Respiratory dysfunction in myotonic dystrophy type 1: a systematic review
AM Hawkins, CL Hawkins, KA Razak, TK Khoo… - Neuromuscular …, 2019 - Elsevier
Abstract Myotonic dystrophy type 1 (DM1) is one of the most common muscular dystrophies
in adults. This review summarises the current literature regarding the natural history of
respiratory dysfunction in DM1, the role of central respiratory drive and peripheral respiratory
muscle involvement and its significance in respiratory function, and investigates the
relationship between genetics (CTG repeat length) and respiratory dysfunction. The review
included all articles that reported spirometry on 10 or more myotonic dystrophy patients. The …
in adults. This review summarises the current literature regarding the natural history of
respiratory dysfunction in DM1, the role of central respiratory drive and peripheral respiratory
muscle involvement and its significance in respiratory function, and investigates the
relationship between genetics (CTG repeat length) and respiratory dysfunction. The review
included all articles that reported spirometry on 10 or more myotonic dystrophy patients. The …