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Background The global SARS-CoV-2 pandemic placed Irish Laboratory Medicine services under sustained and massive strain. Rapid reconfiguration was required to introduce new assays at high capacity for diagnosis and monitoring of COVID-19, while maintaining existing services. Aim The aim of this national survey was to capture Laboratory Medicine’s response across the Republic of Ireland during the first wave of the COVID-19 pandemic. Methods An electronic survey developed using Microsoft Forms® was emailed on 5 October 2020 to 53 local representatives of the PeriAnalytic and Laboratory Medicine Society (PALMSoc), reaching 38 separate pathology departments in the country. Results A total of 45 responses from 38 laboratories were received (72% response rate) representing a range of departments and disciplines. Most laboratories (63%) introduced new tests, and in a time frame of less than 6 weeks (80%). Point-of-care testing (POCT) played a significant role in the response to COVID-19, with almost half of respondents (47%) reporting that additional equipment was introduced. Maintenance of the Quality Management System (QMS) proved challenging, with 60% of respondents indicating that not all aspects were sustained. When asked about changes to staff rostering, 98% of respondents reported that changes were made. All adjustments were made despite staffing challenges; only 18% of respondents described the staffing levels in their department as 100% prior to the onset of the first wave. Conclusions This study confirms an agile and resilient response to the COVID-19 pandemic from Ireland’s Laboratory Medicine services despite many economic and staffing challenges.

Autonomous ship technology is developing at a rapid pace, with the aim of facilitating safe ship operations. Collision avoidance is one of the most critical tasks that autonomous ships must handle. To support the level of safety associated with collision avoidance, this study suggests to provide autonomous ships with the ability to conduct proactive collision avoidance maneuvers. Proactive collision avoidance entails predicting future encounter situations, such that they can preemptively be avoided. However, any such actions must adhere to relevant navigation rules and regulations. As such, it is suggested to predict encounter situations far in advance, i.e. prior to risk of collision existing. Any actions can, therefore, be conducted prior to the applicability of the COLREGs. As such, simple corrective measures, e.g. minor speed and/or heading alterations, can prevent close encounter situations from arising, reducing the overall risk associated with autonomous ship operations, as well as improving traffic flow. This study suggests to facilitate this ability by emulating the development of situation awareness in ship navigators through machine learning. By leveraging historical AIS data to serve as artificial navigational experience, long-range trajectory predictions can be facilitated in a similar manner those conducted by human navigators, where such predictions provide the basis for proactive collision avoidance actions. The development of human situation awareness is, therefore, presented, and relevant machine learning techniques are discussed to emulate the same mechanisms.

Pulmonary embolism (PE) is a significant contributor to morbidity and mortality in the United States. Catheter-directed, ultrasound-assisted thrombolysis (USAT) uses high-frequency, low-energy ultrasound waves to disaggregate uncrosslinked fibrin fibers and increase thrombus penetration of a locally delivered thrombolytic to treat an acute PE. The purpose of this study is to compare the efficacy and safety of catheter-directed USAT versus systemic anticoagulation alone in submassive PE. This was a single-center, retrospective study of patients with a diagnosis of acute submassive PE from April 4, 2014 to May 1, 2019 at a large, academic medical center. Subjects were split into two different groups based on treatment with either USAT with systemic anticoagulation or systemic anticoagulation alone. The primary outcome was the incidence of severe or life-threatening GUSTO bleeding within 72 h or until hospital discharge if sooner. A total of 130 subjects were included (n = 40 in the USAT group and n = 90 in systemic anticoagulation alone group). Significantly fewer subjects in the USAT group had an active diagnosis of cancer at the time of presentation (7.5% vs 28.9%, p = 0.006). There was no difference in severe or life-threatening GUSTO bleeding or any component of the GUSTO bleeding definitions. Administration of USAT with systemic anticoagulation was well-tolerated when compared to systemic anticoagulation alone, but bias may have led to selection of patients for USAT with a lower bleeding risk and higher functional status at baseline.

Background We describe the clinical and neuropathological features of a patient with T-cell-mediated paraneoplastic limbic encephalitis, parkinsonism, hypothermia, and narcolepsy-like presentation associated with endometrial carcinoma. Objectives This patient with prominent parkinsonism and narcolepsy broadens the phenotype of known paraneoplastic syndromes and demonstrates the importance of investigation for occult malignancy even in the absence of paraneoplastic antibodies. Methods This is a case report with diagnosis confirmed at postmortem. Results Paraneoplastic antibodies were not detected. The initial improvement with immunosuppression was short lived, and postmortem neuropathological examination demonstrated encephalitis with predominant T-cell infiltration affecting the hypothalamus and extending to the brainstem, suggestive of a paraneoplastic syndrome. Conclusions Although the possibility of a novel antibody cannot be ruled out, consideration must also be given to recent demonstration of purely T-cell-mediated neuronal destruction in the context of paraneoplastic syndromes.

Disclaimer In an effort to expedite the publication of articles related to the COVID-19 pandemic, AJHP is posting these manuscripts online as soon as possible after acceptance. Accepted manuscripts have been peer-reviewed and copyedited, but are posted online before technical formatting and author proofing. These manuscripts are not the final version of record and will be replaced with the final article (formatted per AJHP style and proofed by the authors) at a later time.

Purpose Obtaining an accurate medication history from patients on hospital admission is a priority in pharmacy practice. Timely and accurate histories are imperative as they may help determine the etiology of illness and prevent medication errors. We conducted a quality improvement project to assess the accuracy of alternate-source medication histories obtained for critically ill patients who were delirious or mechanically ventilated at the time of intensive care unit admission. Methods Included patients were 18 years of age or older, admitted to the medical intensive care unit from August 2017 through January 2018, and had a medication history obtained from a family member or outpatient pharmacy due to active delirium or mechanical ventilation. Patients were directly interviewed after resolution of delirium or extubation. Discrepancies between the initial and follow-up histories were documented and categorized using the National Coordinating Council for Medication Error Reporting and Prevention (NCC MERP) Index for Categorizing Medication Errors. Results Forty patients were included. One hundred four discrepancies were documented, with a median of 2 discrepancies per patient. The most common types of discrepancies were addition (51.9%), followed by omission (24.0%). NCC MERP index category A (51%) was the most common error classification identified. Conclusion Discrepancies between initial and follow-up medication histories occurred at a frequent rate in delirious or mechanically ventilated patients; however, these discrepancies tended to be of low risk severity.

An operationally implementable predictive model has been developed to forecast the number of COVID-19 infections in the patient population, hospital floor and ICU censuses, ventilator and related supply chain demand. The model is intended for clinical, operational, financial and supply chain leaders and executives of a comprehensive healthcare system responsible for making decisions that depend on epidemiological contingencies. This paper describes the model that was implemented at NorthShore University HealthSystem and is applicable to any communicable disease whose risk of reinfection for the duration of the pandemic is negligible.

This study presents a deep learning framework to support regional ship behavior prediction using historical AIS data. The framework is meant to aid in proactive collision avoidance, in order to enhance the safety of maritime transportation systems. In this study, it is suggested to decompose the historical ship behavior in a given geographical region into clusters. Each cluster will contain trajectories with similar behavior characteristics. For each unique cluster, the method generates a local model to describe the local behavior in the cluster. In this manner, higher fidelity predictions can be facilitated compared to training a model on all available historical behavior. The study suggests to cluster historical trajectories using a variational recurrent autoencoder and the Hierarchical Density-Based Spatial Clustering of Applications with Noise algorithm. The past behavior of a selected vessel is then classified to the most likely clusters of behavior based on the softmax distribution. Each local model consists of a sequence-to-sequence model with attention. When utilizing the deep learning framework, a user inputs the past trajectory of a selected vessel, and the framework outputs the most likely future trajectories. The model was evaluated using a geographical region as a test case, with successful results.

Aim: To assess the cost–effectiveness of onabotulinumtoxinA (onabotA), implantable sacral nerve stimulation devices, percutaneous tibial nerve stimulation, anticholinergic medications and mirabegron compared with best supportive care (BSC) for management of refractory overactive bladder (OAB). Methods: A Markov model was developed to compare the cost–effectiveness of treatment options with BSC over a 10-year time horizon. Resource utilization, discontinuation rates and costs were derived from unpublished and published sources. Quality-adjusted life-years (QALYs) and incremental cost–effectiveness ratios were reported. Results: Treatment with onabotA 100U produced the largest gain in QALYs (7.179) and lowest estimated incremental cost–effectiveness ratio ($32,680/QALY) of all assessed treatments compared with BSC. Conclusion: Compared with BSC, onabotA 100U was the most cost-effective treatment option for patients with refractory OAB.

SummaryModern legislation in the UK addressing the issue of decision-making ability uses tests of mental capacity based on the individual's ability to understand relevant information given to them. Alternative models of mental capacity do exist, but are largely considered defunct. This article reviews these alternative models and considers their importance. Far from being irrelevant to modern views on mental capacity, these models have already been incorporated into legislation such as the Mental Capacity Act 2005. A better understanding of these models can improve clinicians’ understanding of mental capacity in general. Modern controversies such as the impact of the United Nations Convention on the Rights of Persons with Disabilities (CRPD) are discussed and ways in which our understanding of mental capacity may have to change in the future are addressed.LEARNING OBJECTIVES•Appreciate the different ways of considering an individual's mental capacity•Understand the primacy and limitations of the functional test of mental capacity•Use this understanding to manage potential conflicts or disagreements when considering an individual's mental capacity

Objective: Inhalation injury is an independent risk factor for mortality in burn patients. The purpose of this study was to observe the effect of inhaled iloprost on gas exchange in patients with inhalation injury and acute respiratory failure as measured by an improved PaO2/FiO2 ratio. Methods: Patients admitted to the burn intensive care unit from 2013 to 2014 meeting Berlin criteria for acute respiratory distress syndrome (ARDS) with a diagnosis of inhalation injury and who received inhaled iloprost were included. Medical records were reviewed to collect patient demographics, characterize iloprost prescribing practices, and observe changes in oxygenation and hemodynamic status after iloprost administration. Differences were evaluated using a t-test with cluster corrected standard errors. Results: A total of eight patients were included with 157 different PaO2/FiO2 ratios calculated. All patients had moderate or severe ARDS with a baseline PaO2/FiO2 ratio of 131.9 mmHg (IQR 119.3–197.3). Median duration of iloprost therapy was 5 days (IQR ± 7). A statistically significant increase in PaO2/FiO2 ratio was observed after iloprost administration with a mean increase of 9.7 mmHg (95% CI 1.8–17.7, p = 0.023). Inhaled iloprost had no effect on hemodynamic parameters. Conclusions: Inhaled iloprost for inhalation injury and ARDS after burn injury was associated with a small but statistically significant improvement in oxygenation. Keywords: Iloprost, Inhaled prostacyclin, Inhalation injury

We show that spironolactone use was associated with an increased rate of all-cause hospitalizations, but no difference in hospitalizations for heart failure or pulmonary arterial hypertension, in patients with World Health Organization Group 1 pulmonary arterial hypertension. A possible reason for this finding is confounding from retrospective study design.

Provision of specialist mental health services for older adults (previously referred to as ‘psychogeriatrics’) is recommended by the World Health Organization. In the UK, this extends to specialist wards for older inpatients with mental health problems. This chapter explores the rationale and provision of specialist older adult mental health beds in the UK and the different types of service offered. Beyond the macro level, it aims to provide a handbook for anyone wishing to set up such a ward from scratch: it explores what a ward designed with older adults in mind would look like (design) and what resources and staffing are necessary. The needs specific to this demographic of the population are explored, including issues such as physical health, frailty, and the most common mental health conditions one might encounter on such as ward (dementia, late-life depression, and late-life psychosis).

During pandemics and global crises, drug shortages become critical as a result of increased demand, shortages in personnel and lockdown restrictions that disrupt the supply chain. The pharmaceutical industry is therefore moving towards continuous manufacturing instead of conventional batch manufacturing involving numerous steps, that normally occur at different sites. In order to validate the use of large-scale industrial processes, feasibility studies need to be performed using small-scale laboratory equipment. To that end, the scale-up of a continuous process and its effect on the critical quality attributes (CQAs) of the end product were investigated in this work. Hydroxychloroquine Sulphate (HCQS) was used as the model drug, Soluplus® as a model polymeric carrier and both horizontal and vertical twin screw extruders used to undertake this hot melt extrusion (HME) study. Seven formulations were processed using a small-scale horizontal extruder and a pilot-scale vertical extruder at various drug loadings, temperature profiles and screw speeds. When utilising a horizontal extruder, formulations with the highest drug load and processed at the lowest screw speed and temperature had the highest crystallinity with higher drug release rates. Upon scale-up to a vertical extruder, the crystallinity of the HCQS was significantly reduced, with less variation in both crystallinity and release profile across the different extrudates. This study demonstrates improved robustness with the pilot-scale vertical extruder compared to lab-scale horizontal extruder. The reduced variation with the vertical extruder will allow for short increases in production rate, with minimum impact on the CQAs of the final product enabling high-performance continuous manufacturing with minimum waste of raw materials. Finally, this research provides valuable information for the pharmaceutical industry in accessing continuous technologies for the manufacture of pharmaceutical products, allowing for efficient utilisation of resources upon scale-up and mass production during global pandemics and drug shortages.

Autosomal recessive hereditary spastic paraparesis is rare.We present 4 patients with slowly progressive predominantly lower limb spasticity and ataxia. Only one patient had family history of ataxia but without any underlying diagnosis. All of them proved negative for the mutation of Spinocerebelalr ataxia genes SCA 1,2,3 and 6. All had mutation in the SPG 7 gene suggestive of autosomal recessive hereditary spastic paraparesis. One of the heterozygous mutatnts showed a novel c1617delC ,p(Val540fs) frameshift mutation in exon 12 of the SPG 7 gene. SPG7 mutation accounts for 1.5-7% of all the HSP but it is the cause of undiagnosed ataxia in 18.6% in a recent case series. SPG7 mutation should be remembered as an important cause of undiagnosed ataxia especially where next generation sequencing is not widely avaialbale or affordable.

INTRODUCTION: Early commentary on SARS-CoV-2 infection proposed a mechanism of cytokine release syndrome (CRS) to explain severe acute respiratory failure associated with COVID-19 Management strategies have included targeted immunomodulation with biologic agents The role of IL-6 and other cytokines in the pathogenesis of COVID-19 is not well defined Evidence for use of immunomodulators has been mixed and these agents may expose patients to harm This study aims to characterize the expression of cytokines and their association with inflammatory biomarkers and outcomes in critically ill patients with COVID-19 METHODS: This was a single-center, retrospective analysis of critically ill patients with COVID-19 confirmed by PCR Patients were included if they had a partial or full cytokine panel drawn while admitted Descriptive statistics were used to assess demographics, outcomes, and relationships between cytokine levels and inflammatory markers The Mann- Whitney U Test was used to compare IL-6 levels between survivors and nonsurvivors RESULTS: Eighty-nine patients were included with 68 full cytokine panels and 108 IL-6 levels Patients had a mean (range) of 10 ventilator-days (0-47), 15 ICU-days (1-60), 20 hospital-days (3-69) and a mortality rate of 31% Cytokine levels were assessed a median of 10 days from symptom onset and 1 day from ICU admission Levels of IL-1B, IL-2, IL-4, IL-5, IL-8, IL-12, IL-13, IL-17, IFN-G, and TNF-A were undetectable in at least 80% of patients, and expression did not correlate with other inflammatory biomarkers (CRP, ferritin), severity of illness (SOFA), or outcome IL-2R levels were numerically elevated in most patients (n=68;median 1227, range: 76-30670) IL-6 levels were mildly elevated (n=108;median: 31, range: 2-882, SD: 150), and levels were statistically significantly higher in nonsurvivors (p = 0 002) CONCLUSIONS: Assessment of cytokine levels in critically ill patients with COVID-19 does not support a hypothesis of CRS While IL-6 levels were numerically higher in nonsurvivors, the clinical significance of this finding is unknown The role of IL-2R in the pathogenesis of COVID-19 remains unclear Larger studies exploring the role of inflammatory mediators in pathogenesis and targeted immunomudulators in management of COVID-19 are warranted

INTRODUCTION: Severe acute respiratory failure is a common complication of COVID-19, with refractory hypoxemia being a hallmark finding in severe illness and a common cause of mortality With limited therapeutic strategies, management centers on good supportive care Prone positioning has been shown to improve oxygenation and survival in patients with moderate-to-severe acute respiratory distress syndrome (ARDS) but the impact of prone positioning in COVID-19 with severe hypoxemia is unknown This study aims to examine the response to proning as a predictor of COVID-19 related mortality METHODS: This is a single-center, retrospective analysis of critically ill patients with COVID-19 confirmed by PCR Patients were included if they were invasively ventilated, and if supportive care included prone positioning for management of refractory hypoxemia Data points collected include demographics, ventilator settings, rates of mortality and progression to ECMO, ventilator-days, and time between symptom onset and intubation, hospital and ICU admission Endpoints included response in oxygenation (PaO2:FiO2) and mortality RESULTS: Forty-nine patients were included in the analysis The average age was 56 9, and 61% of the patients were male Patients had an average of 19 ventilator-days (2-52), 21 ICU-days (4-54), 26 hospital-days (8-65), an ECMO rate of 27%, and a mortality rate of 55% Of the 22 survivors, there was an average increase in PaO2:FiO2 by 108, 93 1, and 93 for each of the first three pronations respectively For the 27 nonsurvivors, there was an average increase in PaO2:FiO2 by 76 1, 84 3, and 50 9 for the first three pronations The difference in improvement in PaO2:FiO2 was not statistically significant between survivors and non-survivors There was no inflection point that could be determined that provided a high sensitivity and specificity to predict mortality or need for ECMO based on response to pronation at any of the time points CONCLUSIONS: Proning improves PaO2:FiO2 in patients with severe hypoxemia related to COVID-19 Survivors in our study had a numerically greater response to proning, but this finding was not statistically significant The clinical significance remains unclear Larger studies assessing the efficacy of proning in critically ill patients with COVID-19 are needed

A 31 year-old man with a history of a depressive episode presented with acute severe ‘thunderclap’ headache. Magnetic resonance imaging (MRI) revealed abnormalities typical of cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL), which was subsequently confirmed by genetic analysis. The psychiatric features of this genetic cause of depression and headache are discussed.

Previous randomized controlled trials have reported a 6.1-6.9% incidence of clean intermittent catheterization (CIC) following treatment with onabotulinumtoxinA in non-neurogenic overactive bladder (OAB) patients who were inadequately managed by ≥1 anticholinergic. A multi-center retrospective chart review assessed the real-world rate of voiding dysfunction requiring catheterization.Patients received onabotulinumtoxinA 100 U (approved dose) administered by experienced injectors between January 2013 and June 2015. Patients using CIC or an indwelling catheter for ≥24 hours for voiding dysfunction prior to onabotulinumtoxinA injections were excluded. The primary outcome was post-treatment CIC (lasting24 hours; per individual physician's clinical judgment considering patient's voiding symptoms, post-void residual [PVR] urine volumes and patient bother). Potential baseline predictors of CIC (history of pelvic prolapse, cystocele, diabetes, PVR urine volume and age) were assessed using multivariable logistic regression.Overall, 299 patients received their first treatment with onabotulinumtoxinA 100 U. Mean age was 66.4 years; 98.3% were female. The incidence of CIC was 2.7% in the total study population after the first treatment with onabotulinumtoxinA. The de novo CIC rate in treatments 2 and 3 combined was similarly low (3.2%). None of the evaluated baseline characteristics were significant predictors of CIC initiation due to the low CIC incidence.This real-world study of non-neurogenic OAB patients treated with onabotulinumtoxinA suggests that the CIC rate is lower than the rates reported in previous studies. There were no significant correlations between baseline predictors and CIC initiation, although statistical significance may not have been reached because of the low incidence of CIC.

Novel low power silicon photonic PAM-4 transmitter and receiver, both operating at 15 Gbaud and with a combined power consumption of 10.1 pJ/Bit are demonstrated in an optical link with up to 40 km of SSMF using electronic dispersion compensation.

Acquired von Willebrand disease increases bleeding risk in patients implanted with a continuous-flow left ventricular assist device. Lower aspirin (ASA) doses decrease the risk of bleeding without an increased risk of embolic events. No published studies in the United States have compared the incidence of bleeding and thrombotic events between antithrombotic regimens with and without ASA. A single-center, retrospective analysis was conducted of adult patients implanted with a HeartMate II (HM II). Patients received warfarin and ASA 81 mg daily or warfarin alone. The primary end-point was a composite of death, bleeding events, and thrombotic events from the date of HM II implantation to first event or 18 months. Secondary end-points included the individual components of the primary end-point and the proportion of patients alive with HM II or transplanted. The Wilcoxon rank sum test and Fisher's exact test were used for statistical analysis. Of the 76 patients meeting inclusion criteria, 44 received warfarin and ASA and 32 received warfarin alone. Baseline characteristics were similar between groups. Warfarin alone was not associated with an increased risk of the primary composite outcome (53 vs. 59%, respectively, p = 0.64). No significant difference was observed in any bleeding event (34 vs. 43%, respectively, p = 0.48) nor any thrombotic event (9 vs. 11%, respectively, p = 1.00) with warfarin alone compared with warfarin and ASA. Elimination of antiplatelet therapy from the HM II antithrombotic regimen was associated with no significant difference in the composite outcome of bleeding events, thrombotic events, or death, nor the individual components of each end-point.

Rolling element bearings are widely used in almost all complex engineering structures. Failure of bearings could lead to a failure of the whole structure. Therefore, early damage diagnosis of bearings is of great importance for the structural integrity of the complex engineering structures. The novel nonlinear non-stationary wavelet bicoherence technology is applied for the first time in worldwide terms for rolling bearing condition monitoring. High effectiveness of early bearing diagnostics by the technology has been experimentally revealed, which has been further substantiated using the Fisher criterion.

Background: Ipilimumab has been shown to improve overall survival in patients with metastatic melanoma; however, complete responses (CRs) are uncommon. Immune-related side effects usually involve the skin or gastrointestinal tract. Neurologic events occur less frequently but are well described. Case Report: We report the case of a 58-year-old man with metastatic melanoma who commenced ipilimumab post spinal decompression and radiation. He developed a colitis post cycle 2 and ipilimumab was discontinued. Imaging, however, documented a radiological CR. 8 weeks later, he developed paraplegia and a myelitis despite an ongoing radiological CR. Steroid use resulted in some improvement radiologically, without clinical improvement. Conclusion: We report myelitis with consequent paraplegia as a potential neurological immune-related side effect of ipilimumab. We further describe a patient with a CR after 2 cycles of ipilimumab in the setting of radiation.

Background: Partial thrombosis of the false lumen has been reported as a significant predictor of mortality during follow-up in patients with acute type B aortic dissection. The purpose of this study was to investigate the correlation of false lumen thrombosis and aortic expansion during follow-up in patients with acute type B aortic dissection. Methods: All medically treated patients with acute type B aortic dissection observed in 4 cardiovascular referral centers between 1998 and 2011, with admission and follow-up computed tomography or magnetic resonance imaging scans, were included. Aortic diameters of the dissected aortas were measured at 4 levels on the baseline and follow-up scans, and annual growth rates were calculated. Univariate and multivariate regression analyses were used to investigate the effect of false lumen thrombosis on aortic growth rate. Results: A total of 84 patients were included, of whom 40 (47.6%) had a partially thrombosed false lumen, 7 (8.3%) had a completely thrombosed false lumen, and 37 (44.0%) had a patent false lumen. A total of 273 of the 336 (81.3%) evaluated aortic levels were dissected segments. Overall, the mean aortic diameter increased significantly at all evaluated levels (P

A novel diagnosis technology combining the benefits of spectral kurtosis and wavelet transform is proposed and validated for early defect diagnosis of rolling element bearings. A systematic procedure for feature calculation is proposed and rules for selection of technology parameters are explained. Experimental validation of the proposed method carried out for early detection of the inner race defect. A comparison between frequency band selection through wavelets and spectral kurtosis is also presented. It has been observed that the frequency band selected using spectral kurtosis provide better separation between healthy and defective bearings compared to the frequency band selection using wavelet. In terms of Fisher criterion the use of spectral kurtosis has a gain of 2.75 times compared to the wavelet.

INTRODUCTION: Emerging evidence from epidemiologic studies suggests living near major roads may be linked to cognitive decline. Little is known about its relationship with dementia incidence, and e...

Keywords: caudate and putamen; collapsin response mediator protein 5 (CRMP5); 18-fluorodeoxyglucose positron emission tomography (18FDG PET) brain; paraneoplastic Parkinsonism

Frontotemporal dementia (FTD) is a neurodegenerative disorer characterised by circumscribed and often asymmetric lobar trophy of the frontal parts of the brain. In some cases it can be assoiated with amyotrophic lateral sclerosis [1], although there are iverse clinical manifestations. FTD accounts for as much as 25% of resenile dementias [2]. Some patients with FTD can develop new, r enhanced nascent artistic skills [3], despite progressive demenia, however not all patients have prior artistic training and typically ost have the semantic dementia subtype.

To explore possible causes of the poor survival of Scottish lung cancer patients, a retrospective registry-based audit was conducted comparing demography, treatment and survival of 3833 Scottish patients and 2073 from British Columbia (BC). Patients from Scotland were older, had a lower rate of pathological confirmation (74% vs 89%, p

Many have noted the lack of human resource management research on employee benefits, which is surprising because employer-sponsored benefits are a primary concern of executives and employees alike. Moreover, of special interest to scholars, benefits provide a unique opportunity to examine fundamental theoretical and empirical questions about employee behavior and contemporary employment relationships. This paper provides a foundation for such research by providing an overview of the context from which U.S. employer-provided benefit programs evolved and the contemporary state of benefits research in human resource management. Propositions related to primary employee benefit research are provided.

IntroductionLung cancer survival in Scotland has historically been poor but many changes to the lung cancer services have been introduced and this study was conducted to investigate the impact of these changes on treatment and survival.MethodsData obtained from the Scottish Cancer Registry, South-East Scotland Cancer Network audit and Edinburgh Cancer Centre database were used to conduct a comparison of the management and outcomes of lung cancer patients from South-East Scotland diagnosed in 1995 and in 2002.ResultsData on 971 patients diagnosed in 2002 and 927 in 1995 were analyzed and demonstrated that though the use of treatment overall had not changed (62% in 2002 versus 63% in 1995) the use of potentially curative radiotherapy (15 versus 5% χ2 p < 0.001) and chemotherapy for non-small cell lung cancer (18 versus 7% χ2 p < 0.001) had increased, but not resection rates (11 versus 10%). The use of palliative radiotherapy declined (38% versus 31% χ2 p < 0.001). Patients diagnosed in 2002 had an adjusted hazard of death of 0.7 (95% confidence interval, 0.6–0.8) compared with 1995, with median survival from date of diagnosis of 5.2 versus 4.1 month and 2 year overall survival 15 versus 11% (log rank p = 0.004). Localized disease and younger age were also associated with a reduced hazard of death.ConclusionsPatients diagnosed with lung cancer in Scotland in 2002 had a reduced hazard of death and improved survival compared with 1995. It is hypothesized that this was due in part to improvements in service organization and increased use of treatments likely to increase survival.

PurposeThe purpose of this paper is to contribute to the development of a conceptual model of service quality in airports by conducting an empirical investigation into passengers' expectations for this service industry.Design/methodology/approachThe paper is a qualitative exploration of the airport experience from the passengers' perspective was combined with a review of relevant literature to identify variables, to clarify basic concepts and to generate a conceptual model of airport service quality expectations. The paper's quantitative research was used to develop a self‐report scale to measure passenger expectations of airport service quality, to test dimensionality and to evaluate scale reliability and validity.FindingsQualitative and quantitative research on nearly 1,000 airport users provided results suggesting that passengers' expectations of airport service quality is a multidimensional, hierarchical construct that includes three key dimensions: function, interaction and diversion.Research limitations/implicationsBy bringing together different literatures and research paradigms to conceptualize service quality in a novel environment, the study contributes to the ongoing extension of service quality research. It is limited insofar as efforts to define a global expectations construct may have “homogenized” results.Practical implicationsThis paper builds on the extant literature on service quality to propose an approach for measuring passengers' expectations of airport service quality that can serve as a foundation of a concise and easy‐to‐administer self‐report measure for identifying and managing airport service quality strategies.Originality/valueThe paper shows that by going beyond traditional service performance measures used in the airport industry and by introducing new variables to the service quality literature, such as Csikszentmihalyi's taxonomy of activity, this study broadens and enriches both practice and theory.

Higher education employees often participate in university-sponsored defined contribution pension plans that place the investment decision responsibility upon them. In order to examine investment decision-making behavior with retirement savings plans we investigated attitude-mediated, individual difference determinants of risky decision-making behavior in employer-sponsored defined contribution retirement plans. Using a field survey of 795 higher education employees, who participated in one of four defined contribution savings plans, we tested a risk taking model using data on the employees’ investment allocations in their defined contribution savings plan. We found that an attitudinal preference toward risk and a perception of opportunity served as mediators for the relationships among employees’ dispositional characteristics and their retirement savings behavior. We also found that investment knowledge directly affected risk taking behavior. Implications of the results for higher education institutions are discussed.

Stiff person syndrome (SPS) is an unusual cause of muscle rigidity and spasms. It is believed to have an autoimmune pathogenesis and is associated with autoantibodies to glutamic acid decarboxylase (GAD). Paraneoplastic SPS (PSPS) has been described mainly in relation to breast cancer and is associated with antibodies to amphiphysin. Few reports of PSPS document the finding of GAD autoantibodies. We present the first reported case of anti-GAD positive PSPS in a 53-year-old male with occult renal carcinoma. Clinical benefit was marked following nephrectomy and intravenous immunoglobulin treatment. Renal carcinoma should be considered in patients with SPS.

A 37-year-old female patient was referred to the hospital with pelvic pain and progressive lower limb weakness of 6 months’ duration. This weakness began during the final trimester of her pregnancy and had continued after the birth of her child. There was no associated sphincter disturbance. On questioning, the patient reported experiencing some right lower extremity weakness approximately 12 months previously, for which no cause was found despite normal brain magnetic resonance imaging and cervical spine imaging. Physical examination revealed positive Babinski signs and significant reduced power of hip flexion bilaterally, with no loss of power detected in other lower limb muscle groups. A sensory level was found at the T7 and T8 levels. A 3 Tesla magnetic resonance imaging examination of the cervical and thoracic spine, with and without contrast, was performed (Figs. 1–2). This demonstrated an avidly enhancing tumor mass in the posterior epidural space, extending craniocaudally from the level of T6 to T9 for a distance of 7 cm. Enhancing epidural soft tissue was detected, extending into the left T7–T8 neural exit foramen. There was spinal cord compression, most marked at T7 and T8. The tumor mass was surgically resected. Histopathologic evaluation confirmed a spinal angiolipoma (Figs. 3–4). Spinal angiolipoma accounts for 0.04%–1.2% of all spinal axis tumors. It predominantly occurs in the mid-thoracic region. Pregnancy is an aggravating factor in some patients. Prognosis post-resection is excellent [1].

Antibodies to voltage gated potassium channels (VGKC) can result in a reversible form of encephalopathy [1]. Since first described, the clinical phenotype of patients with antibodies to VGKC continues to expand [2]. To our knowledge this is the first reported case of VGKC encephalopathy mimicking a stroke resulting in misdiagnosis. A 73 year old woman presented with aphasia and right upper limb weakness, 5 days post-carotid endarterectomy for an asymptomatic 90% left carotid stenosis. She had experienced atrial fibrillation (warfarin had not yet been reintroduced), hypertension, diabetes and was an ex-smoker. A CT brain done within 12 h of presentation did not show any evidence of infarction (Fig. 1). Diffusion weighted (DWI) magnetic resonance imaging (MRI) of the brain, done 2 days after presentation, demonstrated an increased signal in the left temporal lobe in the region of the hippocampus, amygdala and extending to the pulvinar region of the thalamus (Fig. 2). Restricted diffusion was seen on apparent diffusion coefficient (ADC) in the same territory, consistent with a thromboembolic infarct. A diagnosis of thromboembolic event to the left posterior cerebral artery (PCA) was made and the patient was transferred to the acute stroke unit. Warfarin was restarted and, following improvement over a month, she was transferred to a rehabilitation facility. However, after 2 weeks the patient developed visual hallucinations and was re-admitted. On arrival she had three convulsive seizures and was treated with intravenous phenytoin. A repeat MRI brain was performed (Fig. 3), 2 months from initial presentation, again showing high signal on DWI and corresponding restricted diffusion on ADC map in the same distribution as the initial imaging. Following review of the MRI imaging, the lesions were deemed to be inconsistent with a PCA infarct. Cerebrospinal fluid protein was normal and an electroencephalogram showed delta slowing in the left temporal region and intermittent sharp waves. A whole body fluorodeoxyglucose positron emission tomography (FDG–PET) scan did not show any evidence of malignancy. Voltage gated potassium channels (VGKC) levels were 1,726 pico Moles (normal \100 pM), paraneoplastic antibodies were negative. A diagnosis of autoimmune limbic encephalitis (LE) was made, as described with VGKC antibodies [1–4]. The patient was treated with methylprednisolone with an improvement in both receptive and expressive components of language. Repeat antibodies were unrecordable following initial treatment and she was discharged on oral prednisolone. She was re-admitted on two further occasions while on oral steroids. On each recurrent admission, she received methylprednisolone with a corresponding decrease of VGKC antibodies. Mycophenolate mofetil was added when a level of 2,026 pM was recorded. A wide clinical phenotype is seen in VGKC autoimmunity [2] including: Isaacs syndrome [5], Morvan H. Kearney B. Murray K. O’Rourke P. Kelly T. Lynch (&) The Dublin Neurological Institute at the Mater Misericordiae University Hospital, 57 Eccles Street, Dublin 7, Ireland e-mail: Tlynch@mater.ie URL: http://www.neurologicalinstitute.ie

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