Respiratory involvement in neuromuscular disease
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TLDR
In neuromuscular disease (NMD), respiratory muscle weakness is common and death often results from respiratory failure, with sleep disruption initially causing sleep-related hypoventilation, with daytime respiratory failure ensues.Abstract:
#### Key points
In neuromuscular disease (NMD), respiratory muscle weakness (RMW) is common and death often results from respiratory failure. RMW initially causes sleep-related hypoventilation, with sleep disruption. With progression, daytime respiratory failure ensues. Bulbar muscle weaknessread more
Citations
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Journal ArticleDOI
Physiology of respiratory disturbances in muscular dystrophies
TL;DR: The respiratory disturbances in muscular dystrophy are restrictive pulmonary function, hypoventilation, altered thoracoabdominal pattern, hypercapnia, dyspnoea, impaired regulation of breathing, inefficient cough and sleep disordered breathing.
Journal ArticleDOI
Characterization of pulmonary function in 10-18 year old patients with Duchenne muscular dystrophy.
Thomas Meier,Christian Rummey,Mika Leinonen,Paolo Spagnolo,Oscar H. Mayer,Gunnar M. Buyse,G. Bernert,F. Knipp,G. Buyse,Nathalie Goemans,M. van den Hauwe,Thomas Voit,V. Doppler,T. Gidaro,Jean-Marie Cuisset,S. Coopman,Ulrike Schara,S. Lutz,Janbernd Kirschner,S. Borell,Matthew J. Will,Maria Grazia D'Angelo,Erika Brighina,S. Gandossini,Ksenija Gorni,E. Falcier,Luisa Politano,P. D’Ambrosio,Antonella Taglia,Jan J.G.M. Verschuuren,Chiara S. M. Straathof,J.J. Vílchez Padilla,N. Muelas Gómez,Thomas Sejersen,M. Hovmöller,Pierre-Yves Jeannet,Clemens Bloetzer,Susan T. Iannaccone,Diana Castro,Gihan Tennekoon,Richard S. Finkel,Carsten G. Bönnemann,Craig M. McDonald,Erik K Henricson,Nanette C. Joyce,Susan D. Apkon,R.C. Richardson,R.C. Richardson +47 more
TL;DR: A considerable drop in dynamic pulmonary function parameters was associated with loss of upper limb function and the understanding of the reliability, correlation and evolution of different pulmonary function measures in DMD patients who are in the pulmonary function decline phase is expanded.
Journal ArticleDOI
Respiratory measures in amyotrophic lateral sclerosis.
Noah Lechtzin,Merit Cudkowicz,Mamede de Carvalho,Angela Genge,Orla Hardiman,Hiroshi Mitsumoto,Jesus S. Mora,Jeremy M. Shefner,Leonard H. van den Berg,Jinsy A. Andrews +9 more
TL;DR: The most commonly used measurement, forced vital capacity (VC), has been shown to correlate with clinical milestones including survival, but also requires good motor coordination and facial strength to form a tight seal around a mouthpiece.
Journal ArticleDOI
Treatment effect of idebenone on inspiratory function in patients with Duchenne muscular dystrophy
Gunnar M. Buyse,Thomas Voit,Ulrike Schara,Chiara S. M. Straathof,Maria Grazia D'Angelo,G. Bernert,Jean Marie Cuisset,Richard S. Finkel,Nathalie Goemans,Christian Rummey,Mika Leinonen,Oscar H. Mayer,Paolo Spagnolo,Thomas Meier,Craig M. McDonald +14 more
TL;DR: Findings from a randomized controlled study suggest that idebenone preserved inspiratory muscle function as assessed by V'I,max(FVC) and IFR in patients with DMD.
Journal ArticleDOI
Long-Term Ventilation in Neuromuscular Patients: Review of Concerns, Beliefs, and Ethical Dilemmas
TL;DR: NIV should be used early in the course of respiratory muscle involvement in NMD patients and its requirements may increase over time, therefore, training on technical equipment at home and advice on problem solving are warranted.
References
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Journal ArticleDOI
Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care
Katharine Bushby,Richard S. Finkel,David J. Birnkrant,Laura E. Case,Paula R. Clemens,Linda H. Cripe,Ajay Kaul,Kathi Kinnett,Craig M. McDonald,Shree Pandya,James Poysky,Frederic Shapiro,Jean Tomezsko,Carolyn M. Constantin +13 more
TL;DR: A comprehensive set of DMD care recommendations for management of rehabilitation, orthopaedic, respiratory, cardiovascular, gastroenterology/nutrition, and pain issues, as well as general surgical and emergency-room precautions are presented.
Journal ArticleDOI
Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation.
TL;DR: The notes of 197 patients with Duchenne muscular dystrophy whose treatment was managed at the Newcastle muscle centre from 1967 to 2002 were reviewed, to determine whether survival has improved over the decades and whether the impact of nocturnal ventilation altered the pattern of survival.
Journal ArticleDOI
Effects of non-invasive ventilation on survival and quality of life in patients with amyotrophic lateral sclerosis: a randomised controlled trial.
TL;DR: In patients with amyotrophic lateral sclerosis without severe bulbar dysfunction, NIV improves survival with maintenance of, and improvement in, quality of life and the survival benefit from NIV in this group is much greater than that from currently available neuroprotective therapy.
Journal ArticleDOI
BTS guideline for emergency oxygen use in adult patients
TL;DR: The philosophy of the guideline and assessment of patients, monitoring and maintenance of target saturation, and oxygen administration are reviewed.
Journal ArticleDOI
Randomised controlled trial of non-invasive ventilation (NIV) for nocturnal hypoventilation in neuromuscular and chest wall disease patients with daytime normocapnia
TL;DR: Patients with neuromuscular disease with nocturnal hypoventilation are likely to deteriorate with the development of daytime hypercapnia and/or progressive symptoms within 2 years and may benefit from the introduction of noCTurnal NIV before daytimehypercapnia ensues.