Clinical features and outcomes in children with seronegative autoimmune encephalitis.

AIM: To characterize the presenting features and outcomes in children with seronegative autoimmune encephalitis, and to evaluate whether scores at nadir for the Modified Rankin Scale (mRS) and Clinical Assessment Scale for Autoimmune Encephalitis (CASE) or its paediatric-specific modification (ped-CASE) are predictive of outcomes. METHOD: This observational study included children younger than 18 years of age with seronegative autoimmune encephalitis. Demographics and clinical data were collected. The mRS and CASE/ped-CASE scores were used to evaluate disease severity. Descriptive statistics and logistic regression were used for data analysis and to evaluate associations between scale scores and outcomes. RESULTS: Sixty-three children were included (39 [62%] females, median age 7 years, interquartile range [IQR] 4 years 1 months-11 years 6 months), with follow-up available for 56 out of 63 patients (median follow-up 12.2 months, IQR 13.4-17.8). The most frequent presenting neurological manifestation was encephalopathy (81%). Median CASE/ped-CASE and mRS scores at nadir were 12.0 (IQR 7.0-17.0) and 1.0 (IQR 0-2.0) respectively. Thirty-three patients (59%) had persistent neurological deficits at follow-up. Both scoring systems suggested good functional recovery (mRS score ≤2, 95%; CASE/ped-CASE score <5, 91%). CASE/ped-CASE score was more likely than mRS to distinguish children with worse outcomes. INTERPRETATION: Children with seronegative autoimmune encephalitis are likely to have neurological deficits at follow-up. CASE/ped-CASE is more likely to distinguish children with worse outcomes than MRS.

Case-Informed Learning in Medical Education: A Call for Ontological Fidelity.

Case-informed learning is an umbrella term we use to classify pedagogical approaches that use text-based cases for learning. Examples include Problem-Based, Case-Based, and Team-Based approaches, amongst others. We contend that the cases at the heart of case-informed learning are philosophical artefacts that reveal traditional positivist orientations of medical education and medicine, more broadly, through their centering scientific knowledge and objective fact. This positivist orientation, however, leads to an absence of the human experience of medicine in most cases. One of the rationales for using cases is that they allow for learning in context, representing aspects of real-life medical practice in controlled environments. Cases are, therefore, a form of simulation. Yet issues of fidelity, widely discussed in the broader simulation literature, have yet to enter discussions of case-informed learning. We propose the concept of ontological fidelity as a way to approach ontological questions (i.e., questions regarding what we assume to be real), so that they might centre narrative and experiential elements of medicine. Ontological fidelity can help medical educators grapple with what information should be included in a case by encouraging an exploration of the philosophical questions: What is real? Which (and whose) reality do we want to simulate through cases? What are the essential elements of a case that make it feel real? What is the clinical story we want to reproduce in case format? In this Eye-Opener, we explore what it would mean to create cases from a position of ontological fidelity and provide suggestions for how to do this in everyday medical education.

Songwriting and Youth Self-Concept.

Background: Current literature on the therapeutic outcomes of youth engagement in active music-based interventions is limited in terms of both the number of studies and methodology. This pilot study combined phenomenology and quantitative measures of self-esteem and self-efficacy to explore the impact of storytelling through lyrical and musical composition on youth self-concept. Methods: Thirteen youth ages 12 to 16 engaged in a series of 5 consecutive daily music workshops, and themes related to participating in the workshop were identified. Participants also took the Rosenberg Self-Esteem Scale and Sherer General Self-Efficacy Scale before and after completing the workshops, and pre- and posttest scores were compared using t-tests. Results: Key themes that emerged from the analysis were empowerment and accomplishment. Although the results of the t-tests were not significant, the qualitative data from this pilot study suggest that the workshops had a positive impact on participant self-concept. Conclusions: These preliminary results could be used to inform further research and to plan active music interventions to positively impact youth mental health.

How an Arts-Based Clinical Skills Set Can Be Assessed During OSCEs.

Background: Arts-based activities' roles in medical education is to challenge students to cultivate clinical skills using ART (aesthetics, reflection, time). ART activities offer opportunities for students to cultivate creative dimensions of their clinical skills and to reflect on their responses to uncertainty and ambiguity. Faculty, however, are challenged to structure these learning activities in diverse, sometimes unfamiliar, health care settings. Methods: This study explored preclerkship medical students' responses to participating in ART activities presented in the common medical educational format of an objective structured clinical exam (OSCE). Activities included interpreting fine art (eg, images and poetry) and drawing a simulated patient. The discussion section transcript and student sketchbooks were analyzed to identify themes related to participating in the study. Results: Use of arts-based activities elicited behaviors similar to those observed in students' responses to formal summative OSCEs, although students also wrestled with challenges and expressed their subjective impressions. Conclusions: This study offers an arts-based tool set capable of being delivered within the familiar medical education setting and established structure of the OSCE.

Portraits of Children With Epilepsy.

This article examines the challenges of raising a child with epilepsy and the role of portraiture in helping clinicians consider the impact of epilepsy not only on the patient but also on family caregivers. Portraiture facilitates the development of a visual narrative of how a child is seen by others, both figuratively and physically.

Successful treatment of severe infantile hyperekplexia with low-dose clobazam.

We report two cases of severe infantile hyperekplexia successfully treated with low-dose clobazam. The first case presented at 6 weeks of age with multiple episodes consisting of difficulty diapering because of stiffness and loud inspiratory noises followed by breath-holding in inspiration. She was diagnosed with hyperekplexia and started on clonazepam 0.05 mg daily. This was discontinued because of excessive sleepiness. The second case presented at 3 weeks of age with episodes of crying that would change in pitch and then abruptly stop, followed by leg and arm extension and stiffening. On occasion, there was cyanosis, and she received mouth-to-mouth resuscitation. She was diagnosed with hyperekplexia at 9 months of age. Both infants were treated with clobazam (0.25 and 0.3 mg/kg/day respectively), resulting in resolution of symptoms with no side effects. During treatment, both had minimal startle response to various stimuli and have now been successfully weaned from clobazam. Low-dose clobazam is effective in the treatment of hyperekplexia and is well tolerated in infants.

Brainstem calcification in Möbius syndrome.

Möbius syndrome is characterized by congenital facial diplegia, and may be associated with limb or orofacial malformations. A number of mechanisms have been proposed to explain the pathogenesis, including prenatal ischemia. We identified seven children with Möbius syndrome over the 10-year interval 1992-2001, all of whom manifested incomplete bilateral facial palsy. Associated limb and orofacial anomalies were observed in six cases. Computed tomographic scans were available in six children, and five of them manifested brainstem calcification which was most prominent in the floor of the fourth ventricle. The calcification was detected as early as 7 days of age and did not change with time, suggesting a static condition of prenatal onset. These observations support the hypothesis that the pathology in Möbius syndrome is secondary to prenatal brain ischemia.

Phylogeographic structure and postglacial evolutionary history of water voles (Arvicola terrestris) in the United Kingdom.

The phylogeographic pattern of mitochondrial DNA variation in water voles (Arvicola terrestris) from 57 localities across the United Kingdom and representative samples from Spain, France, Switzerland and Finland was determined from sequence variation in the central portion of the control region. Twenty-seven different haplotypes were resolved which formed two distinct phylogenetic clades. This major division separated haplotypes found in Scotland from those found in England and Wales. Nested clade analysis of haplotypes indicated that such a division was a consequence of allopatric fragmentation. The haplotypes found in Switzerland, France and Spain clustered with Scottish haplotypes, whereas the haplotype from Finland clustered with the English/Welsh haplotypes. These patterns indicate that contemporary Scottish populations are derived from an Iberian glacial refugium, whereas English and Welsh populations are derived from an eastern European refugium. As such, the postglacial recolonization of the United Kingdom must have involved two colonization events, either in different localities with no subsequent contact, or as two waves separated over time, with the second wave of colonizers displacing the first. An analysis of molecular variance (AMOVA) identified significant population genetic divergence within both the major clades, indicative of restricted gene flow and regional population isolation. The implications of both phylogeographical and population genetic structure are discussed in context with the conservation of water voles in Britain.