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

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

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

With increasing life expectancy, upper extremity (UE) function becomes more and more important in boys with Duchenne muscular dystrophy (DMD). Knowledge of UE function in these children is, however, limited. The aim of this study was to gain insight into the changing patterns of UE function during the course of DMD. A Web-based questionnaire on UE function, covering all domains of the International Classification of Functioning Disability and Health, was distributed worldwide. Primary domains of the questionnaire were: participant characteristics, UE pain and stiffness, UE activities, and social participation. Data were described per disease stage and analyzed using descriptive analysis. A total of 213 boys/men with DMD (1-35 years) were included in this study. UE pain, stiffness, and activity limitations increased with disease stage. UE activity limitations already occurred in the early ambulatory stage. Compared to the healthy population, social participation was restricted in DMD patients and about 70 % of the respondents experienced UE limitations when performing social activities. Despite the existence of UE impairments, only 9 % of the respondents used supportive aids. Functional capacities and activities of the UE are limited already in the early ambulatory stage of patients with DMD affecting their social participation. Therefore, clinicians should pay attention to UE limitations before DMD patients lose their capacity to walk. Effective and adequate aids as well as attention for pain and stiffness in the therapeutic management could help to reduce UE activity limitations and related restrictions in social participation

Which assessment tools address the categories of the Brief ICF Core Set for Hand Conditions?

Introduction The purpose of this study was to explore whether assessment tools address aspects that are relevant according to the Brief ICF Core Set for Hand Conditions (BICF-CS). Methods Assessment tools meant to assess functioning and/or environmental factors in adults with hand conditions were reviewed. MEDLINE and CINAHL databases, previously published reviews, the book Clinical Assessment Recommendations of the ASHT, and websites of assessment tools were used for the content comparison and linking to the 23 categories of the BICF-CS. The updated version of the linking rules was applied by two reviewers. Results Forty-six assessment tools, known within the areas of hand therapy and hand surgery, were linked to the 23 categories of the BICF-CS. Regarding Body functions and body structures, the categories that were most frequently addressed were b730 “Muscle power functions,” b280 “Sensation of pain,” b710 “Mobility of joint functions,” and s730 “Structure of upper extremity.” Regarding Activities and Participation, d440 “Fine hand use” was addressed mostly and 25 assessment tools (with a total of 146 items) were linked to this category. Regarding Environmental Factors, only one assessment tool was identified that could be linked to two categories. Fifteen points of discussion were encountered in the linking process. Conclusions Content comparison of 46 assessment tools revealed that 19 of the 23 categories of the BICF-CS were addressed. The environmental factors were hardly addressed

Patients with Severe Poststroke Fatigue Show a Psychosocial Profile Comparable to Patients with Other Chronic Disease: Implications for Diagnosis and Treatment

Objective. To obtain a psychosocial profile of patients with poststroke fatigue (PSF), which could aid in optimizing treatment strategies. Methods. Eighty-eight outpatients with severe PSF measured with the Checklist Individual Strength-fatigue subscale (CIS-f) and the Fatigue Severity Scale (FSS) were selected. Depression and anxiety, psychological distress, coping, social support, and self-efficacy of this group were compared to reference groups of healthy controls and patients with other chronic diseases. Associations between psychosocial characteristics and fatigue were calculated. Results. Compared to healthy controls, patients with PSF reported more psychological distress, less problem-focused coping, and more positive social support. Minor or no differences were found in comparison with other chronic patients. The CIS-f correlated with somatic complaints and the FSS with cognitive complaints. Conclusion. Patients with PSF show a psychosocial profile comparable to patients with other chronic disease. Implications for diagnosis and treatment are discussed

Validity and reliability of the VOAA-DDD to assess spontaneous hand use with a video observation tool in children with spastic unilateral cerebral palsy

Contains fulltext : 80999.pdf (publisher's version ) (Open Access)BACKGROUND: In 2003 new computer software, the VOAA (Video Observations Aarts and Aarts), was designed to score and evaluate two important aspects of spontaneous upper limb use, i.e. overall duration and frequency of specific behaviours. The aim of this study was to investigate the test-retest, interrater and intrarater reliability and the construct validity of a new module, the VOAA-DDD, to determine developmental disregard in children with spastic unilateral cerebral palsy (CP). METHODS: A test-retest design with three raters for reliability and a two-group design for construct validity were used. Subjects were a total of 20 children with spastic unilateral CP equally divided in two age groups (2.5-5 and 5-8 years), and 56 healthy children of the same age groups. Overall duration and frequency of specific behaviours of the affected arm and hand were assessed during a task demanding ('stringing beads') and a task stimulating ('decorating a muffin') the use of both hands. Reliability was estimated by intraclass correlation coefficients (ICCs). Construct validity was assessed by comparing children with CP to healthy children. RESULTS: All ICCs exceeded 0.87. In contrast with healthy children, children with CP used their affected hand less during the 'muffin' task compared to the 'beads' task. Of the children with CP, 90% in the age group of 2.5-5 years and 50% in the age group of 5-8 years showed values exceeding the extreme values of healthy controls, respectively, indicating developmental disregard. CONCLUSION: The VOAA-DDD is a reliable and valid instrument to assess spontaneous use of the affected arm and hand in order to determine developmental disregard in children with spastic unilateral CP

Visuomotor processing is altered after peripheral nerve damage in neuralgic amyotrophy

Neuralgic amyotrophy is a common peripheral nerve disorder caused by autoimmune inflammation of the brachial plexus, clinically characterized by acute pain and weakness of the shoulder muscles, followed by motor impairment. Despite recovery of the peripheral nerves, patients often have residual motor dysfunction of the upper extremity, leading to persistent pain related to altered biomechanics of the shoulder region. Building on clinical signs that suggest a role for cerebral mechanisms in these residual complaints, here we show and characterize cerebral alterations following neuralgic amyotrophy. Neuralgic amyotrophy patients often develop alternative motor strategies, which suggests that (mal)adaptations may occur in somatomotor and/or visuomotor brain areas. Here, we tested where changes in cerebral sensorimotor representations occur in neuralgic amyotrophy, while controlling for altered motor execution due to peripheral neuropathy. We additionally explore the relation between potential cerebral alterations in neuralgic amyotrophy and clinical symptoms. During functional MRI scanning, 39 neuralgic amyotrophy patients with persistent, lateralized symptoms in the right upper extremity and 23 matched healthy participants solved a hand laterality judgement task that can activate sensorimotor representations of the upper extremity, across somatomotor and visuomotor brain areas. Behavioural and cerebral responses confirmed the involvement of embodied, sensorimotor processes across groups. Compared with healthy participants, neuralgic amyotrophy patients were slower in hand laterality judgement and had decreased cerebral activity specific to their affected limb in two higher-order visual brain regions: the right extrastriate cortex and the parieto-occipital sulcus. Exploratory analyses revealed that across patients, extrastriate activity specific to the affected limb decreased as persistent pain increased, and affected limb-related parieto-occipital activity decreased as imagery performance of the affected limb became slower. These findings suggest that maladaptive cerebral plasticity in visuomotor areas involved in sensorimotor integration plays a role in residual motor dysfunction and subsequent persistent pain in neuralgic amyotrophy. Rehabilitation interventions that apply visuomotor strategies to improve sensorimotor integration may help to treat neuralgic amyotrophy patients

Search for the standard model Higgs boson in the H to ZZ to 2l 2nu channel in pp collisions at sqrt(s) = 7 TeV

A search for the standard model Higgs boson in the H to ZZ to 2l 2nu decay channel, where l = e or mu, in pp collisions at a center-of-mass energy of 7 TeV is presented. The data were collected at the LHC, with the CMS detector, and correspond to an integrated luminosity of 4.6 inverse femtobarns. No significant excess is observed above the background expectation, and upper limits are set on the Higgs boson production cross section. The presence of the standard model Higgs boson with a mass in the 270-440 GeV range is excluded at 95% confidence level.Comment: Submitted to JHE

Search for New Physics with Jets and Missing Transverse Momentum in pp collisions at sqrt(s) = 7 TeV

A search for new physics is presented based on an event signature of at least three jets accompanied by large missing transverse momentum, using a data sample corresponding to an integrated luminosity of 36 inverse picobarns collected in proton--proton collisions at sqrt(s)=7 TeV with the CMS detector at the LHC. No excess of events is observed above the expected standard model backgrounds, which are all estimated from the data. Exclusion limits are presented for the constrained minimal supersymmetric extension of the standard model. Cross section limits are also presented using simplified models with new particles decaying to an undetected particle and one or two jets

Combined search for the quarks of a sequential fourth generation

Results are presented from a search for a fourth generation of quarks produced singly or in pairs in a data set corresponding to an integrated luminosity of 5 inverse femtobarns recorded by the CMS experiment at the LHC in 2011. A novel strategy has been developed for a combined search for quarks of the up and down type in decay channels with at least one isolated muon or electron. Limits on the mass of the fourth-generation quarks and the relevant Cabibbo-Kobayashi-Maskawa matrix elements are derived in the context of a simple extension of the standard model with a sequential fourth generation of fermions. The existence of mass-degenerate fourth-generation quarks with masses below 685 GeV is excluded at 95% confidence level for minimal off-diagonal mixing between the third- and the fourth-generation quarks. With a mass difference of 25 GeV between the quark masses, the obtained limit on the masses of the fourth-generation quarks shifts by about +/- 20 GeV. These results significantly reduce the allowed parameter space for a fourth generation of fermions.Comment: Replaced with published version. Added journal reference and DO

Search for anomalous t t-bar production in the highly-boosted all-hadronic final state

A search is presented for a massive particle, generically referred to as a Z', decaying into a t t-bar pair. The search focuses on Z' resonances that are sufficiently massive to produce highly Lorentz-boosted top quarks, which yield collimated decay products that are partially or fully merged into single jets. The analysis uses new methods to analyze jet substructure, providing suppression of the non-top multijet backgrounds. The analysis is based on a data sample of proton-proton collisions at a center-of-mass energy of 7 TeV, corresponding to an integrated luminosity of 5 inverse femtobarns. Upper limits in the range of 1 pb are set on the product of the production cross section and branching fraction for a topcolor Z' modeled for several widths, as well as for a Randall--Sundrum Kaluza--Klein gluon. In addition, the results constrain any enhancement in t t-bar production beyond expectations of the standard model for t t-bar invariant masses larger than 1 TeV.Comment: Submitted to the Journal of High Energy Physics; this version includes a minor typo correction that will be submitted as an erratu