P4.57 Effects of air stacking on peak cough flows and forced vital capacity in patients with muscular dystrophy and spinal muscular atrophy

2011 ◽  
Vol 21 (9-10) ◽  
pp. 721 ◽  
Author(s):  
T.B. Carvalho ◽  
M.B. Resende ◽  
E. Zanoteli ◽  
J.M. Salge ◽  
A.S. Nakashima ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Spinal Muscular Atrophy ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Muscular Atrophy

Performance of the Upper Limb Module—A Reliable and Valid Evaluation for Chinese Patients with Duchenne Muscular Dystrophy

2020 ◽  
Author(s):  
Yuen Yee Alice Chiu ◽  
Chun Wai Lo ◽  
Chi Kuk Connie Hui ◽  
Wai Chong Susanna Choi ◽  
So Lun Lee ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Upper Limb ◽  
Forced Vital Capacity ◽  
Motor Performance ◽  
Vital Capacity ◽  
High Reliability ◽  
Reliability And Validity ◽  
Chinese Patients ◽  
Rater Reliability

Abstract Background Duchenne muscular dystrophy is a genetic disease leading to progressive muscle weakness and degeneration. Effective assessment tool is needed to allow monitoring of progress to guide the management. This study assessed the reliability and validity of the Performance of Upper Limb (PUL) Module when used in patients with Duchenne Muscular Dystrophy (DMD). MethodsTotal thirty-three Chinese DMD patients were included. Twenty-five video-recorded PUL Module version 1.3 assessments were performed for the recruited patients with three raters evaluated the same recorded video for inter-rater reliability and evaluated the same performance one month later for intra-rater reliability. Construct validity was assessed correlating the PUL Module scores with the patients’ age, their forced vital capacity (N=25) and their Hammersmith motor scale scores (N=25) performed on the same day. ResultsThe intra-rater and inter-rater reliability (ICC 0.92 - 0.99), internal consistency (Cronbach’s alpha 0.97 - 0.99) and known groups validity (AUC 0.97) of PUL module were excellent. PUL was negatively correlated with age (r = -0.912), and positively correlated with the forced vital capacity (r = 0.87) and the Hammersmith motor scale (r = 0.84). The findings confirm the high reliability and validity of PUL module, and its high clinical relevancy in monitoring the deteriorating upper limb motor performance that strongly correlated with the lung function and generalized motor performance as age increased in DMD. ConclusionThis first study of PUL module in Chinese patients with DMD confirmed that it is a reliable valid tool to monitor clinical progress and outcome for DMD.

Becker muscular dystrophy or spinal muscular atrophy?—Dystrophin studies resolve conflicting results of electromyography and muscle biopsy

1991 ◽  
Vol 1 (3) ◽  
pp. 195-200 ◽  
Author(s):  
Thomas D. McDonald ◽  
Rossella Medori ◽  
David S. Younger ◽  
Hai W. Chang ◽  
Carlo Minetti ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Spinal Muscular Atrophy ◽  
Muscle Biopsy ◽  
Muscular Atrophy ◽  
Becker Muscular Dystrophy

scholarly journals Limb-girdle muscular dystrophy or spinal muscular atrophy: a source of diagnostic confusion?

1997 ◽  
Vol 34 (11) ◽  
pp. 958-959 ◽  
Author(s):  
R Pogue ◽  
T Jackson ◽  
B Sayli ◽  
A Curtis ◽  
K M Bushby
Keyword(s):  
Muscular Dystrophy ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Limb Girdle Muscular Dystrophy ◽  
Diagnostic Confusion

Winging of the Scapula: The Underlying Biomechanics and an Orthotic Solution

1995 ◽  
Vol 209 (4) ◽  
pp. 215-223 ◽  
Author(s):  
N D Barnett ◽  
M Mander ◽  
J C Peacock ◽  
K Bushby ◽  
D Gardner-Medwin ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Surgical Technique ◽  
Spinal Muscular Atrophy ◽  
Nerve Injury ◽  
Muscular Atrophy ◽  
Sensor System ◽  
Functional Improvement ◽  
Medial Border ◽  
Non Invasive ◽  
Muscular Disorders

Winging of the scapula occurring in muscular disorders (muscular dystrophy and spinal muscular atrophy) or nerve injury has been investigated, resulting in a thorough understanding and presentation of the underlying biomechanics causing this occurrence. This includes a biomechanical explanation of the characteristic prominence of the medial border of the scapula upon attempted elevation, together with the biomechanical reasons for the rotation of the scapula in a direction contrary to the normal scapulohumeral rhythm. Based on these findings, a non-invasive alternative to the surgical technique for scapular stabilization has been devised, using an inflatable orthosis, placed between the scapula and an external restraint (such as a spinal jacket). The device has been tested on one subject using a 3SPACE™ Isotrak™ electromagnetic source and sensor system, and gave encouraging results. Elevation increased by up to 35° (37 per cent), and functional improvement in the use of the hand around the head and face has been achieved. The technique needs no aftercare or physiotherapy and is therefore both economical and functionally effective.

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