Prevalence and predictor factors of respiratory impairment in a large cohort of patients with Myotonic Dystrophy type 1 (DM1): A retrospective, cross sectional study

Journal of the Neurological Sciences
Salvatore RossiGabriella Silvestri

Abstract

Respiratory complications are relevant in DM1, leading to a significantly increased morbidity and mortality risk in these patients; however, so far only few studies concerning respiratory function have been conducted in DM1 patients. We report a retrospective, multicenter, cross sectional study on a large cohort of DM1 patients widely characterized in the phenotype, to assess prevalence and identify predictors of restrictive respiratory syndrome. 268 DM1 subjects aged >18 years, who had recently performed spirometric tests were included; restrictive syndrome was diagnosed if forced vital capacity (FVC) <80% of predicted. This cut-off was used for statistical univariate and multivariate analysis. 51.9% patients showed a restrictive syndrome, and half of them had indication to non-invasive ventilation (NIV), yet only 50% resulted compliant to NIV. CTG expansion size in leukocytes, clinical muscle severity, most functional parameters of respiratory muscle involvement, presence of cardiac conduction disturbances, pacemaker (PMK), exertion dyspnea, obstructive sleep apnea, and indication and compliance to NIV were all significantly associated with restrictive syndrome at the univariate analysis; in the multivariate model only the fi...Continue Reading

Citations

Jul 12, 2020·Acta neurologica Belgica·Marco MazzoliGiuliana Galassi
Aug 28, 2019·Journal of Clinical Neuromuscular Disease·David Lacomis
May 7, 2020·Frontiers in Neurology·Mao LiXusheng Huang
Nov 3, 2020·Disability and Rehabilitation·Kateri RaymondCynthia Gagnon
Dec 8, 2020·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·Matteo GaribaldiGiovanni Antonini
Oct 30, 2020·Frontiers in Neurology·Costanza SimonciniGabriele Siciliano
Mar 7, 2021·International Journal of Environmental Research and Public Health·Tiago MateusSandra Rebelo
Sep 26, 2020·Practical Neurology·Caroline KramarzBrendan Mclean
Nov 10, 2021·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·Matteo GaribaldiGiovanni Antonini

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