Long-term outcomes for females with early-onset dystrophinopathy.

AIM: To study long-term disease course for females with early-onset dystrophinopathy, including common (female) symptoms, challenges in social participation, the need for care, and current healthcare management to support guideline development. METHOD: Twelve females with early-onset dystrophinopathy were followed for a median period of more than 17 years (range 1-36). RESULTS: One patient died owing to end-stage cardiac failure. Cardiac abnormalities were observed in three of the remaining 11 participants. Respiratory function was reduced in seven of 10 participants. Fatigue, myalgia, lower back pain, and arthralgia were reported in more than six of the participants. Functional status varied from exercise intolerance to wheelchair dependency. Most or all of the 10 participants reported restrictions in participation in work (n = 10), household duties (n = 10), sports (n = 9), and education (n = 8). Only a few participants received followed-up pulmonary (n = 2) or rehabilitation (n = 3) care. INTERPRETATION: Females with early-onset dystrophinopathy experience a wide range of impairments, comorbidities, limitations in activities, and restrictions in social participation. The whole spectrum should be acknowledged in the healthcare setting. Neuromuscular and cardiac follow-up are indispensable. Additional respiratory assessment and rehabilitation care are expected to improve health status and support daily activities and participation. WHAT THIS PAPER ADDS: No standard diagnostic procedures seem to exist for female patients suspected for dystrophinopathy. Female participants with early-onset dystrophinopathy experienced a broad scope of burdening symptoms, such as fatigue, myalgia, lower back pain, and arthralgia. None of participants worked full time, all felt restricted in paid work, and most felt restricted in education. Most participants showed decreased lung function, while only one was symptomatic. Availability of rehabilitation care may improve support for daily activities and participation for females with early-onset dystrophinopathy.

Using PRPP-Assessment for measuring change in everyday activities by home-based videos: An exploratory case series study in children with multiple disabilities.

BACKGROUND: Currently, paediatric health care aims to use a child-centred tailor-made approach. In order to design tailored occupational therapy, the implementation of personalised occupation-based measurements that guide and evaluate goal setting and are responsive to change is necessary. PURPOSE: Primarily, this study explored the potential of the Perceive, Recall, Plan, and Perform (PRPP) assessment to measure the change in the performance of children with multiple disabilities. As a secondary evaluation, the feasibility of the PRPP-Intervention in a home-based program to enable activities was described. The overall aim is to show the potential of the PRPP-Assessment as an outcome measure to use as a base for designing tailor-made person-centred care. METHODS: An exploratory longitudinal multiple case series mixed-methods design was used. The PRPP-Assessment, scored by multiple raters, was conducted based on parent-provided videos. The assessed activities were chosen by the child and/or parents. Responsiveness was evaluated by hypotheses formulated a priori and by comparing measured change with change on concurrent measures: Goal Attainment Scaling (GAS) and Canadian Occupational Performance Measure (COPM). Over a 6-week period, children and their parents (or caregivers) participated in an online home-based video coaching program where parents were coached in the implementation of the training, based on the PRPP-Intervention, by paediatric occupational therapists on a weekly basis. The feasibility of the intervention was explored using semi-structured interviews with children, parents, and the treating occupational therapists and was analysed by directed content analysis. RESULTS: Three out of 17 eligible children agreed to participate and completed post-intervention measurement, of which two completed the intervention. Quantitative results showed that eight out of nine activities improved on the PRPP-Assessment and the COPM, and nine improved on the GAS. In total, 13 out of 15 hypotheses for responsiveness were accepted. Participants experienced the intervention as successful and acceptable. Facilitators and concerns over demand, implementation, practicality, integration, and adaptation were shared. CONCLUSION: The PRPP-Assessment showed the potential to measure change in a heterogeneous group of children. The results indicated a positive tendency for the intervention and also provide directions for further development.

Martial Arts Training for Boys With Duchenne Muscular Dystrophy.

PURPOSE: The primary aim of this pilot study was to investigate the safety and feasibility of a 3-month martial arts-based training (MAT) program for patients with Duchenne muscular dystrophy (DMD). The secondary aim was to examine changes in physical and psychosocial abilities after participating in the MAT program. METHODS: Twelve patients with DMD (10 ambulant and 2 nonambulant) were included. The MAT program was evaluated on feasibility and safety. Changes in physical abilities were measured using the Motor Function Measure, Performance of Upper Limb scale, and the North Star Ambulatory Assessment. Changes in psychosocial abilities were measured using the Strength and Difficulties Questionnaire, Personal Adjustment and Role Skills for DMD, and the Self-Perception Profile for Children/Adolescents. RESULTS: Two participants did not complete the MAT program. Attendance rate for the 10 remaining participants was 91%. Eleven falls were reported during the training, but these falls did not result in injuries. Therefore, the MAT program was found feasible and safe. After completing the MAT program, most participants showed an improvement of their psychosocial abilities, and their physical abilities did not show deterioration. CONCLUSION: The MAT program is feasible and safe for boys with DMD.

Occurrence of symptoms in different stages of Duchenne muscular dystrophy and their impact on social participation.

INTRODUCTION/AIMS: As life expectancy improves for patients with Duchenne muscular dystrophy (DMD), new symptoms are likely to arise. This aims of this study are: (1) to explore the prevalence of a broad variety of symptoms in the various stages of DMD (with and without steroid use); (2) to explore the prevalence of common secondary diagnoses; and (3) to evaluate the social participation level of patients with DMD older than 16 y of age; and to explore correlations between social participation and symptoms. METHODS: A cross-sectional self-report questionnaire, including questions on functional level and health status, as well as a standardized participation scale was distributed among Dutch patients with DMD. RESULTS: Eighty-four male patients with a mean age of 22.0 (SD = 10.0) y were enrolled. The most prevalent and limiting symptoms were difficulty coughing (58%), coldness of hands (57%), contractures (51%), stiffness (49%), fatigue (40%), myalgia (38%), and low speech volume (33%). Prevalent secondary diagnoses included cardiac disease (14%), neurobehavioral diagnosis (13%), low blood pressure (13%), and arthrosis (5%). Social participation correlated negatively with coldness of hands (r = - .29; P < .03), decreased intelligibility (r = - .40; P < .003), and chewing problems (r = - .33; P < .02). DISCUSSION: The prevalence of a broad spectrum of symptoms and secondary diagnoses is high in patients with DMD, and some of these symptoms are correlated with social participation. Growing awareness of new symptoms and secondary diagnoses among patients, caregivers, and professionals can enhance their recognition, possibly facilitating prevention and early treatment.

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

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.

Orofacial muscles may be affected in early stages of Becker muscular dystrophy: A preliminary study.

BACKGROUND: Dysphagia is reported in patients with Duchenne or Becker muscular dystrophy. Our clinical experience suggests that, compared with Duchenne patients, impaired mastication and swallowing occur early in Becker patients relative to their skeletal muscle involvement. The aim of this study was to assess dysphagia in Duchenne and Becker patients in relation to ambulatory capacity. METHODS: In patients in the early ambulatory stage, clinical symptoms, quantitative muscle ultrasound of the orofacial muscles, and maximum bite force were assessed. The 6-Minute Walk Test (6MWT) was used to measure ambulatory capacity. RESULTS: Eleven Duchenne and 11 Becker patients were included. Although Becker patients had a greater 6MWT distance than Duchenne patients, the occurrence of mastication and swallowing difficulties was similar. The temporalis muscle was significantly thicker in Becker patients. CONCLUSIONS: Clinicians should be aware of dysphagia in both groups, even when ambulation is still well preserved.

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.

Quantitative description of upper extremity function and activity of people with spinal muscular atrophy.

BACKGROUND: Therapeutic management of the upper extremity (UE) function of people with spinal muscular atrophy (SMA) requires sensitive and objective assessment. Therefore, we aimed to measure physiologic UE function of SMA patients with different functional abilities and evaluate the relation between these physiologic measures and functional UE scales. METHODS: 12 male and 5 female SMA patients (mean age 42 years; range 6-62 years) participated in this explorative study. Concerning the physiologic level, the maximal muscle torque, the maximal and normalized surface electromyography (sEMG) amplitudes, and the maximal passive and active joint angles were measured. Concerning the activity level, the Performance of the Upper Limb (PUL) scale was used, and hand function was examined using the Nine-Hole Peg Test and the Timed Test of In-Hand Manipulation (TIHM). RESULTS: Outcome measures that significantly related to the functional ability were: the PUL score (all dimensions); the finger to palm task of the Timed TIHM; biceps, triceps, and forearm extensor strength; and the active range of motion of shoulder abduction, shoulder flexion, and wrist extension. In addition, the following physiologic variables were related to the activity level (PUL score): hand function (the Nine-Hole Peg Test; Rs = - 0.61), the Timed TIHM (Rs = - 0.53), the maximal muscle torque (Rs = 0.74), the maximal sEMG amplitude (Rs = 0.79), and the maximal active joint angle (Rs = 0.88). CONCLUSIONS: Muscle functions in SMA patients are already affected before activity limitations are noticeable. Consequently, monitoring the maximal muscle strength and the normalized muscle activity during task performance could play a role in the early detection of UE limitations. The mechanism behind the loss of arm activities due to SMA is primarily caused by decreasing muscle capacity, which influences the ability to move an arm actively. In clinical practices, these dimensions should be considered separately when monitoring disease progression in order to better evaluate the need for interventions.

Facioscapulohumeral Dystrophy in Childhood: A Nationwide Natural History Study.

OBJECTIVE: Facioscapulohumeral dystrophy (FSHD) is one of the most frequent heritable muscular dystrophies, with a large variety in age at onset and disease severity. The natural history and molecular characteristics of FSHD in childhood are incompletely understood. Our objective is to clinically and genetically characterize FSHD in childhood. METHODS: We performed a nationwide, single-investigator, natural history study on FSHD in childhood. RESULTS: Multiple-source recruitment resulted in 32 patients with FSHD (0-17 years), leading to an estimated prevalence of 1 in 100,000 children in The Netherlands. This series of 32 children with FSHD revealed a heterogeneous phenotype and genotype in childhood. The phenotypic hallmarks of FSHD in childhood are: facial weakness with normal or only mildly affected motor performance, decreased functional exercise capacity (6-minute walk test), lumbar hyperlordosis, and increased echo intensity on muscle ultrasonography. In addition, pain and fatigue were frequent and patients experienced a lower quality of life compared to healthy peers. In contrast to the literature on early-onset FSHD, systemic features such as hearing loss and retinal and cardiac abnormalities were infrequent and subclinical, and epilepsy and intellectual disability were absent. Genotypically, patients had a mean D4Z4 repeat array of 5 units (range, 2-9), and 14% of the mutations were de novo. INTERPRETATION: FSHD in childhood is more prevalent than previously known and the genotype resembles classic FSHD. Importantly, FSHD mainly affects functional exercise capacity and quality of life in children. As such, these results are paramount for counseling, clinical management, and stratification in clinical research. Ann Neurol 2018;84:635-645.

Daily Functioning and Quality of Life in Patients with Sjögren-Larsson Syndrome.

AIM: Sjögren-Larsson syndrome (SLS) is an autosomal recessively inherited neurometabolic disease caused by an enzyme defect in lipid metabolism. Patients suffer from intellectual disability, bilateral spastic paresis, ichthyosis, visual impairment, and photophobia. Knowledge about the meaning of having SLS in daily life is lacking. METHODS: Sixteen parents or caregivers of patients with SLS were asked to fill out online questionnaires about daily functioning, quality of life, feeding and swallowing problems, skin treatment, female hormonal status, and greatest problems. RESULTS: Questionnaires were filled out by parents or caregivers of six children and 10 adult patients, age range 11 to 58 years. The median quality of life score was 73 (range: 26-100). Most often reported problems were itchy skin, reduced mobility, and dependency. Feeding and swallowing problems were reported in 75% of the patients. Mood problems were rarely mentioned. DISCUSSION: Despite the large disruptions of daily functioning, patients with SLS are according to their parents generally content with their quality of life and participation. There was a broad range in reported problems. We found it very useful to systematically ask parents about their children's feelings and needs, to better understand the meaning of living with a complex disorder like SLS.

Ataxia-telangiectasia: recommendations for multidisciplinary treatment.

Ataxia-telangiectasia is a rare, neurodegenerative, and multisystem disease, characterized by cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, progressive respiratory failure, and an increased risk of malignancies. It demands specialized care tailored to the individual patient's needs. Besides the classic ataxia-telangiectasia phenotype, a variant phenotype exists with partly overlapping but some distinctive disease characteristics. This guideline summarizes frequently encountered medical problems in the disease course of patients with classic and variant ataxia-telangiectasia, in the domains of neurology, immunology and infectious diseases, pulmonology, anaesthetic and perioperative risk, oncology, endocrinology, and nutrition. Furthermore, it provides a practical guide with evidence- and expert-based recommendations for the follow-up and treatment of all these different clinical topics.

Dynamic arm study: quantitative description of upper extremity function and activity of boys and men with duchenne muscular dystrophy.

BACKGROUND: Therapeutic 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. METHODS: Twenty-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. RESULTS: Outcome 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). CONCLUSIONS: The 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.

Surface EMG signals in very late-stage of Duchenne muscular dystrophy: a case study.

BACKGROUND: Robotic arm supports aim at improving the quality of life for adults with Duchenne muscular dystrophy (DMD) by augmenting their residual functional abilities. A critical component of robotic arm supports is the control interface, as is it responsible for the human-machine interaction. Our previous studies showed the feasibility of using surface electromyography (sEMG) as a control interface to operate robotic arm supports in adults with DMD (22-24 years-old). However, in the biomedical engineering community there is an often raised skepticism on whether adults with DMD at the last stage of their disease have sEMG signals that can be measured and used for control. FINDINGS: In this study sEMG signals from Biceps and Triceps Brachii muscles were measured for the first time in a 37 year-old man with DMD (Brooke 6) that lost his arm function 15 years ago. The sEMG signals were measured during maximal and sub-maximal voluntary isometric contractions and evaluated in terms of signal-to-noise ratio and co-activation ratio. Beyond the profound deterioration of the muscles, we found that sEMG signals from both Biceps and Triceps muscles were measurable in this individual, although with a maximum signal amplitude 100 times lower compared to sEMG from healthy subjects. The participant was able to voluntarily modulate the required level of muscle activation during the sub-maximal voluntary isometric contractions. Despite the low sEMG amplitude and a considerable level of muscle co-activation, simulations of an elbow orthosis using the measured sEMG as driving signal indicated that the sEMG signals of the participant had the potential to provide control of elbow movements. CONCLUSIONS: To the best of our knowledge this is the first time that sEMG signals from a man with DMD at the last-stage of the disease were measured, analyzed and reported. These findings offer promising perspectives to the use of sEMG as an intuitive and natural control interface for robotic arm supports in adults with DMD until the last stage of the disease.

Multidisciplinary Treatment for Adolescents with Chronic Pain and/or Fatigue: Who Will Benefit?

PURPOSE: The aims of this study were, first, to determine the percentage of adolescents with chronic pain/fatigue successfully treated with rehabilitation treatment for chronic pain/fatigue and, second, to identify predictors for a successful rehabilitation treatment. METHODS: Treatment success is scored based on a combination of predefined clinically relevant changes in 4 outcome measures: level of pain/fatigue, school absence, physical functioning, and psychosocial functioning. A forward stepwise logistic regression analysis with treatment success as a dependent variable is performed to identify predictors for successful treatment. RESULTS: A total of 172 adolescents (mean age 16.2 [SD = 2.5]; 85.5% girls) participated. Almost half (49.6%) of the adolescents had a successful treatment. The explained variance for the complete model explaining treatment success was 49% (R2 = 0.487). Patients with a higher level of pain/fatigue and a passive coping style pretreatment improved most, and these factors could thus be indicated as predictors for successful treatment. Also, gender significantly contributed to the prediction, in favor of boys. CONCLUSIONS: Regarding the first aim, using predefined treatment success based on clinically relevant changes, half of the participants had a successful treatment. Concerning the second aim, adolescents with a high level of pain/fatigue and those with a high passive coping style pretreatment have a better ability to change their functioning during treatment. Boys benefit more than girls.

International Paediatric Mitochondrial Disease Scale.

OBJECTIVE: There is an urgent need for reliable and universally applicable outcome measures for children with mitochondrial diseases. In this study, we aimed to adapt the currently available Newcastle Paediatric Mitochondrial Disease Scale (NPMDS) to the International Paediatric Mitochondrial Disease Scale (IPMDS) during a Delphi-based process with input from international collaborators, patients and caretakers, as well as a pilot reliability study in eight patients. Subsequently, we aimed to test the feasibility, construct validity and reliability of the IPMDS in a multicentre study. METHODS: A clinically, biochemically and genetically heterogeneous group of 17 patients (age 1.6-16 years) from five different expert centres from four different continents were evaluated in this study. RESULTS: The feasibility of the IPMDS was good, as indicated by a low number of missing items (4 %) and the positive evaluation of patients, parents and users. Principal component analysis of our small sample identified three factors, which explained 57.9 % of the variance. Good construct validity was found using hypothesis testing. The overall interrater reliability was good [median intraclass correlation coefficient for agreement between raters (ICCagreement) 0.85; range 0.23-0.99). CONCLUSION: In conclusion, we suggest using the IPMDS for assessing natural history in children with mitochondrial diseases. These data should be used to further explore construct validity of the IPMDS and to set age limits. In parallel, responsiveness and the minimal clinically important difference should be studied to facilitate sample size calculations in future clinical trials.

Facioscapulohumeral dystrophy in children: design of a prospective, observational study on natural history, predictors and clinical impact (iFocus FSHD).

BACKGROUND: Facioscapulohumeral muscular dystrophy (FSHD; OMIM 158900 & 158901) is a progressive skeletal muscle dystrophy, characterized by an autosomal dominant inheritance pattern. One of the major unsolved questions in FSHD is the marked clinical heterogeneity, ranging from asymptomatic individuals to severely affected patients with an early onset. An estimated 10% of FSHD patients have an early onset (onset before 10 years of age) and are traditionally classified as infantile FSHD. This subgroup is regarded as severely affected and extra-muscular symptoms, such as hearing loss and retinopathy, are frequently described. However, information on the prevalence, natural history and clinical management of early onset FSHD is currently lacking, thereby hampering adequate patient counselling and management. Therefore, a population-based prospective cohort study on FSHD in children is highly needed. METHODS/DESIGN: This explorative study aims to recruit all children (aged 0-17 years) with a genetically confirmed diagnosis of FSHD in The Netherlands. The children will be assessed at baseline and at 2-year follow-up. The general aim of the study is the description of the clinical features and genetic characteristics of this paediatric cohort. The primary outcome is the motor function as measured by the Motor Function Measure. Secondary outcomes include quantitative and qualitative description of the clinical phenotype, muscle imaging, genotyping and prevalence estimations. The ultimate objective will be a thorough description of the natural history, predictors of disease severity and quality of life in children with FSHD. DISCUSSION: The results of this population-based study are vital for adequate patient management and clinical trial-readiness. Furthermore, this study is expected to provide additional insight in the epigenetic and environmental disease modifying factors. In addition to improve counselling, this could contribute to unravelling the aetiology of FSHD. TRIAL REGISTRATION: clinicaltrials.gov NCT02625662.

Erratum to: Design and pilot validation of A-gear: a novel wearable dynamic arm support.

Unfortunately, the original version of this article [1] contained an error. Equation 6 was included incorrectly: in the original equation variable slinks3 was missing.The correct Equation 6 can be found below:

Design and pilot validation of A-gear: a novel wearable dynamic arm support.

BACKGROUND: Persons 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. METHODS: In 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. RESULTS: Measurements 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. CONCLUSION: The A-gear prototype is a step towards inconspicuousness and therefore well-received dynamic arm supports for people with muscular weakness.

Development of the writing readiness inventory tool in context (WRITIC).

This article describes the development of the Writing Readiness Inventory Tool in Context (WRITIC), a measurement evaluating writing readiness in Dutch kindergarten children (5 and 6 years old). Content validity was established through 10 expert evaluations in three rounds. Construct validity was established with 251 children following regular education. To identify scale constructs, factor analysis was performed. Discriminative validity was established by examining contrast groups with good (n = 142) and poor (n = 109) performers in paper-and-pencil tasks. Content validity was high with 94.4% agreement among the experts. Two reliable factors were found in the performance of paper-and-pencil tasks with Cronbach's alphas of 0.82 and 0.69 respectively. The contrast groups differed significantly in two WRITIC subdomains: "Sustained attention" and "Task performance". Our findings indicated that the WRITIC is feasible for use in the classroom.

Writing Readiness Inventory Tool in Context (WRITIC): reliability and convergent validity.

BACKGROUND/AIM: This study examined the reliability and convergent validity of the Writing Readiness Inventory Tool in Context, a measurement evaluating writing readiness in kindergarten children (aged from five to six years). METHODS: Test-retest reliability was established with 59 children, inter-rater reliability with 72 children and convergent validity with 119 children. All participants were typically developing kindergarten children. Convergent validity was examined with the Beery-Buktenica Developmental Test of Visual-Motor Integration and the Nine-Hole Peg Test. RESULTS: We found excellent test-retest and inter-rater reliability on the future norm-referenced subdomain 'Task performance' of Writing Readiness Inventory Tool in Context with intra-class correlation coefficient ranging from 0.92 to 0.95. On the other criterion-referenced subdomains, we found fair to good reliability with intra-class correlation coefficient ranging from 0.70 to 1.0 and weighted Kappa ranging from 0.30 to 0.89. Correlations with the Beery-Buktenica Developmental Test of Visual-Motor Integration and the Nine-Hole Peg Test were moderate with rs ranging from 0.34 to 0.40 and these are comparable with correlations in other handwriting studies. CONCLUSION: Writing Readiness Inventory Tool in Context is an assessment of writing readiness that is stable over time and between raters. The expected moderate correlations with the Beery-Buktenica Developmental Test of Visual-Motor Integration and the Nine-Hole Peg Test support the construct of writing readiness.