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Introduction: Despite the prevalence of congenital limb deficiencies (CLDs) occurring in around 7.9/10,000 births (Am J Med Genet A. 2011;155A:1225-1235), there is still a gap in the knowledge regarding the etiology of a large proportion of cases, and literature addressing this topic is sparser compared with other conditions. Objective: The aim of this study is to assess the literature surrounding the causes of CLDs and use this to suggest the area in which a potential gap in the information on the causes of CLDs lies. Study Design: This study is a literature review on articles published from the year 2000 onward. Methods: A search was conducted on five databases (CINAHL, Scopus, PubMed, Embase, Cochrane Library) and the ProQuest platform resulting in 18 articles to be discussed, after inclusion and exclusion criteria were applied and critical appraisals were completed. Results and Discussion: The main findings could be separated into four themes including genetics, environment, drugs, and vascular. Comparisons were made among similar literature, although within some topics, this was less effective, given the specificity and reduced volume of research. Positive correlations were seen in all studies; however, the biggest underlying factor for most causes seemed to originate from a vascular disruption. Conclusions: Future studies should focus on an underlying vascular disruption as a base for further research to attempt to find reasoning for the proportion of CLDs with an unknown cause. Further attempts should be made into creating a consensus on classifying and recording CLDs to ensure a more uniform approach to this topic worldwide, allowing comparisons to be drawn more easily. Clinical Relevance: Understanding relevant terminology is vital to ensure that families experiencing CLD receive accurate information about their child's condition. This understanding will also lead to improvements in the quality of recorded data, such as clinical notes and registry inputs. [ABSTRACT FROM AUTHOR]

Introduction: Understanding repair patterns of upper-limb (UL) prosthetic devices have received little attention compared with their lower-limb counterparts. This study focuses on a retrospective analysis of anonymized UL prosthetic maintenance data to establish if there were any patterns of repairs at a regional prosthetic limb-fitting center in the United Kingdom. A secondary aim of this study is to describe the patient demographics of this center. Methods: Data containing prosthetic repair log and demographic description (n = 212) were acquired through our clinical partners and subjected to statistical analyses. Results: On average, each client visited the center 0.2 times/year for a new device and 0.9 times/year for maintenance-related activities. It is found that the repair rates are generally higher for body-powered devices (1.28 visits/device per year) compared with passive (0.94 visits/device per year) and externally powered devices (0.90 visits/device per year). In keeping with the typical UK UL-deficient population, there is a high male-to-female ratio, and higher instances of traumatic amputations were noticed for males at the center. There is a very high preponderance of congenital cases and an overall emphasis on prescribing passive devices to a majority of patients at the center. Conclusions: The data from our study are similar to previously published data from other centers and show a consistent pattern in terms of relative rates of maintenance attendances for different types of UL prostheses. Clinical Relevance: This study provides a longitudinal perspective and insights on ever-changing requirements (prostheses and related care) of the users at the level of a regional limb-fitting center. This study underscores current gaps in prosthetic device durability/reliability and opens up avenues for improvement of prosthetic services and devices. [ABSTRACT FROM AUTHOR]

Assessments of upper limb performance should require participants to perform tasks that challenge the limits of their ability. In order to select appropriate tasks, it is important to know which joints are used to perform the movement and how reliably those movements can be measured. The purpose of this work was to quantify the reliability of upper limb and trunk joint angles in healthy adults during common activities of daily living (ADLs). Nineteen participants performed six ADLs with the right arm (applying deodorant, turning a doorknob, answering a desk telephone, placing a pushpin in a bulletin board, wiping a plate with a towel, and pouring water from a pitcher) during two separate sessions. Within- and between-session reliability was quantified using intraclass correlation coefficients (ICCs) and minimum detectable change values (MDCs). Reliability was generally better within-session than between-session. The ICCs exceeded 0.75 for 88% of the joint angles and exceeded 0.90 for 32% of the angles. All MDCs were less than 25° and 61% were also less than 10°. The MDCs represented a larger percent of the average angles for the trunk (61%) and wrist (62%) compared to the shoulder (18%) and elbow (26%). Although these results show that most angles can be measured reliably for these six ADLs, reliability varied considerably between joints. It is therefore important to select tasks for assessing of upper limb performance based on which specific joints need to be evaluated. [ABSTRACT FROM AUTHOR]

Purpose: Upper limb assessments in children with hemiparesis rely on clinical measurements, which despite standardization are prone to error. Recently, 3D movement analysis using optoelectronic setups has been used to measure upper limb movement, but generalization is hindered by time and cost. Body worn inertial sensors may provide a simple, cost-effective alternative. Methods: We instrumented a subset of 30 participants in a mirror therapy clinical trial at baseline, post-treatment, and follow-up clinical assessments, with wireless inertial sensors positioned on the arms and trunk to monitor motion during reaching tasks. Results: Inertial sensor measurements distinguished paretic and non-paretic limbs with significant differences (P < 0.01) in movement duration, power, range of angular velocity, elevation, and smoothness (normalized jerk index and spectral arc length). Inertial sensor measurements correlated with functional clinical tests (Melbourne Assessment 2); movement duration and complexity (Higuchi fractal dimension) showed moderate to strong negative correlations with clinical measures of amplitude, accuracy, and fluency. Conclusion: Inertial sensor measurements reliably identify paresis and correlate with clinical measurements; they can therefore provide a complementary dimension of assessment in clinical practice and during clinical trials aimed at improving upper limb function. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background: There has been limited research on upper limb dysfunction in people with multiple sclerosis (PwMS). Objective: The objective of this paper is to study unilateral and bilateral upper limb dysfunction at different International Classification of Functioning (ICF) levels according to overall disability in PwMS. Methods: A total of 105 PwMS (16 with EDSS<4 (mild); 17 with EDSS 4–5.5 (moderate); 37 with EDSS 6–6.5 (severe); 35 with EDSS>6.5 (severe non-ambulant)) were recruited from two rehabilitation centers and assessed in a cross-sectional study. Results: The whole sample showed a diminished sensory function (median (first/third interquartile)) score of 3 (2/3) on the Monofilament Test and a reduced strength 91 (76/100) points on the Motricity Index (Body-Function level). Sensory dysfunction did not increase with higher EDSS while strength decreased from 100 (86/100) in the mild subgroups to 91 (80/100) points in the severe subgroup. All showed diminished dexterity, scoring 0.28 peg/s (0.17/0.35) on the Nine-Hole Peg Test (NHPT) (activity level). Score was better for the mild (0.38 (0.35/0.46)) peg/s compared to the severe subgroup (0.28 (0.17/0.35)). Sixty-eight percent, 44% and 75% of PwMS showed bilateral disorders in sensation, strength and dexterity, respectively. The Community Integration Questionnaire (participation level) showed a 35% reduction in home activities, even among PwMS with EDSS<4. Conclusion: This study showed uni-/bilateral upper limb abnormalities at all ICF levels increasing with the overall disability. [ABSTRACT FROM AUTHOR]

The Oberg, Manske and Tonkin (OMT) Classification of congenital anomalies of the hand and upper limb uses dysmorphological terminology, placing conditions in one of three groups: Malformations, Deformations and Dysplasias. The main group, Malformations, is further subdivided according to whether the whole of the limb is affected or the hand plate alone, and whether the primary insult involves one of the three axes of limb development and patterning or is non-axial. The common surgical diagnoses, such as thumb duplication and thumb hypoplasia, are then placed within this framework. Recently the International Federation of Societies for Surgery of the Hand Scientific Committee for Congenital Conditions approved the OMT Classification as a timely and appropriate replacement of the previously accepted Swanson Classification. This review charts the development of and modifications to the OMT Classification and its current status. [ABSTRACT FROM AUTHOR]

Background: Deep-seated lipomas are mature fat tissues which may occur in intermuscular, intramuscular, or submuscular locations. Unlike benign subcutaneous lipoma, which usually only grow on skin surface, deep-seated lipoma generally grow slowly and may sometimes infiltrate deeply and wrap around nerves. Methods: A retrospective review of all patients with deep-seated lipomas of the upper limb, operated on between 2002 and 2009 at our center, was carried out. Clinical and radiological characteristics, treatment, and evolution profile of these patients were also evaluated. Results: Five patients were identified. The mean age of patients was 71 years (range 60 to 77 years). The mean follow-up period was 2 years and there was no recurrence after the surgery. Conclusions: The complicated growing patterns of deep-seated lipoma have brought about difficulties during diagnostic and surgical procedures. A marked similarity of physical characteristics between a deep-seated lipoma and other soft-tissue masses can cause several confusions, while a careless dissection can cause severe damage to nearby nerves. Level of Evidence: Level V, therapeutic study [ABSTRACT FROM AUTHOR]

Aim: To investigate the effect of intermittent pneumatic compression (IPC) pumps on upper extremity impairments in breast cancer- related lymphedema. Materials and methods: Twenty-five patients with lymphedema were randomized into 2 groups. For 3 weeks, the pneumatic compression group (n = 12) underwent a treatment program including skin care, compression bandage, exercise therapy, manual lymph drainage (MLD), and IPC. The control group (n = 13) participated in the same program, but without IPC. The range of motion (ROM) of the upper extremities was measured with goniometry, and dysfunction of the shoulder was assessed with the Constant-Murley scale. Results: Significant improvements were observed in the ROM of the shoulder when we evaluated pre- and posttreatment values within both groups, and the improvements were still significant at 1-month follow-up. Likewise, we found significant differences in the visual analogue scale (VAS) and the Constant-Murley scores in both groups when we compared pre-treatment and posttreatment values, and significant differences were still present at 1-month follow-up. However, there were no significant differences between the groups in the upper limb's ROM, the VAS, or the Constant-Murley scale after the therapy or at the 1-month follow-up. Conclusion: Upper extremity impairments may improve with conservative treatment of lymphedema. However, the addition of IPC to the therapy may not provide any additional benefit for upper extremity impairments. [ABSTRACT FROM AUTHOR]

Ultrasound is a powerful screening examination for athletes with upper limb injuries. It is very effective in demonstrating injuries to muscle, tendon, ligament, and nerves. The technique is particularly useful in excluding joint effusion and in detecting soft tissue foreign bodies. Dynamic ultrasound examination is especially useful in detecting subluxation of tendon and nerves; it may be the only means of demonstrating the cause of snapping. Detailed anatomical knowledge is mandatory, and the examination should be structured and systematic. Ultrasound may overlook disorders of the deep structures in joints and bone disease. Those employing the technique must understand the strengths of ultrasound examination compared with and in combination with MRI. [ABSTRACT FROM AUTHOR]

Abstract: This study describes a novel pediatric upper limb motion index (PULMI) for children with cerebral palsy (CP). The PULMI is based on three-dimensional kinematics and provides quantitative information about upper limb motion during the Reach & Grasp Cycle. We also report key temporal–spatial parameters for children with spastic, dyskinetic, and ataxic CP. Participants included 30 typically-developing (TD) children (age=10.9±4.1 years) and 25 children with CP and upper limb involvement (age=12.3±3.7 years), Manual Ability Classification System (MACS) levels I–IV. The PULMI is calculated from the root-mean-square difference for eight kinematic variables between each child with CP and the average TD values, and scaled such that the TD PULMI is 100±10. The PULMI was significantly lower among children with CP compared to TD children (Wilcoxon Z=−5.06, p<.0001). PULMI scores were significantly lower among children with dyskinetic CP compared to spastic CP (Z=−2.47, p<.0135). There was a strong negative correlation between PULMI and MACS among children with CP (Spearman’s rho=−.78, p<.0001). Temporal–spatial values were significantly different between CP and TD children: movement time (Z=4.06, p<.0001), index of curvature during reach (Z=3.68, p=.0002), number of movement units (Z=3.72, p=.0002), angular velocity of elbow extension during reach (Z=−3.96, p<.0001), and transport1:reach peak velocities (Z=−2.48, p=.0129). A logistic regression of four temporal–spatial parameters, the Pediatric Upper Limb Temporal–Spatial Equation (PULTSE), correctly predicted 19/22 movement disorder subtypes (spastic versus dyskinetic CP). The PULMI, PULTSE, and key temporal–spatial parameters of the Reach & Grasp Cycle offer a quantitative approach to analyzing upper limb function in children with CP. [Copyright &y& Elsevier]

Abstract: The aim of this study was to measure which three-dimensional spatiotemporal and kinematic parameters differentiate upper limb movement characteristics in children with hemiplegic cerebral palsy (HCP) from those in typically developing children (TDC), during various clinically relevant tasks. We used a standardized protocol containing three reach tasks (forwards, upwards, and sideways), two reach-to-grasp tasks (with objects requiring different hand orientations), and three gross motor tasks. Spatiotemporal (movement duration, trajectory straightness, maximum velocity, and timing of maximum velocity), as well as kinematic parameters (discrete angles and waveforms of the trunk, scapula, shoulder, elbow and wrist), were compared between 20 children with HCP (age 10.9±2.9years) and 20 individually age-matched TDC (age 10.9±3.0years). Kinematic calculations followed the recommendations from the International Society of Biomechanics. Results showed that children with HCP had longer movement durations, less straight hand trajectories, and lower maximum velocities compared to the TDC. Timing of maximum velocity did not differ between both groups. The movement pathology in children with HCP was highlighted by increased trunk movements and reduced shoulder elevation during reaching and reach-to-grasp. We also measured an increased anterior tilting and protraction of the scapula in children with HCP, although differences were not significant for all tasks. Finally, compared to the TDC, children with HCP used less elbow extension and supination and more wrist flexion to execute all tasks. This study reported distinct 3D upper limb movement characteristics in children with HCP and age-matched TDC, establishing the discriminative ability of the measurement procedure. From a clinical perspective, combining spatiotemporal and kinematic parameters may facilitate the identification of the pathological movement patterns seen in children with HCP and thereby add to a well-targeted upper limb treatment planning. [Copyright &y& Elsevier]

Abstract: A common problem in children with hemiplegic cerebral palsy (CP) is the asymmetrical development of arm and hand capacity caused by the lack of use of the affected upper limb, or developmental disregard. In this paper, we provide a neuropsychological model that relates developmental disregard to attentional processes and motor learning. From this model, we hypothesize that high attentional demands associated with the use of the affected upper limb might hinder its use in daily life, and therefore may be a factor in developmental disregard. This can be assessed with a dual-task paradigm. However, until now, this has not been applied to children with CP. We provide recommendations for using a dual-task paradigm in children with CP based on empirical studies in typically developing children and children with developmental coordination disorder. Ultimately, these dual-task studies may be used to improve interventions aimed at reducing developmental disregard. [Copyright &y& Elsevier]

Abstract: The objective of this study was to characterize normal temporal–spatial patterns during the Reach & Grasp Cycle and to identify upper limb motor deficits in children with cerebral palsy (CP). The Reach & Grasp Cycle encompasses six sequential tasks: reach, grasp cylinder, transport to self (T 1), transport back to table (T 2), release cylinder, and return to initial position. Three-dimensional motion data were recorded from 25 typically developing children (11 males, 14 females; ages 5–18 years) and 12 children with hemiplegic CP (2 males, 10 females; ages 5–17 years). Within-day and between-day coefficients of variation for the control group ranged from 0 to 0.19, indicating good repeatability of all parameters. The mean duration of the Cycle for children with CP was nearly twice as long as controls, 9.5±4.3s versus 5.1±1.2s (U =37.0, P =.002), partly due to prolonged grasp and release durations. Peak hand velocity occurred at approximately 40% of each phase and was greater during the transport (T 1, T 2) than non-transport phases (reach, return) in controls (P <.001). Index of curvature was lower during transport versus non-transport phases for all children. Children with CP demonstrated an increased index of curvature during reach (U =46.0, P =.0074) and an increased total number of movement units (U =16.5, P <.0001) compared to controls, indicating less efficient and less smooth movements. Total duration of the Reach & Grasp Cycle (rho=.957, P <.0001), index of curvature during reach and T 1 (rho=.873, P =.0002 and rho=.778, P =.0028), and total number of movement units (rho=.907, P <.0001) correlated strongly with MACS score. The consistent normative data and the substantial differences between children with CP and controls reflect utility of the Reach & Grasp Cycle for quantitative evaluation of upper limb motor deficits. [Copyright &y& Elsevier]

The article presents a study which describes the intensity, prevalence, and functional impact of the different types of pain associated with the loss of upper limb which include the residual limb and phantom limb. The study was conducted through cross-sectional survey of 104 respondents who loss their upper limbs. Results of the study show that nearly all the respondents reported pain.

Abstract: Background: Recent studies have documented motions of the upper limbs of healthy subjects during activities of daily living. The aim of this study was to investigate compensatory motions of the upper extremity and torso during tasks for transradial prosthesis users and to determine if bracing simulates prosthesis use. Methods: Seven transradial myoelectric prosthesis users and 10 non-amputee volunteers performed four common tasks. Bracing was used to simulate the use of a transradial prosthesis by the non-amputee subjects. Range of motion of the glenohumeral (shoulder) joint, elbow joint and torso were calculated from optical motion analysis data. The motions between the non-braced, braced and transradial prosthesis user groups were statistically compared. Degree of asymmetry between the affected and unaffected arm was computed for the bilateral tasks. Findings: Myoelectric transradial prosthesis users compensate for lack of wrist and forearm movement differently depending on the task. Compensatory motion in torso bending occurs while opening a door. For the box lift task, prosthesis users rely more on the sound arm and torso bending. While drinking from a cup, decreasing flexion of the glenohumeral joint and increasing elbow flexion was shown while using a prosthesis. While turning a steering wheel, prosthesis users are similar to non-amputee subjects. Interpretation: By looking at the compensatory motions caused by limiting forearm and wrist movement, a greater understanding of the problems with transradial prosthetic design can be developed. Although bracing intact subjects showed similar mechanisms of compensation in most tasks, the magnitude of compensation was greater for prosthesis users. [Copyright &y& Elsevier]

Key Clinical Message Inflammatory bowel disease can present with extraintestinal features as the patient's only complaint. The erythema nodosum ( EN) initially affected the upper limbs only, reminding us that signs do not always present in a classical fashion. The presence of EN should prompt the clinician to look for any underlying cause. [ABSTRACT FROM AUTHOR]

Background: Proprioceptive sense plays a significant role in the generation and correction of skilled movements and, consequently, in most activities of daily living. We developed a new proprioception assessment protocol that enables the quantification of elbow position sense without using the opposite arm, involving active movement of the evaluated limb or relying on working memory. The aims of this descriptive study were to validate this assessment protocol by quantifying the elbow position sense of healthy adults, before using it in individuals who sustained a stroke, and to investigate its test-retest reliability.Methods: Elbow joint position sense was quantified using a robotic device and a virtual reality system. Two assessments were performed, by the same evaluator, with a one-week interval. While the participant's arms and hands were occluded from vision, the exoskeleton passively moved the dominant arm from an initial to a target position. Then, a virtual arm representation was projected on a screen placed over the participant's arm. This virtual representation and the real arm were not perfectly superimposed, however. Participants had to indicate verbally the relative position of their arm (more flexed or more extended; two-alternative forced choice paradigm) compared to the virtual representation. Each participant completed a total of 136 trials, distributed in three phases. The angular differences between the participant's arm and the virtual representation ranged from 1° to 27° and changed pseudo-randomly across trials. No feedback about results was provided to the participants during the task. A discrimination threshold was statistically extracted from a sigmoid curve fit representing the relationship between the angular difference and the percentage of successful trials. Test-retest reliability was evaluated with 3 different complementary approaches, i.e. a Bland-Altman analysis, an intraclass correlation coefficient (ICC) and a standard error of measurement (SEm).Results: Thirty participants (24.6 years old; 17 males, 25 right-handed) completed both assessments. The mean discrimination thresholds were 7.0 ± 2.4 (mean ± standard deviation) and 5.9 ± 2.1 degrees for the first and the second assessment session, respectively. This small difference between assessments was significant (- 1.1 ± 2.2 degrees), however. The assessment protocol was characterized by a fair to good test-retest reliability (ICC = 0.47).Conclusion: This study demonstrated the potential of this assessment protocol to objectively quantify elbow position sense in healthy individuals. Futures studies will validate this protocol in older adults and in individuals who sustained a stroke. [ABSTRACT FROM AUTHOR]

Objective The Modified Frenchay Scale (MFS) measures active upper limb function in hemiparesis based on a video review of 10 everyday living tasks, each rated on a 10-point visual analogic scale. Six tasks are bimanual and four are unimanual performed with the paretic hand. We measured intra- and inter-rater reliability of MFS in hemiparetic patients among trained raters. Material/patients and methods After a group training session consisting in reviewing and commenting upon ten patient videos, 10 raters assessed the videos of 10 adults with chronic hemiparesis (age 52 ± 13, mean ± SD) on two occasions one week apart. Intra- and inter-rater reliability was assessed using interclass correlation coefficients, mean intra- and inter-rater differences, coefficients of variation between raters and the agreement frequency procedure, agreement being defined as a difference equal or less than 0.5 between two individual ratings. Results For the overall MFS scores, mean intra- and inter-rater ICC coefficients were respectively 0.99 [0.95; 1.00] (mean [95%CI]) and 0.98 [0.98; 1.00]. The mean intra-rater score difference between two ratings one-week apart was 0.26 ± 0.08 and the mean inter-rater standard deviation was 0.61 ± 0.17. Inter-rating differences of less than 0.5 were found in 86% of cases of intra-rater comparisons and in 52% of inter-rater comparisons. Mean inter-rater coefficient of variation was 0.11 ± 0.03. Discussion - conclusion After a group training session in using the scale, the Modified Frenchay Scale has excellent intra- and inter-rater reliability. [ABSTRACT FROM AUTHOR]

Background Following a cerebrovascular accident (CVA or stroke), many patients experience learned non-use of the paretic arm (LNU UL ). One part of LNU is explained by the fact that the patient moves his trunk forward instead of extending his arm to reach a given object, even though he is capable of extending his arm. This is because using the trunk for this task demands less energy and concentration than using the paretic arm. This phenomenon is known to be detrimental to neuroplasticity and recovery. Objective The aim of this study is to quantify this part of learned non-use of the paretic upper limb during a hand-reaching task using 3D movement analysis. Methods Thirty-four post-supratentorial stroke participants were asked to reach a cone placed in front of them at 80% of their arm length. The reaching movement was repeated 5 times with the paretic hand, and then 5 times with the less-impaired hand. This sequence was first performed with the trunk free, then with the trunk restrained. Learned non-use of the upper limb (LNU UL ) was obtained from the difference of the amount of trunk compensation between the free trunk condition and the restrained trunk condition. Results LNU UL was significantly higher for the paretic hand, with individual values ranging from 1 to 43%, and one half of the patients with a LNU UL higher than 15%. Discussion/conclusion This quantification of LNU UL can be used to objectively pinpoint patients who have maladaptive trunk compensation and need specific trunk rehabilitation. It can be also used for monitoring the rehabilitation progress. This quantification of LNU UL may guide upper limb rehabilitation towards more optimal motor recovery avoiding maladaptive trunk compensation and its consequences on neuroplasticity. Considering LNU U could advance both theoretical and practical knowledge about the recovery of arm use after a CVA. [ABSTRACT FROM AUTHOR]