Imaging respiratory muscle quality and function in Duchenne muscular dystrophy

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Respiratory muscle training in Duchenne muscular dystrophy.

Eighteen boys with Duchenne muscular dystrophy were entered into trials to assess the effects of specific ventilatory strength and endurance training programmes. The findings showed an improvement in ventilatory muscle endurance but not in strength as a result of specific respiratory muscle training. The clinical significance of these findings is uncertain, however, and needs further evaluation.

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Respiratory magnetic resonance imaging biomarkers in Duchenne muscular dystrophy

OBJECTIVE To examine the diaphragm and chest wall dynamics with cine breathing magnetic resonance imaging (MRI) in ambulatory boys with Duchenne muscular dystrophy (DMD) without respiratory symptoms and controls. METHODS In 11 DMD boys and 15 controls, cine MRI of maximal breathing was recorded for 10 sec. The lung segmentations were done by an automated pipeline based on a Holistically-Neste...

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P164: Adeno-Associated Viral Vectors in Duchenne Muscular Dystrophy

Duchenne muscular dystrophy (BMD) is an inherited X-link disease. The incidence of this muscle-wasting disease is 1:5000 male live births. Mutation in the gene coding for dystrophin is the main cause of BMD. Most cases of this disease succumb to respiratory and cardiac failure in 3rd to 4th decades. The slow progression of BMD and recent achievement of gene therapies make it as an appropriate c...

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Duchenne Muscular Dystrophy and Brain Function

Muscular dystrophies have historically been characterised according to clinical criteria, however in the genomic age the muscular dystrophies are now subdivided into groups according to the primary gene defect. Currently identified are 29 different loci and encoded proteins, giving rise to 34 distinct forms of muscular dystrophy (Dalkilic & Kunkel 2003; Hsu 2004). The majority of these types of...

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ژورنال

عنوان ژورنال: Journal of Neurology

سال: 2019

ISSN: 0340-5354,1432-1459

DOI: 10.1007/s00415-019-09481-z