Monitoring daily physical activity of upper extremity in young and adolescent boys with Duchenne muscular dystrophy: A pilot study.

INTRODUCTION: Accelerometry of the upper extremity (UE) potentially provides information on the extent of activities in daily life in patients with Duchenne muscular dystrophy (DMD). The objective of this study is to evaluate the validity of home measurements of UE accelerometry. METHODS: This was a cross-sectional study in 16 patients with DMD (aged 7-17 years). Patients were monitored for 1 to 3 days with two accelerometers on the UE and one accelerometer on the wheelchair. RESULTS: The mean intensity of activity and the mean frequency of transfers of arm elevation from low to middle were approximately twofold higher in patients with a Brooke scale score of 1 or 2 than in patients with a Brooke scale score of 3 or 4. Correlations with the Performance of Upper Limb scale score were high for intensity and for the total frequency of arm elevations per hour. DISCUSSION: Intensity, percentage of time in middle orientation, and frequency of transfers of the upper arm correlated well with functional measurements.

A Novel Setup and Protocol to Measure the Range of Motion of the Wrist and the Hand.

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.

Upper extremity kinematics and muscle activation patterns in subjects with facioscapulohumeral dystrophy.

OBJECTIVE: To compare the kinematics and muscle activity of subjects with facioscapulohumeral dystrophy (FSHD) and healthy control subjects during the performance of standardized upper extremity tasks. DESIGN: Exploratory case-control study. SETTING: A movement laboratory. PARTICIPANTS: Subjects (N=19) with FSHD (n=11) and healthy control subjects (n=8) were measured. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURES: Kinematic data were recorded using a 3-dimensional motion capturing system. Muscle activities, recorded using electromyography, were obtained from 6 superficial muscles around the glenohumeral joint. Shoulder elevation and elbow flexion angles, and maximum electromyographic activity during the movements as a percentage of maximum voluntary contraction (MVC) were calculated. RESULTS: Kinematic differences between the FSHD group and the healthy control group were found in the shoulder elevation angle during single shoulder movements and both reaching tasks. In general, subjects with FSHD had higher percentages of muscle activation. The median activity of the trapezius was close to the MVC activity during the single shoulder movements. Moreover, deltoid and pectoralis muscles were also highly active. CONCLUSIONS: Higher activation of the trapezius in subjects with FSHD indicates a mechanism that could help relieve impaired shoulder muscles during arm elevation around shoulder height. Compared with healthy subjects, persons with FSHD activated their shoulder muscles to a greater extent during movements that required arm elevation.

Head support in wheelchairs (scoping review): state-of-the-art and beyond.

BACKGROUND: Many wheelchair users experience disabilities in stabilising and positioning of the head. For these users, adequate head support is required. Although several types of head supports are available, further development of these systems is needed to improve functionality and quality of life, especially for the group of severely challenged users. For this group, user needs have not been clearly established. In this article, we provide an overview of the state-of-the-art in wheelchair mounted head supports and associated scientific evidence in order to identify requirements for the next generation of head support systems. MATERIALS AND METHODS: A scoping review was performed including scientific literature (PubMed/Scopus), patents (Espacenet/Google Scholar) and commercial information. Types of head support and important system characteristics for future head support systems were proposed from consultations with wheelchair users (n = 3), occupational therapists (n = 3) and an expert panel. RESULTS: Forty scientific papers, 90 patents and 80 descriptions of commercial devices were included in the scoping review. The identified head support systems were categorised per head support type. Only limited scientific clinical evidence with respect to the effectiveness of existing head support systems was found. From the user and expert consultations, a need was identified for personalised head support systems that intuitively combine changes in sitting and head position with continuous optimal support of the head to accommodate severely challenged users. CONCLUSIONS: This study presents the state-of-the-art in head support systems. Additionally, several important system characteristics are introduced that provide guidance for the development and improvement of head supports.Implications for rehabilitationEspecially for the group of severely challenged wheelchair users, current head support systems require further development to improve their users' quality of life.The desired system characteristics which are discussed in this review are an important step in the definition of requirements for the next generation of head supports.

Exploring physiological signals on people with Duchenne muscular dystrophy for an active trunk support: a case study.

BACKGROUND: Arm support devices are available to support people with Duchenne muscular dystrophy (DMD), but active trunk support devices are lacking. An active trunk support device can potentially extend the reach of the arm and stabilize the unstable trunk of people with DMD. In a previous study, we showed that healthy people were able to control an active trunk support using four different control interfaces (based on joystick, force on feet, force on sternum and surface electromyography). All four control interfaces had different advantages and disadvantages. The aim of this study was to explore which of the four inputs is detectably used by people with DMD to control an active trunk support. RESULTS: The results were subject-dependent in both experiments. In the active experiment, the joystick was the most promising control interface. Regarding the static experiment, surface electromyography and force on feet worked for two out of the three subjects. CONCLUSIONS: To our knowledge, this is the first time that people with DMD have engaged in a control task using signals other than those related to their arm muscles. According to our findings, the control interfaces have to be customised to every DMD subject.

Evaluation of intuitive trunk and non-intuitive leg sEMG control interfaces as command input for a 2-D Fitts's law style task.

Duchenne muscular dystrophy (DMD) is a muscular condition that leads to muscle loss. Orthotic devices may present a solution for people with DMD to perform activities of daily living (ADL). One such device is the active trunk support but it needs a control interface to identify the user's intention. Myoelectric control interfaces can be used to detect the user's intention and consequently control an active trunk support. Current research on the control of orthotic devices that use surface electromyography (sEMG) signals as control inputs, focuses mainly on muscles that are directly linked to the movement being performed (intuitive control). However in some cases, it is hard to detect a proper sEMG signal (e.g., when there is significant amount of fat), which can result in poor control performance. A way to overcome this problem might be the introduction of other, non-intuitive forms of control. This paper presents an explorative study on the comparison and learning behavior of two different control interfaces, one using sEMG of trunk muscles (intuitive) and one using sEMG of leg muscles that can be potentially used for an active trunk support (non-intuitive). Six healthy subjects undertook a 2-D Fitts's law style task. They were asked to steer a cursor into targets that were radially distributed symmetrically in five directions. The results show that the subjects were generally able to learn to control the tasks using either of the control interfaces and improve their performance over time. Comparison of both control interfaces demonstrated that the subjects were able to learn the leg control interface task faster than the trunk control interface task. Moreover, the performance on the diagonal-targets was significantly lower compared to the one directional-targets for both control interfaces. Overall, the results show that the subjects were able to control a non-intuitive control interface with high performance. Moreover, the results indicate that the non-intuitive control may be a viable solution for controlling an active trunk support.

Evaluation of Control Interfaces for Active Trunk Support.

A feasibility study was performed to evaluate the control interfaces for a novel trunk support assistive device (Trunk Drive), namely, joystick, force on sternum, force on feet, and electromyography (EMG) to be used by adult men with Duchene muscular dystrophy. The objective of this paper was to evaluate the performance of the different control interfaces during a discrete position tracking task. We built a one degree of freedom flexion-extension active trunk support device that was tested on 10 healthy men. An experiment, based on the Fitts law, was conducted, whereby subjects were asked to steer a cursor representing the angle of the Trunk Drive into a target that was shown on a graphical user interface, using the above-mentioned control interfaces. The users could operate the Trunk Drive via each of the control interfaces. In general, the joystick and force on sternum were the fastest in movement time (more than 40%) without any significant difference between them, but there was a significant difference between force on sternum on the one hand, and EMG and force on feet on the other. All control interfaces proved to be feasible solutions for controlling an active trunk support, each of which had specific advantages.

The modular socket system in a rural setting in Indonesia.

BACKGROUND: Prosthetic services are inaccessible to people living in rural areas. Systems like the modular socket system have the potential to be fabricated outside of the prosthetic workshop. OBJECTIVES: This study aimed to evaluate the patient's performance and satisfaction with the use of the modular socket system, and the technical feasibility of its implementation in a rural setting. STUDY DESIGN: A quantitative longitudinal descriptive study design was followed. METHODS: A total of 15 persons with a lower limb amputation were fitted with the modular socket system and followed over 4-6 months. Performance was measured using a 2-min walk test, 10-m walk test and mobility and function questionnaire. Satisfaction was measured by the Socket Fit Comfort Score, Prosthesis Evaluation Questionnaire and EuroQoL 5 Dimensions 5 Levels. Notes on technical feasibility were taken at the moment of fitting ( t0), at 1-3 months post fitting ( t1) and at the end evaluation at 4-6 months post fitting ( t2). RESULTS: Performance did not change between t0 and t2. The comfort of the socket fit reduced between t0 and t2. Satisfaction with prosthesis and general health status stayed constant over time. The average fitting-time for the modular socket system was 6.4 h. CONCLUSION: The modular socket system can be considered a useful alternative for use in rural settings. Clinical relevance The use of the modular socket system is feasible and can improve accessibility to prosthetic technology in rural areas. Experienced prosthetic users were satisfied with the performance and the device. The shorter manufacturing time and use of only hand-held tools makes it an ideal alternative for use in remote and rural settings.

Different profiles of upper limb function in four types of neuromuscular disorders.

The aim of this research was to study impairments, activity limitations and participation restrictions due to upper limb involvement in people with four different types of neuromuscular disorders (NMD) - FacioScapuloHumeral Dystrophy (FSHD), Limb-Girdle Muscular Dystrophy (LGMD), Spinal Muscular Atrophy (SMA) and Duchenne Muscular Dystrophy (DMD) - and to investigate whether common or different profiles could be identified. Total of 267 respondents with NMD from the Netherlands answered a set of questionnaires covering upper limb impairments (pain and stiffness), activity limitations and participation restrictions. Pain was most often reported by persons with FSHD. Problems in the FSHD group were mainly characterized by relatively high scores for pain and stiffness and low scores for activity limitations. People with LGMD reported also low scores for activity limitations. Conversely, people with SMA and DMD experienced in general relatively low scores for pain and stiffness and high scores for activity limitations. Although people with FSHD and LGMD had relatively few activity limitations, all NMD groups experienced restrictions when participating in social activities. Our results indicate specific profiles of upper limb function in different types of NMD. While the profile observed in persons with FSHD seems to reflect overuse, the profile seen in persons with DMD and SMA is suspicious of disuse, each requiring a specific rehabilitation strategy.

Upper limb function and activity in people with facioscapulohumeral muscular dystrophy: a web-based survey.

Purpose To investigate the upper extremity (UE) at the level of impairments and related activity limitations and participation restrictions in people with facioscapulohumeral muscular dystrophy (FSHD). Methods The study was conducted using web-based questionnaires that were distributed amongst people with FSHD in the Netherlands. Eighty-eight respondents started the survey, and 71 completed it. The questionnaires covered the following dimensions: Function, Activity and Participation of the International Classification of Functioning Disability and Health. Results More than 40% of the respondents experienced pain in one arm or both the arms. Increased pain and stiffness scores and longer disease duration were associated with increased limitation scores. For basic activities, lifting the arm above shoulder-level was most frequently reported as most limited, coherent with the clinical picture of FSHD. Among the respondents, 50% indicated restrictions at school, 78% indicated restrictions at work and more than 80% indicated restrictions whilst participating in sports, hobbies, household activities and romantic relationships. Conclusions This study has shown that alongside the well-known problem of lifting the arms above shoulder-level, UE activities below shoulder height during vocational and occupational activities are also problematic in patients with FSHD. Alongside disease duration, pain and stiffness are associated with UE activity limitations. Implications for Rehabilitation Attention is needed for pain and experienced stiffness in the upper extremity as it is frequently present in patients with FSHD. Rehabilitation professionals need to be aware that patients with FSHD not only experience problems with activities above shoulder height, but also with activities below shoulder height. At least 50% of the patients with FSHD experience restrictions in participation as a result of limitations in their UE.

Domains of Daily Physical Activity in Children with Mitochondrial Disease: A 3D Accelerometry Approach.

Feasible, sensitive and clinically relevant outcome measures are of extreme importance when designing clinical trials. For paediatric mitochondrial disease, no robust end point has been described to date. The aim of this study was to select the domains of daily physical activity, which can be measured by 3D accelerometry, that could serve as sensitive end points in future clinical trials in children with mitochondrial disorders.In this exploratory observational study, 17 patients with mitochondrial disease and 16 age- and sex-matched controls wore 3D accelerometers at the upper leg, upper arm, lower arm and chest during one weekend. Using the raw data obtained by the accelerometers, we calculated the following outcome measures: (1) average amount of counts per hour the sensors were worn; (2) the maximal intensity; (3) the largest area under the curve during 30 min and (4) categorized activities lying, standing or being dynamically active. Measuring physical activity during the whole weekend was practically feasible in all participants. We found good face validity by visually correlating the validation videos and activity diaries to the accelerometer data-graphs. Patients with mitochondrial disorders had significantly lower peak intensity and were resting more, compared to their age- and sex-matched peers.Finally, we suggest domains of physical activity that could be included when measuring daily physical activity in children with mitochondrial disorders, preferably using more user-friendly devices. These include peak activity parameters for the arms (all patients) and legs (ambulatory patients). We recommend using or developing devices that measure these domains of physical activity in future clinical studies.

Upper extremity function and activity in facioscapulohumeral dystrophy and limb-girdle muscular dystrophies: a systematic review.

PURPOSE: The aims of this review were (1) to provide insight into the natural course of upper-extremity (UE) impairments and UE activity limitations associated with facioscapulohumeral dystrophy (FSHD) and limb-girdle muscular dystrophies (LGMD), and (2) to provide an overview of outcome measures used to evaluate UE function and activity in patients with FSHD and LGMD. METHODS: Scientific literature databases (PubMed, MEDLINE, EMBASE, CINAHL and Cochrane) were searched for relevant publications. INCLUSION CRITERIA: (1) studies that included persons with a diagnosis of FSHD or LGMD; and (2) studies that reported the natural course of the UE functions and/or activity with outcome measures at these levels. RESULTS: 247 publications were screened, of which 16 fulfilled the selection criteria. Most studies used manual muscle testing (MMT) to evaluate UE function and the Brooke Scale to evaluate UE mobility activities. The clinical picture of UE impairments and limitations of UE activities in FSHD and LGMD patients was highly variable. In general, FSHD and LGMD patients experience difficulty elevating their upper extremities and the execution of tasks takes considerably longer time. CONCLUSIONS: The clinical course of UE impairments and activity limitations associated with FSHD and LGMD is difficult to predict due to its high variability. Although measures like MMT and the Brooke Scale are often used, there is a lack of more specific outcome measures to assess UE function and UE capacity and performance in daily life. Measures such as 3D motion analysis and electromyography (EMG) recordings are recommended to provide additional insight in UE function. Questionnaires like the Abilhand are recommended to assess UE capacity and accelerometry to assess UE performance in daily life. IMPLICATIONS FOR REHABILITATION: There is a need for specific outcome measures on the level of UE activity. Both the level of capacity and performance should be assessed. Possible outcome measures include 3D motion analysis to assess UE function, questionnaires like the Abilhand to assess UE capacity and accelerometry to assess performance of UE activities in daily life.

Patterns of decline in upper limb function of boys and men with DMD: an international survey.

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.

An overview and categorization of dynamic arm supports for people with decreased arm function.

BACKGROUND: Assistive devices that augment arm function were already introduced during the polio era. Devices are still being developed, but a review has not been performed thus far. OBJECTIVE: To create an overview and categorize assistive devices facilitating arm function in activities of daily living for people with decreased arm function. STUDY DESIGN: Literature review. METHODS: A systematic review in three scientific literature databases. Conference proceedings, assistive technology databases, and references were searched and experts consulted. This resulted in a database of dynamic arm supports. Product information was added, and the devices were categorized. RESULTS: A total of 104 dynamic arm supports were found. These could be categorized as nonactuated devices (N = 39), passively actuated devices (N = 24), actively actuated devices (N = 34), or devices using the functional electrical stimulation principle (N = 7). Functionality analysis resulted in second-level categorization: tremor suppression, facilitation of anti-gravity movement, and assistance of specific joint motion. CONCLUSION: All devices could be ordered in a categorization of low complexity. Many have been developed; most have disappeared and have been succeeded by similar devices. Limitations of the devices found mainly concern interfacing and the range of motion facilitated. Future devices could make use of whatever residual strength is available in the users' arm for control. CLINICAL RELEVANCE: The provided overview of devices in this article and the classification developed is relevant for practitioners seeking assistive solutions for their clients as it makes the range of developed solutions both accessible and comprehensible.

An inverse dynamic analysis on the influence of upper limb gravity compensation during reaching.

Muscular dystrophies (MDs) are characterized by progressive muscle wasting and weakness. Several studies have been conducted to investigate the influence of arm supports in an attempt to restore arm function. Lowering the load allows the user to employ the residual muscle force for movement as well as for posture stabilization. In this pilot study three conditions were investigated during a reaching task performed by three healthy subjects and three MD subjects: a control condition involving reaching; a similar movement with gravity compensation using braces to support the forearm; an identical reaching movement in simulated zero-gravity. In the control condition the highest values of shoulder moments were present, with a maximum of about 6 Nm for shoulder flexion and abduction. In the gravity compensation and zero gravity conditions the maximum shoulder moments were decreased by more than 70% and instead of increasing during reaching, they remained almost unvaried, fluctuating around an offset value less than 1 Nm. Similarly, the elbow moments in the control condition were the highest with a peak around 3.3 Nm for elbow flexion, while the moments were substantially reduced in the remaining two conditions, fluctuating around offset values between 0 to 0.5 Nm. In conclusion, gravity compensation by lower arm support is effective in healthy subjects and MD subjects and lowers the amount of shoulder and elbow moments by an amount comparable to a zero gravity environment. However the influence of gravity compensation still needs to be investigated on more people with MDs in order to quantify any beneficial effect on this population.