Multifocal motor neuropathy as a mimic of amyotrophic lateral sclerosis: Serum neurofilament light chain as a reliable diagnostic biomarker.

Introduction/aims: The clinical presentation of multifocal motor neuropathy (MMN) may mimic early amyotrophic lateral sclerosis (ALS) with predominant lower motor neuron (LMN) involvement, posing a diagnostic challenge. Both diseases have specific treatments and prognoses, highlighting the importance of early diagnosis. The aim of this study was to assess the diagnostic value of serum neurofilament light chain (NfL) in differentiating MMN from LMN dominant ALS.

Significant pain decrease in children with non-systemic Juvenile Idiopathic Arthritis treated to target: results over 24 months of follow up.

Background: The aim of this study was to compare pain-scores in three targeted treatment-strategies in JIA-patients and to identify characteristics predicting persistent pain.

Methods: In the BeSt-for-Kids-study 92 DMARD-naïve JIA-patients were randomized in 3 treatment-strategies: 1) initial sequential DMARD-monotherapy 2) initial methotrexate (MTX)/prednisolone-bridging or 3) initial MTX/etanercept. Potential differences in VAS pain scores (0-100 mm) over time between treatment-strategies were compared using linear mixed models with visits clustered within patients.

Linking paranormal and conspiracy beliefs to illusory pattern perception through signal detection theory.

Previous research indicates that irrational beliefs (Paranormal beliefs & conspiracy theory endorsement) are associated with the perception of patterns in noise, but the previous findings do not conclusively describe this relationship. This study aims to disentangle the underlying parameters of this association by applying a signal detection theory approach, thus allowing to distinguish illusory pattern perception (false alarms) from perceptual sensitivity and response tendencies-while also taking base rate information into account. Results from a large sample (N = 723) indicate that paranormal beliefs relate to a more liberal response bias and a lower perceptual sensitivity, and that this relationship is driven by illusory pattern perception.

Clinical Symptoms, Laboratory Parameters and Long-Term Follow-up in a National DADA2 Cohort.

Deficiency of adenosine deaminase-2 (DADA2) is an autosomal recessive autoinflammatory disease with an extremely variable disease presentation. This paper provides a comprehensive overview of the Dutch DADA2 cohort. We performed a retrospective cohort study in 29 ADA2-deficient patients from 23 families with a median age at inclusion of 26 years.

"More life and more days"-patient and care characteristics in a specialized acute pediatric palliative care inpatient unit.

Unlabelled: Only a few acute hospital inpatient units dedicated to pediatric palliative care (PPC) patients exist today. Clinical data on the patients and care provided at specialized acute PPC inpatient units (PPCUs) are scarce. This study aims at describing patient and care characteristics on our PPCU to learn about the complexity and relevance of inpatient PPC.

Illusory perception of visual patterns in pure noise is associated with COVID-19 conspiracy beliefs.

Just as perceptual heuristics can lead to visual illusions, cognitive heuristics can lead to biased judgements, such as "illusory pattern perception" (i.e., seeing patterns in unrelated events).

Acute water intoxication in an adult man: 'dental hyponatraemia' revisited.

The differential diagnosis of hyponatraemia is notoriously wide. However, only a minority is acute, ie develops in less than 48 hours. We describe an unusual cause of water intoxication due to toothache.

Risk of strong antibody decline in dialysis and transplant patients after SARS-CoV-2mRNA vaccination: Six months data from the observational Dia-Vacc study.

Background: Vulnerable dialysis and kidney transplant patients show impaired seroconversion rates compared to medical personnel eight weeks after SARS-CoV-2mRNA vaccination.

Methods: We evaluated six months follow up data in our observational Dia-Vacc study exploring specific cellular (interferon-γ release assay) or/and humoral immune responses after 2x SARS-CoV-2mRNA vaccination in 1205 participants including medical personnel (125 MP), dialysis patients (970 DP) and kidney transplant recipients (110 KTR) with seroconversion ( IgA or IgG antibody positivity by ELISA) after eight weeks.

Findings: Six months after vaccination, seroconversion remained positive in 98% of MP, but 91%/87% of DP/KTR ( = 0·005), respectively.

Acceptance and Adherence to COVID-19 Preventive Measures are Shaped Predominantly by Conspiracy Beliefs, Mistrust in Science and Fear - A Comparison of More than 20 Psychological Variables.

The global coronavirus (COVID-19) pandemic sparked a great interest in psychological factors that determine or explain peoples' responses to the novel threatening situation and the preventive measures (e.g. wearing masks, social distancing).

Metformin and sodium dichloroacetate effects on proliferation, apoptosis, and metabolic activity tested alone and in combination in a canine prostate and a bladder cancer cell line.

An important approach in tumor therapy is combining substances with different action mechanisms aiming to enhance the antineoplastic effect, decrease the therapeutic dosage, and avoid resistance mechanisms. Moreover, evaluating compounds already approved for the treatment of non-neoplastic diseases is promising for new antineoplastic therapies. Sodium dichloroacetate (DCA) reactivates oxidative phosphorylation in the cancer cell mitochondria, reducing apoptosis resistance in cancer cells.

Humoral and cellular immunity to SARS-CoV-2 vaccination in renal transplant versus dialysis patients: A prospective, multicenter observational study using mRNA-1273 or BNT162b2 mRNA vaccine.

Background: Dialysis and kidney transplant patients are vulnerable populations for COVID-19 related disease and mortality.

Methods: We conducted a prospective study exploring the eight week time course of specific cellular (interferon-γ release assay and flow cytometry) or/and humoral immune responses (ELISA) to SARS-CoV-2 boost vaccination in more than 3100 participants including medical personnel, dialysis patients and kidney transplant recipients using mRNA vaccines BNT162b2 or mRNA-1273.

Results: SARS-CoV-2-vaccination induced seroconversion efficacy in dialysis patients was similar to medical personnel (> 95%), but markedly impaired in kidney transplant recipients (42%).

Dutch patients, caregivers and healthcare professionals generate first nationwide research agenda for juvenile idiopathic arthritis.

Background: Involving the end-users of scientific research (patients, carers and clinicians) in setting research priorities is important to formulate research questions that truly make a difference and are in tune with the needs of patients. We therefore aimed to generate a national research agenda for Juvenile Idiopathic Arthritis (JIA) together with patients, their caregivers and healthcare professionals through conducting a nationwide survey among these stakeholders.

Methods: The James Lind Alliance method was used, tailored with additional focus groups held to involve younger patients.

Macrophage activation syndrome in a newborn: report of a case associated with neonatal lupus erythematosus and a summary of the literature.

Background: Macrophage activation syndrome (MAS) is a life-threatening hyperinflammatory syndrome and is caused by a severely dysregulated immune response. It has rarely been associated with neonatal lupus.

Case Presentation: We present a female neonate with MAS born to a mother who had cutaneous lupus erythematosus with circulating anti-nuclear antibodies (ANA), anti-SSA, anti-SSB and anti-extractable nuclear antigen (anti-ENA) antibodies.

In the borderland of multifocal motor neuropathy and chronic inflammatory demyelinating polyradiculopathy.

Chronic inflammatory demyelinating polyradiculopathy (CIDP) and multifocal motor neuropathy (MMN) are seen as distinct entities with marked differences in pathophysiology and clinical, laboratory, and imaging features. We report a patient with an immune-mediated neuropathy in the borderland of CIDP and MMN, whose magnetic resonance imaging and cerebrospinal fluid (CSF) features strongly resembled CIDP, while the clinical course and treatment response suggested the diagnosis of MMN without conduction blocks. There is strong evidence that MMN is not a variant of CIDP and that these conditions can be separated pathologically.

Inter-laboratory study on standardized MPS libraries: evaluation of performance, concordance, and sensitivity using mixtures and degraded DNA.

We present results from an inter-laboratory massively parallel sequencing (MPS) study in the framework of the SeqForSTRs project to evaluate forensically relevant parameters, such as performance, concordance, and sensitivity, using a standardized sequencing library including reference material, mixtures, and ancient DNA samples. The standardized library was prepared using the ForenSeq DNA Signature Prep Kit (primer mix A). The library was shared between eight European laboratories located in Austria, France, Germany, The Netherlands, and Sweden to perform MPS on their particular MiSeq FGx sequencers.

Massively parallel sequence data of 31 autosomal STR loci from 496 Spanish individuals revealed concordance with CE-STR technology and enhanced discrimination power.

This study reports Short Tandem Repeat (STR) sequence-based allele data from 496 Spanish individuals across 31 autosomal STR (auSTR) loci included in the Precision ID GlobalFiler™ NGS STR Panel v2: D12S391, D13S317, D8S1179, D21S11, D3S1358, D5S818, D1S1656, D2S1338, vWA, D2S441, D5S2800, D7S820, D16S539, D6S474, D12ATA63, D4S2408, D6S1043, D19S433, D14S1434, CSF1PO, D10S1248, D18S51, D1S1677, D22S1045, D2S1776, D3S4529, FGA, Penta D, Penta E, TH01 and TPOX. The sequence of each allele was aligned to the reference sequence GRCh37 (hg19) and formatted according to the guidance of the International Society for Forensic Genetics. A subset of 221 samples was evaluated for testing concordance with allele calls derived from CE-based analysis using PowerPlex Fusion 6C, and there was 99.

Influence of bioactive glass-coating of zirconia implant surfaces on human osteoblast behavior in vitro.

Objective: The recently developed bioactive glass PC-XG3, which is suitable to coat zirconia implant surfaces with high adhesion strength may reduce the time of osseointegration and the marginal bone loss following implantation. The glass composition has been previously evaluated for cytotoxicity on fibroblast cells, and will now be used to evaluate the cell behavior of osteoblast cells.

Methods: Three different surface morphologies were created with PC-XG3 on zirconia discs.

Draft Genome Sequences of Three Porcine Streptococcus suis Isolates Which Differ in Their Susceptibility to Penicillin.

The draft genome sequences of three Streptococcus suis isolates, IMT40343, IMT40201, and IMT40738, are presented here. These isolates were obtained from bronchoalveolar lavage fluid of healthy and diseased weaners from different German piglet-producing farms and differed in their susceptibility to penicillin.

Plasmid-located dfrA14 gene in Pasteurella multocida isolates from three different pig-producing farms in Germany.

Pasteurella multocida is an important respiratory tract pathogen in intensive livestock farming, especially in pigs. Antimicrobial agents are frequently used to combat infections caused by this pathogen. In a study on antimicrobial resistance among respiratory tract pathogens of pigs from 30 German pig-producing farms, P.

High efficacy of rituximab for myasthenia gravis: a comprehensive nationwide study in Austria.

Background: Most patients with myasthenia gravis (MG) need long-term immunosuppressive therapy. However, conventional agents may have intolerable side effects, take too long or fail to achieve disease control. Rituximab (RTX) has emerged as an off-label treatment for refractory MG, but data on its use are still sparse.

GCSH antisense regulation determines breast cancer cells' viability.

Since it is known that cancer cells exhibit a preference for increased glycine consumption, the respective glycine metabolizing enzymes are in focus of many research projects. However, no cancer associated studies are available for the Glycine Cleavage System Protein H (GCSH) to date. Our initial analysis revealed a GCSH-overexpression of the protein-coding transcript variant 1 (Tv1) in breast cancer cells and tissue.

Multifocal motor neuropathy in Austria: a nationwide survey of clinical features and response to treatment.

Background And Objectives: Multifocal motor neuropathy (MMN) is a rare neuropathy and detailed descriptions of larger patient cohorts are scarce. The objective of this study was to evaluate epidemiological, clinical, and laboratory features of MMN patients and their response to treatment in Austria and to compare these data with those from the literature.

Methods: Anonymized demographic and clinical data about MMN patients until 31.

Dutch juvenile idiopathic arthritis patients, carers and clinicians create a research agenda together following the James Lind Alliance method: a study protocol.

Background: Research on Juvenile Idiopathic Arthritis (JIA) should support patients, caregivers/parents (carers) and clinicians to make important decisions in the consulting room and eventually to improve the lives of patients with JIA. Thus far these end-users of JIA-research have rarely been involved in the prioritisation of future research.

Main Body: Dutch organisations of patients, carers and clinicians will collaboratively develop a research agenda for JIA, following the James Lind Alliance (JLA) methodology.