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Longitudinal course of lung function in myotonic dystrophy type 1
Author(s) -
Thil Catherine,
Agrinier Nelly,
Chenuel Bruno,
Poussel Mathias
Publication year - 2017
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25604
Subject(s) - myotonic dystrophy , vital capacity , pulmonary function testing , medicine , lung volumes , lung , cardiology , lung function , diffusing capacity
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 (FEV 1 ) –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