Racemose neurocysticercosis.

BACKGROUND: Neurocysticercosis (NCC) is an invasive parasitic infection of the central nervous system caused by the larval stage of the tapeworm Taenia solium. The clinical manifestations of NCC depend on the parasitic load and location of infection, as well as the developmental stage of the cysticerci and host immune response, with symptoms ranging from subclinical headaches to seizures, cerebrovascular events, and life-threatening hydrocephalus. Racemose NCC represents a particularly severe variant of extraparenchymal NCC characterized by the presence of multiple confluent cysts within the subarachnoid space and is associated with increased morbidity and mortality, as well as a decreased response to treatment. Albendazole is the preferred drug for the treatment of racemose NCC due to its superior cerebrospinal fluid penetration compared to praziquantel and the ability to be used concomitantly with steroids. CASE DESCRIPTION: In this report, we describe a 39-year-old man recently emigrated from Mexico with racemose NCC and hydrocephalus successfully treated with prolonged albendazole treatment, high-dose dexamethasone, and ventriculoperitoneal shunt placement for the relief of obstructive hydrocephalus. CONCLUSIONS: Treatment of racemose NCC represents a significant clinical challenge requiring multimodal intervention to minimize infectious- and treatment-related morbidity. We review the clinical, diagnostic, and therapeutic features relevant to the management of this aggressive form of NCC

A Rare Case of a Systemic Non-Langerhans Histiocytosis Presenting with Diabetes Insipidus and a Tentorial Mass

Introduction The histiocytoses are a group of clinically diverse diseases distinguished from one another based on the specific immunophenotype of the lesional cells, implying derivation from the same precursor cell. Langerhans cell histiocytoses (LCH) diseases stem from abnormal dendritic cell lineages, while the non-Langerhans cell histiocytoses (non-LCH) are usually derived from an abnormal monocyte/macrophage cell line.1 Non-LCH with central nervous system (CNS) involvement is predictive of poor outcome. Histopathology is used to make a diagnosis of non-LCH. Immunohistochemistry and the clinical setting are used to differentiate between the various subtypes of non-LCH.1 The non-LCH can be divided into cutaneous non-LCH, cutaneous with a major systemic component, and systemic non-LCH.1 Erdheim-Chester disease (ECD) and Rosai-Dorfman disease (RDD) are systemic non-LCH diseases. First described in 1930, ECD is characterized by xanthogranulomatous accumulations. The extent of infiltration is heterogeneous and can include skin, bones, lungs, kidneys, and the CNS. Approximately 500 cases have been reported so far.2 The majority of ECD patients harbor an activating mutation of the proto-oncogene BRAF, namely BRAF-V600E.3 Recent studies indicate CNS involvement as a predictor of highest mortality among ECD patients.4 First described in 1969, RDD is characterized by accumulation of histiocytes exhibiting emperipolesis in lymph nodes, in the head and neck or in extranodal sites. Extranodal sites include the CNS, skin, soft tissue and gastrointestinal tract. The clinical presentation is typically painless cervical lymphadenopathy with leukocytosis and a fever.5 The etiology of RDD is unknown.6 RDD with CNS involvement is rare and approximately 210 cases have been reported. CNS involvement typically lacks extracranial lymphadenopathy and resembles meningioma radiologically and clinically. 1 Select cases have demonstrated a combined presentation of ECD and RDD.2 In this report we describe a rare case presenting with headache and with clinically and pathologically overlapping features of RDD and ECD. We describe treatment and complications and review the existing literature regarding diagnosis and treatment for these rare conditions

A district case study : how one school district engaged in sustainable systemic change to include students with significant cognitive disabilities in general education classes

History, trends, and data indicate the harm of segregation for persons with significant cognitive disabilities (SCD; Burton & Blatt; 1966; Nielsen, 2013). One school district in the northeastern part of the United States began facilitating systemic change in 2017. This qualitative case study investigated this school district’s multi-year experience with facilitating the development of inclusive education practices for students with SCD. This case study was developed to: (a) understand how one school district addressed sustainable systemic change related to the inclusion of students with significant cognitive disabilities in general education classes, and (b) understand the impact of those efforts on students, their parents, instructional personnel, and administrators. The Principal Investigator collected qualitative and quantitative data to better understand the experiences of one district engaged in efforts for sustainable systemic change related to the use of inclusive education practices for students with SCD. The results of this case study are organized by Themes (n = 5) and Subthemes (n = 13) that emerged from the documents, data, and interviews collected. This discussion section synthesizes the results of the study to answer the research questions and provides: (a) summary of findings, (b) discussion of findings, (c) implications for future research, policy, and practice, (d) limitations, (e) researcher positionality, and (f) conclusions. Limitations of this study and implications for research, policy, and practice are discussed

Roles for Treg expansion and HMGB1 signaling through the TLR1-2-6 axis in determining the magnitude of the antigen-specific immune response to MVA85A

© 2013 Matsumiya et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are creditedA better understanding of the relationships between vaccine, immunogenicity and protection from disease would greatly facilitate vaccine development. Modified vaccinia virus Ankara expressing antigen 85A (MVA85A) is a novel tuberculosis vaccine candidate designed to enhance responses induced by BCG. Antigen-specific interferon-γ (IFN-γ) production is greatly enhanced by MVA85A, however the variability between healthy individuals is extensive. In this study we have sought to characterize the early changes in gene expression in humans following vaccination with MVA85A and relate these to long-term immunogenicity. Two days post-vaccination, MVA85A induces a strong interferon and inflammatory response. Separating volunteers into high and low responders on the basis of T cell responses to 85A peptides measured during the trial, an expansion of circulating CD4+ CD25+ Foxp3+ cells is seen in low but not high responders. Additionally, high levels of Toll-like Receptor (TLR) 1 on day of vaccination are associated with an increased response to antigen 85A. In a classification model, combined expression levels of TLR1, TICAM2 and CD14 on day of vaccination and CTLA4 and IL2Rα two days post-vaccination can classify high and low responders with over 80% accuracy. Furthermore, administering MVA85A in mice with anti-TLR2 antibodies may abrogate high responses, and neutralising antibodies to TLRs 1, 2 or 6 or HMGB1 decrease CXCL2 production during in vitro stimulation with MVA85A. HMGB1 is released into the supernatant following atimulation with MVA85A and we propose this signal may be the trigger activating the TLR pathway. This study suggests an important role for an endogenous ligand in innate sensing of MVA and demonstrates the importance of pattern recognition receptors and regulatory T cell responses in determining the magnitude of the antigen specific immune response to vaccination with MVA85A in humans.This work was funded by the Wellcome Trust. MM has a Wellcome Trust PhD studentship and HM is a Wellcome Trust Senior Fello

Developing secondary prospective teachers’ ability to respond to student work

© 2018, Springer Nature B.V. This paper highlights the second phase of a multi-university research project focusing on improving secondary prospective mathematics teachers’ abilities to respond to student thinking. This work is based on an Interview Module, created by the authors, which showed gains in prospective teachers’ (PSTs) abilities to attend to and interpret student thinking but did not show the same gains in their abilities to respond to student thinking. The Interview Module originally included a pre–post-video, readings, discussion, and one-on-one interview with a secondary student (Lesseig et al. in Math Teach Educ 5(1):29–46, 2016). This second phase adds a take-home assignment based on solutions from the secondary students who were interviewed in Phase 1. This take-home assignment was designed to specifically improve PSTs’ abilities to respond to student thinking. Results indicate that gains were made in their ability to respond, as was intended, but, unexpectedly, greater gains were also found in their abilities to attend to and interpret student thinking compared with Phase 1

The Rna-Binding Motif 45 (Rbm45) Protein Accumulates In Inclusion Bodies In Amyotrophic Lateral Sclerosis (Als) And Frontotemporal Lobar Degeneration With Tdp-43 Inclusions (Ftld-Tdp) Patients

RNA-binding protein pathology now represents one of the best characterized pathologic features of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration patients with TDP-43 or FUS pathology (FTLD-TDP and FTLD-FUS). Using liquid chromatography tandem mass spectrometry, we identified altered levels of the RNA-binding motif 45 (RBM45) protein in the cerebrospinal fluid (CSF) of ALS patients. This protein contains sequence similarities to TAR DNA-binding protein 43 (TDP-43) and fused-in-sarcoma (FUS) that are contained in cytoplasmic inclusions of ALS and FTLDTDP or FTLD-FUS patients. To further characterize RBM45, we first verified the presence of RBM45 in CSF and spinal cord tissue extracts of ALS patients by immunoblot. We next used immunohistochemistry to examine the subcellular distribution of RBM45 and observed in a punctate staining pattern within nuclei of neurons and glia in the brain and spinal cord. We also detected RBM45 cytoplasmic inclusions in 91 % of ALS, 100 % of FTLDTDP and 75 % of Alzheimer\u27s disease (AD) cases. The most extensive RBM45 pathology was observed in patients that harbor the C9ORF72 hexanucleotide repeat expansion. These RBM45 inclusions were observed in spinal cord motor neurons, glia and neurons of the dentate gyrus. By confocal microscopy, RBM45 co-localizes with ubiquitin and TDP-43 in inclusion bodies. In neurons containing RBM45 cytoplasmic inclusions we often detected the protein in a punctate pattern within the nucleus that lacked either TDP-43 or ubiquitin. We identified RBM45 using a proteomic screen of CSF from ALS and control subjects for candidate biomarkers, and link this RNA-binding protein to inclusion pathology in ALS, FTLD-TDP and AD. © 2012 Springer-Verlag