Explore the vast range of titles available.

Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. Advances in autoimmune encephalitis research in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to these disorders. However, existing criteria for autoimmune encephalitis are too reliant on antibody testing and response to immunotherapy, which might delay the diagnosis. We reviewed the literature and gathered the experience of a team of experts with the aims of developing a practical, syndrome-based diagnostic approach to autoimmune encephalitis and providing guidelines to navigate through the differential diagnosis. Because autoantibody test results and response to therapy are not available at disease onset, we based the initial diagnostic approach on neurological assessment and conventional tests that are accessible to most clinicians. Through logical differential diagnosis, levels of evidence for autoimmune encephalitis (possible, probable, or definite) are achieved, which can lead to prompt immunotherapy.

Increasing evidence suggests that seizures and status epilepticus can be immune-mediated. We aimed to describe the clinical features of a new epileptic disorder, and to establish the target antigen and the effects of patients' antibodies on neuronal cultures. In this observational study, we selected serum and CSF samples for antigen characterisation from 140 patients with encephalitis, seizures or status epilepticus, and antibodies to unknown neuropil antigens. The samples were obtained from worldwide referrals of patients with disorders suspected to be autoimmune between April 28, 2006, and April 25, 2013. We used samples from 75 healthy individuals and 416 patients with a range of neurological diseases as controls. We assessed the samples using immunoprecipitation, mass spectrometry, cell-based assay, and analysis of antibody effects in cultured rat hippocampal neurons with confocal microscopy. Neuronal cell-membrane immunoprecipitation with serum of two index patients revealed GABAA receptor sequences. Cell-based assay with HEK293 expressing α1/β3 subunits of the GABAA receptor showed high titre serum antibodies (>1:160) and CSF antibodies in six patients. All six patients (age 3–63 years, median 22 years; five male patients) developed refractory status epilepticus or epilepsia partialis continua along with extensive cortical-subcortical MRI abnormalities; four patients needed pharmacologically induced coma. 12 of 416 control patients with other diseases, but none of the healthy controls, had low-titre GABAA receptor antibodies detectable in only serum samples, five of them also had GAD-65 antibodies. These 12 patients (age 2–74 years, median 26·5 years; seven male patients) developed a broader spectrum of symptoms probably indicative of coexisting autoimmune disorders: six had encephalitis with seizures (one with status epilepticus needing pharmacologically induced coma; one with epilepsia partialis continua), four had stiff-person syndrome (one with seizures and limbic involvement), and two had opsoclonus-myoclonus. Overall, 12 of 15 patients for whom treatment and outcome were assessable had full (three patients) or partial (nine patients) response to immunotherapy or symptomatic treatment, and three died. Patients' antibodies caused a selective reduction of GABAA receptor clusters at synapses, but not along dendrites, without altering NMDA receptors and gephyrin (a protein that anchors the GABAA receptor). High titres of serum and CSF GABAA receptor antibodies are associated with a severe form of encephalitis with seizures, refractory status epilepticus, or both. The antibodies cause a selective reduction of synaptic GABAA receptors. The disorder often occurs with GABAergic and other coexisting autoimmune disorders and is potentially treatable. The National Institutes of Health, the McKnight Neuroscience of Brain Disorders, the Fondo de Investigaciones Sanitarias, Fundació la Marató de TV3, the Netherlands Organisation for Scientific Research (Veni-incentive), the Dutch Epilepsy Foundation.

Background/Objectives: Juvenile idiopathic arthritis (JIA) and inflammatory bowel disease (IBD) are chronic autoimmune conditions that impact the physical and psychological well-being of pediatric patients. While previous studies have shown a high prevalence of mental health challenges among youth with chronic conditions, the prevalence of mental health issues in Canadian pediatric patients with JIA and IBD remains unclear. We aimed to estimate the prevalence of documented mental health disorders and related medication use of youth with JIA or IBD at a tertiary care centre. Methods: We conducted a retrospective chart review of youths aged 12–17 diagnosed with JIA or IBD at McMaster Children’s Hospital (MCH) to understand the prevalence of generalized anxiety disorder (GAD), separation anxiety disorder, social anxiety disorder (SAD), obsessive–compulsive disorders (OCD), eating disorders, major depressive disorder (MDD), adolescent adjustment disorder, suicide attempt/suicide ideation, self-harm behaviour, substance use disorder, and attention deficit disorders (ADD). Results: We reviewed 429 patient charts, including 303 patients with IBD and 126 with JIA. Our findings identified 90 IBD patients and 20 JIA patients who had one or more documented mental health conditions. Proportionately, there was a higher prevalence of mental health conditions among IBD patients (30%) compared to JIA patients (16%). The most frequently observed conditions in both IBD and JIA patients were GAD (63%, 50%), ADD (33%, 35%), and MDD (29%, 15%). Conclusions: These findings highlight the critical need for early mental health screening and integrated care approaches that address both medical and psychosocial needs in adolescents with chronic illnesses. Future research should incorporate prospective study designs, include diverse geographic and demographic populations, and explore targeted interventions to improve mental and physical health outcomes in this vulnerable group.

: Canadian Advanced Physiotherapist Practitioner (APP) roles have existed for over 25 years in pediatric rheumatology. The APP can manage many common pediatric rheumatic conditions most often in Shared-Care Models (SCMs) with pediatric rheumatologists (PRs). The quality of care children receive in an APP SCM compared to traditional care is unknown. The Pediatric Rheumatology Care and Outcomes Improvement Network (PR-COIN) tracks quality measures as Key Performance Indicators (KPIs) in juvenile idiopathic arthritis (JIA) care. This study aimed to analyze the frequency of KPIs documented in a pediatric rheumatology APP SCM from a single center and compare to PR-COIN's performance targets to assess the quality and safety of care. : A retrospective chart review of JIA cases managed in a pediatric rheumatology APP SCM over a 2-year period was conducted. KPIs for disease activity, safety monitoring and access to care were evaluated. Frequency of KPI documentation by the APP were compared to target performance goals (≥40, ≥70 or ≥80% documentation rate depending on KPI) and with PR-COIN data from the Same Center (SC) (three rheumatologists) and PR-COIN (15 centers). : Documented KPIs were compared between the APP SCM, SC and PR-COIN registry (138; 140; 11,431 eligible visits, respectively) between June 2022-May 2024. Demographics were similar between groups. Increased percentages of patients with polyarticular rheumatoid factor positive and psoriatic subtypes were seen by APP compared to SC and PR-COIN. Documentation frequency of all disease activity and safety monitoring KPI performance goals were either higher in the APP SCM or comparable to SC and PR-COIN. : The pediatric rheumatology APP SCM exceeded PR-COIN performance goals for KPI documentation, establishing a high level of quality and safety of care for children with JIA when managed in this model of care. Next steps include replicating this study in other pediatric rheumatology centers with an APP SCM.

Objective The transition from pediatric to adult rheumatology care represents a particularly vulnerable time for patients with juvenile idiopathic arthritis (JIA) and childhood‐onset systemic lupus erythematosus (cSLE). Improving self‐management skills is important in optimizing health care transition. The study’s objectives were to 1) examine variability in transition readiness of adolescents and young adults within and between different ages, sexes, and disease types; 2) determine the association between age and transition readiness; and 3) identify specific challenges to transition readiness for adolescents. Methods Over 1 year, patients 14 to 20 years of age with JIA or cSLE were recruited from pediatric transition and young adult clinics at a single academic institution. Participants completed the 14‐item Transition‐Q at a single time point. Total scores range from 0 to 100; higher scores indicate greater health care self‐management skills as a proxy for transition readiness. Descriptive statistics summarized patient characteristics and Transition‐Q scores for the population. Regression analyses determined the association between age, sex, and disease type and Transition‐Q score. Results Among 70 participants, 61 had JIA and 9 cSLE (mean disease duration 4.6 years). The mean (SD) total Transition‐Q score was 59.8 (14.9). Age was significantly associated with Transition‐Q score (standardized β = 0.372l P = 0.002). The most commonly reported challenges were seeing the physician alone (without parents), making one’s own appointments, picking up prescriptions, and independent transportation for appointments. Conclusion Transition readiness appears to increase with patient age. There is significant variability in Transition‐Q scores between patients of the same age, suggesting that an individualized approach to improving self‐management skills is necessary.

Objective: Adolescents with chronic rheumatic disease must increasingly take on more responsibility for disease management from parents as they transition from pediatric to adult care. Yet, there are limited resources to inform and support parents about transition. Here, we evaluate the impact of a Transition Toolkit, geared towards parents and adolescents, on transition readiness, and explore the potential impact of parent–adolescent communication. Methods: A prospective cohort study of youths aged 14–18 years old and their parents was performed. Participant demographics, disease characteristics, transition readiness scores (Transition-Q, max 100), and parent–adolescent communication scores (PACS, max 100) were collected at enrollment (when the Transition Toolkit was shared with adolescents and their parents. Generalized estimating equation (GEE) analyses determined the influence of the Toolkit on transition readiness and explored the role of parent–adolescent communication quality. Subgroup analyses were conducted by sex. Results: A total of 21 patients were included; 19 completed one post-intervention Transition-Q and 16 completed two. Transition-Q scores increased over time and the rate of increase doubled after the Toolkit was shared (β = 7.8, p < 0.05, and β = 15.5, p < 0.05, respectively). Conclusion: Transition readiness improved at each follow-up, the greatest increase was seen after the Toolkit was shared. Parent–adolescent communication quality did not appear to impact changes in transition readiness.

The objective of this paper is to evaluate available evidence for each step in autoimmune encephalitis management and provide expert opinion when evidence is lacking. The paper approaches autoimmune encephalitis as a broad category rather than focusing on individual antibody syndromes. Core authors from the Autoimmune Encephalitis Alliance Clinicians Network reviewed literature and developed the first draft. Where evidence was lacking or controversial, an electronic survey was distributed to all members to solicit individual responses. Sixty-eight members from 17 countries answered the survey. Corticosteroids alone or combined with other agents (intravenous IG or plasmapheresis) were selected as a first-line therapy by 84% of responders for patients with a general presentation, 74% for patients presenting with faciobrachial dystonic seizures, 63% for NMDAR-IgG encephalitis and 48.5% for classical paraneoplastic encephalitis. Half the responders indicated they would add a second-line agent only if there was no response to more than one first-line agent, 32% indicated adding a second-line agent if there was no response to one first-line agent, while only 15% indicated using a second-line agent in all patients. As for the preferred second-line agent, 80% of responders chose rituximab while only 10% chose cyclophosphamide in a clinical scenario with unknown antibodies. Detailed survey results are presented in the manuscript and a summary of the diagnostic and therapeutic recommendations is presented at the conclusion.

Objective To identify barriers and facilitators to the uptake of information from research by parents of children with juvenile idiopathic arthritis (JIA). Methods Parents of children with JIA participated in focus group and telephone interviews at four Canadian pediatric rheumatology centers. The semistructured interviews focused on perceptions about JIA research, how new information about JIA was obtained and used, and what information was of most interest. Transcripts were analyzed using a general inductive approach. Results Twenty‐eight parents participated in the study. Parents were very interested in research that addresses the outcomes of JIA and side effects of medications. Parents communicated an expectation that information from research be communicated to them by their child's pediatric rheumatologist as part of clinical care. Parents felt that it would be helpful to have information available to them in a variety of formats including written, video, and online. The timing of information delivery is an important factor, with parents being most interested and engaged in learning about new information about JIA at diagnosis and disease flares. We found that parents were overall unaware of new findings from JIA research and therefore may not be optimally utilizing this potentially helpful information in the care of their children. Conclusion This study has led to an understanding of Canadian parents’ perceptions about research and existing gaps in the translation of research knowledge. This information will facilitate the development, implementation, and evaluation of future knowledge translation interventions aimed at improving the uptake of research information in the care of children with JIA.