P35 Upper limb muscle fat-water quantification MRI in non-ambulant Duchenne muscular dystrophy

2014 ◽  
Vol 24 ◽  
pp. S16 ◽  
Author(s):  
V. Ricotti ◽  
M.R.B. Evans ◽  
C.D.J. Sinclair ◽  
J.M. Morrow ◽  
J.W. Butler ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Upper Limb ◽  
Limb Muscle ◽  
Water Quantification

scholarly journals Upper Limb Evaluation in Duchenne Muscular Dystrophy: Fat-Water Quantification by MRI, Muscle Force and Function Define Endpoints for Clinical Trials

PLoS ONE ◽  
2016 ◽  
Vol 11 (9) ◽  
pp. e0162542 ◽  
Author(s):  
Valeria Ricotti ◽  
Matthew R. B. Evans ◽  
Christopher D. J. Sinclair ◽  
Jordan W. Butler ◽  
Deborah A. Ridout ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Upper Limb ◽  
Muscle Force ◽  
Water Quantification ◽  
And Function

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.

scholarly journals Upper limb function in Duchenne muscular dystrophy: 24 month longitudinal data

PLoS ONE ◽  
2018 ◽  
Vol 13 (6) ◽  
pp. e0199223 ◽  
Author(s):  
Marika Pane ◽  
Giorgia Coratti ◽  
Claudia Brogna ◽  
Elena Stacy Mazzone ◽  
Anna Mayhew ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Longitudinal Data ◽  
Upper Limb ◽  
Limb Function ◽  
Upper Limb Function

Manual ability and upper limb performance in nonambulatory stage of Duchenne muscular dystrophy

2020 ◽  
Vol 27 (6) ◽  
pp. 304-309
Author(s):  
G. Demir ◽  
N. Bulut ◽  
Ö. Yılmaz ◽  
A. Karaduman ◽  
İ. Alemdaroğlu-Gürbüz
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Upper Limb

scholarly journals Compensatory movements during functional activities in ambulatory children with Duchenne muscular dystrophy

2014 ◽  
Vol 72 (1) ◽  
pp. 5-11 ◽  
Author(s):  
Joyce Martini ◽  
Mariana Callil Voos ◽  
Michele Emy Hukuda ◽  
Maria Bernadete Dutra de Resende ◽  
Fátima Aparecida Caromano
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Upper Limb ◽  
Lower Limbs ◽  
Functional Evaluation ◽  
Functional Activities ◽  
Lumbar Hyperlordosis ◽  
Base Of Support ◽  
Transitional Phase

Objective: During the transitional phase (ambulatory to non-ambulatory), synergies characterize the evolution of Duchenne muscular dystrophy (DMD). This study was performed to describe and quantify compensatory movements while sitting down on/rising from the floor and climbing up/down steps. Method: Eighty videos (5 children × 4 assessments × 4 tasks) were recorded quarterly in the year prior to gait loss. Compensatory movements from the videos were registered based on the Functional Evaluation Scale for DMD. Results: The most frequently observed compensatory movements were upper limb support on lower limbs/floor/handrail during all the tasks and lumbar hyperlordosis, trunk support on handrail, equinus foot, increased base of support, non-alternated descent, and pauses while climbing up/down steps. Conclusion: Climbing up/down steps showed a higher number of compensatory movements than sitting down on/rising from the floor, which seemed to be lost before climbing up/down steps in ambulatory children with DMD.

scholarly journals Reliability of the Performance of Upper Limb assessment in Duchenne muscular dystrophy

2014 ◽  
Vol 24 (3) ◽  
pp. 201-206 ◽  
Author(s):  
Marika Pane ◽  
Elena S. Mazzone ◽  
Lavinia Fanelli ◽  
Roberto De Sanctis ◽  
Flaviana Bianco ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Upper Limb

scholarly journals RESPONSIVENESS OF UPPER LIMB SCALES AND TRUNK CONTROL FOR THE EVOLUTION OF PATIENTS WITH DUCHENNE MUSCULAR DYSTROPHY

2021 ◽  
Vol 39 ◽  
Author(s):  
Flaviana Kelly de Lima Maciel ◽  
Ana Lúcia Yaeko da Silva Santos ◽  
Cristina dos Santos Cardoso de Sá
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Upper Limb ◽  
Repeated Measures ◽  
Comparison Method ◽  
Time Effect ◽  
Initial Assessment ◽  
Trunk Control ◽  
Limb Function ◽  
Upper Limb Function

ABSTRACT Objective: To verify the interval of responsiveness to the scales Segmental Assessment of Trunk Control (SATCo-BR), Performance of Upper Limbs (PUL), and Jebsen Taylor Test (JTT) in patients with Duchenne Muscular Dystrophy (DMD). Methods: We assessed patients with DMD aged 6 to 19 years old and with mini-mental (MMSE) score above 10 points. The assessments were performed individually, in a single session. The upper limb function was performed by PUL and JTT, and trunk control by SATCo-BR. Assessments were repeated six and 12 months after the initial assessment. The repeated-measures analysis of variance model and Bonferroni’s multiple comparison method were employed as post hoc analysis; when the ANOVA assumptions were not met, the Friedman test was applied. Results: The sample consisted of 28 patients evaluated in three moments (initial, and six and 12 months after the beginning). There was a time effect for the Upper Limb function performance in the total JTT, and for the subtests, except for subtests 1 and 6, which did not show a difference between the different moments. There was also a time effect for the score of total PUL, proximal PUL, intermediate PUL, and distal PUL. In the SATCo-BR, this effect was observed between the initial and 6 months, and between the initial and 12 months. Conclusions: The JTT, PUL, and SATCo-BR scales can detect changes over time, and they showed responsiveness to detect the evolution of the disease in the 6-month interval.

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