Reduced mandibular range of motion in Duchenne Muscular Dystrophy: predictive factors

2015 ◽  
Vol 42 (6) ◽  
pp. 430-438 ◽  
Author(s):  
H. W. van Bruggen ◽  
L. Van Den Engel-Hoek ◽  
M.H. Steenks ◽  
E.M. Bronkhorst ◽  
N.H.J. Creugers ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Range Of Motion ◽  
Predictive Factors

Predictive factors for masticatory performance in Duchenne muscular dystrophy

2014 ◽  
Vol 24 (8) ◽  
pp. 684-692 ◽  
Author(s):  
H.W. van Bruggen ◽  
L. van de Engel-Hoek ◽  
M.H. Steenks ◽  
E.M. Bronkhorst ◽  
N.H.J. Creugers ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Predictive Factors ◽  
Masticatory Performance

Knee Strength and Ankle Range of Motion Impacts on Timed Function Tests in Duchenne Muscular Dystrophy: In the Era of Glucocorticoids

2021 ◽  
pp. 1-13
Author(s):  
Tina Duong ◽  
Jennifer Canbek ◽  
Alicia Fernandez-Fernandez ◽  
Erik Henricson ◽  
Marisa Birkmeier ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Range Of Motion ◽  
Rate Of Change ◽  
Standards Of Care ◽  
Muscle Testing ◽  
Knee Strength ◽  
Function Tests ◽  
Ambulatory Ability ◽  
Loss Of Strength

Background: Duchenne Muscular Dystrophy (DMD) is a neuromuscular disorder that presents in childhood and is characterized by slowly progressive proximal weakness and lower extremity contractures that limit ambulatory ability [1, 2]. Contractures develop in the ankles, knees, and hips due to muscle imbalances, fibrotic changes, loss of strength, and static positioning [2, 5]. Currently, standards of care guidelines emphasize the importance of maintaining good musculoskeletal alignment through stretching, bracing, and glucocorticoid (GC) therapy to preserve strength and function. Methods: This is a retrospective analysis of prospectively collected data through the CINRG Duchenne Natural history study (DNHS). The objectives of this analysis are to understand the progression of ankle contractures for individuals with DMD and to investigate the relationship between progressive lower limb contractures, knee strength, and Timed Function Tests. A collection of TFTs including supine to stand (STS), 10 meter walk test (10MWT), and timed stair climbing (4SC) have been used to monitor disease progression and are predictive of loss of ambulation in these patients [4]. Multiple factors contribute to loss of ambulation, including progressive loss of strength and contracture development that leads to changing biomechanical demands for ambulation. A better understanding of the changes in strength and range of motion (ROM) that contribute to loss of function is important in a more individualized rehabilitation management plan. In this longitudinal study, we measured strength using quantitative muscle testing (QMT) with the CINRG Quantitative Measurement System (CQMS)), ROM was measuresed with a goniometer and TFTs were measured using a standard stopwatch and methodology. Results: We enrolled 440 participants; mean baseline age was 8.9 (2.1, 28.0) years with 1321 observations used for analysis. GC use was stratified based on duration on drug with 18.7%at <  6 months or naïve; 4.3%<1 year; 58.0%1 <  10 years; and 19.3%between 10-25 years of GC use. Ankle ROM was better for those on GC compared to GC naive but did not significantly influence long-term progression rates. QMT, ROM, age and GCs contribute to speed of TFTs. Knee extension (KE) strength and Dorsiflexion (DF) ROM are significant predictors of speed for all TFTs (p <  0.001). Of the variables used in this analysis, KE strength is the primary predictor of walking speed, estimating that every pound increase in KE results in a 0.042 m/s improvement in 10MWT, and a smaller similar increase of 0.009 m/s with every degree of ankle DF ROM. Conclusion: GC use provides an improvement in strength and ROM but does not affect rate of change. Knee strength has a greater influence on speed of TFTs than DF ROM, although both are statistically significant predictors of speed. Results show that retaining knee strength [1, 2], along with joint flexibility, may be important factors in the ability to perform walking, climbing and supine to stand activities.

G.P.10 05 Predictive factors for the development of scoliosis in Duchenne muscular dystrophy

2006 ◽  
Vol 16 (9-10) ◽  
pp. 719
Author(s):  
M. Kinali ◽  
R.K. Knight ◽  
M. Main ◽  
E. Mercuri ◽  
S. Messina ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Predictive Factors

Predictive factors for the development of scoliosis in Duchenne muscular dystrophy

2007 ◽  
Vol 11 (3) ◽  
pp. 160-166 ◽  
Author(s):  
M. Kinali ◽  
M. Main ◽  
J. Eliahoo ◽  
S. Messina ◽  
R.K. Knight ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Predictive Factors

Improving Temporomandibular Range of Motion in People With Duchenne Muscular Dystrophy and Spinal Muscular Atrophy

2020 ◽  
Vol 74 (2) ◽  
pp. 7402205080p1
Author(s):  
Evan Harry Lloyd Morris ◽  
Timothy Estilow ◽  
Allan M. Glanzman ◽  
Stacy Victoria Cusack ◽  
Sabrina W. Yum
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Spinal Muscular Atrophy ◽  
Range Of Motion ◽  
Muscular Atrophy

Predictive Factors of Cessation of Ambulation in Patients with Duchenne Muscular Dystrophy

2002 ◽  
Vol 81 (12) ◽  
pp. 906-912 ◽  
Author(s):  
Jan P. J. Bakker ◽  
Imelda J. M. de Groot ◽  
Anita Beelen ◽  
Gustaaf J. Lankhorst
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Predictive Factors

G.P.12.12 Predictive factors for progressive foot deformity in non-ambulant boys with Duchenne muscular dystrophy

2008 ◽  
Vol 18 (9-10) ◽  
pp. 807
Author(s):  
C. Nicholson ◽  
M. Main ◽  
M. Kinali ◽  
F. Muntoni ◽  
E. Mercuri
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Predictive Factors ◽  
Foot Deformity

Identifying Potential Predictive Factors of Sleep Quality in Duchenne Muscular Dystrophy

2019 ◽  
Author(s):  
J. Newitt ◽  
H. Gordish-Dressman ◽  
P. Clemens ◽  
P.J. Strollo ◽  
CINRG DHNS Investigators
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Sleep Quality ◽  
Predictive Factors

scholarly journals Feasibility and effectiveness of a novel dynamic arm support in persons with spinal muscular atrophy and duchenne muscular dystrophy

2021 ◽  
Vol 18 (1) ◽  
Author(s):  
Mariska M. H. P. Janssen ◽  
Jolinda Horstik ◽  
Paulien Klap ◽  
Imelda J. M. de Groot
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Spinal Muscular Atrophy ◽  
Range Of Motion ◽  
Upper Extremity ◽  
Muscular Atrophy ◽  
Neuromuscular Disorders ◽  
Median Increase ◽  
Active Range Of Motion ◽  
Arm Support

Abstract Background Neuromuscular disorders (NMD) commonly affect the upper extremity. Due to muscle weakness, performance of daily activities becomes increasingly difficult, which leads to reduced independence and quality of life. In order to support the performance of upper extremity tasks, dynamic arm supports may be used. The Yumen Arm is a novel dynamic arm support specially developed for people with NMD. The aim of this study is to evaluate the feasibility and effectiveness of the Yumen Arm in persons with Duchenne Muscular Dystrophy (DMD) and persons with Spinal Muscular Atrophy (SMA). Methods Three persons with DMD and three persons with SMA participated in this study. All participants conducted a set of measures with and without the Yumen Arm. Outcome measures were: active range of motion of the arm and trunk (i.e. Reachable Workspace, Functional Workspace, and trunk movement), fatigue (OMNI-RPE), Performance of Upper Limb (PUL) scale and some additional activities of daily living. User experiences were collected using a questionnaire. Results The Yumen Arm could be used by all participants. Results showed a median increase in active range of motion (4% relative surface area), and a median increase of function ability (> 11% PUL score) when using the Yumen Arm. In addition, three out of four (data from 2 participants was missing) participants indicated that activity performance was less fatiguing when using the Yumen Arm. Four out of five (data from 1 participant was missing) participants indicated that they would like to use the Yumen Arm in their daily lives. Conclusion This study is one of the first studies describing a range of objective measures to examine the effectiveness of a dynamic arm support. Based on these measurements we can conclude that the Yumen Arm effectively improves arm function in NMD patients, however the effectiveness varies a lot between individual subjects. We provided detailed recommendations for the improvement of the Yumen Arm, and possible also for the development of other dynamic arm supports. This study showed a lot of variability between individual subjects, which emphasizes the importance of tuning dynamic arm supports based on individual user characteristics, such as scoliosis, functional capacity and muscle strength.

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