Development of the clinical assessment scale in autoimmune encephalitis.

OBJECTIVE: There is no scale for rating the severity of autoimmune encephalitis (AE). In this study, we aimed to develop a novel scale for rating severity in patients with diverse AE syndromes and to verify the reliability and validity of the developed scale. METHODS: The key items were generated by a panel of experts and selected according to content validity ratios. The developed scale was initially applied to 50 patients with AE (development cohort) to evaluate its acceptability, reproducibility, internal consistency, and construct validity. Then, the scale was applied to another independent cohort (validation cohort, n = 38). RESULTS: A new scale consisting of 9 items (seizure, memory dysfunction, psychiatric symptoms, consciousness, language problems, dyskinesia/dystonia, gait instability and ataxia, brainstem dysfunction, and weakness) was developed. Each item was assigned a value of up to 3 points. The total score could therefore range from 0 to 27. We named the scale the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The new scale showed excellent interobserver (intraclass correlation coefficient [ICC] = 0.97) and intraobserver (ICC = 0.96) reliability for total scores, was highly correlated with modified Rankin scale (r = 0.86, p < 0.001), and had acceptable internal consistency (Cronbach α = 0.88). Additionally, in the validation cohort, the scale showed high interobserver reliability (ICC = 0.99) and internal consistency (Cronbach α = 0.92). INTERPRETATION: CASE is a novel clinical scale for AE with a high level of clinimetric properties. It would be suitable for application in clinical practice and might help overcome the limitations of current outcome scales for AE. ANN NEUROL 2019;85:352-358.

Psychometric Evaluation of the Caregiver Burden Inventory in Children and Adolescents With PANS.

Objectives: To establish the psychometric properties of the Caregiver Burden Inventory (CBI) in patients with Pediatric Acute-onset Neuropsychiatric Syndrome (PANS), which is characterized by the abrupt onset of obsessive-compulsive disorder and/or restricted eating and at least two additional psychiatric symptoms. Parents of patients with PANS have reported high caregiver burden. However, no validated instrument of burden exists for this population. Methods: Study took place at a community-based PANS clinic where the CBI is administered as part of routine clinical care. The first CBI available during an active disease flare was analyzed (N =104). Construct validity was evaluated within a confirmatory factor analytic framework. Associations between the CBI and patient/family characteristics were explored, and preliminary normative data for this population are presented. Results: Item-factor loadings were strong, and the overall fit of the model was good (root mean square error of approximation = .061). Strict/metric measurement invariance was demonstrated across age. The mean Total Score in this sample was 36.72 ± 19.84 (interquartile range 19-53). Total Scores on the CBI were significantly elevated for parents of children who switched schools because of their illness (Cohen's d = 0.75, 95% confidence interval [CI] 0.28-1.22) and for those who had reduced work hours to accommodate the child's illness (Cohen's d = 0.65, 95% CI 0.10-1.20). However, in this relatively high-status sample, socioeconomic variables did not predict Total Scores. Conclusions: Parents of patients with PANS experience high caregiver burden. The CBI may be confidently used to assess caregiver burden in this population.

Why Finding a Treatment for Alopecia Areata Is Important: A Multifaceted Perspective.

Alopecia areata is an autoimmune disease that alters one's appearance. Some define it as a cosmetic disease despite evidence that substantial psychosocial burden is associated with it. As a physician, support group leader, consultant for the National Alopecia Areata Foundation, and patient, I discuss the evidence behind the psychosocial impact of alopecia areata and the importance of comprehensive treatment.

Targeting Medication Non-Adherence Behavior in Selected Autoimmune Diseases: A Systematic Approach to Digital Health Program Development.

BACKGROUND: 29 autoimmune diseases, including Rheumatoid Arthritis, gout, Crohn's Disease, and Systematic Lupus Erythematosus affect 7.6-9.4% of the population. While effective therapy is available, many patients do not follow treatment or use medications as directed. Digital health and Web 2.0 interventions have demonstrated much promise in increasing medication and treatment adherence, but to date many Internet tools have proven disappointing. In fact, most digital interventions continue to suffer from high attrition in patient populations, are burdensome for healthcare professionals, and have relatively short life spans. OBJECTIVE: Digital health tools have traditionally centered on the transformation of existing interventions (such as diaries, trackers, stage-based or cognitive behavioral therapy programs, coupons, or symptom checklists) to electronic format. Advanced digital interventions have also incorporated attributes of Web 2.0 such as social networking, text messaging, and the use of video. Despite these efforts, there has not been little measurable impact in non-adherence for illnesses that require medical interventions, and research must look to other strategies or development methodologies. As a first step in investigating the feasibility of developing such a tool, the objective of the current study is to systematically rate factors of non-adherence that have been reported in past research studies. METHODS: Grounded Theory, recognized as a rigorous method that facilitates the emergence of new themes through systematic analysis, data collection and coding, was used to analyze quantitative, qualitative and mixed method studies addressing the following autoimmune diseases: Rheumatoid Arthritis, gout, Crohn's Disease, Systematic Lupus Erythematosus, and inflammatory bowel disease. Studies were only included if they contained primary data addressing the relationship with non-adherence. RESULTS: Out of the 27 studies, four non-modifiable and 11 modifiable risk factors were discovered. Over one third of articles identified the following risk factors as common contributors to medication non-adherence (percent of studies reporting): patients not understanding treatment (44%), side effects (41%), age (37%), dose regimen (33%), and perceived medication ineffectiveness (33%). An unanticipated finding that emerged was the need for risk stratification tools (81%) with patient-centric approaches (67%). CONCLUSIONS: This study systematically identifies and categorizes medication non-adherence risk factors in select autoimmune diseases. Findings indicate that patients understanding of their disease and the role of medication are paramount. An unexpected finding was that the majority of research articles called for the creation of tailored, patient-centric interventions that dispel personal misconceptions about disease, pharmacotherapy, and how the body responds to treatment. To our knowledge, these interventions do not yet exist in digital format. Rather than adopting a systems level approach, digital health programs should focus on cohorts with heterogeneous needs, and develop tailored interventions based on individual non-adherence patterns.

Ro52 autoantibody-positive women's experience of being pregnant and giving birth to a child with congenital heart block.

OBJECTIVE: congenital heart block may develop in the fetus of women with Ro/SSA autoantibodies. The aim of this study was to investigate how women expecting a child with congenital heart block (CHB) experienced their pregnancy and post-partum period. DESIGN, SETTING AND PARTICIPANTS: women giving birth to a child with CHB in Sweden during 2000-2009 were identified in a population-based manner and individually interviewed post-pregnancy using a semi-structured interview guide. The interviews (n=21) were audiotaped, transcribed verbatim and analysed by qualitative content analysis. FINDINGS: three categories emerged from the responses: learning, suspense and facing. Learning contained both learning about the child's heart block, but frequently also about autoantibody-positivity and a potential rheumatic diagnosis in the mother (16/21). The medical procedures and information differed considerably depending on the area of residence and who was encountered in the health-care system. In many cases, ignorance about this rare condition caused a delay in treatment and surveillance. Suspense described the women's struggle to cope with the feeling of guilt and that the child had a serious heart condition and might not survive the pregnancy. Facing included the post-partum period, leaving the hospital and adjusting to everyday life. The women had tended to put their pregnancies 'on hold', and some described that they needed prolonged time to bond with their newborn child. CONCLUSION: increased awareness and knowledge of CHB are needed to provide adequate care. Offering psychological support by a health-care professional to facilitate early bonding with the child should be considered. IMPLICATIONS FOR PRACTICE: there is a need for structured programs for surveillance of the pregnancies. Such programme should implement guidelines for the involved personnel in the chain of care and make relevant information accessible for the women and families.