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 RehabilitationThere 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

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

Musical Women and Identity-Building in Early Independent Mexico (1821-1854)

This thesis investigates music in Mexico City, with an emphasis on women's relationship to Romanticism, education, consumption, domestic music-making and public performance. During the first decades after independence in 1821, Mexicans began the process of constructing an identity, which musically speaking meant an expansion of the secular musical world. Such construction involved the development of internal activity alongside a conditional receptivity to external influence in the form of the visits of Italian opera companies such as those of Manuel García and Max Maretzek, and travelling virtuosi such as pianist virtuoso Henri Herz, who brought new repertoire and performance practices to Mexican theatres and homes. As consumers and as musicians, women were at the centre of such developments. In Mexico, both European music and that of local musicians was disseminated by means of ladies’ journals and imported and locally-printed sheet music by foreign and Mexican composers, in order to supply a growing home market for amateurs. Abundant surviving repertoire for the home, the widespread availability of musical instruction as revealed through advertisements, and witness accounts of soirées and concerts in the theatre reveal a budding musical world that has hitherto been overlooked and which occurred during a period generally deemed of little importance in Mexican musical history. By investigating a key period in the social-cultural history of Mexican music, this thesis crafts a narrative of intersections between the musical life of Mexican women and the incipient construction of a musical-cultural identity

Design of a dynamic and adaptive head support

For people with severe muscle weakness or paresis in the trunk and neck muscles, adequate head support is required. Although several assistive devices exist that can support a person’s head position, there is an absence of devices that are capable to support head movements in a natural and safe way. The large individual variation between users requires an individual match between user and assistive device. From initial market research it can be concluded that there is a need for assistive devices that provide dynamic adjustability by combining changes in position of the trunk and head with continuous stabilization. Within the project, the main objectives were to characterize this need for support, and to develop a first proof-of-concept of a dynamic and adaptive head support. Position control was implemented on an actuated head support system with four degrees of freedom (e.g. three translations and one rotation in flexion-extension), using a six degree-of-freedom force sensor as a joystick interface. For the current control method, manipulation of the joystick results in the head support following part of the natural flexion-extension motion of the head, coupling multiple degrees of freedom of the actuated system. Additionally, the system can autonomously adapt the head support position according to the back seat angle of the electric wheelchair, to compensate for changes in posture relative to the wheelchair seat caused by changing seat settings. Initial functional testing shows that the current prototype matches the majority of the requirements set in the design phase. Compared to current solutions, the presented system can steer the head support position in 3D in a more efficient and natural way. Therefore, it can be concluded that the redesigned system is a promising first step in the development of a new generation of dynamic and adaptive head supports that are intelligent enough to autonomously personalize their behavior to the user

1st Workshop on Upper-Extremity Assistive Technology for People with Duchenne: State of the art, emerging avenues, and challenges: April 27th 2015, London, United Kingdom

status: publishe