prepOSCE: A Virtual, Scalable Solution to Prepare Residents for Their OSCE Examination.

The Royal College Comprehensive Objective Examination in Neurology provides certification for Canadian neurologists and consists of a written examination and the Observed Structured Clinical Encounter (OSCE). The OSCE portion of the certification involves residents visiting several patient stations where they address case scenarios with an examiner. Unfortunately, residents lack exam preparation time due to demanding work hours. In response to resident needs, we created a novel, virtual preparation OSCE program - "prepOSCE" - and evaluated its efficacy. The prepOSCE program employed a proprietary virtual solution from CTC Communications Corp. Ten virtual sessions accommodated 70 residents totally. Seven Canadian physicians and two co-chairs created case scenarios for the stations. On session day, seven residents arrived in a virtual plenary room for briefing followed by assignment to a station by CTC. Residents then moved virtually through prepOSCE stations a different examiner and case scenario in each. Following their session, residents and evaluators were surveyed to capture experiences. The average program rating was 4.22 out of 5 (n = 36 residents of 70 residents who participated in the program) and 4.35 (n = 17 evaluators). Ninety-two percent of residents agreed or strongly agreed that they would recommend this program to their peers; they would like prepOSCE to continue next year; and the program was relevant and added value to their studies. The positive feedback received from prepOSCE participants indicates there is a need for a program like prepOSCE. This model has potential for expansion and it is hoped that specialties outside of neurology could benefit from a similar program.

Competence committees decision-making; an interplay of data, group orientation, and intangible impressions.

BACKGROUND: The implementation of competency-based medical education and utilization of competence committees (CC) represents a paradigm shift in residency education. This qualitative study aimed to explore the methods used by two operational CC and their members to make decisions about progression and competence of their residents. METHODS: An instrumental case study methodology was used to study the CC of two postgraduate training programs. Transcripts from observed CC meetings, member interviews, and guiding documents were analyzed using a constructivist grounded theory approach to reveal themes explaining the decision-making process. RESULTS: Our study found that the CC followed a process that began within a social decision schema model and evolved to a discussion that invoked social influence theory, shared mental models, and social judgment scheme to clarify the points of contention. We identified that the CC decision-making was at risk of bias, primarily influenced by the group composition, the group orientation and individual members' mindset, as well as their personal experiences with the trainees. CONCLUSIONS: Increased awareness of the sources of bias in CC functioning and familiarity with the CC role in competency-based medical education would enable committees to provide valuable feedback to all trainees regardless of their trajectory.

Neuroimaging and Pathology Findings Associated With Rapid Onset Obesity, Hypothalamic Dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) Syndrome.

Rapid onset Obesity, Hypothalamic dysfunction, Hypoventilation, and Autonomic Dysregulation (ROHHAD) is a rare syndrome whose underlying pathophysiology and etiology remain elusive. We present the case of a 36-month-old boy with the classic symptoms of ROHHAD and a neuroendocrine tumor, who progressed rapidly and subsequently succumbed to cardiorespiratory arrest because of hypoventilation. His magnetic resonance imaging findings at the initial diagnosis and the brain autopsy results are detailed. The literature was reviewed to summarize the current understanding of the underlying mechanism of this rare disorder.

Thrombophilia risk is not increased in children after perinatal stroke.

Perinatal stroke causes cerebral palsy and lifelong disability. Specific diseases are definable, but mechanisms are poorly understood. Evidence suggests possible associations between arterial perinatal stroke and prothrombotic disorders, but population-based, controlled, disease-specific studies are limited. Understanding thrombophilia in perinatal stroke informs pathogenesis models and clinical management. We conducted a population-based, prospective, case-control study to determine the association of specific perinatal stroke diseases with known thrombophilias. Children with idiopathic magnetic resonance imaging-classified neonatal arterial ischemic stroke (NAIS), arterial presumed perinatal ischemic stroke (APPIS), or fetal periventricular venous infarction (PVI) were recruited. Standardized thrombophilia evaluations were performed after 12 months of age on stroke cases and controls, including quantified proteins C and S, antithrombin, factors VIII/IX/XI, fibrinogen, lipoprotein(a), homocysteine, lupus anticoagulant, anticardiolipin antibodies and genotyping of factor V Leiden (FVL), factor II G20210A (FII), and methylenetetrahydrofolate reductase C677T. A total of 212 children were studied: 46 with NAIS, 34 with APPIS, 55 with PVI, and 77 controls (male, 53%; median age, 4.8 years). Of 14 parameters, no differences were observed in 12, including all common thrombophilias. Mean prothrombin time was shorter in arterial strokes (P < .001). Rates of antiphospholipid antibodies were low, comparable to those in controls, and resolved on repeat testing. FVL and FII rates were comparable to population norms. Total number of possible abnormalities did not differ between cases and controls. Our prospective, population-based, controlled, disease-specific study suggests minimal association between perinatal stroke and thrombophilia. This does not exclude the possibility of disordered coagulation at the time of stroke but suggests testing in childhood is not indicated.

Longitudinally extensive transverse myelitis with positive aquaporin-4 IgG associated with dengue infection: a case report and systematic review of cases.

BACKGROUND: Neuromyelitis Optica Spectrum Disorder can be associated with parainfectious and post-infectious triggers. Dengue virus infection is one of the most common arbovirus infections in the world, and may present with neurological manifestations. OBJECTIVES: We present a case of DENV-associated with LETM and positive aquaporin-4 IgG, and a systematic review of published cases. METHODS: Medline (Ovid) and PubMed were search through June 2021, for case reports, series and observational studies that described patients with DENV-associated LETM and/or NMOSD. RESULTS: An adolescent girl who had recently immigrated from a Dengue-endemic region presented with a LETM with high positive AQP4-IgG titer and seropositive DENV IgM/IgG antibodies. She responded well to steroids and subsequently started maintenance rituximab for her NMOSD diagnosis. LITERATURE REVIEW: 22 publications describing 27 patients met inclusion criteria. In addition to this case, three published cases met current criteria for NMOSD with serological evidence of acute DENV infection. CONCLUSIONS: It is unknown whether there is a pathophysiological association between DENV infection and NMOSD. Regardless, if an immune-mediated event is suspected, particularly NMOSD, appropriate immunotherapy should be considered early. Decision regarding long term immunotherapy may depend on index of suspicion of true NMOSD, and this is where AQP4-IgG status and follow-up is helpful.

Single-center experience with Beta-propeller protein-associated neurodegeneration (BPAN); expanding the phenotypic spectrum.

Beta-propeller protein-associated neurodegeneration (BPAN) is a subtype of neurodegeneration with brain iron accumulation (NBIA) that presents with childhood developmental delay (especially speech delay), occasionally associated with epileptic encephalopathy, autism, or Rett-like syndrome. The majority of children described to date have been severely affected, with little to no expressive speech function, severe developmental delay, and cognitive impairment. Herein, five additional patients with BPAN identified in the same center in Canada are described, four with the typical severe phenotype and one with a milder phenotype. Our findings provide further evidence that a spectrum of severity exists for this rare and newly described condition. Challenges in identifying iron accumulation on brain MRI are also addressed. Additionally, the importance of including the WDR45 gene on epilepsy and Rett-like syndrome genetic panels is highlighted.