Stiff person syndrome presenting with sudden onset of shortness of breath and difficulty moving the right arm: a case report

<p>Abstract</p> <p>Introduction</p> <p>First described in 1956, stiff person syndrome is characterized by episodes of slowly progressive stiffness and rigidity in both the paraspinal and limb muscles. Although considered a rare disorder, stiff person syndrome is likely to be under-diagnosed due to a general lack of awareness of the disease in the medical community.</p> <p>Case presentation</p> <p>A 27-year-old Hispanic woman presented to our emergency department with a sudden onset of shortness of breath and difficulty moving her right arm. Her physical examination was remarkable in that her abdomen was firm to palpation and her right upper extremity was rigid on passive and active ranges of motion. Her right fingers were clenched in a fist. Her electromyography findings were consistent with stiff person syndrome in the right clinical setting. Stiff person syndrome is confirmed by testing for the anti-glutamic acid decarboxylase antibody. Her test for this was positive.</p> <p>Conclusion</p> <p>Stiff person syndrome may not be a common condition. However, if disregarded in the differential diagnosis, it can lead to several unnecessary tests being carried out causing a delay in treatment. This case report reveals some of the characteristic features of stiff person syndrome with an atypical presentation.</p

Recall termination in free recall

Although much is known about the dynamics of memory search in the free recall task, relatively little is known about the factors related to recall termination. Reanalyzing individual trial data from 14 prior studies (1,079 participants in 28,015 trials) and defining termination as occurring when a final response is followed by a long nonresponse interval, we observed that termination probability increased throughout the recall period and that retrieval was more likely to terminate following an error than following a correct response. Among errors, termination probability was higher following prior-list intrusions and repetitions than following extralist intrusions. To verify that this pattern of results can be seen in a single study, we report a new experiment in which 80 participants contributed recall data from a total of 9,122 trials. This experiment replicated the pattern observed in the aggregate analysis of the prior studies

Shear-Mediated Dilation of the Internal Carotid Artery Occurs Independent of Hypercapnia.

Evidence for shear stress as a regulator of carotid artery dilation in response to increased arterial carbon dioxide was recently demonstrated in humans during sustained elevations in CO2 (hypercapnia); however, the relative contributions of CO2 and shear stress to this response remains unclear. We examined the hypothesis that, following a 30-second transient increase in arterial CO2 tension and consequent increase in internal carotid artery shear stress, internal carotid artery diameter would increase, indicating shear-mediated dilation, in the absence of concurrent hypercapnia. In 27 healthy participants the partial pressures of end-tidal O2 and CO2, ventilation (pneumotachography), blood pressure (finger-photoplethysmography), heart-rate (electrocardiogram), internal carotid artery flow, diameter and shear stress (high resolution duplex ultrasound) and middle cerebral artery blood velocity (transcranial Doppler) were measured during 4-minute steady state and transient 30-second hypercapnic tests (both +9mmHg CO2). Internal carotid artery dilation was lower in the transient, compared to the steady state hypercapnia (3.3±1.9% vs. 5.3±2.9%, respectively; P<0.03). Increases in internal carotid artery shear stress preceded increases in diameter in both the transient (time: 16.8±13.2s vs. 59.4±60.3s; P<0.01) and steady state (time: 18.2±14.2s vs. 110.3±79.6s; P<0.01) tests. Internal carotid artery dilation was positively correlated with shear rate area under the curve in the transient (r(2)=0.44; P<0.01), but not steady state (r(2)=0.02; P=0.53) trial. Collectively, these results suggest that hypercapnia induces shear-mediated dilation of the internal carotid artery in humans. This study further promotes the application and development of hypercapnia as a clinical strategy for the assessment of cerebrovascular vasodilatory function and health in humans

The effect of graphite and carbon black ratios on conductive ink performance

Conductive inks based on graphite and carbon black are used in a host of applications including energy storage, energy harvesting, electrochemical sensors and printed heaters. This requires accurate control of electrical properties tailored to the application; ink formulation is a fundamental element of this. Data on how formulation relates to properties have tended to apply to only single types of conductor at any time, with data on mixed types of carbon only empirical thus far. Therefore, screen printable carbon inks with differing graphite, carbon black and vinyl polymer content were formulated and printed to establish the effect on rheology, deposition and conductivity. The study found that at a higher total carbon loading ink of 29.4% by mass, optimal conductivity (0.029 Ω cm) was achieved at a graphite to carbon black ratio of 2.6 to 1. For a lower total carbon loading (21.7 mass %), this ratio was reduced to 1.8 to 1. Formulation affected viscosity and hence ink transfer and also surface roughness due to retention of features from the screen printing mesh and the inherent roughness of the carbon components, as well as the ability of features to be reproduced consistently

Feasibility and acceptability of point of care HIV testing in community outreach and GUM drop-in services in the North West of England: A programmatic evaluation

Background: In Liverpool, injecting drug users (IDUs), men-who-have-sex-with-men (MSM) and UK Africans experience a disproportionate burden of HIV, yet services do not reach out to these groups and late presentations continue. We set out to: increase testing uptake in targeted marginalized groups through a community and genitourinary medicine (GUM)-based point of care testing (POCT) programme; and conduct a process evaluation to examine service provider inputs and document service user perceptions of the programme. Methods: Mixed quantitative, qualitative and process evaluation methods were used. Service providers were trained to use fourth generation rapid antibody/antigen HIV tests. Existing outreach services incorporated POCT into routine practice. Clients completed a semi-structured questionnaire and focus group discussions (FGDs) were held with service providers. Results: Between September 2009 and June 2010, 953 individuals underwent POCT (GUM: 556 [59%]; communitybased sites: 397 [42%]). Participants in the community were more likely to be male (p = 0.028), older (p < 0.001), of UK African origin (p < 0.001) and IDUs (p < 0.001) than participants from the GUM clinic. Seventeen new HIV diagnoses were confirmed (prevalence = 1.8%), 16 of whom were in risk exposure categories (prevalence: 16/517, 3.1%). Questionnaires and FGDs showed that clients and service providers were supportive of POCT, highlighting benefits of reaching out to marginalised communities and incorporating HIV prevention messages. Conclusions: Community and GUM clinic-based POCT for HIV was feasible and acceptable to clients and service providers in a low prevalence setting. It successfully reached target groups, many of whom would not have otherwise tested. We recommend POCT be considered among strategies to increase the uptake of HIV testing among groups who are currently underserved

Subclonal TP53 copy number is associated with prognosis in multiple myeloma

Multiple myeloma (MM) is a genetically heterogeneous cancer of bone marrow plasma cells with variable outcome. To assess the prognostic relevance of clonal heterogeneity of TP53 copy number, we profiled tumors from 1777 newly diagnosed Myeloma XI trial patients with multiplex ligation-dependent probe amplification (MLPA). Subclonal TP53 deletions were independently associated with shorter overall survival, with a hazard ratio of 1.8 (95% confidence interval, 1.2-2.8; P = .01). Clonal, but not subclonal, TP53 deletions were associated with clinical markers of advanced disease, specifically lower platelet counts (P < .001) and increased lactate dehydrogenase (P < .001), as well as a higher frequency of features indicative of genomic instability, del(13q) (P = .002) or del(1p) (P = .006). Biallelic TP53 loss-of-function by mutation and deletion was rare (2.4%) and associated with advanced disease. We present a framework for identifying subclonal TP53 deletions by MLPA, to improve patient stratification in MM and tailor therapy, enabling management strategies

Use of a Cybex NORM dynamometer to assess muscle function in patients with thoracic cancer

<p>Abstract</p> <p>Background</p> <p>The cachexia-anorexia syndrome impacts on patients' physical independence and quality of life. New treatments are required and need to be evaluated using acceptable and reliable outcome measures, e.g. the assessment of muscle function. The aims of this study were to: (i) examine the acceptability and reliability of the Cybex NORM dynamometer to assess muscle function in people with non-small cell lung cancer or mesothelioma; (ii) compare muscle function in this group with healthy volunteers and; (iii) explore changes in muscle function over one month.</p> <p>Methods</p> <p>The test consisted of 25 repetitions of isokinetic knee flexion and extension at maximal effort while seated on a Cybex NORM dynamometer. Strength and endurance for the quadriceps and hamstrings were assessed as peak torque and total work and an endurance ratio respectively. Thirteen patients and 26 volunteers completed the test on three separate visits. Acceptability was assessed by questionnaire, reliability by intraclass correlation coefficients (ICC) and tests of difference compared outcomes between and within groups.</p> <p>Results</p> <p>All subjects found the test acceptable. Peak torque and work done were reliable measures (ICC >0.80), but the endurance ratio was not. Muscle function did not differ significantly between the patient and a matched volunteer group or in either group when repeated after one month.</p> <p>Conclusion</p> <p>For patients with non-small cell lung cancer or mesothelioma, the Cybex NORM dynamometer provides an acceptable and reliable method of assessing muscle strength and work done. Muscle function appears to be relatively well preserved in this group and it appears feasible to explore interventions which aim to maintain or even improve this.</p

First Neutrino Observations from the Sudbury Neutrino Observatory

The first neutrino observations from the Sudbury Neutrino Observatory are presented from preliminary analyses. Based on energy, direction and location, the data in the region of interest appear to be dominated by 8B solar neutrinos, detected by the charged current reaction on deuterium and elastic scattering from electrons, with very little background. Measurements of radioactive backgrounds indicate that the measurement of all active neutrino types via the neutral current reaction on deuterium will be possible with small systematic uncertainties. Quantitative results for the fluxes observed with these reactions will be provided when further calibrations have been completed.Comment: Latex, 7 pages, 10 figures, Invited paper at Neutrino 2000 Conference, Sudbury, Canada, June 16-21, 2000 to be published in the Proceeding