Stronger relation between impairment and manual capacity in the non-dominant hand than the dominant hand in congenital hand differences; implications for surgical and therapeutic interventions.

OBJECTIVES: To evaluate manual activity capacity (i.e. activity capacity to perform hand activities) and its relation with body functions of the hand and forearm in children with congenital hand differences (CHD) METHODS: We assessed 10-14 year-old children with CHD (N = 106) using a functional handgrips test. Measurements of body functions included joint mobility and muscle strength. Patient characteristics were hand dominance and severity. RESULTS: We found a stronger relation between body functions and manual activity capacity in non-dominant hands than dominant hands. Dominant hands scored significantly higher on manual activity capacity than nondominant hands that were similarly impaired at body functions level. Severity of the CHD and body functions had only small effects on manual activity capacity. CONCLUSION: The relation between body functions and manual activity capacity is stronger in non-dominant hands than dominant hands, indicating that improvement in body functions lead to larger changes in manual activity capacity in the non-dominant hand. This may suggest that in bilaterally-affected children surgery should be done at the non-dominant hand first since this hand would benefit most from surgery-induced body functions improvement.

Musculoskeletal complaints and disability in a group of young adults with major congenital upper limb differences in The Netherlands.

PURPOSE: To determine prevalence of musculoskeletal complaints (MSCs) in adults with major congenital upper limb differences (CoULD) compared to able-bodied controls, and to examine associations of MSCs and disability with various biopsychosocial factors. MATERIALS AND METHODS: Questionnaire-based cross-sectional study assessing MSCs, disability (using the Disabilities of the Arm, Shoulder and Hand questionnaire (DASH)), general and mental health status, physical work demands, and upper extremity range of motion. RESULTS: Seventy-one individuals with CoULD (participation rate: 41%) and 71 controls matched on age, gender, and education were included (49% female, mean age 28.9 years). Year prevalence of MSCs was significantly higher in the CoULD group (35%) than in the control group (18%). The CoULD group was less often employed and had lower scores on all measures of upper limb range of motion and hand grip. MSCs were associated with higher DASH scores and higher reported work demands. Disability was associated with female gender, more joints with limited range of motion, unemployment, and lower general and mental health. Factors associated with disability did not differ between groups. CONCLUSIONS: MSCs are a frequent problem in young adults with major CoULD. To prevent or reduce MSC and disability, clinicians and researchers should be aware of the associated factors. Implications for rehabilitationThe year prevalence of musculoskeletal complaints (MSCs) in those with major congenital upper limb differences (CoULD) was approximately double to that of the control group, implying a potential relationship between CoULD and MSCs.Rehabilitation professionals should develop personalized strategies to manage work demands in those with CoULD, considering the association between MSCs and higher reported work demands.Recognizing the impact of a negatively perceived body image on mental health, clinicians should integrate psychological counseling into rehabilitation treatments to support mental well-being and improve overall quality of life in those with CoULD.Rehabilitation professionals should educate individuals with CoULD about the potential associations between upper limb work demands, MSCs, and disability.

Diaphragmatic dysfunction in neuromuscular disease, an MRI study.

The aim of this exploratory study was to evaluate diaphragmatic function across various neuromuscular diseases using spirometry-controlled MRI. We measured motion of the diaphragm relative to that of the thoracic wall (cranial-caudal ratio vs. anterior posterior ratio; CC-AP ratio), and changes in the diaphragmatic curvature (diaphragm height and area ratio) during inspiration in 12 adults with a neuromuscular disease having signs of respiratory muscle weakness, 18 healthy controls, and 35 adult Pompe patients - a group with prominent diaphragmatic weakness. CC-AP ratio was lower in patients with myopathies (n=7, 1.25±0.30) and motor neuron diseases (n=5, 1.30±0.10) than in healthy controls (1.37±0.14; p=0.001 and p=0.008), but not as abnormal as in Pompe patients (1.12±0.18; p=0.011 and p=0.024). The mean diaphragm height ratio was 1.17±0.33 in patients with myopathies, pointing at an insufficient diaphragmatic contraction. This was also seen in patients with Pompe disease (1.28±0.36), but not in healthy controls (0.82±0.33) or patients with motor neuron disease (0.82±0.24). We conclude that spirometry-controlled MRI enables us to investigate respiratory dysfunction across neuromuscular diseases, suggesting that the diaphragm is affected in a different way in myopathies and motor neuron diseases. Whether MRI can also be used to evaluate progression of diaphragmatic dysfunction requires additional studies.

Perception and control of low cable operation forces in voluntary closing body-powered upper-limb prostheses.

Operating a body-powered prosthesis can be painful and tiring due to high cable operation forces, illustrating that low cable operation forces are a desirable design property for body-powered prostheses. However, lower operation forces might negatively affect controllability and force perception, which is plausible but not known. This study aims to quantify the accuracy of cable force perception and control for body-powered prostheses in a low cable operation force range by utilizing isometric and dynamic force reproduction experiments. Twenty-five subjects with trans-radial absence conducted two force reproduction tasks; first an isometric task of reproducing 10, 15, 20, 25, 30 or 40 N and second a force reproduction task of 10 and 20 N, for cable excursions of 10, 20, 40, 60 and 80 mm. Task performance was quantified by the force reproduction error and the variability in the generated force. The results of the isometric experiment demonstrated that increasing force levels enlarge the force variability, but do not influence the force reproduction error for the tested force range. The second experiment showed that increased cable excursions resulted in a decreased force reproduction error, for both tested force levels, whereas the force variability remained unchanged. In conclusion, the design recommendations for voluntary closing body-powered prostheses suggested by this study are to minimize cable operation forces: this does not affect force reproduction error but does reduce force variability. Furthermore, increased cable excursions facilitate users with additional information to meet a target force more accurately.

Living with transversal upper limb reduction deficiency: limitations experienced by young adults during their transition to adulthood.

INTRODUCTION: During transition to adulthood young adults with disabilities are at risk of experiencing limitations due to changing physical and social requirements. PURPOSE: To determine whether young adults with transversal upper limb reduction deficiency (tULRD) have experienced limitations in various domains of participation during transition to adulthood and how they dealt with these limitations. PARTICIPANTS: Fifteen participants (mean age 21.4 years) with tULRD. METHODS: A qualitative study was performed using a semi-structured interview based on the Rotterdam Transition Profile to identify the limitations experienced in participation domains. RESULTS: Almost all the participants reported difficulties in finding a suitable study or job. Most young adults were convinced they were suitable for almost any study or job, but their teachers and potential employers were more reserved. Few difficulties were reported on the domains leisure activities, intimate relationships/sexuality, housing/housekeeping and transportation. Participants preferred to develop their own strategies for dealing with limitations. Various aids, adaptations and prostheses were used to overcome limitations. Rehabilitation teams were infrequently consulted for advice in solving transitional problems. CONCLUSION: Young adults with tULRD experience limitations mainly in choosing and finding a suitable study or job. Rehabilitation teams may play a more extensive role in supporting individuals with transitional problems. Implications for rehabilitation Most young adults with transversal upper limb reduction deficiency (tULRD) experience limitations in study and job selection during transition to adulthood, but they do not consult the rehabilitation team. Assessment of abilities in relation to job interests and practicing job specific bimanual activities may be helpful for young adults with a tULRD. How the rehabilitation teams can meet the needs of young adults with tULRD during transitional phases, when autonomy is of growing importance, should be investigated further.

Assessment of arm/hand functioning in children with a congenital transverse or longitudinal reduction deficiency of the upper limb.

PURPOSE: Selection of appropriate functional tests and questionnaires to assess capacity (tests) and performance (questionnaires) of arm/hand functioning in children with congenital transverse or longitudinal (radius dysplasia) reduction deficiencies of the upper limb. METHOD: A PubMed Medline search was performed. Tests and questionnaires were evaluated according to three criteria: (1) items represent bimanual daily activities, (2a) quality of movement is scored (tests) or (2b) difficulty in performing a task (questionnaires), (3) instrument is attractive for children aged 4 - 12. RESULTS: We found 14 functional tests and nine questionnaires to measure arm/hand functioning. Three tests, the Assisting Hand Assessment (AHA), Unilateral Below Elbow Test (UBET) and University of New Brunswick Test of prosthetic function (UNB Test) and two questionnaires, the Prosthetic Upper limb Functional Index (PUFI) and the children's version of the ABILHAND (ABILHAND-Kids) met the criteria. CONCLUSIONS: Two functional tests (AHA and UBET) and two questionnaires (ABILHAND-Kids and PUFI) were considered appropriate to assess arm/hand functioning in children with congenital reduction deficiencies of the upper limb, but require further study on psychometric properties for these patient groups.

Determinants of the sit-to-stand movement: a review.

BACKGROUND AND PURPOSE: The sit-to-stand (STS) movement is a skill that helps determine the functional level of a person. Assessment of the STS movement has been done using quantitative and semiquantitative techniques. The purposes of this study were to identify the determinants of the STS movement and to describe their influence on the performance of the STS movement. METHODS: A search was made using MEDLINE (1980-2001) and the Science Citation Index Expanded of the Institute for Scientific Information (1988-2001) using the key words "chair," "mobility," "rising," "sit-to-stand," and "standing." Relevant references such as textbooks, presentations, and reports also were included. Of the 160 identified studies, only those in which the determinants of STS movement performance were examined using an experimental setup (n=39) were included in this review. RESULTS: The literature indicates that chair seat height, use of armrests, and foot position have a major influence on the ability to do an STS movement. Using a higher chair seat resulted in lower moments at knee level (up to 60%) and hip level (up to 50%); lowering the chair seat increased the need for momentum generation or repositioning of the feet to lower the needed moments. Using the armrests lowered the moments needed at the hip by 50%, probably without influencing the range of motion of the joints. Repositioning of feet influenced the strategy of the STS movement, enabling lower maximum mean extension moments at the hip (148.8 N m versus 32.7 N m when the foot position changed from anterior to posterior). DISCUSSION AND CONCLUSION: The ability to do an STS movement, according to the research reviewed, is strongly influenced by the height of the chair seat, use of armrests, and foot position. More study of the interaction among the different determinants is needed. Failing to account for these variables may lead to erroneous measurements of changes in STS performance.

Relationships among manual body functions, manual capacity, and bimanual performance using the prosthetic upper extremity functional index in children with congenital hand differences.

BACKGROUND: Most surgical techniques intervene at the level of body functions of the upper limb, aiming to improve manual capacity and activity performance. However, the nature of the relationships among these levels of functioning and evidence for hand function variables predicting performance have scarcely been investigated. OBJECTIVE: The primary aim of this study was to assess aspects of hand function and manual capacity that influence bimanual performance in children with congenital hand differences (CHDs), ranging from surgically corrected polydactyly or syndactyly to radial dysplasia. A secondary aim was to assess whether the number of items on the Prosthetic Upper Extremity Functional Index (PUFI) can be reduced without losing information on bimanual performance in this population. DESIGN: A cross-sectional design was used. METHODS: One hundred six 10- to 14-year-old children with CHD participated in the study, which was conducted in a university hospital's outpatient clinic. Bimanual performance was evaluated with child self-reports on an adapted version of the PUFI, calculating ease of performance and actual use of the affected hand. Additionally, hand function and manual capacity were assessed. RESULTS: The median score on ease of performance was high, and, on average, the children used their affected hand actively in 97% of all activities. Manual capacity of the nondominant hand and lateral pinch strength of the dominant hand predicted attainment of maximum PUFI scores. Nonmaximum PUFI scores were predicted by opposition strength of the nondominant hand and lateral pinch strength of the dominant hand. In addition, in this patient group, only 6 items of the PUFI explained all variance in PUFI scores. LIMITATIONS: The generalizability of the results is limited by the carefully selected age range. Second, the cross-sectional design of the study limits statements on causality on the relationships found. CONCLUSION: Children with a CHD generally have good bimanual performance and, on average, perform activities with active use of the affected hand. Therapy directed toward increasing manual capacity and finger muscle strength might assist in improving bimanual performance in children with CHD. Furthermore, the number of items on the PUFI could be reduced from 38 to 6 items in children with CHD.

Opinions of youngsters with congenital below-elbow deficiency, and those of their parents and professionals concerning prosthetic use and rehabilitation treatment.

BACKGROUND: Youngsters with unilateral congenital below-elbow deficiency (UCBED) seem to function well with or without a prosthesis. Reasons for rejecting prostheses have been reported earlier, but unfortunately not those of the children themselves. Furthermore, reasons for acceptance are underexplored in the literature. OBJECTIVES: To investigate opinions of children and early and late adolescents with UCBED, and those of their parents and healthcare professionals, concerning (1) reasons to wear or not to wear prostheses and (2) about rehabilitation care. METHODS: During one week of online focus group interviews, 42 children of 8-12 y/o, early and late adolescents of 13-16 and 17-20 y/o, 17 parents, and 19 healthcare professionals provided their opinions on various topics. This study addresses prosthetic use or non-use of prosthetics and rehabilitation care. Data were analyzed using the framework approach. RESULTS: Cosmesis was considered to be the prime factor for choosing and wearing a prosthesis, since this was deemed especially useful in avoiding stares from others. Although participants functioned well without prostheses, they agreed that it was an adjuvant in daily-life activities and sports. Weight and limited functionality constituted rejection reasons for a prosthesis. Children and adolescents who had accepted that they were different no longer needed the prosthesis to avoid being stared at. The majority of participants highly valued the peer-to-peer contact provided by the healthcare professionals. CONCLUSIONS: For children and adolescents with UCBED, prostheses appeared particularly important for social integration, but much less so for functionality. Peer-to-peer contact seemed to provide support during the process of achieving social integration and should be embedded in the healthcare process.

Activity and participation of children and adolescents with unilateral congenital below elbow deficiency: an online focus group study.

OBJECTIVE: To assess whether children/adolescents with unilateral congenital below elbow deficiency experience activity or participation limitations and how they deal with those limitations. METHODS: A qualitative study using online focus group interviews was held with 42 children/adolescents (in 3 age groups: 8-12, 13-16, and 17-20 years), 17 parents and 19 health professionals. Questions were posted concerning activities, participation, prosthetic use, psychosocial functioning, and rehabilitation care. This study concerns the first two topics; activities and participation. RESULTS: Children/adolescents experienced only a few limitations, and there were no activities or participation situations that were impossible. The limitations experienced could be attributed mainly to environmental factors, e.g. people who lack knowledge of the child's capacities. Those factors were particularly decisive in transition phases. Children/adolescents and parents described numerous strategies applied to deal with the deficiency. Professionals described fewer strategies and emphasized the use of adaptive devices and prostheses more than other participants did. CONCLUSION: Having unilateral congenital below elbow deficiency did not interfere with any activity, but not all children/adolescents had the ability to perform all activities. The strategies described by children/adolescents in managing their deficiency should be integrated into healthcare by providing realistic education about the various creative solutions and possibilities of adaptive devices and prostheses, and should be combined with specific training.

Mixed feelings of children and adolescents with unilateral congenital below elbow deficiency: an online focus group study.

The existing literature is inconsistent about the psychosocial functioning of children and adolescents with Unilateral Congenital Below Elbow Deficiency (UCBED). The objective of this qualitative study was to explore the psychosocial functioning of children and adolescents with UCBED in terms of their feelings about the deficiency and what helps them to cope with those feelings. Additionally, the perspectives of prosthesis wearers and non-wearers were compared, as were the perspectives of children, adolescents, parents and health professionals. Online focus group interviews were carried out with 42 children and adolescents (aged 8-12, 13-16 and 17-20), 16 parents and 19 health professionals. Questions were asked about psychosocial functioning, activities, participation, prosthetic use or non-use, and rehabilitation care. This study concerned remarks about psychosocial functioning. Children and adolescents with UCBED had mixed feelings about their deficiency. Both negative and positive feelings were often felt simultaneously and mainly depended on the way people in the children's environment reacted to the deficiency. People staring affected the children negatively, while support from others helped them to cope with the deficiency. Wearing a prosthesis and peer-to-peer contact were also helpful. Non-wearers tended to be more resilient than prosthesis wearers. Wearers wore their prosthesis for cosmetic reasons and to prevent them from negative reactions from the environment. We recommend that rehabilitation teams make parents aware of their great influence on the psychosocial functioning of their child with UCBED, to adjust or extend the currently available psychosocial help, and to encourage peer-to-peer contact.

The hypothesis of overwork weakness in Charcot-Marie-Tooth: a critical evaluation.

OBJECTIVE: It has been reported that the non-dominant hand of patients with Charcot-Marie-Tooth disease is stronger than the dominant hand as a result of overwork weakness. The objective of this study was to determine if this hypothesis could be verified in our population. DESIGN: Survey. SUBJECTS: Twenty-eight patients with Charcot-Marie-Tooth disease type I or II from a rehabilitation department of a university hospital in the Netherlands. METHODS: The strength of 3 intrinsic muscle groups of the dominant and non-dominant hand were determined using the Medical Research Council scale and the Rotterdam Intrinsic Hand Myometer. Furthermore, grip strength, pinch and key grip strength were measured. RESULTS: We found no differences in muscle strength for the dominant and non-dominant hand, except for a stronger key grip strength of the dominant hand in patients with Charcot-Marie-Tooth disease type II. CONCLUSION: In our population, the dominant hand of patients with Charcot-Marie-Tooth disease type I and II was equally strong as the non-dominant hand, suggesting that there is no presence of overwork weakness in the dominant hand in our group of patients. This implies that patients with Charcot-Marie-Tooth disease do not have to limit the use of their hands in daily life in order to prevent muscle strength loss.

Sensitivity of accelerometry to assess balance control during sit-to-stand movement.

Accelerometry has the potential to measure balance, defined as high-frequency body sway, ambulatorily in a simple and inexpensive way. The aim of this study was to determine and compare the sensitivity of accelerometric balance parameters during the sit-to-stand (STS) movement. Eleven healthy subjects (four males, 28.2 +/-7.9 years) and 31 patients with stroke (21 males; 63.3+/-12.8 years) were included. The healthy subjects performed STS movements in four conditions with different levels of difficulty. Data of the patients were compared 1) with healthy subjects, 2) between patient subgroups, and 3) between different phases of recovery to assess the sensitivity of accelerometry for differences in balance control. Accelerometers were attached to the trunk, and force plate measurements were simultaneously done in the healthy subjects. Main outcome measures were root mean square (rms) and area under the curve (AUC) derived from the high-frequency component of the transversal acceleration signal of the trunk. In all comparisons there was a significant difference in AUC data ( p < 0.05), and AUC appeared to be more sensitive than rms. Variability in AUC was not completely or mainly the result of changes and differences in the duration of the STS movement. As a conclusion, accelerometry is a potentially valuable technique to measure balance during the STS movement.

Validity of accelerometry in assessing the duration of the sit-to-stand movement.

Accelerometry is frequently used in movement analysis to assess body postures and motions. Here, we assessed the validity of ambulatory accelerometric measurement of the sit-to-stand (STS) movement duration. We compared accelerometric and opto-electronic assessment of the STS movement duration under four conditions (comfortable, slow, fast movement and exaggerated trunk flexion) with six healthy subjects and six subjects with stroke who performed movements six times under each condition. Accelerometric and opto-electronic data of STS movement duration were strongly related (r = 0.98). Accelerometry showed a fixed bias of 0.07 s (95% CI 0.008, 0.141) in healthy subjects and 0.32 s (95% CI 0.223, 0.422) in stroke subjects. In healthy subjects, a significant negative proportional bias of 0.1 was detected (95% CI -0.160, -0.032). Accelerometry showed discriminative validity in comparing stroke subjects to healthy subjects, and in comparing speed conditions. Our results indicate that accelerometry can provide valid data on the STS movement duration, furthermore during its use additional information on the STS movement, such as balance control, can be recorded.

Hand function and activity performance of children with longitudinal radial deficiency.

BACKGROUND: The effects of treatment of children with longitudinal radial deficiency are generally evaluated by measuring grip and pinch strength and joint mobility. Insight into limitations of activities of children with radial deficiency is scarce. In this study, we used standardized instruments to assess impairments in hand function and activity limitations and explored the relationship between the two. METHODS: We evaluated the hand function of twenty children with radial deficiency who were between four and twelve years of age. Impairments in hand function were assessed by measuring grip and pinch strength and the active range of motion of the wrist and of the metacarpophalangeal and proximal interphalangeal joints of the second digit. Functional activities were assessed with use of the Assisting Hand Assessment (AHA), to measure the effectiveness of the affected hand, and the Prosthetic Upper Extremity Functional Index (PUFI), to evaluate the ease of activity performance. The relationship between hand function and activity performance and the relationship of those measures with the type of radial deficiency were determined. RESULTS: The average grip and pinch strengths were 36% and 30% of reference values. We found reductions in the active range of motion, particularly of the metacarpophalangeal and proximal interphalangeal joints. The mean AHA score was 85.5 points and the mean PUFI score was 81.8 points, with both measured on a 0 to 100-point scale. Grip and pinch strength, the active range of joint motion, and the sum scores on the two functional tests were related to the type of radial deficiency. Significant relationships were found between impairments in hand function and activity performance. There was a large variation in the activity performance of the children with poor strength, whereas a more linear relationship was found between the active ranges of motion of the wrist and finger joints and activity performance. CONCLUSIONS: Despite marked impairments in hand function, children with radial deficiency performed functional activities fairly well. Relationships between impairments in hand function and limitation of activities were not linear. We recommend that evaluations of the results of treatment include assessment of both aspects of hand function.

Sensory evaluation of the hands in patients with Charcot-Marie-Tooth disease using Semmes-Weinstein monofilaments.

In this study, the intra- and interobserver reliability of the Semmes-Weinstein monofilaments (SWMFs) was determined in the hands of 15 patients with Charcot-Marie-Tooth disease. In addition, the amount and distribution of sensory loss in the hand, and the relation between sensory loss, intrinsic muscle strength, and hand dexterity was explored in 45 patients. SWMF testing had good intra- and interobserver reliability with intraclass correlation coefficients of 0.91 and 0.86, respectively. The SWMF testing revealed normal sensory function in 43% of all six locations. The average loss of the intrinsic hand muscle strength was 57%. Poor strength was found in patients with both poor and with good sensory function. The correlation between the measurements of intrinsic muscle strength and the Sollerman test for dexterity was 0.70.

Comparison of instruments to assess hand function in children with radius deficiencies.

PURPOSE: For treatment evaluation of children with radius deficiencies (RDs), standardized assessment of hand function in performing activities is required. Instruments to measure hand function have been developed for other diagnoses. The current study aimed to find additional evidence for validity, reliability, and usefulness of these instruments for children with RDs. METHODS: In this study, 20 children with RDs (aged 4-12 years) participated; 16 were boys, and 13 children were unilaterally affected. Children were assessed using the Assisting Hand Assessment, the Unilateral Below Elbow Test, the Prosthetic Upper Extremity Functional Index, and ABILHAND-Kids. Construct and convergent validity of the instruments were studied focusing on predefined hypotheses and relationships with other instruments and the therapist's global assessment. Test-retest reliability was assessed in 10 children by means of the intraclass correlation coefficients and the smallest detectable differences. RESULTS: For children with RDs, the Assisting Hand Assessment and the Prosthetic Upper Extremity Functional Index appeared to be the most valid function test and questionnaire according to the relationships found with type of RD, functional hand grips, and the therapist's global assessment of hand function. Regarding test-retest reliability, intraclass correlation coefficients ranged from 0.82 to 0.91, and smallest detectable differences were acceptably small. CONCLUSIONS: The current results contribute to the evidence that the instruments, especially the Assisting Hand Assessment and the Prosthetic Upper Extremity Functional Index, provide valid and reliable results in children with RDs. TYPE OF STUDY/LEVEL OF EVIDENCE: Diagnostic I.