Objectives:
The aim of this study was to assess the Dutch nationwide incidence of myelin oligodendrocyte glycoprotein (MOG)-IgG-associated acquired demyelinating syndromes (ADS) and to describe the ...clinical and serological characteristics of these patients.
Methods:
All serum samples for routine diagnostics from February 2014 to December 2017 were sent to the single central reference laboratory for the full-length MOG-IgG cell-based assay (CBA) in the Netherlands. Clinical data from patients known in our National ADS centre were available.
Results:
A total of 1414 samples of 1277 patients were received; of these, 92 patients (7%) were MOG-IgG-seropositive. The mean incidence was 0.16/100,000 people, with higher seropositivity in children (0.31/100,000) than in adults (0.13/100,000). In MOG-IgG-positive patients at the National ADS centre (61/92, 66%), the most common presenting phenotype is acute disseminated encephalomyelitis (ADEM, 56%) in children and optic neuritis (ON, 44%) in adults. Relapsing disease occurred in 9/34 (26%) children and 11/27 (41%) adults during median follow-up of 27.5 months. Patients were tested MOG-IgG-positive >200 months after the initial attack, suggesting an extended time to first relapse (TTFR). Longitudinal analysis of MOG-IgG (25/61, 41%) showed that 67% of the monophasic patients remain seropositive and 60% in relapsing patients. Majority of seronegative patients had no relapses (89%).
Conclusion:
This nationwide study shows that the overall incidence of MOG-IgG-seropositive disorders is 0.16 per 100,000 people. The distribution over the clinical phenotypes differs between adults and children. Seropositivity can be maintained over years even without clinical activity, while seronegative patients generally had no relapses.
Background:
Radiologically isolated syndrome (RIS) is typified by multiple sclerosis (MS)-like lesions on imaging, without clinical MS symptoms. The prevalence of pediatric RIS is largely unknown.
...Objective:
The objective of the study is to provide an estimated RIS prevalence in a population-based cohort of children.
Methods:
We used data from the Generation R study to identify the childhood RIS prevalence.
Results:
In 5238 participants, only one RIS case was identified (prevalence: 0.02%; 95% confidence interval (CI): 0.00–0.11). During a 62-month follow-up, imaging examinations showed accrual of new focal demyelinating lesions; however, no clinical MS symptoms occurred.
Conclusions:
This study shows that the occurrence of RIS in children from the general population is rare.
Background and purpose
Neuromyelitis optica spectrum disorders (NMOSDs) are a group of rare inflammatory demyelinating disorders of the central nervous system. The identification of specific ...antibodies directed to aquaporin 4 (AQP4‐IgG) led to the distinction from multiple sclerosis. However, up to 25% of the clinically diagnosed NMO patients are seronegative for AQP4‐IgG. A subgroup of these patients might be identified by antibodies directed to myelin oligodendrocyte glycoprotein (MOG‐IgG). Our objective was to investigate whether the clinical characteristics of these patients differ.
Methods
Using a cell‐based assay, samples of 61 AQP4‐IgG seronegative patients and 41 AQP4‐IgG seropositive patients with clinically NMOSD were analysed for the presence of MOG‐IgG. Clinical characteristics of the AQP4‐IgG, MOG‐IgG seropositive and double seronegative NMOSD patients were compared.
Results
Twenty of the 61 AQP4‐IgG seronegative patients tested MOG‐IgG seropositive (33%). MOG‐IgG seropositive patients were more frequently males in contrast to AQP4‐IgG seropositive patients (55% vs. 15%, P < 0.01) and Caucasians (90% vs. 63%, P = 0.03). They more frequently presented with coincident optic neuritis and transverse myelitis (40% vs. 12%, P = 0.02) and had a monophasic disease course (70% vs. 29%, P < 0.01). AQP4‐IgG seropositive patients were 2.4 times more likely to suffer from relapses compared with MOG‐IgG seropositive patients (relative risk 2.4, 95% confidence interval 1.2–4.7). AQP4‐IgG seropositive patients had higher Expanded Disability Status Scale levels at last follow‐up (P < 0.01).
Conclusion
Antibodies directed to MOG identify a subgroup of AQP4‐IgG seronegative NMO patients with generally a favourable monophasic disease course.
Background and purpose
Clinically isolated syndrome (CIS) is a first demyelinating event of the central nervous system and can be a single event. After CIS, a chronic disease course with ongoing ...inflammation and relapses might occur, resulting in a diagnosis of multiple sclerosis (MS). As yet, there has been no prospective exploration of whether children and adults with CIS have the same disease course.
Methods
Patients with CIS, whose age ranged from 1 to 50 years, were prospectively followed. We divided the patients into three different age groups, i.e. 1–10, 11–17 and 18–50 years old. Demographic data, disease course, time to MS diagnosis and annualized relapse rates (ARRs) were compared among these groups.
Results
We included 383 patients with CIS, of whom 218 (56.9%) were diagnosed with MS. Children of between 11 and 17 years old had the highest rate of MS conversion (83.5% vs. 50.0% in the other age groups together, P < 0.01) and the shortest time to MS diagnosis median time 2.6 months (interquartile range, 0.6–6.0) vs. 8.2 months (interquartile range, 1.9–28.2) in the other age groups together, P < 0.01). ARRs corrected for follow‐up were higher in children of <18 years old than in adults of ≥18 years old with MS (mean ARR, 0.65 vs. 0.43, P < 0.01).
Conclusion
Children with CIS tend to have a more inflammatory disease course appearing from higher ARRs in all children and the highest rate of MS conversion in 11–17‐year‐old children. This supports early initiation of disease‐modifying therapy in children, perhaps even at the first event in children at high risk for MS in line with clinical practice in adults.
Click here to view the accompanying paper in this issue.
Studies showed that adverse events within health care settings can lead to two victims. The first victim is the patient and family and the second victim is the involved healthcare professionals. ...However, there is a lack of research studying the experiences of healthcare professionals encountering clinical incidents in Hong Kong. This paper reports a qualitative study in exploring the healthcare professional experiences of clinical incident, their impacts and needs.
This study is the second part of the mixed research method with two studies conducted in a cluster of hospitals in Hong Kong. Study 1 was a quantitative questionnaire survey and Study 2 was a qualitative In-Depth Interview. In study 2, a semi-interview guide was used.
Results showed that symptoms experienced after the clinical incident were mostly from psychological, physical, then social and lastly spiritual aspects which were consistent with those found in study 1 and other studies. Using content analysis for analyzing the impacts, four themes were identified. Concerning the impacts immediately from the clinical incident, two themes emerged were 1) facing emotion distress and 2) maintaining rationality. Regarding the impacts after the clinical incident, another two themes were 3) managing further emotional distress 4) restoring personal wellness. With regard to the needs after clinical incidents, three themes emerged were 1) self-recovery; 2) senior good mentoring and 3) positive organization climate with emphasis on enhancement of training and development of a positive practice culture.
Great impacts are found with healthcare professionals encountering clinical incidents from a holistic perspective. They need time for self-recovery with support from good supervisors, peers and a caring environment. Some recommendations based on the findings of the study are made.
Abstract Highly virulent community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) has been associated with morbidity and mortality in various countries of the world. We characterized ...the clinical and molecular features of pediatric invasive CA-MRSA infections in Taiwan. Between July 2000 and June 2005, 31 previously healthy children with invasive CA-MRSA infections were identified from 423 children with community-onset methicillin-resistant S. aureus infections. The medical records were reviewed. The clinical isolates, if available, were collected for molecular characterization. Sixteen (51.6%) patients were male, and the mean age was 5.7 years. Adolescents accounted for 9 (29%) cases. Eighteen children had bone and/or joint infections, 14 had deep-seated soft tissue infections, 11 had pneumonia, and 2 had central nervous system infections. Multiorgan involvement was identified in 8 of 20 bacteremic cases. Twenty-two patients (71%) required surgical interventions. The mean hospital stay was 27.4 days. All of the 15 available isolates were classified as sequence type (ST) 59 or its single locus variant and belonged to 2 previously reported community-associated clones containing staphylococcal cassette chromosome mec (SCC mec ) type IV or type VT in Taiwan. Most of the isolates were multiresistant to clindamycin (94%) and erythromycin (97%). Eleven (73.3%) isolates carried pvl genes, and the strains harboring pvl genes were significantly associated with lung involvement. In conclusion, invasive CA-MRSA infections in pediatric population were not limited to young children. Surgical interventions were often required, and a prolonged course of antibiotic therapy was needed. A multiresistant CA-MRSA clone characterized as ST59 was identified from these children in Taiwan.
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