A critical review of functional assessment tools for upper limbs in Duchenne muscular dystrophy

Article (PDF Available)inDevelopmental Medicine & Child Neurology 54(10):879-885 · June 2012with74 Reads
DOI: 10.1111/j.1469-8749.2012.04345.x · Source: PubMed
The recent development of therapeutic approaches for Duchenne muscular dystrophy (DMD) has highlighted the need to identify clinical outcome measures for planned efficacy studies. Although several studies have reported the value of functional scales, timed tests, and measures of endurance aimed at ambulant individuals, less has been done to identify reliable measures of function in individuals who have lost ambulation. The aim of this paper is to provide a critical review of the existing literature on functional measures assessing upper extremity function in DMD. Four observer-rated, performance-based measures and four self-reported scales have been previously used in DMD. Each scale provides useful information but none reflects all the different levels of functional ability in activities of daily living observed in individuals with DMD at different ages.

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Available from: Anna Mayhew, Oct 16, 2014
    • "The specific roles of these authors are articulated in the 'author contributions' section. patients include observer-rated performance in a controlled environment and self-reported questionnaires [38][39][40][41][42] . Recently, collaborative efforts among medical doctors, physiotherapists , and patients have led to development of a novel scale for assessing performance of upper limbs [43], a stereo camera-based reachable workspace analysis system [44,45] or a skeletal tracking system [46]. "
    [Show abstract] [Hide abstract] ABSTRACT: Trial registration: ClinicalTrials.gov NCT01611597.
    Full-text · Article · Jun 2016
    • "With new therapies emerging for myopathies [1,2], the need for reliable outcome measures of the upper limb has become crucial, particularly for clinical trials including non-ambulant patients. Change in wrist strength and function may impact upper limb abilities and therefore, the quality of life of these patients. "
    [Show abstract] [Hide abstract] ABSTRACT: Background Wrist movements become impaired with disease progression in various neuromuscular disorders. With the development of new therapies, thorough measurement of muscle strength is crucial to document natural disease progression and to assess treatment efficacy. We developed a new dynamometer enabling wrist flexion and extension torque measurement with high sensitivity. The aims of the present study were to collect norms for healthy children and adults, to compute predictive equations, to assess the reliability of the measurements and to test the feasibility of using the device in patients with a neuromuscular disease.Methods The peak isometric torque of wrist flexion and extension was measured with the MyoWrist dynamometer in 345 healthy subjects aged between 5 and 80 years old and in 9 patients with limb girdle muscle dystrophy type 2 C (LGMD2C) aged between 16 and 38 years old.ResultsPredictive equations are proposed for the wrist flexion and extension strength in children and adults. Intra-rater and inter-rater reliability was good with ICCs higher than 0.9 for both wrist flexion and extension. However, retest values were significantly higher by 4% than test results. The dynamometer was applied with no difficulty to patients with LGMD2C and was sensitive enough to detect strength as weak as 0.82 N.m. From our models, we quantified the mean strength of wrist extension in LGMD2C patients to 39¿±¿17% of their predicted values.Conclusions The MyoWrist dynamometer provides reliable and sensitive measurement of both wrist flexion and extension torques. However, a training session is recommended before starting a study as a small but significant learning effect was observed. Strength deficit can be quantified from predictive equations that were computed from norms of healthy children and adults.
    Full-text · Article · Jan 2015
    • "There are many clinical outcome measures in use pertaining to the upper extremity, such as: manual and quantitative muscle test (MMT, QMT), range of motion (ROM) [1], standardized timed function tests (e.g. 9-hole peg test) [2], Jebsen-Taylor hand function test [3], Wolf Motor Function Test [4], Brooke test [5], Fugl-Meyer assessment, and many others that were developed to evaluate impairments in specific conditions (e.g., [6][7][8][9]). One of the most commonly used standard measures across multiple conditions is ROM. "
    [Show abstract] [Hide abstract] ABSTRACT: In clinical evaluation of upper extremity, there is a lack of assessment methods that are quantitative, reliable, and informative of the overall functional capability of an individual. We present new methodology for the assessment of upper extremity impairments based on the concept of 3-dimensional reachable workspace using Microsoft Kinect. We quantify the reachable workspace by the relative surface area representing the portion of the unit hemi-sphere that is covered by the hand movement. We examine accuracy of joint positions, joint angles, and reachable workspace computation between the Kinect and motion capture system. The results of our analysis in 10 healthy subjects showed that the accuracy of the joint positions was within 66.3 mm for our experimental protocol. We found that the dynamic angle measurements had relatively large deviations (between 9° to 28°. The acquired reachable workspace envelope showed high agreement between the two systems with high repeatability between trials (correlation coefficients between 0.86 and 0.93). The findings indicate that the proposed Kinect-based 3D reachable workspace analysis provides sufficiently accurate and reliable results as compared to motion capture system. The proposed method could be promising for clinical evaluation of upper extremity in neurological or musculoskeletal conditions.
    Full-text · Article · Nov 2013
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