With increasing life expectancy, upper extremity (UE) function becomes more and more important in boys with Duchenne muscular dystrophy (DMD). Knowledge of UE function in these children is, however, limited. The aim of this study was to gain insight into the changing patterns of UE function during the course of DMD. A Web-based questionnaire on UE function, covering all domains of the International Classification of Functioning Disability and Health, was distributed worldwide. Primary domains of the questionnaire were: participant characteristics, UE pain and stiffness, UE activities, and social participation. Data were described per disease stage and analyzed using descriptive analysis. A total of 213 boys/men with DMD (1-35 years) were included in this study. UE pain, stiffness, and activity limitations increased with disease stage. UE activity limitations already occurred in the early ambulatory stage. Compared to the healthy population, social participation was restricted in DMD patients and about 70% of the respondents experienced UE limitations when performing social activities. Despite the existence of UE impairments, only 9% of the respondents used supportive aids. Functional capacities and activities of the UE are limited already in the early ambulatory stage of patients with DMD affecting their social participation. Therefore, clinicians should pay attention to UE limitations before DMD patients lose their capacity to walk. Effective and adequate aids as well as attention for pain and stiffness in the therapeutic management could help to reduce UE activity limitations and related restrictions in social participation.

While there is an extensive number of studies on the development and evaluation of electromyography (EMG)- and force-based control interfaces for assistive devices, no studies have focused on testing these control strategies for the specific case of adults with Duchenne muscular dystrophy (DMD). This paper presents a feasibility study on the use of EMG and force as control interfaces for the operation of active arm supports for men with DMD. We have built an experimental active elbow support, with a threefold objective: 1) to investigate whether adult men with DMD could use EMG- and force-based control interfaces; 2) to evaluate their performance during a discrete position-tracking task; and 3) to examine users' acceptance of the control methods. The system was tested in three adults with DMD (21-22 years). Although none of the three participants had performed any voluntary movements with their arms for the past 3-5 years, all of them were 100% successful in performing the series of tracking tasks using both control interfaces (mean task completion time EMG: [Formula: see text] , force: [Formula: see text] ). While movements with the force-based control were considerably smoother in Subject 3 and faster in Subject 1, EMG based-control was perceived as less fatiguing by all three subjects. Both EMG- and force-based interfaces are feasible solutions for the control of active elbow supports in adults with DMD and should be considered for further investigations on multi-DOF control.

BackgroundPersons suffering from progressive muscular weakness, like those with Duchenne muscular dystrophy (DMD), gradually lose the ability to stand, walk and to use their arms. This hinders them from performing daily activities, social participation and being independent. Wheelchairs are used to overcome the loss of walking. However, there are currently few efficient functional substitutes to support the arms. Arm supports or robotic arms can be mounted to wheelchairs to aid in arm motion, but they are quite visible (stigmatizing), and limited in their possibilities due to their fixation to the wheelchair. The users prefer inconspicuous arm supports that are comfortable to wear and easy to control.MethodsIn this paper the design, characterization, and pilot validation of a passive arm support prototype, which is worn on the body, is presented. The A-gear runs along the body from the contact surface between seat and upper legs via torso and upper arm to the forearm. Freedom of motion is accomplished by mechanical joints, which are nearly aligned with the human joints. The system compensates for the arm weight, using elastic bands for static balance, in every position of the arm. As opposed to existing devices, the proposed kinematic structure allows trunk motion and requires fewer links and less joint space without compromising balancing precision.The functional prototype has been validated in three DMD patients, using 3D motion analysis.ResultsMeasurements have shown increased arm performance when the subjects were wearing the prototype. Upward and forward movements were easier to perform. The arm support is easy to put on and remove. Moreover, the device felt comfortable for the subjects. However, downward movements were more difficult, and the patients would prefer the device to be even more inconspicuous.ConclusionThe A-gear prototype is a step towards inconspicuousness and therefore well-received dynamic arm supports for people with muscular weakness.Electronic supplementary materialThe online version of this article (doi:10.1186/s12984-015-0072-y) contains supplementary material, which is available to authorized users.

The human hand is important for the performance of activities of daily living which are directly related to quality of life. Various conditions, such as Duchenne muscular dystrophy (DMD) can affect the function of the human hand and wrist. The ability to assess the impairment in the hand and the wrist by measuring the range of motion (ROM), is essential for the development of effective rehabilitation protocols. Currently the clinical standard is the goniometer. In this study we explore the feasibility and reliability of an optical sensor (Leap motion sensor) in measuring active hand/wrist ROM. We measured the hand/wrist ROM of 20 healthy adults with the goniometer and the Leap motion sensor, in order to check the agreement between the two methods and additionally, we performed a test-retest of the Leap motion sensor with 12 of them, to assess its reliability. The results suggest low agreement between the goniometer and the leap motion sensor, yet showing a large decrease in measurement time and high reliability when using the later. Despite the low agreement between the two methods, we believe that the Leap motion sensor shows potential to contribute to the development of hand rehabilitation protocols and be used with patients in a clinical setting.

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BackgroundTherapeutic management of upper extremity (UE) function of boys and men with Duchenne Muscular Dystrophy (DMD) requires sensitive and objective assessment. Therefore, we aimed to measure physiologic UE function of healthy subjects and DMD patients in different disease stages, and to evaluate the relation between these physiologic measures and functional UE scales.MethodsTwenty-three DMD patients and twenty healthy controls (7–23 years) participated in this explorative case–control study. Maximal muscle torque, maximal and normalized surface electromyography (sEMG) amplitudes, muscle thickness, echogenicity and maximal passive and active joint angles were measured. At activity level, Brooke upper extremity rating scale and the Performance of Upper Limb (PUL) scale were used.ResultsOutcome measures related to proximal UE function could discriminate between disease stages. Increased normalized sEMG amplitudes were found in patients, even in early disease stages. Maximal active joint angles showed the strongest relation to Brooke scale (R 2 = 0.88) and PUL scale (R 2 = 0.85).ConclusionsThe decline of muscle functions precedes the decline in performance of UE activities, and therefore may play a role in early detection of UE limitations. Increased sEMG levels demonstrate that DMD patients use more of their muscle capacity compared to healthy subjects, to perform daily activities. This might result in increased fatigability. Active maximal joint angles are highly related to functional scales, so preserving the ability to use the full range of motion is important for the performance of daily activities. Close monitoring of active joint angles could therefore help in starting interventions that minimize functional UE decline in DMD patients timely.

BackgroundAdults with Duchenne muscular dystrophy (DMD) can benefit from devices that actively support their arm function. A critical component of such devices is the control interface as it is responsible for the human-machine interaction. Our previous work indicated that surface electromyography (sEMG) and force-based control with active gravity and joint-stiffness compensation were feasible solutions for the support of elbow movements (one degree of freedom). In this paper, we extend the evaluation of sEMG- and force-based control interfaces to simultaneous and proportional control of planar arm movements (two degrees of freedom).MethodsThree men with DMD (18–23 years-old) with different levels of arm function (i.e. Brooke scores of 4, 5 and 6) performed a series of line-tracing tasks over a tabletop surface using an experimental active arm support. The arm movements were controlled using three control methods: sEMG-based control, force-based control with stiffness compensation (FSC), and force-based control with no compensation (FNC). The movement performance was evaluated in terms of percentage of task completion, tracing error, smoothness and speed.ResultsFor subject S1 (Brooke 4) FNC was the preferred method and performed better than FSC and sEMG. FNC was not usable for subject S2 (Brooke 5) and S3 (Brooke 6). Subject S2 presented significantly lower movement speed with sEMG than with FSC, yet he preferred sEMG since FSC was perceived to be too fatiguing. Subject S3 could not successfully use neither of the two force-based control methods, while with sEMG he could reach almost his entire workspace.ConclusionsMovement performance and subjective preference of the three control methods differed with the level of arm function of the participants. Our results indicate that all three control methods have to be considered in real applications, as they present complementary advantages and disadvantages. The fact that the two weaker subjects (S2 and S3) experienced the force-based control interfaces as fatiguing suggests that sEMG-based control interfaces could be a better solution for adults with DMD. Yet force-based control interfaces can be a better alternative for those cases in which voluntary forces are higher than the stiffness forces of the arms.

Due to progressive muscle weakness, the arm function in boys with Duchenne muscular dystrophy (DMD) reduces. An arm support can compensate for this loss of function. Existing arm supports are wheelchair bound, which restricts the ability to perform trunk movements. To evaluate the function of a body-bound arm support, a prototype (based on the Wilmington robotic exoskeleton (WREX) arm support) that allows trunk movements was built. In order to examine the effect of this device and to compare it with an existing wheelchair-bound device, three healthy subjects performed single joint movements (SJMs) and activities of daily living (ADL) with and without the devices. The range of motion (RoM) of the arm and the surface electromyography (sEMG) signal of five different arm muscles were measured. The range of motion increased when compared to the wheelchair-bound device, and the trunk motion was perceived as important to make specific movements easier and more natural, especially the more extreme movements like reaching for a far object and reaching to the top of the head. The sEMG signal was comparable to that of the wheelchair-bound device. This means that an arm support with trunk motion capability can increase the range of motion of the user, while the amount of support to the arm is equal.