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Sample records for profound muscle weakness

  1. Skeletal muscle weakness in osteogenesis imperfecta mice.

    Science.gov (United States)

    Gentry, Bettina A; Ferreira, J Andries; McCambridge, Amanda J; Brown, Marybeth; Phillips, Charlotte L

    2010-09-01

    Exercise intolerance, muscle fatigue and weakness are often-reported, little-investigated concerns of patients with osteogenesis imperfecta (OI). OI is a heritable connective tissue disorder hallmarked by bone fragility resulting primarily from dominant mutations in the proα1(I) or proα2(I) collagen genes and the recently discovered recessive mutations in post-translational modifying proteins of type I collagen. In this study we examined the soleus (S), plantaris (P), gastrocnemius (G), tibialis anterior (TA) and quadriceps (Q) muscles of mice expressing mild (+/oim) and moderately severe (oim/oim) OI for evidence of inherent muscle pathology. In particular, muscle weight, fiber cross-sectional area (CSA), fiber type, fiber histomorphology, fibrillar collagen content, absolute, relative and specific peak tetanic force (P(o), P(o)/mg and P(o)/CSA respectively) of individual muscles were evaluated. Oim/oim mouse muscles were generally smaller, contained less fibrillar collagen, had decreased P(o) and an inability to sustain P(o) for the 300-ms testing duration for specific muscles; +/oim mice had a similar but milder skeletal muscle phenotype. +/oim mice had mild weakness of specific muscles but were less affected than their oim/oim counterparts which demonstrated readily apparent skeletal muscle pathology. Therefore muscle weakness in oim mice reflects inherent skeletal muscle pathology. Copyright © 2010 Elsevier B.V. All rights reserved.

  2. Sex hormones and skeletal muscle weakness

    DEFF Research Database (Denmark)

    Sipilä, Sarianna; Narici, Marco; Kjaer, Michael

    2013-01-01

    Human ageing is accompanied with deterioration in endocrine functions the most notable and well characterized of which being the decrease in the production of sex hormones. Current research literature suggests that low sex hormone concentration may be among the key mechanism for sarcopenia...... and muscle weakness. Within the European large scale MYOAGE project, the role of sex hormones, estrogens and testosterone, in causing the aging-related loss of muscle mass and function was further investigated. Hormone replacement therapy (HRT) in women is shown to diminish age-associated muscle loss, loss...... properties. HRT influences gene expression in e.g. cytoskeletal and cell-matrix proteins, has a stimulating effect upon IGF-I, and a role in IL-6 and adipokine regulation. Despite low circulating steroid-hormone level, postmenopausal women have a high local concentration of steroidogenic enzymes in skeletal...

  3. A weak balance: the contribution of muscle weakness to postural instability and falls.

    NARCIS (Netherlands)

    Horlings, G.C.; Engelen, B.G.M. van; Allum, J.H.J.; Bloem, B.R.

    2008-01-01

    Muscle strength is a potentially important factor contributing to postural control. In this article, we consider the influence of muscle weakness on postural instability and falling. We searched the literature for research evaluating muscle weakness as a risk factor for falls in community-dwelling

  4. Muscle weakness, afferent sensory dysfunction and exercise in knee osteoarthritis

    DEFF Research Database (Denmark)

    Roos, Ewa M.; Herzog, Walter; Block, Joel A

    2011-01-01

    Lower-extremity muscle strength and afferent sensory dysfunction, such as reduced proprioceptive acuity, are potentially modifiable putative risk factors for knee osteoarthritis (OA). Findings from current studies suggest that muscle weakness is a predictor of knee OA onset, while there is confli...... with previous knee injuries) are easily identified, and may benefit from exercise interventions to prevent or delay OA onset....... there is conflicting evidence regarding the role of muscle weakness in OA progression. In contrast, the literature suggests a role for afferent sensory dysfunction in OA progression but not necessarily in OA onset. The few pilot exercise studies performed in patients who are at risk of incident OA indicate...... a possibility for achieving preventive structure or load modifications. In contrast, large randomized controlled trials of patients with established OA have failed to demonstrate beneficial effects of strengthening exercises. Subgroups of individuals who are at increased risk of knee OA (such as those...

  5. Neck extensor muscle weakness (Dropped head syndrome) following radiotherapy

    International Nuclear Information System (INIS)

    Bhatia, S.; Miller, R.C.; Lachance, D.L.

    2006-01-01

    Background. Dropped head syndrome is an unusual condition in which the head cannot be held upright in its normal anatomic position secondary to pronounced, isolated, neck extensor muscle weakness. Case report. A case of dropped head syndrome in a female with a history of radiotherapy for Hodgkin's lymphoma and a clinical history consistent with multiple sclerosis is presented, and potential etiologies are discussed. Conclusions. Muscular atrophy and lower motor neuron injury secondary to isolated anterior horn cell injury from radiotherapy emerge as the most likely etiology. (author)

  6. Inherited and Acquired Muscle Weakness: A Moving Target for Diagnostic Muscle Biopsy.

    Science.gov (United States)

    Stenzel, Werner; Schoser, Benedikt

    2017-08-01

    Inherited and acquired muscular weakness is caused by multiple conditions. While the inherited ones are mostly caused by mutations in genes coding for myopathic or neurogenic diseases, the acquired ones occur due to inflammatory, endocrine, or toxic etiologies. Precise diagnosis of a specific disease may be challenging and may require a multidisciplinary approach. What is the current place for a diagnostic biopsy of skeletal muscle? Diagnostic muscle biopsy lost in this context its first-tier place in the primary diagnostic workup for some diseases, but it is still mandatory for others. We here summarize conditions in which we believe a diagnostic sample is most relevant and mention those in which a biopsy may be secondary or can even be left out. We would like to stress that muscle biopsy nowadays has a new important place in description and definition of new diseases, for example, discovered by modern genetic approaches. Georg Thieme Verlag KG Stuttgart, New York.

  7. Muscle Weakness Thresholds for Prediction of Diabetes in Adults.

    Science.gov (United States)

    Peterson, Mark D; Zhang, Peng; Choksi, Palak; Markides, Kyriakos S; Al Snih, Soham

    2016-05-01

    Despite the known links between weakness and early mortality, what remains to be fully understood is the extent to which strength preservation is associated with protection from cardiometabolic diseases, such as diabetes. The purposes of this study were to determine the association between muscle strength and diabetes among adults, and to identify age- and sex-specific thresholds of low strength for detection of risk. A population-representative sample of 4066 individuals, aged 20-85 years, was included from the combined 2011-2012 National Health and Nutrition Examination Survey (NHANES) data sets. Strength was assessed using a handheld dynamometer, and the single highest reading from either hand was normalized to body mass. A logistic regression model was used to assess the association between normalized grip strength and risk of diabetes, as determined by haemoglobin A1c levels ≥6.5 % (≥48 mmol/mol), while controlling for sociodemographic characteristics, anthropometric measures and television viewing time. For every 0.05 decrement in normalized strength, there were 1.26 times increased adjusted odds for diabetes in men and women. Women were at lower odds of having diabetes (odds ratio 0.49; 95 % confidence interval 0.29-0.82). Age, waist circumference and lower income were also associated with diabetes. The optimal sex- and age-specific weakness thresholds to detect diabetes were 0.56, 0.50 and 0.45 for men at ages of 20-39, 40-59 and 60-80 years, respectively, and 0.42, 0.38 and 0.33 for women at ages of 20-39, 40-59 and 60-80 years, respectively. We present thresholds of strength that can be incorporated into a clinical setting for identifying adults who are at risk of developing diabetes and might benefit from lifestyle interventions to reduce risk.

  8. Fatigue is associated with muscle weakness in Ehlers-Danlos syndrome: an explorative study.

    Science.gov (United States)

    Voermans, N C; Knoop, H; Bleijenberg, G; van Engelen, B G

    2011-06-01

    Ehlers-Danlos syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders characterised by joint hypermobility, skin hyperextensibility and tissue fragility. It has recently been shown that muscle weakness occurs frequently in EDS, and that fatigue is a common and clinically important symptom. The aim of this study was to investigate the relationship between fatigue severity and subjective and objective measures of muscle weakness. Furthermore, the predictive value of muscle weakness for fatigue severity was determined, together with that of pain and physical activity. An explorative, cross-sectional, observational study. Thirty EDS patients, recruited from the Dutch patient association, were investigated at the neuromuscular outpatient department of a tertiary referral centre in The Netherlands. Muscle strength measured with manual muscle strength testing and hand-held dynamometry. Self-reported muscle weakness, pain, physical activity levels and fatigue were assessed with standardised questionnaires. Fatigue severity in EDS was significantly correlated with measured and self-reported muscle weakness (r=-0.408 for manual muscle strength, r=0.461 for hand-held dynamometry and r=0.603 for self-reported muscle weakness). Both muscle weakness and pain severity were significant predictors of fatigue severity in a multiple regression analysis. The results suggest a positive and direct relationship between fatigue severity and muscle weakness in EDS. Future research should focus on the relationship between fatigue, muscle weakness and objectively measured physical activity, preferably in a larger cohort of EDS patients. Copyright © 2010 Chartered Society of Physiotherapy. Published by Elsevier Ltd. All rights reserved.

  9. Difference in distribution of muscle weakness between myasthenia gravis and the Lambert-Eaton myasthenic syndrome

    NARCIS (Netherlands)

    Wirtz, P. W.; Sotodeh, M.; Nijnuis, M.; van Doorn, P. A.; van Engelen, B. G. M.; Hintzen, R. Q.; de Kort, P. L. M.; Kuks, J. B.; Twijnstra, A.; de Visser, M.; Visser, L. H.; Wokke, J. H.; Wintzen, A. R.; Verschuuren, J. J.

    2002-01-01

    Background: Myasthenia gravis and the Lambert-Eaton myasthenic syndrome (LEMS) may have a similar distribution of muscle weakness. Deciding on a diagnosis of myasthenia gravis or LEMS on clinical grounds may therefore be difficult. Objective: To compare the localisation of initial muscle weakness

  10. Respiratory muscle weakness and respiratory muscle training in severely disabled multiple sclerosis patients.

    Science.gov (United States)

    Gosselink, R; Kovacs, L; Ketelaer, P; Carton, H; Decramer, M

    2000-06-01

    To evaluate the contribution of respiratory muscle weakness (part 1) and respiratory muscle training (part 2) to pulmonary function, cough efficacy, and functional status in patients with advanced multiple sclerosis (MS). Survey (part 1) and randomized controlled trial (part 2). Rehabilitation center for MS. Twenty-eight bedridden or wheelchair-bound MS patients (part 1); 18 patients were randomly assigned to a training group (n = 9) or a control group (n = 9) (part 2). The training group (part 2) performed three series of 15 contractions against an expiratory resistance (60% maximum expiratory pressure [PEmax]) two times a day, whereas the control group performed breathing exercises to enhance maximal inspirations. Forced vital capacity (FVC), inspiratory, and expiratory muscle strength (PImax and PEmax), neck flexion force (NFF), cough efficacy by means of the Pulmonary Index (PI), and functional status by means of the Extended Disability Status Scale (EDSS). Part 1 revealed a significantly reduced FVC (43% +/- 26% predicted), PEmax (18% +/- 8% predicted), and PImax (27% +/- 11% predicted), whereas NFF was only mildly reduced (93% +/- 26% predicted). The PI (median score, 10) and EDSS (median score, 8.5) were severely reduced. PEmax was significantly correlated to FVC, EDSS, and PI (r = .77, -.79, and -.47, respectively). In stepwise multiple regression analysis. PEmax was the only factor contributing to the explained variance in FVC (R2 = .60), whereas body weight (R2 = .41) was the only factor for the PI. In part 2, changes in PImax and PEmax tended to be higher in the training group (p = .06 and p = .07, respectively). The PI was significantly improved after 3 months of training compared with the control group (p functional status. Expiratory muscle training tended to enhance inspiratory and expiratory muscle strength. In addition, subjectively and objectively rated cough efficacy improved significantly and lasted for 3 months after training cessation.

  11. Progressive Muscle Atrophy and Weakness After Treatment by Mantle Field Radiotherapy in Hodgkin Lymphoma Survivors

    International Nuclear Information System (INIS)

    Leeuwen-Segarceanu, Elena M. van; Dorresteijn, Lucille D.A.; Pillen, Sigrid; Biesma, Douwe H.; Vogels, Oscar J.M.; Alfen, Nens van

    2012-01-01

    Purpose: To describe the damage to the muscles and propose a pathophysiologic mechanism for muscle atrophy and weakness after mantle field radiotherapy in Hodgkin lymphoma (HL) survivors. Methods and Materials: We examined 12 patients treated by mantle field radiotherapy between 1969 and 1998. Besides evaluation of their symptoms, the following tests were performed: dynamometry; ultrasound of the sternocleidomastoid, biceps, and antebrachial flexor muscles; and needle electromyography of the neck, deltoid, and ultrasonographically affected arm muscles. Results: Ten patients (83%) experienced neck complaints, mostly pain and muscle weakness. On clinical examination, neck flexors were more often affected than neck extensors. On ultrasound, the sternocleidomastoid was severely atrophic in 8 patients, but abnormal echo intensity was seen in only 3 patients. Electromyography of the neck muscles showed mostly myogenic changes, whereas the deltoid, biceps, and antebrachial flexor muscles seemed to have mostly neurogenic damage. Conclusions: Many patients previously treated by mantle field radiotherapy develop severe atrophy and weakness of the neck muscles. Neck muscles within the radiation field show mostly myogenic damage, and muscles outside the mantle field show mostly neurogenic damage. The discrepancy between echo intensity and atrophy suggests that muscle damage is most likely caused by an extrinsic factor such as progressive microvascular fibrosis. This is also presumed to cause damage to nerves within the radiated field, resulting in neurogenic damage of the deltoid and arm muscles.

  12. Simulating the effect of muscle weakness and contracture on neuromuscular control of normal gait in children.

    Science.gov (United States)

    Fox, Aaron S; Carty, Christopher P; Modenese, Luca; Barber, Lee A; Lichtwark, Glen A

    2018-03-01

    Altered neural control of movement and musculoskeletal deficiencies are common in children with spastic cerebral palsy (SCP), with muscle weakness and contracture commonly experienced. Both neural and musculoskeletal deficiencies are likely to contribute to abnormal gait, such as equinus gait (toe-walking), in children with SCP. However, it is not known whether the musculoskeletal deficiencies prevent normal gait or if neural control could be altered to achieve normal gait. This study examined the effect of simulated muscle weakness and contracture of the major plantarflexor/dorsiflexor muscles on the neuromuscular requirements for achieving normal walking gait in children. Initial muscle-driven simulations of walking with normal musculoskeletal properties by typically developing children were undertaken. Additional simulations with altered musculoskeletal properties were then undertaken; with muscle weakness and contracture simulated by reducing the maximum isometric force and tendon slack length, respectively, of selected muscles. Muscle activations and forces required across all simulations were then compared via waveform analysis. Maintenance of normal gait appeared robust to muscle weakness in isolation, with increased activation of weakened muscles the major compensatory strategy. With muscle contracture, reduced activation of the plantarflexors was required across the mid-portion of stance suggesting a greater contribution from passive forces. Increased activation and force during swing was also required from the tibialis anterior to counteract the increased passive forces from the simulated dorsiflexor muscle contracture. Improvements in plantarflexor and dorsiflexor motor function and muscle strength, concomitant with reductions in plantarflexor muscle stiffness may target the deficits associated with SCP that limit normal gait. Copyright © 2018 Elsevier B.V. All rights reserved.

  13. Cytokine genes as potential biomarkers for muscle weakness in OPMD

    DEFF Research Database (Denmark)

    Riaz, Muhammad; Raz, Yotam; van der Slujis, Barbara

    2016-01-01

    is a dominant, late-onset myopathy, caused by an alanine-expansion mutation in the gene encoding for poly(A) binding protein nuclear 1 (expPABPN1). Here, we investigated the hypothesis that cytokines could mark OPMD disease state. We determined cytokines levels the vastus lateralis muscle from genetically...... confirmed expPABPN1 carriers at a symptomatic or a presymptomatic stage. We identified cytokine-related genes candidates from a transcriptome study in a mouse overexpressing exp PABPN1 Six cytokines were found to be consistently down-regulated in OPMD vastus lateralis muscles. Expression levels...

  14. Elbow flexor and extensor muscle weakness in lateral epicondylalgia.

    Science.gov (United States)

    Coombes, Brooke K; Bisset, Leanne; Vicenzino, Bill

    2012-05-01

    To evaluate whether deficits of elbow flexor and extensor muscle strength exist in lateral epicondylalgia (LE) in comparison with a healthy control population. Cross-sectional study. 150 participants with unilateral LE were compared with 54 healthy control participants. Maximal isometric elbow flexion and extension strength were measured bilaterally using a purpose-built standing frame such that gripping was avoided. The authors found significant side differences in elbow extensor (-6.54 N, 95% CI -11.43 to -1.65, p=0.008, standardised mean difference (SMD) -0.45) and flexor muscle strength (-11.26 N, 95% CI -19.59 to -2.94, p=0.009, SMD -0.46) between LE and control groups. Within the LE group, only elbow extensor muscle strength deficits between sides was significant (affected-unaffected: -2.94 N, 95% CI -5.44 to -0.44). Small significant deficits of elbow extensor and flexor muscle strength exist in the affected arm of unilateral LE in comparison with healthy controls. Notably, comparing elbow strength between the affected and unaffected sides in unilateral epicondylalgia is likely to underestimate these deficits. Trial Registration Australian New Zealand Clinical Trials Register ACTRN12609000051246.

  15. Proximal muscle weakness as a result of osteomalacia associated with celiac disease: a case report.

    Science.gov (United States)

    Oz, B; Akan, O; Kocyigit, H; Gürgan, H A

    2016-02-01

    A 24-year-old woman suffering from back and hip pain with difficulty in walking was reported. She had proximal muscle weakness. Laboratory findings led to the diagnosis of osteomalacia. Positivity of antibodies strengthened suspicion of celiac disease. In patients with proximal muscle weakness, osteomalacia should be considered in differential diagnosis even in a young woman. A 24-year-old woman suffering from back pain, bilateral hip pain, and difficulty in walking was reported. Her symptoms had started in the first trimester of pregnancy. In her physical examination, proximal muscle weakness and waddling gait pattern were determined. Her lumbar spine and hip MRI revealed no obvious pathological findings. Electromyography showed a myophatic pattern. Physical examination, normal values of creatine kinase, and muscle biopsy were supplied to exclude the diagnosis of primer muscle diseases. Laboratory findings led to the diagnosis of osteomalacia with normal renal function. Gastrointestinal symptoms and positivity of anti-gliadin and anti-endomysium antibodies strengthened the suspicion of celiac disease as a cause of the osteomalacia. The diagnosis of celiac disease was confirmed with duodenal mucosal biopsy. In patients with proximal muscle weakness and waddling gait pattern, osteomalacia should be considered in differential diagnosis even in a young woman and underlying disease should be investigated.

  16. Prevalence of joint contractures and muscle weakness in people with multiple sclerosis.

    Science.gov (United States)

    Hoang, Phu Dinh; Gandevia, Simon C; Herbert, Robert D

    2014-01-01

    To investigate the prevalence of joint contracture (limited passive range of joint motion) and muscle weakness in a population with multiple sclerosis (MS). A secondary aim was to establish normative data of functional tests of mobility and balance of people with MS who are still ambulant. Cross-sectional study. People with MS living in metropolitan Sydney, Australia. 330 people with MS living in metropolitan Sydney, Australia were randomly sampled on 23 July 2009 from the MS Australia register and invited to participate. Passive range of motion of large joints of the limbs and muscle strength. Tests of walking and balance were also conducted. 156 people (109 females, 47 males; mean age 54.2 years; mean time since diagnosis 14.9 years) agreed to participate and were assessed. Fifty-six per cent (56%) of participants had contracture in at least one major joint of upper or lower limb. The most common site of contracture was the ankle (43.9%). Seventy per cent (70%) of participants had muscle weakness in one or more muscle groups. As muscle weakness, joint contractures were present at early stage of MS and the prevalence was associated with the progression of the disease. These data show that in addition to muscle weakness joint contractures are highly prevalent among people with MS, especially in the ankle joint. This implicates that prevention of contracture is crucial in providing rehabilitation to people with MS.

  17. Effects of respiratory muscle training (RMT) in children with infantile-onset Pompe disease and respiratory muscle weakness.

    Science.gov (United States)

    Jones, Harrison N; Crisp, Kelly D; Moss, Tronda; Strollo, Katherine; Robey, Randy; Sank, Jeffrey; Canfield, Michelle; Case, Laura E; Mahler, Leslie; Kravitz, Richard M; Kishnani, Priya S

    2014-01-01

    Respiratory muscle weakness is a primary therapeutic challenge for patients with infantile Pompe disease. We previously described the clinical implementation of a respiratory muscle training (RMT) regimen in two adults with late-onset Pompe disease; both demonstrated marked increases in inspiratory and expiratory muscle strength in response to RMT. However, the use of RMT in pediatric survivors of infantile Pompe disease has not been previously reported. We report the effects of an intensive RMT program on maximum inspiratory pressure (MIP) and maximum expiratory pressure (MEP) using A-B-A (baseline-treatment-posttest) single subject experimental design in two pediatric survivors of infantile Pompe disease. Both subjects had persistent respiratory muscle weakness despite long-term treatment with alglucosidase alfa. Subject 1 demonstrated negligible to modest increases in MIP/MEP (6% increase in MIP, d=0.25; 19% increase in MEP, d=0.87), while Subject 2 demonstrated very large increases in MIP/MEP (45% increase in MIP, d=2.38; 81% increase in MEP, d=4.31). Following three-month RMT withdrawal, both subjects maintained these strength increases and demonstrated maximal MIP and MEP values at follow-up. Intensive RMT may be a beneficial treatment for respiratory muscle weakness in pediatric survivors of infantile Pompe disease.

  18. Weakness

    Science.gov (United States)

    ... by a slipped disk in the spine) Stroke MUSCLE DISEASES Becker muscular dystrophy Dermatomyositis Muscular dystrophy (Duchenne) Myotonic dystrophy POISONING Botulism Poisoning ( insecticides , nerve gas) ...

  19. Knee extensor muscle weakness is a risk factor for development of knee osteoarthritis

    DEFF Research Database (Denmark)

    Oiestad, B E; Juhl, C B; Eitzen, I

    2015-01-01

    The objective of this study was to perform a systematic review and meta-analysis on the association between knee extensor muscle weakness and the risk of developing knee osteoarthritis. A systematic review and meta-analysis was conducted with literature searches in Medline, SPORTDiscus, EMBASE......, CINAHL, and AMED. Eligible studies had to include participants with no radiographic or symptomatic knee osteoarthritis at baseline; have a follow-up time of a minimum of 2 years, and include a measure of knee extensor muscle strength. Hierarchies for extracting data on knee osteoarthritis and knee...... extensor muscle strength were defined prior to data extraction. Meta-analysis was applied on the basis of the odds ratios (ORs) of developing symptomatic knee osteoarthritis or radiographic knee osteoarthritis in subjects with knee extensor muscle weakness. ORs for knee osteoarthritis and 95% confidence...

  20. Non-neural Muscle Weakness Has Limited Influence on Complexity of Motor Control during Gait

    Directory of Open Access Journals (Sweden)

    Marije Goudriaan

    2018-01-01

    Full Text Available Cerebral palsy (CP and Duchenne muscular dystrophy (DMD are neuromuscular disorders characterized by muscle weakness. Weakness in CP has neural and non-neural components, whereas in DMD, weakness can be considered as a predominantly non-neural problem. Despite the different underlying causes, weakness is a constraint for the central nervous system when controlling gait. CP demonstrates decreased complexity of motor control during gait from muscle synergy analysis, which is reflected by a higher total variance accounted for by one synergy (tVAF1. However, it remains unclear if weakness directly contributes to higher tVAF1 in CP, or whether altered tVAF1 reflects mainly neural impairments. If muscle weakness directly contributes to higher tVAF1, then tVAF1 should also be increased in DMD. To examine the etiology of increased tVAF1, muscle activity data of gluteus medius, rectus femoris, medial hamstrings, medial gastrocnemius, and tibialis anterior were measured at self-selected walking speed, and strength data from knee extensors, knee flexors, dorsiflexors and plantar flexors, were analyzed in 15 children with CP [median (IQR age: 8.9 (2.2], 15 boys with DMD [8.7 (3.1], and 15 typical developing (TD children [8.6 (2.7]. We computed tVAF1 from 10 concatenated steps with non-negative matrix factorization, and compared tVAF1 between the three groups with a Mann-Whiney U-test. Spearman's rank correlation coefficients were used to determine if weakness in specific muscle groups contributed to altered tVAF1. No significant differences in tVAF1 were found between DMD [tVAF1: 0.60 (0.07] and TD children [0.65 (0.07], while tVAF1 was significantly higher in CP [(0.74 (0.09] than in the other groups (both p < 0.005. In CP, weakness in the plantar flexors was related to higher tVAF1 (r = −0.72. In DMD, knee extensor weakness related to increased tVAF1 (r = −0.50. These results suggest that the non-neural weakness in DMD had limited influence on

  1. Agrin mutations lead to a congenital myasthenic syndrome with distal muscle weakness and atrophy.

    Science.gov (United States)

    Nicole, Sophie; Chaouch, Amina; Torbergsen, Torberg; Bauché, Stéphanie; de Bruyckere, Elodie; Fontenille, Marie-Joséphine; Horn, Morten A; van Ghelue, Marijke; Løseth, Sissel; Issop, Yasmin; Cox, Daniel; Müller, Juliane S; Evangelista, Teresinha; Stålberg, Erik; Ioos, Christine; Barois, Annie; Brochier, Guy; Sternberg, Damien; Fournier, Emmanuel; Hantaï, Daniel; Abicht, Angela; Dusl, Marina; Laval, Steven H; Griffin, Helen; Eymard, Bruno; Lochmüller, Hanns

    2014-09-01

    Congenital myasthenic syndromes are a clinically and genetically heterogeneous group of rare diseases resulting from impaired neuromuscular transmission. Their clinical hallmark is fatigable muscle weakness associated with a decremental muscle response to repetitive nerve stimulation and frequently related to postsynaptic defects. Distal myopathies form another clinically and genetically heterogeneous group of primary muscle disorders where weakness and atrophy are restricted to distal muscles, at least initially. In both congenital myasthenic syndromes and distal myopathies, a significant number of patients remain genetically undiagnosed. Here, we report five patients from three unrelated families with a strikingly homogenous clinical entity combining congenital myasthenia with distal muscle weakness and atrophy reminiscent of a distal myopathy. MRI and neurophysiological studies were compatible with mild myopathy restricted to distal limb muscles, but decrement (up to 72%) in response to 3 Hz repetitive nerve stimulation pointed towards a neuromuscular transmission defect. Post-exercise increment (up to 285%) was observed in the distal limb muscles in all cases suggesting presynaptic congenital myasthenic syndrome. Immunofluorescence and ultrastructural analyses of muscle end-plate regions showed synaptic remodelling with denervation-reinnervation events. We performed whole-exome sequencing in two kinships and Sanger sequencing in one isolated case and identified five new recessive mutations in the gene encoding agrin. This synaptic proteoglycan with critical function at the neuromuscular junction was previously found mutated in more typical forms of congenital myasthenic syndrome. In our patients, we found two missense mutations residing in the N-terminal agrin domain, which reduced acetylcholine receptors clustering activity of agrin in vitro. Our findings expand the spectrum of congenital myasthenic syndromes due to agrin mutations and show an unexpected

  2. Pharmacological inhibition of myostatin protects against skeletal muscle atrophy and weakness after anterior cruciate ligament tear.

    Science.gov (United States)

    Wurtzel, Caroline Nw; Gumucio, Jonathan P; Grekin, Jeremy A; Khouri, Roger K; Russell, Alan J; Bedi, Asheesh; Mendias, Christopher L

    2017-11-01

    Anterior cruciate ligament (ACL) tears are among the most frequent knee injuries in sports medicine, with tear rates in the US up to 250,000 per year. Many patients who suffer from ACL tears have persistent atrophy and weakness even after considerable rehabilitation. Myostatin is a cytokine that directly induces muscle atrophy, and previous studies rodent models and patients have demonstrated an upregulation of myostatin after ACL tear. Using a preclinical rat model, our objective was to determine if the use of a bioneutralizing antibody against myostatin could prevent muscle atrophy and weakness after ACL tear. Rats underwent a surgically induced ACL tear and were treated with either a bioneutralizing antibody against myostatin (10B3, GlaxoSmithKline) or a sham antibody (E1-82.15, GlaxoSmithKline). Muscles were harvested at either 7 or 21 days after induction of a tear to measure changes in contractile function, fiber size, and genes involved in muscle atrophy and hypertrophy. These time points were selected to evaluate early and later changes in muscle structure and function. Compared to the sham antibody group, 7 days after ACL tear, myostatin inhibition reduced the expression of proteolytic genes and induced the expression of hypertrophy genes. These early changes in gene expression lead to a 22% increase in muscle fiber cross-sectional area and a 10% improvement in maximum isometric force production that were observed 21 days after ACL tear. Overall, myostatin inhibition lead to several favorable, although modest, changes in molecular biomarkers of muscle regeneration and reduced muscle atrophy and weakness following ACL tear. © 2017 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 35:2499-2505, 2017. © 2017 Orthopaedic Research Society. Published by Wiley Periodicals, Inc.

  3. Ankle-foot orthoses that restrict dorsiflexion improve walking in polio survivors with calf muscle weakness

    NARCIS (Netherlands)

    Ploeger, Hilde E.; Bus, Sicco A.; Brehm, Merel-Anne; Nollet, Frans

    2014-01-01

    In polio survivors with calf muscle weakness, dorsiflexion-restricting ankle-foot orthoses (DR-AFOs) aim to improve gait in order to reduce walking-related problems such as instability or increased energy cost. However, evidence on the efficacy of DR-AFOs in polio survivors is lacking. We

  4. Fatigue is associated with muscle weakness in Ehlers-Danlos syndrome: an explorative study

    NARCIS (Netherlands)

    Voermans, N.C.; Knoop, H.; Bleijenberg, G.; Engelen, B.G.M. van

    2011-01-01

    OBJECTIVES: Ehlers-Danlos syndrome (EDS) is a clinically and genetically heterogeneous group of inherited connective tissue disorders characterised by joint hypermobility, skin hyperextensibility and tissue fragility. It has recently been shown that muscle weakness occurs frequently in EDS, and that

  5. Prevalence of clinically relevant muscle weakness and its association with vitamin D status among older adults in Ecuador.

    Science.gov (United States)

    Orces, Carlos H

    2017-10-01

    Muscle weakness and 25-hydroxyvitamin D (25(OH)D) deficiency have been associated with adverse outcomes among older adults. However, little is known about the relationship between clinically relevant muscle weakness and 25(OH)D levels in Ecuador. To examine the prevalence of muscle weakness and its association with 25(OH)D status among subjects aged 60 years and older in Ecuador. The present study was based on data from 2205 participants in the first National Survey of Health, Wellbeing, and Aging. The Foundation for the National Institute of Health Sarcopenia Project criteria was used to examine muscle weakness prevalence rates. Gender-specific general linear and logistic regression models adjusted for potential confounders were created to compare mean 25(OH)D concentrations and 25(OH)D deficiency across muscle strength categories, respectively. An estimated 32.2% of women and 33.4% of men had evidence of clinically relevant muscle weakness in Ecuador. In general, increased muscle weakness prevalence rates were present among Indigenous, residents in the rural Andes Mountains, underweight subjects, and those with a sedentary lifestyle. Muscle strength was significantly and directly correlated with mean 25(OH)D levels. After controlling for potential confounders, 25(OH)D deficiency prevalence rates were 31 and 43% higher among men and women with muscle weakness than those with normal strength, respectively. One-third of older adults nationwide had evidence of muscle weakness. While the present study found a significant correlation between muscle strength and 25(OH)D concentrations, further research is needed to examine whether optimizing 25(OH)D levels may improve muscle weakness among older adults.

  6. Environmentally profound

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Rushu [China Yangzte Three Gorges Project Development Corp., Yichang Hubei (China)

    1999-09-01

    The Three Gorges dam project on the Yangtze river will have a profound effect on the people, the environment and cultural heritage sites. The dam will be the world's biggest and will provide almost 85 BkWh of hydro power per annum. A noticeable benefit will be a greatly reduced incidence of flooding in the Jingjiang reaches of the river. Additional benefits will be improved local navigation, climate and enhanced water quality. The main unwelcome impacts were loss of farmland and resettlement of people but here the government have been particularly careful to provide the relocated people with a reasonable standard of new accommodation and farmland. The loss of natural vegetation will be small but there are endangered species of birds and animals living in the region. A number of negative environmental affects (dust noise, incidence of certain diseases) and how they are to be mitigated are mentioned. (UK)

  7. Ca2+ sensitizers: An emerging class of agents for counterbalancing weakness in skeletal muscle diseases?

    Science.gov (United States)

    Ochala, Julien

    2010-02-01

    Ca(2+) ions are key regulators of skeletal muscle contraction. By binding to contractile proteins, they initiate a cascade of molecular events leading to cross-bridge formation and ultimately, muscle shortening and force production. The ability of contractile proteins to respond to Ca(2+) attachment, also known as Ca(2+) sensitivity, is often compromised in acquired and congenital skeletal muscle disorders. It constitutes, undoubtedly, a major physiological cause of weakness for patients. In this review, we discuss recent studies giving strong molecular and cellular evidence that pharmacological modulators of some of the contractile proteins, also termed Ca(2+) sensitizers, are efficient agents to improve Ca(2+) sensitivity and function in diseased skeletal muscle cells. In fact, they compensate for the impaired contractile proteins response to Ca(2+) binding. Currently, such Ca(2+) sensitizing compounds are successfully used for reducing problems in cardiac disorders. Therefore, in the future, under certain conditions, these agents may represent an emerging class of agents to enhance the quality of life of patients suffering from skeletal muscle weakness. Copyright 2009 Elsevier B.V. All rights reserved.

  8. A 13 year old girl with muscle weakness and ventricular tachycardia

    International Nuclear Information System (INIS)

    Rauf, M.; Zeb, S.; Adil, M.; Gul, A.M.; Hafizullah, M.

    2012-01-01

    Gitelman's syndrome is characterized by hypokalemia, hypomagnesemia and hypocalciuria. It is an autosomal recessive renal disorder and mostly present with asymptomatic hypokalemia but muscle cramps, dizziness, fatigue, muscle weakness and arrhythmias are the usual presentation. Same is the case with us, young girl presented with multiple symptoms and arrhythmia was worked up for electrolyte imbalance. Long term prognosis in terms of maintaining growth, renal function and life expectancy is excellent. Family screening is important for its early detection and treatment. This needs future genetic studies. (author)

  9. The spectrum of muscle histopathologic findings in 42 weak scleroderma patients

    Science.gov (United States)

    Paik, Julie J.; Wigley, Fredrick M.; Lloyd, Thomas E.; Corse, Andrea M.; Casciola-Rosen, Livia; Shah, Ami A.; Boin, Francesco; Hummers, Laura K.; Mammen, Andrew L.

    2015-01-01

    Objective To determine if distinct muscle pathological features exist in scleroderma subjects with weakness. Methods This retrospective study included weak scleroderma subjects with muscle biopsies available for review. Biopsies were systematically assessed for individual pathologic features including inflammation, necrosis, fibrosis, and acute neurogenic atrophy. Based on the aggregate individual features, biopsies were assigned a histopathologic category of polymyositis, dermatomyositis, necrotizing myopathy, non-specific myositis, “acute denervation”, “fibrosis only”, or “other”. Clinical data analyzed included autoantibody profiles, scleroderma subtype and disease duration, Medsger muscle severity scores, creatine kinase (CK), electromyography (EMG), and muscle magnetic resonance imaging (MRI). Results 42 subjects (79% female and 64% diffuse scleroderma) were included in this study. Necrosis (67%), inflammation (48%), acute neurogenic atrophy (48%), and fibrosis (33%) were the most prevalent pathologic features. The presence of fibrosis was strongly associated with anti-PM-Scl antibodies. Histopathologic categories included non-specific myositis (36%), necrotizing myopathy (21%), dermatomyositis (7%), “acute denervation” (7%), “fibrosis only” (7%), and polymyositis (5%). Disease duration of scleroderma at the time of muscle biopsy was shorter in polymyositis than other histopathologic categories. Patients with anti-PM-Scl and Scl-70 antibodies also had a shorter disease duration than those with other auto-antibody profiles. Conclusion Non-specific myositis and necrotizing myopathy were the most common histopathologic categories in weak scleroderma subjects. Surprisingly, nearly half of the subjects studied had histological evidence of acute motor denervation (acute neurogenic atrophy); this has not been previously reported. Taken together, these observations suggest that a variety of pathologic mechanisms may underlie the development of

  10. MUSCLE WEAKNESS, FATIGUE, AND TORQUE VARIABILITY: EFFECTS OF AGE AND MOBILITY STATUS

    Science.gov (United States)

    KENT-BRAUN, JANE A.; CALLAHAN, DAMIEN M.; FAY, JESSICA L.; FOULIS, STEPHEN A.; BUONACCORSI, JOHN P.

    2013-01-01

    Introduction Whereas deficits in muscle function, particularly power production, develop in old age and are risk factors for mobility impairment, a complete understanding of muscle fatigue during dynamic contractions is lacking. We tested hypotheses related to torque-producing capacity, fatigue resistance, and variability of torque production during repeated maximal contractions in healthy older, mobility-impaired older, and young women. Methods Knee extensor fatigue (decline in torque) was measured during 4 min of dynamic contractions. Torque variability was characterized using a novel 4-component logistic regression model. Results Young women produced more torque at baseline and during the protocol than older women (P torque variability differed by group (P = 0.022) and was greater in older impaired compared with young women (P = 0.010). Conclusions These results suggest that increased torque variability may combine with baseline muscle weakness to limit function, particularly in older adults with mobility impairments. PMID:23674266

  11. Simvastatin reduces fibrosis and protects against muscle weakness after massive rotator cuff tear.

    Science.gov (United States)

    Davis, Max E; Korn, Michael A; Gumucio, Jonathan P; Harning, Julie A; Saripalli, Anjali L; Bedi, Asheesh; Mendias, Christopher L

    2015-02-01

    Chronic rotator cuff tears are a common source of shoulder pain and disability, and patients with chronic cuff tears often have substantial weakness, fibrosis, inflammation, and fat accumulation. Identifying therapies to prevent the development of these pathologic processes will likely have a positive impact on clinical outcomes. Simvastatin is a drug with demonstrated anti-inflammatory and antifibrotic effects in many tissues but had not previously been studied in the context of rotator cuff tears. We hypothesized that after the induction of a massive supraspinatus tear, simvastatin would protect muscles from a loss of force production and fibrosis. We measured changes in muscle fiber contractility, histology, and biochemical markers of fibrosis and fatty infiltration in rats that received a full-thickness supraspinatus tear and were treated with either carrier alone or simvastatin. Compared with vehicle-treated controls, simvastatin did not have an appreciable effect on muscle fiber size, but treatment did increase muscle fiber specific force by 20%. Simvastatin also reduced collagen accumulation by 50% but did not affect triglyceride content of muscles. Several favorable changes in the expression of genes and other markers of inflammation, fibrosis, and regeneration were also observed. Simvastatin partially protected muscles from the weakness that occurs as a result of chronic rotator cuff tear. Fibrosis was also markedly reduced in simvastatin-treated animals. Whereas further studies are necessary, statin medication could potentially help improve outcomes for patients with rotator cuff tears. Copyright © 2015 Journal of Shoulder and Elbow Surgery Board of Trustees. Published by Elsevier Inc. All rights reserved.

  12. Compensatory strategies during manual wheelchair propulsion in response to weakness in individual muscle groups: A simulation study.

    Science.gov (United States)

    Slowik, Jonathan S; McNitt-Gray, Jill L; Requejo, Philip S; Mulroy, Sara J; Neptune, Richard R

    2016-03-01

    The considerable physical demand placed on the upper extremity during manual wheelchair propulsion is distributed among individual muscles. The strategy used to distribute the workload is likely influenced by the relative force-generating capacities of individual muscles, and some strategies may be associated with a higher injury risk than others. The objective of this study was to use forward dynamics simulations of manual wheelchair propulsion to identify compensatory strategies that can be used to overcome weakness in individual muscle groups and identify specific strategies that may increase injury risk. Identifying these strategies can provide rationale for the design of targeted rehabilitation programs aimed at preventing the development of pain and injury in manual wheelchair users. Muscle-actuated forward dynamics simulations of manual wheelchair propulsion were analyzed to identify compensatory strategies in response to individual muscle group weakness using individual muscle mechanical power and stress as measures of upper extremity demand. The simulation analyses found the upper extremity to be robust to weakness in any single muscle group as the remaining groups were able to compensate and restore normal propulsion mechanics. The rotator cuff muscles experienced relatively high muscle stress levels and exhibited compensatory relationships with the deltoid muscles. These results underline the importance of strengthening the rotator cuff muscles and supporting muscles whose contributions do not increase the potential for impingement (i.e., the thoracohumeral depressors) and minimize the risk of upper extremity injury in manual wheelchair users. Copyright © 2016 Elsevier Ltd. All rights reserved.

  13. Feeding during the resting phase causes profound changes in physiology and desynchronization between liver and muscle rhythms of rats.

    Science.gov (United States)

    Opperhuizen, Anne-Loes; Wang, Dawei; Foppen, Ewout; Jansen, Remi; Boudzovitch-Surovtseva, Olga; de Vries, Janneke; Fliers, Eric; Kalsbeek, Andries

    2016-11-01

    Shiftworkers run an increased risk of developing metabolic disorders, presumably as a result of disturbed circadian physiology. Eating at a time-of-day that is normally dedicated to resting and fasting, may contribute to this association. The hypothalamus is the key brain area that integrates different inputs, including environmental time information from the central biological clock in the suprachiasmatic nuclei, with peripheral information on energy status to maintain energy homeostasis. The orexin system within the lateral hypothalamus is an important output of the suprachiasmatic nuclei involved in the control of sleep/wake behavior and glucose homeostasis, among other functions. In this study, we tested the hypothesis that feeding during the rest period disturbs the orexin system as a possible underlying contributor to metabolic health problems. Male Wistar rats were exposed to an 8-week protocol in which food was available ad libitum for 24-h, for 12-h during the light phase (i.e., unnatural feeding time) or for 12-h during the dark phase (i.e., restricted feeding, but at the natural time-of-day). Animals forced to eat at an unnatural time, i.e., during the light period, showed no changes in orexin and orexin-receptor gene expression in the hypothalamus, but the rhythmic expression of clock genes in the lateral hypothalamus was absent in these animals. Light fed animals did show adverse changes in whole-body physiology and internal desynchronization of muscle and liver clock and metabolic gene expression. Eating at the 'wrong' time-of-day thus causes internal desynchronization at different levels, which in the long run may disrupt body physiology. © 2016 Federation of European Neuroscience Societies and John Wiley & Sons Ltd.

  14. Sildenafil reduces respiratory muscle weakness and fibrosis in the mdx mouse model of Duchenne muscular dystrophy.

    Science.gov (United States)

    Percival, Justin M; Whitehead, Nicholas P; Adams, Marvin E; Adamo, Candace M; Beavo, Joseph A; Froehner, Stanley C

    2012-09-01

    Duchenne muscular dystrophy (DMD) is the most common form of muscular dystrophy caused by mutations in the dystrophin gene. Loss of dystrophin initiates a progressive decline in skeletal muscle integrity and contractile capacity which weakens respiratory muscles including the diaphragm, culminating in respiratory failure, the leading cause of morbidity and mortality in DMD patients. At present, corticosteroid treatment is the primary pharmacological intervention in DMD, but has limited efficacy and adverse side effects. Thus, there is an urgent need for new safe, cost-effective, and rapidly implementable treatments that slow disease progression. One promising new approach is the amplification of nitric oxide-cyclic guanosine monophosphate (NO-cGMP) signalling pathways with phosphodiesterase 5 (PDE5) inhibitors. PDE5 inhibitors serve to amplify NO signalling that is attenuated in many neuromuscular diseases including DMD. We report here that a 14-week treatment of the mdx mouse model of DMD with the PDE5 inhibitor sildenafil (Viagra(®), Revatio(®)) significantly reduced mdx diaphragm muscle weakness without impacting fatigue resistance. In addition to enhancing respiratory muscle contractility, sildenafil also promoted normal extracellular matrix organization. PDE5 inhibition slowed the establishment of mdx diaphragm fibrosis and reduced matrix metalloproteinase-13 (MMP-13) expression. Sildenafil also normalized the expression of the pro-fibrotic (and pro-inflammatory) cytokine tumour necrosis factor α (TNFα). Sildenafil-treated mdx diaphragms accumulated significantly less Evans Blue tracer dye than untreated controls, which is also indicative of improved diaphragm muscle health. We conclude that sildenafil-mediated PDE5 inhibition significantly reduces diaphragm respiratory muscle dysfunction and pathology in the mdx mouse model of Duchenne muscular dystrophy. This study provides new insights into the therapeutic utility of targeting defects in NO

  15. A decline in PABPN1 induces progressive muscle weakness in oculopharyngeal muscle dystrophy and in muscle aging

    DEFF Research Database (Denmark)

    Anvar, Seyed Yahya; Raz, Yotam; Verway, Nisha

    2013-01-01

    Oculopharyngeal muscular dystrophy (OPMD) is caused by trinucleotide repeat expansion mutations in Poly(A) binding protein 1 (PABPN1). PABPN1 is a regulator of mRNA stability and is ubiquitously expressed. Here we investigated how symptoms in OPMD initiate only at midlife and why a subset...... of skeletal muscles is predominantly affected. Genome-wide RNA expression profiles from Vastus lateralis muscles human carriers of expanded-PABPN1 at pre-symptomatic and symptomatic stages were compared with healthy controls. Major expression changes were found to be associated with age rather than...... with expression of expanded-PABPN1, instead transcriptomes of OPMD and elderly muscles were significantly similar (P...

  16. Hypogonadism in patients with chronic obstructive pulmonary disease: relationship with airflow limitation, muscle weakness and systemic inflammation

    Directory of Open Access Journals (Sweden)

    Rasha Galal Daabis

    2016-03-01

    Conclusion: Hypogonadism is highly prevalent in clinically stable COPD patients and is particularly related to the severity of the airway obstruction. Systemic inflammation is present in stable COPD patients and its intensity is related to the severity of the underlying disease and it predisposes to skeletal muscle weakness and exercise intolerance. However, we failed to find a significant association between hypogonadism and muscle weakness or systemic inflammation.

  17. Hereditary sensory ataxic neuropathy associated with proximal muscle weakness in the lower extremities.

    Science.gov (United States)

    Murakami, Tatsufumi; Fukai, Yuta; Rikimaru, Mitsue; Henmi, Shoji; Ohsawa, Yutaka; Sunada, Yoshihide

    2010-04-15

    We describe three patients from the same family with hereditary sensory ataxic neuropathy followed by proximal muscle weakness in the lower extremities. Sensory ataxic gait began as an initial symptom when patients were in their 50s. Mild proximal weakness in the lower extremities appeared several years later. Serum creatine kinase was mildly elevated. Nerve conduction studies revealed sensory dominant axonal neuropathy, and short sensory evoked potentials showed involvement of the sensory nerve axon, dorsal root ganglia and posterior funiculus of the spinal cord. Needle electromyography showed fibrillation, positive sharp waves, and multiple giant motor unit potentials, suggesting the involvement of anterior horn motor neurons or the anterior root. Autosomal recessive inheritance was considered, because of consanguinity. The disorder described here may be a new clinical entity with unique clinical manifestations. Copyright 2009 Elsevier B.V. All rights reserved.

  18. Age-related weakness of proximal muscle studied with motor cortical mapping: a TMS study.

    Directory of Open Access Journals (Sweden)

    Ela B Plow

    Full Text Available Aging-related weakness is due in part to degeneration within the central nervous system. However, it is unknown how changes to the representation of corticospinal output in the primary motor cortex (M1 relate to such weakness. Transcranial magnetic stimulation (TMS is a noninvasive method of cortical stimulation that can map representation of corticospinal output devoted to a muscle. Using TMS, we examined age-related alterations in maps devoted to biceps brachii muscle to determine whether they predicted its age-induced weakness. Forty-seven right-handed subjects participated: 20 young (22.6 ± 0.90 years and 27 old (74.96 ± 1.35 years. We measured strength as force of elbow flexion and electromyographic activation of biceps brachii during maximum voluntary contraction. Mapping variables included: 1 center of gravity or weighted mean location of corticospinal output, 2 size of map, 3 volume or excitation of corticospinal output, and 4 response density or corticospinal excitation per unit area. Center of gravity was more anterior in old than in young (p<0.001, though there was no significant difference in strength between the age groups. Map size, volume, and response density showed no significant difference between groups. Regardless of age, center of gravity significantly predicted strength (β = -0.34, p = 0.005, while volume adjacent to the core of map predicted voluntary activation of biceps (β = 0.32, p = 0.008. Overall, the anterior shift of the map in older adults may reflect an adaptive change that allowed for the maintenance of strength. Laterally located center of gravity and higher excitation in the region adjacent to the core in weaker individuals could reflect compensatory recruitment of synergistic muscles. Thus, our study substantiates the role of M1 in adapting to aging-related weakness and subtending strength and muscle activation across age groups. Mapping from M1 may offer foundation for an examination of mechanisms that

  19. High-intensity interval training prevents oxidant-mediated diaphragm muscle weakness in hypertensive mice.

    Science.gov (United States)

    Bowen, T Scott; Eisenkolb, Sophia; Drobner, Juliane; Fischer, Tina; Werner, Sarah; Linke, Axel; Mangner, Norman; Schuler, Gerhard; Adams, Volker

    2017-01-01

    Hypertension is a key risk factor for heart failure, with the latter characterized by diaphragm muscle weakness that is mediated in part by increased oxidative stress. In the present study, we used a deoxycorticosterone acetate (DOCA)-salt mouse model to determine whether hypertension could independently induce diaphragm dysfunction and further investigated the effects of high-intensity interval training (HIIT). Sham-treated (n = 11), DOCA-salt-treated (n = 11), and DOCA-salt+HIIT-treated (n = 15) mice were studied over 4 wk. Diaphragm contractile function, protein expression, enzyme activity, and fiber cross-sectional area and type were subsequently determined. Elevated blood pressure confirmed hypertension in DOCA-salt mice independent of HIIT (P HIIT. Myosin heavy chain (MyHC) protein expression tended to decrease (∼30%; P = 0.06) in DOCA-salt vs. sham- and DOCA-salt+HIIT mice, whereas oxidative stress increased (P HIIT further prevented direct oxidant-mediated diaphragm contractile dysfunction (P hypertension induces diaphragm contractile dysfunction via an oxidant-mediated mechanism that is prevented by HIIT.-Bowen, T. S., Eisenkolb, S., Drobner, J., Fischer, T., Werner, S., Linke, A., Mangner, N., Schuler, G., Adams, V. High-intensity interval training prevents oxidant-mediated diaphragm muscle weakness in hypertensive mice. © FASEB.

  20. Relative contribution of different altered motor unit control to muscle weakness in stroke: a simulation study

    Science.gov (United States)

    Shin, Henry; Suresh, Nina L.; Zev Rymer, William; Hu, Xiaogang

    2018-02-01

    Objective. Chronic muscle weakness impacts the majority of individuals after a stroke. The origins of this hemiparesis is multifaceted, and an altered spinal control of the motor unit (MU) pool can lead to muscle weakness. However, the relative contribution of different MU recruitment and discharge organization is not well understood. In this study, we sought to examine these different effects by utilizing a MU simulation with variations set to mimic the changes of MU control in stroke. Approach. Using a well-established model of the MU pool, this study quantified the changes in force output caused by changes in MU recruitment range and recruitment order, as well as MU firing rate organization at the population level. We additionally expanded the original model to include a fatigue component, which variably decreased the output force with increasing length of contraction. Differences in the force output at both the peak and fatigued time points across different excitation levels were quantified and compared across different sets of MU parameters. Main results. Across the different simulation parameters, we found that the main driving factor of the reduced force output was due to the compressed range of MU recruitment. Recruitment compression caused a decrease in total force across all excitation levels. Additionally, a compression of the range of MU firing rates also demonstrated a decrease in the force output mainly at the higher excitation levels. Lastly, changes to the recruitment order of MUs appeared to minimally impact the force output. Significance. We found that altered control of MUs alone, as simulated in this study, can lead to a substantial reduction in muscle force generation in stroke survivors. These findings may provide valuable insight for both clinicians and researchers in prescribing and developing different types of therapies for the rehabilitation and restoration of lost strength after stroke.

  1. Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons

    OpenAIRE

    Lee, Young il; Mikesh, Michelle; Smith, Ian; Rimer, Mendell; Thompson, Wesley

    2011-01-01

    A mouse model of the devastating human disease "spinal muscular atrophy" (SMA) was used to investigate the severe muscle weakness and spasticity that precedes the death of these animals near the end of the 2nd postnatal week. Counts of motor units to the soleus muscle as well as of axons in the soleus muscle nerve showed no loss of motor neurons. Similarly, neither immunostaining of neuromuscular junctions nor the measurement of the tension generated by nerve stimulation gave evidence of any ...

  2. High prevalence of respiratory muscle weakness in hospitalized acute heart failure elderly patients.

    Directory of Open Access Journals (Sweden)

    Pedro Verissimo

    Full Text Available Respiratory Muscle Weakness (RMW has been defined when the maximum inspiratory pressure (MIP is lower than 70% of the predictive value. The prevalence of RMW in chronic heart failure patients is 30 to 50%. So far there are no studies on the prevalence of RMW in acute heart failure (AHF patients.Evaluate the prevalence of RMW in patients admitted because of AHF and the condition of respiratory muscle strength on discharge from the hospital.Sixty-three patients had their MIP measured on two occasions: at the beginning of the hospital stay, after they had reached respiratory, hemodynamic and clinical stability and before discharge from the hospital. The apparatus and technique to measure MIP were adapted because of age-related limitations of the patients. Data on cardiac ejection fraction, ECG, brain natriuretic peptide (BNP levels and on the use of noninvasive ventilation (NIV were collected.The mean age of the 63 patients under study was 75 years. On admission the mean ejection fraction was 33% (95% CI: 31-35 and the BNP hormone median value was 726.5 pg/ml (range: 217 to 2283 pg/ml; 65% of the patients used NIV. The median value of MIP measured after clinical stabilization was -52.7 cmH2O (range: -20 to -120 cmH2O; 76% of the patients had MIP values below 70% of the predictive value. On discharge, after a median hospital stay of 11 days, the median MIP was -53.5 cmH2O (range:-20 to -150 cmH2O; 71% of the patients maintained their MIP values below 70% of the predictive value. The differences found were not statistically significant.Elderly patients admitted with AHF may present a high prevalence of RMW on admission; this condition may be maintained at similar levels on discharge in a large percentage of these patients, even after clinical stabilization of the heart condition.

  3. Neuromuscular electrical stimulation of the cricothyroid muscle in patients with suspected superior laryngeal nerve weakness.

    Science.gov (United States)

    Guzman, Marco; Rubin, Adam; Cox, Paul; Landini, Fernando; Jackson-Menaldi, Cristina

    2014-03-01

    In this retrospective case study, we report the apparent clinical effectiveness of neuromuscular electrical stimulation (NMES) in combination with voice therapy (VT) for rehabilitating dysphonia secondary to suspected superior laryngeal nerve (SLN) weakness in two female patients. Both patients failed or plateaued with traditional VT but had significant improvement with the addition of NMES of the cricothyroid muscle and SLN using a VitalStim unit. Stimulation was provided simultaneously with voice exercises based on musical phonatory tasks. Both acoustic analysis and endoscopic evaluation demonstrated important improvements after treatment. In the first patient, the major change was obtained within the primo passaggio region; specifically, a decrease in voice breaks was demonstrated. In the second patient, an improvement in voice quality (less breathiness) and vocal range were the most important findings. Additionally, each patient reported a significant improvement in their voice complaints. Neuromuscular laryngeal electrical stimulation in combination with vocal exercises might be a useful tool to improve voice quality in patients with SLN injury. Copyright © 2014 The Voice Foundation. Published by Mosby, Inc. All rights reserved.

  4. Enzyme replacement therapy rescues weakness and improves muscle pathology in mice with X-linked myotubular myopathy.

    Science.gov (United States)

    Lawlor, Michael W; Armstrong, Dustin; Viola, Marissa G; Widrick, Jeffrey J; Meng, Hui; Grange, Robert W; Childers, Martin K; Hsu, Cynthia P; O'Callaghan, Michael; Pierson, Christopher R; Buj-Bello, Anna; Beggs, Alan H

    2013-04-15

    No effective treatment exists for patients with X-linked myotubular myopathy (XLMTM), a fatal congenital muscle disease caused by deficiency of the lipid phosphatase, myotubularin. The Mtm1δ4 and Mtm1 p.R69C mice model severely and moderately symptomatic XLMTM, respectively, due to differences in the degree of myotubularin deficiency. Contractile function of intact extensor digitorum longus (EDL) and soleus muscles from Mtm1δ4 mice, which produce no myotubularin, is markedly impaired. Contractile forces generated by chemically skinned single fiber preparations from Mtm1δ4 muscle were largely preserved, indicating that weakness was largely due to impaired excitation contraction coupling. Mtm1 p.R69C mice, which produce small amounts of myotubularin, showed impaired contractile function only in EDL muscles. Short-term replacement of myotubularin with a prototypical targeted protein replacement agent (3E10Fv-MTM1) in Mtm1δ4 mice improved contractile function and muscle pathology. These promising findings suggest that even low levels of myotubularin protein replacement can improve the muscle weakness and reverse the pathology that characterizes XLMTM.

  5. What is profound?

    DEFF Research Database (Denmark)

    Sobisch, Jan-Ulrich

    2013-01-01

    Discussing the question, which elements on the path are to be considered profound. While a general view is that the most subtle practises are also the most profound, 'Jig-rten-mgon-po maintains that the most fundamental one's are to be considered the most profound....

  6. Detecting intrinsic muscle weakness of the hallux as an addition to early-stage screening of the feet in patients with diabetes

    NARCIS (Netherlands)

    Mahieu, R.; Coenen, M. N. O.; van Bemmel, T.; van der Zaag-Loonen, H. J.; Theuvenet, W. J.

    Aims: Present-day screening of the diabetic foot involves the Semmes Weinstein Monofilament Test for evaluating loss of sensibility, while testing for intrinsic muscle weakness is not implied. Just as with the early detection of sensibility loss, early detection of intrinsic muscle weakness might

  7. Lung volume recruitment acutely increases respiratory system compliance in individuals with severe respiratory muscle weakness

    Directory of Open Access Journals (Sweden)

    Yannick Molgat-Seon

    2017-03-01

    Full Text Available The aim of the present study was to determine whether lung volume recruitment (LVR acutely increases respiratory system compliance (Crs in individuals with severe respiratory muscle weakness (RMW. Individuals with RMW resulting from neuromuscular disease or quadriplegia (n=12 and healthy controls (n=12 underwent pulmonary function testing and the measurement of Crs at baseline, immediately after, 1 h after and 2 h after a single standardised session of LVR. The LVR session involved 10 consecutive supramaximal lung inflations with a manual resuscitation bag to the highest tolerable mouth pressure or a maximum of 50 cmH2O. Each LVR inflation was followed by brief breath-hold and a maximal expiration to residual volume. At baseline, individuals with RMW had lower Crs than controls (37±5 cmH2O versus 109±10 mL·cmH2O−1, p0.05. LVR had no significant effect on measures of pulmonary function at any time point in either group (all p>0.05. During inflations, mean arterial pressure decreased significantly relative to baseline by 10.4±2.8 mmHg and 17.3±3.0 mmHg in individuals with RMW and controls, respectively (both p<0.05. LVR acutely increases Crs in individuals with RMW. However, the high airway pressures during inflations cause reductions in mean arterial pressure that should be considered when applying this technique.

  8. Diaphragm Muscle Fiber Weakness and Ubiquitin-Proteasome Activation in Critically Ill Patients

    NARCIS (Netherlands)

    Hooijman, P.E.; Beishuizen, A.; Witt, C.C.; de Waard, M.C.; Girbes, A.R.J.; Spoelstra-de Man, A.M.E.; Niessen, H.W.; Manders, E.; van Hees, H.W.H.; van den Brom, C.E.; Silderhuis, V.; Lawlor, M.W.; Labeit, S.; Stienen, G.J.M.; Hartemink, K.J.; Paul, M.A.; Heunks, L.M.A.; Ottenheijm, C.A.C.

    2015-01-01

    RATIONALE: The clinical significance of diaphragm weakness in critically ill patients is evident: it prolongs ventilator dependency, and increases morbidity and duration of hospital stay. To date, the nature of diaphragm weakness and its underlying pathophysiologic mechanisms are poorly understood.

  9. Effect of early implementation of electrical muscle stimulation to prevent muscle atrophy and weakness in patients after anterior cruciate ligament reconstruction.

    Science.gov (United States)

    Hasegawa, Satoshi; Kobayashi, Masahiko; Arai, Ryuzo; Tamaki, Akira; Nakamura, Takashi; Moritani, Toshio

    2011-08-01

    Following anterior cruciate ligament (ACL) reconstruction, restricted weight bearing and immobilization results in thigh and calf muscle atrophy and weakness. The purpose of this study was to assess the effect of electrical muscle stimulation (EMS) on prevention of muscle atrophy in patients during the early rehabilitation stage after ACL reconstruction. Twenty patients with acute ACL tears were divided into two groups randomly. The control group (CON group) participated in only the usual rehabilitation program. In addition to this protocol, the electrical muscle stimulation group (EMS group) received EMS training using the wave form of 20 Hz exponential pulse from the 2nd post-operative day to 4 weeks after the surgery. Muscle thickness of vastus lateralis and calf increased significantly 4 weeks after surgery in the EMS group, while it decreased significantly in the CON group. The decline of knee extension strength was significantly less in the EMS group than in the CON group at 4 weeks after the surgery, and the EMS group showed greater recovery of knee extension strength at 3 months after surgery. EMS implemented during the early rehabilitation stage is effective in maintaining and increasing muscle thickness and strength in the operated limb. Copyright © 2011 Elsevier Ltd. All rights reserved.

  10. Deletion of the Ste20-like kinase SLK in skeletal muscle results in a progressive myopathy and muscle weakness.

    Science.gov (United States)

    Pryce, Benjamin R; Al-Zahrani, Khalid N; Dufresne, Sébastien; Belkina, Natalya; Labrèche, Cédrik; Patino-Lopez, Genaro; Frenette, Jérôme; Shaw, Stephen; Sabourin, Luc A

    2017-02-02

    The Ste20-like kinase, SLK, plays an important role in cell proliferation and cytoskeletal remodeling. In fibroblasts, SLK has been shown to respond to FAK/Src signaling and regulate focal adhesion turnover through Paxillin phosphorylation. Full-length SLK has also been shown to be essential for embryonic development. In myoblasts, the overexpression of a dominant negative SLK is sufficient to block myoblast fusion. In this study, we crossed the Myf5-Cre mouse model with our conditional SLK knockout model to delete SLK in skeletal muscle. A thorough analysis of skeletal muscle tissue was undertaken in order to identify defects in muscle development caused by the lack of SLK. Isometric force analysis was performed on adult knockout mice and compared to age-matched wild-type mice. Furthermore, cardiotoxin injections were performed followed by immunohistochemistry for myogenic markers to assess the efficiency muscle regeneration following SLK deletion. We show here that early deletion of SLK from the myogenic lineage does not markedly impair skeletal muscle development but delays the regenerative process. Interestingly, adult mice (~6 months) display an increase in the proportion of central nuclei and increased p38 activation. Furthermore, mice as young as 3 months old present with decreased force generation, suggesting that the loss of SLK impairs myofiber stability and function. Assessment of structural components revealed aberrant localization of focal adhesion proteins, such as FAK and paxillin. Our data show that the loss of SLK results in unstable myofibers resulting in a progressive myopathy. Additionally, the loss of SLK resulted in a delay in muscle regeneration following cardiotoxin injections. Our results show that SLK is dispensable for muscle development and regeneration but is required for myofiber stability and optimal force generation.

  11. Diaphragm Muscle Weakness Following Acute Sustained Hypoxic Stress in the Mouse Is Prevented by Pretreatment with N-Acetyl Cysteine

    Directory of Open Access Journals (Sweden)

    Andrew J. O’Leary

    2018-01-01

    Full Text Available Oxygen deficit (hypoxia is a major feature of cardiorespiratory diseases characterized by diaphragm dysfunction, yet the putative role of hypoxic stress as a driver of diaphragm dysfunction is understudied. We explored the cellular and functional consequences of sustained hypoxic stress in a mouse model. Adult male mice were exposed to 8 hours of normoxia, or hypoxia (FiO2 = 0.10 with or without antioxidant pretreatment (N-acetyl cysteine, 200 mg/kg i.p.. Ventilation and metabolism were measured. Diaphragm muscle contractile function, myofibre size and distribution, gene expression, protein signalling cascades, and oxidative stress (TBARS were determined. Hypoxia caused pronounced diaphragm muscle weakness, unrelated to increased respiratory muscle work. Hypoxia increased diaphragm HIF-1α protein content and activated MAPK, mTOR, Akt, and FoxO3a signalling pathways, largely favouring protein synthesis. Hypoxia increased diaphragm lipid peroxidation, indicative of oxidative stress. FoxO3 and MuRF-1 gene expression were increased. Diaphragm 20S proteasome activity and muscle fibre size and distribution were unaffected by acute hypoxia. Pretreatment with N-acetyl cysteine substantially enhanced cell survival signalling, prevented hypoxia-induced diaphragm oxidative stress, and prevented hypoxia-induced diaphragm dysfunction. Hypoxia is a potent driver of diaphragm weakness, causing myofibre dysfunction without attendant atrophy. N-acetyl cysteine protects the hypoxic diaphragm and may have application as a potential adjunctive therapy.

  12. Mice deficient in ribosomal protein S6 phosphorylation suffer from muscle weakness that reflects a growth defect and energy deficit.

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    Igor Ruvinsky

    Full Text Available BACKGROUND: Mice, whose ribosomal protein S6 cannot be phosphorylated due to replacement of all five phosphorylatable serine residues by alanines (rpS6(P-/-, are viable and fertile. However, phenotypic characterization of these mice and embryo fibroblasts derived from them, has established the role of these modifications in the regulation of the size of several cell types, as well as pancreatic beta-cell function and glucose homeostasis. A relatively passive behavior of these mice has raised the possibility that they suffer from muscle weakness, which has, indeed, been confirmed by a variety of physical performance tests. METHODOLOGY/PRINCIPAL FINDINGS: A large variety of experimental methodologies, including morphometric measurements of histological preparations, high throughput proteomic analysis, positron emission tomography (PET and numerous biochemical assays, were used in an attempt to establish the mechanism underlying the relative weakness of rpS6(P-/- muscles. Collectively, these experiments have demonstrated that the physical inferiority appears to result from two defects: a a decrease in total muscle mass that reflects impaired growth, rather than aberrant differentiation of myofibers, as well as a diminished abundance of contractile proteins; and b a reduced content of ATP and phosphocreatine, two readily available energy sources. The abundance of three mitochondrial proteins has been shown to diminish in the knockin mouse. However, the apparent energy deficiency in this genotype does not result from a lower mitochondrial mass or compromised activity of enzymes of the oxidative phosphorylation, nor does it reflect a decline in insulin-dependent glucose uptake, or diminution in storage of glycogen or triacylglycerol (TG in the muscle. CONCLUSIONS/SIGNIFICANCE: This study establishes rpS6 phosphorylation as a determinant of muscle strength through its role in regulation of myofiber growth and energy content. Interestingly, a similar

  13. [A 34-year-old woman with delayed motor milestones, high arched palate, and proximal muscle weakness].

    Science.gov (United States)

    Yamamoto, T; Kitada, T; Hirasawa, E; Mori, H; Mizuno, Y

    1996-07-01

    We report a right-handed 34-year-old woman with diffuse muscle atrophy. The patient was a full-term infant of uneventful delivery, however, motor milestones were delayed in that neck control was obtained at 10 months of the age and she started to walk unassisted at 2 years of the age. Mental development was normal. She was unable to run with her mates at her kindergarten and she required a handrail when she walk up the stairs. She could not close her mouth completely at the primary school. She was unable to use a straw as a middle school pupil. Recently, she noted difficulty in raising her head from the supine position, and has become unable to walk a long distance. She was admitted to our hospital in September 17, 1994 when she was 34-year-old. On admission, general physical examination revealed that she looked slender weighing 38 kg with 149.5 cm height. She showed a high arched palate, slight scoliosis, and pes equinus. Otherwise general physical examination was unremarkable. Upon neurologic examination, she was alert and well oriented. Cranial nerves were unremarkable except for bilateral facial atrophy and moderate weakness. Her voice was of nasal quality, and swallowing was slightly difficult. No atrophy was noted in the sternocleidomastoid muscle. She showed waddling gait and positive Gowers' sign. Diffuse muscle atrophy was noted and mild to moderate weakness was presented more in the proximal part in both upper and lower extremities, however, deltoid muscles retained normal power. No ataxia was noted. All the deep tendon reflexes were lost. Sensation was intact. Routine laboratory examination was unremarkable. Serum CK was 56 IU/l. Electromyography revealed myogenic changes in the deltoid, biceps, and quadriceps muscles. A diagnostic biopsy was performed in the left biceps brachii muscle. The patient was discussed in the neurologic CPC, and the chief discussant arrived at the conclusion that the patient had nemaline myopathy. Opinions were divided among

  14. Muscles in a mouse model of spinal muscular atrophy show profound defects in neuromuscular development even in the absence of failure in neuromuscular transmission or loss of motor neurons.

    Science.gov (United States)

    Lee, Young Il; Mikesh, Michelle; Smith, Ian; Rimer, Mendell; Thompson, Wesley

    2011-08-15

    A mouse model of the devastating human disease "spinal muscular atrophy" (SMA) was used to investigate the severe muscle weakness and spasticity that precede the death of these animals near the end of the 2nd postnatal week. Counts of motor units to the soleus muscle as well as of axons in the soleus muscle nerve showed no loss of motor neurons. Similarly, neither immunostaining of neuromuscular junctions nor the measurement of the tension generated by nerve stimulation gave evidence of any significant impairment in neuromuscular transmission, even when animals were maintained up to 5days longer via a supplementary diet. However, the muscles were clearly weaker, generating less than half their normal tension. Weakness in 3 muscles examined in the study appears due to a severe but uniform reduction in muscle fiber size. The size reduction results from a failure of muscle fibers to grow during early postnatal development and, in soleus, to a reduction in number of fibers generated. Neuromuscular development is severely delayed in these mutant animals: expression of myosin heavy chain isoforms, the elimination of polyneuronal innervation, the maturation in the shape of the AChR plaque, the arrival of SCs at the junctions and their coverage of the nerve terminal, the development of junctional folds. Thus, if SMA in this particular mouse is a disease of motor neurons, it can act in a manner that does not result in their death or disconnection from their targets but nonetheless alters many aspects of neuromuscular development. Copyright © 2011 Elsevier Inc. All rights reserved.

  15. A new strength assessment to evaluate the association between muscle weakness and gait pathology in children with cerebral palsy.

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    Marije Goudriaan

    Full Text Available The main goal of this validation study was to evaluate whether lower limb muscle weakness and plantar flexor rate of force development (RFD related to altered gait parameters in children with cerebral palsy (CP, when weakness was assessed with maximal voluntary isometric contractions (MVICs in a gait related test position. As a subgoal, we analyzed intra- and intertester reliability of this new strength measurement method.Part 1 -Intra- and intertester reliability were determined with the intra-class correlation coefficient (ICC2,1 in 10 typical developing (TD children (age: 5-15. We collected MVICs in four lower limb muscle groups to define maximum joint torques, as well as plantar flexor RFD. Part 2 -Validity of the strength assessment was explored by analyzing the relations of lower limb joint torques and RFD to a series of kinematic- and kinetic gait features, the GDI (gait deviation index, and the GDI-kinetic in 23 children with CP (GMFCS I-II; age: 5-15 and 23 TD children (age: 5-15 with Spearman's rank correlation coefficients.Part 1 -The best reliability was found for the torque data (Nm, with the highest ICC2,1 (0.951 for knee extension strength (inter and the lowest (0.693 for dorsiflexion strength (intra. For plantar flexor RFD, the most reliable window size was 300 milliseconds (ICC2,1: 0.828 (inter and 0.692 (intra. Part 2 -The children with CP were significantly weaker than the TD children (p <0.001. Weakness of the dorsiflexors and plantar flexors associated with delayed and decreased knee flexion angle during swing, respectively. No other significant correlations were found.While our new strength assessment was reliable, intra-joint correlations between weakness, RFD, and gait deviations were low. However, we found inter-joint associations, reflected by a strong association between plantar- and dorsiflexor weakness, and decreased and delayed knee flexion angle during swing.

  16. Skeletal Muscle and Lymphocyte Mitochondrial Dysfunctions in Septic Shock Trigger ICU-Acquired Weakness and Sepsis-Induced Immunoparalysis

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    Quentin Maestraggi

    2017-01-01

    Full Text Available Fundamental events driving the pathological processes of septic shock-induced multiorgan failure (MOF at the cellular and subcellular levels remain debated. Emerging data implicate mitochondrial dysfunction as a critical factor in the pathogenesis of sepsis-associated MOF. If macrocirculatory and microcirculatory dysfunctions undoubtedly participate in organ dysfunction at the early stage of septic shock, an intrinsic bioenergetic failure, sometimes called “cytopathic hypoxia,” perpetuates cellular dysfunction. Short-term failure of vital organs immediately threatens patient survival but long-term recovery is also severely hindered by persistent dysfunction of organs traditionally described as nonvital, such as skeletal muscle and peripheral blood mononuclear cells (PBMCs. In this review, we will stress how and why a persistent mitochondrial dysfunction in skeletal muscles and PBMC could impair survival in patients who overcome the first acute phase of their septic episode. First, muscle wasting protracts weaning from mechanical ventilation, increases the risk of mechanical ventilator-associated pneumonia, and creates a state of ICU-acquired muscle weakness, compelling the patient to bed. Second, failure of the immune system (“immunoparalysis” translates into its inability to clear infectious foci and predisposes the patient to recurrent nosocomial infections. We will finally emphasize how mitochondrial-targeted therapies could represent a realistic strategy to promote long-term recovery after sepsis.

  17. Neuromuscular electrical stimulation for preventing skeletal-muscle weakness and wasting in critically ill patients

    DEFF Research Database (Denmark)

    Maffiuletti, Nicola A.; Roig, Marc; Karatzanos, Eleftherios

    2013-01-01

    ill patients, in comparison with usual care. Methods: We searched PubMed, CENTRAL, CINAHL, Web of Science, and PEDro to identify randomized controlled trials exploring the effect of NMES in critically ill patients, which had a well-defined NMES protocol, provided outcomes related to skeletal......-muscle strength and/or mass, and for which full text was available. Two independent reviewers extracted data on muscle-related outcomes (strength and mass), and participant and intervention characteristics, and assessed the methodological quality of the studies. Owing to the lack of means and standard deviations...... yielded 8 eligible studies involving 172 patients. The methodological quality of the studies was moderate to high. Five studies reported an increase in strength or better preservation of strength with NMES, with one study having a large effect size. Two studies found better preservation of muscle mass...

  18. Forearm Flexor Muscles in Children with Cerebral Palsy Are Weak, Thin and Stiff

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    Eva Pontén

    2017-04-01

    Full Text Available Children with cerebral palsy (CP often develop reduced passive range of motion with age. The determining factor underlying this process is believed to be progressive development of contracture in skeletal muscle that likely changes the biomechanics of the joints. Consequently, to identify the underlying mechanisms, we modeled the mechanical characteristics of the forearm flexors acting across the wrist joint. We investigated skeletal muscle strength (Grippit® and passive stiffness and viscosity of the forearm flexors in 15 typically developing (TD children (10 boys/5 girls, mean age 12 years, range 8–18 yrs and nine children with CP Nine children (6 boys/3 girls, mean age 11 ± 3 years (yrs, range 7–15 yrs using the NeuroFlexor® apparatus. The muscle stiffness we estimate and report is the instantaneous mechanical response of the tissue that is independent of reflex activity. Furthermore, we assessed cross-sectional area of the flexor carpi radialis (FCR muscle using ultrasound. Age and body weight did not differ significantly between the two groups. Children with CP had a significantly weaker (−65%, p < 0.01 grip and had smaller cross-sectional area (−43%, p < 0.01 of the FCR muscle. Passive stiffness of the forearm muscles in children with CP was increased 2-fold (p < 0.05 whereas viscosity did not differ significantly between CP and TD children. FCR cross-sectional area correlated to age (R2 = 0.58, p < 0.01, body weight (R2 = 0.92, p < 0.0001 and grip strength (R2 = 0.82, p < 0.0001 in TD children but only to grip strength (R2 = 0.60, p < 0.05 in children with CP. We conclude that children with CP have weaker, thinner, and stiffer forearm flexors as compared to typically developing children.

  19. Somatic mosaicism of a point mutation in the dystrophin gene in a patient presenting with an asymmetrical muscle weakness and contractures

    NARCIS (Netherlands)

    Helderman-van den Enden, A. T. J. M.; Ginjaar, H. B.; Kneppers, A. L. J.; Bakker, E.; Breuning, M. H.; de Visser, M.

    2003-01-01

    We describe a patient with somatic mosaicism of a point mutation in the dystrophin gene causing benign muscular dystrophy with an unusual asymmetrical distribution of muscle weakness and contractures. To our knowledge this is the first patient with asymmetrical weakness and contractures in an

  20. Muscle weakness and lack of reflex gain adaptation predominate during post-stroke posture control of the wrist

    Directory of Open Access Journals (Sweden)

    van der Helm Frans CT

    2009-07-01

    Full Text Available Abstract Background Instead of hyper-reflexia as sole paradigm, post-stroke movement disorders are currently considered the result of a complex interplay between neuronal and muscular properties, modified by level of activity. We used a closed loop system identification technique to quantify individual contributors to wrist joint stiffness during an active posture task. Methods Continuous random torque perturbations applied to the wrist joint by a haptic manipulator had to be resisted maximally. Reflex provoking conditions were applied i.e. additional viscous loads and reduced perturbation signal bandwidth. Linear system identification and neuromuscular modeling were used to separate joint stiffness into the intrinsic resistance of the muscles including co-contraction and the reflex mediated contribution. Results Compared to an age and sex matched control group, patients showed an overall 50% drop in intrinsic elasticity while their reflexive contribution did not respond to provoking conditions. Patients showed an increased mechanical stability compared to control subjects. Conclusion Post stroke, we found active posture tasking to be dominated by: 1 muscle weakness and 2 lack of reflex adaptation. This adds to existing doubts on reflex blocking therapy as the sole paradigm to improve active task performance and draws attention to muscle strength and power recovery and the role of the inability to modulate reflexes in post stroke movement disorders.

  1. Diospyros rhodocalyx (Tako-Na), a Thai folk medicine, associated with hypokalemia and generalized muscle weakness: a case series.

    Science.gov (United States)

    Othong, Rittirak; Trakulsrichai, Satariya; Wananukul, Winai

    2017-11-01

    Diospyros rhodocalyx (Tako-Na) is a Thai folk medicine purported to promote longevity, treat impotence, etc. We present patients with hypokalemia, weakness and hypertension after consuming Tako-Na tea. Case 1: A 61-year-old man was brought in nine hours after drinking 400-500 mL of Tako-Na tea. One handful of Tako-Na bark was boiled in water to make tea. He had vomiting and watery diarrhea six hours after drinking it. He took no medications and had no history of hypertension. The only remarkable vital sign was BP 167/90 mmHg. Physical examination revealed generalized muscle weakness. Laboratory findings were potassium 2.7 mmol/L, bicarbonate 24 mmol/L, and transtubular potassium gradient (TTKG) 5.6. He was discharged the next day with a BP 140/90 mmHg and potassium 4.2 mmol/L. Case 2: A 78-year-old man, a friend of case 1, also drank Tako-Na tea from the same pot at the same time as case 1. He also had vomiting and diarrhea six hours later. He took no medications despite past history of hypertension (baseline SBP 140-160). Initial BP was 230/70 mmHg. He also had muscle weakness. Laboratory findings were potassium 3.3 mmol/L, bicarbonate 24 mmol/L, TTKG 7.37 and normal thyroid function. He was also discharged the next day with a BP 148/70 mmHg and potassium 4.2 mmol/L. Case 3-7: These were patients reported to a poison center and their potassium concentrations were 1.4, 1.4, 3.3, 1.3 and 1.2 mmol/L, respectively. Three of them were intubated and case 3 died. Tako-Na contains betulin, betulinic acid, taraxerone, lupeol, and lupenone. Their structures are similar to glycyrrhetic acid, the active metabolite of glycyrrhizic acid found in licorice which is well known to cause pseudoaldosteronism. Glycyrrhetic acid is potent in inhibiting 11-beta-hydroxysteroid dehydrogenase, and causes pseudoaldosteronism. We hypothesize that the compounds in Tako-Na act in the same way as glycyrrhetic acid in producing pseudoaldosteronism.

  2. Muscle Weakness Is Associated With an Increase of Left Ventricular Mass Through Excessive Blood Pressure Elevation During Exercise in Patients With Hypertension.

    Science.gov (United States)

    Kamada, Yumi; Masuda, Takashi; Tanaka, Shinya; Akiyama, Ayako; Nakamura, Takeshi; Hamazaki, Nobuaki; Okubo, Michihito; Kobayashi, Naoyuki; Ako, Junya

    2017-08-03

    Autonomic imbalance in hypertension induces excessive blood pressure (BP) elevation during exercise, thereby increasing left ventricular mass (LVM). Although muscle weakness enhances autonomic imbalance by stimulating muscle sympathetic activity during exercise, it is unclear whether muscle weakness is associated with an increase of LVM in patients with hypertension. This study aimed to investigate the relationships between muscle weakness, BP elevation during exercise, and LVM in these patients. Eighty-six hypertensive patients aged 69 ± 8 years with controlled resting BP (ie, exercise test performed at moderate intensity (ΔNORA and ΔPWV, respectively). A difference between baseline and peak systolic BP during the exercise test was defined as BP elevation during exercise (ΔSBP). Relationships between muscle strength, ΔNORA, ΔPWV, ΔSBP, BNP, and LVMI were analyzed, and significant factors increasing LVM were identified using univariate and multivariate regression analyses. Muscle strength was negatively correlated with ΔNORA (r = -0.202, P = 0.048), ΔPWV (r = -0.328, P = 0.002), ΔSBP (r = -0.230, P = 0.033), BNP (r = -0.265, P = 0.014), and LVMI (r = -0.233, P = 0.031). LVMI was positively correlated with ΔPWV (r = 0.246, P = 0.023) and ΔSBP (r = 0.307, P = 0.004). Muscle strength was a significant independent factor associated with LVMI (β = -0.331, P = 0.010). Our findings suggest that muscle weakness is associated with an increase of LVM through excessive BP elevation during exercise in patients with hypertension.

  3. Intellectual disability, muscle weakness and characteristic face in three siblings: A newly described recessive syndrome mapping to 3p24.3-p25.3

    NARCIS (Netherlands)

    Kariminejad, Ariana; Nafissi, Shahriar; Nilipoor, Yalda; Tavasoli, Alireza; van Veldhoven, Paul P.; Bonnard, Carine; Ng, Yeng Ting; Majoie, Charles B.; Reversade, Bruno; Hennekam, Raoul C.

    2015-01-01

    We report on a sister and two brothers born to healthy Iranian parents with mild intellectual disability, progressive muscle weakness, and characteristic facies. including highly arched eyebrows, down-slanting palpebral fissures, prominent nasal bridge, prominent nose, columella extending below alae

  4. Describing a new syndrome in L5-S1 disc herniation: Sexual and sphincter dysfunction without pain and muscle weakness

    Directory of Open Access Journals (Sweden)

    Nezih Akca

    2014-01-01

    Full Text Available Context: Little seems to be known about the sexual dysfunction (SD in lumbar intervertebral disc herniation. Aims: Investigation of sexual and sphincter dysfunction in patient with lumbar disc hernitions. Settings and Design: A retrospective analysis. Materials and Methods: Sexual and sphincter dysfunction in patients admitted with lumbar disc herniations between September 2012-March 2014. Statistical Analysis Used: Statistical analysis was performed using the Predictive Analytics SoftWare (PASW Statistics 18.0 for Windows (Statistical Package for the Social Sciences, SPSS Inc., Chicago, Illinois. The statistical significance was set at P < 0.05. The Wilcoxon signed ranks test was used to evaluate the difference between patients. Results: Four patients with sexual and sphincter dysfunction were found, including two women and two men, aged between 20 and 52 years. All of them admitted without low back pain. In addition, on neurological examination, reflex and motor deficit were not found. However, almost all patients had perianal sensory deficit and sexual and sphincter dysfunction. Magnetic resonance imaging (MRI of three patients displayed a large extruded disc fragment at L5-S1 level on the left side. In fourth patient, there were not prominent disc herniations. There was not statistically significant difference between pre-operative and post-operative sexual function, anal-urethral sphincter function, and perianal sensation score. A syndrome in L5-S1 disc herniation with sexual and sphincter dysfunction without pain and muscle weakness was noted. We think that it is crucial for neurosurgeons to early realise that paralysis of the sphincter and sexual dysfunction are possible in patients with lumbar L5-S1 disc disease. Conclusion: A syndrome with perianal sensory deficit, paralysis of the sphincter, and sexual dysfunction may occur in patients with lumbar L5-S1 disc disease. The improvement of perianal sensory deficit after surgery was

  5. Mutations in PTRH2 cause novel infantile-onset multisystem disease with intellectual disability, microcephaly, progressive ataxia, and muscle weakness.

    Science.gov (United States)

    Hu, Hao; Matter, Michelle L; Issa-Jahns, Lina; Jijiwa, Mayumi; Kraemer, Nadine; Musante, Luciana; de la Vega, Michelle; Ninnemann, Olaf; Schindler, Detlev; Damatova, Natalia; Eirich, Katharina; Sifringer, Marco; Schrötter, Sandra; Eickholt, Britta J; van den Heuvel, Lambert; Casamina, Chanel; Stoltenburg-Didinger, Gisela; Ropers, Hans-Hilger; Wienker, Thomas F; Hübner, Christoph; Kaindl, Angela M

    2014-12-01

    To identify the cause of a so-far unreported phenotype of infantile-onset multisystem neurologic, endocrine, and pancreatic disease (IMNEPD). We characterized a consanguineous family of Yazidian-Turkish descent with IMNEPD. The two affected children suffer from intellectual disability, postnatal microcephaly, growth retardation, progressive ataxia, distal muscle weakness, peripheral demyelinating sensorimotor neuropathy, sensorineural deafness, exocrine pancreas insufficiency, hypothyroidism, and show signs of liver fibrosis. We performed whole-exome sequencing followed by bioinformatic analysis and Sanger sequencing on affected and unaffected family members. The effect of mutations in the candidate gene was studied in wild-type and mutant mice and in patient and control fibroblasts. In a consanguineous family with two individuals with IMNEPD, we identified a homozygous frameshift mutation in the previously not disease-associated peptidyl-tRNA hydrolase 2 (PTRH2) gene. PTRH2 encodes a primarily mitochondrial protein involved in integrin-mediated cell survival and apoptosis signaling. We show that PTRH2 is highly expressed in the developing brain and is a key determinant in maintaining cell survival during human tissue development. Moreover, we link PTRH2 to the mTOR pathway and thus the control of cell size. The pathology suggested by the human phenotype and neuroimaging studies is supported by analysis of mutant mice and patient fibroblasts. We report a novel disease phenotype, show that the genetic cause is a homozygous mutation in the PTRH2 gene, and demonstrate functional effects in mouse and human tissues. Mutations in PTRH2 should be considered in patients with undiagnosed multisystem neurologic, endocrine, and pancreatic disease.

  6. Mutations in PTRH2 cause novel infantile-onset multisystem disease with intellectual disability, microcephaly, progressive ataxia, and muscle weakness

    Science.gov (United States)

    Hu, Hao; Matter, Michelle L; Issa-Jahns, Lina; Jijiwa, Mayumi; Kraemer, Nadine; Musante, Luciana; de la Vega, Michelle; Ninnemann, Olaf; Schindler, Detlev; Damatova, Natalia; Eirich, Katharina; Sifringer, Marco; Schrötter, Sandra; Eickholt, Britta J; van den Heuvel, Lambert; Casamina, Chanel; Stoltenburg-Didinger, Gisela; Ropers, Hans-Hilger; Wienker, Thomas F; Hübner, Christoph; Kaindl, Angela M

    2014-01-01

    Objective To identify the cause of a so-far unreported phenotype of infantile-onset multisystem neurologic, endocrine, and pancreatic disease (IMNEPD). Methods We characterized a consanguineous family of Yazidian-Turkish descent with IMNEPD. The two affected children suffer from intellectual disability, postnatal microcephaly, growth retardation, progressive ataxia, distal muscle weakness, peripheral demyelinating sensorimotor neuropathy, sensorineural deafness, exocrine pancreas insufficiency, hypothyroidism, and show signs of liver fibrosis. We performed whole-exome sequencing followed by bioinformatic analysis and Sanger sequencing on affected and unaffected family members. The effect of mutations in the candidate gene was studied in wild-type and mutant mice and in patient and control fibroblasts. Results In a consanguineous family with two individuals with IMNEPD, we identified a homozygous frameshift mutation in the previously not disease-associated peptidyl-tRNA hydrolase 2 (PTRH2) gene. PTRH2 encodes a primarily mitochondrial protein involved in integrin-mediated cell survival and apoptosis signaling. We show that PTRH2 is highly expressed in the developing brain and is a key determinant in maintaining cell survival during human tissue development. Moreover, we link PTRH2 to the mTOR pathway and thus the control of cell size. The pathology suggested by the human phenotype and neuroimaging studies is supported by analysis of mutant mice and patient fibroblasts. Interpretation We report a novel disease phenotype, show that the genetic cause is a homozygous mutation in the PTRH2 gene, and demonstrate functional effects in mouse and human tissues. Mutations in PTRH2 should be considered in patients with undiagnosed multisystem neurologic, endocrine, and pancreatic disease. PMID:25574476

  7. An Objective Functional Characterisation of Head Movement Impairment in Individuals with Neck Muscle Weakness Due to Amyotrophic Lateral Sclerosis.

    Directory of Open Access Journals (Sweden)

    Silvia Pancani

    Full Text Available Neck muscle weakness and head drop are well recognised in patients with Amyotrophic lateral sclerosis (ALS, but an objective characterisation of the consequent head movement impairment is lacking. The aim of this study was to quantitatively characterise head movements in ALS compared to aged matched controls.We evaluated two groups, one of thirteen patients with ALS and one of thirteen age-matched controls, during the execution of a series of controlled head movements, performed while wearing two inertial sensors attached on the forehead and sternum, respectively. We quantified the differences between the two groups from the sensor data using indices of velocity, smoothness and movement coupling (intended as a measure of undesired out of plane movements.Results confirmed a general limitation in the ability of the ALS patients to perform and control head movements. High inter-patient variability was observed due to a wide range of observed functional impairment levels. The ability to extend the head backward and flex it laterally were the most compromised, with significantly lower angular velocity (P 0.8, reduced smoothness and greater presence of coupled movements with respect to the controls. A significant reduction of angular velocity (P 0.8 in extension, axial rotation and lateral flexion was observed when patients were asked to perform the movements as fast as possible.This pilot study is the first study providing a functional objective quantification of head movements in ALS. Further work involving different body areas and correlation with existing methods of evaluating neuromuscular function, such as dynamometry and EMG, is needed to explore the use of this approach as a marker of disease progression in ALS.

  8. Early Life Exposure to Chronic Intermittent Hypoxia Primes Increased Susceptibility to Hypoxia-Induced Weakness in Rat Sternohyoid Muscle During Adulthood

    Directory of Open Access Journals (Sweden)

    Fiona B Mcdonald

    2016-03-01

    Full Text Available Intermittent hypoxia is a feature of apnea of prematurity, chronic lung disease and sleep apnea. Despite the clinical relevance, the long-term effects of hypoxic exposure in early life on respiratory control are not well defined. We recently reported that exposure to chronic intermittent hypoxia (CIH during postnatal development (pCIH causes upper airway muscle weakness in both sexes, which persists for several weeks. We sought to examine if there are persistent sex-dependent effects of pCIH on respiratory muscle function into adulthood and/or increased susceptibility to re-exposure to CIH in adulthood in animals previously exposed to CIH during postnatal development. We hypothesized that pCIH would cause long-lasting muscle impairment and increased susceptibility to subsequent hypoxia. Within 24 hours of delivery, pups and their respective dams were exposed to CIH: 90s of hypoxia reaching 5% O2 at nadir; once every 5 min, 8 hrs per day for 3 weeks. Sham groups were exposed to normoxia in parallel. Three groups were studied: sham; pCIH; and pCIH combined with adult CIH (p+aCIH, where a subset of the pCIH-exposed pups were re-exposed to the same CIH paradigm beginning at 13 weeks. Following gas exposures, sternohyoid and diaphragm muscle isometric contractile and endurance properties were examined ex vivo. There was no apparent lasting effect of pCIH on respiratory muscle function in adults. However, in both males and females, re-exposure to CIH in adulthood in pCIH-exposed animals caused sternohyoid (but not diaphragm weakness. Exposure to this paradigm of CIH in adulthood alone had no effect on muscle function. Persistent susceptibility in pCIH-exposed airway dilator muscle to subsequent hypoxic insult may have implications for the control of airway patency in adult humans exposed to intermittent hypoxic stress during early life.

  9. Early life exposure to chronic intermittent Hypoxia Primes Increased Susceptibility to Hypoxia-Induced Weakness in Rat Sternohyoid Muscle during adulthood.

    LENUS (Irish Health Repository)

    McDonald, Fiona B

    2016-03-01

    Intermittent hypoxia is a feature of apnea of prematurity (AOP), chronic lung disease, and sleep apnea. Despite the clinical relevance, the long-term effects of hypoxic exposure in early life on respiratory control are not well defined. We recently reported that exposure to chronic intermittent hypoxia (CIH) during postnatal development (pCIH) causes upper airway muscle weakness in both sexes, which persists for several weeks. We sought to examine if there are persistent sex-dependent effects of pCIH on respiratory muscle function into adulthood and\\/or increased susceptibility to re-exposure to CIH in adulthood in animals previously exposed to CIH during postnatal development. We hypothesized that pCIH would cause long-lasting muscle impairment and increased susceptibility to subsequent hypoxia. Within 24 h of delivery, pups and their respective dams were exposed to CIH: 90 s of hypoxia reaching 5% O2 at nadir; once every 5 min, 8 h per day for 3 weeks. Sham groups were exposed to normoxia in parallel. Three groups were studied: sham; pCIH; and pCIH combined with adult CIH (p+aCIH), where a subset of the pCIH-exposed pups were re-exposed to the same CIH paradigm beginning at 13 weeks. Following gas exposures, sternohyoid and diaphragm muscle isometric contractile and endurance properties were examined ex vivo. There was no apparent lasting effect of pCIH on respiratory muscle function in adults. However, in both males and females, re-exposure to CIH in adulthood in pCIH-exposed animals caused sternohyoid (but not diaphragm) weakness. Exposure to this paradigm of CIH in adulthood alone had no effect on muscle function. Persistent susceptibility in pCIH-exposed airway dilator muscle to subsequent hypoxic insult may have implications for the control of airway patency in adult humans exposed to intermittent hypoxic stress during early life.

  10. Fitness and mobility training in patients with Intensive Care Unit-acquired muscle weakness (FITonICU): study protocol for a randomised controlled trial

    OpenAIRE

    Mehrholz, Jan; Thomas, Simone; Burridge, Jane H.; Schmidt, Andr?; Scheffler, Bettina; Schellin, Ralph; R?ckriem, Stefan; Mei?ner, Daniel; Mehrholz, Katja; Sauter, Wolfgang; Bodechtel, Ulf; Elsner, Bernhard

    2016-01-01

    Background Critical illness myopathy (CIM) and polyneuropathy (CIP) are a common complication of critical illness. Both cause intensive-care-unit-acquired (ICU-acquired) muscle weakness (ICUAW) which increases morbidity and delays rehabilitation and recovery of activities of daily living such as walking ability. Focused physical rehabilitation of people with ICUAW is, therefore, of great importance at both an individual and a societal level. A recent systematic Cochrane review found no random...

  11. Facial-muscle weakness, speech disorders and dysphagia are common in patients with classic infantile Pompe disease treated with enzyme therapy.

    Science.gov (United States)

    van Gelder, C M; van Capelle, C I; Ebbink, B J; Moor-van Nugteren, I; van den Hout, J M P; Hakkesteegt, M M; van Doorn, P A; de Coo, I F M; Reuser, A J J; de Gier, H H W; van der Ploeg, A T

    2012-05-01

    Classic infantile Pompe disease is an inherited generalized glycogen storage disorder caused by deficiency of lysosomal acid α-glucosidase. If left untreated, patients die before one year of age. Although enzyme-replacement therapy (ERT) has significantly prolonged lifespan, it has also revealed new aspects of the disease. For up to 11 years, we investigated the frequency and consequences of facial-muscle weakness, speech disorders and dysphagia in long-term survivors. Sequential photographs were used to determine the timing and severity of facial-muscle weakness. Using standardized articulation tests and fibreoptic endoscopic evaluation of swallowing, we investigated speech and swallowing function in a subset of patients. This study included 11 patients with classic infantile Pompe disease. Median age at the start of ERT was 2.4 months (range 0.1-8.3 months), and median age at the end of the study was 4.3 years (range 7.7 months -12.2 years). All patients developed facial-muscle weakness before the age of 15 months. Speech was studied in four patients. Articulation was disordered, with hypernasal resonance and reduced speech intelligibility in all four. Swallowing function was studied in six patients, the most important findings being ineffective swallowing with residues of food (5/6), penetration or aspiration (3/6), and reduced pharyngeal and/or laryngeal sensibility (2/6). We conclude that facial-muscle weakness, speech disorders and dysphagia are common in long-term survivors receiving ERT for classic infantile Pompe disease. To improve speech and reduce the risk for aspiration, early treatment by a speech therapist and regular swallowing assessments are recommended.

  12. Development of Orally Bioavailable Therapeutics by the Chloroplast Expression System to Counter Muscle Degeneration, Weakness, and Fibrosis in DMD

    Science.gov (United States)

    2016-11-01

    strength in the diaphragm muscles . However, by 2 months of treatment the benefits were reduced back to untreated controls. We are continuing to analyze...Enzyme 2 Angiotensin-(1-7) Fibrosis Duchenne Muscular Dystrophy Mdx Skeletal muscle Chloroplast expression Oral bioavailability 3... treatment regimen to determine if any acute functional benefit was achieved. While no significant improvement was observed in EDL muscles , the diaphragms

  13. Mitochondrial Function in an In Vitro Model of Skeletal Muscle of Patients With Protracted Critical Illness and Intensive Care Unit-Acquired Weakness.

    Science.gov (United States)

    Jiroutková, Kateřina; Krajčová, Adéla; Žiak, Jakub; Fric, Michal; Gojda, Jan; Džupa, Valér; Kalous, Martin; Tůmová, Jana; Trnka, Jan; Duška, František

    2017-09-01

    Functional mitochondria in skeletal muscle of patients with protracted critical illness and intensive care unit-acquired weakness are depleted, but remaining mitochondria have increased functional capacities of respiratory complexes II and III. This can be an adaptation to relative abundancy of fatty acid over glucose caused by insulin resistance. We hypothesized that the capacity of muscle mitochondria to oxidize fatty acid is increased in protracted critical illness. We assessed fatty acid oxidation (FAO) and mitochondrial functional indices in vitro by using extracellular flux analysis in cultured myotubes obtained by isolating and culturing satellite cells from vastus lateralis muscle biopsy samples from patients with ICU-acquired weakness (n = 6) and age-matched healthy controls (n = 7). Bioenergetic measurements were performed at baseline and after 6 days of exposure to free fatty acids (FFAs). Mitochondrial density in myotubes from ICU patients was 69% of healthy controls ( P = .051). After adjustment to mitochondrial content, there were no differences in adenosine triphosphate (ATP) synthesis or the capacity and coupling of the respiratory chain. FAO capacity in ICU patients was 157% of FAO capacity in controls ( P = .015). In myotubes of ICU patients, unlike healthy controls, the exposure to FFA significantly ( P = .009) increased maximum respiratory chain capacity. In an in vitro model of skeletal muscle of patients with protracted critical illness, we have shown signs of adaptation to increased FAO. Even in the presence of glucose and insulin, elevation of FFAs in the extracellular environment increased maximal capacity of the respiratory chain.

  14. Muscle Weakness in the Empty and Full Can Tests Cannot Differentiate Rotator Cuff Tear from Cervical Spondylotic Amyotrophy: Pain Provocation is a Useful Finding.

    Science.gov (United States)

    Iwata, Eiichiro; Shigematsu, Hideki; Inoue, Kazuya; Egawa, Takuya; Sakamoto, Yoshihiro; Tanaka, Yasuhito

    2017-01-01

    Rotator cuff tears and cervical spondylotic amyotrophy (CSA) are often confused as the main symptom in those with difficulty in shoulder elevation. Empty and full can tests are frequently used for the clinical diagnosis of rotator cuff tears. The aim of the present study was to investigate whether the empty and full can test results can help differentiate rotator cuff tears from CSA. Twenty-seven consecutive patients with rotator cuff tears and 25 with CSA were enrolled. We prospectively performed empty and full can tests in patients with rotator cuff tears and CSA. The following signs were considered positive: (a) muscle weakness during the empty can test, (b) muscle weakness during the full can test, (c) pain provocation during the empty can test, and (d) pain provocation during the full can test. We calculated the sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of rotator cuff tears for each positive finding. The sensitivity and specificity of each index were as follows (sensitivity, specificity, PPV, NPV): (a) 77.8%, 0%, 45.7%, 0%; (b) 66.7%, 4.0%, 42.9%, 10.0%; (c) 88.9%, 96.0%, 96.0%, 88.9%; and (d) 74.1%, 96.0%, 95.2%, 77.4%. There were significant differences for each index. Muscle weakness during the empty and full can tests was not useful in differentiating rotator cuff tears from CSA because of low specificity and PPV. However, pain provocation was useful in differentiating these two conditions because of high specificity and PPV.

  15. Contribution of direct actions of the oxime HI-6 in reversing soman-induced muscle weakness in the rat diaphragm

    Energy Technology Data Exchange (ETDEWEB)

    Alder, M.; Maxwell, D.M.; Filbert, M.G.; Deshpande, S.S.

    1994-12-31

    The actions of the bispyridinium oxime HI-6 ((4-AMINOCARBONYL) PYRIDINO-methoxymethyl- 2-(HYDROXYIMINO)METHYL- pyridinium dichloride) were investigated in vitro on rat phrenic nerve-hemidiaphragm preparations. Isometric twitch and tetanic tensions were elicited at 37 deg C with supramaximal nerve stimulation at frequencies of 20 and 50 Hz. To approximate normal respiration patterns, trials consisting of 30 successive 0.55 5 trains were alternated with 1.25 5 rest periods. Under control conditions, the above stimulation pattern generated tensions that were well maintained at both frequencies. In contrast, a marked depression of muscle tension was observed in diaphragms removed from rats administered 339 ug/kg soman (3 LD50) and tested in vitro. Addition of HI-6, 4 h after soman exposure, led to a nearly complete recovery of muscle tension at 20 Hz. At 50 Hz, muscle tensions still declined especially when trains were elicited at 1.25 and 3 5 intervals. The recovery by HI-6 observed in this study appears to be mediated by mechanisms unrelated to acetylcholinesterase reactivation since no increase of enzymatic activity was detected and the effect was reversed by a brief washout in oxime-free physiological solution. The results suggest that the direct action of HI-6 may play a role in restoring soman-induced diaphragmatic failure but this effect would be significant primarily under low use conditions.

  16. Assessment of the Sheffield Support Snood, an innovative cervical orthosis designed for people affected by neck muscle weakness

    OpenAIRE

    Pancani, Silvia; Rowson, Jennifer; Tindale, Wendy; Heron, Nicola; Langley, Joe; McCarthy, Avril D.; Quinn, Ann; Reed, Heath; Stanton, Andrew; Shaw, Pamela J.; McDermott, Christopher J.; Mazzà, Claudia

    2016-01-01

    The aim of this study was to quantify the biomechanical features of the Sheffield Support Snood (SSS), a cervical orthosis specifically designed for patients with neck weakness. The orthosis is designed to be adaptable to a patient’s level of functional limitation using adjustable removable supports, which contribute support and restrict movement only in desired anatomical planes. \\ud Methods: The SSS was evaluated along with two commercially available orthoses, the Vista and Headmaster. The ...

  17. Human dignity and the profoundly disabled: a theological perspective.

    Science.gov (United States)

    Matthews, Pia

    2011-01-01

    One challenge to the concept of human dignity is that it is a rootless notion invoked simply to mask inequalities that inevitably exist between human beings. This privileging of humans is speciesist and its weak point is the profoundly disabled human being. This article argues that far from being a weak point, the profoundly disabled person is a source of strength and witness to the intrinsic dignity that all human beings have by virtue of being human. The disabled represent the reality of human existence that is both strong and fragile. Although human dignity can be understood philosophically its depth is rooted in Christian theological insights. The profoundly disabled occupy a privileged position and share in a theology of mission since they testify to the interdependence of every human being and human dependence on God to a myopic world that only values strength, autonomy and independence.

  18. Antagonism of botulinum toxin-induced muscle weakness by aminopyridines in rat phrenic nerve-hemidiaphragm preparations

    Energy Technology Data Exchange (ETDEWEB)

    Adler, M.; Scovill, J.; Deshpande, S.S.

    1993-05-13

    The effects of the potassium channel inhibitor and putative botulinum toxin antagonists 4-aminopyridine (4-AP) and 3,4-diaminopyridine (3,4-DAP) were investigated in vitro on the contractile and electrophysiological properties of rat diaphragm muscle. In the presence of 300 pM botulinum toxin A (BoTx A), twitches elicited by supramaximal nerve stimulation (0. 1 Hz) were reduced by over 80% in 3 hr. The time to block decreased with increases in temperature, toxin concentration and stimulation frequency. Addition of 4-AP or 3,4-DAP led to a prompt reversal of the BoTx A-induced depression of twitch tension. This reversal was concentration-dependent such that, in the presence of 1 mM 4-AP, reversal of the BoTx A-induced blockade was complete in 6.7 min. The beneficial effect of the APs were well maintained and persisted for up to 6 hr after addition. Application of 1 microns M neostigmine 1 hr after 3,4-DAP produced a further potentiation of twitch tensions, but this action lasted for < 5 min and led to the appearance of tetanic fade during repetitive stimulation. It is concluded that the APs are of benefit in antagonizing the muscle paralysis following exposure to botulinum toxin. Co-application of neostigmine, however, appears to confer no additional benefit.

  19. Intellectual disability, muscle weakness and characteristic face in three siblings: A newly described recessive syndrome mapping to 3p24.3-p25.3.

    Science.gov (United States)

    Kariminejad, Ariana; Nafissi, Shahriar; Nilipoor, Yalda; Tavasoli, Alireza; Van Veldhoven, Paul P; Bonnard, Carine; Ng, Yeng Ting; Majoie, Charles B; Reversade, Bruno; Hennekam, Raoul C

    2015-11-01

    We report on a sister and two brothers born to healthy Iranian parents with mild intellectual disability, progressive muscle weakness, and characteristic facies. including highly arched eyebrows, down-slanting palpebral fissures, prominent nasal bridge, prominent nose, columella extending below alae nasi, narrow mouth, narrow palate, and dental caries, and in one of them an inability to abduct the left eye. Electrophysiological studies showed signs of myopathy, and muscle biopsies demonstrated only nonspecific signs. Brain MRIs in two of the sibs showed leukencephalopathy with delayed myelination, frontal and parietal hyperintensities, and hippocampal atrophy in one. We have been unable to find a description of this association of features in literature. Based on the occurrence in siblings, no significant difference in phenotype between the brothers and sister, absence of manifestations in parents, and a likely consanguinity between parents we performed a homozygosity mapping. A single identical-by-descent bloc encompassing 57 genes located at 3p24.3-p25.3 was found to segregate within the family with this phenotype. © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.

  20. Do hip muscle weakness and dynamic knee valgus matter for the clinical evaluation and decision-making process in patients with patellofemoral pain?

    Science.gov (United States)

    Rabelo, Nayra Deise Dos Anjos; Lucareli, Paulo Roberto Garcia

    Patellofemoral pain is a very common musculoskeletal condition. In the last years, evidence regarding this disease increased exponentially. Although widely investigated, this problem still frustrates patients and clinicians for having an unfavorable prognosis. Some gaps still exist in the understanding and managing of patellofemoral pain. Numerous cross-sectional association studies show an association between gluteus muscular strength and dynamic knee valgus in patients with patellofemoral pain. In spite of this biological plausibility, many evidences challenge the direct relationship between these factors. Recent studies have concluded that women with patellofemoral pain show muscular weakness of the hip based on the cross-sectional studies, however prospective studies indicate that hip weakness cannot be considered a risk for development of patellofemoral pain. In addition, some clinical trials have demonstrated that strength training of the gluteal muscles promotes significant improvement in symptoms but not alter the kinematics of the patients with patellofemoral pain. These findings cast doubt on whether the cause of this condition is really being treated, whether all individuals suffering from patellofemoral pain present dynamic knee valgus or if this is a disturbance present in only a subgroup of patients and whether the strengthening of the hip musculature is an option to consider for prevention of patellofemoral pain. Certainly, more studies should be conducted to clarify the influence of mechanical patterns on this condition, but with the existing evidence so far, the importance given to these issues in the evaluation and clinical decision on treatment of these patients seems questionable. Therefore, this masterclass explores the understanding about patellofemoral pain, highlighting mainly the importance of muscular strength and dynamic knee valgus, as well as other possible factors that must be consider during the evaluation and the decision making in

  1. Lúpus Eritematoso Sistêmico com fraqueza muscular por Miastenia Gravis Systemic Lupus Erythematosus with muscle weakness due to Myasthenia Gravis

    Directory of Open Access Journals (Sweden)

    Sâmia Araújo de Sousa Studart

    2011-06-01

    Full Text Available O Lúpus Eritematoso Sistêmico (LES e a Miastenia Gravis (MG são doenças autoimunes cuja associação em um mesmo paciente é raramente descrita. Essas patologias compartilham algumas características como acometimento de mulheres jovens, positividade para anticorpos antinucleares, evolução em períodos de exacerbações e remissões. O presente relato de caso analisa as possíveis hipóteses diagnósticas para um quadro clínico de ptose palpebral e fraqueza muscular proximal em uma paciente portadora de lúpus recente que evoluiu com MG associada.Systemic lupus erythematosus (SLE and myasthenia gravis (MG are autoimmune diseases, whose association in the same patient is rarely reported. Both pathologies share the following characteristics: affect mainly young women; alternate exacerbation and remission periods; and have positive antinuclear antibody (ANA test. This case report assesses possible diagnostic hypotheses for the clinical findings of eyelid ptosis and proximal muscle weakness in a female patient recently diagnosed with SLE, who evolved with associated MG.

  2. Association between muscle atrophy/weakness and health care costs and utilization among patients receiving total knee replacement surgery: a retrospective cohort study

    Directory of Open Access Journals (Sweden)

    Chen SY

    2013-08-01

    Full Text Available Shih-Yin Chen,1 Ning Wu,1 Yuan-Chi Lee,1 Yang Zhao21Health Economics and Epidemiology, Evidera, Lexington, Massachusetts, 2Health Economics and Outcomes Research, Novartis Pharmaceuticals Corporation, East Hanover, New Jersey, USAPurpose: The aim of the study reported here was to examine health care resource utilization, costs, and risk of rehospitalization for total knee replacement (TKR patients with and without muscle atrophy/weakness (MAW.Patients and methods: Individuals aged 50–64 years with commercial insurance or 65+ years with Medicare Supplemental Insurance (Medicare who had a hospitalization for TKR between January 1, 2006 and September 30, 2009 were identified from a large US claims database. First hospitalization for TKR was defined as the index stay. All patients were classified into three cohorts according to when MAW was diagnosed relative to TKR: pre-MAW, post-MAW, and no MAW. The association between MAW and health care costs over the 12-month post-index period and the probability of rehospitalization were assessed via multivariate regressions.Results: The study sample included 53,696 Medicare and 46,058 commercial insurance TKR patients. Controlling for cross-cohort differences, both the pre- and post-MAW cohorts had significantly higher total health care costs (Medicare US$4,201 and US$9,404 higher, commercial insurance US$2,737 and US$6,640 higher, respectively than the no MAW cohort (all P < 0.05. The post-MAW cohort in both populations was also more likely to have any all-cause or replacement-related rehospitalization compared with the no MAW cohort.Conclusion: Among US patients undergoing TKR, those with MAW had higher health care utilization and costs than patients without MAW.Keywords: rehospitalization, resource utilization, Medicare, health insurance, USA

  3. Severe metabolic alkalosis, hypokalemia, and respiratory acidosis induced by the Chinese herbal medicine yokukansan in an elderly patient with muscle weakness and drowsiness.

    Science.gov (United States)

    Yamada, Shunsuke; Tokumoto, Masanori; Kansui, Yasuo; Wakisaka, Yoshinobu; Uchizono, Yuji; Tsuruya, Kazuhiko; Ooboshi, Hiroaki

    2013-05-01

    Yokukansan is a Chinese herbal medicine containing licorice that has been shown to alleviate the behavioral and psychological symptoms of Alzheimer's disease, with few adverse effects. Increasing numbers of patients with Alzheimer's disease in Japan are now being treated with this drug. However, yokukansan should be used with caution because of its potential to induce pseudoaldosteronism through the inhibition of 11-beta-hydroxysteroid dehydrogenase type 2, which metabolizes cortisol into cortisone. We present the case of an 88-year-old woman with a history of Alzheimer's disease who was transferred to our emergency department because of drowsiness, anorexia, and muscle weakness. Her blood pressure was 168/90 mmHg. Laboratory data showed serum potassium of 1.9 mmol/l, metabolic alkalosis (pH 7.54; HCO 3 - , 50.5 mmol/l; chloride, 81 mmol/l; sodium, 140 mmol/l), and respiratory disorders (pCO 2 , 60.5 mmHg; pO 2 , 63.8 mmHg). Plasma renin activity and aldosterone concentration were suppressed, and urinary potassium excretion was 22 mmol/l (calculated transtubular potassium gradient 12.9). An electrocardiogram showed flat T-waves and U-waves with ventricular premature contractions. Echocardiography denied volume depletion. Medical interview disclosed that she had been treated with a Chinese herbal medicine (yokukansan) containing licorice. The final diagnosis was pseudoaldosteronism and respiratory acidosis induced by licorice. Hypokalemia, metabolic alkalosis, and respiratory acidosis all subsided shortly after the discontinuation of yokukansan and initiation of intravenous potassium replacement. This case highlights the need for nephrologists to consider the possible involvement of Chinese herbal medicines, including yokukansan, when they encounter hypokalemia in elderly patients.

  4. Cancer cachexia decreases specific force and accelerates fatigue in limb muscle

    Energy Technology Data Exchange (ETDEWEB)

    Roberts, B. M. [1225 Center Drive, HPNP Building Room 1142, Department of Physical Therapy, University of Florida, Gainesville, FL 32610 (United States); Frye, G. S.; Ahn, B.; Ferreira, L. F. [1864 Stadium Road, Department of Applied Physiology and Kinesiology, University of Florida, Gainesville, FL 32610 (United States); Judge, A.R., E-mail: arjudge@phhp.ufl.edu [1225 Center Drive, HPNP Building Room 1142, Department of Physical Therapy, University of Florida, Gainesville, FL 32610 (United States)

    2013-06-07

    soleus is also important for normal locomotion, we further performed a fatigue trial in the soleus and found that the decrease in relative force was greater and more rapid in solei from C-26 mice compared to controls. These data demonstrate that severe cancer cachexia causes profound muscle weakness that is not entirely explained by the muscle atrophy. In addition, cancer cachexia decreases the fatigue resistance of the soleus muscle, a postural muscle typically resistant to fatigue. Thus, specifically targeting contractile dysfunction represents an additional means to counter muscle weakness in cancer cachexia, in addition to targeting the prevention of muscle atrophy.

  5. Cancer cachexia decreases specific force and accelerates fatigue in limb muscle

    International Nuclear Information System (INIS)

    Roberts, B.M.; Frye, G.S.; Ahn, B.; Ferreira, L.F.; Judge, A.R.

    2013-01-01

    soleus is also important for normal locomotion, we further performed a fatigue trial in the soleus and found that the decrease in relative force was greater and more rapid in solei from C-26 mice compared to controls. These data demonstrate that severe cancer cachexia causes profound muscle weakness that is not entirely explained by the muscle atrophy. In addition, cancer cachexia decreases the fatigue resistance of the soleus muscle, a postural muscle typically resistant to fatigue. Thus, specifically targeting contractile dysfunction represents an additional means to counter muscle weakness in cancer cachexia, in addition to targeting the prevention of muscle atrophy

  6. Novel excitation-contraction coupling related genes reveal aspects of muscle weakness beyond atrophy—new hopes for treatment of musculoskeletal diseases

    Science.gov (United States)

    Manring, Heather; Abreu, Eduardo; Brotto, Leticia; Weisleder, Noah; Brotto, Marco

    2013-01-01

    Research over the last decade strengthened the understanding that skeletal muscles are not only the major tissue in the body from a volume point of view but also function as a master regulator contributing to optimal organismal health. These new contributions to the available body of knowledge triggered great interest in the roles of skeletal muscle beyond contraction. The World Health Organization, through its Global Burden of Disease (GBD) report, recently raised further awareness about the key importance of skeletal muscles as the GDB reported musculoskeletal (MSK) diseases have become the second greatest cause of disability, with more than 1.7 billion people in the globe affected by a diversity of MSK conditions. Besides their role in MSK disorders, skeletal muscles are also seen as principal metabolic organs with essential contributions to metabolic disorders, especially those linked to physical inactivity. In this review, we have focused on the unique function of new genes/proteins (i.e., MTMR14, MG29, sarcalumenin, KLF15) that during the last few years have helped provide novel insights about muscle function in health and disease, muscle fatigue, muscle metabolism, and muscle aging. Next, we provide an in depth discussion of how these genes/proteins converge into a common function of acting as regulators of intracellular calcium homeostasis. A clear link between dysfunctional calcium homeostasis is established and the special role of store-operated calcium entry is analyzed. The new knowledge that has been generated by the understanding of the roles of previously unknown modulatory genes of the skeletal muscle excitation-contraction coupling (ECC) process brings exciting new possibilities for treatment of MSK diseases, muscle regeneration, and skeletal muscle tissue engineering. The next decade of skeletal muscle and MSK research is bound to bring to fruition applied knowledge that will hopefully offset the current heavy and sad burden of MSK diseases on the

  7. Weak currents

    International Nuclear Information System (INIS)

    Leite Lopes, J.

    1976-01-01

    A survey of the fundamental ideas on weak currents such as CVC and PCAC and a presentation of the Cabibbo current and the neutral weak currents according to the Salam-Weinberg model and the Glashow-Iliopoulos-Miami model are given [fr

  8. Weak decays

    International Nuclear Information System (INIS)

    Wojcicki, S.

    1978-11-01

    Lectures are given on weak decays from a phenomenological point of view, emphasizing new results and ideas and the relation of recent results to the new standard theoretical model. The general framework within which the weak decay is viewed and relevant fundamental questions, weak decays of noncharmed hadrons, decays of muons and the tau, and the decays of charmed particles are covered. Limitation is made to the discussion of those topics that either have received recent experimental attention or are relevant to the new physics. (JFP) 178 references

  9. Weak interactions

    International Nuclear Information System (INIS)

    Ogava, S.; Savada, S.; Nakagava, M.

    1983-01-01

    The problem of the use of weak interaction laws to study models of elementary particles is discussed. The most typical examples of weak interaction is beta-decay of nucleons and muons. Beta-interaction is presented by quark currents in the form of universal interaction of the V-A type. Universality of weak interactions is well confirmed using as examples e- and μ-channels of pion decay. Hypothesis on partial preservation of axial current is applicable to the analysis of processes with pion participation. In the framework of the model with four flavours lepton decays of hadrons are considered. Weak interaction without lepton participation are also considered. Properties of neutral currents are described briefly

  10. Weak interactions

    International Nuclear Information System (INIS)

    Chanda, R.

    1981-01-01

    The theoretical and experimental evidences to form a basis for Lagrangian Quantum field theory for Weak Interactions are discussed. In this context, gauge invariance aspects of such interactions are showed. (L.C.) [pt

  11. Achieving profound anesthesia using the intraosseous technique.

    Science.gov (United States)

    Coury, K A

    1997-10-01

    The intraosseous technique has been described as a useful adjunct to primary anesthetic administration. It has several advantages (Table 3) over other supplemental techniques in that it is relatively simple to implement into routine practice, it affords fast, predictable results, and it is relatively painless. The technique has been shown to be very successful in achieving profound pulpal anesthesia when administered as a supplement to the inferior alveolar nerve block and is effective in achieving profound anesthesia in irreversibly inflamed teeth, especially mandibular molars.

  12. Acute muscular weakness in children

    Directory of Open Access Journals (Sweden)

    Ricardo Pablo Javier Erazo Torricelli

    Full Text Available ABSTRACT Acute muscle weakness in children is a pediatric emergency. During the diagnostic approach, it is crucial to obtain a detailed case history, including: onset of weakness, history of associated febrile states, ingestion of toxic substances/toxins, immunizations, and family history. Neurological examination must be meticulous as well. In this review, we describe the most common diseases related to acute muscle weakness, grouped into the site of origin (from the upper motor neuron to the motor unit. Early detection of hyperCKemia may lead to a myositis diagnosis, and hypokalemia points to the diagnosis of periodic paralysis. Ophthalmoparesis, ptosis and bulbar signs are suggestive of myasthenia gravis or botulism. Distal weakness and hyporeflexia are clinical features of Guillain-Barré syndrome, the most frequent cause of acute muscle weakness. If all studies are normal, a psychogenic cause should be considered. Finding the etiology of acute muscle weakness is essential to execute treatment in a timely manner, improving the prognosis of affected children.

  13. Weak interactions

    International Nuclear Information System (INIS)

    Bjorken, J.D.

    1978-01-01

    Weak interactions are studied from a phenomenological point of view, by using a minimal number of theoretical hypotheses. Charged-current phenomenology, and then neutral-current phenomenology are discussed. This all is described in terms of a global SU(2) symmetry plus an electromagnetic correction. The intermediate-boson hypothesis is introduced and lower bounds on the range of the weak force are inferred. This phenomenology does not yet reconstruct all the predictions of the conventional SU(2)xU(1) gauge theory. To do that requires an additional assumption of restoration of SU(2) symmetry at asymptotic energies

  14. Signal processing for the profoundly deaf.

    Science.gov (United States)

    Boothyroyd, A

    1990-01-01

    Profound deafness, defined here as a hearing loss in excess of 90 dB, is characterized by high thresholds, reduced hearing range in the intensity and frequency domains, and poor resolution in the frequency and time domains. The high thresholds call for hearing aids with unusually high gains or remote microphones that can be placed close to the signal source. The former option creates acoustic feedback problems for which digital signal processing may yet offer solutions. The latter option calls for carrier wave technology that is already available. The reduced frequency and intensity ranges would appear to call for frequency and/or amplitude compression. It might also be argued, however, that any attempts to compress the acoustic signal into the limited hearing range of the profoundly deaf will be counterproductive because of poor frequency and time resolution, especially when the signal is present in noise. In experiments with a 2-channel compression system, only 1 of 9 subjects showed an improvement of perception with the introduction of fast-release (20 ms) compression. The other 8 experienced no benefit or a slight deterioration of performance. These results support the concept of providing the profoundly deaf with simpler, rather than more complex, patterns, perhaps through the use of feature extraction hearing aids. Data from users of cochlear implants already employing feature extraction techniques also support this concept.

  15. Profound bradycardia associated with NIV removal.

    Science.gov (United States)

    Echevarria, C; Bourke, S C; Gibson, G J

    2012-01-01

    A patient with lower-limb onset ALS presented with a one-month history of vasovagal episodes and a one-week history of cough productive of green sputum and lethargy. She was drowsy and in acute on chronic type-two respiratory failure. She responded to non-invasive ventilation, however she suffered recurrent episodes of profound bradycardia on removal of the mask, which gradually resolved over ten days. We have reviewed the literature and offer a potential explanation for these events.

  16. Asystole Following Profound Vagal Stimulation During Hepatectomy

    Directory of Open Access Journals (Sweden)

    Preeta John

    2008-01-01

    Full Text Available Asystole in a non laparoscopic upper abdominal surgery following intense vagal stimulation is a rare event. This case report highlights the need for awareness of such a complication when a thoracic epidural anaesthetic has been given in addition to a general anaesthetic for an upper abdominal procedure. A combined thoracic epidural and general anaesthetic was given. The anterior abdominal wall was retracted forty minutes after administration of the epidural bolus. This maneuver resulted in a profound vagal response with bradycardia and asystole. The patient was resuscitated successfully with a cardiac massage, atropine and adrenaline and the surgery was resumed. Surgery lasted eleven hours and was uneventful.

  17. Weak relativity

    CERN Document Server

    Selleri, Franco

    2015-01-01

    Weak Relativity is an equivalent theory to Special Relativity according to Reichenbach’s definition, where the parameter epsilon equals to 0. It formulates a Neo-Lorentzian approach by replacing the Lorentz transformations with a new set named “Inertial Transformations”, thus explaining the Sagnac effect, the twin paradox and the trip from the future to the past in an easy and elegant way. The cosmic microwave background is suggested as a possible privileged reference system. Most importantly, being a theory based on experimental proofs, rather than mutual consensus, it offers a physical description of reality independent of the human observation.

  18. Contribution of oxidative stress to pathology in diaphragm and limb muscles with Duchenne muscular dystrophy.

    Science.gov (United States)

    Kim, Jong-Hee; Kwak, Hyo-Bum; Thompson, LaDora V; Lawler, John M

    2013-02-01

    Duchenne muscular dystrophy (DMD) is a degenerative skeletal muscle disease that makes walking and breathing difficult. DMD is caused by an X-linked (Xp21) mutation in the dystrophin gene. Dystrophin is a scaffolding protein located in the sarcolemmal cytoskeleton, important in maintaining structural integrity and regulating muscle cell (muscle fiber) growth and repair. Dystrophin deficiency in mouse models (e.g., mdx mouse) destabilizes the interface between muscle fibers and the extracellular matrix, resulting in profound damage, inflammation, and weakness in diaphragm and limb muscles. While the link between dystrophin deficiency with inflammation and pathology is multi-factorial, elevated oxidative stress has been proposed as a central mediator. Unfortunately, the use of non-specific antioxidant scavengers in mouse and human studies has led to inconsistent results, obscuring our understanding of the importance of redox signaling in pathology of muscular dystrophy. However, recent studies with more mechanistic approaches in mdx mice suggest that NAD(P)H oxidase and nuclear factor-kappaB are important in amplifying dystrophin-deficient muscle pathology. Therefore, more targeted antioxidant therapeutics may ameliorate damage and weakness in human population, thus promoting better muscle function and quality of life. This review will focus upon the pathobiology of dystrophin deficiency in diaphragm and limb muscle primarily in mouse models, with a rationale for development of targeted therapeutic antioxidants in DMD patients.

  19. Persistent Thalamic Sound Processing Despite Profound Cochlear Denervation

    Directory of Open Access Journals (Sweden)

    Anna R. Chambers

    2016-08-01

    Full Text Available Neurons at higher stages of sensory processing can partially compensate for a sudden drop in input from the periphery through a homeostatic plasticity process that increases the gain on weak afferent inputs. Even after a profound unilateral auditory neuropathy where > 95% of synapses between auditory nerve fibers and inner hair cells have been eliminated with ouabain, central gain can restore the cortical processing and perceptual detection of basic sounds delivered to the denervated ear. In this model of profound auditory neuropathy, cortical processing and perception recover despite the absence of an auditory brainstem response (ABR or brainstem acoustic reflexes, and only a partial recovery of sound processing at the level of the inferior colliculus (IC, an auditory midbrain nucleus. In this study, we induced a profound cochlear neuropathy with ouabain and asked whether central gain enabled a compensatory plasticity in the auditory thalamus comparable to the full recovery of function previously observed in the auditory cortex (ACtx, the partial recovery observed in the IC, or something different entirely. Unilateral ouabain treatment in adult mice effectively eliminated the ABR, yet robust sound-evoked activity persisted in a minority of units recorded from the contralateral medial geniculate body (MGB of awake mice. Sound-driven MGB units could decode moderate and high-intensity sounds with accuracies comparable to sham-treated control mice, but low-intensity classification was near chance. Pure tone receptive fields and synchronization to broadband pulse trains also persisted, albeit with significantly reduced quality and precision, respectively. MGB decoding of temporally modulated pulse trains and speech tokens were both greatly impaired in ouabain-treated mice. Taken together, the absence of an ABR belied a persistent auditory processing at the level of the MGB that was likely enabled through increased central gain. Compensatory

  20. Staffs' documentation of participation for adults with profound intellectual disability or profound intellectual and multiple disabilities.

    Science.gov (United States)

    Talman, Lena; Gustafsson, Christine; Stier, Jonas; Wilder, Jenny

    2017-06-21

    This study investigated what areas of International Classification of Functioning, Disability and Health were documented in implementation plans for adults with profound intellectual disability or profound intellectual and multiple disabilities with focus on participation. A document analysis of 17 implementation plans was performed and International Classification of Functioning, Disability and Health was used as an analytic tool. One hundred and sixty-three different codes were identified, especially in the components Activities and participation and Environmental factors. Participation was most frequently coded in the chapters Community, social and civic life and Self-care. Overall, the results showed that focus in the implementation plans concerned Self-care and Community, social and civic life. The other life areas in Activities and participation were seldom, or not at all, documented. A deeper focus on participation in the implementation plans and all life areas in the component Activities and participation is needed. It is important that the documentation clearly shows what the adult wants, wishes, and likes in everyday life. It is also important to ensure that the job description for staff contains both life areas and individual preferences so that staff have the possibility to work to fulfill social and individual participation for the target group. Implications for rehabilitation There is a need for functioning working models to increase participation significantly for adults with profound intellectual disability or profound intellectual and multiple disabilities. For these adults, participation is achieved through the assistance of others and support and services carried out must be documented in an implementation plan. The International Classification of Functioning, Disability and Health can be used to support staff and ensure that information about the most important factors in an individual's functioning in their environment is not omitted in

  1. A young woman with weakness of the legs

    African Journals Online (AJOL)

    A previously well 22-year-old woman presented with progressive weakness of her legs and urinary incontinence over 7 days. Clinically she was healthy, with no skin rashes. On neurological examination she had profound bilateral weakness of the lower limbs, hypertonia, hyperreflexia, a positive Babinski sign and a T6 ...

  2. Feasibility, Test-Retest Reliability, and Interrater Reliability of the Modified Ashworth Scale and Modified Tardieu Scale in Persons with Profound Intellectual and Multiple Disabilities

    Science.gov (United States)

    Waninge, A.; Rook, R. A.; Dijkhuizen, A.; Gielen, E.; van der Schans, C. P.

    2011-01-01

    Caregivers of persons with profound intellectual and multiple disabilities (PIMD) often describe the quality of the daily movements of these persons in terms of flexibility or stiffness. Objective outcome measures for flexibility and stiffness are muscle tone or level of spasticity. Two instruments used to grade muscle tone and spasticity are the…

  3. Reconstructing weak values without weak measurements

    International Nuclear Information System (INIS)

    Johansen, Lars M.

    2007-01-01

    I propose a scheme for reconstructing the weak value of an observable without the need for weak measurements. The post-selection in weak measurements is replaced by an initial projector measurement. The observable can be measured using any form of interaction, including projective measurements. The reconstruction is effected by measuring the change in the expectation value of the observable due to the projector measurement. The weak value may take nonclassical values if the projector measurement disturbs the expectation value of the observable

  4. Nuclear Positioning in Muscle Development and Disease

    OpenAIRE

    Eric eFolker; Mary eBaylies

    2013-01-01

    Muscle disease as a group is characterized by muscle weakness, muscle loss, and impaired muscle function. Although the phenotype is the same, the underlying cellular pathologies, and the molecular causes of these pathologies, are diverse. One common feature of many muscle disorders is the mispositioning of myonuclei. In unaffected individuals myonuclei are spaced throughout the periphery of the muscle fiber such that the distance between nuclei is maximized. However, in diseased muscles, th...

  5. Peripheral facial weakness (Bell's palsy).

    Science.gov (United States)

    Basić-Kes, Vanja; Dobrota, Vesna Dermanović; Cesarik, Marijan; Matovina, Lucija Zadro; Madzar, Zrinko; Zavoreo, Iris; Demarin, Vida

    2013-06-01

    Peripheral facial weakness is a facial nerve damage that results in muscle weakness on one side of the face. It may be idiopathic (Bell's palsy) or may have a detectable cause. Almost 80% of peripheral facial weakness cases are primary and the rest of them are secondary. The most frequent causes of secondary peripheral facial weakness are systemic viral infections, trauma, surgery, diabetes, local infections, tumor, immune disorders, drugs, degenerative diseases of the central nervous system, etc. The diagnosis relies upon the presence of typical signs and symptoms, blood chemistry tests, cerebrospinal fluid investigations, nerve conduction studies and neuroimaging methods (cerebral MRI, x-ray of the skull and mastoid). Treatment of secondary peripheral facial weakness is based on therapy for the underlying disorder, unlike the treatment of Bell's palsy that is controversial due to the lack of large, randomized, controlled, prospective studies. There are some indications that steroids or antiviral agents are beneficial but there are also studies that show no beneficial effect. Additional treatments include eye protection, physiotherapy, acupuncture, botulinum toxin, or surgery. Bell's palsy has a benign prognosis with complete recovery in about 80% of patients, 15% experience some mode of permanent nerve damage and severe consequences remain in 5% of patients.

  6. Proximal processes of children with profound multiple disabilities

    OpenAIRE

    Wilder, Jenny

    2008-01-01

    In this thesis four empirical studies dealt with children with profound multiple disabilities and their parents with regard to: (a) how parents perceived interaction with their children (b) how observed child/parent interaction was linked to behavior style of the children as perceived by the parents (c) how parents of children with profound multiple disabilities perceived child/parent interaction and behavior style of their children in comparison to parents to children without disabilities ma...

  7. Muscle strength in myasthenia gravis

    DEFF Research Database (Denmark)

    Cejvanovic, S; Vissing, J

    2014-01-01

    OBJECTIVE: Myasthenia gravis (MG) is characterized by fatigue and fluctuating muscle weakness as a result of impaired neuromuscular transmission (NMT). Although MG is a prototypic fatiguing disorder, little is known about how the condition affects fixed weakness, and if present, whether weakness...

  8. Static and Dynamic Balance in Congenital Severe to Profound Hearing-Impaired Children

    Directory of Open Access Journals (Sweden)

    Farideh HajiHeydari

    2011-09-01

    Full Text Available Background and Aim: Research conducted since the early 1900s has consistently identified differences between deaf and hearing children on performance of a wide variety of motor tasks, most notably balance. Our study was performed to test static and dynamic balance skills in congenital severe to profound hearing impaired children in comparison with normal age-matched children.Methods: This cross-sectional study was conducted on 30 severe to profound hearing impaired and 40 normal children with age 6 to 10 years old. Bruininks-Oseretsky test of motor proficiency 2, balance subset with 9 parts was used for evaluation of balance skills.Results: Hearing-impaired children showed 16.7 to 100% fail results in 7 parts of the balance subset. In normal children fail result was revealed just in 3 parts of the balance subset from 2.5 to 57.5%, and differences between two groups were significant (p<0.0001. There was a significant difference between two groups in two static balance skills of standing on one leg on a line and standing on one leg on a balance beam with eyes closed (p<0.0001.conclusion: It seems that development of static balance skills are longer than dynamic ones. Because severe to profound hearing-impaired children showed more weakness than normal children in both static and dynamic balance abilities, functional tests of balance proficiency can help to identify balance disorders in these children.

  9. Dietary nitrate increases tetanic [Ca2+]i and contractile force in mouse fast-twitch muscle.

    Science.gov (United States)

    Hernández, Andrés; Schiffer, Tomas A; Ivarsson, Niklas; Cheng, Arthur J; Bruton, Joseph D; Lundberg, Jon O; Weitzberg, Eddie; Westerblad, Håkan

    2012-08-01

    Dietary inorganic nitrate has profound effects on health and physiological responses to exercise. Here, we examined if nitrate, in doses readily achievable via a normal diet, could improve Ca(2+) handling and contractile function using fast- and slow-twitch skeletal muscles from C57bl/6 male mice given 1 mm sodium nitrate in water for 7 days. Age matched controls were provided water without added nitrate. In fast-twitch muscle fibres dissected from nitrate treated mice, myoplasmic free [Ca(2+)] was significantly greater than in Control fibres at stimulation frequencies from 20 to 150 Hz, which resulted in a major increase in contractile force at ≤ 50 Hz. At 100 Hz stimulation, the rate of force development was ∼35% faster in the nitrate group. These changes in nitrate treated mice were accompanied by increased expression of the Ca(2+) handling proteins calsequestrin 1 and the dihydropyridine receptor. No changes in force or calsequestrin 1 and dihydropyridine receptor expression were measured in slow-twitch muscles. In conclusion, these results show a striking effect of nitrate supplementation on intracellular Ca(2+) handling in fast-twitch muscle resulting in increased force production. A new mechanism is revealed by which nitrate can exert effects on muscle function with applications to performance and a potential therapeutic role in conditions with muscle weakness.

  10. Weakly clopen functions

    International Nuclear Information System (INIS)

    Son, Mi Jung; Park, Jin Han; Lim, Ki Moon

    2007-01-01

    We introduce a new class of functions called weakly clopen function which includes the class of almost clopen functions due to Ekici [Ekici E. Generalization of perfectly continuous, regular set-connected and clopen functions. Acta Math Hungar 2005;107:193-206] and is included in the class of weakly continuous functions due to Levine [Levine N. A decomposition of continuity in topological spaces. Am Math Mon 1961;68:44-6]. Some characterizations and several properties concerning weakly clopenness are obtained. Furthermore, relationships among weak clopenness, almost clopenness, clopenness and weak continuity are investigated

  11. Weak value controversy

    Science.gov (United States)

    Vaidman, L.

    2017-10-01

    Recent controversy regarding the meaning and usefulness of weak values is reviewed. It is argued that in spite of recent statistical arguments by Ferrie and Combes, experiments with anomalous weak values provide useful amplification techniques for precision measurements of small effects in many realistic situations. The statistical nature of weak values is questioned. Although measuring weak values requires an ensemble, it is argued that the weak value, similarly to an eigenvalue, is a property of a single pre- and post-selected quantum system. This article is part of the themed issue `Second quantum revolution: foundational questions'.

  12. Music Training for Severely and Profoundly Retarded Individuals.

    Science.gov (United States)

    Kesler, Buford; Richmond, Bert O.

    Investigated were the effects of sex, ability and training method on the musical instrument playing ability of 16 institutionalized severely and profoundly retarded persons ages 7 to 20 years. Ss were randomly assigned to one of four treatment groups, and the time required to reach criterion playing a familiar tune was recorded. Data indicated…

  13. Acceleration of Object Permanence with Severely and Profoundly Retarded Children.

    Science.gov (United States)

    Kahn, James V.

    Examined was the effectiveness of training four severely and profoundly retarded children (3-6 years old) to improve their level of functioning on a measure of object permanence and to demonstrate generalization to other areas of sensorimotor intelligence. Ss were given a pretest and posttest on the I. Uzgiris and J. Hunt instrument which consists…

  14. Factors which Motivate Job Acceptance and Profoundly Mentally Retarded Children.

    Science.gov (United States)

    Marozas, Donald S.; May, Deborah C.

    1980-01-01

    The study involving 360 Pennsylvania teachers was designed to identify factors which motivate job acceptance among teachers of severely and profoundly mentally retarded children. The responses of 235 teachers indicated that challenge and practicum experiences were the two most prevalent motivational factors underlying job acceptance. (Author)

  15. Pre-Language Activities for the Profoundly Mentally Retarded.

    Science.gov (United States)

    Poole, Marilyn R.; And Others

    Provided are sample lesson plans for a program to develop pre-language skills in profoundly retarded children and adults. Characteristic of the suggested activities is the stimulation of all sensory channels through structured infant-like play activities in five general areas: oral stimulation, sensory arousal, motor stimulation, vocal play, and…

  16. A Grounded Theory of Effective Reading by Profoundly Deaf Adults

    Science.gov (United States)

    Silvestri, Julia; Wang, Ye

    2018-01-01

    The purpose of the study was to uncover and describe psycholinguistic and sociocognitive factors facilitating effective reading by signing adults who are profoundly deaf and do not use hearing technology. The sample comprised four groups, each consisting of 15 adults, for a total of 60 participants. The four groups were "deaf…

  17. Deming's System of Profound Knowledge: An Overview for International Educators.

    Science.gov (United States)

    Evans, Thomas J.

    W. Edwards Deming called for the transformation to a new style of organizational management based on greater cooperation between managers and employees. This transformation could be achieved by introducing "profound knowledge" into the system. This paper is a presentation outline that was used to introduce the basics of Deming's theory…

  18. Feasibility, test-retest reliability, and interrater reliability of the Modified Ashworth Scale and Modified Tardieu Scale in persons with profound intellectual and multiple disabilities

    NARCIS (Netherlands)

    Waninge, A.; Rook, R.A.; Dijkhuizen, A.; Gielen, E.; van der Schans, C.P.

    2011-01-01

    Caregivers of persons with profound intellectual and multiple disabilities (PIMD) often describe the quality of the daily movements of these persons in terms of flexibility or stiffness. Objective outcome measures for flexibility and stiffness are muscle tone or level of spasticity. Two instruments

  19. Proximal Limb Weakness Reverting After CSF Diversion In Intracranial Hypertension

    Directory of Open Access Journals (Sweden)

    Sinha S

    2005-01-01

    Full Text Available We report about two young girls who developed progressive visual failure secondary to increased intracranial pressure and had significant proximal muscle weakness of limbs. Patients with elevated intracranial pressure (ICP may present with "false localizing signs", besides having headache, vomiting and papilledema. Radicular pain as a manifestation of raised ICP is rare and motor weakness attributable to polyradiculopathy is exceptional. Two patients with increased intracranial pressure without lateralizing signs′ had singnificant muscle weakness. Clinical evaluation and laboratory tests did not disclose any other cause for weakness. Following theco-peritoneal shunt, in both patients, there was variable recovery of vision but the proximal weakness and symptoms of elevated ICP improved rapidly. Recognition of this uncommon manifestation of raised ICP may obviate the need for unnecessary investigation and reduce morbidity due to weakness by CSF diversion procedure.

  20. Outcomes of laryngohyoid suspension techniques in an ovine model of profound oropharyngeal dysphagia.

    Science.gov (United States)

    Johnson, Christopher M; Venkatesan, Naren N; Siddiqui, M Tausif; Cates, Daniel J; Kuhn, Maggie A; Postma, Gregory M; Belafsky, Peter C

    2017-12-01

    To evaluate the efficacy of various techniques of laryngohyoid suspension in the elimination of aspiration utilizing a cadaveric ovine model of profound oropharyngeal dysphagia. Animal study. The head and neck of a Dorper cross ewe was placed in the lateral fluoroscopic view. Five conditions were tested: baseline, thyroid cartilage to hyoid approximation (THA), thyroid cartilage to hyoid to mandible (laryngohyoid) suspension (LHS), LHS with cricopharyngeus muscle myotomy (LHS-CPM), and cricopharyngeus muscle myotomy (CPM) alone. Five 20-mL trials of barium sulfate were delivered into the oropharynx under fluoroscopy for each condition. Outcome measures included the penetration aspiration scale (PAS) and the National Institutes of Health (NIH) Swallow Safety Scale (NIH-SSS). Median baseline PAS and NIH-SSS scores were 8 and 6, respectively, indicating severe impairment. THA scores were not improved from baseline. LHS alone reduced the PAS to 1 (P = .025) and NIH-SSS to 2 (P = .025) from baseline. LHS-CPM reduced the PAS to 1 (P = .025) and NIH-SSS to 0 (P = .025) from baseline. CPM alone did not improve scores. LHS-CPM displayed improved NIH-SSS over LHS alone (P = .003). This cadaveric model represents end-stage profound oropharyngeal dysphagia such as what could result from severe neurological insult. CPM alone failed to improve fluoroscopic outcomes in this model. Thyrohyoid approximation also failed to improve outcomes. LHS significantly improved both PAS and NIH-SSS. The addition of CPM to LHS resulted in improvement over suspension alone. NA. Laryngoscope, 127:E422-E427, 2017. © 2017 The American Laryngological, Rhinological and Otological Society, Inc.

  1. Do colors exist? and other profound physics questions

    CERN Document Server

    Cottrell, Seth Stannard

    2018-01-01

    Why do polished stones look wet? How does the Twin Paradox work? Why are orbits ellipses? How can we be sure that pi never repeats? How does a quantum computer break encryption? Discover the answers to these, and other profound physics questions! This fascinating book presents a collection of articles based on conversations and correspondences between the author and complete strangers about physics and math. The author, a researcher in mathematical physics, responds to dozens of questions posed by inquiring minds from all over the world, ranging from the everyday to the profound. Rather than unnecessarily complex explanations mired in mysterious terminology and symbols, the reader is presented with the reasoning, experiments, and mathematics in a casual, conversational, and often comical style. Neither over-simplified nor over-technical, the lucid and entertaining writing will guide the reader from the each innocent question to a better understanding of the weird and beautiful universe around us. Advance prai...

  2. Second class weak currents

    International Nuclear Information System (INIS)

    Delorme, J.

    1978-01-01

    The definition and general properties of weak second class currents are recalled and various detection possibilities briefly reviewed. It is shown that the existing data on nuclear beta decay can be consistently analysed in terms of a phenomenological model. Their implication on the fundamental structure of weak interactions is discussed [fr

  3. Weak C* Hopf Symmetry

    OpenAIRE

    Rehren, K. -H.

    1996-01-01

    Weak C* Hopf algebras can act as global symmetries in low-dimensional quantum field theories, when braid group statistics prevents group symmetries. Possibilities to construct field algebras with weak C* Hopf symmetry from a given theory of local observables are discussed.

  4. Bagging Weak Predictors

    DEFF Research Database (Denmark)

    Lukas, Manuel; Hillebrand, Eric

    Relations between economic variables can often not be exploited for forecasting, suggesting that predictors are weak in the sense that estimation uncertainty is larger than bias from ignoring the relation. In this paper, we propose a novel bagging predictor designed for such weak predictor variab...

  5. Observations on Working Psychoanalytically with a Profoundly Amnesic Patient

    Directory of Open Access Journals (Sweden)

    Paul A. Moore

    2017-08-01

    Full Text Available Individuals with profound amnesia are markedly impaired in explicitly recalling new episodic events, but appear to preserve the capacity to use information from other sources. Amongst these preserved capacities is the ability to form new memories of an emotional nature – a skill at the heart of developing and sustaining interpersonal relationships. The psychoanalytic study of individuals with profound amnesia might contribute to the understanding the importance of each memory system, including effects on key analytic processes such as transference and countertransference. However, psychoanalytic work in the presence of profound amnesia might also require important technical modifications. In the first report of its kind, we describe observations from a long term psychoanalytic process (72 sessions with an individual (JL who has profound amnesia after an anoxic episode. The nature of therapy was shaped by JL’s impairment in connecting elements that belong to distant (and even relatively close moments in the therapeutic process. However, we were also able to document areas of preservation, in what appears to be a functioning therapeutic alliance. As regards transference, the relationship between JL and his analyst can be viewed as the evolution of a narcissistic transference, and case material is provided that maps this into three phases: (i rejecting; (ii starting to take in; and (iii full use of the analytic space – where each phase exhibits differing degrees of permeability between JL and the analyst. This investigation appears to have important theoretical implications for psychoanalytic practice, and for psychotherapy in general – and not only with regard to brain injured populations. We especially note that it raises questions concerning the mechanism of therapeutic action in psychoanalysis and psychotherapy, and the apparent unimportance of episodic memory for many elements of therapeutic change.

  6. Foveal Processing Under Concurrent Peripheral Load in Profoundly Deaf Adults

    Science.gov (United States)

    2016-01-01

    Development of the visual system typically proceeds in concert with the development of audition. One result is that the visual system of profoundly deaf individuals differs from that of those with typical auditory systems. While past research has suggested deaf people have enhanced attention in the visual periphery, it is still unclear whether or not this enhancement entails deficits in central vision. Profoundly deaf and typically hearing adults were administered a variant of the useful field of view task that independently assessed performance on concurrent central and peripheral tasks. Identification of a foveated target was impaired by a concurrent selective peripheral attention task, more so in profoundly deaf adults than in the typically hearing. Previous findings of enhanced performance on the peripheral task were not replicated. These data are discussed in terms of flexible allocation of spatial attention targeted towards perceived task demands, and support a modified “division of labor” hypothesis whereby attentional resources co-opted to process peripheral space result in reduced resources in the central visual field. PMID:26657078

  7. Electro-weak theory

    International Nuclear Information System (INIS)

    Deshpande, N.G.

    1980-01-01

    By electro-weak theory is meant the unified field theory that describes both weak and electro-magnetic interactions. The development of a unified electro-weak theory is certainly the most dramatic achievement in theoretical physics to occur in the second half of this century. It puts weak interactions on the same sound theoretical footing as quantum elecrodynamics. Many theorists have contributed to this development, which culminated in the works of Glashow, Weinberg and Salam, who were jointly awarded the 1979 Nobel Prize in physics. Some of the important ideas that contributed to this development are the theory of beta decay formulated by Fermi, Parity violation suggested by Lee and Yang, and incorporated into immensely successful V-A theory of weak interactions by Sudarshan and Marshak. At the same time ideas of gauge invariance were applied to weak interaction by Schwinger, Bludman and Glashow. Weinberg and Salam then went one step further and wrote a theory that is renormalizable, i.e., all higher order corrections are finite, no mean feat for a quantum field theory. The theory had to await the development of the quark model of hadrons for its completion. A description of the electro-weak theory is given

  8. Weak interactions with nuclei

    International Nuclear Information System (INIS)

    Walecka, J.D.

    1983-01-01

    Nuclei provide systems where the strong, electomagnetic, and weak interactions are all present. The current picture of the strong interactions is based on quarks and quantum chromodynamics (QCD). The symmetry structure of this theory is SU(3)/sub C/ x SU(2)/sub W/ x U(1)/sub W/. The electroweak interactions in nuclei can be used to probe this structure. Semileptonic weak interactions are considered. The processes under consideration include beta decay, neutrino scattering and weak neutral-current interactions. The starting point in the analysis is the effective Lagrangian of the Standard Model

  9. History of Weak Interactions

    Science.gov (United States)

    Lee, T. D.

    1970-07-01

    While the phenomenon of beta-decay was discovered near the end of the last century, the notion that the weak interaction forms a separate field of physical forces evolved rather gradually. This became clear only after the experimental discoveries of other weak reactions such as muon-decay, muon-capture, etc., and the theoretical observation that all these reactions can be described by approximately the same coupling constant, thus giving rise to the notion of a universal weak interaction. Only then did one slowly recognize that the weak interaction force forms an independent field, perhaps on the same footing as the gravitational force, the electromagnetic force, and the strong nuclear and sub-nuclear forces.

  10. Hunting the weak bosons

    International Nuclear Information System (INIS)

    Anon.

    1979-01-01

    The possibility of the production of weak bosons in the proton-antiproton colliding beam facilities which are currently being developed, is discussed. The production, decay and predicted properties of these particles are described. (W.D.L.).

  11. Diaphragm muscle fiber dysfunction in chronic obstructive pulmonary disease: toward a pathophysiological concept.

    NARCIS (Netherlands)

    Ottenheijm, C.A.C.; Heunks, L.M.A.; Dekhuijzen, P.N.R.

    2007-01-01

    Inspiratory muscle weakness in patients with chronic obstructive pulmonary disease (COPD) is of major clinical relevance; maximum inspiratory pressure generation is an independent determinant of survival in severe COPD. Traditionally, inspiratory muscle weakness has been ascribed to

  12. Charged weak currents

    International Nuclear Information System (INIS)

    Turlay, R.

    1979-01-01

    In this review of charged weak currents I shall concentrate on inclusive high energy neutrino physics. There are surely still things to learn from the low energy weak interaction but I will not discuss it here. Furthermore B. Tallini will discuss the hadronic final state of neutrino interactions. Since the Tokyo conference a few experimental results have appeared on charged current interaction, I will present them and will also comment on important topics which have been published during the last past year. (orig.)

  13. Weakly oval electron lense

    International Nuclear Information System (INIS)

    Daumenov, T.D.; Alizarovskaya, I.M.; Khizirova, M.A.

    2001-01-01

    The method of the weakly oval electrical field getting generated by the axially-symmetrical field is shown. Such system may be designed with help of the cylindric form coaxial electrodes with the built-in quadrupole duplet. The singularity of the indicated weakly oval lense consists of that it provides the conducting both mechanical and electronic adjustment. Such lense can be useful for elimination of the near-axis astigmatism in the electron-optical system

  14. Immunosenescence of the CD8(+) T cell compartment is associated with HIV-infection, but only weakly reflects age-related processes of adipose tissue, metabolism, and muscle in antiretroviral therapy-treated HIV-infected patients and controls

    DEFF Research Database (Denmark)

    Tavenier, Juliette; Langkilde, Anne; Haupt, Thomas Huneck

    2015-01-01

    of immunosenescence is not well established. Studying immunosenescence in HIV-infection could give insight into its role in ageing processes. In this cross-sectional study, we aimed to investigate whether ART-treated HIV-infected patients exhibit immunosenescence; and whether immunosenescence is associated with age......BACKGROUND: Despite effective antiretroviral therapy (ART), HIV-infected patients exhibit systemic inflammation, early onset of age-related diseases, and features of immunosenescence. The role of inflammation in the development of age-related diseases is widely recognized. However, the role......-related processes of inflammation, metabolism, adipose tissue, and muscle. T cell immunosenescence and exhaustion were assessed by flow cytometry analysis of CD8 (+) cells from 43 ART-treated HIV-infected patients (HIV(+)) and ten Controls using markers of differentiation: CD27/CD28; maturation: CD27/CD45RA...

  15. Inducible satellite cell depletion attenuates skeletal muscle regrowth following a scald-burn injury.

    Science.gov (United States)

    Finnerty, Celeste C; McKenna, Colleen F; Cambias, Lauren A; Brightwell, Camille R; Prasai, Anesh; Wang, Ye; El Ayadi, Amina; Herndon, David N; Suman, Oscar E; Fry, Christopher S

    2017-11-01

    Severe burns result in significant skeletal muscle cachexia that impedes recovery. Activity of satellite cells, skeletal muscle stem cells, is altered following a burn injury and likely hinders regrowth of muscle. Severe burn injury induces satellite cell proliferation and fusion into myofibres with greater activity in muscles proximal to the injury site. Conditional depletion of satellite cells attenuates recovery of myofibre area and volume following a scald burn injury in mice. Skeletal muscle regrowth following a burn injury requires satellite cell activity, underscoring the therapeutic potential of satellite cells in the prevention of prolonged frailty in burn survivors. Severe burns result in profound skeletal muscle atrophy; persistent muscle atrophy and weakness are major complications that hamper recovery from burn injury. Many factors contribute to the erosion of muscle mass following burn trauma, and we have previously shown concurrent activation and apoptosis of muscle satellite cells following a burn injury in paediatric patients. To determine the necessity of satellite cells during muscle recovery following a burn injury, we utilized a genetically modified mouse model (Pax7 CreER -DTA) that allows for the conditional depletion of satellite cells in skeletal muscle. Additionally, mice were provided 5-ethynyl-2'-deoxyuridine to determine satellite cell proliferation, activation and fusion. Juvenile satellite cell-wild-type (SC-WT) and satellite cell-depleted (SC-Dep) mice (8 weeks of age) were randomized to sham or burn injury consisting of a dorsal scald burn injury covering 30% of total body surface area. Both hindlimb and dorsal muscles were studied at 7, 14 and 21 days post-burn. SC-Dep mice had >93% depletion of satellite cells compared to SC-WT (P satellite cell proliferation and fusion. Depletion of satellite cells impaired post-burn recovery of both muscle fibre cross-sectional area and volume (P satellite cells in the aetiology of lean

  16. Bipolar Affective Disorder in a Patient of Profound Deafness.

    Science.gov (United States)

    Zubair, Usama Bin; Mumtaz, Humza; Mansoor, Sawera

    2018-03-01

    Profound deafness is a lifelong impairment, leading to the physical disability as well as poor psychological adjustment. We herein present a mental health disorder rarely seen among the patients of profound deafness. A 16-year deaf and dumb girl, previously treated for depression, presented with unusual laughter, irritability, flight of ideas, decreased sleep, ideas of self importance, and decreased social functioning and educational performance. These problems were understood by the parents via sign language, who interpreted them to the interviewer. Her Young Mania Rating Scale (YMRS) score was 19 and Brief Psychiatric Rating Scale (BPRS) score was 52. She was diagnosed as a case of bipolar affective disorder (BPAD). Marked improvement in the symptoms and social and educational performance was noted after two weeks of the treatment with sodium valproate, resperidone and clonazepam. Treatment options were explained to the patient with risks and benefits, and she was involved in the decision-making. This case report highlights the importance of accurately diagnosing and managing a rare mental health disorder among the physically handicapped people, especially those who cannot communicate effectively and explain their unusual subjective experiences.

  17. Effects of oblique muscle surgery on the rectus muscle pulley

    International Nuclear Information System (INIS)

    Okanobu, Hirotaka; Kono, Reika; Ohtsuki, Hiroshi

    2011-01-01

    The purpose of this study was to determine the position of rectus muscle pulleys in Japanese eyes and to evaluate the effect of oblique muscle surgery on rectus muscle pulleys. Quasi-coronal plane MRI was used to determine area centroids of the 4 rectus muscles. The area centroids of the rectus muscles were transformed to 2-dimensional coordinates to represent pulley positions. The effects of oblique muscle surgery on the rectus muscle pulley positions in the coronal plane were evaluated in 10 subjects with cyclovertical strabismus and, as a control, pulley locations in 7 normal Japanese subjects were calculated. The mean positions of the rectus muscle pulleys in the coronal plane did not significantly differ from previous reports on normal populations, including Caucasians. There were significant positional shifts of the individual horizontal and vertical rectus muscle pulleys in 3 (100%) patients with inferior oblique advancement, but not in eyes with inferior oblique recession and superior oblique tendon advancement surgery. The surgical cyclorotatory effect was significantly correlated with the change in the angle of inclination formed by the line connecting the vertical rectus muscles (p=0.0234), but weakly correlated with that of the horizontal rectus muscles. The most important factor that affects the pulley position is the amount of ocular torsion, not the difference in surgical procedure induced by oblique muscle surgery. (author)

  18. Muscle Deoxygenation Causes Muscle Fatigue

    Science.gov (United States)

    Murthy, G.; Hargens, A. R.; Lehman, S.; Rempel, D.

    1999-01-01

    Muscle fatigue is a common musculoskeletal disorder in the work place, and may be a harbinger for more disabling cumulative trauma disorders. Although the cause of fatigue is multifactorial, reduced blood flow and muscle oxygenation may be the primary factor in causing muscle fatigue during low intensity muscle exertion. Muscle fatigue is defined as a reduction in muscle force production, and also occurs among astronauts who are subjected to postural constraints while performing lengthy, repetitive tasks. The objectives of this research are to: 1) develop an objective tool to study the role of decreased muscle oxygenation on muscle force production, and 2) to evaluate muscle fatigue during prolonged glovebox work.

  19. Muscle Cramps

    Science.gov (United States)

    ... Talk to your provider about the risks and benefits of medicines. How can I prevent muscle cramps? To prevent muscle cramps, you can Stretch your muscles, especially before exercising. If you often get leg cramps at night, ...

  20. Weak radiative hyperon decays

    International Nuclear Information System (INIS)

    Roberts, B.L.; Booth, E.C.; Gall, K.P.; McIntyre, E.K.; Miller, J.P.; Whitehouse, D.A.; Bassalleck, B.; Hall, J.R.; Larson, K.D.; Wolfe, D.M.; Fickinger, W.J.; Robinson, D.K.; Hallin, A.L.; Hasinoff, M.D.; Measday, D.F.; Noble, A.J.; Waltham, C.E.; Hessey, N.P.; Lowe, J.; Horvath, D.; Salomon, M.

    1990-01-01

    New measurements of the Σ + and Λ weak radiative decays are discussed. The hyperons were produced at rest by the reaction K - p → Yπ where Y = Σ + or Λ. The monoenergetic pion was used to tag the hyperon production, and the branching ratios were determined from the relative amplitudes of Σ + → pγ to Σ + → pπ 0 and Λ → nγ to Λ → nπ 0 . The photons from weak radiative decays and from π 0 decays were detected with modular NaI arrays. (orig.)

  1. Satellite cells in human skeletal muscle plasticity.

    Science.gov (United States)

    Snijders, Tim; Nederveen, Joshua P; McKay, Bryon R; Joanisse, Sophie; Verdijk, Lex B; van Loon, Luc J C; Parise, Gianni

    2015-01-01

    Skeletal muscle satellite cells are considered to play a crucial role in muscle fiber maintenance, repair and remodeling. Our knowledge of the role of satellite cells in muscle fiber adaptation has traditionally relied on in vitro cell and in vivo animal models. Over the past decade, a genuine effort has been made to translate these results to humans under physiological conditions. Findings from in vivo human studies suggest that satellite cells play a key role in skeletal muscle fiber repair/remodeling in response to exercise. Mounting evidence indicates that aging has a profound impact on the regulation of satellite cells in human skeletal muscle. Yet, the precise role of satellite cells in the development of muscle fiber atrophy with age remains unresolved. This review seeks to integrate recent results from in vivo human studies on satellite cell function in muscle fiber repair/remodeling in the wider context of satellite cell biology whose literature is largely based on animal and cell models.

  2. A weak equivalence principle test on a suborbital rocket

    Energy Technology Data Exchange (ETDEWEB)

    Reasenberg, Robert D; Phillips, James D, E-mail: reasenberg@cfa.harvard.ed [Smithsonian Astrophysical Observatory, Harvard-Smithsonian Center for Astrophysics, Cambridge, MA 02138 (United States)

    2010-05-07

    We describe a Galilean test of the weak equivalence principle, to be conducted during the free fall portion of a sounding rocket flight. The test of a single pair of substances is aimed at a measurement uncertainty of sigma(eta) < 10{sup -16} after averaging the results of eight separate drops. The weak equivalence principle measurement is made with a set of four laser gauges that are expected to achieve 0.1 pm Hz{sup -1/2}. The discovery of a violation (eta not = 0) would have profound implications for physics, astrophysics and cosmology.

  3. Profound hyper natremia due to central diabetes insipidus

    International Nuclear Information System (INIS)

    Vaqar, A.; Javaid, K.H.; Parveen, R.; Sadaf, R.

    2012-01-01

    Diabetes insipidus is a rare endocrine disorder in paediatric patients. Polyuria is a cardinal manifestation that is extremely difficult to recognize in diapered infants. Careful urine quantification is the key to diagnosis in appropriate clinical setting. We report a case of a 4 months old infant presenting with an acute life threatening event following an episode of vomiting and decreased oral intake. She had profound hyper natremia which persisted after stabilization. Polyuria unrecognized by the mother was revealed by 24-hour urine output measurement. A diagnosis of diabetes insipidus was made after appropriate laboratory investigations including serum and urine osmolality. The central nature of the disease was confirmed by neuroimaging which showed holoprosencephaly. (author)

  4. Disease-Induced Skeletal Muscle Atrophy and Fatigue

    NARCIS (Netherlands)

    Powers, Scott K.; Lynch, Gordon S.; Murphy, Kate T.; Reid, Michael B.; Zijdewind, Inge

    2016-01-01

    Numerous health problems including acute critical illness, cancer, diseases associated with chronic inflammation, and neurological disorders often result in skeletal muscle weakness and fatigue. Disease-related muscle atrophy and fatigue is an important clinical problem because acquired skeletal

  5. Startpoints via weak contractions

    OpenAIRE

    Agyingi, Collins Amburo; Gaba, Yaé Ulrich

    2018-01-01

    Startpoints (resp. endpoints) can be defined as "oriented fixed points". They arise naturally in the study of fixed for multi-valued maps defined on quasi-metric spaces. In this article, we give a new result in the startpoint theory for quasi-pseudometric spaces. The result we present is obtained via a generalized weakly contractive set-valued map.

  6. Weakly Coretractable Modules

    Science.gov (United States)

    Hadi, Inaam M. A.; Al-aeashi, Shukur N.

    2018-05-01

    If R is a ring with identity and M is a unitary right R-module. Here we introduce the class of weakly coretractable module. Some basic properties are investigated and some relationships between these modules and other related one are introduced.

  7. Pseudohypertrophy of the calf muscles in a patient with diabetic neuropathy: a case report

    International Nuclear Information System (INIS)

    Lee, Eun Jin; Lee, Young Hwan; Jung, Kyung Jae; Park, Young Chan; Kim, Ho Kyun; Kim, Ok Dong

    2007-01-01

    Partial or complete loss of innervation of skeletal muscle leads to muscle weakness and atrophic changes, resulting in decreased muscle volume with fatty replacement. Rarely, enlargement of the affected muscle may occur, related to two processes: true hypertrophy and pseudohypertrophy. We report CT and MR findings of the pseudohypertrophy of calf muscles, especially the soleus and gastrocnemius muscles, in a patient with diabetic neuropathy that showed increased muscle volume with diffuse fatty replacement and the presence of scanty muscle fibers

  8. Pseudohypertrophy of the calf muscles in a patient with diabetic neuropathy: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Eun Jin; Lee, Young Hwan; Jung, Kyung Jae; Park, Young Chan; Kim, Ho Kyun; Kim, Ok Dong [School of Medicine, Catholic University of Daegu, Daegu (Korea, Republic of)

    2007-09-15

    Partial or complete loss of innervation of skeletal muscle leads to muscle weakness and atrophic changes, resulting in decreased muscle volume with fatty replacement. Rarely, enlargement of the affected muscle may occur, related to two processes: true hypertrophy and pseudohypertrophy. We report CT and MR findings of the pseudohypertrophy of calf muscles, especially the soleus and gastrocnemius muscles, in a patient with diabetic neuropathy that showed increased muscle volume with diffuse fatty replacement and the presence of scanty muscle fibers.

  9. THE MAKE BREAK TEST AS A DIAGNOSTIC-TOOL IN FUNCTIONAL WEAKNESS

    NARCIS (Netherlands)

    VANDERPLOEG, RJO; OOSTERHUIS, HJGH

    Strength was measured in four major muscle groups with a hand-held dynamometer. The "make" and "break" technique was used with and without encouragement, and fatiguability was tested in patients with organic weakness and patients with functional weakness. Patients with functional weakness could be

  10. Partial muscle carnitine palmitoyltransferase-A deficiency

    International Nuclear Information System (INIS)

    Ross, N.S.; Hoppel, C.L.

    1987-01-01

    After initiation of ibuprofen therapy, a 45-year-old woman developed muscle weakness and tenderness with rhabdomyolysis, culminating in respiratory failure. A muscle biopsy specimen showed a vacuolar myopathy, and markedly decreased muscle carnitine content and carnitine palmitoyltransferase activity. Following recovery, muscle carnitine content was normal but carnitine palmitoyltransferase activity was still abnormally low. The ratio of palmitoyl-coenzyme A plus carnitine to palmitoylcarnitine oxidation by muscle mitochondria isolated from the patient was markedly decreased. The authors conclude that transiently decreased muscle carnitine content interacted with partial deficiency of carnitine palmitoyltransferase-A to produce rhabdomyolysis and respiratory failure and that ibuprofen may have precipitated the clinical event

  11. Partial muscle carnitine palmitoyltransferase-A deficiency

    Energy Technology Data Exchange (ETDEWEB)

    Ross, N.S.; Hoppel, C.L.

    1987-01-02

    After initiation of ibuprofen therapy, a 45-year-old woman developed muscle weakness and tenderness with rhabdomyolysis, culminating in respiratory failure. A muscle biopsy specimen showed a vacuolar myopathy, and markedly decreased muscle carnitine content and carnitine palmitoyltransferase activity. Following recovery, muscle carnitine content was normal but carnitine palmitoyltransferase activity was still abnormally low. The ratio of palmitoyl-coenzyme A plus carnitine to palmitoylcarnitine oxidation by muscle mitochondria isolated from the patient was markedly decreased. The authors conclude that transiently decreased muscle carnitine content interacted with partial deficiency of carnitine palmitoyltransferase-A to produce rhabdomyolysis and respiratory failure and that ibuprofen may have precipitated the clinical event.

  12. Nuclear Positioning in Muscle Development and Disease

    Directory of Open Access Journals (Sweden)

    Eric eFolker

    2013-12-01

    Full Text Available Muscle disease as a group is characterized by muscle weakness, muscle loss, and impaired muscle function. Although the phenotype is the same, the underlying cellular pathologies, and the molecular causes of these pathologies, are diverse. One common feature of many muscle disorders is the mispositioning of myonuclei. In unaffected individuals myonuclei are spaced throughout the periphery of the muscle fiber such that the distance between nuclei is maximized. However, in diseased muscles, the nuclei are often clustered within the center of the muscle cell. Although this phenotype has been acknowledged for several decades, it is often ignored as a contributor to muscle weakness. Rather, these nuclei are taken only as a sign of muscle repair. Here we review the evidence that mispositioned myonuclei are not merely a symptom of muscle disease but also a cause. Additionally, we review the working models for how myonuclei move from two different perspectives, from that of the nucleus and from that of the cytoskeleton. We further compare and contrast these mechanisms with the mechanisms of nuclear movement in other cell types both to draw general themes for nuclear movement and to identify muscle-specific considerations. Finally, we focus on factors that can be linked to muscle disease and find that genes that regulate myonuclear movement and positioning have been linked to muscular dystrophy. Although the cause-effect relationship is largely speculative, recent data indicate that the position of nuclei should no longer be considered only a means to diagnose muscle disease.

  13. Introduction to weak interactions

    International Nuclear Information System (INIS)

    Leite Lopes, J.

    An account is first given of the electromagnetic interactions of complex, scalar, vector and spinor fields. It is shown that the electromagnetic field may be considered as a gauge field. Yang-Mills fields and the field theory invariant with respect to the non-Abelian gauge transformation group are then described. The construction, owing to this invariance principle, of conserved isospin currents associated with gauge fields is also demonstrated. This is followed by a historical survey of the development of the weak interaction theory, established at first to describe beta disintegration processes by analogy with electrodynamics. The various stages are mentioned from the discovery of principles and rules and violation of principles, such as those of invariance with respect to spatial reflection and charge conjugation to the formulation of the effective current-current Lagrangian and research on the structure of weak currents [fr

  14. An official American Thoracic Society Clinical Practice guideline: the diagnosis of intensive care unit-acquired weakness in adults.

    Science.gov (United States)

    Fan, Eddy; Cheek, Fern; Chlan, Linda; Gosselink, Rik; Hart, Nicholas; Herridge, Margaret S; Hopkins, Ramona O; Hough, Catherine L; Kress, John P; Latronico, Nicola; Moss, Marc; Needham, Dale M; Rich, Mark M; Stevens, Robert D; Wilson, Kevin C; Winkelman, Chris; Zochodne, Doug W; Ali, Naeem A

    2014-12-15

    Profound muscle weakness during and after critical illness is termed intensive care unit-acquired weakness (ICUAW). To develop diagnostic recommendations for ICUAW. A multidisciplinary expert committee generated diagnostic questions. A systematic review was performed, and recommendations were developed using the Grading, Recommendations, Assessment, Development, and Evaluation (GRADE) approach. Severe sepsis, difficult ventilator liberation, and prolonged mechanical ventilation are associated with ICUAW. Physical rehabilitation improves outcomes in heterogeneous populations of ICU patients. Because it may not be feasible to provide universal physical rehabilitation, an alternative approach is to identify patients most likely to benefit. Patients with ICUAW may be such a group. Our review identified only one case series of patients with ICUAW who received physical therapy. When compared with a case series of patients with ICUAW who did not receive structured physical therapy, evidence suggested those who receive physical rehabilitation were more frequently discharged home rather than to a rehabilitative facility, although confidence intervals included no difference. Other interventions show promise, but fewer data proving patient benefit existed, thus precluding specific comment. Additionally, prior comorbidity was insufficiently defined to determine its influence on outcome, treatment response, or patient preferences for diagnostic efforts. We recommend controlled clinical trials in patients with ICUAW that compare physical rehabilitation with usual care and further research in understanding risk and patient preferences. Research that identifies treatments that benefit patients with ICUAW is necessary to determine whether the benefits of diagnostic testing for ICUAW outweigh its burdens.

  15. Weak states and security

    OpenAIRE

    Rakipi, Albert

    2006-01-01

    Cataloged from PDF version of article. Although the weak 1 failing states have often been deseribed as the single most important problem for the international order s ince the en d of Cold W ar (F .Fukuyaına 2004:92) several dimensions of this phenomenon still remain unexplored. While this phenomenon has been present in the international politics even earlier, only the post Cold W ar period accentuated its relationship with security issues. Following the Cold W ar' s "peacef...

  16. Composite weak bosons

    Energy Technology Data Exchange (ETDEWEB)

    Suzuki, M.

    1988-04-01

    Dynamical mechanism of composite W and Z is studied in a 1/N field theory model with four-fermion interactions in which global weak SU(2) symmetry is broken explicitly by electromagnetic interaction. Issues involved in such a model are discussed in detail. Deviation from gauge coupling due to compositeness and higher order loop corrections are examined to show that this class of models are consistent not only theoretically but also experimentally.

  17. Survival and weak chaos.

    Science.gov (United States)

    Nee, Sean

    2018-05-01

    Survival analysis in biology and reliability theory in engineering concern the dynamical functioning of bio/electro/mechanical units. Here we incorporate effects of chaotic dynamics into the classical theory. Dynamical systems theory now distinguishes strong and weak chaos. Strong chaos generates Type II survivorship curves entirely as a result of the internal operation of the system, without any age-independent, external, random forces of mortality. Weak chaos exhibits (a) intermittency and (b) Type III survivorship, defined as a decreasing per capita mortality rate: engineering explicitly defines this pattern of decreasing hazard as 'infant mortality'. Weak chaos generates two phenomena from the normal functioning of the same system. First, infant mortality- sensu engineering-without any external explanatory factors, such as manufacturing defects, which is followed by increased average longevity of survivors. Second, sudden failure of units during their normal period of operation, before the onset of age-dependent mortality arising from senescence. The relevance of these phenomena encompasses, for example: no-fault-found failure of electronic devices; high rates of human early spontaneous miscarriage/abortion; runaway pacemakers; sudden cardiac death in young adults; bipolar disorder; and epilepsy.

  18. Finger extension weakness and downbeat nystagmus motor neuron disease syndrome: A novel motor neuron disorder?

    Science.gov (United States)

    Delva, Aline; Thakore, Nimish; Pioro, Erik P; Poesen, Koen; Saunders-Pullman, Rachel; Meijer, Inge A; Rucker, Janet C; Kissel, John T; Van Damme, Philip

    2017-12-01

    Disturbances of eye movements are infrequently encountered in motor neuron diseases (MNDs) or motor neuropathies, and there is no known syndrome that combines progressive muscle weakness with downbeat nystagmus. To describe the core clinical features of a syndrome of MND associated with downbeat nystagmus, clinical features were collected from 6 patients. All patients had slowly progressive muscle weakness and wasting in combination with downbeat nystagmus, which was clinically most obvious in downward and lateral gaze. Onset was in the second to fourth decade with finger extension weakness, progressing to other distal and sometimes more proximal muscles. Visual complaints were not always present. Electrodiagnostic testing showed signs of regional motor axonal loss in all patients. The etiology of this syndrome remains elusive. Because finger extension weakness and downbeat nystagmus are the discriminating clinical features of this MND, we propose the name FEWDON-MND syndrome. Muscle Nerve 56: 1164-1168, 2017. © 2017 The Authors Muscle & Nerve Published by Wiley Periodicals, Inc.

  19. Systemic inflammatory response syndrome increases immobility-induced neuromuscular weakness.

    Science.gov (United States)

    Fink, Heidrun; Helming, Marc; Unterbuchner, Christoph; Lenz, Andrea; Neff, Frauke; Martyn, J A Jeevendra; Blobner, Manfred

    2008-03-01

    Inflammation and immobility are comorbid etiological factors inducing muscle weakness in critically ill patients. This study establishes a rat model to examine the effect of inflammation and immobilization alone and in combination on muscle contraction, histology, and acetylcholine receptor regulation. Prospective, randomized, experimental study. Animal laboratory of a university hospital. Sprague-Dawley rats. To produce systemic inflammation, rats (n = 34) received three consecutive intravenous injections of Corynebacterium parvum on days 0, 4, and 8. Control rats (n = 21) received saline. Both groups were further divided to have one hind limb either immobilized by pinning of knee and ankle joints or sham-immobilized (surgical leg). The contralateral nonsurgical leg of each animal served as control (nonsurgical leg). After 12 days, body weight and muscle mass were significantly reduced in all C. parvum animals compared with saline-injected rats. Immobilization led to local muscle atrophy. Normalized to muscle mass, tetanic contraction was reduced in the surgical leg after immobilization (7.64 +/- 1.91 N/g) and after inflammation (8.71 +/- 2.0 N/g; both p < .05 vs. sham immobilization and saline injection, 11.03 +/- 2.26 N/g). Histology showed an increase in inflammatory cells in all C. parvum-injected animals. Immobilization in combination with C. parvum injection had an additive effect on inflammation. Acetylcholine receptors were increased in immobilized muscles and in all muscles of C. parvum-injected animals. The muscle weakness in critically ill patients can be replicated in our novel rat model. Inflammation and immobilization independently lead to muscle weakness.

  20. Otolithic organ function in patients with profound sensorineural hearing loss

    Directory of Open Access Journals (Sweden)

    Yujuan Zhou

    2016-06-01

    Full Text Available Profound sensorineural hearing loss (PSHL is not uncommonly encountered in otology. In clinics, there is a high incidence of otolithic damage in patients with PSHL, but relevant reports are few. Sharing a continuous membranous structure and similar receptor cell ultrastructures, the cochlea and vestibule may be susceptible to the same harmful factors. Disorders of the inner ear may result in a variety of manifestations, including vertigo, spatial disorientation, blurred vision, impaired articulation, and hearing impairment. Considering the diversity of clinical symptoms associated with PSHL with otolithic dysfunction, it may be frequently misdiagnosed, and objective means of testing the function of otolithic organs should be recommended for hearing-impaired patients. Vestibular-evoked myogenic potentials (VEMPs via air-conducted sound are of great importance for the diagnosis of otolithic function. Hearing devices such as cochlear implants are commonly accepted treatments for PSHL, and early identification and treatment of vestibular disorders may increase the success rate of cochlear implantation. Therefore, it is necessary to increase awareness of otolithic functional states in patients with PSHL.

  1. Cancer prevention knowledge of people with profound hearing loss.

    Science.gov (United States)

    Zazove, Philip; Meador, Helen E; Reed, Barbara D; Sen, Ananda; Gorenflo, Daniel W

    2009-03-01

    Deaf persons, a documented minority population, have low reading levels and difficulty communicating with physicians. The effect of these on their knowledge of cancer prevention recommendations is unknown. A cross-sectional study of 222 d/Deaf persons in Michigan, age 18 and older, chose one of four ways (voice, video of a certified American Sign Language interpreter, captions, or printed English) to complete a self-administered computer video questionnaire about demographics, hearing loss, language history, health-care utilization, and health-care information sources, as well as family and social variables. Twelve questions tested their knowledge of cancer prevention recommendations. The outcome measures were the percentage of correct answers to the questions and the association of multiple variables with these responses. Participants averaged 22.9% correct answers with no gender difference. Univariate analysis revealed that smoking history, types of medical problems, last physician visit, and women having previous cancer preventive tests did not affect scores. Improved scores occurred with computer use (p = 0.05), higher education (p internet (p = 0.02), and believing that smoking is bad (p bad (p = 0.05) were associated with improved scores. Persons with profound hearing loss have poor knowledge of recommended cancer prevention interventions. English use in multiple settings was strongly associated with increased knowledge.

  2. Histones induce rapid and profound thrombocytopenia in mice

    Science.gov (United States)

    Bhandari, Ashish A.

    2011-01-01

    Histones are released from dying cells and contribute to antimicrobial defense during infection. However, extracellular histones are a double-edged sword because they also damage host tissue and may cause death. We studied the interactions of histones with platelets. Histones bound to platelets, induced calcium influx, and recruited plasma adhesion proteins such as fibrinogen to induce platelet aggregation. Hereby fibrinogen cross-linked histone-bearing platelets and triggered microaggregation. Fibrinogen interactions with αIIbβ3 integrins were not required for this process but were necessary for the formation of large platelet aggregates. Infused histones associated with platelets in vivo and caused a profound thrombocytopenia within minutes after administration. Mice lacking platelets or αIIbβ3 integrins were protected from histone-induced death but not from histone-induced tissue damage. Heparin, at high concentrations, prevented histone interactions with platelets and protected mice from histone-induced thrombocytopenia, tissue damage, and death. Heparin and histones are evolutionary maintained. Histones may combine microbicidal with prothrombotic properties to fight invading microbes and maintain hemostasis after injury. Heparin may provide an innate counter mechanism to neutralize histones and diminish collateral tissue damage. PMID:21700775

  3. Hypernuclear weak decay puzzle

    International Nuclear Information System (INIS)

    Barbero, C.; Horvat, D.; Narancic, Z.; Krmpotic, F.; Kuo, T.T.S.; Tadic, D.

    2002-01-01

    A general shell model formalism for the nonmesonic weak decay of the hypernuclei has been developed. It involves a partial wave expansion of the emitted nucleon waves, preserves naturally the antisymmetrization between the escaping particles and the residual core, and contains as a particular case the weak Λ-core coupling formalism. The extreme particle-hole model and the quasiparticle Tamm-Dancoff approximation are explicitly worked out. It is shown that the nuclear structure manifests itself basically through the Pauli principle, and a very simple expression is derived for the neutron- and proton-induced decays rates Γ n and Γ p , which does not involve the spectroscopic factors. We use the standard strangeness-changing weak ΛN→NN transition potential which comprises the exchange of the complete pseudoscalar and vector meson octets (π,η,K,ρ,ω,K * ), taking into account some important parity-violating transition operators that are systematically omitted in the literature. The interplay between different mesons in the decay of Λ 12 C is carefully analyzed. With the commonly used parametrization in the one-meson-exchange model (OMEM), the calculated rate Γ NM =Γ n +Γ p is of the order of the free Λ decay rate Γ 0 (Γ NM th congruent with Γ 0 ) and is consistent with experiments. Yet the measurements of Γ n/p =Γ n /Γ p and of Γ p are not well accounted for by the theory (Γ n/p th p th > or approx. 0.60Γ 0 ). It is suggested that, unless additional degrees of freedom are incorporated, the OMEM parameters should be radically modified

  4. Atypical presentation of GNE myopathy with asymmetric hand weakness

    Science.gov (United States)

    de Dios, John Karl L.; Shrader, Joseph A.; Joe, Galen O.; McClean, Jeffrey C.; Williams, Kayla; Evers, Robert; Malicdan, May Christine V.; Ciccone, Carla; Mankodi, Ami; Huizing, Marjan; McKew, John C.; Bluemke, David A.; Gahl, William A.; Carrillo-Carrasco, Nuria

    2014-01-01

    GNE myopathy is a rare autosomal recessive muscle disease caused by mutations in GNE, the gene encoding the rate-limiting enzyme in sialic acid biosynthesis. GNE myopathy usually manifests in early adulthood with distal myopathy that progresses slowly and symmetrically, first involving distal muscles of the lower extremities, followed by proximal muscles with relative sparing of the quadriceps. Upper extremities are typically affected later in the disease. We report a patient with GNE myopathy who presented with asymmetric hand weakness. He had considerably decreased left grip strength, atrophy of the left anterior forearm and fibro-fatty tissue replacement of left forearm flexor muscles on T1-weighted magnetic resonance imaging. The patient was an endoscopist and thus the asymmetric hand involvement may be associated with left hand overuse in daily repetitive pinching and gripping movements, highlighting the possible impact of environmental factors on the progression of genetic muscle conditions. PMID:25182749

  5. Measurement of weak radioactivity

    CERN Document Server

    Theodorsson , P

    1996-01-01

    This book is intended for scientists engaged in the measurement of weak alpha, beta, and gamma active samples; in health physics, environmental control, nuclear geophysics, tracer work, radiocarbon dating etc. It describes the underlying principles of radiation measurement and the detectors used. It also covers the sources of background, analyzes their effect on the detector and discusses economic ways to reduce the background. The most important types of low-level counting systems and the measurement of some of the more important radioisotopes are described here. In cases where more than one type can be used, the selection of the most suitable system is shown.

  6. On Weak Markov's Principle

    DEFF Research Database (Denmark)

    Kohlenbach, Ulrich Wilhelm

    2002-01-01

    We show that the so-called weak Markov's principle (WMP) which states that every pseudo-positive real number is positive is underivable in E-HA + AC. Since allows one to formalize (atl eastl arge parts of) Bishop's constructive mathematics, this makes it unlikely that WMP can be proved within...... the framework of Bishop-style mathematics (which has been open for about 20 years). The underivability even holds if the ine.ective schema of full comprehension (in all types) for negated formulas (in particular for -free formulas) is added, which allows one to derive the law of excluded middle...

  7. Weak interaction rates

    International Nuclear Information System (INIS)

    Sugarbaker, E.

    1995-01-01

    I review available techniques for extraction of weak interaction rates in nuclei. The case for using hadron charge exchange reactions to estimate such rates is presented and contrasted with alternate methods. Limitations of the (p,n) reaction as a probe of Gamow-Teller strength are considered. Review of recent comparisons between beta-decay studies and (p,n) is made, leading to cautious optimism regarding the final usefulness of (p,n)- derived GT strengths to the field of astrophysics. copyright 1995 American Institute of Physics

  8. Evaluation of muscle hyperactivity of the grimacing muscles by unilateral tight eyelid closure and stapedius muscle tone.

    Science.gov (United States)

    Shiba, Masato; Matsuo, Kiyoshi; Ban, Ryokuya; Nagai, Fumio

    2012-10-01

    Muscle hyperactivity of grimacing muscles, including the orbicularis oculi and corrugator supercilii muscles that cause crow's feet and a glabellar frown line with ageing, cannot be accurately evaluated by surface observation. In 71 subjects, this study investigated the extent to which grimacing muscles are innervated by the bilateral motor cortices, whether the corticofacial projection to the grimacing muscles affects the facially innervated stapedius muscle tone by measuring static compliance of the tympanic membrane, and whether unilateral tight eyelid closure with contraction of the grimacing muscles changes static compliance. Unilateral tight eyelid closure and its subsequent change in the contralateral vertical medial eyebrow position revealed that motor neurons of the orbicularis oculi and corrugator supercilii muscles were innervated by the bilateral motor cortices with weak-to-strong contralateral dominance. The orbicularis oculi, corrugator supercilii, and stapedius muscles innervated by the bilateral motor cortices had increased muscle hyperactivity, which lowered the vertical medial eyebrow position and decreased the static compliance of the tympanic membrane more than those innervated by the unilateral motor cortex. Unilateral enhanced tight eyelid closure with contraction of the grimacing muscles in certain subjects ipsilaterally decreased the static compliance with increased contraction of the stapedius muscle, which probably occurs to immobilise the tympanic membrane and protect the inner ear from loud sound. Evaluation of unilateral tight eyelid closure and the subsequent change in the contralateral vertical medial eyebrow position as well as a measurement of the static compliance for the stapedius muscle tone has revealed muscle hyperactivity of grimacing muscles.

  9. Cochlear implantation in autistic children with profound sensorineural hearing loss.

    Science.gov (United States)

    Lachowska, Magdalena; Pastuszka, Agnieszka; Łukaszewicz-Moszyńska, Zuzanna; Mikołajewska, Lidia; Niemczyk, Kazimierz

    2016-11-19

    Cochlear implants have become the method of choice for the treatment of severe-to-profound hearing loss in both children and adults. Its benefits are well documented in the pediatric and adult population. Also deaf children with additional needs, including autism, have been covered by this treatment. The aim of this study was to assess the benefits from cochlear implantation in deafened children with autism as the only additional disability. This study analyzes data of six children. The follow-up time was at least 43 months. The following data were analyzed: medical history, reaction to music and sound, Ling's six sounds test, onomatopoeic word test, reaction to spoken child's name, response to requests, questionnaire given to parents, sound processor fitting sessions and data. After cochlear implantation each child presented other communication skills. In some children, the symptoms of speech understanding were observed. No increased hyperactivity associated with daily use cochlear implant was observed. The study showed that in autistic children the perception is very important for a child's sense of security and makes contact with parents easier. Our study showed that oral communication is not likely to be a realistic goal in children with cochlear implants and autism. The implantation results showed benefits that varied among those children. The traditional methods of evaluating the results of cochlear implantation in children with autism are usually insufficient to fully assess the functional benefits. These benefits should be assessed in a more comprehensive manner taking into account the limitations of communication resulting from the essence of autism. It is important that we share knowledge about these complex children with cochlear implants. Copyright © 2016 Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial. Published by Elsevier Editora Ltda. All rights reserved.

  10. Weakly Supervised Dictionary Learning

    Science.gov (United States)

    You, Zeyu; Raich, Raviv; Fern, Xiaoli Z.; Kim, Jinsub

    2018-05-01

    We present a probabilistic modeling and inference framework for discriminative analysis dictionary learning under a weak supervision setting. Dictionary learning approaches have been widely used for tasks such as low-level signal denoising and restoration as well as high-level classification tasks, which can be applied to audio and image analysis. Synthesis dictionary learning aims at jointly learning a dictionary and corresponding sparse coefficients to provide accurate data representation. This approach is useful for denoising and signal restoration, but may lead to sub-optimal classification performance. By contrast, analysis dictionary learning provides a transform that maps data to a sparse discriminative representation suitable for classification. We consider the problem of analysis dictionary learning for time-series data under a weak supervision setting in which signals are assigned with a global label instead of an instantaneous label signal. We propose a discriminative probabilistic model that incorporates both label information and sparsity constraints on the underlying latent instantaneous label signal using cardinality control. We present the expectation maximization (EM) procedure for maximum likelihood estimation (MLE) of the proposed model. To facilitate a computationally efficient E-step, we propose both a chain and a novel tree graph reformulation of the graphical model. The performance of the proposed model is demonstrated on both synthetic and real-world data.

  11. Ethanol Exposure Causes Muscle Degeneration in Zebrafish

    Directory of Open Access Journals (Sweden)

    Elizabeth C. Coffey

    2018-03-01

    Full Text Available Alcoholic myopathies are characterized by neuromusculoskeletal symptoms such as compromised movement and weakness. Although these symptoms have been attributed to neurological damage, EtOH may also target skeletal muscle. EtOH exposure during zebrafish primary muscle development or adulthood results in smaller muscle fibers. However, the effects of EtOH exposure on skeletal muscle during the growth period that follows primary muscle development are not well understood. We determined the effects of EtOH exposure on muscle during this phase of development. Strikingly, muscle fibers at this stage are acutely sensitive to EtOH treatment: EtOH induces muscle degeneration. The severity of EtOH-induced muscle damage varies but muscle becomes more refractory to EtOH as muscle develops. NF-kB induction in muscle indicates that EtOH triggers a pro-inflammatory response. EtOH-induced muscle damage is p53-independent. Uptake of Evans blue dye shows that EtOH treatment causes sarcolemmal instability before muscle fiber detachment. Dystrophin-null sapje mutant zebrafish also exhibit sarcolemmal instability. We tested whether Trichostatin A (TSA, which reduces muscle degeneration in sapje mutants, would affect EtOH-treated zebrafish. We found that TSA and EtOH are a lethal combination. EtOH does, however, exacerbate muscle degeneration in sapje mutants. EtOH also disrupts adhesion of muscle fibers to their extracellular matrix at the myotendinous junction: some detached muscle fibers retain beta-Dystroglycan indicating failure of muscle end attachments. Overexpression of Paxillin, which reduces muscle degeneration in zebrafish deficient for beta-Dystroglycan, is not sufficient to rescue degeneration. Taken together, our results suggest that EtOH exposure has pleiotropic deleterious effects on skeletal muscle.

  12. Muscle histochemistry in chronic alcoholism

    Directory of Open Access Journals (Sweden)

    M. L. Ferraz

    1989-06-01

    Full Text Available Twenty-two chronic acoholic patients were assessed by neurologic examination and muscle biopsy. The patients manifested proximal muscular weakness to a variable extent. One case presented as an acute bout of myopathy, according to the Manual Muscle Test, MMT. The most prominent histologic feature observed was muscle atrophy (95.3% better evidenced through the ATPase stain with the predominance of type II A fibers (71.4%. Lack of the mosaic pattern (type grouping seen in 76% of the cases and an important mitochondrial proliferation with intrasarcoplasmatic lipid accumulation in 63% of the patients. In case of acute presentation of muscle weakness the. pathological substrate is quite different, i.e. presence of myositis mainly interstitial characterized by lymphoplasmocytic infiltrate and several spots of necrosis like Zencker degeneration. Based on histologic criteria, our data suggest that: the main determinant of muscle weakness seen in chronic alcoholic patients is neurogenic in origin (alcoholic polineuropathy; the direct toxic action of ethanol under the skeletal muscle is closely related to the mitochondrial metabolism; the so-called acute alcoholic myopathy has probably viral etiology.

  13. A muscle model for hybrid muscle activation

    Directory of Open Access Journals (Sweden)

    Klauer Christian

    2015-09-01

    Full Text Available To develop model-based control strategies for Functional Electrical Stimulation (FES in order to support weak voluntary muscle contractions, a hybrid model for describing joint motions induced by concurrent voluntary-and FES induced muscle activation is proposed. It is based on a Hammerstein model – as commonly used in feedback controlled FES – and exemplarily applied to describe the shoulder abduction joint angle. Main component of a Hammerstein muscle model is usually a static input nonlinearity depending on the stimulation intensity. To additionally incorporate voluntary contributions, we extended the static non-linearity by a second input describing the intensity of the voluntary contribution that is estimated by electromyography (EMG measurements – even during active FES. An Artificial Neural Network (ANN is used to describe the static input non-linearity. The output of the ANN drives a second-order linear dynamical system that describes the combined muscle activation and joint angle dynamics. The tunable parameters are adapted to the individual subject by a system identification approach using previously recorded I/O-data. The model has been validated in two healthy subjects yielding RMS values for the joint angle error of 3.56° and 3.44°, respectively.

  14. Nanotechnological Inventions and Nanomaterials Produce A Profound Effect

    Directory of Open Access Journals (Sweden)

    VLASOV Vladimir Alexeevich

    2015-02-01

    Full Text Available The inventions in the area of nanotechnologies and nanomaterials produce a profound effect in construction, housing and communal services and adjacent economic fields as they allow us: to increase mechanical strength, coefficient of elasticity, alkali resistance and temperature of products vitrification; to obtain nanostructured coatings with the property of shape memory on the steel; to raise the dynamics of coal burning and its full burnout in the boilers of thermoelectric power station; to produce metal nanopowders with increased stored energy 10–15% etc. For example, the invention «Epoxy composition for high strength, alkali resistant structures» refers to epoxy composition used as a binder for production of high strength, thermal- and alkali-resistant glass-fiber material which can be applied in the manufacture process of construction reinforcement to strengthen concrete structures. The invention «The method to produce nanostructured reaction foil» can be used to join different materials including metal alloys, ceramics, amorphous materials and elements of microelectronic devices that are sensible to the heating. This process provides decreased labour-output ratio and energy consumption as well as the condition to manufacture foil with specified stored energy and high mechanical properties. The invention «The method of intensification of burning lowreactionary coal in the boilers of thermoelectric power station» refers to the thermal energy and can be implemented at the thermal plants. The increased dynamics of inflaming and burning leads to full burnout of powdered-coal low-reactionary fuel and decreased mechanical underfiring. The specialists may be also interested in the following inventions: fine dispersed organic suspension of carbon metal-containing nanostructures and the method to produce it; the dispersion of carbon nanotubes; the composition for reinforcement of building structures; the reinforced plate element made of

  15. Muscle channelopathies and electrophysiological approach

    Directory of Open Access Journals (Sweden)

    Cherian Ajith

    2008-01-01

    Full Text Available Myotonic syndromes and periodic paralyses are rare disorders of skeletal muscle characterized mainly by muscle stiffness or episodic attacks of weakness. Familial forms are caused by mutation in genes coding for skeletal muscle voltage ionic channels. Familial periodic paralysis and nondystrophic myotonias are disorders of skeletal muscle excitability caused by mutations in genes coding for voltage-gated ion channels. These diseases are characterized by episodic failure of motor activity due to muscle weakness (paralysis or stiffness (myotonia. Clinical studies have identified two forms of periodic paralyses: hypokalemic periodic paralysis (hypoKPP and hyperkalemic periodic paralysis (hyperKPP, based on changes in serum potassium levels during the attacks, and three distinct forms of myotonias: paramyotonia congenita (PC, potassium-aggravated myotonia (PAM, and myotonia congenita (MC. PC and PAM have been linked to missense mutations in the SCN4A gene, which encodes α subunit of the voltage-gated sodium channel, whereas MC is caused by mutations in the chloride channel gene (CLCN1. Exercise is known to trigger, aggravate, or relieve symptoms. Therefore, exercise can be used as a functional test in electromyography to improve the diagnosis of these muscle disorders. Abnormal changes in the compound muscle action potential can be disclosed using different exercise tests. Five electromyographic (EMG patterns (I-V that may be used in clinical practice as guides for molecular diagnosis are discussed.

  16. Hip abduction weakness in elite junior footballers is common but easy to correct quickly: a prospective sports team cohort based study

    Directory of Open Access Journals (Sweden)

    Osborne Hamish R

    2012-10-01

    Full Text Available Abstract Background Hip abduction weakness has never been documented on a population basis as a common finding in a healthy group of athletes and would not normally be found in an elite adolescent athlete. This study aimed to show that hip abduction weakness not only occurs in this group but also is common and easy to correct with an unsupervised home based program. Methods A prospective sports team cohort based study was performed with thirty elite adolescent under-17 Australian Rules Footballers in the Australian Institute of Sport/Australian Football League Under-17 training academy. The players had their hip abduction performance assessed and were then instructed in a hip abduction muscle training exercise. This was performed on a daily basis for two months and then they were reassessed. Results The results showed 14 of 28 athletes who completed the protocol had marked weakness or a side-to-side difference of more than 25% at baseline. Two months later ten players recorded an improvement of ≥ 80% in their recorded scores. The mean muscle performance on the right side improved from 151 Newton (N to 202 N (p Conclusions The baseline values show widespread profound deficiencies in hip abduction performance not previously reported. Very large performance increases can be achieved, unsupervised, in a short period of time to potentially allow large clinically significant gains. This assessment should be an integral part of preparticipation screening and assessed in those with lower limb injuries. This particular exercise should be used clinically and more research is needed to determine its injury prevention and performance enhancement implications.

  17. Fraqueza muscular esquelética e intolerância ao exercício em pacientes com doença pulmonar obstrutiva crônica Skeletal muscle weakness and exercise intolerance in patients with chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    KR Silva

    2008-06-01

    21cmH2O, p<0.05, palm grip strength (38±6 versus 47±5kg, p<0.05, peak torque (103±21 versus 138±18Nm, p<0.05 and total work of the quadriceps femoris (1570±395 versus 2333±568J, p<0.05 when compared with the control group (independent Student’s t test. There was no correlation between FEV1 and the variables studied, while the FFM correlated with the total work of the quadriceps (Pearson, r=0.6290, p<0.05. CONCLUSIONS: These results indicate that patients with COPD show weakness of the inspiratory and quadriceps muscles and lower functional capacity, when compared with a healthy group. Moreover, they suggest that the degree of airflow obstruction is not a good predictor for quantifying the nutritional and muscle impairments in patients with COPD.

  18. Muscle Contraction.

    Science.gov (United States)

    Sweeney, H Lee; Hammers, David W

    2018-02-01

    SUMMARYMuscle cells are designed to generate force and movement. There are three types of mammalian muscles-skeletal, cardiac, and smooth. Skeletal muscles are attached to bones and move them relative to each other. Cardiac muscle comprises the heart, which pumps blood through the vasculature. Skeletal and cardiac muscles are known as striated muscles, because the filaments of actin and myosin that power their contraction are organized into repeating arrays, called sarcomeres, that have a striated microscopic appearance. Smooth muscle does not contain sarcomeres but uses the contraction of filaments of actin and myosin to constrict blood vessels and move the contents of hollow organs in the body. Here, we review the principal molecular organization of the three types of muscle and their contractile regulation through signaling mechanisms and discuss their major structural and functional similarities that hint at the possible evolutionary relationships between the cell types. Copyright © 2018 Cold Spring Harbor Laboratory Press; all rights reserved.

  19. RhoE deficiency produces postnatal lethality, profound motor deficits and neurodevelopmental delay in mice.

    Directory of Open Access Journals (Sweden)

    Enric Mocholí

    Full Text Available Rnd proteins are a subfamily of Rho GTPases involved in the control of actin cytoskeleton dynamics and other cell functions such as motility, proliferation and survival. Unlike other members of the Rho family, Rnd proteins lack GTPase activity and therefore remain constitutively active. We have recently described that RhoE/Rnd3 is expressed in the Central Nervous System and that it has a role in promoting neurite formation. Despite their possible relevance during development, the role of Rnd proteins in vivo is not known. To get insight into the in vivo function of RhoE we have generated mice lacking RhoE expression by an exon trapping cassette. RhoE null mice (RhoE gt/gt are smaller at birth, display growth retardation and early postnatal death since only half of RhoE gt/gt mice survive beyond postnatal day (PD 15 and 100% are dead by PD 29. RhoE gt/gt mice show an abnormal body position with profound motor impairment and impaired performance in most neurobehavioral tests. Null mutant mice are hypoactive, show an immature locomotor pattern and display a significant delay in the appearance of the hindlimb mature responses. Moreover, they perform worse than the control littermates in the wire suspension, vertical climbing and clinging, righting reflex and negative geotaxis tests. Also, RhoE ablation results in a delay of neuromuscular maturation and in a reduction in the number of spinal motor neurons. Finally, RhoE gt/gt mice lack the common peroneal nerve and, consequently, show a complete atrophy of the target muscles. This is the first model to study the in vivo functions of a member of the Rnd subfamily of proteins, revealing the important role of Rnd3/RhoE in the normal development and suggesting the possible involvement of this protein in neurological disorders.

  20. How much muscle strength is required to walk in a crouch gait?

    NARCIS (Netherlands)

    Steele, K.M.; van der Krogt, M.M.; Schwartz, M.H.; Delp, S.L.

    2012-01-01

    Muscle weakness is commonly cited as a cause of crouch gait in individuals with cerebral palsy; however, outcomes after strength training are variable and mechanisms by which muscle weakness may contribute to crouch gait are unclear. Understanding how much muscle strength is required to walk in a

  1. Lifting the nebula: novel insights into skeletal muscle contractility.

    Science.gov (United States)

    Ottenheijm, Coen A C; Granzier, Henk

    2010-10-01

    Nebulin is a giant protein and a constituent of the skeletal muscle sarcomere. The name of this protein refers to its unknown (i.e., nebulous) function. However, recent rapid advances reveal that nebulin plays important roles in the regulation of muscle contraction. When these functions of nebulin are compromised, muscle weakness ensues, as is the case in patients with nemaline myopathy.

  2. A Replication to Increase Happiness Indices among Some People with Profound Multiple Disabilities.

    Science.gov (United States)

    Ivancic, Martin T.; And Others

    1997-01-01

    The happiness indices in a Fun Time activity were systematically increased for three of four people with profound multiple disabilities by providing their preferred stimuli. The fourth showed decreased unhappiness. However, three people with profound disabilities and minimal movement failed to show any indices of happiness or unhappiness.…

  3. Teaching Individuals with Profound Multiple Disabilities to Access Preferred Stimuli with Multiple Microswitches

    Science.gov (United States)

    Tam, Gee May; Phillips, Katrina J.; Mudford, Oliver C.

    2011-01-01

    We replicated and extended previous research on microswitch facilitated choice making by individuals with profound multiple disabilities. Following an assessment of stimulus preferences, we taught 6 adults with profound multiple disabilities to emit 2 different responses to activate highly preferred stimuli. All participants learnt to activate…

  4. Standard and Null Weak Values

    OpenAIRE

    Zilberberg, Oded; Romito, Alessandro; Gefen, Yuval

    2013-01-01

    Weak value (WV) is a quantum mechanical measurement protocol, proposed by Aharonov, Albert, and Vaidman. It consists of a weak measurement, which is weighed in, conditional on the outcome of a later, strong measurement. Here we define another two-step measurement protocol, null weak value (NVW), and point out its advantages as compared to WV. We present two alternative derivations of NWVs and compare them to the corresponding derivations of WVs.

  5. Weak openness and almost openness

    Directory of Open Access Journals (Sweden)

    David A. Rose

    1984-01-01

    Full Text Available Weak openness and almost openness for arbitrary functions between topological spaces are defined as duals to the weak continuity of Levine and the almost continuity of Husain respectively. Independence of these two openness conditions is noted and comparison is made between these and the almost openness of Singal and Singal. Some results dual to those known for weak continuity and almost continuity are obtained. Nearly almost openness is defined and used to obtain an improved link from weak continuity to almost continuity.

  6. Weak measurements and quantum weak values for NOON states

    Science.gov (United States)

    Rosales-Zárate, L.; Opanchuk, B.; Reid, M. D.

    2018-03-01

    Quantum weak values arise when the mean outcome of a weak measurement made on certain preselected and postselected quantum systems goes beyond the eigenvalue range for a quantum observable. Here, we propose how to determine quantum weak values for superpositions of states with a macroscopically or mesoscopically distinct mode number, that might be realized as two-mode Bose-Einstein condensate or photonic NOON states. Specifically, we give a model for a weak measurement of the Schwinger spin of a two-mode NOON state, for arbitrary N . The weak measurement arises from a nondestructive measurement of the two-mode occupation number difference, which for atomic NOON states might be realized via phase contrast imaging and the ac Stark effect using an optical meter prepared in a coherent state. The meter-system coupling results in an entangled cat-state. By subsequently evolving the system under the action of a nonlinear Josephson Hamiltonian, we show how postselection leads to quantum weak values, for arbitrary N . Since the weak measurement can be shown to be minimally invasive, the weak values provide a useful strategy for a Leggett-Garg test of N -scopic realism.

  7. Your Muscles

    Science.gov (United States)

    ... and you need to throw up. The muscles push the food back out of the stomach so it comes up ... body the power it needs to lift and push things. Muscles in your neck and the top part of your back aren't as large, but they are capable ...

  8. Exercise-induced muscle modifications

    International Nuclear Information System (INIS)

    Kerviler, E. de; Willig, A.L.; Jehenson, P.; Duboc, D.; Syrota, A.

    1990-01-01

    This paper compares changes in muscle proton T2 after exercise in normal subjects and in patients with muscular glycogenoses. Four patients suffering from muscular glycogenosis and eight normal volunteers were studied. Muscle T2s were measured in forearm muscles at rest and after exercise, with a 0.5-T imager. The exercise was performed with handgrips and was evaluated by P-31 spectroscopy (end-exercise decrease in pH and phosphocreatine) performed with a 2-T magnet. In normal subjects, a relative T2 increase, ranging from 14% to 44%, was observed in the exercised muscles. In the patients, who cannot produce lactate during exercise, weak pH variation occurred, and only a slight T2 increase (7% - 9%) was observed

  9. Weak decays of stable particles

    International Nuclear Information System (INIS)

    Brown, R.M.

    1988-09-01

    In this article we review recent advances in the field of weak decays and consider their implications for quantum chromodynamics (the theory of strong interactions) and electroweak theory (the combined theory of electromagnetic and weak interactions), which together form the ''Standard Model'' of elementary particles. (author)

  10. Contractures and muscle disease.

    Science.gov (United States)

    Walters, R Jon

    2016-08-01

    Contractures are one of a handful of signs in muscle disease, besides weakness and its distribution, whose presence can help guide us diagnostically, a welcome star on the horizon. Contractures are associated with several myopathies, some with important cardiac manifestations, and consequently are important to recognise; their presence may also provide us with a potential satisfying 'penny dropping' diagnostic moment. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

  11. Misperceiving Bullshit as Profound Is Associated with Favorable Views of Cruz, Rubio, Trump and Conservatism.

    Directory of Open Access Journals (Sweden)

    Stefan Pfattheicher

    Full Text Available The present research investigates the associations between holding favorable views of potential Democratic or Republican candidates for the US presidency 2016 and seeing profoundness in bullshit statements. In this contribution, bullshit is used as a technical term which is defined as communicative expression that lacks content, logic, or truth from the perspective of natural science. We used the Bullshit Receptivity scale (BSR to measure seeing profoundness in bullshit statements. The BSR scale contains statements that have a correct syntactic structure and seem to be sound and meaningful on first reading but are actually vacuous. Participants (N = 196; obtained via Amazon Mechanical Turk rated the profoundness of bullshit statements (using the BSR and provided favorability ratings of three Democratic (Hillary Clinton, Martin O'Malley, and Bernie Sanders and three Republican candidates for US president (Ted Cruz, Marco Rubio, and Donald Trump. Participants also completed a measure of political liberalism/conservatism. Results revealed that favorable views of all three Republican candidates were positively related to judging bullshit statements as profound. The smallest correlation was found for Donald Trump. Although we observe a positive association between bullshit and support for the three Democrat candidates, this relationship is both substantively small and statistically insignificant. The general measure of political liberalism/conservatism was also related to judging bullshit statements as profound in that individuals who were more politically conservative had a higher tendency to see profoundness in bullshit statements. Of note, these results were not due to a general tendency among conservatives to see profoundness in everything: Favorable views of Republican candidates and conservatism were not significantly related to profoundness ratings of mundane statements. In contrast, this was the case for Hillary Clinton and Martin O

  12. Misperceiving Bullshit as Profound Is Associated with Favorable Views of Cruz, Rubio, Trump and Conservatism.

    Science.gov (United States)

    Pfattheicher, Stefan; Schindler, Simon

    2016-01-01

    The present research investigates the associations between holding favorable views of potential Democratic or Republican candidates for the US presidency 2016 and seeing profoundness in bullshit statements. In this contribution, bullshit is used as a technical term which is defined as communicative expression that lacks content, logic, or truth from the perspective of natural science. We used the Bullshit Receptivity scale (BSR) to measure seeing profoundness in bullshit statements. The BSR scale contains statements that have a correct syntactic structure and seem to be sound and meaningful on first reading but are actually vacuous. Participants (N = 196; obtained via Amazon Mechanical Turk) rated the profoundness of bullshit statements (using the BSR) and provided favorability ratings of three Democratic (Hillary Clinton, Martin O'Malley, and Bernie Sanders) and three Republican candidates for US president (Ted Cruz, Marco Rubio, and Donald Trump). Participants also completed a measure of political liberalism/conservatism. Results revealed that favorable views of all three Republican candidates were positively related to judging bullshit statements as profound. The smallest correlation was found for Donald Trump. Although we observe a positive association between bullshit and support for the three Democrat candidates, this relationship is both substantively small and statistically insignificant. The general measure of political liberalism/conservatism was also related to judging bullshit statements as profound in that individuals who were more politically conservative had a higher tendency to see profoundness in bullshit statements. Of note, these results were not due to a general tendency among conservatives to see profoundness in everything: Favorable views of Republican candidates and conservatism were not significantly related to profoundness ratings of mundane statements. In contrast, this was the case for Hillary Clinton and Martin O'Malley. Overall, small

  13. Electromagnetic current in weak interactions

    International Nuclear Information System (INIS)

    Ma, E.

    1983-01-01

    In gauge models which unify weak and electromagnetic interactions, the weak neutral-current interaction also involves the electromagnetic current. The exact nature of such a component can be explored using e + e - experimental data. In recent years, the existence of a new component of the weak interaction has become firmly established, i.e., the neutral-current interaction. As such, it competes with the electromagnetic interaction whenever the particles involved are also charged, but at a very much lower rate because its effective strength is so small. Hence neutrino processes are best for the detection of the neutral-current interaction. However, in any gauge model which unifies weak and electromagnetic interactions, the weak neutral-current interaction also involves the electromagnetic current

  14. Weak values in collision theory

    Science.gov (United States)

    de Castro, Leonardo Andreta; Brasil, Carlos Alexandre; Napolitano, Reginaldo de Jesus

    2018-05-01

    Weak measurements have an increasing number of applications in contemporary quantum mechanics. They were originally described as a weak interaction that slightly entangled the translational degrees of freedom of a particle to its spin, yielding surprising results after post-selection. That description often ignores the kinetic energy of the particle and its movement in three dimensions. Here, we include these elements and re-obtain the weak values within the context of collision theory by two different approaches, and prove that the results are compatible with each other and with the results from the traditional approach. To provide a more complete description, we generalize weak values into weak tensors and use them to provide a more realistic description of the Stern-Gerlach apparatus.

  15. Correction of Malocclusion by Botulinum Neurotoxin Injection into Masticatory Muscles

    OpenAIRE

    Seok, Hyun; Kim, Seong-Gon

    2018-01-01

    Botulinum toxin (BTX) is a neurotoxin, and its injection in masticatory muscles induces muscle weakness and paralysis. This paralytic effect of BTX induces growth retardation of the maxillofacial bones, changes in dental eruption and occlusion state, and facial asymmetry. Using masticatory muscle paralysis and its effect via BTX, BTX can be used for the correction of malocclusion after orthognathic surgery and mandible fracture. The paralysis of specific masticatory muscles by BTX injection r...

  16. Association between statin-associated myopathy and skeletal muscle damage.

    OpenAIRE

    Mohaupt Markus G; Karas Richard H; Babiychuk Eduard B; Sanchez-Freire Verónica; Monastyrskaya Katia; Iyer Lakshmanan; Hoppeler Hans; Breil Fabio; Draeger Annette

    2009-01-01

    BACKGROUND Many patients taking statins often complain of muscle pain and weakness. The extent to which muscle pain reflects muscle injury is unknown. METHODS We obtained biopsy samples from the vastus lateralis muscle of 83 patients. Of the 44 patients with clinically diagnosed statin associated myopathy 29 were currently taking a statin and 15 had discontinued statin therapy before the biopsy (minimal duration of discontinuation 3 weeks). We also included 19 patients who were taking stat...

  17. Subunit Stoichiometry of Human Muscle Chloride Channels

    OpenAIRE

    Fahlke, Christoph; Knittle, Timothy; Gurnett, Christina A.; Campbell, Kevin P.; George, Alfred L.

    1997-01-01

    Voltage-gated Cl? channels belonging to the ClC family appear to function as homomultimers, but the number of subunits needed to form a functional channel is controversial. To determine subunit stoichiometry, we constructed dimeric human skeletal muscle Cl? channels in which one subunit was tagged by a mutation (D136G) that causes profound changes in voltage-dependent gating. Sucrose-density gradient centrifugation experiments indicate that both monomeric and dimeric hClC-1 channels in their ...

  18. Hartman effect and weak measurements that are not really weak

    International Nuclear Information System (INIS)

    Sokolovski, D.; Akhmatskaya, E.

    2011-01-01

    We show that in wave packet tunneling, localization of the transmitted particle amounts to a quantum measurement of the delay it experiences in the barrier. With no external degree of freedom involved, the envelope of the wave packet plays the role of the initial pointer state. Under tunneling conditions such ''self-measurement'' is necessarily weak, and the Hartman effect just reflects the general tendency of weak values to diverge, as postselection in the final state becomes improbable. We also demonstrate that it is a good precision, or a 'not really weak' quantum measurement: no matter how wide the barrier d, it is possible to transmit a wave packet with a width σ small compared to the observed advancement. As is the case with all weak measurements, the probability of transmission rapidly decreases with the ratio σ/d.

  19. Reversal of profound rocuronium neuromuscular blockade by sugammadex in anesthetized rhesus monkeys.

    NARCIS (Netherlands)

    Boer, H.D. de; Egmond, J. van; Pol, F. van de; Bom, A.; Booij, L.H.D.J.

    2006-01-01

    BACKGROUND: Reversal of neuromuscular blockade can be accomplished by chemical encapsulation of rocuronium by sugammadex, a synthetic gamma-cyclodextrin derivative. The current study determined the feasibility of reversal of rocuronium-induced profound neuromuscular blockade with sugammadex in the

  20. Insulin signaling in skeletal muscle and liver of neonatal pigs during endotoxemia

    Science.gov (United States)

    Sepsis has been associated with tumor necrosis factor alpha (TNF-alpha) and nitric oxide (NO) overproduction, insulin resistance, and a profound suppression of muscle protein synthesis. However, lesser suppression of muscle protein synthesis in neonatal pigs occurs in response to endotoxin (LPS) whe...

  1. Weak Measurement and Quantum Correlation

    Indian Academy of Sciences (India)

    Arun Kumar Pati

    Entanglement: Two quantum systems can be in a strongly correlated state even if .... These are resources which can be used to design quantum computer, quantum ...... Weak measurements have found numerous applications starting from the ...

  2. Weakly infinite-dimensional spaces

    International Nuclear Information System (INIS)

    Fedorchuk, Vitalii V

    2007-01-01

    In this survey article two new classes of spaces are considered: m-C-spaces and w-m-C-spaces, m=2,3,...,∞. They are intermediate between the class of weakly infinite-dimensional spaces in the Alexandroff sense and the class of C-spaces. The classes of 2-C-spaces and w-2-C-spaces coincide with the class of weakly infinite-dimensional spaces, while the compact ∞-C-spaces are exactly the C-compact spaces of Haver. The main results of the theory of weakly infinite-dimensional spaces, including classification via transfinite Lebesgue dimensions and Luzin-Sierpinsky indices, extend to these new classes of spaces. Weak m-C-spaces are characterised by means of essential maps to Henderson's m-compacta. The existence of hereditarily m-strongly infinite-dimensional spaces is proved.

  3. Weak interactions and presupernova evolution

    International Nuclear Information System (INIS)

    Aufderheide, M.B.; State Univ. of New York

    1991-01-01

    The role of weak interactions, particularly electron capture and β - decay, in presupernova evolution is discussed. The present uncertainty in these rates is examined and the possibility of improving the situation is addressed. 12 refs., 4 figs

  4. Muscle cramps

    Science.gov (United States)

    ... the lower leg/calf Back of the thigh (hamstrings) Front of the thigh (quadriceps) Cramps in the ... Names Cramps - muscle Images Chest stretch Groin stretch Hamstring stretch Hip stretch Thigh stretch Triceps stretch References ...

  5. Muscle atrophy

    Science.gov (United States)

    ... People who cannot actively move one or more joints can do exercises using braces or splints . When ... A.M. Editorial team. Muscle Disorders Read more Neuromuscular Disorders Read more NIH MedlinePlus Magazine Read more ...

  6. Weak Deeply Virtual Compton Scattering

    International Nuclear Information System (INIS)

    Ales Psaker; Wolodymyr Melnitchouk; Anatoly Radyushkin

    2006-01-01

    We extend the analysis of the deeply virtual Compton scattering process to the weak interaction sector in the generalized Bjorken limit. The virtual Compton scattering amplitudes for the weak neutral and charged currents are calculated at the leading twist within the framework of the nonlocal light-cone expansion via coordinate space QCD string operators. Using a simple model, we estimate cross sections for neutrino scattering off the nucleon, relevant for future high intensity neutrino beam facilities

  7. Weakly compact operators and interpolation

    OpenAIRE

    Maligranda, Lech

    1992-01-01

    The class of weakly compact operators is, as well as the class of compact operators, a fundamental operator ideal. They were investigated strongly in the last twenty years. In this survey, we have collected and ordered some of this (partly very new) knowledge. We have also included some comments, remarks and examples. The class of weakly compact operators is, as well as the class of compact operators, a fundamental operator ideal. They were investigated strongly in the last twenty years. I...

  8. Feeding during the resting phase causes profound changes in physiology and desynchronization between liver and muscle rhythms of rats

    NARCIS (Netherlands)

    Opperhuizen, Anne-Loes; Wang, Dawei; Foppen, E.; Jansen, Remi; Boudzovitch-Surovtseva, Olga; de Vries, Janneke; Fliers, Eric; Kalsbeek, A.

    2016-01-01

    Shiftworkers run an increased risk of developing metabolic disorders, presumably as a result of disturbed circadian physiology. Eating at a time-of-day that is normally dedicated to resting and fasting, may contribute to this association. The hypothalamus is the key brain area that integrates

  9. Catechins activate muscle stem cells by Myf5 induction and stimulate muscle regeneration.

    Science.gov (United States)

    Kim, A Rum; Kim, Kyung Min; Byun, Mi Ran; Hwang, Jun-Ha; Park, Jung Il; Oh, Ho Taek; Kim, Hyo Kyeong; Jeong, Mi Gyeong; Hwang, Eun Sook; Hong, Jeong-Ho

    2017-07-22

    Muscle weakness is one of the most common symptoms in aged individuals and increases risk of mortality. Thus, maintenance of muscle mass is important for inhibiting aging. In this study, we investigated the effect of catechins, polyphenol compounds in green tea, on muscle regeneration. We found that (-)-epicatechin gallate (ECG) and (-)-epigallocatechin-3-gallate (EGCG) activate satellite cells by induction of Myf5 transcription factors. For satellite cell activation, Akt kinase was significantly induced after ECG treatment and ECG-induced satellite cell activation was blocked in the presence of Akt inhibitor. ECG also promotes myogenic differentiation through the induction of myogenic markers, including Myogenin and Muscle creatine kinase (MCK), in satellite and C2C12 myoblast cells. Finally, EGCG administration to mice significantly increased muscle fiber size for regeneration. Taken together, the results suggest that catechins stimulate muscle stem cell activation and differentiation for muscle regeneration. Copyright © 2017 Elsevier Inc. All rights reserved.

  10. An Active Learning Mammalian Skeletal Muscle Lab Demonstrating Contractile and Kinetic Properties of Fast- and Slow-Twitch Muscle

    Science.gov (United States)

    Head, S. I.; Arber, M. B.

    2013-01-01

    The fact that humans possess fast and slow-twitch muscle in the ratio of approximately 50% has profound implications for designing exercise training strategies for power and endurance activities. With the growth of exercise and sport science courses, we have seen the need to develop an undergraduate student laboratory that demonstrates the basic…

  11. DMPD: Gram-negative endotoxin: an extraordinary lipid with profound effects oneukaryotic signal transduction. [Dynamic Macrophage Pathway CSML Database

    Lifescience Database Archive (English)

    Full Text Available 1916089 Gram-negative endotoxin: an extraordinary lipid with profound effects oneuk...ep;5(12):2652-60. (.png) (.svg) (.html) (.csml) Show Gram-negative endotoxin: an extraordinary lipid with profound effects...tive endotoxin: an extraordinary lipid with profound effects oneukaryotic signal transduction. Authors Raetz

  12. Cosmology with weak lensing surveys

    International Nuclear Information System (INIS)

    Munshi, Dipak; Valageas, Patrick; Waerbeke, Ludovic van; Heavens, Alan

    2008-01-01

    Weak gravitational lensing is responsible for the shearing and magnification of the images of high-redshift sources due to the presence of intervening matter. The distortions are due to fluctuations in the gravitational potential, and are directly related to the distribution of matter and to the geometry and dynamics of the Universe. As a consequence, weak gravitational lensing offers unique possibilities for probing the Dark Matter and Dark Energy in the Universe. In this review, we summarise the theoretical and observational state of the subject, focussing on the statistical aspects of weak lensing, and consider the prospects for weak lensing surveys in the future. Weak gravitational lensing surveys are complementary to both galaxy surveys and cosmic microwave background (CMB) observations as they probe the unbiased non-linear matter power spectrum at modest redshifts. Most of the cosmological parameters are accurately estimated from CMB and large-scale galaxy surveys, so the focus of attention is shifting to understanding the nature of Dark Matter and Dark Energy. On the theoretical side, recent advances in the use of 3D information of the sources from photometric redshifts promise greater statistical power, and these are further enhanced by the use of statistics beyond two-point quantities such as the power spectrum. The use of 3D information also alleviates difficulties arising from physical effects such as the intrinsic alignment of galaxies, which can mimic weak lensing to some extent. On the observational side, in the next few years weak lensing surveys such as CFHTLS, VST-KIDS and Pan-STARRS, and the planned Dark Energy Survey, will provide the first weak lensing surveys covering very large sky areas and depth. In the long run even more ambitious programmes such as DUNE, the Supernova Anisotropy Probe (SNAP) and Large-aperture Synoptic Survey Telescope (LSST) are planned. Weak lensing of diffuse components such as the CMB and 21 cm emission can also

  13. Cosmology with weak lensing surveys

    Energy Technology Data Exchange (ETDEWEB)

    Munshi, Dipak [Institute of Astronomy, Madingley Road, Cambridge, CB3 OHA (United Kingdom); Astrophysics Group, Cavendish Laboratory, Madingley Road, Cambridge CB3 OHE (United Kingdom)], E-mail: munshi@ast.cam.ac.uk; Valageas, Patrick [Service de Physique Theorique, CEA Saclay, 91191 Gif-sur-Yvette (France); Waerbeke, Ludovic van [University of British Columbia, Department of Physics and Astronomy, 6224 Agricultural Road, Vancouver, BC V6T 1Z1 (Canada); Heavens, Alan [SUPA - Scottish Universities Physics Alliance, Institute for Astronomy, University of Edinburgh, Blackford Hill, Edinburgh EH9 3HJ (United Kingdom)

    2008-06-15

    Weak gravitational lensing is responsible for the shearing and magnification of the images of high-redshift sources due to the presence of intervening matter. The distortions are due to fluctuations in the gravitational potential, and are directly related to the distribution of matter and to the geometry and dynamics of the Universe. As a consequence, weak gravitational lensing offers unique possibilities for probing the Dark Matter and Dark Energy in the Universe. In this review, we summarise the theoretical and observational state of the subject, focussing on the statistical aspects of weak lensing, and consider the prospects for weak lensing surveys in the future. Weak gravitational lensing surveys are complementary to both galaxy surveys and cosmic microwave background (CMB) observations as they probe the unbiased non-linear matter power spectrum at modest redshifts. Most of the cosmological parameters are accurately estimated from CMB and large-scale galaxy surveys, so the focus of attention is shifting to understanding the nature of Dark Matter and Dark Energy. On the theoretical side, recent advances in the use of 3D information of the sources from photometric redshifts promise greater statistical power, and these are further enhanced by the use of statistics beyond two-point quantities such as the power spectrum. The use of 3D information also alleviates difficulties arising from physical effects such as the intrinsic alignment of galaxies, which can mimic weak lensing to some extent. On the observational side, in the next few years weak lensing surveys such as CFHTLS, VST-KIDS and Pan-STARRS, and the planned Dark Energy Survey, will provide the first weak lensing surveys covering very large sky areas and depth. In the long run even more ambitious programmes such as DUNE, the Supernova Anisotropy Probe (SNAP) and Large-aperture Synoptic Survey Telescope (LSST) are planned. Weak lensing of diffuse components such as the CMB and 21 cm emission can also

  14. Quantum discord with weak measurements

    International Nuclear Information System (INIS)

    Singh, Uttam; Pati, Arun Kumar

    2014-01-01

    Weak measurements cause small change to quantum states, thereby opening up the possibility of new ways of manipulating and controlling quantum systems. We ask, can weak measurements reveal more quantum correlation in a composite quantum state? We prove that the weak measurement induced quantum discord, called as the “super quantum discord”, is always larger than the quantum discord captured by the strong measurement. Moreover, we prove the monotonicity of the super quantum discord as a function of the measurement strength and in the limit of strong projective measurement the super quantum discord becomes the normal quantum discord. We find that unlike the normal discord, for pure entangled states, the super quantum discord can exceed the quantum entanglement. Our results provide new insights on the nature of quantum correlation and suggest that the notion of quantum correlation is not only observer dependent but also depends on how weakly one perturbs the composite system. We illustrate the key results for pure as well as mixed entangled states. -- Highlights: •Introduced the role of weak measurements in quantifying quantum correlation. •We have introduced the notion of the super quantum discord (SQD). •For pure entangled state, we show that the SQD exceeds the entanglement entropy. •This shows that quantum correlation depends not only on observer but also on measurement strength

  15. Satellite cells in human skeletal muscle plasticity

    Directory of Open Access Journals (Sweden)

    Tim eSnijders

    2015-10-01

    Full Text Available Skeletal muscle satellite cells are considered to play a crucial role in muscle fiber maintenance, repair and remodelling. Our knowledge of the role of satellite cells in muscle fiber adaptation has traditionally relied on in vitro cell and in vivo animal models. Over the past decade, a genuine effort has been made to translate these results to humans under physiological conditions. Findings from in vivo human studies suggest that satellite cells play a key role in skeletal muscle fiber repair/remodelling in response to exercise. Mounting evidence indicates that aging has a profound impact on the regulation of satellite cells in human skeletal muscle. Yet, the precise role of satellite cells in the development of muscle fiber atrophy with age remains unresolved. This review seeks to integrate recent results from in vivo human studies on satellite cell function in muscle fiber repair/remodelling in the wider context of satellite cell biology whose literature is largely based on animal and cell models.

  16. An animal model for human masseter muscle: histochemical characterization of mouse, rat, rabbit, cat, dog, pig, and cow masseter muscle

    DEFF Research Database (Denmark)

    Tuxen, A; Kirkeby, S

    1990-01-01

    The masseter muscle of several animal species was investigated by use of a histochemical method for the demonstration of acid-stable and alkali-stable myosin adenosine triphosphatase (ATPase). The following subdivisions of fiber types were used: Type I fibers show weak ATPase activity at pH 9...... II and I fibers, with type II predominating. Cow masseter muscle consisted mainly of type I fibers, although some cow masseter muscles contained a very small number of type II fibers. Pig masseter muscle had both type I, II, and IM fibers. One of the characteristics of human masseter muscle is type...... IM fibers, which are rarely seen in muscles other than the masticatory muscles. Therefore, pig masseter muscle might be a suitable animal model for experimental studies, such as an investigation of the distribution and diameter of fiber types in the masticatory muscles before and after orthognathic...

  17. ProFound: Source Extraction and Application to Modern Survey Data

    Science.gov (United States)

    Robotham, A. S. G.; Davies, L. J. M.; Driver, S. P.; Koushan, S.; Taranu, D. S.; Casura, S.; Liske, J.

    2018-05-01

    We introduce PROFOUND, a source finding and image analysis package. PROFOUND provides methods to detect sources in noisy images, generate segmentation maps identifying the pixels belonging to each source, and measure statistics like flux, size, and ellipticity. These inputs are key requirements of PROFIT, our recently released galaxy profiling package, where the design aim is that these two software packages will be used in unison to semi-automatically profile large samples of galaxies. The key novel feature introduced in PROFOUND is that all photometry is executed on dilated segmentation maps that fully contain the identifiable flux, rather than using more traditional circular or ellipse-based photometry. Also, to be less sensitive to pathological segmentation issues, the de-blending is made across saddle points in flux. We apply PROFOUND in a number of simulated and real-world cases, and demonstrate that it behaves reasonably given its stated design goals. In particular, it offers good initial parameter estimation for PROFIT, and also segmentation maps that follow the sometimes complex geometry of resolved sources, whilst capturing nearly all of the flux. A number of bulge-disc decomposition projects are already making use of the PROFOUND and PROFIT pipeline, and adoption is being encouraged by publicly releasing the software for the open source R data analysis platform under an LGPL-3 license on GitHub (github.com/asgr/ProFound).

  18. A Prognostic Model for Development of Profound Shock among Children Presenting with Dengue Shock Syndrome.

    Directory of Open Access Journals (Sweden)

    Phung Khanh Lam

    Full Text Available To identify risk factors and develop a prediction model for the development of profound and recurrent shock amongst children presenting with dengue shock syndrome (DSS.We analyzed data from a prospective cohort of children with DSS recruited at the Paediatric Intensive Care Unit of the Hospital for Tropical Disease in Ho Chi Minh City, Vietnam. The primary endpoint was "profound DSS", defined as ≥2 recurrent shock episodes (for subjects presenting in compensated shock, or ≥1 recurrent shock episodes (for subjects presenting initially with decompensated/hypotensive shock, and/or requirement for inotropic support. Recurrent shock was evaluated as a secondary endpoint. Risk factors were pre-defined clinical and laboratory variables collected at the time of presentation with shock. Prognostic model development was based on logistic regression and compared to several alternative approaches.The analysis population included 1207 children of whom 222 (18% progressed to "profound DSS" and 433 (36% had recurrent shock. Independent risk factors for both endpoints included younger age, earlier presentation, higher pulse rate, higher temperature, higher haematocrit and, for females, worse hemodynamic status at presentation. The final prognostic model for "profound DSS" showed acceptable discrimination (AUC=0.69 for internal validation and calibration and is presented as a simple score-chart.Several risk factors for development of profound or recurrent shock among children presenting with DSS were identified. The score-chart derived from the prognostic models should improve triage and management of children presenting with DSS in dengue-endemic areas.

  19. Weak-interacting holographic QCD

    International Nuclear Information System (INIS)

    Gazit, D.; Yee, H.-U.

    2008-06-01

    We propose a simple prescription for including low-energy weak-interactions into the frame- work of holographic QCD, based on the standard AdS/CFT dictionary of double-trace deformations. As our proposal enables us to calculate various electro-weak observables involving strongly coupled QCD, it opens a new perspective on phenomenological applications of holographic QCD. We illustrate efficiency and usefulness of our method by performing a few exemplar calculations; neutron beta decay, charged pion weak decay, and meson-nucleon parity non-conserving (PNC) couplings. The idea is general enough to be implemented in both Sakai-Sugimoto as well as Hard/Soft Wall models. (author)

  20. Plane waves with weak singularities

    International Nuclear Information System (INIS)

    David, Justin R.

    2003-03-01

    We study a class of time dependent solutions of the vacuum Einstein equations which are plane waves with weak null singularities. This singularity is weak in the sense that though the tidal forces diverge at the singularity, the rate of divergence is such that the distortion suffered by a freely falling observer remains finite. Among such weak singular plane waves there is a sub-class which does not exhibit large back reaction in the presence of test scalar probes. String propagation in these backgrounds is smooth and there is a natural way to continue the metric beyond the singularity. This continued metric admits string propagation without the string becoming infinitely excited. We construct a one parameter family of smooth metrics which are at a finite distance in the space of metrics from the extended metric and a well defined operator in the string sigma model which resolves the singularity. (author)

  1. A young lady with swelling and stiffness of calf muscles

    Directory of Open Access Journals (Sweden)

    H S Kiran

    2011-01-01

    Full Text Available Hypothyroidism causes a variety of changes in the body. Though uncommon, hypothyroidism can present as myopathy. Hoffman′s syndrome is a specific, rare form of hypothyroid myopathy, which causes proximal weakness and pseudohypertrophy of muscles.

  2. Vitamin D and muscle function.

    Science.gov (United States)

    Dawson-Hughes, Bess

    2017-10-01

    Muscle weakness is a hallmark of severe vitamin D deficiency, but the effect of milder vitamin D deficiency or insufficiency on muscle mass and performance and risk of falling is uncertain. In this presentation, I review the evidence that vitamin D influences muscle mass and performance, balance, and risk of falling in older adults. Special consideration is given to the impact of both the starting 25-hydroxyvitamin D [25(OH)D] level and the dose administered on the clinical response to supplemental vitamin D in older men and women. Based on available evidence, older adults with serum 25(OH)D levels vitamin D dose range of 800-1000 IU per day has been effective in many studies; lower doses have generally been ineffective and several doses above this range have increased the risk of falls. In conclusion, older adults with serum 25(OH)D levels vitamin D. Copyright © 2017 Elsevier Ltd. All rights reserved.

  3. Cosmology and the weak interaction

    Energy Technology Data Exchange (ETDEWEB)

    Schramm, D.N. (Fermi National Accelerator Lab., Batavia, IL (USA)):(Chicago Univ., IL (USA))

    1989-12-01

    The weak interaction plays a critical role in modern Big Bang cosmology. This review will emphasize two of its most publicized cosmological connections: Big Bang nucleosynthesis and Dark Matter. The first of these is connected to the cosmological prediction of Neutrino Flavours, N{sub {nu}} {approximately} 3 which is now being confirmed at SLC and LEP. The second is interrelated to the whole problem of galaxy and structure formation in the universe. This review will demonstrate the role of the weak interaction both for dark matter candidates and for the problem of generating seeds to form structure. 87 refs., 3 figs., 5 tabs.

  4. Cosmology and the weak interaction

    International Nuclear Information System (INIS)

    Schramm, D.N.

    1989-12-01

    The weak interaction plays a critical role in modern Big Bang cosmology. This review will emphasize two of its most publicized cosmological connections: Big Bang nucleosynthesis and Dark Matter. The first of these is connected to the cosmological prediction of Neutrino Flavours, N ν ∼ 3 which is now being confirmed at SLC and LEP. The second is interrelated to the whole problem of galaxy and structure formation in the universe. This review will demonstrate the role of the weak interaction both for dark matter candidates and for the problem of generating seeds to form structure. 87 refs., 3 figs., 5 tabs

  5. Nonlinear waves and weak turbulence

    CERN Document Server

    Zakharov, V E

    1997-01-01

    This book is a collection of papers on dynamical and statistical theory of nonlinear wave propagation in dispersive conservative media. Emphasis is on waves on the surface of an ideal fluid and on Rossby waves in the atmosphere. Although the book deals mainly with weakly nonlinear waves, it is more than simply a description of standard perturbation techniques. The goal is to show that the theory of weakly interacting waves is naturally related to such areas of mathematics as Diophantine equations, differential geometry of waves, Poincaré normal forms, and the inverse scattering method.

  6. Weak disorder in Fibonacci sequences

    Energy Technology Data Exchange (ETDEWEB)

    Ben-Naim, E [Theoretical Division and Center for Nonlinear Studies, Los Alamos National Laboratory, Los Alamos, NM 87545 (United States); Krapivsky, P L [Department of Physics and Center for Molecular Cybernetics, Boston University, Boston, MA 02215 (United States)

    2006-05-19

    We study how weak disorder affects the growth of the Fibonacci series. We introduce a family of stochastic sequences that grow by the normal Fibonacci recursion with probability 1 - {epsilon}, but follow a different recursion rule with a small probability {epsilon}. We focus on the weak disorder limit and obtain the Lyapunov exponent that characterizes the typical growth of the sequence elements, using perturbation theory. The limiting distribution for the ratio of consecutive sequence elements is obtained as well. A number of variations to the basic Fibonacci recursion including shift, doubling and copying are considered. (letter to the editor)

  7. Weak interactions at high energies

    International Nuclear Information System (INIS)

    Ellis, J.

    1978-08-01

    Review lectures are presented on the phenomenological implications of the modern spontaneously broken gauge theories of the weak and electromagnetic interactions, and some observations are made about which high energy experiments probe what aspects of gauge theories. Basic quantum chromodynamics phenomenology is covered including momentum dependent effective quark distributions, the transverse momentum cutoff, search for gluons as sources of hadron jets, the status and prospects for the spectroscopy of fundamental fermions and how fermions may be used to probe aspects of the weak and electromagnetic gauge theory, studies of intermediate vector bosons, and miscellaneous possibilities suggested by gauge theories from the Higgs bosons to speculations about proton decay. 187 references

  8. Attuning: A Communication Process between People with Severe and Profound Intellectual Disability and Their Interaction Partners.

    Science.gov (United States)

    Griffiths, Colin; Smith, Martine

    2016-03-01

    People with severe and profound intellectual disability typically demonstrate a limited ability to communicate effectively. Most of their communications are non-verbal, often idiosyncratic and ambiguous. This article aims to identify the process that regulates communications of this group of people with others and to describe the methodological approach that was used to achieve this. In this qualitative study, two dyads consisting of a person with severe or profound intellectual and multiple disability and a teacher or carer were filmed as they engaged in school-based activities. Two 1-hour videotapes were transcribed and analysed using grounded theory. Attuning was identified within the theory proposed here as a central process that calibrates and regulates communication. Attuning is conceptualized as a bidirectional, dyadic communication process. Understanding this process may support more effective communication between people with severe or profound intellectual and multiple disability and their interaction partners. © 2015 John Wiley & Sons Ltd.

  9. Prevalence of Auditory Neuropathy in a Population of Children with Severe to Profound Hearing Loss

    Directory of Open Access Journals (Sweden)

    Nader Saki

    2013-04-01

    Full Text Available Background: The purpose of this investigation is to determine auditory neuropathy in the students with severe to profound hearing losses in Ahwaz.Materials and Methods: In this cross-sectional study, 212 children of 7-11 year old with severe to profound hearing loss performed ordinary audiometric evaluations as well as ABR and OAE. The patients with normal DPOAE who had no record of acoustic reflex having normal ABR, were considered as the patients with auditory neuropathy. Results: The neuropathic complication found in 14 children was appeared in 8 ones as one-sided (57.14% and in 6 ones (42.86% as two-sided. 68% of the patients as diagnosed had a very low Speech Discrimination Score (SDS.Conclusion: we must be very vigilant in auditory neuropathy diagnosis for the purpose to be successful in appropriate treatment of severe to profound hearing losses.

  10. Interleukin-6 markedly decreases skeletal muscle protein turnover and increases nonmuscle amino acid utilization in healthy individuals

    DEFF Research Database (Denmark)

    van Hall, Gerrit; Steensberg, Adam; Fischer, Christian

    2008-01-01

    CONTEXT: IL-6 is a key modulator of immune function and suggested to be involved in skeletal muscle wasting as seen in sepsis. OBJECTIVE: Our objective was to determine the role of IL-6 in human in vivo systemic and skeletal muscle amino acid metabolism and protein turnover. SUBJECTS AND METHODS...... synthesis was more suppressed than breakdown, causing a small increase in net muscle protein breakdown. Furthermore, rhIL-6 decreased arterial amino acid concentration with 20-40%, despite the increase net release from muscle. CONCLUSIONS: We demonstrated that IL-6 profoundly alters amino acid turnover....... A substantial decrease in plasma amino acids was observed with a concomitant 50% decrease in muscle protein turnover, however, modest increase in net muscle degradation. We hypothesize that the profound reduction in muscle protein turnover and modest increase in net degradation are primarily caused...

  11. Weak localization of seismic waves

    International Nuclear Information System (INIS)

    Larose, E.; Margerin, L.; Tiggelen, B.A. van; Campillo, M.

    2004-01-01

    We report the observation of weak localization of seismic waves in a natural environment. It emerges as a doubling of the seismic energy around the source within a spot of the width of a wavelength, which is several tens of meters in our case. The characteristic time for its onset is the scattering mean-free time that quantifies the internal heterogeneity

  12. On Weak-BCC-Algebras

    Science.gov (United States)

    Thomys, Janus; Zhang, Xiaohong

    2013-01-01

    We describe weak-BCC-algebras (also called BZ-algebras) in which the condition (x∗y)∗z = (x∗z)∗y is satisfied only in the case when elements x, y belong to the same branch. We also characterize ideals, nilradicals, and nilpotent elements of such algebras. PMID:24311983

  13. Voltage Weak DC Distribution Grids

    NARCIS (Netherlands)

    Hailu, T.G.; Mackay, L.J.; Ramirez Elizondo, L.M.; Ferreira, J.A.

    2017-01-01

    This paper describes the behavior of voltage weak DC distribution systems. These systems have relatively small system capacitance. The size of system capacitance, which stores energy, has a considerable effect on the value of fault currents, control complexity, and system reliability. A number of

  14. The structure of weak interaction

    International Nuclear Information System (INIS)

    Zee, A.

    1977-01-01

    The effect of introducing righthanded currents on the structure of weak interaction is discussed. The ΔI=1/2 rule is in the spotlight. The discussion provides an interesting example in which the so-called Iizuka-Okubo-Zweing rule is not only evaded, but completely negated

  15. Coverings, Networks and Weak Topologies

    Czech Academy of Sciences Publication Activity Database

    Dow, A.; Junnila, H.; Pelant, Jan

    2006-01-01

    Roč. 53, č. 2 (2006), s. 287-320 ISSN 0025-5793 R&D Projects: GA ČR GA201/97/0216 Institutional research plan: CEZ:AV0Z10190503 Keywords : Banach spaces * weak topologies * networks topologies Subject RIV: BA - General Mathematics

  16. Weak differentiability of product measures

    NARCIS (Netherlands)

    Heidergott, B.F.; Leahu, H.

    2010-01-01

    In this paper, we study cost functions over a finite collection of random variables. For these types of models, a calculus of differentiation is developed that allows us to obtain a closed-form expression for derivatives where "differentiation" has to be understood in the weak sense. The technique

  17. Weak lensing and dark energy

    International Nuclear Information System (INIS)

    Huterer, Dragan

    2002-01-01

    We study the power of upcoming weak lensing surveys to probe dark energy. Dark energy modifies the distance-redshift relation as well as the matter power spectrum, both of which affect the weak lensing convergence power spectrum. Some dark-energy models predict additional clustering on very large scales, but this probably cannot be detected by weak lensing alone due to cosmic variance. With reasonable prior information on other cosmological parameters, we find that a survey covering 1000 sq deg down to a limiting magnitude of R=27 can impose constraints comparable to those expected from upcoming type Ia supernova and number-count surveys. This result, however, is contingent on the control of both observational and theoretical systematics. Concentrating on the latter, we find that the nonlinear power spectrum of matter perturbations and the redshift distribution of source galaxies both need to be determined accurately in order for weak lensing to achieve its full potential. Finally, we discuss the sensitivity of the three-point statistics to dark energy

  18. Weak pion production from nuclei

    Indian Academy of Sciences (India)

    effect of Pauli blocking, Fermi motion and renormalization of weak ∆ properties ... Furthermore, the angular distribution and the energy distribution of ... Here ψα(p ) and u(p) are the Rarita Schwinger and Dirac spinors for ∆ and nucleon.

  19. Weak transitions in 44Ca

    International Nuclear Information System (INIS)

    Tauhata, L.; Marques, A.

    1972-01-01

    Energy levels and gamma radiation transitions of Ca 44 are experimentally determined, mainly the weak transition at 564 KeV and 728 KeV. The decay scheme and the method used (coincidence with Ge-Li detector) are also presented [pt

  20. Patients with polymyositis show changes in muscle protein charges

    DEFF Research Database (Denmark)

    Bartels, E M; Jacobsen, Søren; Rasmussen, L

    1989-01-01

    Polymyositis (PM) appears with indolent proximal muscle weakness and is an inflammatory disease with breakdown of muscle cells. In our study the protein charge concentrations of the contractile proteins in the A and I bands were determined, applying a microelectrode technique. Patients with PM sh...

  1. Repair of gluteus medius muscle avulsion following transgluteal hip replacement

    NARCIS (Netherlands)

    Groot, Diederik; van Ooij, Andre; Haverkamp, Daniel; Morrenhof, Wim

    2011-01-01

    During a transgluteal approach to the hip joint the anterior part of the gluteus medius and minimus muscles are detached and subsequently reattached to the greater trochanter. Avulsion or rupture of these muscles may result in weak abduction, pain and/or instability. 15 patients with these symptoms

  2. Classroom Procedures for the Measurement of Behavior State among Students with Profound Disabilities.

    Science.gov (United States)

    Guy, Barbara; And Others

    1993-01-01

    This study examined whether teacher-implemented classroom measurement procedures of short duration are as reliable as methods used in research studies to determine behavior state (e.g., awake active, awake inactive, asleep, drowsed) of six students with profound mental disabilities. Results indicated that more frequent but briefer measurements…

  3. The Role of Sound in Residential Facilities for People With Profound Intellectual and Multiple Disabilities

    NARCIS (Netherlands)

    van den Bosch, Kirsten A.; Andringa, Tjeerd C.; Baskent, Deniz; Vlaskamp, Carla

    2016-01-01

    Attention to the auditory environment of people with profound intellectual and multiple disabilities (PIMD) is limited, both in research and practice. As there is a dynamic interplay between the quality of the auditory environment and well-being, a study was undertaken to test the validity of the

  4. The Influence of Phonological Mechanisms in Written Spelling of Profoundly Deaf Children

    Science.gov (United States)

    Colombo, Lucia; Arfe, Barbara; Bronte, Tiziana

    2012-01-01

    In the present study, the effect of phonological and working memory mechanisms involved in spelling Italian single words was explored in two groups of children matched for grade level: a group of normally hearing children and a group of pre-verbally deaf children, with severe-to-profound hearing loss. Three-syllable and four-syllable familiar…

  5. Self-Concept of Severely to Profoundly Hearing-Impaired Children.

    Science.gov (United States)

    Warren, Charlotte; Hasenstab, Suzanne

    1986-01-01

    A study examined demographic, impairment-related, and parental variables that best predicted self-concept among 49 severely to profoundly hearing-impaired 5- to 11-year-olds. A strong relationship was observed between self-concept and parental indulgence, parental rejection, parental protection, parental discipline, and extent of language…

  6. A Data Based Multidimensional Oral Hygiene Curriculum for the Severely and Profoundly Handicapped.

    Science.gov (United States)

    Feldman, David; Elliott, Thomas A.

    A multidimensional oral hygiene curriculum appropriate for the moderately to profoundly retarded and composed of tooth brushing, flossing, and gum stimulation/massage is proposed. Task analyses are included for manual tooth brushing, utilizing an oral irrigation appliance, manual flossing, and use of an adaptive aid for flossing. Also provided are…

  7. Operationalizing quality of life for people with profound multiple disabilities : a Delphi study

    NARCIS (Netherlands)

    Petry, K.; Maes, B.; Vlaskamp, C.

    Background: In a recent study, we constructed an item pool that contains items on the quality of life (QOL) and related aspects of support of people with profound multiple disabilities (PMD). In the present study, a panel of experts assessed the content and the structure of this item pool in order

  8. The Curriculum for Children with Severe and Profound Learning Difficulties at Stephen Hawking School

    Science.gov (United States)

    Rayner, Matthew

    2011-01-01

    The increasing number of children with profound and multiple learning difficulties means that many schools for children with severe learning difficulties are having to review the curriculum that they offer. In addition, these schools are continuing to question whether a subject-based approach, in line with the National Curriculum, is the most…

  9. Reversal of rocuronium-induced profound neuromuscular block by sugammadex in Duchenne muscular dystrophy.

    NARCIS (Netherlands)

    Boer, H.D. de; Egmond, J. van; Booij, L.H.D.J.; Driessen, J.J.

    2009-01-01

    A case is reported in which a child with Duchenne muscular dystrophy received a dose of sugammadex to reverse a rocuronium-induced profound neuromuscular block. Sugammadex is the first selective relaxant binding agent and reverses rocuronium- and vecuronium-induced neuromuscular block. A fast and

  10. Connections that Count: Brain-Computer Interface Enables the Profoundly Paralyzed to Communicate

    Science.gov (United States)

    ... Home Current Issue Past Issues Connections that Count: Brain-Computer Interface Enables the Profoundly Paralyzed to Communicate Past Issues / ... of this page please turn Javascript on. A brain-computer interface (BCI) system This brain-computer interface (BCI) system ...

  11. A Preliminary Investigation of the Suitability of Aquatics for People with Severe and Profound Intellectual Disabilities

    Science.gov (United States)

    Aherne, Cian; Coughlan, Barry

    2017-01-01

    Background: Aquatics is an under-researched but possibly appropriate form of activity for people with severe to profound intellectual disabilities (SPIDs). Aim: The current pilot study investigates the suitability of an aquatics programme for service users with SPIDs. Method: Four service users with SPID completed an 8-12-session aquatics…

  12. Determining alertness in individuals with profound intellectual and multiple disabilities : the reliability of an observation list

    NARCIS (Netherlands)

    Munde, V.S.; Vlaskamp, C.; Ruijssenaars, A.J.J.M.; Nakken, H.

    In the support of individuals with profound intellectual and multiple disabilities (PIMD), assessing the level of alertness is a recurring issue for parents and other direct support persons. Although observations show clear advantages above and beyond other assessment methods, there are problems

  13. Social Peer Interactions in Persons with Profound Intellectual and Multiple Disabilities: A Literature Review

    Science.gov (United States)

    Nijs, Sara; Maes, Bea

    2014-01-01

    Social interactions may positively influence developmental and quality of life outcomes. Research in persons with profound intellectual and multiple disabilities (PIMD) mostly investigated interactions with caregivers. This literature review focuses on peer interactions of persons with PIMD. A computerized literature search of three databases was…

  14. Normality and Impairment following Profound Early Institutional Deprivation: A Longitudinal follow-up into Early Adolescence

    Science.gov (United States)

    Kreppner, Jana M.; Rutter, Michael; Beckett, Celia; Castle, Jenny; Colvert, Emma; Groothues, Christine; Hawkins, Amanda; O'Connor, Thomas G.; Stevens, Suzanne; Sonuga-Barke, Edmund J. S.

    2007-01-01

    Longitudinal analyses on normal versus impaired functioning across 7 domains were conducted in children who had experienced profound institutional deprivation up to the age of 42 months and were adopted from Romania into U.K. families. Comparisons were made with noninstitutionalized children adopted from Romania and with nondeprived within-U.K.…

  15. The pros and cons of inclusive education for children with Profound Intellectual and Multiple Disabilities

    NARCIS (Netherlands)

    Everaarts, Sanne; de Boer, Anke; van der Putten, Annette; Minnaert, Alexander

    2016-01-01

    Aim: Although children with disabilities have the right to be included into the school system, children with Profound Intellectual and Multiple Disabilities (PIMD) are often not included. The aim of this study is to gather knowledge about inclusive education for children with PIMD by identifying

  16. Bereavement and Loss: Developing a Memory Box to Support a Young Woman with Profound Learning Disabilities

    Science.gov (United States)

    Young, Hannah; Garrard, Brenda

    2016-01-01

    Supporting bereaved people with profound learning disabilities still remains an under-researched area. Moreover, the barriers of communication and disenfranchised grief mean that they often do not receive the support they require, leading to emotional and behavioural difficulties. This article describes research using a case study design, which…

  17. Visual impairments in people with severe and profound multiple disabilities: An inventory of visual functioning

    NARCIS (Netherlands)

    van den Broek, E.G.C.; Janssen, C.G.C.; van Ramshorst, T.; Deen, L.

    2006-01-01

    Background: The prevalence of visual impairments in people with severe and profound multiple disabilities (SPMD) is the subject of considerable debate and is difficult to assess. Methods: In a typical Dutch care organization, all clients with SPMD (n = 76) participated in the study and specific

  18. Is more profound knowledge in the field of radiation nece--ssary for doctors

    International Nuclear Information System (INIS)

    Klener, V.

    1981-01-01

    Reasons are listed why doctors should have more profound knowledge in the field of radiation. Despite imperceptibility by human senses of ionizing radiation, the parameters characterizing irradiation can accurately be measured. The units of the said parameters are listed and characterized and the relationships are explained of the radiation dose and non-stochastic and stochastic radiation effects. (Ha)

  19. HEART RATE AND PHYSICAL ACTIVITY PATTERNS IN PERSONS WITH PROFOUND INTELLECTUAL AND MULTIPLE DISABILITIES

    NARCIS (Netherlands)

    Waninge, Aly; van der Putten, Annette A. J.; Stewart, Roy E.; Steenbergen, Bert; van Wijck, Ruud; van der Schans, Cees P.

    2013-01-01

    Because physical fitness and health are related to physical activity, it is important to gain an insight into the physical activity levels of persons with profound intellectual and multiple disabilities (PIMD). The purpose of this study was to examine heart rate patterns to measure the activity

  20. Attentional Processes in Interactions between People with Profound Intellectual and Multiple Disabilities and Direct Support Staff

    Science.gov (United States)

    Ine, Hostyn; Heleen, Neerinckx; Bea, Maes

    2011-01-01

    Few studies have examined joint attention in interactions with persons with profound intellectual and multiple disabilities (PIMD), despite its important role in high-quality interaction. The purpose of this study is to describe the attention-directing behaviours of persons with PIMD and their direct support staff and the attention episodes…

  1. Interaction between Persons with Profound Intellectual and Multiple Disabilities and Their Partners: A Literature Review

    Science.gov (United States)

    Hostyn, Ine; Maes, Bea

    2009-01-01

    Background: High quality interactions are of crucial importance for quality of life of persons with profound intellectual and multiple disabilities (PIMD). This literature review describes and synthesises studies addressing the interaction between persons with PIMD and their partners. Method: A computerised literature search using defined…

  2. A Vibrotactile Interface to Motivate Movement for Children with Severe to Profound Disabilities

    DEFF Research Database (Denmark)

    Manresa-Yee, Cristina; Morrison, Ann; Larsen, Jeppe Veirum

    2014-01-01

    V-Sense is a vibrotactile interface that encourages children with severe or profound cognitive, sensory and physical impairments to move. The interface makes use of touch, in particular vibrations, as a supportive function to motivate users' actions. Specifically, we propose a vibrotactile...

  3. Attuning: A Communication Process between People with Severe and Profound Intellectual Disability and Their Interaction Partners

    Science.gov (United States)

    Griffiths, Colin; Smith, Martine

    2016-01-01

    Background: People with severe and profound intellectual disability typically demonstrate a limited ability to communicate effectively. Most of their communications are non-verbal, often idiosyncratic and ambiguous. This article aims to identify the process that regulates communications of this group of people with others and to describe the…

  4. Electrically controllable artificial PAN muscles

    Science.gov (United States)

    Salehpoor, Karim; Shahinpoor, Mohsen; Mojarrad, Mehran

    1996-02-01

    Artificial muscles made with polyacrylonitrile (PAN) fibers are traditionally activated in electrolytic solution by changing the pH of the solution by the addition of acids and/or bases. This usually consumes a considerable amount of weak acids or bases. Furthermore, the synthetic muscle (PAN) itself has to be impregnated with an acid or a base and must have an appropriate enclosure or provision for waste collection after actuation. This work introduces a method by which the PAN muscle may be elongated or contracted in an electric field. We believe this is the first time that this has been achieved with PAN fibers as artificial muscles. In this new development the PAN muscle is first put in close contact with one of the two platinum wires (electrodes) immersed in an aqueous solution of sodium chloride. Applying an electric voltage between the two wires changes the local acidity of the solution in the regions close to the platinum wires. This is because of the ionization of sodium chloride molecules and the accumulation of Na+ and Cl- ions at the negative and positive electrode sites, respectively. This ion accumulation, in turn, is accompanied by a sharp increase and decrease of the local acidity in regions close to either of the platinum wires, respectively. An artificial muscle, in close contact with the platinum wire, because of the change in the local acidity will contract or expand depending on the polarity of the electric field. This scheme allows the experimenter to use a fixed flexible container of an electrolytic solution whose local pH can be modulated by an imposed electric field while the produced ions are basically trapped to stay in the neighborhood of a given electrode. This method of artificial muscle activation has several advantages. First, the need to use a large quantity of acidic or alkaline solutions is eliminated. Second, the use of a compact PAN muscular system is facilitated for applications in active musculoskeletal structures. Third, the

  5. Evaluation of muscle MRI in amyotrophic lateral sclerosis

    International Nuclear Information System (INIS)

    Baba, Yuri; Kuroiwa, Yoshiyuki

    2005-01-01

    Various objective measurements can be used to diagnose amyotrophic lateral sclerosis (ALS). T2-weighted brain MRI images revealed high signal areas at the posterior limb of the internal capsules in ALS patients. Recently, muscle MRI proved useful to evaluate abnormalities of the muscle in myositis patients. Therefore, in the present study, we examined muscle MRI of leg muscles in ALS patients, and correlated MRI with functional measurements, such as muscle strength, and compound muscle action potential amplitude of the tibialis anterior (TA) after stimulation of the peroneal nerve. The subjects consisted of 10 ALS patients (7 males and 3 females), ranging in age from 49 to 87. Neurologic symptoms at the onset of ALS consisted of bulbar dysfunction in one patient, upper extremity involvement in three patients, and lower extremity involvement in six patients. Muscle MRI of the legs was performed in 9 (ALS patients. A peripheral nerve conduction study was performed on the peroneal nerve, with the recording electrode over the TA. The T2-weighted muscle MRI images revealed high signal aeras in the muscle in six ALS patients, whose muscle weakness existed predominantly in the lower extremities. Extracellular fluid accumulation has been proposed to be responsible for the signal increase of denervated muscles on T2-weighted muscle MRI images. We assume that muscle MRI is useful to demonstrate the distribution of muscle involvement in ALS patients and to assess the disease's stage. (author)

  6. Light weakly interacting massive particles

    Science.gov (United States)

    Gelmini, Graciela B.

    2017-08-01

    Light weakly interacting massive particles (WIMPs) are dark matter particle candidates with weak scale interaction with the known particles, and mass in the GeV to tens of GeV range. Hints of light WIMPs have appeared in several dark matter searches in the last decade. The unprecedented possible coincidence into tantalizingly close regions of mass and cross section of four separate direct detection experimental hints and a potential indirect detection signal in gamma rays from the galactic center, aroused considerable interest in our field. Even if these hints did not so far result in a discovery, they have had a significant impact in our field. Here we review the evidence for and against light WIMPs as dark matter candidates and discuss future relevant experiments and observations.

  7. (Weakly) three-dimensional caseology

    International Nuclear Information System (INIS)

    Pomraning, G.C.

    1996-01-01

    The singular eigenfunction technique of Case for solving one-dimensional planar symmetry linear transport problems is extended to a restricted class of three-dimensional problems. This class involves planar geometry, but with forcing terms (either boundary conditions or internal sources) which are weakly dependent upon the transverse spatial variables. Our analysis involves a singular perturbation about the classic planar analysis, and leads to the usual Case discrete and continuum modes, but modulated by weakly dependent three-dimensional spatial functions. These functions satisfy parabolic differential equations, with a different diffusion coefficient for each mode. Representative one-speed time-independent transport problems are solved in terms of these generalised Case eigenfunctions. Our treatment is very heuristic, but may provide an impetus for more rigorous analysis. (author)

  8. Molecular mechanisms of muscle atrophy in myotonic dystrophies

    OpenAIRE

    Timchenko, Lubov

    2013-01-01

    Myotonic dystrophy type 1 (DM1) and myotonic dystrophy type 2 (DM2) are multisystemic diseases that primarily affect skeletal muscle, causing myotonia, muscle atrophy, and muscle weakness. DM1 and DM2 pathologies are caused by expansion of CTG and CCTG repeats in non-coding regions of the genes encoding myotonic dystrophy protein kinase (DMPK) and Zinc finger protein 9 (ZNF9) respectively. These expansions cause DM pathologies through accumulation of mutant RNAs that alter RNA metabolism in p...

  9. History of the weak interactions

    International Nuclear Information System (INIS)

    Lee, T.D.

    1987-01-01

    At the 'Jackfest' marking the 65th birthday of Jack Steinberger (see July/August 1986 issue, page 29), T.D. Lee gave an account of the history of the weak interactions. This edited version omits some of Lee's tributes to Steinberger, but retains the impressive insight into the subtleties of a key area of modern physics by one who played a vital role in its development. (orig./HSI).

  10. Weak neutral-current interactions

    International Nuclear Information System (INIS)

    Barnett, R.M.

    1978-08-01

    The roles of each type of experiment in establishing uniquely the values of the the neutral-current couplings of u and d quarks are analyzed together with their implications for gauge models of the weak and electromagnetic interactions. An analysis of the neutral-current couplings of electrons and of the data based on the assumption that only one Z 0 boson exists is given. Also a model-independent analysis of parity violation experiments is discussed. 85 references

  11. Submanifolds weakly associated with graphs

    Indian Academy of Sciences (India)

    A CARRIAZO, L M FERN ´ANDEZ and A RODRÍGUEZ-HIDALGO. Department of Geometry and Topology, ..... by means of trees (connected graphs without cycles) and forests (disjoint unions of trees, see [6]) given in [3], by extending it to weak ... CR-submanifold. In this case, every tree is a K2. Finally, Theorem 3.8 of [3] can ...

  12. Elderly persons with ICU-acquired weakness: the potential role for β-hydroxy-β-methylbutyrate (HMB) supplementation?

    Science.gov (United States)

    Rahman, Adam; Wilund, Kenneth; Fitschen, Peter J; Jeejeebhoy, Khursheed; Agarwala, Ravi; Drover, John W; Mourtzakis, Marina

    2014-07-01

    Intensive care unit (ICU)-acquired weakness is common and characterized by muscle loss, weakness, and paralysis. It is associated with poor short-term outcomes, including increased mortality, but the consequences of reduced long-term outcomes, including decreased physical function and quality of life, can be just as devastating. ICU-acquired weakness is particularly relevant to elderly patients who are increasingly consuming ICU resources and are at increased risk for ICU-acquired weakness and complications, including mortality. Elderly patients often enter critical illness with reduced muscle mass and function and are also at increased risk for accelerated disuse atrophy with acute illness. Increasingly, intensivists and researchers are focusing on strategies and therapies aimed at improving long-term neuromuscular function. β-Hydroxy-β-methylbutyrate (HMB), an ergogenic supplement, has shown efficacy in elderly patients and certain clinical populations in counteracting muscle loss. The present review discusses ICU-acquired weakness, as well as the unique physiology of muscle loss and skeletal muscle function in elderly patients, and then summarizes the evidence for HMB in elderly patients and in clinical populations. We subsequently postulate on the potential role and strategies in studying HMB in elderly ICU patients to improve muscle mass and function. © 2013 American Society for Parenteral and Enteral Nutrition.

  13. Gossip and Distributed Kalman Filtering: Weak Consensus Under Weak Detectability

    Science.gov (United States)

    Kar, Soummya; Moura, José M. F.

    2011-04-01

    The paper presents the gossip interactive Kalman filter (GIKF) for distributed Kalman filtering for networked systems and sensor networks, where inter-sensor communication and observations occur at the same time-scale. The communication among sensors is random; each sensor occasionally exchanges its filtering state information with a neighbor depending on the availability of the appropriate network link. We show that under a weak distributed detectability condition: 1. the GIKF error process remains stochastically bounded, irrespective of the instability properties of the random process dynamics; and 2. the network achieves \\emph{weak consensus}, i.e., the conditional estimation error covariance at a (uniformly) randomly selected sensor converges in distribution to a unique invariant measure on the space of positive semi-definite matrices (independent of the initial state.) To prove these results, we interpret the filtered states (estimates and error covariances) at each node in the GIKF as stochastic particles with local interactions. We analyze the asymptotic properties of the error process by studying as a random dynamical system the associated switched (random) Riccati equation, the switching being dictated by a non-stationary Markov chain on the network graph.

  14. Oral Communication Development in Severe to Profound Hearing Impaired Children After Receiving Aural Habilitation

    Directory of Open Access Journals (Sweden)

    Soleimani Farin

    2009-10-01

    Full Text Available Communication, cognition, language, and speech are interrelated and develop together. It should come as no surprise to us that the key to intervention with deaf children is to establish, as early as possible, a functional communication system for the child and the parents. Early intervention programs need to be multidisciplinary, technologically sound and most important, it should take cognizance of the specific context (community, country in which the child and family function. The main aim of this study was to obtain oral communication development regarding current status of the intervention (aural habilitation and speech therapyfor children with severe to profound hearing impairment in Iran. A prospective longitudinal study was undertaken on a consecutive group of children with severe to profound deafness. Nine severe to profound hearing-impaired children out of the primer 42 cases, who were detected below two years old, had been selected in the previous study to receive aural habilitation. The average of their speech intelligibility scores was near 70% at age 6, which was accounted as poor oral communication and only two of them were able to communicate by spoken language. An integrated intervention services continued again for one year and their oral communication skill was assessed by their speech intelligibility. The intelligibility test of children was recorded on audio-tape, when they read 10 questions such as where is your home. This can be answered only in one word. Each tape was presented to10 normal hearing listeners, and their task was to write down, the answers in Persian orthography. At the beginning (at age 6 the average speech intelligibility score of these children was 72% and only two of them had score of 90% and 100%. At age 7, all of the severe groups were over 90%, and only two profound ones achieved the score of 48% and 62%. All of severe groups develop oral communication, but profound ones had a semi-intelligible speech

  15. [Long-term clinical course of sequelae in patients with neonatal anoxic encephalopathy resulting in profound mental retardation and motor disturbance].

    Science.gov (United States)

    Ishizaki, A; Kubota, M; Fueki, N; Shinozaki, M; Kurata, K; Takei, M; Sakamoto, K

    1993-01-01

    A long-term observation has been made in 58 patients (30 males and 28 females) with severe sequelae of neonatal anoxic encephalopathy. They aged from 8 months to 65 years. All of them had motor disturbances and profound mental retardation. Motor function was improved in 4 patients with aging. In contrast, motor activity deteriorated in 11 cases, of which 4 showed a mental regression. Among them, patients who had originally better motor ability than sitting were likely to deteriorate by uncontrollable epilepsy and/or excessive administration of anticonvulsants. Regression of the patients with worse motor ability like bedridden appeared to attributable hypertonia of muscles and bodily deformation. Fifteen cases showed an exacerbation of general condition which originated predominantly to respiratory distress. Twelve patients died including 6 exacerbated cases. Exacerbation or death may have occurred frequently in specific periods of infancy, adolescence and youth with the patients who showed very low motor function such as bedridden and no locomotion.

  16. Hyperglycemia-induced diaphragm weakness is mediated by oxidative stress

    Science.gov (United States)

    2014-01-01

    Introduction A major consequence of ICU-acquired weakness (ICUAW) is diaphragm weakness, which prolongs the duration of mechanical ventilation. Hyperglycemia (HG) is a risk factor for ICUAW. However, the mechanisms underlying HG-induced respiratory muscle weakness are not known. Excessive reactive oxygen species (ROS) injure multiple tissues during HG, but only one study suggests that excessive ROS generation may be linked to HG-induced diaphragm weakness. We hypothesized that HG-induced diaphragm dysfunction is mediated by excessive superoxide generation and that administration of a specific superoxide scavenger, polyethylene glycol superoxide dismutase (PEG-SOD), would ameliorate these effects. Methods HG was induced in rats using streptozotocin (60 mg/kg intravenously) and the following groups assessed at two weeks: controls, HG, HG + PEG-SOD (2,000U/kg/d intraperitoneally for seven days), and HG + denatured (dn)PEG-SOD (2000U/kg/d intraperitoneally for seven days). PEG-SOD and dnPEG-SOD were administered on day 8, we measured diaphragm specific force generation in muscle strips, force-pCa relationships in single permeabilized fibers, contractile protein content and indices of oxidative stress. Results HG reduced diaphragm specific force generation, altered single fiber force-pCa relationships, depleted troponin T, and increased oxidative stress. PEG-SOD prevented HG-induced reductions in diaphragm specific force generation (for example 80 Hz force was 26.4 ± 0.9, 15.4 ± 0.9, 24.0 ± 1.5 and 14.9 ± 0.9 N/cm2 for control, HG, HG + PEG-SOD, and HG + dnPEG-SOD groups, respectively, P hyperglycemia-induced diaphragm dysfunction. This new mechanistic information could explain how HG alters diaphragm function during critical illness. PMID:24886999

  17. Why is gravity so weak?

    International Nuclear Information System (INIS)

    Goradia, S.G.

    2006-01-01

    Why is gravity weak? Gravity is plagued with this and many other questions. After decades of exhausting work we do not have a clear answer. In view of this fact it will be shown in the following pages that there are reasons for thinking that gravity is just a composite force consisting of the long-range manifestations of short range nuclear forces that are too tiny to be measured at illuminated or long ranges by particle colliders. This is consistent with Einstein's proposal in 1919

  18. Weak decays of heavy quarks

    International Nuclear Information System (INIS)

    Gaillard, M.K.

    1978-08-01

    The properties that may help to identify the two additional quark flavors that are expected to be discovered. These properties are lifetime, branching ratios, selection rules, and lepton decay spectra. It is also noted that CP violation may manifest itself more strongly in heavy particle decays than elsewhere providing a new probe of its origin. The theoretical progress in the understanding of nonleptonic transitions among lighter quarks, nonleptonic K and hyperon decay amplitudes, omega minus and charmed particle decay predictions, and lastly the Kobayashi--Maskawa model for the weak coupling of heavy quarks together with the details of its implications for topology and bottomology are treated. 48 references

  19. Weak consistency and strong paraconsistency

    Directory of Open Access Journals (Sweden)

    Gemma Robles

    2009-11-01

    Full Text Available In a standard sense, consistency and paraconsistency are understood as, respectively, the absence of any contradiction and as the absence of the ECQ (“E contradictione quodlibet” rule that allows us to conclude any well formed formula from any contradiction. The aim of this paper is to explain the concepts of weak consistency alternative to the standard one, the concepts of paraconsistency related to them and the concept of strong paraconsistency, all of which have been defined by the author together with José M. Méndez.

  20. Electromagnetic weak turbulence theory revisited

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, P. H. [IPST, University of Maryland, College Park, Maryland 20742 (United States); Ziebell, L. F. [Instituto de Fisica, UFRGS, Porto Alegre, RS (Brazil); Gaelzer, R.; Pavan, J. [Instituto de Fisica e Matematica, UFPel, Pelotas, RS (Brazil)

    2012-10-15

    The statistical mechanical reformulation of weak turbulence theory for unmagnetized plasmas including fully electromagnetic effects was carried out by Yoon [Phys. Plasmas 13, 022302 (2006)]. However, the wave kinetic equation for the transverse wave ignores the nonlinear three-wave interaction that involves two transverse waves and a Langmuir wave, the incoherent analogue of the so-called Raman scattering process, which may account for the third and higher-harmonic plasma emissions. The present paper extends the previous formalism by including such a term.

  1. The relationship between hamstring length and gluteal muscle strength in individuals with sacroiliac joint dysfunction.

    Science.gov (United States)

    Massoud Arab, Amir; Reza Nourbakhsh, Mohammad; Mohammadifar, Ali

    2011-02-01

    It has been suggested that tight hamstring muscle, due to its anatomical connections, could be a compensatory mechanism for providing sacroiliac (SI) joint stability in patients with gluteal muscle weakness and SIJ dysfunction. The purpose of this study was to determine the relationship between hamstring muscle length and gluteal muscle strength in subjects with sacroiliac joint dysfunction. A total of 159 subjects with and without low back pain (LBP) between the ages of 20 and 65 years participate in the study. Subjects were categorized into three groups: LBP without SIJ involvement (n = 53); back pain with SIJ dysfunction (n = 53); and no low back pain (n = 53). Hamstring muscle length and gluteal muscle strength were measured in all subjects. The number of individuals with gluteal weakness was significantly (P = 0.02) higher in subjects with SI joint dysfunction (66%) compared to those with LBP without SI joint dysfunctions (34%). In pooled data, there was no significant difference (P = 0.31) in hamstring muscle length between subjects with SI joint dysfunction and those with back pain without SI involvement. In subjects with SI joint dysfunction, however, those with gluteal muscle weakness had significantly (P = 0.02) shorter hamstring muscle length (mean = 158±11°) compared to individuals without gluteal weakness (mean = 165±10°). There was no statistically significant difference (P>0.05) in hamstring muscle length between individuals with and without gluteal muscle weakness in other groups. In conclusion, hamstring tightness in subjects with SI joint dysfunction could be related to gluteal muscle weakness. The slight difference in hamstring muscle length found in this study, although statistically significant, was not sufficient for making any definite conclusions. Further studies are needed to establish the role of hamstring muscle in SI joint stability.

  2. Reversal of profound vecuronium-induced neuromuscular block under sevoflurane anesthesia: sugammadex versus neostigmine.

    Directory of Open Access Journals (Sweden)

    Lemmens Hendrikus JM

    2010-09-01

    Full Text Available Abstract Background Acetylcholinesterase inhibitors cannot rapidly reverse profound neuromuscular block. Sugammadex, a selective relaxant binding agent, reverses the effects of rocuronium and vecuronium by encapsulation. This study assessed the efficacy of sugammadex compared with neostigmine in reversal of profound vecuronium-induced neuromuscular block under sevoflurane anesthesia. Methods Patients aged ≥18 years, American Society of Anesthesiologists class 1-4, scheduled to undergo surgery under general anesthesia were enrolled in this phase III, multicenter, randomized, safety-assessor blinded study. Sevoflurane anesthetized patients received vecuronium 0.1 mg/kg for intubation, with maintenance doses of 0.015 mg/kg as required. Patients were randomized to receive sugammadex 4 mg/kg or neostigmine 70 μg/kg with glycopyrrolate 14 μg/kg at 1-2 post-tetanic counts. The primary efficacy variable was time from start of study drug administration to recovery of the train-of-four ratio to 0.9. Safety assessments included physical examination, laboratory data, vital signs, and adverse events. Results Eighty three patients were included in the intent-to-treat population (sugammadex, n = 47; neostigmine, n = 36. Geometric mean time to recovery of the train-of-four ratio to 0.9 was 15-fold faster with sugammadex (4.5 minutes compared with neostigmine (66.2 minutes; p Conclusions Recovery from profound vecuronium-induced block is significantly faster with sugammadex, compared with neostigmine. Neostigmine did not rapidly reverse profound neuromuscular block (Trial registration number: NCT00473694.

  3. Speech timing and working memory in profoundly deaf children after cochlear implantation

    OpenAIRE

    Burkholder, Rose A.; Pisoni, David B.

    2003-01-01

    Thirty-seven profoundly deaf children between 8- and 9-years-old with cochlear implants and a comparison group of normal-hearing children were studied to measure speaking rates, digit spans, and speech timing during digit span recall. The deaf children displayed longer sentence durations and pauses during recall and shorter digit spans compared to the normal-hearing children. Articulation rates, measured from sentence durations, were strongly correlated with immediate memory span in both norm...

  4. Epidemiology of fractures in people with severe and profound developmental disabilities

    Science.gov (United States)

    Glick, N.R.; Fischer, M.H.; Heisey, D.M.; Leverson, G.E.; Mann, D.C.

    2005-01-01

    Fractures are more prevalent among people with severe and profound developmental disabilities than in the general population. In order to characterize the tendency of these people to fracture, and to identify features that may guide the development of preventive strategies, we analyzed fracture epidemiology in people with severe and profound developmental disabilities who lived in a stable environment. Data from a 23-year longitudinal cohort registry of 1434 people with severe and profound developmental disabilities were analyzed to determine the effects of age, gender, mobility, bone fractured, month of fracture, and fracture history upon fracture rates. Eighty-five percent of all fractures involved the extremities. The overall fracture rate increased as mobility increased. In contrast, femoral shaft fracture risk was substantially higher in the least mobile [relative risk (RR), 10.36; 95% confidence interval (CI), 3.29-32.66] compared with the most mobile group. Although the overall fracture rate was not associated with age, the femoral shaft fractures decreased but hand/foot fractures increased with age. Overall fracture risk declined in August and September (RR, 0.70; 95% CI, 0.55-0.89), being especially prominent for tibial/fibular fractures (RR, 0.31; 95% CI, 0.13-0.70). Gender was not a factor in fracture risk. Two primary fracture mechanisms are apparent: one, largely associated with lack of weight-bearing in people with the least mobility, is exemplified by femoral fractures during non-traumatic events as simple as diapering or transfers; the other, probably due to movement- or fall-related trauma, is exemplified by hand/foot fractures in people who ambulate. The fracture experience of people with severe and profound developmental disabilities is unique and, because it differs qualitatively from postmenopausal osteoporosis, may require population-specific methods for assessing risk, for improving bone integrity, and for reduction of falls and accidents

  5. Weak interactions at the SSC

    International Nuclear Information System (INIS)

    Chanowitz, M.S.

    1986-03-01

    Prospects for the study of standard model weak interactions at the SSC are reviewed, with emphasis on the unique capability of the SSC to study the mechanism of electroweak symmetry breaking whether the associated new quanta are at the TeV scale or higher. Symmetry breaking by the minimal Higgs mechanism and by related strong interaction dynamical variants is summarized. A set of measurements is outlined that would calibrate the proton structure functions and the backgrounds to new physics. The ability to measure the three weak gauge boson vertex is found to complement LEP II, with measurements extending to larger Q 2 at a comparable statistical level in detectable decays. B factory physics is briefly reviewed as one example of a possible broad program of high statistics studies of sub-TeV scale phenomena. The largest section of the talk is devoted to the possible manifestations of symmetry breaking in the WW and ZZ production cross sections. Some new results are presented bearing on the ability to detect high mass WW and ZZ pairs. The principal conclusion is that although nonstandard model scenarios are typically more forgiving, the capability to study symmetry breaking in the standard model (and in related strong interaction dynamical variants) requires achieving the SSC design goals of √ s,L = 40Tev, 10 33 cm -2 sec -1 . 28 refs., 5 figs

  6. Probing supervoids with weak lensing

    Science.gov (United States)

    Higuchi, Yuichi; Inoue, Kaiki Taro

    2018-05-01

    The cosmic microwave background (CMB) has non-Gaussian features in the temperature fluctuations. An anomalous cold spot surrounded with a hot ring, called the Cold Spot, is one of such features. If a large underdense region (supervoid) resides towards the Cold Spot, we would be able to detect a systematic shape distortion in the images of background source galaxies via weak lensing effect. In order to estimate the detectability of such signals, we used the data of N-body simulations to simulate full-sky ray-tracing of source galaxies. We searched for a most prominent underdense region using the simulated convergence maps smoothed at a scale of 20° and obtained tangential shears around it. The lensing signal expected in a concordant Λ cold dark matter model can be detected at a signal-to-noise ratio S/N ˜ 3. If a supervoid with a radius of ˜200 h-1 Mpc and a density contrast δ0 ˜ -0.3 at the centre resides at a redshift z ˜ 0.2, on-going and near-future weak gravitational lensing surveys would detect a lensing signal with S/N ≳ 4 without resorting to stacking. From the tangential shear profile, we can obtain a constraint on the projected mass distribution of the supervoid.

  7. Like cognitive function, decision making across the life span shows profound age-related changes.

    Science.gov (United States)

    Tymula, Agnieszka; Rosenberg Belmaker, Lior A; Ruderman, Lital; Glimcher, Paul W; Levy, Ifat

    2013-10-15

    It has long been known that human cognitive function improves through young adulthood and then declines across the later life span. Here we examined how decision-making function changes across the life span by measuring risk and ambiguity attitudes in the gain and loss domains, as well as choice consistency, in an urban cohort ranging in age from 12 to 90 y. We identified several important age-related patterns in decision making under uncertainty: First, we found that healthy elders between the ages of 65 and 90 were strikingly inconsistent in their choices compared with younger subjects. Just as elders show profound declines in cognitive function, they also show profound declines in choice rationality compared with their younger peers. Second, we found that the widely documented phenomenon of ambiguity aversion is specific to the gain domain and does not occur in the loss domain, except for a slight effect in older adults. Finally, extending an earlier report by our group, we found that risk attitudes across the life span show an inverted U-shaped function; both elders and adolescents are more risk-averse than their midlife counterparts. Taken together, these characterizations of decision-making function across the life span in this urban cohort strengthen the conclusions of previous reports suggesting a profound impact of aging on cognitive function in this domain.

  8. Myopathy in Childhood Muscle-Specific Kinase Myasthenia Gravis.

    Science.gov (United States)

    Kirzinger, Lukas; Khomenko, Andrei; Schulte-Mattler, Wilhelm; Backhaus, Roland; Platen, Sabine; Schalke, Berthold

    2016-12-01

    Adult and pediatric patients suffering from MuSK (muscle-specific kinase) -antibody positive myasthenia gravis exhibit similar features to individuals with acetylcholine receptor (AChR) antibodies, but they differ in several characteristics such as a predominant bulbar, respiratory and neck weakness, a generally worse disease severity and a tendency to develop muscle atrophy. Muscle atrophy is a rare phenomenon that is usually restricted to the facial muscles. We describe a girl with MuSK-antibody positive myasthenia gravis who developed a myopathy with severe generalized muscular weakness, muscle atrophy, and myopathic changes on electromyography. This is the first published example of a generalized myopathic syndrome in myasthenia gravis. We review the relevant literature and discuss the hypothesis of a mitochondrial myopathy as a pathogenic mechanism in MuSK-antibody positive myasthenia gravis. Copyright © 2016 Elsevier Inc. All rights reserved.

  9. Prevalence of adult Pompe disease in patients with proximal myopathic syndrome and undiagnosed muscle biopsy.

    Science.gov (United States)

    Golsari, Amir; Nasimzadah, Arzoo; Thomalla, Götz; Keller, Sarah; Gerloff, Christian; Magnus, Tim

    2018-03-01

    We examined patients with limb-girdle muscle weakness and/or hyper-CKaemia and undiagnosed muscle biopsy for late onset Pompe disease (LOPD). Patients with an inconclusive limb-girdle muscle weakness who presented at our neuromuscular centre between 2005 and 2015 with undiagnosed muscle biopsies were examined by dry blood spot testing (DBS) including determination of the enzyme activity of acid alpha-glucosidase (GAA). In the case of depressed enzyme activity, additional gene testing of the GAA gene was carried out. Of the 340 evaluated muscle biopsies, 69 patients fulfilled the inclusion criteria and were examined with DBS. Among those patients, 76% showed a limb-girdle muscle weakness and 14% showed a hyper-CKaemia. A diagnosis of LOPD could be established in the case of two patients (2.9%) with reduced GAA enzyme activity and proof of mutations in the GAA gene. One of the two patients presents in the muscle biopsy suggestive features of Pompe disease including vacuoles with positive acid phosphatase reaction. In summary, our results show that a muscle biopsy can be helpful in identifying LOPD patients, but vacuolation with glycogen storage can also be absent. An inconspicuous muscle biopsy does not rule out Pompe disease. Consequently, all patients with limb-girdle muscle weakness should be examined by DBS before conducting a muscle biopsy. Copyright © 2017 Elsevier B.V. All rights reserved.

  10. Weak KAM for commuting Hamiltonians

    International Nuclear Information System (INIS)

    Zavidovique, M

    2010-01-01

    For two commuting Tonelli Hamiltonians, we recover the commutation of the Lax–Oleinik semi-groups, a result of Barles and Tourin (2001 Indiana Univ. Math. J. 50 1523–44), using a direct geometrical method (Stoke's theorem). We also obtain a 'generalization' of a theorem of Maderna (2002 Bull. Soc. Math. France 130 493–506). More precisely, we prove that if the phase space is the cotangent of a compact manifold then the weak KAM solutions (or viscosity solutions of the critical stationary Hamilton–Jacobi equation) for G and for H are the same. As a corollary we obtain the equality of the Aubry sets and of the Peierls barrier. This is also related to works of Sorrentino (2009 On the Integrability of Tonelli Hamiltonians Preprint) and Bernard (2007 Duke Math. J. 136 401–20)

  11. Esterase profile of human masseter muscle

    DEFF Research Database (Denmark)

    Kirkeby, S; Moe, D; Vilmann, H

    1988-01-01

    The esterase profile of fresh human masseter muscle was investigated by use of histochemistry and electrophoresis. The histochemical methods included reactions for alpha-naphthyl esterase, myofibrillar ATPase, reverse myofibrillar ATPase and succinic dehydrogenase. In frozen sections of the muscle...... the coloured reaction product for esterases was present both as a diffuse sarcoplasmic coloration and as distinct granules. The intensity of diffuse reaction was used to classify the muscle fibres as strongly, moderately and weakly reacting. The fibres with strong esterase activity belonged to Type I and ii......C. iM and Type II A fibres showed a moderate esterase reaction and Type II B fibres had a low activity. The electrophoretic gels stained for esterase activity showed that the human masseter muscle possesses a slow migrating double band with high enzyme activity and a cascade of faster migrating...

  12. Extraocular muscle function testing

    Science.gov (United States)

    ... medlineplus.gov/ency/article/003397.htm Extraocular muscle function testing To use the sharing features on this page, please enable JavaScript. Extraocular muscle function testing examines the function of the eye muscles. ...

  13. Fermi and the Theory of Weak Interactions

    Indian Academy of Sciences (India)

    IAS Admin

    Quantum Field Theory created by Dirac and used by Fermi to describe weak ... of classical electrodynamics (from which the electric field and magnetic field can be obtained .... Universe. However, thanks to weak interactions, this can be done.

  14. Nuclear beta decay and the weak interaction

    International Nuclear Information System (INIS)

    Kean, D.C.

    1975-11-01

    Short notes are presented on various aspects of nuclear beta decay and weak interactions including: super-allowed transitions, parity violation, interaction strengths, coupling constants, and the current-current formalism of weak interaction. (R.L.)

  15. The relationship between hamstring length and gluteal muscle strength in individuals with sacroiliac joint dysfunction

    OpenAIRE

    Massoud Arab, Amir; Reza Nourbakhsh, Mohammad; Mohammadifar, Ali

    2011-01-01

    It has been suggested that tight hamstring muscle, due to its anatomical connections, could be a compensatory mechanism for providing sacroiliac (SI) joint stability in patients with gluteal muscle weakness and SIJ dysfunction. The purpose of this study was to determine the relationship between hamstring muscle length and gluteal muscle strength in subjects with sacroiliac joint dysfunction. A total of 159 subjects with and without low back pain (LBP) between the ages of 20 and 65 years parti...

  16. Weakly distributive modules. Applications to supplement submodules

    Indian Academy of Sciences (India)

    Abstract. In this paper, we define and study weakly distributive modules as a proper generalization of distributive modules. We prove that, weakly distributive supplemented modules are amply supplemented. In a weakly distributive supplemented module every submodule has a unique coclosure. This generalizes a result of ...

  17. Effect of experimental hyperthyroidism on protein turnover in skeletal and cardiac muscle.

    Science.gov (United States)

    Carter, W J; Van Der Weijden Benjamin, W S; Faas, F H

    1980-10-01

    Since experimental hyperthyroidism reduces skeletal muscle mass while simultaneously increasing cardiac muscle mass, the effect of hyperthyroidism on muscle protein degradation was compared in skeletal and cardiac muscle. Pulse-labeling studies using (3H) leucine and (14C) carboxyl labeled aspartate and glutamate were carried out. Hyperthyroidism caused a 25%-29% increase in protein breakdown in both sarcoplasmic and myofibrillar fractions of skeletal muscle. Increased muscle protein degradation may be a major factor in the development of skeletal muscle wasting and weakness in hyperthyroidism. In contrast, protein breakdown appeared to be reduced 22% in the sarcoplasmic fraction of hyperthyroid heart muscle and was unchanged in the myofibrillar fraction. Possible reasons for the contrasting effects of hyperthyroidism on skeletal and cardiac muscle include increased sensitivity of the hyperthyroid heart to catecholamines, increased cardiac work caused by the hemodynamic effects of hyperthyroidism, and a different direct effect of thyroid hormone at the nuclear level in cardiac as opposed to skeletal muscle.

  18. EFFECT OF MUSCLE ENERGY TECHNIQUE ON FLEXIBILITY OF HAMSTRING AND CALF MUSCLES AND SPRINTING PERFORMANCE IN SPRINTERS

    Directory of Open Access Journals (Sweden)

    M. Prasad Naik

    2015-10-01

    Full Text Available Background: Muscle energy technique is used for restoring normal tone in hypertonic muscles, strengthening weak muscles, preparing muscle for subsequent stretching, one of the main uses of this method is to normalize joint range which may help in increase flexibility and performance in sprinters. The aim of the study is to evaluate the effect of muscle energy technique on flexibility of hamstrings and calf muscles and sprinting performance in sprinters. The objective of the study is to determine the muscle energy technique on hamstrings and calf muscle flexibility and sprinting performance in sprinters by using goniometer and timing of sprinting performance. Method: The study design is an experimental study in which 30 male sprinters were recruited in this study. The study sample included all male healthy sprinters, aged between 15 -30 years. All subjects received warm up, muscle energy technique and cool down exercises daily for a period of 6weeks.The outcome measures are 90°-90°popliteal angle for assessing hamstring flexibility and ROM of ankle joint for calf muscles by universal goniometer and sprinting performance time by using stopwatch. Results: Independent t-test and paired t- test are used to analyse the data. A significant difference was found between pre and post values of hamstring and calf muscle flexibility and sprinting performance after the analysis in this study. Conclusion: This study shows that there was a significant effect of MET on hamstring and calf muscle flexibility and sprinting performance.

  19. Function of skeletal muscle tissue formed after myoblast transplantation into irradiated mouse muscles.

    Science.gov (United States)

    Wernig, A; Zweyer, M; Irintchev, A

    2000-01-15

    1. Pretreatment of muscles with ionising radiation enhances tissue formation by transplanted myoblasts but little is known about the effects on muscle function. We implanted myoblasts from an expanded, male-donor-derived, culture (i28) into X-ray irradiated (16 Gy) or irradiated and damaged soleus muscles of female syngeneic mice (Balb/c). Three to 6 months later the isometric contractile properties of the muscles were studied in vitro, and donor nuclei were visualised in muscle sections with a Y chromosome-specific DNA probe. 2. Irradiated sham-injected muscles had smaller masses than untreated solei and produced less twitch and tetanic force (all by about 18 %). Injection of 106 myoblasts abolished these deficiencies and innervation appeared normal. 3. Cryodamage of irradiated solei produced muscle remnants with few (1-50) or no fibres. Additional myoblast implantation led to formation of large muscles (25 % above normal) containing numerous small-diameter fibres. Upon direct electrical stimulation, these muscles produced considerable twitch (53 % of normal) and tetanic forces (35 % of normal) but innervation was insufficient as indicated by weak nerve-evoked contractions and elevated ACh sensitivity. 4. In control experiments on irradiated muscles, reinnervation was found to be less complete after botulinum toxin paralysis than after nerve crush indicating that proliferative arrest of irradiated Schwann cells may account for the observed innervation deficits. 5. Irradiation appears to be an effective pretreatment for improving myoblast transplantation. The injected cells can even produce organised contractile tissue replacing whole muscle. However, impaired nerve regeneration limits the functional performance of the new muscle.

  20. Maturation of the mitochondrial redox response to profound asphyxia in fetal sheep.

    Directory of Open Access Journals (Sweden)

    Paul P Drury

    Full Text Available Fetal susceptibility to hypoxic brain injury increases over the last third of gestation. This study examined the hypothesis that this is associated with impaired mitochondrial adaptation, as measured by more rapid oxidation of cytochrome oxidase (CytOx during profound asphyxia.Chronically instrumented fetal sheep at 0.6, 0.7, and 0.85 gestation were subjected to either 30 min (0.6 gestational age (ga, n = 6, 25 min (0.7 ga, n = 27 or 15 min (0.85 ga, n = 17 of complete umbilical cord occlusion. Fetal EEG, cerebral impedance (to measure brain swelling and near-infrared spectroscopy-derived intra-cerebral oxygenation (ΔHb = HbO(2 - Hb, total hemoglobin (THb and CytOx redox state were monitored continuously. Occlusion was associated with profound, rapid fall in ΔHb in all groups to a plateau from 6 min, greatest at 0.85 ga compared to 0.6 and 0.7 ga (p<0.05. THb initially increased at all ages, with the greatest rise at 0.85 ga (p<0.05, followed by a progressive fall from 7 min in all groups. CytOx initially increased in all groups with the greatest rise at 0.85 ga (p<0.05, followed by a further, delayed increase in preterm fetuses, but a striking fall in the 0.85 group after 6 min of occlusion. Cerebral impedance (a measure of cytotoxic edema increased earlier and more rapidly with greater gestation. In conclusion, the more rapid rise in CytOx and cortical impedance during profound asphyxia with greater maturation is consistent with increasing dependence on oxidative metabolism leading to earlier onset of neural energy failure before the onset of systemic hypotension.

  1. The Differential Effects of Attentional Focus in Children with Moderate and Profound Visual Impairments

    Directory of Open Access Journals (Sweden)

    Scott W. T. McNamara

    2017-10-01

    Full Text Available It has been consistently reported that an external focus of attention leads to better motor performance than an internal focus, but no research to date has explored this effect in a population with visual impairments (VI. External focus statements typically reference something in the environment (e.g., target that may be difficult to conceptualize for people with VI since they cannot generate a visual representation of the object of focus. Internal focus statements could be more closely identifiable with proprioception that is not impaired in this population. Recent studies have reported that sighted adults with temporarily obstructed vision are able to receive an external focus benefit when performing discrete tasks (i.e., golf putt and vertical jump, however, it is unclear if those with VI would experience the same benefit. The purpose of this investigation was to compare how an internal focus and external focus impact the balance of children with VI. Eighteen children with VI were grouped into a moderate (n = 11 and a profound VI group (n = 7. Participants completed a familiarization trial, an internal focus trial (i.e., focusing on feet and an external focus trial (i.e., focusing on markers in a counterbalanced order. The moderate VI group had a lower root mean square error while using an external focus (p = 0.04, while the profound VI group did not differ between conditions (p > 0.05. These results suggest that while performing a task reliant on sensory feedback, an external focus benefit may be dependent on the severity of VI. Further research is needed to examine whether external focus statements can be presented in a way that may be more intuitive to those with profound VI. These findings may help to influence how professionals in health-related fields (e.g., physical therapist and physical educators give instructions on motor performance to populations with VI.

  2. Correlation between audiovestibular function tests and hearing outcomes in severe to profound sudden sensorineural hearing loss.

    Science.gov (United States)

    Wang, Chi-Te; Huang, Tsung-Wei; Kuo, Shih-Wei; Cheng, Po-Wen

    2009-02-01

    This study investigated whether audiovestibular function tests, namely auditory brain stem response (ABR) and vestibular-evoked myogenic potential (VEMP) tests were correlated to hearing outcomes after controlling the effects of other potential confounding factors in severe to profound sudden sensorineural hearing loss (SSHL). Eighty-eight patients with severe to profound SSHL were enrolled in this study. Pretreatment hearing levels, results of audiovestibular function tests, and final hearing outcomes were recorded from retrospective chart reviews. Other factors, including age, gender, delay of treatment, vertigo, diabetes mellitus, and hypertension, were collected as well. Comparative analysis between multiple variables and hearing outcomes was conducted using the cumulative logits model in overall subjects. Further, multivariate analysis of prognostic factors was conducted in the stratified groups of severe (70 dB HL 90 dB HL) SSHL. Multivariate analysis showed that pretreatment hearing levels, presence of vertigo, and results of ABR and VEMP testing were significant outcome predictors in the overall subjects. Stratification analysis demonstrated that both the presence of ABR and VEMP waveforms were significantly correlated with better hearing outcomes in the group of severe SSHL [ABR: adjusted odds ratio (aOR) = 14.7, 95% confidence interval (CI) = 1.78 to 122, p = 0.01; VEMP: aOR = 5.91, 95% CI = 1.18 to 29.5, p = 0.03], whereas the presence of vertigo was the only significant negative prognostic factor in the group of profound SSHL (aOR = 0.24, 95% CI = 0.06 to 0.95, p = 0.04). Other variables, including age, gender, diabetes mellitus, hypertension, and delay of treatment, were not significantly related to hearing outcomes in both groups (p > 0.05). A predictive hearing recovery table with the combined ABR and VEMP results was proposed for the group of severe SSHL. ABR and VEMP tests should be included in the battery of neurootological examinations in

  3. Spider Silk Violin Strings with a Unique Packing Structure Generate a Soft and Profound Timbre

    Science.gov (United States)

    Osaki, Shigeyoshi

    2012-04-01

    We overcome the difficulties in pulling long draglines from spiders, twist bundles of dragline filaments, and succeed in preparing violin strings. The twisting is found to change the cross section shapes of filaments from circular to polygonal and to optimize the packing structure with no openings among filaments providing mechanically strong and elastic strings. The spider string signal peaks of overtones for the violin are relatively large at high frequencies, generating a soft and profound timbre. Such a preferable timbre is considered to be due to the unique polygonal packing structure which provides valuable knowledge for developing new types of materials.

  4. Experience with cochlear implants in Greenlanders with profound hearing loss living in Greenland

    DEFF Research Database (Denmark)

    Homøe, Preben; Andersen, Ture; Grøntved, Aksel

    2013-01-01

    OBJECTIVE: Cochlear implant (CI) treatment was introduced to the world in the 1980s and has become a routine treatment for congenital or acquired severe-to-profound hearing loss. CI treatment requires access to a highly skilled team of ear, nose and throat specialists, audiologists and speech...... years are in need of a CI every second year in Greenland often due to sequelae from meningitis, which may cause postinfectious deafness. Screening of new-borns for hearing has been started in Greenland establishing the basis for early diagnosis of congenital hearing impairment and subsequent...

  5. Profound vision loss impairs psychological well-being in young and middle-aged individuals.

    Science.gov (United States)

    Garcia, Giancarlo A; Khoshnevis, Matin; Gale, Jesse; Frousiakis, Starleen E; Hwang, Tiffany J; Poincenot, Lissa; Karanjia, Rustum; Baron, David; Sadun, Alfredo A

    2017-01-01

    The aim of this study was to evaluate the effects of profound vision loss on psychological well-being in adolescents, young adults, and middle-aged adults with regard to mood, interpersonal interactions, and career-related goals. In addition, we assessed the significance of the resources that may be used to enhance psychological well-being in cases of profound vision loss, and in particular, examined the utility of low vision aids and the role of the ophthalmologist as a provider of emotional support. A questionnaire was issued to individuals aged 13-65 years with profound vision loss resulting from Leber's hereditary optic neuropathy (LHON). Depression prevalence was evaluated with questions regarding major depressive disorder symptomatology. Participants appraised the effects of vision loss on their interpersonal interactions and career goals by providing an impact rating (IR) on a 21-point psychometric scale from -10 to +10. Social well-being index was defined as the average of interpersonal IR and career IR. Subjects were additionally asked about the use of low vision aids and sources of emotional support. A total of 103 participants (mean age =26.4±11.2 years at LHON diagnosis; mean ± standard deviation) completed the questionnaire. Nearly half (49.5%) met the depression criteria after vision loss. Negative impacts on interpersonal interactions (median IR = -5) and career goals (median IR = -6) were observed; both ratings were worse ( P negative interpersonal IR and career IR. Sixty-eight percent of subjects used electronic vision aids; controlling for age, social well-being index was higher among these individuals than for those who did not use electronic aids ( P =0.03). Over half of the participants (52.4%) asserted that they derived emotional support from their ophthalmologist. Profound vision loss in adolescents, young adults, and middle-aged adults is associated with significant negative psychological and psychosocial effects, which are influenced by

  6. Developmental dysgraphia with profound hearing impairment: intervention by auditory methods enabled by cochlear implant.

    Science.gov (United States)

    Fukushima, Kunihiro; Kawasaki, Akihiro; Nagayasu, Rie; Kunisue, Kazuya; Maeda, Yukihide; Kariya, Shin; Kataoka, Yuko; Nishizaki, Kazunori

    2008-06-01

    Learning disability combined with hearing impairment (LDHI) is a poor prognostic factor for the language development of hearing impaired children after educational intervention. A typical example of a child with LDHI and effective interventions provided by cochlear implants are presented in this report. A case of congenital cytomegaloviral infection that showed dysgraphia as well as profound deafness was reported and an underlying visual processing problem diagnosed in the present case caused the patient's dysgraphia. The dysgraphia could be circumvented by the use of auditory memory fairly established by a cochlear implant.

  7. Slow-Adhering Stem Cells Derived from Injured Skeletal Muscle Have Improved Regenerative Capacity

    Science.gov (United States)

    2011-08-01

    stress urinary incontinence . Urology 2006, 68:449–454 15. Chermansky CJ, Tarin T, Kwon DD, Jankowski RJ, Cannon TW, de Groat WC, Huard J, Chancellor...from control noninjured muscle. These data suggest that traumatic injury may modify stem cell characteristics through trophic factors and improve the...alter the microenvironment of resident muscle cells (ie, stimu- lating cell dedifferentiation on various trophic factors )20,21 and result in profound

  8. A Universe without Weak Interactions

    Energy Technology Data Exchange (ETDEWEB)

    Harnik, Roni; Kribs, Graham D.; Perez, Gilad

    2006-04-07

    A universe without weak interactions is constructed that undergoes big-bang nucleosynthesis, matter domination, structure formation, and star formation. The stars in this universe are able to burn for billions of years, synthesize elements up to iron, and undergo supernova explosions, dispersing heavy elements into the interstellar medium. These definitive claims are supported by a detailed analysis where this hypothetical ''Weakless Universe'' is matched to our Universe by simultaneously adjusting Standard Model and cosmological parameters. For instance, chemistry and nuclear physics are essentially unchanged. The apparent habitability of the Weakless Universe suggests that the anthropic principle does not determine the scale of electroweak breaking, or even require that it be smaller than the Planck scale, so long as technically natural parameters may be suitably adjusted. Whether the multi-parameter adjustment is realized or probable is dependent on the ultraviolet completion, such as the string landscape. Considering a similar analysis for the cosmological constant, however, we argue that no adjustments of other parameters are able to allow the cosmological constant to raise up even remotely close to the Planck scale while obtaining macroscopic structure. The fine-tuning problems associated with the electroweak breaking scale and the cosmological constant therefore appear to be qualitatively different from the perspective of obtaining a habitable universe.

  9. A Universe without Weak Interactions

    International Nuclear Information System (INIS)

    Harnik, R

    2006-01-01

    A universe without weak interactions is constructed that undergoes big-bang nucleosynthesis, matter domination, structure formation, and star formation. The stars in this universe are able to burn for billions of years, synthesize elements up to iron, and undergo supernova explosions, dispersing heavy elements into the interstellar medium. These definitive claims are supported by a detailed analysis where this hypothetical ''Weakless Universe'' is matched to our Universe by simultaneously adjusting Standard Model and cosmological parameters. For instance, chemistry and nuclear physics are essentially unchanged. The apparent habitability of the Weakless Universe suggests that the anthropic principle does not determine the scale of electroweak breaking, or even require that it be smaller than the Planck scale, so long as technically natural parameters may be suitably adjusted. Whether the multi-parameter adjustment is realized or probable is dependent on the ultraviolet completion, such as the string landscape. Considering a similar analysis for the cosmological constant, however, we argue that no adjustments of other parameters are able to allow the cosmological constant to raise up even remotely close to the Planck scale while obtaining macroscopic structure. The fine-tuning problems associated with the electroweak breaking scale and the cosmological constant therefore appear to be qualitatively different from the perspective of obtaining a habitable universe

  10. A universe without weak interactions

    International Nuclear Information System (INIS)

    Harnik, Roni; Kribs, Graham D.; Perez, Gilad

    2006-01-01

    A universe without weak interactions is constructed that undergoes big-bang nucleosynthesis, matter domination, structure formation, and star formation. The stars in this universe are able to burn for billions of years, synthesize elements up to iron, and undergo supernova explosions, dispersing heavy elements into the interstellar medium. These definitive claims are supported by a detailed analysis where this hypothetical ''weakless universe'' is matched to our Universe by simultaneously adjusting standard model and cosmological parameters. For instance, chemistry and nuclear physics are essentially unchanged. The apparent habitability of the weakless universe suggests that the anthropic principle does not determine the scale of electroweak breaking, or even require that it be smaller than the Planck scale, so long as technically natural parameters may be suitably adjusted. Whether the multiparameter adjustment is realized or probable is dependent on the ultraviolet completion, such as the string landscape. Considering a similar analysis for the cosmological constant, however, we argue that no adjustments of other parameters are able to allow the cosmological constant to raise up even remotely close to the Planck scale while obtaining macroscopic structure. The fine-tuning problems associated with the electroweak breaking scale and the cosmological constant therefore appear to be qualitatively different from the perspective of obtaining a habitable universe

  11. Measurements of weak conversion lines

    International Nuclear Information System (INIS)

    Feoktistov, A.I.; Frantsev, Yu.E.

    1979-01-01

    Described is a new methods for measuring weak conversion lines with the help of the β spectrometer of the π √ 2 type which permits to increase the reliability of the results obtained. According to this method the measurements were carried out by short series with the storage of the information obtained on the punched tape. The spectrometer magnetic field was stabilized during the measuring of the conversion spectra with the help of three nmr recorders. Instead of the dependence of the pulse calculation rate on the magnetic field value was measured the dependence of the calculation rate on the value of the voltage applied between the source and the spectrometer chamber. A short description of the automatic set-up for measuring conversion lines according to the method proposed is given. The main set-up elements are the voltage multiplexer timer, printer, scaler and the pulse analyzer. With the help of the above methods obtained is the K 1035, 8 keV 182 Ta line. It is obtained as a result of the composition of 96 measurement series. Each measurement time constitutes 640 s 12 points are taken on the line

  12. Methodology for analyzing weak spectra

    International Nuclear Information System (INIS)

    Yankovich, T.L.; Swainson, I.P.

    2000-02-01

    There is considerable interest in quantifying radionuclide transfer between environmental compartments. However, in many cases, it can be a challenge to detect concentrations of gamma-emitting radionuclides due to their low levels in environmental samples. As a result, it is valuable to develop analytical protocols to ensure consistent analysis of the areas under weak peaks. The current study has focused on testing how reproducibly peak areas and baselines can be determined using two analytical approaches. The first approach, which can be carried out using Maestro software, involves extracting net counts under a curve without fitting a functional form to the peak, whereas the second approach, which is used by most other peak fitting programs, determines net counts from spectra by fitting a Gaussian form to the data. It was found that the second approach produces more consistent peak area and baseline measurements, with the ability to de-convolute multiple, overlapping peaks. In addition, programs, such as Peak Fit, which can be used to fit a form to spectral data, often provide goodness of fit analyses, since the Gaussian form can be described using a characteristic equation against which peak data can be tested for their statistical significance. (author)

  13. Homological properties of modules with finite weak injective and weak flat dimensions

    OpenAIRE

    Zhao, Tiwei

    2017-01-01

    In this paper, we define a class of relative derived functors in terms of left or right weak flat resolutions to compute the weak flat dimension of modules. Moreover, we investigate two classes of modules larger than that of weak injective and weak flat modules, study the existence of covers and preenvelopes, and give some applications.

  14. Whole exome sequencing identifies mutations in Usher syndrome genes in profoundly deaf Tunisian patients.

    Science.gov (United States)

    Riahi, Zied; Bonnet, Crystel; Zainine, Rim; Lahbib, Saida; Bouyacoub, Yosra; Bechraoui, Rym; Marrakchi, Jihène; Hardelin, Jean-Pierre; Louha, Malek; Largueche, Leila; Ben Yahia, Salim; Kheirallah, Moncef; Elmatri, Leila; Besbes, Ghazi; Abdelhak, Sonia; Petit, Christine

    2015-01-01

    Usher syndrome (USH) is an autosomal recessive disorder characterized by combined deafness-blindness. It accounts for about 50% of all hereditary deafness blindness cases. Three clinical subtypes (USH1, USH2, and USH3) are described, of which USH1 is the most severe form, characterized by congenital profound deafness, constant vestibular dysfunction, and a prepubertal onset of retinitis pigmentosa. We performed whole exome sequencing in four unrelated Tunisian patients affected by apparently isolated, congenital profound deafness, with reportedly normal ocular fundus examination. Four biallelic mutations were identified in two USH1 genes: a splice acceptor site mutation, c.2283-1G>T, and a novel missense mutation, c.5434G>A (p.Glu1812Lys), in MYO7A, and two previously unreported mutations in USH1G, i.e. a frameshift mutation, c.1195_1196delAG (p.Leu399Alafs*24), and a nonsense mutation, c.52A>T (p.Lys18*). Another ophthalmological examination including optical coherence tomography actually showed the presence of retinitis pigmentosa in all the patients. Our findings provide evidence that USH is under-diagnosed in Tunisian deaf patients. Yet, early diagnosis of USH is of utmost importance because these patients should undergo cochlear implant surgery in early childhood, in anticipation of the visual loss.

  15. Profound microcephaly, primordial dwarfism with developmental brain malformations: a new syndrome.

    Science.gov (United States)

    Abdel-Salam, Ghada M H; Abdel-Hamid, Mohamed S; Saleem, Sahar N; Ahmed, Mahmoud K H; Issa, Mahmoud; Effat, Laila K; Kayed, Hisham F; Zaki, Maha S; Gaber, Khaled R

    2012-08-01

    We describe two sibs with a lethal form of profound congenital microcephaly, intrauterine and postnatal growth retardation, subtle skeletal changes, and poorly developed brain. The sibs had striking absent cranial vault with sloping of the forehead, large beaked nose, relatively large ears, and mandibular micro-retrognathia. Brain magnetic resonance imaging (MRI) revealed extremely simplified gyral pattern, large interhemispheric cyst and agenesis of corpus callosum, abnormally shaped hippocampus, and proportionately affected cerebellum and brainstem. In addition, fundus examination showed foveal hypoplasia with optic nerve atrophy. No abnormalities of the internal organs were found. This profound form of microcephaly was identified at 17 weeks gestation by ultrasound and fetal brain MRI helped in characterizing the developmental brain malformations in the second sib. Molecular analysis excluded mutations in potentially related genes such as RNU4ATAC, SLC25A19, and ASPM. These clinical and imaging findings are unlike that of any recognized severe forms of microcephaly which is believed to be a new microcephalic primordial dwarfism (MPD) with developmental brain malformations with most probably autosomal recessive inheritance based on consanguinity and similarly affected male and female sibs. Copyright © 2012 Wiley Periodicals, Inc.

  16. Profound neonatal hypoglycemia and lactic acidosis caused by pyridoxine-dependent epilepsy.

    Science.gov (United States)

    Mercimek-Mahmutoglu, Saadet; Horvath, Gabriella A; Coulter-Mackie, Marion; Nelson, Tanya; Waters, Paula J; Sargent, Michael; Struys, Eduard; Jakobs, Cornelis; Stockler-Ipsiroglu, Sylvia; Connolly, Mary B

    2012-05-01

    Pyridoxine-dependent epilepsy (PDE) was first described in 1954. The ALDH7A1 gene mutations resulting in α-aminoadipic semialdehyde dehydrogenase deficiency as a cause of PDE was identified only in 2005. Neonatal epileptic encephalopathy is the presenting feature in >50% of patients with classic PDE. We report the case of a 13-month-old girl with profound neonatal hypoglycemia (0.6 mmol/L; reference range >2.4), lactic acidosis (11 mmol/L; reference range A (p.Val278Val), and a novel putative pathogenic missense mutation c.1192G>C (p.Gly398Arg) in the ALDH7A1 gene. She has been seizure-free since 1.5 months of age on treatment with pyridoxine alone. She has motor delay and central hypotonia but normal language and social development at the age of 13 months. This case is the first description of a patient with PDE due to mutations in the ALDH7A1 gene who presented with profound neonatal hypoglycemia and lactic acidosis masquerading as a neonatal-onset gluconeogenesis defect. PDE should be included in the differential diagnosis of hypoglycemia and lactic acidosis in addition to medically refractory neonatal seizures.

  17. Quantification of structural changes in the corpus callosumin children with profound hypoxic-ischaemic brain injury

    Energy Technology Data Exchange (ETDEWEB)

    Stivaros, Stavros M. [Manchester Academic Health Science Centre, Academic Unit of Paediatric Radiology, Royal Manchester Children' s Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester (United Kingdom); University of Manchester, Centre for Imaging Sciences, Institute of Population Health, Manchester (United Kingdom); Radon, Mark R. [The Walton Centre NHS Foundation Trust, Department of Neuroradiology, Liverpool (United Kingdom); Mileva, Reneta; Gledson, Ann; Keane, John A. [University of Manchester, School of Computer Science, Manchester (United Kingdom); Connolly, Daniel J.A.; Batty, Ruth [Sheffield Children' s Hospital NHS Foundation Trust, Department of Neuroradiology, Sheffield (United Kingdom); Cowell, Patricia E. [University of Sheffield, Department of Human Communication Sciences, Sheffield (United Kingdom); Hoggard, Nigel; Griffiths, Paul D. [University of Sheffield, Academic Unit of Radiology, Sheffield (United Kingdom); Wright, Neville B.; Tang, Vivian [Manchester Academic Health Science Centre, Academic Unit of Paediatric Radiology, Royal Manchester Children' s Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester (United Kingdom)

    2016-01-15

    Birth-related acute profound hypoxic-ischaemic brain injury has specific patterns of damage including the paracentral lobules. To test the hypothesis that there is anatomically coherent regional volume loss of the corpus callosum as a result of this hemispheric abnormality. Study subjects included 13 children with proven acute profound hypoxic-ischaemic brain injury and 13 children with developmental delay but no brain abnormalities. A computerised system divided the corpus callosum into 100 segments, measuring each width. Principal component analysis grouped the widths into contiguous anatomical regions. We conducted analysis of variance of corpus callosum widths as well as support vector machine stratification into patient groups. There was statistically significant narrowing of the mid-posterior body and genu of the corpus callosum in children with hypoxic-ischaemic brain injury. Support vector machine analysis yielded over 95% accuracy in patient group stratification using the corpus callosum centile widths. Focal volume loss is seen in the corpus callosum of children with hypoxic-ischaemic brain injury secondary to loss of commissural fibres arising in the paracentral lobules. Support vector machine stratification into the hypoxic-ischaemic brain injury group or the control group on the basis of corpus callosum width is highly accurate and points towards rapid clinical translation of this technique as a potential biomarker of hypoxic-ischaemic brain injury. (orig.)

  18. Validation of the Spanish version of the Baylor Profound Mental Status Examination.

    Science.gov (United States)

    Salmerón, Sergio; Huedo, Isabel; López-Utiel, Melisa; Soler-Moratalla, Isabel; Flores-Ruano, Teresa; Fernández-Sánchez, Miguel; Noguerón, Alicia; Doody, Rachelle S; Abizanda, Pedro

    2016-01-01

    There are no short valid instruments to evaluate cognitive status in severe Alzheimer's disease (AD) patients in the Spanish language. To validate the Spanish version of the Baylor Profound Mental Status Examination (BPMSE-Sp). The Baylor Profound Mental Status Examination (BPMSE) was translated to Spanish and back translated. Validation was conducted in 100 patients with severe probable AD with a Mini-Mental State Examination (MMSE) reliability. The mean age of patients was 84.9; 74% were female; 64% were institutionalized. The mean MMSE was 5.6; the mean BPMSE-Sp was 13.6; the mean BPMSE-Sp behavior was 1.2; the mean SIB was 42.2; and the mean NPI-Q was 4.7. BPMSE-Sp presented good internal consistency (Cronbach α= 0.84). There were significant correlations between the BPMSE-Sp and MMSE (r = 0.86, p reliability were in both cases excellent, ranging between 0.96 and 0.99 (p <  0.001). BPMSE-Sp had fewer floor and ceiling effects than the MMSE. The BPMSE-Sp is a valid tool for use in daily practice and research in the evaluation of cognitive function of patients with severe AD.

  19. Loss of CIB2 Causes Profound Hearing Loss and Abolishes Mechanoelectrical Transduction in Mice

    Directory of Open Access Journals (Sweden)

    Yanfei Wang

    2017-12-01

    Full Text Available Calcium and integrin-binding protein 2 (CIB2 belongs to a protein family with four known members, CIB1 through CIB4, which are characterized by multiple calcium-binding EF-hand domains. Among the family members, the Cib1 and Cib2 genes are expressed in mouse cochlear hair cells, and mutations in the human CIB2 gene have been associated with nonsyndromic deafness DFNB48 and syndromic deafness USH1J. To further explore the function of CIB1 and CIB2 in hearing, we established Cib1 and Cib2 knockout mice using the clustered regularly interspaced short palindromic repeat (CRISPR-associated Cas9 nuclease (CRISPR/Cas9 genome editing technique. We found that loss of CIB1 protein does not affect auditory function, whereas loss of CIB2 protein causes profound hearing loss in mice. Further investigation revealed that hair cell stereocilia development is affected in Cib2 knockout mice. Noticeably, loss of CIB2 abolishes mechanoelectrical transduction (MET currents in auditory hair cells. In conclusion, we show here that although both CIB1 and CIB2 are readily detected in the cochlea, only loss of CIB2 results in profound hearing loss, and that CIB2 is essential for auditory hair cell MET.

  20. Whole exome sequencing identifies mutations in Usher syndrome genes in profoundly deaf Tunisian patients.

    Directory of Open Access Journals (Sweden)

    Zied Riahi

    Full Text Available Usher syndrome (USH is an autosomal recessive disorder characterized by combined deafness-blindness. It accounts for about 50% of all hereditary deafness blindness cases. Three clinical subtypes (USH1, USH2, and USH3 are described, of which USH1 is the most severe form, characterized by congenital profound deafness, constant vestibular dysfunction, and a prepubertal onset of retinitis pigmentosa. We performed whole exome sequencing in four unrelated Tunisian patients affected by apparently isolated, congenital profound deafness, with reportedly normal ocular fundus examination. Four biallelic mutations were identified in two USH1 genes: a splice acceptor site mutation, c.2283-1G>T, and a novel missense mutation, c.5434G>A (p.Glu1812Lys, in MYO7A, and two previously unreported mutations in USH1G, i.e. a frameshift mutation, c.1195_1196delAG (p.Leu399Alafs*24, and a nonsense mutation, c.52A>T (p.Lys18*. Another ophthalmological examination including optical coherence tomography actually showed the presence of retinitis pigmentosa in all the patients. Our findings provide evidence that USH is under-diagnosed in Tunisian deaf patients. Yet, early diagnosis of USH is of utmost importance because these patients should undergo cochlear implant surgery in early childhood, in anticipation of the visual loss.

  1. Quantification of structural changes in the corpus callosumin children with profound hypoxic-ischaemic brain injury

    International Nuclear Information System (INIS)

    Stivaros, Stavros M.; Radon, Mark R.; Mileva, Reneta; Gledson, Ann; Keane, John A.; Connolly, Daniel J.A.; Batty, Ruth; Cowell, Patricia E.; Hoggard, Nigel; Griffiths, Paul D.; Wright, Neville B.; Tang, Vivian

    2016-01-01

    Birth-related acute profound hypoxic-ischaemic brain injury has specific patterns of damage including the paracentral lobules. To test the hypothesis that there is anatomically coherent regional volume loss of the corpus callosum as a result of this hemispheric abnormality. Study subjects included 13 children with proven acute profound hypoxic-ischaemic brain injury and 13 children with developmental delay but no brain abnormalities. A computerised system divided the corpus callosum into 100 segments, measuring each width. Principal component analysis grouped the widths into contiguous anatomical regions. We conducted analysis of variance of corpus callosum widths as well as support vector machine stratification into patient groups. There was statistically significant narrowing of the mid-posterior body and genu of the corpus callosum in children with hypoxic-ischaemic brain injury. Support vector machine analysis yielded over 95% accuracy in patient group stratification using the corpus callosum centile widths. Focal volume loss is seen in the corpus callosum of children with hypoxic-ischaemic brain injury secondary to loss of commissural fibres arising in the paracentral lobules. Support vector machine stratification into the hypoxic-ischaemic brain injury group or the control group on the basis of corpus callosum width is highly accurate and points towards rapid clinical translation of this technique as a potential biomarker of hypoxic-ischaemic brain injury. (orig.)

  2. Weak boson emission in hadron collider processes

    International Nuclear Information System (INIS)

    Baur, U.

    2007-01-01

    The O(α) virtual weak radiative corrections to many hadron collider processes are known to become large and negative at high energies, due to the appearance of Sudakov-like logarithms. At the same order in perturbation theory, weak boson emission diagrams contribute. Since the W and Z bosons are massive, the O(α) virtual weak radiative corrections and the contributions from weak boson emission are separately finite. Thus, unlike in QED or QCD calculations, there is no technical reason for including gauge boson emission diagrams in calculations of electroweak radiative corrections. In most calculations of the O(α) electroweak radiative corrections, weak boson emission diagrams are therefore not taken into account. Another reason for not including these diagrams is that they lead to final states which differ from that of the original process. However, in experiment, one usually considers partially inclusive final states. Weak boson emission diagrams thus should be included in calculations of electroweak radiative corrections. In this paper, I examine the role of weak boson emission in those processes at the Fermilab Tevatron and the CERN LHC for which the one-loop electroweak radiative corrections are known to become large at high energies (inclusive jet, isolated photon, Z+1 jet, Drell-Yan, di-boson, tt, and single top production). In general, I find that the cross section for weak boson emission is substantial at high energies and that weak boson emission and the O(α) virtual weak radiative corrections partially cancel

  3. Advancements in stem cells treatment of skeletal muscle wasting

    Directory of Open Access Journals (Sweden)

    mirella emeregalli

    2014-02-01

    Full Text Available Muscular dystrophies (MDs are a heterogeneous group of inherited disorders, in which progressive muscle wasting and weakness is often associated with exhaustion of muscle regeneration potential. Although physiological properties of skeletal muscle tissue are now well known, no treatments are effective for these diseases. Muscle regeneration was attempted by means transplantation of myogenic cells (from myoblast to embryonic stem cells and also by interfering with the malignant processes that originate in pathological tissues, such as uncontrolled fibrosis and inflammation. Taking into account the advances in the isolation of new subpopulation of stem cells and in the creation of artificial stem cell niches, we discuss how these emerging technologies offer great promises for therapeutic approaches to muscle diseases and muscle wasting associated with aging.

  4. Muscle response to leg lengthening during distraction osteogenesis.

    Science.gov (United States)

    Thorey, Fritz; Bruenger, Jens; Windhagen, Henning; Witte, Frank

    2009-04-01

    Continuous lengthening of intact muscles during distraction osteogenesis leads to an increase of sarcomeres and enhances the regeneration of tendons and blood vessels. A high distraction rate leads to an excessive leg and muscle lengthening and might cause damages of muscle fibers with fibrosis, necrosis, and muscle weakness. Complications like muscle contractures or atrophy after postoperative immobilization emphazize the importance of muscles and their function in the clinical outcome. In an animal model of distraction osteogenesis, 18 sheep were operated with an external fixator followed by 4 days latency, 21 days distraction (1.25 mm per day) and 51 days consolidation. The anatomical location (gastrocnemius, peroneus tertius, and first flexor digitorum longus muscle), dimension and occurrence of muscular defects were characterized histologically. The callus formation and leg axis was monitored by weekly X-rays. Additionally, serum creatine kinase was analyzed during a distraction and consolidation period. Significant signs of muscle lesions in all three observed muscles can be found postoperatively, whereas normal callus formation and regular leg axis was observed radiologically. The peroneus tertius and first flexor digitorum longus muscles were found to have significantly more signs of fibrosis, inflammatory, and necrosis. Creatine kinase showed two peaks: 4 and 39 days postoperative as an indication of muscle damage and regeneration. The study implicates that muscle damages should be considered when a long-distance distraction osteogenesis is planned. The surgeon should consider these muscle responses and individually discuss a two-stage treatment or additional muscle tendon releases to minimize the risk of muscle damages.

  5. Developing a questionnaire on physical activity support of people with (profound) intellectual (and multiple) disabilities : Experiences from the Netherlands

    NARCIS (Netherlands)

    Bossink, Leontien; van der Putten, Annette; Vlaskamp, Carla

    2017-01-01

    Introduction: People with intellectual disabilities (ID) undertake extremely low levels of physical activity, which is even more true in people with profound intellectual and multiple disabilities (PIMD). Physical activity approaches, particularly for people with PIMD, are more likely to be

  6. Early reversal of profound rocuronium-induced neuromuscular blockade by sugammadex in a randomized multicenter study - Efficacy, safety, and pharmacokinetics

    NARCIS (Netherlands)

    Sparr, Harald J.; Vermeyen, Karel M.; Beaufort, Anton M.; Rietbergen, Henk; Proost, Johannes H.; Saldien, Vera; Velik-Salchner, Corinna; Wierda, J. Mark K. H.

    Background: Sugammadex reverses the neuromuscular blocking effects of rocuronium by chemical encapsulation. The efficacy, safety, and pharmacokinetics of sugammadex for reversal of profound rocuronium-induced neuromuscular blockade were evaluated. Methods: Ninety-eight male adult patients were

  7. Examining relationships between staff attributions of soundscapes and core affect in people with severe or profound intellectual and visual disabilities

    NARCIS (Netherlands)

    van den Bosch, Kirsten A.; Vlaskamp, Carla; Andringa, Tjeerd C.; Post, Wendy J.; Ruijssenaars, Wied A.J.J.M.

    Background: People with profound intellectual disabilities experience a high prevalence of visual disabilities, making them more dependent on sound. However, research addressing the influence of the auditory environment is scarce. Method: Observations of the auditory environments (soundscapes) and

  8. An atypical case of successful resuscitation of an accidental profound hypothermia patient, occurring in a temperate climate.

    LENUS (Irish Health Repository)

    Coleman, E

    2010-03-01

    Cases of accidental profound hypothermia occur most frequently in cold, northern climates. We describe an atypical case, occurring in a temperate climate, where a hypothermic cardiac-arrested patient was successfully resuscitated using extracorporeal circulation (ECC).

  9. Informal social networks of people with profound intellectual and multiple disabilities : Relationship with age, communicative abilities and current living arrangements

    NARCIS (Netherlands)

    Kamstra, A.; van der Putten, A.A.J.; Post, W.J.; Vlaskamp, C.

    2015-01-01

    BACKGROUND: People with profound intellectual and multiple disabilities (PIMD) have limited informal social contacts. Research to determine the factors which can positively influence establishing sound informal social contacts is required. MATERIALS AND METHODS: Regression analysis for 200 people

  10. Classical field approach to quantum weak measurements.

    Science.gov (United States)

    Dressel, Justin; Bliokh, Konstantin Y; Nori, Franco

    2014-03-21

    By generalizing the quantum weak measurement protocol to the case of quantum fields, we show that weak measurements probe an effective classical background field that describes the average field configuration in the spacetime region between pre- and postselection boundary conditions. The classical field is itself a weak value of the corresponding quantum field operator and satisfies equations of motion that extremize an effective action. Weak measurements perturb this effective action, producing measurable changes to the classical field dynamics. As such, weakly measured effects always correspond to an effective classical field. This general result explains why these effects appear to be robust for pre- and postselected ensembles, and why they can also be measured using classical field techniques that are not weak for individual excitations of the field.

  11. Contribution of elastic tissues to the mechanics and energetics of muscle function during movement.

    Science.gov (United States)

    Roberts, Thomas J

    2016-01-01

    Muscle force production occurs within an environment of tissues that exhibit spring-like behavior, and this elasticity is a critical determinant of muscle performance during locomotion. Muscle force and power output both depend on the speed of contraction, as described by the isotonic force-velocity curve. By influencing the speed of contractile elements, elastic structures can have a profound effect on muscle force, power and work. In very rapid movements, elastic mechanisms can amplify muscle power by storing the work of muscle contraction slowly and releasing it rapidly. When energy must be dissipated rapidly, such as in landing from a jump, energy stored rapidly in elastic elements can be released more slowly to stretch muscle contractile elements, reducing the power input to muscle and possibly protecting it from damage. Elastic mechanisms identified so far rely primarily on in-series tendons, but many structures within muscles exhibit spring-like properties. Actomyosin cross-bridges, actin and myosin filaments, titin, and the connective tissue scaffolding of the extracellular matrix all have the potential to store and recover elastic energy during muscle contraction. The potential contribution of these elements can be assessed from their stiffness and estimates of the strain they undergo during muscle function. Such calculations provide boundaries for the possible roles these springs might play in locomotion, and may help to direct future studies of the uses of elastic elements in muscle. © 2016. Published by The Company of Biologists Ltd.

  12. Co-activation: its association with weakness and specific neurological pathology

    Directory of Open Access Journals (Sweden)

    Wiles Charles M

    2006-11-01

    Full Text Available Abstract Background Net agonist muscle strength is in part determined by the degree of antagonist co-activation. The level of co-activation might vary in different neurological disorders causing weakness or might vary with agonist strength. Aim This study investigated whether antagonist co-activation changed a with the degree of muscle weakness and b with the nature of the neurological lesion causing weakness. Methods Measures of isometric quadriceps and hamstrings strength were obtained. Antagonist (hamstring co-activation during knee extension was calculated as a ratio of hamstrings over quadriceps activity both during an isometric and during a functional sit to stand (STS task (using kinematics in groups of patients with extrapyramidal (n = 15, upper motor neuron (UMN (n = 12, lower motor neuron (LMN with (n = 18 or without (n = 12 sensory loss, primary muscle or neuromuscular junction disorder (n = 17 and in healthy matched controls (n = 32. Independent t-tests or Mann Witney U tests were used to compare between the groups. Correlations between variables were also investigated. Results In healthy subjects mean (SD co-activation of hamstrings during isometric knee extension was 11.8 (6.2% and during STS was 20.5 (12.9%. In patients, co-activation ranged from 7 to 17% during isometric knee extension and 15 to 25% during STS. Only the extrapyramidal group had lower co-activation levels than healthy matched controls (p Conclusion It is concluded that antagonist co-activation does not systematically vary with the site of neurological pathology when compared to healthy matched controls or, in most patient groups, with strength. The lower co-activation levels found in the extrapyramidal group require confirmation and further investigation. Co-activation may be relevant to individuals with muscle weakness. Within patient serial studies in the presence of changing muscle strength may help to understand these relationships more clearly.

  13. Instrumental systematics and weak gravitational lensing

    International Nuclear Information System (INIS)

    Mandelbaum, R.

    2015-01-01

    We present a pedagogical review of the weak gravitational lensing measurement process and its connection to major scientific questions such as dark matter and dark energy. Then we describe common ways of parametrizing systematic errors and understanding how they affect weak lensing measurements. Finally, we discuss several instrumental systematics and how they fit into this context, and conclude with some future perspective on how progress can be made in understanding the impact of instrumental systematics on weak lensing measurements

  14. Fixed points of occasionally weakly biased mappings

    OpenAIRE

    Y. Mahendra Singh, M. R. Singh

    2012-01-01

    Common fixed point results due to Pant et al. [Pant et al., Weak reciprocal continuity and fixed point theorems, Ann Univ Ferrara, 57(1), 181-190 (2011)] are extended to a class of non commuting operators called occasionally weakly biased pair[ N. Hussain, M. A. Khamsi A. Latif, Commonfixed points for JH-operators and occasionally weakly biased pairs under relaxed conditions, Nonlinear Analysis, 74, 2133-2140 (2011)]. We also provideillustrative examples to justify the improvements. Abstract....

  15. Robust weak measurements on finite samples

    International Nuclear Information System (INIS)

    Tollaksen, Jeff

    2007-01-01

    A new weak measurement procedure is introduced for finite samples which yields accurate weak values that are outside the range of eigenvalues and which do not require an exponentially rare ensemble. This procedure provides a unique advantage in the amplification of small nonrandom signals by minimizing uncertainties in determining the weak value and by minimizing sample size. This procedure can also extend the strength of the coupling between the system and measuring device to a new regime

  16. Spin effects in the weak interaction

    International Nuclear Information System (INIS)

    Freedman, S.J.; Chicago Univ., IL; Chicago Univ., IL

    1990-01-01

    Modern experiments investigating the beta decay of the neutron and light nuclei are still providing important constraints on the theory of the weak interaction. Beta decay experiments are yielding more precise values for allowed and induced weak coupling constants and putting constraints on possible extensions to the standard electroweak model. Here we emphasize the implications of recent experiments to pin down the strengths of the weak vector and axial vector couplings of the nucleon

  17. Family matters: The experiences and opinions of family members of persons with (severe) or profound intellectual disabilities

    OpenAIRE

    Luijkx, Jorien

    2016-01-01

    “I love my sister, but sometimes I don’t”. This is one of the statements made in the study focused on the experiences of family members of people with (profound) intellectual (and multiple) disabilities (both of individuals living in a residential facility as persons living at home). In recent years, there has been greater recognition of the important position of parents and siblings of people with (profound) intellectual (and multiple) disabilities and the importance of the wellbeing of all ...

  18. Weak strange particle production: advantages and difficulties

    International Nuclear Information System (INIS)

    Angelescu, Tatiana; Baker, O.K.

    2002-01-01

    Electromagnetic strange particle production developed at Jefferson Laboratory was an important source of information on strange particle electromagnetic formfactors and induced and transferred polarization. The high quality of the beam and the detection techniques involved could be an argument for detecting strange particles in weak interactions and answer questions about cross sections, weak formfactors, neutrino properties, which have not been investigated yet. The paper analyses some aspects related to the weak lambda production and detection with the Hall C facilities at Jefferson Laboratory and the limitations in measuring the weak interaction quantities. (authors)

  19. Celiac Disease Presenting as Profound Diarrhea and Weight Loss ? A Celiac Crisis

    OpenAIRE

    Bul, Vadim; Sleesman, Brett; Boulay, Brian

    2016-01-01

    Patient: Male, 46 Final Diagnosis: Celiac crisis Symptoms: Abdominal pain ? chronic diarrhea ? lightheadedness ? weakness ? weight loss Medication: ? Clinical Procedure: ? Specialty: Gastroenterology and Hepatology Objective: Rare disease Background: Celiac disease is a hypersensitivity enteropathy that can have various presentations in adults. Rarely, patients can present with severe lab abnormalities, dehydration and weight loss caused by celiac disease ? a celiac crisis. Case Report: A 46-...

  20. Dengue: muscle biopsy findings in 15 patients

    Directory of Open Access Journals (Sweden)

    S.M.F. Malheiros

    1993-06-01

    Full Text Available Dengue is known to produce a syndrome involving muscles, tendons and joints. The hallmark of this syndrome is severe myalgia but includes fever, cutaneous rash, and headache. The neuromuscular aspects of this infection are outlined only in isolated reports, and the muscle histopathological features during myalgia have not been described. In order to ascertain the actual neuromuscular involvement in dengue and better comprehend the histological nature of myalgia, we performed a clinical and neurological evaluation, a serum CPK level and a muscle biopsy (with histochemistry in 15 patients (4 males, median age 23 years (range 14-47 with classic dengue fever, serologically confirmed, during the bra-zilian dengue epidemics from September 1986 to March 1987. All patients had a history of fever, headache and severe myalgia. Upon examination 4 had a cutaneous rash, 3 had fever, and 3 a small hepatomegaly. The neurological examination was unremarkable in all and included a manual muscle test. CPK was mildly elevated in only 3 patients. Muscle biopsy revealed a light to moderate perivascular mononuclear infiltrate in 12 patients and lipid accumulation in 11. Mild mitochondrial proliferation was seen in 3, few central nuclei in 3, rare foci of myonecrosis in 3, and 2 patients had type grouping. Dengue in our patients, produced myalgia but no detectable muscle weakness or other neuromuscular involvement. The main histopathological correlation with myalgia seems to be a perivascular mononuclear infiltrate and lipid accumulation.

  1. Profound hypoglycemia-ınduced by vaccinium corymbosum juice and laurocerasus fruit.

    Science.gov (United States)

    Aktan, Ahmet Hamdi; Ozcelik, Abdullah; Cure, Erkan; Cure, Medine Cumhur; Yuce, Suleyman

    2014-01-01

    An emergency intervention was performed in a 75-year-old male patient with hypoglycemic attack and blackout. Although he was diagnosed with prediabetes before 2 years, he did not take any anti-diabetic drug or follow dietary advice. He drank Vaccinium corymbosum L (VC) juice daily with a belief that it increases sexual potency. Before the development of hypoglycemia, the patient had consumed about 500 ml VC juice in addition to eating 200-300 gram of Laurocerasus officinalis (LO) fruit. The measured plasma glucose (PG) level during loss of consciousness was 30 mg/dl. The profound hypoglycemia may be an unexpected side effect of an interaction between the chemical compositions of the two plants, occurred as a result of LO fruit intake that may have a strong PG-lowering effect or related to excessive intake of VC juice. Both plants may be considered in the alternative treatment of diabetes.

  2. Basic life support and children with profound and multiple learning disabilities.

    Science.gov (United States)

    Cash, Stefan; Shinnick-Page, Andrea

    2008-10-01

    Nurses and other carers of people with learning disabilities must be able to manage choking events and perform basic life support effectively. UK guidelines for assessment of airway obstruction and for resuscitation do not take account of the specific needs of people with profound multiple learning disability. For example, they fail to account for inhibited gag and coughing reflexes, limited body movements or chest deformity. There are no national guidelines to assist in clinical decisions and training for nurses and carers. Basic life support training for students of learning disability nursing at Birmingham City University is supplemented to address these issues. The authors ask whether such training should be provided for all nurses including those caring for children and young people. They also invite comment and discussion on questions related to chest compression and training in basic life support for a person in a seated position.

  3. Hypothalamic obesity in patients with craniopharyngioma: Profound changes of several weight regulatory circuits

    Directory of Open Access Journals (Sweden)

    Christian eRoth

    2011-10-01

    Full Text Available One of the most striking examples of dysfunctional hypothalamic signaling of energy homeostasis is observed in patients with hypothalamic lesions leading to hypothalamic obesity (HO. This drastic condition is frequently seen in patients with craniopharyngioma (CP, an embryological tumor located in the hypothalamic and/or pituitary region, frequently causing not only hypopituitarism, but also leading to damage of medial hypothalamic nuclei due to the tumor and its treatment. HO syndrome in CP patients is characterized by fatigue, decreased physical activity, uncontrolled appetite, and morbid obesity, and is associated with insulin and leptin resistance. Mechanisms leading to the profoundly disturbed energy homeostasis are complex. This review summarizes different aspects of important clinical studies as well as data obtained in rodent studies. In addition a model is provided describing how medial hypothalamic lesion can interact simultaneously with several weight regulating circuitries.

  4. Profound vision loss impairs psychological well-being in young and middle-aged individuals

    Directory of Open Access Journals (Sweden)

    Garcia GA

    2017-02-01

    Full Text Available Giancarlo A Garcia,1,2 Matin Khoshnevis,1,3 Jesse Gale,1,4 Starleen E Frousiakis,1,5 Tiffany J Hwang,1,6 Lissa Poincenot,1 Rustum Karanjia,1,7–9 David Baron,6 Alfredo A Sadun1,7 1Doheny Eye Institute, Los Angeles, CA, USA; 2University of California, Irvine School of Medicine, Irvine, CA, USA; 3Department of Ophthalmology, Temple University, Philadelphia, PA, USA; 4Wellington School of Medicine and Health Sciences, University of Otago, Wellington, NZ; 5Department of Ophthalmology, New York Medical College, Valhalla, NY, USA; 6Department of Psychiatry & The Behavioral Sciences, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA; 7Doheny Eye Centers, Department of Ophthalmology, David Geffen School of Medicine at UCLA, Los Angeles California, CA, USA; 8Department of Ophthalmology, University of Ottawa, Ottawa, ON, Canada; 9Ottawa Hospital Health Research Institute, Ottawa, ON, Canada Purpose: The aim of this study was to evaluate the effects of profound vision loss on psychological well-being in adolescents, young adults, and middle-aged adults with regard to mood, interpersonal interactions, and career-related goals. In addition, we assessed the significance of the resources that may be used to enhance psychological well-being in cases of profound vision loss, and in particular, examined the utility of low vision aids and the role of the ophthalmologist as a provider of emotional support.Methods: A questionnaire was issued to individuals aged 13–65 years with profound vision loss resulting from Leber’s hereditary optic neuropathy (LHON. Depression prevalence was evaluated with questions regarding major depressive disorder symptomatology. Participants appraised the effects of vision loss on their interpersonal interactions and career goals by providing an impact rating (IR on a 21-point psychometric scale from −10 to +10. Social well-being index was defined as the average of interpersonal IR and career IR

  5. Profound Endothelial Damage Predicts Impending Organ Failure and Death in Sepsis

    DEFF Research Database (Denmark)

    Johansen, Maria E; Johansson, Pär I.; Ostrowski, Sisse R

    2015-01-01

    levels at enrollment predicted risk of multiple organ failure during follow-up (HR [> 14 ng/mL vs. organ failure and death in septic......Endothelial damage contributes to organ failure and mortality in sepsis, but the extent of the contribution remains poorly quantified. Here, we examine the association between biomarkers of superficial and profound endothelial damage (syndecan-1 and soluble thrombomodulin [sTM], respectively......), organ failure, and death in sepsis. The data from a clinical trial, including critically ill patients predominantly suffering sepsis (Clinicaltrials.gov: NCT00271752) were studied. Syndecan-1 and sTM levels at the time of study enrollment were determined. The predictive ability of biomarker levels...

  6. Lack of the serum- and glucocorticoid-inducible kinase SGK1 improves muscle force characteristics and attenuates fibrosis in dystrophic mdx mouse muscle

    DEFF Research Database (Denmark)

    Steinberger, Martin; Föller, Michael; Vogelgesang, Silke

    2015-01-01

    Duchenne muscular dystrophy (DMD) is a human genetic disease characterized by fibrosis and severe muscle weakness. Currently, there is no effective treatment available to prevent progressive fibrosis in skeletal muscles. The serum- and glucocorticoid-inducible kinase SGK1 regulates a variety...... of physiological functions and participates in fibrosis stimulation. Here, we investigated whether SGK1 influences structure, function and/or fibrosis of the muscles from the mdx mouse, an animal model for DMD. As expected, mdx muscles showed the typical pathological features of muscular dystrophy including fiber...... size variations, central nuclei of muscle fibers, fibrosis in the diaphragm, and force reduction by 30–50 %. Muscles from sgk1 -/- mice were histologically overall intact and specific force was only slightly reduced compared to wild-type muscles. Surprisingly, soleus and diaphragm muscles of mdx/sgk1...

  7. Vibration sensitivity of human muscle spindles and Golgi tendon organs.

    Science.gov (United States)

    Fallon, James B; Macefield, Vaughan G

    2007-07-01

    The responses of the various muscle receptors to vibration are more complicated than a naïve categorization into stretch (muscle spindle primary ending), length (muscle spindle secondary endings), and tension (Golgi tendon organs) receptors. To emphasize the similarity of responses to small length changes, we recorded from 58 individual muscle afferents subserving receptors in the ankle or toe dorsiflexors of awake human subjects (32 primary endings, 20 secondary endings, and six Golgi tendon organs). Transverse sinusoidal vibration was applied to the distal tendon of the receptor-bearing muscle, while subjects either remained completely relaxed or maintained a weak isometric contraction of the appropriate muscle. In relaxed muscle, few units responded in a 1:1 manner to vibration, and there was no evidence of a preferred frequency of activation. In active muscle the response profiles of all three receptor types overlapped, with no significant difference in threshold between receptor types. These results emphasize that when intramuscular tension increases during a voluntary contraction, Golgi tendon organs and muscle spindle secondary endings, not just muscle spindle primary endings, can effectively encode small imposed length changes.

  8. Non-Coding RNAs in Muscle Dystrophies

    Directory of Open Access Journals (Sweden)

    Alessandra Ferlini

    2013-09-01

    Full Text Available ncRNAs are the most recently identified class of regulatory RNAs with vital functions in gene expression regulation and cell development. Among the variety of roles they play, their involvement in human diseases has opened new avenues of research towards the discovery and development of novel therapeutic approaches. Important data come from the field of hereditary muscle dystrophies, like Duchenne muscle dystrophy and Myotonic dystrophies, rare diseases affecting 1 in 7000–15,000 newborns and is characterized by severe to mild muscle weakness associated with cardiac involvement. Novel therapeutic approaches are now ongoing for these diseases, also based on splicing modulation. In this review we provide an overview about ncRNAs and their behavior in muscular dystrophy and explore their links with diagnosis, prognosis and treatments, highlighting the role of regulatory RNAs in these pathologies.

  9. Muscle autoantibodies in myasthenia gravis: beyond diagnosis?

    Science.gov (United States)

    Meriggioli, Matthew N; Sanders, Donald B

    2012-01-01

    Myasthenia gravis is an autoimmune disorder of the neuromuscular junction. A number of molecules, including ion channels and other proteins at the neuromuscular junction, may be targeted by autoantibodies leading to abnormal neuromuscular transmission. In approximately 85% of patients, autoantibodies, directed against the postsynaptic nicotinic acetylcholine receptor can be detected in the serum and confirm the diagnosis, but in general, do not precisely predict the degree of weakness or response to therapy. Antibodies to the muscle-specific tyrosine kinase are detected in approximately 50% of generalized myasthenia gravis patients who are seronegative for anti-acetylcholine receptor antibodies, and levels of anti-muscle-specific tyrosine kinase antibodies do appear to correlate with disease severity and treatment response. Antibodies to other muscle antigens may be found in the subsets of myasthenia gravis patients, potentially providing clinically useful diagnostic information, but their utility as relevant biomarkers (measures of disease state or response to treatment) is currently unclear. PMID:22882218

  10. S-parameters for weakly excited slots

    DEFF Research Database (Denmark)

    Albertsen, Niels Christian

    1999-01-01

    A simple approach to account for parasitic effects in weakly excited slots cut in the broad wall of a rectangular waveguide is proposed......A simple approach to account for parasitic effects in weakly excited slots cut in the broad wall of a rectangular waveguide is proposed...

  11. Low-energy Electro-weak Reactions

    International Nuclear Information System (INIS)

    Gazit, Doron

    2012-01-01

    Chiral effective field theory (EFT) provides a systematic and controlled approach to low-energy nuclear physics. Here, we use chiral EFT to calculate low-energy weak Gamow-Teller transitions. We put special emphasis on the role of two-body (2b) weak currents within the nucleus and discuss their applications in predicting physical observables.

  12. Weak interaction: past answers, present questions

    International Nuclear Information System (INIS)

    Ne'eman, Y.

    1977-02-01

    A historical sketch of the weak interaction is presented. From beta ray to pion decay, the V-A theory of Marshak and Sudarshan, CVC principle of equivalence, universality as an algebraic condition, PCAC, renormalized weak Hamiltonian in the rehabilitation of field theory, and some current issues are considered in this review. 47 references

  13. Staggering towards a calculation of weak amplitudes

    Energy Technology Data Exchange (ETDEWEB)

    Sharpe, S.R.

    1988-09-01

    An explanation is given of the methods required to calculate hadronic matrix elements of the weak Hamiltonians using lattice QCD with staggered fermions. New results are presented for the 1-loop perturbative mixing of the weak interaction operators. New numerical techniques designed for staggered fermions are described. A preliminary result for the kaon B parameter is presented. 24 refs., 3 figs.

  14. Weak measurements with a qubit meter

    DEFF Research Database (Denmark)

    Wu, Shengjun; Mølmer, Klaus

    2009-01-01

    We derive schemes to measure the so-called weak values of quantum system observables by coupling of the system to a qubit meter system. We highlight, in particular, the meaning of the imaginary part of the weak values, and show how it can be measured directly on equal footing with the real part...

  15. Optimization of strong and weak coordinates

    NARCIS (Netherlands)

    Swart, M.; Bickelhaupt, F.M.

    2006-01-01

    We present a new scheme for the geometry optimization of equilibrium and transition state structures that can be used for both strong and weak coordinates. We use a screening function that depends on atom-pair distances to differentiate strong coordinates from weak coordinates. This differentiation

  16. Analysis of factors related to arm weakness in patients with breast cancer-related lymphedema.

    Science.gov (United States)

    Lee, Daegu; Hwang, Ji Hye; Chu, Inho; Chang, Hyun Ju; Shim, Young Hun; Kim, Jung Hyun

    2015-08-01

    The aim of this study was to evaluate the ratio of significant weakness in the affected arm of breast cancer-related lymphedema patients to their unaffected side. Another purpose was to identify factors related to arm weakness and physical function in patients with breast cancer-related lymphedema. Consecutive patients (n = 80) attended a single evaluation session following their outpatient lymphedema clinic visit. Possible independent factors (i.e., lymphedema, pain, psychological, educational, and behavioral) were evaluated. Handgrip strength was used to assess upper extremity muscle strength and the disabilities of arm, shoulder, and hand (DASH) questionnaire was used to assess upper extremity physical function. Multivariate logistic regression was performed using factors that had significant differences between the handgrip weakness and non-weakness groups. Out of the 80 patients with breast cancer-related lymphedema, 29 patients (36.3 %) had significant weakness in the affected arm. Weakness of the arm with lymphedema was not related to lymphedema itself, but was related to the fear of using the affected limb (odds ratio = 1.76, 95 % confidence interval = 1.30-2.37). Fears of using the affected limb and depression significantly contributed to the variance in DASH scores. Appropriate physical and psychological interventions, including providing accurate information and reassurance of physical activity safety, are necessary to prevent arm weakness and physical dysfunction in patients with breast cancer-related lymphedema.

  17. Manual muscle testing: a method of measuring extremity muscle strength applied to critically ill patients.

    Science.gov (United States)

    Ciesla, Nancy; Dinglas, Victor; Fan, Eddy; Kho, Michelle; Kuramoto, Jill; Needham, Dale

    2011-04-12

    Survivors of acute respiratory distress syndrome (ARDS) and other causes of critical illness often have generalized weakness, reduced exercise tolerance, and persistent nerve and muscle impairments after hospital discharge. Using an explicit protocol with a structured approach to training and quality assurance of research staff, manual muscle testing (MMT) is a highly reliable method for assessing strength, using a standardized clinical examination, for patients following ARDS, and can be completed with mechanically ventilated patients who can tolerate sitting upright in bed and are able to follow two-step commands. (7, 8) This video demonstrates a protocol for MMT, which has been taught to ≥ 43 research staff who have performed >800 assessments on >280 ARDS survivors. Modifications for the bedridden patient are included. Each muscle is tested with specific techniques for positioning, stabilization, resistance, and palpation for each score of the 6-point ordinal Medical Research Council scale. Three upper and three lower extremity muscles are graded in this protocol: shoulder abduction, elbow flexion, wrist extension, hip flexion, knee extension, and ankle dorsiflexion. These muscles were chosen based on the standard approach for evaluating patients for ICU-acquired weakness used in prior publications. (1,2).

  18. The impact of obesity on skeletal muscle strength and structure through adolescence to old age.

    Science.gov (United States)

    Tomlinson, D J; Erskine, R M; Morse, C I; Winwood, K; Onambélé-Pearson, Gladys

    2016-06-01

    Obesity is associated with functional limitations in muscle performance and increased likelihood of developing a functional disability such as mobility, strength, postural and dynamic balance limitations. The consensus is that obese individuals, regardless of age, have a greater absolute maximum muscle strength compared to non-obese persons, suggesting that increased adiposity acts as a chronic overload stimulus on the antigravity muscles (e.g., quadriceps and calf), thus increasing muscle size and strength. However, when maximum muscular strength is normalised to body mass, obese individuals appear weaker. This relative weakness may be caused by reduced mobility, neural adaptations and changes in muscle morphology. Discrepancies in the literature remain for maximal strength normalised to muscle mass (muscle quality) and can potentially be explained through accounting for the measurement protocol contributing to muscle strength capacity that need to be explored in more depth such as antagonist muscle co-activation, muscle architecture, a criterion valid measurement of muscle size and an accurate measurement of physical activity levels. Current evidence demonstrating the effect of obesity on muscle quality is limited. These factors not being recorded in some of the existing literature suggest a potential underestimation of muscle force either in terms of absolute force production or relative to muscle mass; thus the true effect of obesity upon skeletal muscle size, structure and function, including any interactions with ageing effects, remains to be elucidated.

  19. History-dependence of muscle slack length following contraction and stretch in the human vastus lateralis.

    Science.gov (United States)

    Stubbs, Peter W; Walsh, Lee D; D'Souza, Arkiev; Héroux, Martin E; Bolsterlee, Bart; Gandevia, Simon C; Herbert, Robert D

    2018-06-01

    In reduced muscle preparations, the slack length and passive stiffness of muscle fibres have been shown to be influenced by previous muscle contraction or stretch. In human muscles, such behaviours have been inferred from measures of muscle force, joint stiffness and reflex magnitudes and latencies. Using ultrasound imaging, we directly observed that isometric contraction of the vastus lateralis muscle at short lengths reduces the slack lengths of the muscle-tendon unit and muscle fascicles. The effect is apparent 60 s after the contraction. These observations imply that muscle contraction at short lengths causes the formation of bonds which reduce the effective length of structures that generate passive tension in muscles. In reduced muscle preparations, stretch and muscle contraction change the properties of relaxed muscle fibres. In humans, effects of stretch and contraction on properties of relaxed muscles have been inferred from measurements of time taken to develop force, joint stiffness and reflex latencies. The current study used ultrasound imaging to directly observe the effects of stretch and contraction on muscle-tendon slack length and fascicle slack length of the human vastus lateralis muscle in vivo. The muscle was conditioned by (a) strong isometric contractions at long muscle-tendon lengths, (b) strong isometric contractions at short muscle-tendon lengths, (c) weak isometric contractions at long muscle-tendon lengths and (d) slow stretches. One minute after conditioning, ultrasound images were acquired from the relaxed muscle as it was slowly lengthened through its physiological range. The ultrasound image sequences were used to identify muscle-tendon slack angles and fascicle slack lengths. Contraction at short muscle-tendon lengths caused a mean 13.5 degree (95% CI 11.8-15.0 degree) shift in the muscle-tendon slack angle towards shorter muscle-tendon lengths, and a mean 5 mm (95% CI 2-8 mm) reduction in fascicle slack length, compared to the

  20. Blocked muscle fat oxidation during exercise in neutral lipid storage disease

    DEFF Research Database (Denmark)

    Laforêt, Pascal; Ørngreen, Mette; Preisler, Nicolai

    2012-01-01

    To determine whether impaired exercise capacity in neutral lipid storage disease with myopathy is solely caused by muscle weakness or whether a defect in energy metabolism (blocked fat oxidation) may also play a role.......To determine whether impaired exercise capacity in neutral lipid storage disease with myopathy is solely caused by muscle weakness or whether a defect in energy metabolism (blocked fat oxidation) may also play a role....

  1. Healthy Muscles Matter

    Science.gov (United States)

    ... or lying down, and faster when you’re running or playing sports and your skeletal muscles need more blood to help them do their work. What can go wrong? Injuries Almost everyone has had sore muscles after exercising ...

  2. Weak Solution and Weakly Uniformly Bounded Solution of Impulsive Heat Equations Containing “Maximum” Temperature

    Directory of Open Access Journals (Sweden)

    Oyelami, Benjamin Oyediran

    2013-09-01

    Full Text Available In this paper, criteria for the existence of weak solutions and uniformly weak bounded solution of impulsive heat equation containing maximum temperature are investigated and results obtained. An example is given for heat flow system with impulsive temperature using maximum temperature simulator and criteria for the uniformly weak bounded of solutions of the system are obtained.

  3. Oxidative metabolism in muscle.

    OpenAIRE

    Ferrari, M; Binzoni, T; Quaresima, V

    1997-01-01

    Oxidative metabolism is the dominant source of energy for skeletal muscle. Near-infrared spectroscopy allows the non-invasive measurement of local oxygenation, blood flow and oxygen consumption. Although several muscle studies have been made using various near-infrared optical techniques, it is still difficult to interpret the local muscle metabolism properly. The main findings of near-infrared spectroscopy muscle studies in human physiology and clinical medicine are summarized. The advantage...

  4. Diagnosis of functional (psychogenic paresis and weakness

    Directory of Open Access Journals (Sweden)

    Savkov V.S.

    2018-03-01

    Full Text Available Functional (conversion neurological symptoms represent one of the most common situations faced by neurologists in their everyday practice. Among them, acute or subacute functional weakness may mimic very prevalent conditions such as stroke or traumatic injury. In the diagnosis of functional weakness, although elements of the history are helpful, physical signs are often of crucial importance in the diagnosis and positive signs are as important as absence of signs of disease. Hence, accurate and reliable positive signs of functional weakness are valuable for obtaining timely diagnosis and treatment, making it possible to avoid unnecessary or invasive tests and procedures up to thrombolysis. Functional weakness commonly presents as weakness of an entire limb, paraparesis, or hemiparesis, with observable or demonstrable inconsistencies and non-anatomic accompaniments. Documentation of limb movements during sleep, the arm drop test, the Babinski’s trunk-thigh test, Hoover tests, the Sonoo abductor test, and various dynamometer tests can provide useful bedside diagnostic information on functional weakness. We therefore present here a brief overview of the positive neurological signs of functional weakness available, both in the lower and in the upper limbs; but none should be used in isolation and must be interpreted in the overall context of the presentation. It should be borne in mind that a patient may have both a functional and an organic disorder.

  5. Simultaneous versus Sequential Intratympanic Steroid Treatment for Severe-to-Profound Sudden Sensorineural Hearing Loss.

    Science.gov (United States)

    Yoo, Myung Hoon; Lim, Won Sub; Park, Joo Hyun; Kwon, Joong Keun; Lee, Tae-Hoon; An, Yong-Hwi; Kim, Young-Jin; Kim, Jong Yang; Lim, Hyun Woo; Park, Hong Ju

    2016-01-01

    Severe-to-profound sudden sensorineural hearing loss (SSNHL) has a poor prognosis. We aimed to compare the efficacy of simultaneous and sequential oral and intratympanic steroids for this condition. Fifty patients with severe-to-profound SSNHL (>70 dB HL) were included from 7 centers. The simultaneous group (27 patients) received oral and intratympanic steroid injections for 2 weeks. The sequential group (23 patients) was treated with oral steroids for 2 weeks and intratympanic steroids for the subsequent 2 weeks. Pure-tone averages (PTA) and word discrimination scores (WDS) were compared before treatment and 2 weeks and 1 and 2 months after treatment. Treatment outcomes according to the modified American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) criteria were also analyzed. The improvement in PTA and WDS at the 2-week follow-up was 23 ± 21 dB HL and 20 ± 39% in the simultaneous group and 31 ± 29 dB HL and 37 ± 42% in the sequential group; this was not statistically significant. Complete or partial recovery at the 2-week follow-up was observed in 26% of the simultaneous group and 30% of the sequential group; this was also not significant. The improvement in PTA and WDS at the 2-month follow-up was 40 ± 20 dB HL and 37 ± 35% in the simultaneous group and 41 ± 25 dB HL and 48 ± 41% in the sequential group; this was not statistically significant. Complete or partial recovery at the 2-month follow-up was observed in 33% of the simultaneous group and 35% of the sequential group; this was also not significant. Seven patients in the sequential group did not need intratympanic steroid injections for sufficient improvement after oral steroids alone. Simultaneous oral/intratympanic steroid treatment yielded a recovery similar to that produced by sequential treatment. Because the addition of intratympanic steroids can be decided upon based on the improvement after an oral steroid, the sequential regimen can be recommended to avoid unnecessary

  6. Chronic exercise preserves lean muscle mass in masters athletes.

    Science.gov (United States)

    Wroblewski, Andrew P; Amati, Francesca; Smiley, Mark A; Goodpaster, Bret; Wright, Vonda

    2011-09-01

    Aging is commonly associated with a loss of muscle mass and strength, resulting in falls, functional decline, and the subjective feeling of weakness. Exercise modulates the morbidities of muscle aging. Most studies, however, have examined muscle-loss changes in sedentary aging adults. This leaves the question of whether the changes that are commonly associated with muscle aging reflect the true physiology of muscle aging or whether they reflect disuse atrophy. This study evaluated whether high levels of chronic exercise prevents the loss of lean muscle mass and strength experienced in sedentary aging adults. A cross-section of 40 high-level recreational athletes ("masters athletes") who were aged 40 to 81 years and trained 4 to 5 times per week underwent tests of health/activity, body composition, quadriceps peak torque (PT), and magnetic resonance imaging of bilateral quadriceps. Mid-thigh muscle area, quadriceps area (QA), subcutaneous adipose tissue, and intramuscular adipose tissue were quantified in magnetic resonance imaging using medical image processing, analysis, and visualization software. One-way analysis of variance was used to examine age group differences. Relationships were evaluated using Spearman correlations. Mid-thigh muscle area (P = 0.31) and lean mass (P = 0.15) did not increase with age and were significantly related to retention of mid-thigh muscle area (P lean mass (P = 0.4) and PT. This study contradicts the common observation that muscle mass and strength decline as a function of aging alone. Instead, these declines may signal the effect of chronic disuse rather than muscle aging. Evaluation of masters athletes removes disuse as a confounding variable in the study of lower-extremity function and loss of lean muscle mass. This maintenance of muscle mass and strength may decrease or eliminate the falls, functional decline, and loss of independence that are commonly seen in aging adults.

  7. Muscle satellite cell heterogeneity and self-renewal

    Science.gov (United States)

    Motohashi, Norio; Asakura, Atsushi

    2014-01-01

    Adult skeletal muscle possesses extraordinary regeneration capacities. After muscle injury or exercise, large numbers of newly formed muscle fibers are generated within a week as a result of expansion and differentiation of a self-renewing pool of muscle stem cells termed muscle satellite cells. Normally, satellite cells are mitotically quiescent and reside beneath the basal lamina of muscle fibers. Upon regeneration, satellite cells are activated, and give rise to daughter myogenic precursor cells. After several rounds of proliferation, these myogenic precursor cells contribute to the formation of new muscle fibers. During cell division, a minor population of myogenic precursor cells returns to quiescent satellite cells as a self-renewal process. Currently, accumulating evidence has revealed the essential roles of satellite cells in muscle regeneration and the regulatory mechanisms, while it still remains to be elucidated how satellite cell self-renewal is molecularly regulated and how satellite cells are important in aging and diseased muscle. The number of satellite cells is decreased due to the changing niche during ageing, resulting in attenuation of muscle regeneration capacity. Additionally, in Duchenne muscular dystrophy (DMD) patients, the loss of satellite cell regenerative capacity and decreased satellite cell number due to continuous needs for satellite cells lead to progressive muscle weakness with chronic degeneration. Thus, it is necessary to replenish muscle satellite cells continuously. This review outlines recent findings regarding satellite cell heterogeneity, asymmetric division and molecular mechanisms in satellite cell self-renewal which is crucial for maintenance of satellite cells as a muscle stem cell pool throughout life. In addition, we discuss roles in the stem cell niche for satellite cell maintenance, as well as related cell therapies for approaching treatment of DMD. PMID:25364710

  8. Muscle Satellite Cell Heterogeneity and Self-Renewal

    Directory of Open Access Journals (Sweden)

    Norio eMotohashi

    2014-01-01

    Full Text Available Adult skeletal muscle possesses extraordinary regeneration capacities. After muscle injury or exercise, large numbers of newly formed muscle fibers are generated within a week as a result of expansion and differentiation of a self-renewing pool of muscle stem cells termed muscle satellite cells. Normally, satellite cells are mitotically quiescent and reside beneath the basal lamina of muscle fibers. Upon regeneration, satellite cells are activated, and give rise to daughter myogenic precursor cells. After several rounds of proliferation, these myogenic precursor cells contribute to the formation of new muscle fibers. During cell division, a minor population of myogenic precursor cells returns to quiescent satellite cells as a self-renewal process. Currently, accumulating evidence has revealed the essential roles of satellite cells in muscle regeneration and the regulatory mechanisms, while it still remains to be elucidated how satellite cell self-renewal is molecularly regulated and how satellite cells are important in aging and diseased muscle. The number of satellite cells is decreased due to the changing niche during ageing, resulting in attenuation of muscle regeneration capacity. Additionally, in Duchenne muscular dystrophy (DMD patients, the loss of satellite cell regenerative capacity and decreased satellite cell number due to continuous needs for satellite cells lead to progressive muscle weakness with chronic degeneration. Thus, it is necessary to replenish muscle satellite cells continuously. This review outlines recent findings regarding satellite cell heterogeneity, asymmetric division and molecular mechanisms in satellite cell self-renewal which is crucial for maintenance of satellite cells as a muscle stem cell pool throughout life. In addition, we discuss roles in the stem cell niche for satellite cell maintenance, as well as related cell therapies for approaching treatment of DMD.

  9. A study of muscle involvement in scrub typhus.

    Science.gov (United States)

    Kalita, Jayantee; Misra, Usha K; Mani, Vinita E; Mahadevan, Anita; Shankar, Susrala K

    2015-01-15

    Patients with scrub typhus often complain of myalgia, but a comprehensive study on muscle dysfunction is lacking. We therefore report the clinical, electromyographic and muscle biopsy findings in patients with scrub typhus. Consecutive patients with scrub typhus were included, and their clinical and laboratory findings were noted. The patients with myalgia or weakness and elevated serum creatine kinase (CK) were considered to have muscle involvement. Electromyography (EMG) and muscle biopsy were done in some patients. Patients were treated with doxycycline 200mg daily for 7 days, and their clinical and biochemical outcome on discharge and one month were evaluated. 13 out of 33 (39.4%) patients had muscle involvement and their CK levels ranged between 287 and 3166 (859 ± 829) U/L. EMG revealed short duration polyphasic potentials, and muscle histopathology revealed evidence of vasculitis. There were significant correlations between severity of weakness and CK levels (r = -0.6; p scrub typhus. Although muscle histopathology showed evidence of vasculitis, patients responded to doxycycline. Copyright © 2014 Elsevier B.V. All rights reserved.

  10. Targeting superoxide dismutase to endothelial caveolae profoundly alleviates inflammation caused by endotoxin.

    Science.gov (United States)

    Shuvaev, Vladimir V; Kiseleva, Raisa Yu; Arguiri, Evguenia; Villa, Carlos H; Muro, Silvia; Christofidou-Solomidou, Melpo; Stan, Radu V; Muzykantov, Vladimir R

    2018-02-28

    Inflammatory mediators binding to Toll-Like receptors (TLR) induce an influx of superoxide anion in the ensuing endosomes. In endothelial cells, endosomal surplus of superoxide causes pro-inflammatory activation and TLR4 agonists act preferentially via caveolae-derived endosomes. To test the hypothesis that SOD delivery to caveolae may specifically inhibit this pathological pathway, we conjugated SOD with antibodies (Ab/SOD, size ~10nm) to plasmalemmal vesicle-associated protein (Plvap) that is specifically localized to endothelial caveolae in vivo and compared its effects to non-caveolar target CD31/PECAM-1. Plvap Ab/SOD bound to endothelial cells in culture with much lower efficacy than CD31 Ab/SOD, yet blocked the effects of LPS signaling with higher efficiency than CD31 Ab/SOD. Disruption of cholesterol-rich membrane domains by filipin inhibits Plvap Ab/SOD endocytosis and LPS signaling, implicating the caveolae-dependent pathway(s) in both processes. Both Ab/SOD conjugates targeted to Plvap and CD31 accumulated in the lungs after IV injection in mice, but the former more profoundly inhibited LPS-induced pulmonary inflammation and elevation of plasma level of interferon-beta and -gamma and interleukin-27. Taken together, these results indicate that targeted delivery of SOD to specific cellular compartments may offer effective, mechanistically precise interception of pro-inflammatory signaling mediated by reactive oxygen species. Copyright © 2018 Elsevier B.V. All rights reserved.

  11. Smoking cessation induces profound changes in the composition of the intestinal microbiota in humans.

    Directory of Open Access Journals (Sweden)

    Luc Biedermann

    Full Text Available BACKGROUND: The human intestinal microbiota is a crucial factor in the pathogenesis of various diseases, such as metabolic syndrome or inflammatory bowel disease (IBD. Yet, knowledge about the role of environmental factors such as smoking (which is known to influence theses aforementioned disease states on the complex microbial composition is sparse. We aimed to investigate the role of smoking cessation on intestinal microbial composition in 10 healthy smoking subjects undergoing controlled smoking cessation. METHODS: During the observational period of 9 weeks repetitive stool samples were collected. Based on abundance of 16S rRNA genes bacterial composition was analysed and compared to 10 control subjects (5 continuing smokers and 5 non-smokers by means of Terminal Restriction Fragment Length Polymorphism analysis and high-throughput sequencing. RESULTS: Profound shifts in the microbial composition after smoking cessation were observed with an increase of Firmicutes and Actinobacteria and a lower proportion of Bacteroidetes and Proteobacteria on the phylum level. In addition, after smoking cessation there was an increase in microbial diversity. CONCLUSIONS: These results indicate that smoking is an environmental factor modulating the composition of human gut microbiota. The observed changes after smoking cessation revealed to be similar to the previously reported differences in obese compared to lean humans and mice respectively, suggesting a potential pathogenetic link between weight gain and smoking cessation. In addition they give rise to a potential association of smoking status and the course of IBD.

  12. The management of children with Down syndrome and profound hearing loss.

    Science.gov (United States)

    Phelan, E; Pal, R; Henderson, L; Green, K M J; Bruce, I A

    2016-01-01

    Although, the association between Down syndrome (DS) and conductive hearing loss is well recognized, the fact that a small proportion of these children may have a severe to profound sensorineural hearing loss that could benefit from cochlear implantation (CI) is less well understood. The management of significant co-morbidities in children with DS can delay initial diagnosis of hearing impairment and assessment of suitability for CI can likewise be challenging, due to difficulties conditioning to behavioural hearing tests. We performed a retrospective case note review of three children with DS referred to the Manchester Cochlear Implant Programme. Three illustrative cases are described including CI in a 4 years old. Using conventional outcome measurement instruments, the outcome could be considered to be suboptimal with a Categories of Auditory Performance score of 4 at 6 months post-op and at last follow up. In part, this is likely to reflect the delay in implantation, but the role of cognitive impairment must be considered. The cases described emphasize the importance of comprehensive radiological and audiological assessment in children with DS being considered for CI. The influence of cognitive impairment upon outcome of CI must be taken into account, but should not be considered a contra-indication to implantation in children with DS. Benefit that might be considered limited when quantified using existing general outcome measurement instruments, may have a significant impact upon psychosocial development and quality of life in children with significant cognitive impairment, or other additional needs.

  13. Partial neuroprotection by nNOS inhibition during profound asphyxia in preterm fetal sheep.

    Science.gov (United States)

    Drury, Paul P; Davidson, Joanne O; van den Heuij, Lotte G; Tan, Sidhartha; Silverman, Richard B; Ji, Haitao; Blood, Arlin B; Fraser, Mhoyra; Bennet, Laura; Gunn, Alistair Jan

    2013-12-01

    Preterm brain injury is partly associated with hypoxia-ischemia starting before birth. Excessive nitric oxide production during HI may cause nitrosative stress, leading to cell membrane and mitochondrial damage. We therefore tested the hypothesis that therapy with a new, selective neuronal nitric oxide synthase (nNOS) inhibitor, JI-10 (0.022mg/kg bolus, n=8), given 30min before 25min of complete umbilical cord occlusion was protective in preterm fetal sheep at 101-104day gestation (term is 147days), compared to saline (n=8). JI-10 had no effect on fetal blood pressure, heart rate, carotid and femoral blood flow, total EEG power, nuchal activity, temperature or intracerebral oxygenation on near-infrared spectroscopy during or after occlusion. JI-10 was associated with later onset of post-asphyxial seizures compared with saline (p<0.05), and attenuation of the subsequent progressive loss of cytochrome oxidase (p<0.05). After 7days recovery, JI-10 was associated with improved neuronal survival in the caudate nucleus (p<0.05), but not the putamen or hippocampus, and more CNPase positive oligodendrocytes in the periventricular white matter (p<0.05). In conclusion, prophylactic nNOS inhibition before profound asphyxia was associated with delayed onset of seizures, slower decline of cytochrome oxidase and partial white and gray matter protection, consistent with protection of mitochondrial function. © 2013.

  14. Attentional processes in interactions between people with profound intellectual and multiple disabilities and direct support staff.

    Science.gov (United States)

    Hostyn, Ine; Ine, Hostyn; Neerinckx, Heleen; Heleen, Neerinckx; Maes, Bea; Bea, Maes

    2011-01-01

    Few studies have examined joint attention in interactions with persons with profound intellectual and multiple disabilities (PIMD), despite its important role in high-quality interaction. The purpose of this study is to describe the attention-directing behaviours of persons with PIMD and their direct support staff and the attention episodes resulting from their interactions, and to understand how these variables relate to each other. Video observations of 17 staff-client dyads were coded using partial interval recording. The results showed considerable variation across individuals and dyads. In general, persons with PIMD directed the attention of staff members infrequently. The staff members frequently directed their clients' attention towards a topic of interest but did not often use the tactile modality. Within the staff-client dyad, there was not much joint attention; however, shared attention episodes occurred frequently. Shared attention and joint attention are strongly correlated. A negative correlation was found between clients not using attention-directing behaviours and staff members using tactile methods to direct the attention, and joint attention episodes. This study presents both directions for future research and practical implications. Copyright © 2010 Elsevier Ltd. All rights reserved.

  15. The current status of audiologic rehabilitation for profound unilateral sensorineural hearing loss.

    Science.gov (United States)

    Bishop, Charles E; Eby, Thomas L

    2010-03-01

    Audiologic rehabilitation of individuals with profound unilateral sensorineural hearing loss (USNHL) has traditionally been limited to the use of air-conduction contralateral routing of sound (CROS) hearing aids. Treatment for these individuals has expanded with new applications of the bone-anchored hearing aid (BAHA), transcranial hearing aid (t-CROS), and the cochlear implant. In this article, the authors review the literature that addresses these various treatment options. Contemporary review Historical information is available that describes the limited efficacy of air-conduction CROS hearing aids in lifting hearing handicap associated with USNHL. Current investigations on providing cross hearing are generally focused on use of the BAHA. Little is known at present whether new developments in hearing aid technology can improve on conventional air-conduction CROS or t-CROS approaches. Interestingly, the cochlear implant seems to be a viable option for individuals with USNHL and tinnitus who also have intact auditory nerve pathways. There is indication in the literature that BAHA provides greater relief of hearing handicap associated with USNHL than CROS hearing aids; however, both have been found to provide limited patient satisfaction and seemingly fall short of restoring true sound localization. Adequate trials have not been performed comparing BAHA with the best CROS hearing aid technology. Transcranial hearing aids and cochlear implants are experimental methods to treat USNHL and hold promise, although there remains a lack of studies available to fully support this.

  16. Differentiating characteristics of deafblindness and autism in people with congenital deafblindness and profound intellectual disability.

    Science.gov (United States)

    Hoevenaars-van den Boom, M A A; Antonissen, A C F M; Knoors, H; Vervloed, M P J

    2009-06-01

    In persons with deafblindness, it is hard to distinguish autism spectrum disorders from several deafblind specific behaviours caused by the dual sensory impairments, especially when these persons are also intellectually disabled. As a result, there is an over-diagnosis of autism in persons who are deafblind leading to unsuitable interventions. Autism as specified by the DSM-IV was studied in 10 persons with congenital deafblindness with profound intellectual disabilities. Behaviours of people with deafblindness and autism (n = 5) and of people with deafblindness without autism (n = 5) were observed in a semi-standardised assessment. All people with deafblindness showed impairments in social interaction, communication and language. In contrast to persons without autism, people with deafblindness and autism showed significantly more impairments in reciprocity of social interaction, quality of initiatives to contact and the use of adequate communicative signals and functions. No differences between the groups were found for quantity and persistence of stereotyped behaviour, quality of play and exploration and adequate problem-solving strategies. This study indicates that there are some possibilities to differentiate autism from behaviours specific for deafblindness. It also confirms the large overlap in overt behaviours between people with deafblindness and persons with autism.

  17. Anterior cruciate ligament tear induces a sustained loss of muscle fiber force production.

    Science.gov (United States)

    Gumucio, Jonathan P; Sugg, Kristoffer B; Enselman, Elizabeth R Sibilsky; Konja, Alexis C; Eckhardt, Logan R; Bedi, Asheesh; Mendias, Christopher L

    2018-01-18

    Patients with anterior cruciate ligament (ACL) tears have persistent quadriceps strength deficits that are thought to be due to altered neurophysiological function. Our goal was to determine the changes in muscle fiber contractility independent of the ability of motor neurons to activate fibers. We obtained quadriceps biopsies of patients undergoing ACL reconstruction, and additional biopsies 1, 2, and 6 months after surgery. Muscles fiber contractility was assessed in vitro, along with whole muscle strength testing. Compared with controls, patients had a 30% reduction in normalized muscle fiber force at the time of surgery. One month later, the force deficit was 41%, and at 6 months the deficit was 23%. Whole muscle strength testing demonstrated similar trends. While neurophysiological dysfunction contributes to whole muscle weakness, there is also a reduction in the force generating capacity of individual muscle cells independent of alpha motor neuron activation. Muscle Nerve, 2018. © 2018 Wiley Periodicals, Inc.

  18. Effect of Gender, Disease Duration and Treatment on Muscle Strength in Myasthenia Gravis

    DEFF Research Database (Denmark)

    Citirak, Gülsenay; Cejvanovic, Sanja; Andersen, Henning

    2016-01-01

    INTRODUCTION: The aim of this observational, cross-sectional study was to quantify the potential presence of muscle weakness among patients with generalized myasthenia gravis (gMG). The influence of gender, treatment intensity and disease duration on muscle strength and disease progression was also...

  19. Skeletal Muscle Remodelling as a Function of Disease Progression in Amyotrophic Lateral Sclerosis

    DEFF Research Database (Denmark)

    Jensen, L; Jørgensen, L H; Bech, R D

    2016-01-01

    Muscle weakness is considered the pivotal sign of amyotrophic lateral sclerosis (ALS). Knowledge about the skeletal muscle degeneration/regeneration process and the myogenic potential is limited in ALS patients. Therefore, we investigate these processes in a time course perspective by analysing s...

  20. Functional ability and muscle force in healthy children and ambulant Duchenne muscular dystrophy patients

    NARCIS (Netherlands)

    Beenakker, EAC; Maurits, NM; Fock, JM; Brouwer, OF; van der Hoeven, JH

    2005-01-01

    Neuromuscular disorders are characterised by progressive muscle weakness, which in time causes functional impairment. To quantify the extent of disease progression, muscle force and functional ability can be measured. Which of these parameters changes most depends on the disease stage. In a previous

  1. Effect of whole-body vibration on muscle strength, spasticity, and ...

    African Journals Online (AJOL)

    CP) and is characterized by spasticity and muscle weakness of both lower limbs resulting in decreased walking ability. The purpose of this study was to evaluate the effect of whole body vibration (WBV) training on muscle strength, spasticity, and ...

  2. Mouse Plantar Flexor Muscle Size and Strength After Inactivity and Training

    Science.gov (United States)

    2010-07-01

    atrophy and weakness as a function of the intensity of the train- ing ( 15 ). Although the hind limb suspension resulted in loss of muscle mass in...Muscle Biology Laboratory, Department of Health and Kinesiology , Texas A&M University, College Station, TX, and Gordon L. Warren, Ph.D., Division of

  3. Driving to learn in a powered wheelchair: the process of learning joystick use in people with profound cognitive disabilities.

    Science.gov (United States)

    Nilsson, Lisbeth; Eklund, Mona; Nyberg, Per; Thulesius, Hans

    2011-01-01

    The Driving to Learn project explored ways to help people with profound cognitive disabilities practice operating a joystick-operated powered wheelchair. The project used a grounded theory approach with constant comparative analysis and was carried out over 12 yr. The participants were 45 children and adults with profound cognitive disabilities. Reference groups included 17 typically developing infants and 64 participants with lesser degrees of cognitive disability. The data sources included video recordings, field notes, open interviews, and a rich mixture of literature. The findings that emerged yielded strategies for facilitating achievements, an 8-phase learning process, an assessment tool, and a grounded theory of deplateauing explaining the properties necessary for participants to exceed expected limitations and plateaus. Eight participants with profound cognitive disabilities reached goal-directed driving or higher. Participants were empowered by attaining increased control over tool use, improving their autonomy and quality of life.

  4. Reliability of Ultrasonographic Measurement of Cervical Multifidus Muscle Dimensions during Isometric Contraction of Neck Muscles

    Directory of Open Access Journals (Sweden)

    Somayeh Amiri Arimi

    2012-07-01

    Full Text Available Background and Aim: Cervical multifidus is considered as one of the most important neck stabilizers. Weakness and muscular atrophy of this muscle were seen in patients with chronic neck pain. Ultrasonographic imaging is a non-invasive and feasible technique that commonly used to record such changes and measure muscle dimensions. Therefore, the aim of this study was to evaluate the reliability of ultrasonographic measurement of cervical multifidus muscle’s dimensions during isometric contraction of neck muscles. Materials and Method: Ten subjects (5 patients with chronic neck pain and 5 healthy subjects were recruited in this study. Cervical multifidus muscle’s dimensions were measured at the level of forth cervical vertebrae. Ultrasonographic measurement of cervical multifidus muscle at rest, 50% and 100% of maximal voluntary contraction (MVC were performed by one examiner within 1 week interval. The dimensions of cervical multifidus muscle including cross-sectional area (CSA, anterior posterior dimension (APD, and lateral dimension (LD were measured. Intraclass correlation coefficients (ICC, standard error of measurement (SEM and minimal detectable change (MDC were computed for data analysis.Results: The between days reliability of maximum strength of neck muscles and multifidus muscle dimensions at rest, 50% and 100% of MVC of neck muscles were good to excellent (ICC=0.75-0.99.Conclusion: The results of this study showed that ultrasonographic measuring of cervical multifidus muscle’s dimensions during isometric contraction of neck muscles at the level of C4 in females with chronic neck pain and healthy subjects is a reliable and repeatable method.

  5. Exercise and nutritional interventions for improving aging muscle health.

    Science.gov (United States)

    Forbes, Scott C; Little, Jonathan P; Candow, Darren G

    2012-08-01

    Skeletal muscle mass declines with age (i.e., sarcopenia) resulting in muscle weakness and functional limitations. Sarcopenia has been associated with physiological changes in muscle morphology, protein and hormonal kinetics, insulin resistance, inflammation, and oxidative stress. The purpose of this review is to highlight how exercise and nutritional intervention strategies may benefit aging muscle. It is well known that resistance exercise training increases muscle strength and size and evidence also suggests that resistance training can increase mitochondrial content and decrease oxidative stress in older adults. Recent findings suggest that fast-velocity resistance exercise may be an effective intervention for older adults to enhance muscle power and functional capacity. Aerobic exercise training may also benefit aging skeletal muscle by enhancing mitochondrial bioenergetics, improving insulin sensitivity, and/or decreasing oxidative stress. In addition to exercise, creatine monohydrate, milk-based proteins, and essential fatty acids all have biological effects which could enhance some of the physiological adaptations from exercise training in older adults. Additional research is needed to determine whether skeletal muscle adaptations to increased activity in older adults are further enhanced with effective nutritional interventions and whether this is due to enhanced muscle protein synthesis, improved mitochondrial function, and/or a reduced inflammatory response.

  6. Association between statin-associated myopathy and skeletal muscle damage.

    Science.gov (United States)

    Mohaupt, Markus G; Karas, Richard H; Babiychuk, Eduard B; Sanchez-Freire, Verónica; Monastyrskaya, Katia; Iyer, Lakshmanan; Hoppeler, Hans; Breil, Fabio; Draeger, Annette

    2009-07-07

    Many patients taking statins often complain of muscle pain and weakness. The extent to which muscle pain reflects muscle injury is unknown. We obtained biopsy samples from the vastus lateralis muscle of 83 patients. Of the 44 patients with clinically diagnosed statin-associated myopathy, 29 were currently taking a statin, and 15 had discontinued statin therapy before the biopsy (minimal duration of discontinuation 3 weeks). We also included 19 patients who were taking statins and had no myopathy, and 20 patients who had never taken statins and had no myopathy. We classified the muscles as injured if 2% or more of the muscle fibres in a biopsy sample showed damage. Using reverse transcriptase polymerase chain reaction, we evaluated the expression levels of candidate genes potentially related to myocyte injury. Muscle injury was observed in 25 (of 44) patients with myopathy and in 1 patient without myopathy. Only 1 patient with structural injury had a circulating level of creatine phosphokinase that was elevated more than 1950 U/L (10x the upper limit of normal). Expression of ryanodine receptor 3 was significantly upregulated in patients with biopsy evidence of structural damage (1.7, standard error of the mean 0.3). Persistent myopathy in patients taking statins reflects structural muscle damage. A lack of elevated levels of circulating creatine phosphokinase does not rule out structural muscle injury. Upregulation of the expression of ryanodine receptor 3 is suggestive of an intracellular calcium leak.

  7. Weak entropy inequalities and entropic convergence

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    A criterion for algebraic convergence of the entropy is presented and an algebraic convergence result for the entropy of an exclusion process is improved. A weak entropy inequality is considered and its relationship to entropic convergence is discussed.

  8. Current problems in the weak interactions

    International Nuclear Information System (INIS)

    Pais, A.

    1977-01-01

    Some reasons are discussed showing why the recent SU(2) x U(1) gauge theory of weak and electromagnetic interactions is not a complete theory of these interactions, Lepton theory, charm, and the CP problem are considered. 60 references

  9. From Suitable Weak Solutions to Entropy Viscosity

    KAUST Repository

    Guermond, Jean-Luc; Pasquetti, Richard; Popov, Bojan

    2010-01-01

    This paper focuses on the notion of suitable weak solutions for the three-dimensional incompressible Navier-Stokes equations and discusses the relevance of this notion to Computational Fluid Dynamics. The purpose of the paper is twofold (i

  10. Subsidy Competition for FDI: Fierce or Weak?

    OpenAIRE

    Tomáš Havránek

    2009-01-01

    The objective of this paper is to empirically assess the recently introduced models of subsidy competition based on the classical oligopoly theories, using both cross-sectional and panel data. Three crucial scenarios (including coordination, weak competition, and fierce competition) are tested employing OLS, iteratively re-weighted least squares, fixed effects, and Blundell-Bond estimator. The results suggest that none of the scenarios can be strongly supported—although there is some weak sup...

  11. About some distinguishing features of weak interactions

    International Nuclear Information System (INIS)

    Beshtoev, Kh.M.

    1999-01-01

    It is shown that, in contrast to strong and electromagnetic theories, additive conserved numbers (such as lepton, aromatic and another numbers) and γ 5 anomaly do not appear in the standard weak interaction theory. It means that in this interaction the additive numbers cannot be conserved. These results are the consequence of specific character of the weak interaction: the right components of spinors do not participate in this interaction. The schemes of violation of the aromatic and lepton numbers were considered

  12. Weak limits for quantum random walks

    International Nuclear Information System (INIS)

    Grimmett, Geoffrey; Janson, Svante; Scudo, Petra F.

    2004-01-01

    We formulate and prove a general weak limit theorem for quantum random walks in one and more dimensions. With X n denoting position at time n, we show that X n /n converges weakly as n→∞ to a certain distribution which is absolutely continuous and of bounded support. The proof is rigorous and makes use of Fourier transform methods. This approach simplifies and extends certain preceding derivations valid in one dimension that make use of combinatorial and path integral methods

  13. Drift waves in a weakly ionized plasma

    DEFF Research Database (Denmark)

    Popovic, M.; Melchior, H.

    1968-01-01

    A dispersion relation for low frequency drift waves in a weakly ionized plasma has been derived, and through numerical calculations the effect of collisions between the charged and the neutral particles is estimated.......A dispersion relation for low frequency drift waves in a weakly ionized plasma has been derived, and through numerical calculations the effect of collisions between the charged and the neutral particles is estimated....

  14. Energetic aspects of skeletal muscle contraction: implications of fiber types.

    Science.gov (United States)

    Rall, J A

    1985-01-01

    . Unlike economy, maximum efficiency of work production varies little across the animal kingdom. There are difficulties associated with the measurement of maximum efficiency of contraction, and it has yet to be determined unequivocally if the maximum efficiency of contraction varies in different fiber types. The intrinsic properties of force per cross-sectional area, economy, and Vmax determine the basic energetic properties of skeletal muscles. Nonetheless, the mechanics and energetics of skeletal muscles in the body are profoundly influenced by muscle architecture, attachment of muscles to the skeleton, and motor unit organization.(ABSTRACT TRUNCATED AT 400 WORDS)

  15. [Adverse muscle effects of a podofyllotoxin-containing cytotoxic drug product with simvastatin].

    Science.gov (United States)

    Kaipiainen-Seppänen, Oili; Savolainen, Elina; Elfving, Pia; Kononoff, Aulikki

    2009-01-01

    With the ageing population, drug interactions pose an increasing challenge to health professionals. We describe four patients, for whom concurrent administration of a podofyllotoxin-containing cytotoxic drug product and simvastatin caused severe adverse effects on muscles, including muscle pain, soreness or fatigue or weakness, and in some patients also disintegration of muscle tissue, i.e. rhabdomyolysis. The metabolism of both drugs proceeds via the common CYP3A4 enzyme pathway.

  16. Attending to weak signals: the leader's challenge.

    Science.gov (United States)

    Kerfoot, Karlene

    2005-12-01

    Halverson and Isham (2003) quote sources that report the accidental death rate of simply being in a hospital is " ... four hundred times more likely than your risk of death from traveling by train, forty times higher than driving a car, and twenty times higher than flying in a commercial aircraft" (p. 13). High-reliability organizations such as nuclear power plants and aircraft carriers have been pioneers in the business of recognizing weak signals. Weike and Sutcliffe (2001) note that high-reliability organizations distinguish themselves from others because of their mindfulness which enables them to see the significance of weak signals and to give strong interventions to weak signals. To act mindfully, these organizations have an underlying mental model of continually updating, anticipating, and focusing the possibility of failure using the intelligence that weak signals provides. Much of what happens is unexpected in health care. However, with a culture that is continually looking for weak signals, and intervenes and rescues when these signals are detected, the unexpected happens less often. This is the epitome of how leaders can build a culture of safety that focuses on recognizing the weak signals to manage the unforeseen.

  17. Muscle-directed gene therapy for phenylketonuria (PKU): Development of transgenic mice with muscle-specific phenylalanine hydroxylase expression

    Energy Technology Data Exchange (ETDEWEB)

    Harding, C.O.; Messing, A.; Wolff, J.A. [Univ. of Wisconsin, Madison, WI (United States)

    1994-09-01

    Phenylketonuria (PKU) is an attractive target for gene therapy because of shortcomings in current therapy including lifelong commitment to a difficult and expensive diet, persistent mild cognitive deficits in some children despite adequate dietary therapy, and maternal PKU syndrome. Phenylalanine hydroxylase (PAH) is normally expressed only in liver, but we propose to treat PKU by introducing the gene for PAH into muscle. In order to evaluate both the safety and efficacy of this approach, we have a developed a trangenic mouse which expresses PAH in both cardiac and skeletal muscle. The transgene includes promoter and enhancer sequences from the mouse muscle creatine kinase (MCK) gene fused to the mouse liver PAH cDNA. Mice which have inherited the transgene are healthy, active, and do not exhibit any signs of muscle weakness or wasting. Ectopic PAH expression in muscle is not detrimental to the health, neurologic function, or reproduction of the mice. Pah{sup enu2} hyperphenylalaninemic mice, a model of human PAH deficiency, bred to carry the transgene have substantial PAH expression in cardiac and skeletal muscle but none in liver. Muscle PAH expression alone does not complement the hyperphenylalaninemic phenotype of Pah{sup enu2} mice. However, administration of reduced tetrahydrobiopterin to transgenic Pah{sup enu2} mice is associated with a 25% mean decrease in serum phenylalanine levels. We predict that ectopic expression of PAH in muscle along with adequate muscle supplies of reduced biopterin cofactor will decrease hyperphenylalaninemia in PKU.

  18. 38 CFR 4.56 - Evaluation of muscle disabilities.

    Science.gov (United States)

    2010-07-01

    ..., the cardinal signs and symptoms of muscle disability are loss of power, weakness, lowered threshold of fatigue, fatigue-pain, impairment of coordination and uncertainty of movement. (d) Under diagnostic codes... power or lowered threshold of fatigue when compared to the sound side. (3) Moderately severe disability...

  19. Morphology and histochemistry of primary flight muscles in ...

    African Journals Online (AJOL)

    preincubation staining protocol for myosin ATPase. The primary flight muscles, serratus ventralis included type I, type IIa and type IIb fibers. Type I fibers were highly oxidative, as stained dark for NADHTR. Type IIa fibers exhibited relatively weak staining properties for NADH-TR and SDH, indicating an intermediate oxidative ...

  20. A muscle stem cell for every muscle: variability of satellite cell biology among different muscle groups

    Science.gov (United States)

    Randolph, Matthew E.; Pavlath, Grace K.

    2015-01-01

    The human body contains approximately 640 individual skeletal muscles. Despite the fact that all of these muscles are composed of striated muscle tissue, the biology of these muscles and their associated muscle stem cell populations are quite diverse. Skeletal muscles are affected differentially by various muscular dystrophies (MDs), such that certain genetic mutations specifically alter muscle function in only a subset of muscles. Additionally, defective muscle stem cells have been implicated in the pathology of some MDs. The biology of muscle stem cells varies depending on the muscles with which they are associated. Here we review the biology of skeletal muscle stem cell populations of eight different muscle groups. Understanding the biological variation of skeletal muscles and their resident stem cells could provide valuable insight into mechanisms underlying the susceptibility of certain muscles to myopathic disease. PMID:26500547

  1. A Glance on Diagnosis and Ttreatment of Profound Fatigue in Multiple Sclerosis

    Directory of Open Access Journals (Sweden)

    Mojtaba Azimian

    2007-09-01

    Full Text Available Multiple Sclerosis (M.S is one of the prevalent diseases of the central nervous system which cause disability in young adults. This appears as neural signs in different parts and various times. The main defect is local deterioration or destruction of myelin tissue in the brain and spinal cord. Profound fatigue is one the most common symptoms of this disease. Because MS is prevalent in middle ages, so the presence of this symptom affects the life and activity of the patient In these ages people have high amount of activities and this functional disorder creates many difficulties for daily works in patient. For this reason, fatigue problem in patients, always, have caught the attention of researchers. Despite numerous studies, still the exact reason for producing fatigue have not been mentioned But several mechanisms have been discussed, such as: autoimmune mechanism of the disease, Involvement and disorder of endocrine system, involvement of central nervous system and de-synchronization of autonom system. Among these, autoimmune mechanism is considered as a main role in producing fatigue. Lack of proper knowledge regarding exact fatigue mechanism several problems in its treatment. Evaluation is the first step for controlling fatigue, which is done through various tests. Clear and proper evaluation can be obtained by these tests. After evaluation, non-drug and finally drug procedures are helpful. In non-drug treatment the focus is on: sport exercises, suitable diet program, identification and improvement of the patient psychotic disorders, energy conservation methods. Finally, the use of drugs such as Amantadine, Pemoline and Modafini is recommended for fatigue treatment.

  2. Profound tumor-specific Th2 bias in patients with malignant glioma

    International Nuclear Information System (INIS)

    Shimato, Shinji; Maier, Lisa M; Maier, Richard; Bruce, Jeffrey N; Anderson, Richard CE; Anderson, David E

    2012-01-01

    Vaccination against tumor-associated antigens is one promising approach to immunotherapy against malignant gliomas. While previous vaccine efforts have focused exclusively on HLA class I-restricted peptides, class II-restricted peptides are necessary to induce CD4 + helper T cells and sustain effective anti-tumor immunity. In this report we investigated the ability of five candidate peptide epitopes derived from glioma-associated antigens MAGE and IL-13 receptor α2 to detect and characterize CD4 + helper T cell responses in the peripheral blood of patients with malignant gliomas. Primary T cell responses were determined by stimulating freshly isolated PBMCs from patients with primary glioblastoma (GBM) (n = 8), recurrent GBM (n = 5), meningioma (n = 7), and healthy controls (n = 6) with each candidate peptide, as well as anti-CD3 monoclonal antibody (mAb) and an immunodominant peptide epitope derived from myelin basic protein (MBP) serving as positive and negative controls, respectively. ELISA was used to measure IFN-γ and IL-5 levels, and the ratio of IFN-γ/IL-5 was used to determine whether the response had a predominant Th1 or Th2 bias. We demonstrate that novel HLA Class-II restricted MAGE-A3 and IL-13Rα2 peptides can detect T cell responses in patients with GBMs as well as in healthy subjects. Stimulation with a variety of peptide antigens over-expressed by gliomas is associated with a profound reduction in the IFN-γ/IL-5 ratio in GBM patients relative to healthy subjects. This bias is more pronounced in patients with recurrent GBMs. Therapeutic vaccine strategies to shift tumor antigen-specific T cell response to a more immunostimulatory Th1 bias may be needed for immunotherapeutic trials to be more successful clinically

  3. Cochlear implantation: is hearing preservation necessary in severe to profound hearing loss?

    Science.gov (United States)

    Derinsu, Ufuk; Serin, Gediz Murat; Akdaş, Ferda; Batman, Çağlar

    2011-03-01

    The goal of the cochlear implant surgery is to place the electrode array with minimal damage to preserve the residual hearing. Round-window insertion can be performed in a manner that is potentially less traumatic than the standard cochleostomy. The purpose of the study was to investigate audiological results of the round-window approach using standard electrode. A retrospective study was performed to evaluate our experience in patients with implanted through round window between January 2007 and March 2009. Sixty patients had undergone cochlear implant surgery through the round window with full insertion of a standard electrode array. Preoperative and postoperative pure-tone thresholds were measured for implanted ears in the range of 250 to 4000 Hz. Within these 60 cases, 31 patients had been evaluated. The population comprised 16 women and 15 men. The mean age was 15.96 years (range, 4-64 years). Follow-up times ranged from 6 to 26 months. Preservation of low-frequency hearing (250 and 500 Hz) was achieved in 27 (87%) of 31 patients. Complete hearing preservation (all frequencies) was accomplished in 11 patients (35.48%). No hearing could be determined postoperatively in 4 patients (12.9%), having preoperative thresholds of 120 dB at 250, 500, and 1000 Hz. Round-window approach has been widely used for preservation of residual hearing. In our patients with severe to profound hearing loss, we preserved residual hearing. Although the residual hearing cannot be sufficient for using additional acoustic stimulation, the preserved residual hearing means minimal damage and a more convenient cochlea, so this is promising for future development.

  4. Recurrent Vestibular Migraine Vertigo Attacks Associated With the Development of Profound Bilateral Vestibulopathy: A Case Series.

    Science.gov (United States)

    Wester, Jacob L; Ishiyama, Akira; Ishiyama, Gail

    2017-09-01

    Bilateral vestibulopathy (BVP) is a debilitating condition characterized by gait ataxia, oscillopsia, and imbalance. Case series of patients with migraine-linked vertigo spells and profound BVP. PATIENT 1:: A 69-year-old man presented with a history of recurrent severe vertigo spells lasting up to 3 days in duration associated with prostrating migraine headaches starting at age 60. His symptoms were misdiagnosed as an anxiety syndrome. At age 68, electronystagmography (ENG) revealed bilaterally absent caloric responses and complete BVP. His hearing was normal. PATIENT 2:: A 51-year-old man presented with a history of "earthquake-like" vertigo, sharp head pain, and phonophobia. These episodes occurred a handful of times over a 7-year period. Previous ENG testing at age 43 was normal. However, his ENG at age 48 revealed complete BVP. He was started on acetazolamide and noted improved balance, although subsequent ENG was unchanged. PATIENT 3:: A 49-year-old woman presented with a history of recurrent migraines with visual aura associated with vertigo lasting 1 hour. ENG at age 50 revealed complete BVP. Subjectively, she noted improved balance with acetazolamide and subsequent ENG demonstrated mild improvement. PATIENT 4:: A 43-year-old man presented with a 5-year history of optical migraines and recurrent vertigo spells, lasting 30 seconds, which was misdiagnosed as positional vertigo. He additionally had a 10-year history of oscillopsia. ENG at age 61 revealed complete BVP. In these cases, vestibular migraine was linked to recurrent vertigo spells that eventually led to complete bilateral vestibulopathy.

  5. The notochord in Atlantic salmon (Salmo salar L.) undergoes profound morphological and mechanical changes during development.

    Science.gov (United States)

    Kryvi, Harald; Rusten, Iselin; Fjelldal, Per Gunnar; Nordvik, Kari; Totland, Geir K; Karlsen, Tine; Wiig, Helge; Long, John H

    2017-11-01

    We present the development of the notochord of the Atlantic salmon (Salmo salar L.), from early embryo to sexually mature fish. Over the salmon's lifespan, profound morphological changes occur. Cells and gross structures of the notochord reorganize twice. In the embryo, the volume of the notochord is dominated by large, vacuolated chordocytes; each cell can be modeled as a hydrostat organized into a larger cellular-hydrostat network, structurally bound together with desmosomes. After the embryo hatches and grows into a fry, vacuolated chordocytes disappear, replaced by extracellular lacunae. The formation of mineralized, segmental chordacentra stiffens the notochord and creates intervertebral joints, where tissue strain during lateral bending is now focused. As development proceeds towards the parr stage, a process of devacuolization and intracellular filament accumulation occur, forming highly dense, non-vacuolated chordocytes. As extracellular lacunae enlarge, they are enclosed by dense filamentous chordocytes that form transverse intervertebral septa, which are connected to the intervertebral ligaments, and a longitudinal notochordal strand. In the vertebral column of pelagic adults, large vacuolated chordocytes reappear; cells of this secondary population have a volume up to 19 000 times larger than the primary vacuolated chordocytes of the early notochord. In adults the lacunae have diminished in relative size. Hydrostatic pressure within the notochord increases significantly during growth, from 525 Pa in the alevins to 11 500 Pa in adults, at a rate of increase with total body length greater than that expected by static stress similarity. Pressure and morphometric measurements were combined to estimate the stress in the extracellular material of the notochordal sheath and intervertebral ligaments and the flexural stiffness of the axial skeleton. The functional significance of the morphological changes in the axial skeleton is discussed in relation to the

  6. NATURE FACILITATES CONNECTION WITH THE PROFOUND SELF: NEEDS, GOALS AND RESOURCE AWARENESS

    Directory of Open Access Journals (Sweden)

    Nicoleta Răban-Motounu

    2014-11-01

    Full Text Available The present study is based on Kaplan and Kaplan’s (1989 theory explaining the restorative effects that nature has on a person’s psychic. According to this theory, nature exerts a “soft fascination” combining the activation of involuntary attention mechanisms with the reflexive awareness, allowing a spontaneous detachment from stress factors and automatic functioning, and also the feeling of compatibility between personal purposes, and the possibilities for action offered by the environment (a sense of meaning. Our objective was to investigate the effects of nature on Self awareness: the immediate, emotional experience; needs awareness and organization; plans for action, and availability of resources, both personal, and external. We conducted an experiment with an experimental group (persons watching a video with life in nature and an active control group (involved in a psychotherapeutic technique focused on confronting and solving personal difficulties by creative means, accompanied by a short psychological analysis. The effects were assessed in terms of “here and now” emotions and available resources according to a self-evaluation scale, and with open-ended questions regarding personal needs and goals. The results showed that, for the experimental group, the relaxation effects and the awareness of long term goals were stronger, while all the other effects were the same as for the control group. The results suggest that indeed, nature helps a person get in contact with her profound Self, allowing the access to both “here and now” basic needs, and also long term goals (inner sources of meaning, the sense of connection between internal tendencies, personal, and external resources, resulting in increased positive emotions, and decreased negative emotions. Nature contemplation may facilitate a meditative state whit all its positive effects.

  7. Classic Bartter syndrome complicated with profound growth hormone deficiency: a case report.

    Science.gov (United States)

    Adachi, Masanori; Tajima, Toshihiro; Muroya, Koji; Asakura, Yumi

    2013-12-30

    Classic Bartter syndrome is a salt-wasting tubulopathy caused by mutations in the CLCNKB (chloride channel Kb) gene. Although growth hormone deficiency has been suggested as a cause for persistent growth failure in patients with classic Bartter syndrome, in our opinion the diagnoses of growth hormone deficiency has been unconvincing in some reports. Moreover, Gitelman syndrome seems to have been confused with Bartter syndrome in some cases in the literature. In the present work, we describe a new case with CLCNKB gene mutations and review the reported cases of classic Bartter syndrome associated with growth hormone deficiency. Our patient was a Japanese boy diagnosed as having classic Bartter syndrome at eight months of age. The diagnosis of Bartter syndrome was confirmed by CLCNKB gene analysis, which revealed compound heterozygous mutations with deletion of exons 1 to 3 (derived from his mother) and ΔL130 (derived from his father). His medical therapy consisted of potassium (K), sodium chloride, spironolactone, and anti-inflammatory agents; this regime was started at eight months of age. Our patient was very short (131.1cm, -4.9 standard deviation) at 14.3 years and showed profoundly impaired growth hormone responses to pharmacological stimulants: 0.15μg/L to insulin-induced hypoglycemia and 0.39μg/L to arginine. His growth response to growth hormone therapy was excellent. The present case strengthens the association between classic Bartter syndrome and growth hormone deficiency. We propose that growth hormone status should be considered while treating children with classic Bartter syndrome.

  8. Achieving and sustaining profound institutional change in healthcare: case study using neo-institutional theory.

    Science.gov (United States)

    Macfarlane, Fraser; Barton-Sweeney, Cathy; Woodard, Fran; Greenhalgh, Trisha

    2013-03-01

    Change efforts in healthcare sometimes have an ambitious, whole-system remit and seek to achieve fundamental changes in norms and organisational culture rather than (or as well as) restructuring the service. Long-term evaluation of such initiatives is rarely undertaken. We report a secondary analysis of data from an evaluation of a profound institutional change effort in London, England, using a mixed-method longitudinal case study design. The service had received £15 million modernisation funding in 2004, covering multiple organisations and sectors and overseen by a bespoke management and governance infrastructure that was dismantled in 2008. In 2010-11, we gathered data (activity statistics, documents, interviews, questionnaires, site visits) and compared these with data from 2003 to 2008. Data analysis was informed by neo-institutional theory, which considers organisational change as resulting from the material-resource environment and three 'institutional pillars' (regulative, normative and cultural-cognitive), enacted and reproduced via the identities, values and activities of human actors. Explaining the long-term fortunes of the different components of the original programme and their continuing adaptation to a changing context required attention to all three of Scott's pillars and to the interplay between macro institutional structures and embedded human agency. The paper illustrates how neo-institutional theory (which is typically used by academics to theorise macro-level changes in institutional structures over time) can also be applied at a more meso level to inform an empirical analysis of how healthcare organisations achieve change and what helps or hinders efforts to sustain those changes. Copyright © 2013 Elsevier Ltd. All rights reserved.

  9. What parents find important in the support of a child with profound intellectual and multiple disabilities.

    Science.gov (United States)

    Jansen, S L G; van der Putten, A A J; Vlaskamp, C

    2013-05-01

    The importance of a partnership between parents and professionals in the support of children with disabilities is widely acknowledged and is one of the key elements of 'family-centred care'. To what extent family-centred principles are also applied to the support of persons with profound intellectual and multiple disabilities (PIMD) is not yet known. The purpose of this exploratory study was to examine what parents with a child with PIMD find important in the support of their child. In addition, we examined which child or parent characteristics influence these parental opinions. In total, 100 parents completed an adapted version of the Measure of Processes of Care. Mean unweighted and weighted scale scores were computed. Non-parametric tests were used to examine differences in ratings due to child (gender, age, type and number of additional disabilities, type of services used and duration of service use) and parent characteristics (gender, involvement with support and educational level). Parents rated situations related to 'Respectful and Supportive Care' and 'Enabling and Partnership' with averages of 7.07 and 6.87 respectively on a scale from 1 to 10. They were generally satisfied with the services provided, expressed in a mean score of 6.88 overall. The age of the child significantly affected the scores for 'Providing Specific Information about the Child'. Parents of children in the '6-12 years' age group gave significantly higher scores on this scale than did parents of children in the '≥17 years' age group (U = 288, r = -0.34). This study shows that parents with children with PIMD find family-centred principles in the professional support of their children important. Although the majority of parents are satisfied with the support provided for their children, a substantial minority of the parents indicated that they did not receive the support they find important. © 2012 Blackwell Publishing Ltd.

  10. Time use of parents raising children with severe or profound intellectual and multiple disabilities.

    Science.gov (United States)

    Luijkx, J; van der Putten, A A J; Vlaskamp, C

    2017-07-01

    Raising children with severe or profound intellectual and multiple disabilities (PIMD) is expected to put extreme pressure on parental time use patterns. The aim of this study was to examine the total time use of mothers and fathers raising children with PIMD and compare it with the time use of parents of typically developing children. Twenty-seven fathers and 30 mothers raising children with PIMD completed a time use diary on a mobile phone or tablet app, as did 66 fathers and 109 mothers of typically developing children. Independent t-tests and Mann-Whitney tests were performed to compare mean time use. There are no differences in the time use of parents of children with PIMD on contracted time (paid work and educational activities) and necessary time (personal care, eating and drinking and sleeping) when compared with parents of typically developing children. There are significant differences between the parents of children with PIMD and the parents of typically developing children in terms of committed time (time for domestic work and the care and supervision of their children) and free time. The mothers of children with PIMD spend significantly less time on domestic work and more time on care and supervision than mothers of typically developing children. This study shows that the parents of children with PIMD have to spend a significant amount of time on care tasks and have on average 1.5 h less free time per day than parents of typically developing children. This is a striking difference, because leisure time can substantially contribute to well-being. Therefore, it is important not only to consider a child with PIMD's support needs but also to identify what parents need to continue their children's daily care and supervision. © 2017 John Wiley & Sons Ltd.

  11. Unique expression of cytoskeletal proteins in human soft palate muscles.

    Science.gov (United States)

    Shah, Farhan; Berggren, Diana; Holmlund, Thorbjörn; Levring Jäghagen, Eva; Stål, Per

    2016-03-01

    The human oropharyngeal muscles have a unique anatomy with diverse and intricate functions. To investigate if this specialization is also reflected in the cytoarchitecture of muscle fibers, intermediate filament proteins and the dystrophin-associated protein complex have been analyzed in two human palate muscles, musculus uvula (UV) and musculus palatopharyngeus (PP), with immunohistochenmical and morphological techniques. Human limb muscles were used as reference. The findings show that the soft palate muscle fibers have a cytoskeletal architecture that differs from the limb muscles. While all limb muscles showed immunoreaction for a panel of antibodies directed against different domains of cytoskeletal proteins desmin and dystrophin, a subpopulation of palate muscle fibers lacked or had a faint immunoreaction for desmin (UV 11.7% and PP 9.8%) and the C-terminal of the dystrophin molecule (UV 4.2% and PP 6.4%). The vast majority of these fibers expressed slow contractile protein myosin heavy chain I. Furthermore, an unusual staining pattern was also observed in these fibers for β-dystroglycan, caveolin-3 and neuronal nitric oxide synthase nNOS, which are all membrane-linking proteins associated with the dystrophin C-terminus. While the immunoreaction for nNOS was generally weak or absent, β-dystroglycan and caveolin-3 showed a stronger immunostaining. The absence or a low expression of cytoskeletal proteins otherwise considered ubiquitous and important for integration and contraction of muscle cells indicate a unique cytoarchitecture designed to meet the intricate demands of the upper airway muscles. It can be concluded that a subgroup of muscle fibers in the human soft palate appears to have special biomechanical properties, and their unique cytoarchitecture must be taken into account while assessing function and pathology in oropharyngeal muscles. © 2015 Anatomical Society.

  12. Atypical Initial Presentation of Painful Muscle Cramps in a Patient with Amyotrophic Lateral Sclerosis: A Case Report and Brief Review of the Literature

    OpenAIRE

    Kuzel, Aaron R; Lodhi, Muhammad Uzair; Syed, Intekhab Askari; Rahim, Mustafa

    2017-01-01

    Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized clinically by progressive muscle weakness that can occur proximally or distally in either the upper or lower extremities. It includes both upper motor neuron signs (spasticity, hyperreflexia, clonus, and Babinski sign) and lower motor neuron signs (atrophy, weakness, and muscle fasciculation). Initial presentation of progressively painful muscle cramps should lead the physician to screen for other signs of amyot...

  13. Enhancing QKD security with weak measurements

    Science.gov (United States)

    Farinholt, Jacob M.; Troupe, James E.

    2016-10-01

    Publisher's Note: This paper, originally published on 10/24/2016, was replaced with a corrected/revised version on 11/8/2016. If you downloaded the original PDF but are unable to access the revision, please contact SPIE Digital Library Customer Service for assistance. In the late 1980s, Aharonov and colleagues developed the notion of a weak measurement of a quantum observable that does not appreciably disturb the system.1, 2 The measurement results are conditioned on both the pre-selected and post-selected state of the quantum system. While any one measurement reveals very little information, by making the same measurement on a large ensemble of identically prepared pre- and post-selected (PPS) states and averaging the results, one may obtain what is known as the weak value of the observable with respect to that PPS ensemble. Recently, weak measurements have been proposed as a method of assessing the security of QKD in the well-known BB84 protocol.3 This weak value augmented QKD protocol (WV-QKD) works by additionally requiring the receiver, Bob, to make a weak measurement of a particular observable prior to his strong measurement. For the subset of measurement results in which Alice and Bob's measurement bases do not agree, the weak measurement results can be used to detect any attempt by an eavesdropper, Eve, to correlate her measurement results with Bob's. Furthermore, the well-known detector blinding attacks, which are known to perfectly correlate Eve's results with Bob's without being caught by conventional BB84 implementations, actually make the eavesdropper more visible in the new WV-QKD protocol. In this paper, we will introduce the WV-QKD protocol and discuss its generalization to the 6-state single qubit protocol. We will discuss the types of weak measurements that are optimal for this protocol, and compare the predicted performance of the 6- and 4-state WV-QKD protocols.

  14. Weak interactions in astrophysics and cosmology

    International Nuclear Information System (INIS)

    Taylor, R.J.

    1977-01-01

    There ar many problems in astrophysics and cosmology in which the form of the weak interactions, their strength or the number of weakly interacting particles, is very important. It is possible that astronomical observations may give some information about the weak interactions. In the conventional hot big bang cosmological theory the number of leptons with associated neutrinos influences the speed of expansion of the Universe and the chemical composition of pre-galactic matter. The strength of the weak interaction, as exemplified by the half-life of the neutron, has a similar effect. In addition, the form of the weak interactions will determine how effectively neutrino viscosity can smooth out irregularities in the early Universe. Because neutrinos have a very long mean free path, they can escape from the central region of stars whereas photons can only escape from the surface. In late stages of stellar evolution, neutrino luminosity is often believed to be much greater than photon luminosity. This can both accelerate the cooling of dying stars and influence the stages of stellar evolution leading to the onset of supernova explosions. In pre-super-novae it is even possible that very dense stellar cores can be opaque to neutrinos and that the absorption or scattering of neutrinos can cause the explosion. These results depend crucially on the form of the weak interactions, with the discovery of neutral currents being very important. Until the solar neutrino experiment has been reconciled with theory, the possible role of uncertainties in the weak interactions cannot be ignored. (author)

  15. Importance of weak minerals on earthquake mechanics

    Science.gov (United States)

    Kaneki, S.; Hirono, T.

    2017-12-01

    The role of weak minerals such as smectite and talc on earthquake mechanics is one of the important issues, and has been debated for recent several decades. Traditionally weak minerals in fault have been reported to weaken fault strength causing from its low frictional resistance. Furthermore, velocity-strengthening behavior of such weak mineral (talc) is considered to responsible for fault creep (aseismic slip) in the San Andreas fault. In contrast, recent studies reported that large amount of weak smectite in the Japan Trench could facilitate gigantic seismic slip during the 2011 Tohoku-oki earthquake. To investigate the role of weak minerals on rupture propagation process and magnitude of slip, we focus on the frictional properties of carbonaceous materials (CMs), which is the representative weak materials widely distributed in and around the convergent boundaries. Field observation and geochemical analyses revealed that graphitized CMs-layer is distributed along the slip surface of a fossil plate-subduction fault. Laboratory friction experiments demonstrated that pure quartz, bulk mixtures with bituminous coal (1 wt.%), and quartz with layered coal samples exhibited almost similar frictional properties (initial, yield, and dynamic friction). However, mixtures of quartz (99 wt.%) and layered graphite (1 wt.%) showed significantly lower initial and yield friction coefficient (0.31 and 0.50, respectively). Furthermore, the stress ratio S, defined as (yield stress-initial stress)/(initial stress-dynamic stress), increased in layered graphite samples (1.97) compared to quartz samples (0.14). Similar trend was observed in smectite-rich fault gouge. By referring the reported results of dynamic rupture propagation simulation using S ratio of 1.4 (typical value for the Japan Trench) and 2.0 (this study), we confirmed that higher S ratio results in smaller slip distance by approximately 20 %. On the basis of these results, we could conclude that weak minerals have lower

  16. SIMULATION OF SUBGRADE EMBANKMENT ON WEAK BASE

    Directory of Open Access Journals (Sweden)

    V. D. Petrenko

    2015-08-01

    Full Text Available Purpose. This article provides: the question of the sustainability of the subgrade on a weak base is considered in the paper. It is proposed to use the method of jet grouting. Investigation of the possibility of a weak base has an effect on the overall deformation of the subgrade; the identification and optimization of the parameters of subgrade based on studies using numerical simulation. Methodology. The theoretical studies of the stress-strain state of the base and subgrade embankment by modeling in the software package LIRA have been conducted to achieve this goal. Findings. After making the necessary calculations perform building fields of a subsidence, borders cramped thickness, bed’s coefficients of Pasternak and Winkler. The diagrams construction of vertical stress performs at any point of load application. Also, using the software system may perform peer review subsidence, rolls railroad tracks in natural and consolidated basis. Originality. For weak soils is the most appropriate nonlinear model of the base with the existing areas of both elastic and limit equilibrium, mixed problem of the theory of elasticity and plasticity. Practical value. By increasing the load on the weak base as a result of the second track construction, adds embankment or increasing axial load when changing the rolling stock process of sedimentation and consolidation may continue again. Therefore, one of the feasible and promising options for the design and reconstruction of embankments on weak bases is to strengthen the bases with the help of jet grouting. With the expansion of the railway infrastructure, increasing speed and weight of the rolling stock is necessary to ensure the stability of the subgrade on weak bases. LIRA software package allows you to perform all the necessary calculations for the selection of a proper way of strengthening weak bases.

  17. Improving Social Skills in Adolescents and Adults with Autism and Severe to Profound Intellectual Disability: A Review of the Literature

    Science.gov (United States)

    Walton, Katherine M.; Ingersoll, Brooke R.

    2013-01-01

    Social skills are important treatment targets for individuals with autism spectrum disorders (ASD) across the lifespan. However, few treatments are available for adolescents and adults with ASD who also have severe to profound intellectual disability (S/PID). Several social skill interventions have been described that may improve social skills in…

  18. The Hands with Eyes and Nose in the Palm: As Effective Communication Alternatives for Profoundly Deaf People in Zimbabwe

    Science.gov (United States)

    Mutswanga, Phillipa

    2017-01-01

    Drawing from the experiences and testimonies of people with profound deafness, the study qualitatively explored the use of the hands with eyes and nose in the palm as communication alternatives in the field of deafness. The study was prompted by the 27 year old lady, Leah Katz-Hernandez who is deaf who got engaged in March 2015 as the 2016…

  19. How to Facilitate Transition to Adulthood? Innovative Solutions from Parents of Young Adults with Profound Intellectual Disability

    Science.gov (United States)

    Gauthier-Boudreault, Camille; Couture, Mélanie; Gallagher, Frances

    2018-01-01

    Background: At age 21, access to specialised services for youth with profound intellectual disability is reduced. Few studies have focused on parents' views concerning potential solutions to ease the transition to adulthood, and most existing solutions target young adults with less severe intellectual disability. The aim of this study is to…

  20. Reliability of assessing the sensory perception of children with profound intellectual and multiple disabilities : a case study

    NARCIS (Netherlands)

    Vlaskamp, C.; Cuppen-Fonteine, H.

    Background This study describes preliminary stages of developing a checklist to enable practitioners to determine the behavioural responses of children with profound intellectual and multiple disabilities to sensory stimuli. Reliability of currently used checklists is low, with a focus on the

  1. Motor activation in people with profound intellectual and multiple disabilities: research in daily practice in residential facilities

    NARCIS (Netherlands)

    Bossink, Leontien; van der Putten, Annette; Vlaskamp, Carla

    2014-01-01

    Aim: The total study aims at generating knowledge about the best way to motor activate persons with profound intellectual and multiple disabilities (PIMD) in residential facilities. The purpose of the current poster presentation is to present the results of the first step executed in this project

  2. Parental Adaptation to Out-of-Home Placement of a Child with Severe or Profound Developmental Disabilities

    Science.gov (United States)

    Jackson, Jeffrey B.; Roper, Susanne Olsen

    2014-01-01

    Utilizing grounded theory qualitative research methods, a model was developed for describing parental adaptation after voluntary placement of a child with severe or profound developmental disabilities in out-of-home care. Interviews of parents from 20 families were analyzed. Parents' cognitive appraisals of placement outcomes were classified…

  3. The documentation of health problems in relation to prescribed medication in people with profound intellectual and multiple disabilities

    NARCIS (Netherlands)

    van der Heide, D. C.; van der Putten, A. A. J.; van den Berg, P. B.; Taxis, K.; Vlaskamp, C.

    Persons with profound intellectual and multiple disabilities (PIMD) suffer from a wide range of health problems and use a wide range of different drugs. This study investigated for frequently used medication whether there was a health problem documented in the medical notes for the drug prescribed.

  4. You Should be Dancin:! The Role of Performing Arts in the Lives of the Severely and Profoundly Handicapped.

    Science.gov (United States)

    Barringer, Mary Dean

    The manual presents a program planning framework and teaching units for teaching dance and movement to severely and profoundly handicapped individuals. The planning framework contains four components: (1) aesthetic perception/multisensory integration; (2) creative expression; (3) dance heritage/historical and cultural; and (4) aesthetic…

  5. Domains of quality of life of people with profound multiple disabilities : The perspective of parents and direct support staff

    NARCIS (Netherlands)

    Petry, K; Maes, B; Vlaskamp, C

    Background This study considered the general validity of the basic domains of quality of life that appear in theoretical models, in relation to people with profound multiple disabilities. The authors examined how parents and direct support staff operationalized these basic domains for people with

  6. Camera-based microswitch technology to monitor mouth, eyebrow, and eyelid responses of children with profound multiple disabilities

    NARCIS (Netherlands)

    Lancioni, G.E.; Bellini, D.; Oliva, D.; Singh, N.N.; O'Reilly, M.F.; Sigafoos, J.; Lang, R.B.; Didden, H.C.M.

    2011-01-01

    A camera-based microswitch technology was recently used to successfully monitor small eyelid and mouth responses of two adults with profound multiple disabilities (Lancioni et al., Res Dev Disab 31:1509-1514, 2010a). This technology, in contrast with the traditional optic microswitches used for

  7. The Effect of Frequency Transposition on Speech Perception in Adolescents and Young Adults with Profound Hearing Loss

    Science.gov (United States)

    Gou, J.; Smith, J.; Valero, J.; Rubio, I.

    2011-01-01

    This paper reports on a clinical trial evaluating outcomes of a frequency-lowering technique for adolescents and young adults with severe to profound hearing impairment. Outcomes were defined by changes in aided thresholds, speech perception, and acceptance. The participants comprised seven young people aged between 13 and 25 years. They were…

  8. Informal Social Networks of People with Profound Intellectual and Multiple Disabilities: Relationship with Age, Communicative Abilities and Current Living Arrangements

    Science.gov (United States)

    Kamstra, A.; van der Putten, A. A. J.; Post, W. J.; Vlaskamp, C.

    2015-01-01

    Background: People with profound intellectual and multiple disabilities (PIMD) have limited informal social contacts. Research to determine the factors which can positively influence establishing sound informal social contacts is required. Materials and Methods: Regression analysis for 200 people with PIMD was used to analyse how age,…

  9. Family matters : The experiences and opinions of family members of persons with (severe) or profound intellectual disabilities

    NARCIS (Netherlands)

    Luijkx, Jorien

    2016-01-01

    “I love my sister, but sometimes I don’t”. This is one of the statements made in the study focused on the experiences of family members of people with (profound) intellectual (and multiple) disabilities (both of individuals living in a residential facility as persons living at home). In recent

  10. An Evaluation of Social and Adaptive Skills in Adults with Bipolar Disorder and Severe/Profound Intellectual Disability

    Science.gov (United States)

    Matson, Johnny L.; Terlonge, Cindy; Gonzalez, Melissa L.; Rivet, Tessa

    2006-01-01

    The purpose of this study was to explore the interrelationship of social and adaptive skills in adults with bipolar disorder and severe or profound intellectual disability. A bipolar group (N=14), a severe psychopathology group without bipolar disorder (N=14), and a control group with no DSM-IV Axis I diagnosis (N=14) were compared on the…

  11. Therapeutic interventions in the Netherlands and Belgium in support of people with profound intellectual and multiple disabilities

    NARCIS (Netherlands)

    Vlaskamp, Carla; Nakken, Han

    For several reasons, people with profound and multiple disabilities may be offered a variety of therapeutic interventions. Thus far, researchers have shown a limited interest in providing an empirical base for these interventions. Research is needed on the theoretical rationale (if any), the

  12. The Role of the External Personal Assistants for Children with Profound Intellectual and Multiple Disabilities Working in the Children's Home

    Science.gov (United States)

    Axelsson, Anna Karin

    2015-01-01

    Background: Children with profound intellectual and multiple disabilities need support to function in an optimal way. However, there is a limited knowledge about the role of external personal assistants working in the children's home. Materials and Methods: A mixed method study was performed including qualitative data from interviews with 11…

  13. Catch the wave! Time-window sequential analysis of alertness stimulation in individuals with profound intellectual and multiple disabilities

    NARCIS (Netherlands)

    Munde, V. S.; Vlaskamp, C.; Maes, B.; Ruijssenaars, A. J. J. M.

    BackgroundWhile optimally activities are provided at those moments when the individual with profound intellectual and multiple disabilities (PIMD) is focused on the environment' or alert', detailed information about the impact that the design and timing of the activity has on alertness is lacking.

  14. Noonan Syndrome: An Underestimated Cause of Severe to Profound Sensorineural Hearing Impairment. Which Clues to Suspect the Diagnosis?

    Science.gov (United States)

    Ziegler, Alban; Loundon, Natalie; Jonard, Laurence; Cavé, Hélène; Baujat, Geneviève; Gherbi, Souad; Couloigner, Vincent; Marlin, Sandrine

    2017-09-01

    To highlight Noonan syndrome as a clinically recognizable cause of severe to profound sensorineural hearing impairment. New clinical cases and review. Patients evaluated for etiological diagnosis by a medical geneticist in a reference center for hearing impairment. Five patients presenting with confirmed Noonan syndrome and profound sensorineural hearing impairment. Diagnostic and review of the literature. Five patients presented with profound sensorineural hearing impairment and molecularly confirmed Noonan syndrome. Sensorineural hearing impairment has been progressive for three patients. Cardiac echography identified pulmonary stenosis in two patients and was normal for the three other patients. Short stature was found in two patients. Mild intellectual disability was found in one patient. Inconspicuous clinical features as facial dysmorphism, cryptorchidism, or easy bruising were of peculiar interest to reach the diagnosis of Noonan syndrome. Profound sensorineural hearing impairment can be the main feature of Noonan syndrome. Associated features are highly variable; thus, detailed medical history and careful physical examination are mandatory to consider the diagnosis in case of a sensorineural hearing impairment.

  15. Quality-Enhancing Interventions for People with Profound Intellectual and Multiple Disabilities: A Review of the Empirical Research Literature

    Science.gov (United States)

    Maes, Bea; Lambrechts, Greet; Hostyn, Ine; Petry, Katja

    2007-01-01

    Background: This study provides an overview of empirical research on the effectiveness of quality-enhancing interventions for people with profound intellectual and multiple disabilities (PIMD). Method: Through computerised searches of the PsycINFO and ERIC databases, and using several search criteria specifically relating to the target group and…

  16. Staff attributions of the causes of challenging behaviour in children and adults with profound intellectual and multiple disabilities

    NARCIS (Netherlands)

    Poppes, P.; van der Putten, A.A.J.; ten Brug, A.; Vlaskamp, C.

    A study has shown that staff do not generally perceive challenging behaviour in people with profound intellectual and multiple disabilities (PIMD) as being of serious consequence. In this study we aimed to gain a better understanding of the causal explanations that direct care and support staff give

  17. The Problem of Weak Governments and Weak Societies in Eastern Europe

    Directory of Open Access Journals (Sweden)

    Marko Grdešić

    2008-01-01

    Full Text Available This paper argues that, for Eastern Europe, the simultaneous presence of weak governments and weak societies is a crucial obstacle which must be faced by analysts and reformers. The understanding of other normatively significant processes will be deficient without a consciousness-raising deliberation on this problem and its implications. This paper seeks to articulate the “relational” approach to state and society. In addition, the paper lays out a typology of possible patterns of relationship between state and society, dependent on whether the state is weak or strong and whether society is weak or strong. Comparative data are presented in order to provide an empirical support for the theses. Finally, the paper outlines two reform approaches which could enable breaking the vicious circle emerging in the context of weak governments and weak societies.

  18. Geometric phase topology in weak measurement

    Science.gov (United States)

    Samlan, C. T.; Viswanathan, Nirmal K.

    2017-12-01

    The geometric phase visualization proposed by Bhandari (R Bhandari 1997 Phys. Rep. 281 1-64) in the ellipticity-ellipse orientation basis of the polarization ellipse of light is implemented to understand the geometric aspects of weak measurement. The weak interaction of a pre-selected state, acheived via spin-Hall effect of light (SHEL), results in a spread in the polarization ellipticity (η) or ellipse orientation (χ) depending on the resulting spatial or angular shift, respectively. The post-selection leads to the projection of the η spread in the complementary χ basis results in the appearance of a geometric phase with helical phase topology in the η - χ parameter space. By representing the weak measurement on the Poincaré sphere and using Jones calculus, the complex weak value and the geometric phase topology are obtained. This deeper understanding of the weak measurement process enabled us to explore the techniques’ capabilities maximally, as demonstrated via SHEL in two examples—external reflection at glass-air interface and transmission through a tilted half-wave plate.

  19. Weak KAM theory for a weakly coupled system of Hamilton–Jacobi equations

    KAUST Repository

    Figalli, Alessio; Gomes, Diogo A.; Marcon, Diego

    2016-01-01

    Here, we extend the weak KAM and Aubry–Mather theories to optimal switching problems. We consider three issues: the analysis of the calculus of variations problem, the study of a generalized weak KAM theorem for solutions of weakly coupled systems of Hamilton–Jacobi equations, and the long-time behavior of time-dependent systems. We prove the existence and regularity of action minimizers, obtain necessary conditions for minimality, extend Fathi’s weak KAM theorem, and describe the asymptotic limit of the generalized Lax–Oleinik semigroup. © 2016, Springer-Verlag Berlin Heidelberg.

  20. Weak KAM theory for a weakly coupled system of Hamilton–Jacobi equations

    KAUST Repository

    Figalli, Alessio

    2016-06-23

    Here, we extend the weak KAM and Aubry–Mather theories to optimal switching problems. We consider three issues: the analysis of the calculus of variations problem, the study of a generalized weak KAM theorem for solutions of weakly coupled systems of Hamilton–Jacobi equations, and the long-time behavior of time-dependent systems. We prove the existence and regularity of action minimizers, obtain necessary conditions for minimality, extend Fathi’s weak KAM theorem, and describe the asymptotic limit of the generalized Lax–Oleinik semigroup. © 2016, Springer-Verlag Berlin Heidelberg.

  1. Proteomics of Skeletal Muscle

    DEFF Research Database (Denmark)

    Deshmukh, Atul

    2016-01-01

    , of altered protein expressions profiles and/or their posttranslational modifications (PTMs). Mass spectrometry (MS)-based proteomics offer enormous promise for investigating the molecular mechanisms underlying skeletal muscle insulin resistance and exercise-induced adaptation; however, skeletal muscle......Skeletal muscle is the largest tissue in the human body and plays an important role in locomotion and whole body metabolism. It accounts for ~80% of insulin stimulated glucose disposal. Skeletal muscle insulin resistance, a primary feature of Type 2 diabetes, is caused by a decreased ability...... of muscle to respond to circulating insulin. Physical exercise improves insulin sensitivity and whole body metabolism and remains one of the most promising interventions for the prevention of Type 2 diabetes. Insulin resistance and exercise adaptations in skeletal muscle might be a cause, or consequence...

  2. Muscles, exercise and obesity

    DEFF Research Database (Denmark)

    Pedersen, Bente K; Febbraio, Mark A

    2012-01-01

    During the past decade, skeletal muscle has been identified as a secretory organ. Accordingly, we have suggested that cytokines and other peptides that are produced, expressed and released by muscle fibres and exert either autocrine, paracrine or endocrine effects should be classified as myokines....... The finding that the muscle secretome consists of several hundred secreted peptides provides a conceptual basis and a whole new paradigm for understanding how muscles communicate with other organs, such as adipose tissue, liver, pancreas, bones and brain. However, some myokines exert their effects within...... the muscle itself. Thus, myostatin, LIF, IL-6 and IL-7 are involved in muscle hypertrophy and myogenesis, whereas BDNF and IL-6 are involved in AMPK-mediated fat oxidation. IL-6 also appears to have systemic effects on the liver, adipose tissue and the immune system, and mediates crosstalk between intestinal...

  3. The muscle CT of thigh in chronic Werdnig-Hoffmann disease

    International Nuclear Information System (INIS)

    Horikawa, Hirosei; Konagaya, Masaaki; Takayanagi, Tetsuya; Otsuji, Hideaki

    1986-01-01

    In this paper, the muscle CT of thigh in chronic Werdnig-Hoffmann disease (chronic WH) was evaluated. The subjects were five cases of chronic WH (3 males and 2 females, ages ranging from 6 to 22 years) and four control males. All cases showed symmetrical muscular weakness. The proximal muscle were more affected than the distal in the upper limbs. But the muscle strength of hip adduction was relatively spared as compared with other strength of lower limbs. The CT scan was carried out at the upper quarter level between lesser trochanter and medial condyle of the femur. The muscle CT of cases aged 6 and 7 years showed the severely decreased cross-sectional area of muscle without significant decrease in density. The atrophic muscles were surrounded by a large amount of low density area. The hamstring muscles and the adductor muscles, especially adductor longus muscle (ALM), were less affected than the quadriceps femoris muscles. Spotty and moth-eaten low density areas were observed dominantly in the severely affected muscles. In the advanced cases, only ALM could be identified on the CT image. The other muscles were unable to be identified because of severe atrophy with extremely low density. These CT findings suggest the process of muscular wastings of chronic WH as follows; at first muscle fibers are atrophied due to denervation and sooner or later replaced with fat tissue. Moreover, the preservation of ALM suggests that loss of anterior horn cells does not always go on homogeneously. (author)

  4. IL-6 signaling blockade increases inflammation but does not affect muscle function in the mdx mouse

    Directory of Open Access Journals (Sweden)

    Kostek Matthew C

    2012-06-01

    Full Text Available Abstract Background IL-6 is a pleiotropic cytokine that modulates inflammatory responses and plays critical roles in muscle maintenance and remodeling. In the mouse model (mdx of Duchenne Muscular Dystrophy, IL-6 and muscle inflammation are elevated, which is believed to contribute to the chronic inflammation and failure of muscle regeneration in DMD. The purpose of the current study was to examine the effect of blocking IL-6 signaling on the muscle phenotype including muscle weakness and pathology in the mdx mouse. Methods A monoclonal antibody against the IL-6 receptor (IL-6r mAb that blocks local and systemic IL-6 signaling was administered to mdx and BL-10 mice for 5 weeks and muscle function, histology, and inflammation were examined. Results IL-6r mAb treatment increased mdx muscle inflammation including total inflammation score and ICAM-1 positive lumens in muscles. There was no significant improvement in muscle strength nor muscle pathology due to IL-6r mAb treatment in mdx mice. Conclusions These results showed that instead of reducing inflammation, IL-6 signaling blockade for 5 weeks caused an increase in muscle inflammation, with no significant change in indices related to muscle regeneration and muscle function. The results suggest a potential anti-inflammatory instead of the original hypothesized pro-inflammatory role of IL-6 signaling in the mdx mice.

  5. Weakly supervised classification in high energy physics

    International Nuclear Information System (INIS)

    Dery, Lucio Mwinmaarong; Nachman, Benjamin; Rubbo, Francesco; Schwartzman, Ariel

    2017-01-01

    As machine learning algorithms become increasingly sophisticated to exploit subtle features of the data, they often become more dependent on simulations. This paper presents a new approach called weakly supervised classification in which class proportions are the only input into the machine learning algorithm. Using one of the most challenging binary classification tasks in high energy physics — quark versus gluon tagging — we show that weakly supervised classification can match the performance of fully supervised algorithms. Furthermore, by design, the new algorithm is insensitive to any mis-modeling of discriminating features in the data by the simulation. Weakly supervised classification is a general procedure that can be applied to a wide variety of learning problems to boost performance and robustness when detailed simulations are not reliable or not available.

  6. Weakly supervised classification in high energy physics

    Energy Technology Data Exchange (ETDEWEB)

    Dery, Lucio Mwinmaarong [Physics Department, Stanford University,Stanford, CA, 94305 (United States); Nachman, Benjamin [Physics Division, Lawrence Berkeley National Laboratory,1 Cyclotron Rd, Berkeley, CA, 94720 (United States); Rubbo, Francesco; Schwartzman, Ariel [SLAC National Accelerator Laboratory, Stanford University,2575 Sand Hill Rd, Menlo Park, CA, 94025 (United States)

    2017-05-29

    As machine learning algorithms become increasingly sophisticated to exploit subtle features of the data, they often become more dependent on simulations. This paper presents a new approach called weakly supervised classification in which class proportions are the only input into the machine learning algorithm. Using one of the most challenging binary classification tasks in high energy physics — quark versus gluon tagging — we show that weakly supervised classification can match the performance of fully supervised algorithms. Furthermore, by design, the new algorithm is insensitive to any mis-modeling of discriminating features in the data by the simulation. Weakly supervised classification is a general procedure that can be applied to a wide variety of learning problems to boost performance and robustness when detailed simulations are not reliable or not available.

  7. Efficient quantum computing with weak measurements

    International Nuclear Information System (INIS)

    Lund, A P

    2011-01-01

    Projective measurements with high quantum efficiency are often assumed to be required for efficient circuit-based quantum computing. We argue that this is not the case and show that the fact that they are not required was actually known previously but was not deeply explored. We examine this issue by giving an example of how to perform the quantum-ordering-finding algorithm efficiently using non-local weak measurements considering that the measurements used are of bounded weakness and some fixed but arbitrary probability of success less than unity is required. We also show that it is possible to perform the same computation with only local weak measurements, but this must necessarily introduce an exponential overhead.

  8. Weak layer fracture: facets and depth hoar

    Directory of Open Access Journals (Sweden)

    I. Reiweger

    2013-09-01

    Full Text Available Understanding failure initiation within weak snow layers is essential for modeling and predicting dry-snow slab avalanches. We therefore performed laboratory experiments with snow samples containing a weak layer consisting of either faceted crystals or depth hoar. During these experiments the samples were loaded with different loading rates and at various tilt angles until fracture. The strength of the samples decreased with increasing loading rate and increasing tilt angle. Additionally, we took pictures of the side of four samples with a high-speed video camera and calculated the displacement using a particle image velocimetry (PIV algorithm. The fracture process within the weak layer could thus be observed in detail. Catastrophic failure started due to a shear fracture just above the interface between the depth hoar layer and the underlying crust.

  9. Weak lensing in the Dark Energy Survey

    Science.gov (United States)

    Troxel, Michael

    2016-03-01

    I will present the current status of weak lensing results from the Dark Energy Survey (DES). DES will survey 5000 square degrees in five photometric bands (grizY), and has already provided a competitive weak lensing catalog from Science Verification data covering just 3% of the final survey footprint. I will summarize the status of shear catalog production using observations from the first year of the survey and discuss recent weak lensing science results from DES. Finally, I will report on the outlook for future cosmological analyses in DES including the two-point cosmic shear correlation function and discuss challenges that DES and future surveys will face in achieving a control of systematics that allows us to take full advantage of the available statistical power of our shear catalogs.

  10. Weak self-adjoint differential equations

    International Nuclear Information System (INIS)

    Gandarias, M L

    2011-01-01

    The concepts of self-adjoint and quasi self-adjoint equations were introduced by Ibragimov (2006 J. Math. Anal. Appl. 318 742-57; 2007 Arch. ALGA 4 55-60). In Ibragimov (2007 J. Math. Anal. Appl. 333 311-28), a general theorem on conservation laws was proved. In this paper, we generalize the concept of self-adjoint and quasi self-adjoint equations by introducing the definition of weak self-adjoint equations. We find a class of weak self-adjoint quasi-linear parabolic equations. The property of a differential equation to be weak self-adjoint is important for constructing conservation laws associated with symmetries of the differential equation. (fast track communication)

  11. On weakly D-differentiable operators

    DEFF Research Database (Denmark)

    Christensen, Erik

    2016-01-01

    Let DD be a self-adjoint operator on a Hilbert space HH and aa a bounded operator on HH. We say that aa is weakly DD-differentiable, if for any pair of vectors ξ,ηξ,η from HH the function 〈eitDae−itDξ,η〉〈eitDae−itDξ,η〉 is differentiable. We give an elementary example of a bounded operator aa......, such that aa is weakly DD-differentiable, but the function eitDae−itDeitDae−itD is not uniformly differentiable. We show that weak  DD-differentiability   may be characterized by several other properties, some of which are related to the commutator (Da−aD)...

  12. Strong effects in weak nonleptonic decays

    International Nuclear Information System (INIS)

    Wise, M.B.

    1980-04-01

    In this report the weak nonleptonic decays of kaons and hyperons are examined with the hope of gaining insight into a recently proposed mechanism for the ΔI = 1/2 rule. The effective Hamiltonian for ΔS = 1 weak nonleptonic decays and that for K 0 -anti K 0 mixing are calculated in the six-quark model using the leading logarithmic approximation. These are used to examine the CP violation parameters of the kaon system. It is found that if Penguin-type diagrams make important contributions to K → ππ decay amplitudes then upcoming experiments may be able to distinguish the six-quark model for CP violation from the superweak model. The weak radiative decays of hyperons are discussed with an emphasis on what they can teach us about hyperon nonleptonic decays and the ΔI = 1/2 rule

  13. Postural control in children with typical development and children with profound hearing loss

    Directory of Open Access Journals (Sweden)

    Monteiro de Sousa AM

    2012-05-01

    Full Text Available Aneliza Maria Monteiro de Sousa,1 Jônatas de França Barros,2 Brígido Martins de Sousa Neto31Faculty of Health Sciences, University of Brasilia, Brasilia, Federal District, Brazil; 2Department of Physical Education at the Federal University of Rio Grande do Norte, Natal, Rio Grande do Norte, Brazil; 3University Center UNIEURO, Brasilia, Federal District, BrazilPurpose: To describe the behavior of the postural control in children with profound sensorineural hearing loss and compare the results of experimental tests with hearing children aged 7 to 10 years.Patients and methods: This is a cross-sectional study where 100 children were divided into experimental and control groups. We used a force platform, AccuSway Plus, where the tests were conducted under the experimental conditions: open base, eyes open (OBEO; open base, eyes closed (OBEC; closed base, eyes open (CBEO; closed base, eyes closed (CBEC. The body sway velocity (V of the center of pressure, the displacement in the anteroposterior direction (COPap and mediolateral (COPml of the center of pressure were the parameters to evaluate the postural control. For statistical analysis we used the nonparametric Mann–Whitney U test, with a significance level of 5%.Results: In comparisons of variables between the groups, the experimental group outperformed by at least 75% of the control group values. In terms of global trends, the experimental group shows higher values of body oscillations in all experimental conditions and variables evaluated. Children with hearing loss had poorer balance performance compared to the group of hearing. The inferential analysis revealed a statistically significant difference in the balance between deaf and hearing children in the OBEC experimental condition in relation to the COPml parameter (P = 0.04. There were no statistically significant differences in comparisons between the sexes when the groups were analyzed separately. The prevalence of unknown etiology

  14. Binaural auditory outcomes in patients with postlingual profound unilateral hearing loss: 3 years after cochlear implantation.

    Science.gov (United States)

    Mertens, Griet; Kleine Punte, Andrea; De Bodt, Marc; Van de Heyning, Paul

    2015-01-01

    The value of cochlear implants (CI) in patients with profound unilateral hearing loss (UHL) and tinnitus has recently been investigated. The authors previously demonstrated the feasibility of CI in a 12- month outcome study in a prospective UHL cohort. The aim of this study was to investigate the binaural auditory outcomes in this cohort 36 months after CI surgery. The 36-month outcome was evaluated in 22 CI users with postlingual UHL and severe tinnitus. Twelve subjects had contralateral normal hearing (single-sided deafness - SSD group) and 10 subjects had a contralateral, mild to moderate hearing loss and used a hearing aid (asymmetric hearing loss - AHL group). Speech perception in noise was assessed in two listening conditions: the CIoff and the CIon condition. The binaural summation effect (S0N0), binaural squelch effect (S0NCI) and the combined head shadow effect (SCIN0) were investigated. Subjective benefit in daily life was assessed by means of the Speech, Spatial and Qualities of Hearing Scale (SSQ). At 36 months, a significant binaural summation effect was observed for the study cohort (2.00, SD 3.82 dB; p binaural effect was not significant 12 months after CI surgery. A binaural squelch effect was significant for the AHL subgroup at 12 months (2.00, SD 4.38 dB; p < 0.05). A significant combined head shadow and squelch effect was also noted in the spatial configuration SCIN0 for the study cohort (4.00, SD 5.89 dB; p < 0.01) and for the AHL subgroup (5.67, SD 6.66 dB; p < 0.05). The SSQ data show that the perceived benefit in daily life after CI surgery remains stable up to 36 months at CIon. CI can significantly improve speech perception in noise in patients with UHL. The positive effects of CIon speech perception in noise increase over time up to 36 months after CI surgery. Improved subjective benefit in daily life was also shown to be sustained in these patients. © 2015 S. Karger AG, Basel.

  15. Experience with cochlear implants in Greenlanders with profound hearing loss living in Greenland

    Directory of Open Access Journals (Sweden)

    Preben Homøe

    2013-08-01

    Full Text Available Objective. Cochlear implant (CI treatment was introduced to the world in the 1980s and has become a routine treatment for congenital or acquired severe-to-profound hearing loss. CI treatment requires access to a highly skilled team of ear, nose and throat specialists, audiologists and speech-language pathologists for evaluation, surgery and rehabilitation. In particular, children treated with CI are in need of long-term post-operative auditory training and other follow-up support. Design. The study is retrospective with updated information on present performance. Results. Since 2001, a total of 11 Greenlandic patients living in Greenland have been treated with CI, 7 children and 4 adults. Of these children, 4 use oral communication only and are full-time CI-users, 2 with full-time use of CI are still in progress with use of oral communication, and 1 has not acquired oral language yet, but has started auditory and speech training. Six children attend mainstream public school while one child is in kindergarten. Of the adults, only 1 has achieved good speech perception with full-time use of CI while 3 do not use the CI. Discussion. From an epidemiological point of view, approximately 1–3 children below 6 years are in need of a CI every second year in Greenland often due to sequelae from meningitis, which may cause postinfectious deafness. Screening of new-borns for hearing has been started in Greenland establishing the basis for early diagnosis of congenital hearing impairment and subsequent intervention. The logistics and lack of availability of speech therapists in Greenland hampers possibilities for optimal language and speech therapy of CI patients in Greenland. This study aims at describing the results of CI treatment in Greenlanders and the outcome of the CI operations along with the auditory and speech/language outcomes. Finally, we present a suggestion for the future CI treatment and recommendations for an increased effort in the

  16. Muscle Progenitor Cell Regenerative Capacity in the Torn Rotator Cuff

    Science.gov (United States)

    Meyer, Gretchen A.; Farris, Ashley L.; Sato, Eugene; Gibbons, Michael; Lane, John G.; Ward, Samuel R.; Engler, Adam J.

    2014-01-01

    Chronic rotator cuff (RC) tears affect a large portion of the population and result in substantial upper extremity impairment, shoulder weakness, pain and limited range of motion. Regardless of surgical or conservative treatment, persistent atrophic muscle changes limit functional restoration and may contribute to surgical failure. We hypothesized that deficits in the skeletal muscle progenitor (SMP) cell pool could contribute to poor muscle recovery following tendon repair. Biopsies were obtained from patients undergoing arthroscopic RC surgery. The SMP population was quantified, isolated and assayed in culture for its ability to proliferate and fuse in-vitro and in-vivo. The SMP population was larger in muscles from cuffs with partial tears compared with no tears or full thickness tears. However, SMPs from muscles in the partial tear group also exhibited reduced proliferative ability. Cells from all cuff states were able to fuse robustly in culture and engraft when injected into injured mouse muscle, suggesting that when given the correct signals, SMPs are capable of contributing to muscle hypertrophy and regeneration regardless of tear severity. The fact that this does not appear to happen in-vivo helps focus future therapeutic targets for promoting muscle recovery following rotator cuff repairs and may help improve clinical outcomes. PMID:25410765

  17. Precision cosmology with weak gravitational lensing

    Science.gov (United States)

    Hearin, Andrew P.

    In recent years, cosmological science has developed a highly predictive model for the universe on large scales that is in quantitative agreement with a wide range of astronomical observations. While the number and diversity of successes of this model provide great confidence that our general picture of cosmology is correct, numerous puzzles remain. In this dissertation, I analyze the potential of planned and near future galaxy surveys to provide new understanding of several unanswered questions in cosmology, and address some of the leading challenges to this observational program. In particular, I study an emerging technique called cosmic shear, the weak gravitational lensing produced by large scale structure. I focus on developing strategies to optimally use the cosmic shear signal observed in galaxy imaging surveys to uncover the physics of dark energy and the early universe. In chapter 1 I give an overview of a few unsolved mysteries in cosmology and I motivate weak lensing as a cosmological probe. I discuss the use of weak lensing as a test of general relativity in chapter 2 and assess the threat to such tests presented by our uncertainty in the physics of galaxy formation. Interpreting the cosmic shear signal requires knowledge of the redshift distribution of the lensed galaxies. This redshift distribution will be significantly uncertain since it must be determined photometrically. In chapter 3 I investigate the influence of photometric redshift errors on our ability to constrain dark energy models with weak lensing. The ability to study dark energy with cosmic shear is also limited by the imprecision in our understanding of the physics of gravitational collapse. In chapter 4 I present the stringent calibration requirements on this source of uncertainty. I study the potential of weak lensing to resolve a debate over a long-standing anomaly in CMB measurements in chapter 5. Finally, in chapter 6 I summarize my findings and conclude with a brief discussion of my

  18. Extrapolating Weak Selection in Evolutionary Games

    Science.gov (United States)

    Wu, Bin; García, Julián; Hauert, Christoph; Traulsen, Arne

    2013-01-01

    In evolutionary games, reproductive success is determined by payoffs. Weak selection means that even large differences in game outcomes translate into small fitness differences. Many results have been derived using weak selection approximations, in which perturbation analysis facilitates the derivation of analytical results. Here, we ask whether results derived under weak selection are also qualitatively valid for intermediate and strong selection. By “qualitatively valid” we mean that the ranking of strategies induced by an evolutionary process does not change when the intensity of selection increases. For two-strategy games, we show that the ranking obtained under weak selection cannot be carried over to higher selection intensity if the number of players exceeds two. For games with three (or more) strategies, previous examples for multiplayer games have shown that the ranking of strategies can change with the intensity of selection. In particular, rank changes imply that the most abundant strategy at one intensity of selection can become the least abundant for another. We show that this applies already to pairwise interactions for a broad class of evolutionary processes. Even when both weak and strong selection limits lead to consistent predictions, rank changes can occur for intermediate intensities of selection. To analyze how common such games are, we show numerically that for randomly drawn two-player games with three or more strategies, rank changes frequently occur and their likelihood increases rapidly with the number of strategies . In particular, rank changes are almost certain for , which jeopardizes the predictive power of results derived for weak selection. PMID:24339769

  19. Weak Acid Ionization Constants and the Determination of Weak Acid-Weak Base Reaction Equilibrium Constants in the General Chemistry Laboratory

    Science.gov (United States)

    Nyasulu, Frazier; McMills, Lauren; Barlag, Rebecca

    2013-01-01

    A laboratory to determine the equilibrium constants of weak acid negative weak base reactions is described. The equilibrium constants of component reactions when multiplied together equal the numerical value of the equilibrium constant of the summative reaction. The component reactions are weak acid ionization reactions, weak base hydrolysis…

  20. Accessory piriformis muscle

    Directory of Open Access Journals (Sweden)

    Sedat Develi

    2017-03-01

    Full Text Available Piriformis muscle originates from facies pelvica of sacrum and inserts on the trochanter major. It is one of the lateral rotator muscles of the hip and a landmark point in the gluteal region since n. ischiadicus descends to the thigh by passing close to the muscle. This contiguity may be associated with the irritation of the nerve which is known as piriformis syndrome. A rare anatomic variation of the muscle which observed on 74 years old male cadaver is discussed in this case report. [Cukurova Med J 2017; 42(1.000: 182-183

  1. Weak hadronic currents in compensation theory

    International Nuclear Information System (INIS)

    Pappas, R.C.

    1975-01-01

    Working within the framework of a compensation theory of strong and weak interactions, it is shown that: (1) an axial vector baryon number current can be included in the weak current algebra if certain restrictions on the K-meson strong couplings are relaxed; (2) the theory does not permit the introduction of strange currents of the chiral form V + A; and (3) the assumption that the superweak currents of the theory cannot contain certain CP conserving terms can be justified on the basis of compensation requirements

  2. Weak interactions of the b quark

    International Nuclear Information System (INIS)

    Branco, G.C.; Mohapatra, R.N.

    1978-01-01

    In weak-interaction models with two charged W bosons of comparable mass, there exists a novel possibility for the weak interactions of the b quark, in which the (u-barb)/sub R/ current occurs with maximal strength. It is noted that multimuon production in e + e - annihilation at above Q 2 > or approx. = (12 GeV) 2 will distinguish this scheme from the conventional one. We also present a Higgs system that leads naturally to this type of coupling, in a class of gauge models

  3. Fast measure proceeding of weak currents

    International Nuclear Information System (INIS)

    Taieb, J.

    1953-01-01

    The process of fast measure of the weak currents that we are going to describe briefly apply worthy of the provided currents by the sources to elevated value internal resistance, as it is the case for the ionization chamber, the photocells, mass spectroscopic tubes. The problem to measure weak currents is essentially a problem of amplifier and of input circuit. We intended to achieve a whole amplifier and input circuit with advanced performances, meaning that for a measured celerity we wanted to have an signal/noise ratio the most important as in the classic systems and for a same report signal/noise a more quickly done measure. (M.B.) [fr

  4. A Continuation Method for Weakly Kannan Maps

    Directory of Open Access Journals (Sweden)

    Ariza-Ruiz David

    2010-01-01

    Full Text Available The first continuation method for contractive maps in the setting of a metric space was given by Granas. Later, Frigon extended Granas theorem to the class of weakly contractive maps, and recently Agarwal and O'Regan have given the corresponding result for a certain type of quasicontractions which includes maps of Kannan type. In this note we introduce the concept of weakly Kannan maps and give a fixed point theorem, and then a continuation method, for this class of maps.

  5. From Suitable Weak Solutions to Entropy Viscosity

    KAUST Repository

    Guermond, Jean-Luc

    2010-12-16

    This paper focuses on the notion of suitable weak solutions for the three-dimensional incompressible Navier-Stokes equations and discusses the relevance of this notion to Computational Fluid Dynamics. The purpose of the paper is twofold (i) to recall basic mathematical properties of the three-dimensional incompressible Navier-Stokes equations and to show how they might relate to LES (ii) to introduce an entropy viscosity technique based on the notion of suitable weak solution and to illustrate numerically this concept. © 2010 Springer Science+Business Media, LLC.

  6. Weak form factors of beauty baryons

    International Nuclear Information System (INIS)

    Ivanov, M.A.; Lyubovitskij, V.E.

    1992-01-01

    Full analysis of semileptonic decays of beauty baryons with J p =1/2 2 and J p =3/2 2 into charmed ones within the Quark Confinement Model is reported. Weak form factors and decay rates are calculated. Also the heavy quark limit m Q →∞ (Isgur-Wise symmetry) is examined. The weak heavy-baryon form factors in the Isgur-Wise limit and 1/m Q -corrections to them are computered. The Ademollo-Gatto theorem is spin-flavour symmetry of heavy quarks is checked. 33 refs.; 1 fig.; 9 tabs

  7. Weak interactions at high energies. [Lectures, review

    Energy Technology Data Exchange (ETDEWEB)

    Ellis, J.

    1978-08-01

    Review lectures are presented on the phenomenological implications of the modern spontaneously broken gauge theories of the weak and electromagnetic interactions, and some observations are made about which high energy experiments probe what aspects of gauge theories. Basic quantum chromodynamics phenomenology is covered including momentum dependent effective quark distributions, the transverse momentum cutoff, search for gluons as sources of hadron jets, the status and prospects for the spectroscopy of fundamental fermions and how fermions may be used to probe aspects of the weak and electromagnetic gauge theory, studies of intermediate vector bosons, and miscellaneous possibilities suggested by gauge theories from the Higgs bosons to speculations about proton decay. 187 references. (JFP)

  8. Shock waves in weakly compressed granular media.

    Science.gov (United States)

    van den Wildenberg, Siet; van Loo, Rogier; van Hecke, Martin

    2013-11-22

    We experimentally probe nonlinear wave propagation in weakly compressed granular media and observe a crossover from quasilinear sound waves at low impact to shock waves at high impact. We show that this crossover impact grows with the confining pressure P0, whereas the shock wave speed is independent of P0-two hallmarks of granular shocks predicted recently. The shocks exhibit surprising power law attenuation, which we model with a logarithmic law implying that shock dissipation is weak and qualitatively different from other granular dissipation mechanisms. We show that elastic and potential energy balance in the leading part of the shocks.

  9. Categorization of States Beyond Strong and Weak

    Directory of Open Access Journals (Sweden)

    Peter Tikuisis

    2017-09-01

    Full Text Available The discourse on poor state performers has suffered from widely varying definitions on what distinguishes certain weak states from others. Indices that rank states from strong to weak conceal important distinctions that can adversely affect intervention policy. This deficiency is addressed by grouping states according to their performance on three dimensions of statehood: authority, legitimacy, and capacity. The resultant categorization identifies brittle states that are susceptible to regime change, impoverished states often considered as aid darlings, and fragile states that experience disproportionately high levels of violent internal conflict. It also provides a quantifiable means to analyze transitions from one state type to another for more insightful intervention policy.

  10. Theoretical status of weak and electromagnetic interactions

    Energy Technology Data Exchange (ETDEWEB)

    Pandit, L. K.

    1980-07-01

    An extended simple version of the Weinberg gauge model is proposed to bring together weak and electromagnetic interactions under one theory. The essential features of the standard SU/sub 2/ (operating on)U/sub 1/ gauge scheme with four leptons and four quark flavours is recalled. Charged-current and neutral current interactions are described. Non-leptonic decays of strange particles are studied. The treatment is extended to 6-leptons and 6-quark flavours. The short comings of this model are discussed. Speculations on the unification of strong, weak and electromagnetic interactions are made.

  11. CPT non-invariance and weak interactions

    International Nuclear Information System (INIS)

    Hsu, J.P.

    1973-01-01

    In this talk, I will describe a possible violation of CPT invariance in the domain of weak interactions. One can construct a model of weak interactions which, in order to be consistent with all experimental data, must violate CPT maximally. The model predicts many specific results for decay processes which could be tested in the planned neutral hyperon beam or neutrino beam at NAL. The motivations and the physical idea in the model are explained and the implications of the model are discussed. (U.S.)

  12. Relationship between lower extremity isometric muscle strength and standing balance in patients with multiple sclerosis.

    Science.gov (United States)

    Citaker, Seyit; Guclu-Gunduz, Arzu; Yazici, Gokhan; Bayraktar, Deniz; Nazliel, Bijen; Irkec, Ceyla

    2013-01-01

    Muscle strength and standing balance decrease in patients with Multiple Sclerosis (MS). The aim of the present study was to investigate the relationship between the lower extremity isometric muscle strength and standing balance in patients with MS. Forty-seven patients with MS and 10 healthy volunteers were included. Neurological disability level was assessed using Expanded Disability Status Scale (EDSS). Isometric strength of seven lower extremity muscles (hip flexor-extensor-abductor-adductor, knee flexor-extensor, and ankle dorsal flexor) was assessed using hand-held dynamometer. Duration of static one-leg standing balance was measured using digital chronometer. Hip flexor-extensor-abductor-adductor, knee flexor-extensor, and ankle dorsal flexor isometric muscle strength, and duration of one-leg standing balance were decreased in patients with MS when compared with controls (p isometric muscle strength and EDSS level was related duration of one-leg standing balance in patients with MS. All assessed lower extremity isometric muscle strength (except ankle dorsal flexor) was related with EDSS. Hip flexor-extensor-abductor-adductor, knee flexor-extensor, and ankle dorsal flexor isometric muscle strength decreases in ambulatory MS patients. Lower extremity muscle weakness and neurological disability level are related with imbalance in MS population. Hip and knee region muscles weakness increases the neurological disability level. For the better balance and decrease neurological disability level whole lower extremity muscle strengthening should be included in rehabilitation programs.

  13. Magnetic resonance imaging, ultrasound and real-time ultrasound elastography of the thigh muscles in congenital muscle dystrophy

    Energy Technology Data Exchange (ETDEWEB)

    Drakonaki, Eleni E. [University of Crete, Radiology Department, Heraklion (Greece); Allen, Gina M. [Green Templeton College, Oxford (United Kingdom)

    2010-04-15

    Congenital muscle dystrophy includes a range of genetic disorders characterized by muscle weakness and contractures. We report the magnetic resonance (MR), ultrasound (US) and real-time sonoelastography (RTE) imaging findings of the thigh muscles of a 15-year-old boy with Bethlem myopathy diagnosed with clinical, electromyographic and histopathological criteria. Ultrasound and MR showed hyperechoic appearance and high signal intensity on T1- and T2-weighted sequences respectively at the periphery of the vastus lateralis and the long head of the biceps femoris muscles, and at a central area within the rectus femoris muscles. RTE was employed to examine the elastic properties of the muscle. The elastograms were presented as colour-coded maps superimposed on the B-mode images and revealed that the elastographic pattern correlated with the MR and US pattern of involvement. The abnormal muscle areas were stiffer (blue) than the normal-appearing areas (green), a finding that probably correlates with the presence of dystrophic collagen at the affected areas. This report suggests that RTE could be used as an additional imaging tool to evaluate the pattern of muscle changes in congenital myopathy. Further studies are needed to investigate the specificity and clinical value of RTE in the diagnosis and monitoring of neuromuscular disease. (orig.)

  14. Magnetic resonance imaging, ultrasound and real-time ultrasound elastography of the thigh muscles in congenital muscle dystrophy

    International Nuclear Information System (INIS)

    Drakonaki, Eleni E.; Allen, Gina M.

    2010-01-01

    Congenital muscle dystrophy includes a range of genetic disorders characterized by muscle weakness and contractures. We report the magnetic resonance (MR), ultrasound (US) and real-time sonoelastography (RTE) imaging findings of the thigh muscles of a 15-year-old boy with Bethlem myopathy diagnosed with clinical, electromyographic and histopathological criteria. Ultrasound and MR showed hyperechoic appearance and high signal intensity on T1- and T2-weighted sequences respectively at the periphery of the vastus lateralis and the long head of the biceps femoris muscles, and at a central area within the rectus femoris muscles. RTE was employed to examine the elastic properties of the muscle. The elastograms were presented as colour-coded maps superimposed on the B-mode images and revealed that the elastographic pattern correlated with the MR and US pattern of involvement. The abnormal muscle areas were stiffer (blue) than the normal-appearing areas (green), a finding that probably correlates with the presence of dystrophic collagen at the affected areas. This report suggests that RTE could be used as an additional imaging tool to evaluate the pattern of muscle changes in congenital myopathy. Further studies are needed to investigate the specificity and clinical value of RTE in the diagnosis and monitoring of neuromuscular disease. (orig.)

  15. Temporalis muscle hypertrophy and reduced skull eccentricity in Duchenne muscular dystrophy.

    Science.gov (United States)

    Straathof, C S M; Doorenweerd, N; Wokke, B H A; Dumas, E M; van den Bergen, J C; van Buchem, M A; Hendriksen, J G M; Verschuuren, J J G M; Kan, H E

    2014-10-01

    Muscle hypertrophy and muscle weakness are well known in Duchenne muscular dystrophy. Decreased muscle force can have secondary effects on skeletal growth and development such as facial and dental morphology changes. In this study, we quantified temporal muscle thickness, circumference, and eccentricity of the skull and the head on T1-weighted magnetic resonance imaging (MRI) scans of the head of 15 Duchenne muscular dystrophy patients and 15 controls. Average temporal muscle thickness was significantly increased in patients (12.9 ± 5.2 mm) compared to controls (6.8 ± 1.4 mm) (P muscle thickness and skull eccentricity were significantly negatively correlated in patients, and positively in controls. Hypertrophy of the temporal muscles and changes in skull eccentricity appear to occur early in the course of Duchenne muscular dystrophy. Further studies in younger patients are needed to confirm a causal relationship. © The Author(s) 2014.

  16. Acquired muscle contractures in the dog and cat. A review of the literature and case report.

    Science.gov (United States)

    Taylor, J; Tangner, C H

    2007-01-01

    Canine and feline muscle contracture is reported to affect several different muscles, is associated with a number of predisposing factors, and a varying prognosis depending upon which muscle is affected. Most patients suffer some form of trauma weeks to months before the contracture is present. The clinical signs include: lameness, pain, weakness, decreased range of motion, a firmness noted throughout the entire muscle, and usually a characteristic gait. Pre-disposing factors for muscle contracture include: compartment syndrome, infection, trauma, repetitive strains, fractures, infectious diseases, immune-mediated diseases, neoplasia, and ischaemia. There does appear to be some breed and age predilection, however, the sex of the animal does not have an appreciable influence. In general, muscle contractures of the forelimb respond better to treatment and carry a better prognosis than muscle contractures of the hindlimb.

  17. Weakly nonlocal symplectic structures, Whitham method and weakly nonlocal symplectic structures of hydrodynamic type

    International Nuclear Information System (INIS)

    Maltsev, A Ya

    2005-01-01

    We consider the special type of field-theoretical symplectic structures called weakly nonlocal. The structures of this type are, in particular, very common for integrable systems such as KdV or NLS. We introduce here the special class of weakly nonlocal symplectic structures which we call weakly nonlocal symplectic structures of hydrodynamic type. We investigate then the connection of such structures with the Whitham averaging method and propose the procedure of 'averaging' the weakly nonlocal symplectic structures. The averaging procedure gives the weakly nonlocal symplectic structure of hydrodynamic type for the corresponding Whitham system. The procedure also gives 'action variables' corresponding to the wave numbers of m-phase solutions of the initial system which give the additional conservation laws for the Whitham system

  18. Weak organic acid stress in Bacillus subtilis

    NARCIS (Netherlands)

    ter Beek, A.S.

    2009-01-01

    Weak organic acids are commonly used food preservatives that protect food products from bacterial contamination. A variety of spore-forming bacterial species pose a serious problem to the food industry by causing extensive food spoilage or even food poisoning. Understanding the mechanisms of

  19. Common Fixed Points for Weakly Compatible Maps

    Indian Academy of Sciences (India)

    The purpose of this paper is to prove a common fixed point theorem, from the class of compatible continuous maps to a larger class of maps having weakly compatible maps without appeal to continuity, which generalized the results of Jungck [3], Fisher [1], Kang and Kim [8], Jachymski [2], and Rhoades [9].

  20. Weak MSO: automata and expressiveness modulo bisimilarity

    NARCIS (Netherlands)

    Carreiro, F.; Facchini, A.; Venema, Y.; Zanasi, F.

    2014-01-01

    We prove that the bisimulation-invariant fragment of weak monadic second-order logic (WMSO) is equivalent to the fragment of the modal μ-calculus where the application of the least fixpoint operator μp.φ is restricted to formulas φ that are continuous in p. Our proof is automata-theoretic in nature;

  1. Weak lensing probes of modified gravity

    International Nuclear Information System (INIS)

    Schmidt, Fabian

    2008-01-01

    We study the effect of modifications to general relativity on large-scale weak lensing observables. In particular, we consider three modified gravity scenarios: f(R) gravity, the Dvali-Gabadadze-Porrati model, and tensor-vector-scalar theory. Weak lensing is sensitive to the growth of structure and the relation between matter and gravitational potentials, both of which will in general be affected by modified gravity. Restricting ourselves to linear scales, we compare the predictions for galaxy-shear and shear-shear correlations of each modified gravity cosmology to those of an effective dark energy cosmology with the same expansion history. In this way, the effects of modified gravity on the growth of perturbations are separated from the expansion history. We also propose a test which isolates the matter-potential relation from the growth factor and matter power spectrum. For all three modified gravity models, the predictions for galaxy and shear correlations will be discernible from those of dark energy with very high significance in future weak lensing surveys. Furthermore, each model predicts a measurably distinct scale dependence and redshift evolution of galaxy and shear correlations, which can be traced back to the physical foundations of each model. We show that the signal-to-noise for detecting signatures of modified gravity is much higher for weak lensing observables as compared to the integrated Sachs-Wolfe effect, measured via the galaxy-cosmic microwave background cross-correlation.

  2. Weak contractions via $\\lambda$-sequences

    OpenAIRE

    Agyingi, Collins Amburo; Gaba, Yaé Ulrich

    2018-01-01

    In this note, we discuss common fixed point for a family of self mapping defined on a metric type space and satisfying a weakly contractive condition. In our development, we make use of the $\\lambda$-sequence approach and also of a certain class of real valued maps. We derive some implications for self-mappings on quasi-pseudometric type spaces.

  3. Reducing Weak to Strong Bisimilarity in CCP

    Directory of Open Access Journals (Sweden)

    Andrés Aristizábal

    2012-12-01

    Full Text Available Concurrent constraint programming (ccp is a well-established model for concurrency that singles out the fundamental aspects of asynchronous systems whose agents (or processes evolve by posting and querying (partial information in a global medium. Bisimilarity is a standard behavioural equivalence in concurrency theory. However, only recently a well-behaved notion of bisimilarity for ccp, and a ccp partition refinement algorithm for deciding the strong version of this equivalence have been proposed. Weak bisimiliarity is a central behavioural equivalence in process calculi and it is obtained from the strong case by taking into account only the actions that are observable in the system. Typically, the standard partition refinement can also be used for deciding weak bisimilarity simply by using Milner's reduction from weak to strong bisimilarity; a technique referred to as saturation. In this paper we demonstrate that, because of its involved labeled transitions, the above-mentioned saturation technique does not work for ccp. We give an alternative reduction from weak ccp bisimilarity to the strong one that allows us to use the ccp partition refinement algorithm for deciding this equivalence.

  4. Weak radiative baryonic decays of B mesons

    International Nuclear Information System (INIS)

    Kohara, Yoji

    2004-01-01

    Weak radiative baryonic B decays B→B 1 B 2 -barγ are studied under the assumption of the short-distance b→sγ electromagnetic penguin transition dominance. The relations among the decay rates of various decay modes are derived

  5. Weak NNM couplings and nuclear parity violation

    International Nuclear Information System (INIS)

    Holstein, B.R.

    1987-01-01

    After many years of careful theoretical and experimental study of nuclear parity violation, rough empirical values for weak parity violation nucleon-nucleon-meson vertices have been deduced. We address some of the physics which has been learned from this effort and show that it has implications for work going on outside this field. (author)

  6. Weak universality in inhomogeneous Ising quantum chains

    International Nuclear Information System (INIS)

    Karevski, Dragi

    2006-01-01

    The Ising quantum chain with arbitrary coupling distribution {λ i } leading to an anisotropic scaling is considered. The smallest gap of the chain is connected to the surface magnetization by the relation Λ 1 = m s ({λ i })m s ({λ -1 i }). For some aperiodic distribution {λ i }, a weak universality of the critical behaviour is found. (letter to the editor)

  7. Broken color symmetry and weak currents

    International Nuclear Information System (INIS)

    Stech, B.

    1976-01-01

    Broken colour symmetry predicts a very rich spectrum of new particles. If broken colour is relevant at all, charged psi-particles should be found in particular at the 4 GeV region. For the weak hadronic currents no completely satisfactory suggestion exists. Broken colour symmetry describes qualitatively several of the new effects observed recently. (BJ) [de

  8. Solitons and Weakly Nonlinear Waves in Plasmas

    DEFF Research Database (Denmark)

    Pécseli, Hans

    1985-01-01

    Theoretical descriptions of solitons and weakly nonlinear waves propagating in plasma media are reviewed, with particular attention to the Korteweg-de Vries (KDV) equation and the Nonlinear Schrödinger equation (NLS). The modifications of these basic equations due to the effects of resonant...

  9. Efficient bootstrap with weakly dependent processes

    NARCIS (Netherlands)

    Bravo, Francesco; Crudu, Federico

    The efficient bootstrap methodology is developed for overidentified moment conditions models with weakly dependent observation. The resulting bootstrap procedure is shown to be asymptotically valid and can be used to approximate the distributions of t-statistics, the J-statistic for overidentifying

  10. Weak equivalence classes of complex vector bundles

    Czech Academy of Sciences Publication Activity Database

    Le, Hong-Van

    LXXVII, č. 1 (2008), s. 23-30 ISSN 0862-9544 R&D Projects: GA AV ČR IAA100190701 Institutional research plan: CEZ:AV0Z10190503 Keywords : chern classes * complex Grassmannians weak equivalence Subject RIV: BA - General Mathematics

  11. Gauge theories of the weak interactions

    International Nuclear Information System (INIS)

    Quinn, H.

    1978-08-01

    Two lectures are presented on the Weinberg--Salam--Glashow--Iliopoulos--Maiani gauge theory for weak interactions. An attempt is made to give some impressions of the generality of this model, how it was developed, variations found in the literature, and the status of the standard model. 21 references

  12. Electric properties of weakly nonideal plasmas

    Energy Technology Data Exchange (ETDEWEB)

    Guenther, K; Radtke, R

    1984-01-01

    The progress in theory as well as in diagnostics and measurement during the last fifteen years is reviewed. Starting from the transport theory of ideal plasmas physically justified corrections are introduced which allow the quantitative calculation of the transport properties of weakly nonideal plasmas. Essential coefficients and numerical data of the electrical conductivity for plasmas of technical importance are given in tables and diagrams.

  13. Localization on weakly disordered Cayley tree

    International Nuclear Information System (INIS)

    Brezini, A.; Olivier, G.

    1980-08-01

    The localization model of Kumar et al. is critically re-examined for the approximation γ → 0, which describes weak disorder. By using an improved method of approximation, we have studied the displacement of the band and the mobility edges and compared the result of Kumar et al. and Abou-Chacra et al. in the light of the present approximation. (author)

  14. Studying dark matter haloes with weak lensing

    NARCIS (Netherlands)

    Velander, Malin Barbro Margareta

    2012-01-01

    Our Universe is comprised not only of normal matter but also of unknown components: dark matter and dark energy. This Thesis recounts studies of dark matter haloes, using a technique known as weak gravitational lensing, in order to learn more about the nature of these dark components. The haloes

  15. Completely continuous and weakly completely continuous abstract ...

    Indian Academy of Sciences (India)

    An algebra A is called right completely continuous (right weakly completely continuous) ... Moreover, some applications of these results in group algebras are .... A linear subspace S(G) of L1(G) is said to be a Segal algebra, if it satisfies the.

  16. Collisional properties of weakly bound heteronuclear dimers

    NARCIS (Netherlands)

    Marcelis, B.; Kokkelmans, S.J.J.M.F.; Shlyapnikov, G.V.; Petrov, D.S.

    2008-01-01

    We consider collisional properties of weakly bound heteronuclear molecules (dimers) formed in a two-species mixture of atoms with a large mass difference. We focus on dimers containing light fermionic atoms as they manifest collisional stability due to an effective dimer-dimer repulsion originating

  17. Quantum mechanical calculations on weakly interacting complexes

    NARCIS (Netherlands)

    Heijmen, T.G.A.

    1998-01-01

    Symmetry-adapted perturbation theory (SAPT) has been applied to compute the intermolecular potential energy surfaces and the interaction-induced electrical properties of weakly interacting complexes. Asymptotic (large R) expressions have been derived for the contributions to the collision-induced

  18. Weak-interaction rates in stellar conditions

    Science.gov (United States)

    Sarriguren, Pedro

    2018-05-01

    Weak-interaction rates, including β-decay and electron captures, are studied in several mass regions at various densities and temperatures of astrophysical interest. In particular, we study odd-A nuclei in the pf-shell region, which are involved in presupernova formations. Weak rates are relevant to understand the late stages of the stellar evolution, as well as the nucleosynthesis of heavy nuclei. The nuclear structure involved in the weak processes is studied within a quasiparticle proton-neutron random-phase approximation with residual interactions in both particle-hole and particle-particle channels on top of a deformed Skyrme Hartree-Fock mean field with pairing correlations. First, the energy distributions of the Gamow-Teller strength are discussed and compared with the available experimental information, measured under terrestrial conditions from charge-exchange reactions. Then, the sensitivity of the weak-interaction rates to both astrophysical densities and temperatures is studied. Special attention is paid to the relative contribution to these rates of thermally populated excited states in the decaying nucleus and to the electron captures from the degenerate electron plasma.

  19. Black holes and the weak cosmic censorship

    International Nuclear Information System (INIS)

    Krolak, A.

    1984-01-01

    A theory of black holes is developed under the assumption of the weak cosmic censorship. It includes Hawking's theory of black holes in the future asymptotically predictable space-times as a special case but it also applies to the cosmological situations including models with nonzero cosmological constant of both signs. (author)

  20. How weak is the subduction zone interface?

    NARCIS (Netherlands)

    Duarte, João C.; Schellart, Wouter P.; Cruden, Alexander R.

    2015-01-01

    Several lines of evidence suggest that subduction zones are weak and that the unique availability of water on Earth is a critical factor in the weakening process. We have evaluated the strength of subduction zone interfaces using two approaches: (i) from empirical relationships between shear stress