The First Case Report of Schnitzler Syndrome Presenting with Eye Pain

ABSTRACT

Schnitzler syndrome is a rare, auto inflammatory condition known to manifest with bone pain, urticarial rash, fevers, relapsing arthralgia, and fatigue. In this case report, we describe a patient who was diagnosed with Schnitzler Syndrome that had initially presented with a unilateral pressure-type headache with a sensation of a ‘dagger’ stabbing into the back of the eye. He also had an associated ipsilateral redness of the conjunctiva, eyelid swelling, subtle optic disc elevations bilaterally and facial flushing - but with no visual acuity, pupillary, or lacrimatory changes. Anterior segment, fundoscopy, intraocular pressures and extraocular muscle movements were otherwise normal.     

Pituitary Adenylate Cyclase-Activating Polypeptide Attenuates Brain Edema by Protecting Blood–Brain Barrier and Glymphatic System After Subarachnoid Hemorrhage in Rats

Brain edema is a vital contributor to early brain injury after subarachnoid hemorrhage (SAH), which is responsible for prolonged hospitalization and poor outcomes. Pharmacological therapeutic targets on edema formation have been the focus of research for decades. Pituitary adenylate cyclase-activating polypeptide (PACAP) has been shown to participate in neural development and brain injury. Here, we used PACAP knockout CRISPR to demonstrate that endogenous PACAP plays an endogenous neuroprotective role against brain edema formation after SAH in rats. The exogenous PACAP treatment provided both short- and long-term neurological benefits by preserving the function of the blood–brain barrier and glymphatic system after SAH. Pretreatment of inhibitors of PACAP receptors showed that the PACAP-involved anti-edema effect and neuroprotection after SAH was facilitated by the selective PACAP receptor (PAC1). Further administration of adenylyl cyclase (AC) inhibitor and sulfonylurea receptor 1 (SUR1) CRISPR activator suggested that the AC–cyclic adenosine monophosphate (cAMP)–protein kinase A (PKA) axis participated in PACAP signaling after SAH, which inhibited the expression of edema-related proteins, SUR1 and aquaporin-4 (AQP4), through SUR1 phosphorylation. Thus, PACAP may serve as a potential clinical treatment to alleviate brain edema in patients with SAH.Electronic supplementary materialThe online version of this article (10.1007/s13311-020-00925-3) contains supplementary material, which is available to authorized users.Key Words: Subarachnoid hemorrhage, brain edema, pituitary adenylate cyclase-activating polypeptide, blood–brain barrier, glymphatic system     

Mind the gap: Teachers’ conceptions of student-staff partnership and its potential to enhance educational quality

Abstract

Introduction: Student-staff partnerships as a concept to improve medical education have received a growing amount of attention. Such partnerships are collaborations in which students and teachers seek to improve education by each adding their unique contribution to decision-making and implementation processes. Although previous research has demonstrated that students are favourable to this concept, teachers remain hesitant. The present study investigated teachers’ conceptions of student-staff partnerships and of the prerequisites that are necessary to render such partnerships successful and enhance educational quality.

Method: We conducted semi-structured interviews with 14 course coordinators who lead course design teams and also teach in 4 bachelor health programmes, using Bovill and Bulley’s levels of student participation as sensitising concepts during data analysis.

Results: The results pointed to three different conceptions of student-staff partnerships existing among teachers: Teachers teach and students study; teachers teach and value students’ feedback; and teachers and students co-create. The prerequisites for effective co-creation teachers identified were: Teachers must be open to involve students and create dialogues; students must be motivated and have good communication skills; the organisation must be supportive; and teachers should have the final say.

Conclusion: We conclude that teachers’ conceptions are consistent with Bovill and Bulley’s levels of student participation. Under certain conditions, teachers are willing to co-create and reach the highest levels of student participation.     

Barriers and facilitators for cascade testing in genetic conditions: a systematic review

Cascade testing is the process of offering genetic counseling and testing to at-risk relatives of an individual who has been diagnosed with a genetic condition. It is critical for increasing the identification rates of individuals with these conditions and the uptake of appropriate preventive health services. The process of cascade testing is highly varied in clinical practice, and a comprehensive understanding of factors that hinder or enhance its implementation is necessary to improve this process. We conducted a systematic review to identify barriers and facilitators for cascade testing and searched PubMed, CINAHL via EBSCO, Web of Science, EMBASE, and the Cochrane Library for articles published from the databases’ inception to November 2018. Thirty articles met inclusion criteria. Barriers and facilitators identified from these studies at the individual-level were organized into the following categories: (1) demographics, (2) knowledge, (3) attitudes, beliefs, and emotional responses of the individual, and (4) perceptions of relatives, relatives’ responses, and attitudes toward relatives. At the interpersonal-level, barriers and facilitators were categorized as (1) family communication-, support- and dynamics-, and (2) provider-factors. Finally, barriers at the environmental-level relating to accessibility of genetic services were also identified. Our findings suggest that several individual, interpersonal and environmental factors may play a role in cascade testing. Future studies to further investigate these barriers and facilitators are needed to inform future interventions for improving the implementation of cascade testing for genetic conditions in clinical practice.Subject terms: Preventive medicine, Genetic services     

Shared decision-making for the management of small renal masses: Development and acceptability testing of a novel patient decision aid

IntroductionShared decision-making incorporates patients’ values and preferences to achieve high-quality decisions. The objective of this study was to develop an acceptable patient decision aid to facilitate shared decision-making for the management of small renal masses (SRMs).MethodsThe International Patient Decision Aids Standards were used to guide an evidence-based development process. Management options included active surveillance, thermal ablation, partial nephrectomy, and radical nephrectomy. A literature review was performed to provide incidence rates for outcomes of each option. Once a prototype was complete, alpha-testing was performed using a 10-question survey to assess acceptability with patients, patient advocates, urologists, and methodological experts. The primary outcome was acceptability of the decision aid.ResultsA novel patient decision aid was created to facilitate shared decision-making for the management of SRMs. Acceptability testing was performed with 20 patients, 10 urologists, two patient advocates, and one methodological expert. Responders indicated the decision aid was appropriate in length (82%, 27/33), well-balanced (82%, 27/33), and had language that was easy to follow (94%, 31/33). All patient responders felt the decision aid would have been helpful during their consultation and would recommend the decision aid for future patients (100%, 20/20). Most urologists reported they intend to use the decision aid (90%, 9/10).ConclusionsA novel patient decision aid was created to facilitate shared decision-making for management of SRMs. This clinical tool was acceptable with patients, patient advocates, and urologists and is freely available at: https://decisionaid.ohri.ca/decaids.html.     

An uncertainty principle for neural coding: Conjugate representations of position and velocity are mapped onto firing rates and co‐firing rates of neural spike trains

The hippocampal system contains neural populations that encode an animal's position and velocity as it navigates through space. Here, we show that such populations can embed two codes within their spike trains: a firing rate code ( R ) conveyed by within‐cell spike intervals, and a co‐firing rate code () conveyed by between‐cell spike intervals. These two codes behave as conjugates of one another, obeying an analog of the uncertainty principle from physics: information conveyed in R comes at the expense of information in , and vice versa. An exception to this trade‐off occurs when spike trains encode a pair of conjugate variables, such as position and velocity, which do not compete for capacity across R and . To illustrate this, we describe two biologically inspired methods for decoding R and , referred to as sigma and sigma‐chi decoding, respectively. Simulations of head direction and grid cells show that if firing rates are tuned for position (but not velocity), then position is recovered by sigma decoding, whereas velocity is recovered by sigma‐chi decoding. Conversely, simulations of oscillatory interference among theta‐modulated “speed cells” show that if co‐firing rates are tuned for position (but not velocity), then position is recovered by sigma‐chi decoding, whereas velocity is recovered by sigma decoding. Between these two extremes, information about both variables can be distributed across both channels, and partially recovered by both decoders. These results suggest that populations with different spatial and temporal tuning properties—such as speed versus grid cells—might not encode different information, but rather, distribute similar information about position and velocity in different ways across R and . Such conjugate coding of position and velocity may influence how hippocampal populations are interconnected to form functional circuits, and how biological neurons integrate their inputs to decode information from firing rates and spike correlations.     

Managing invasive aspergillosis in haematological patients in the era of resistance polymerase chain reaction and increasing triazole resistance: A modelling study of different strategies

Objectives

Triazole resistance in Aspergillus spp. is emerging and complicates prophylaxis and treatment of invasive aspergillosis (IA) worldwide. New polymerase chain reaction (PCR) tests on broncho-alveolar lavage (BAL) fluid allow for detection of triazole resistance at a genetic level, which has opened up new possibilities for targeted therapy. In the absence of clinical trials, a modelling study delivers estimates of the added value of resistance detection with PCR, and which empiric therapy would be optimal when local resistance rates are known.

A second wave of COVID-19 in Cook County: What lessons can be applied?

During the ongoing public health crisis, many agencies are reporting COVID-19 health outcome information based on the overall population. This practice can lead to misleading results and underestimation of high risk areas. To gain a better understanding of spatial and temporal distribution of COVID-19 deaths; the long term care facility (LTCF) and household population (HP) deaths must be used. This approach allows us to better discern high risk areas and provides policy makers with reliable information for community engagement and mitigation strategies. By focusing on high-risk LTCFs and residential areas, protective measures can be implemented to minimize COVID-19 spread and subsequent mortality.  These areas should be a high priority target when COVID-19 vaccines become available

During the current public health crisis, many agencies and media outlets are reporting COVID-19 health outcome information based on the overall population of Cook County. As we have demonstrated, overall COVID-19 case counts and mortality can be misleading (details in >Story Map 1). Moreover, they offer little guidance for delivering public health interventions to high risk populations, a critical need during this second and potentially more devastating wave of the pandemic. The University of Illinois Chicago School of Public Health’s Public Health Geographic Information System Program (UIC-SPH-PHGIS) and Purdue research team has been examining spatial and temporal patterns of COVID-19 mortality with a focus on the significant loss of life from COVID-19 among Long-Term Care Facility (LTCF) residents in contrast to mortality in the community among residents of private households (non-LTCF; referred to as household population, HP). The goals of the study are:

• Improve the accuracy of commonly quoted COVID-19 mortality indicators;
• Gain a better understanding of spatial and temporal distribution of COVID-19 deaths;
• Examine the role of race, ethnicity, and socioeconomic status in COVID-19 mortality;
• Identify population and organizational parameters that can inform strategies for public health interventions.

Prioritizing the allocation of resources based on reliable information is a prerequisite of a successful mitigation strategy and immunization plan. Findings from our research have significant practical implications. The state and federal government face a series of policy decisions both due to the recent surge in positive cases and, when the time comes, the need to rationalize distribution of vaccines to high priority groups beyond healthcare workers and nursing home residents in critical areas. The research team seeks to modify prevailing practices in order to derive reliable information that guides policy decisions. At this stage of the study, we identified high-risk LTCFs and residential areas (HP) of Cook County from readily available, real-time mortality data.     

Immunoblot and Polymerase Chain Reaction to Diagnose Ocular Syphilis and Neurosyphilis in HIV-positive and HIV-negative Patients

ABSTRACT

Purpose: To evaluate immunoblot (IB) and polymerase chain reaction (PCR) to diagnose ocular- and neurosyphilis.

Methods: Prospective cross-sectional study. Aqueous humor (AH) and cerebrospinal fluid (CSF) samples were tested for treponemal DNA or antibodies to treponemal antigens.

Results: Thirteen of 106 cases had positive syphilis serology of which 69.2% were HIV+ (median CD4+ = 181 cells/µL). Four cases met CDC criteria for neurosyphilis (3 confirmed, 1 probable) and 2 additional cases required neurosyphilis treatment according to UpToDate algorithms. All AH and CSF samples tested PCR negative. Five cases were CSF IB+ and 3 cases AH IB+. Using our classification, eight patients had confirmed neurosyphilis, one had probable neurosyphilis, three had confirmed ocular syphilis and nine had probable ocular syphilis.

Conclusion: Our findings suggest that IB of AH and CSF provides additional evidence to diagnose ocular and neurosyphilis and allows us to classify them as probable or confirmed.