-held spirometry with a face mask. Several clinical outcome measures were correlated to respiratory function. Results: Ninety-two patients were included (57% male, age 18–75 years). At baseline, the spirometry outcomes of 41 patients showed a restrictive ventilatory pattern (FVC < 80% and FEV1/FVC ≥ 70% of predicted) and of 48 patients at follow-up. The mean FVC decreased from baseline to follow-up from 79.0 to 76.7% predicted (p = 0.021). This decrease was driven by a subgroup of 15 patients who had a deterioration of FVC of > 10% predicted. The subgroup of 15 patients was more severely affected at baseline (p = 0.002 for FSHD clinical score and 0.007 for Ricci score). They developed more frequently spinal and thorax deformities (p < 0.001 for kyphoscoliosis and 0.012 for pectus excavatum) and had a larger decline in axial muscle function (p = 0.020). Only weak correlations were found between the change in FVC% predicted and the change in clinical scores between baseline and follow-up. Interpretation: Respiratory function remained stable in most patients with FSHD, but a subgroup of patients showed a pronounced deterioration. They showed more severe muscle weakness including the leg muscles at baseline (Ricci score ≥ 6), had spinal and thorax deformities and a relatively fast decline in axial muscle function at follow-up. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 03405354
- Volume :
- 269
- Issue :
- 7
- Database :
- Academic Search Index
- Journal :
- Journal of Neurology
- Publication Type :
- Academic Journal
- Accession number :
- 157584910
- Full Text :
- https://doi.org/10.1007/s00415-022-10990-7