Longitudinal course of lung function in myotonic dystrophy type 1. Issue 4 (11th April 2017)

• Record Type: Journal Article
• Authors: Thil, Catherine; Agrinier, Nelly; Chenuel, Bruno; Poussel, Mathias
• Is Part Of: Muscle & nerve. Volume 56:Issue 4(2017)
• Page Start: 816
• Page End: 818
• Publication Date: 2017-04-11
• Subjects: hypercapnia -- hypoxemia -- lung function -- lung restriction -- myotonic dystrophy -- neuromuscular disorder; Neuromuscular diseases -- Periodicals; Muscles -- Periodicals; Nerves -- Periodicals; 616.74
• DOI: 10.1002/mus.25604
• Languages: English
• ISSNs: 0148-639X

Abstract

ABSTRACT: Introduction : Quality of life and prognosis among patients with myotonic dystrophy type 1 (DM1) depend on the degree of respiratory impairment. However, the changes over time in pulmonary function in DM1 have not been clearly described. Methods : We retrospectively reviewed pulmonary function tests of 80 DM1 patients followed for at least 5 years. Results : During 9.02 ± 3.4 years of follow‐up, the average annual changes were: forced vital capacity (FVC) –0.034 ± 0.06 L (–0.72 ± 1.7% predicted); forced expiratory volume in 1 second (FEV1 ) –0.043 ± 0.05 L (–1.07 ± 1.7% predicted); and total lung capacity (TLC) –0.047 ± 0.1 L (–1.15 ± 1.7% predicted). Conclusions : These results suggest that, compared with other neuromuscular disorders, DM1 is, overall, associated with slowly progressive impairment of lung function. Muscle Nerve 56 : 816–818, 2017