Deep phenotyping of the neuroimaging and skeletal features in KBG syndrome: a study of 53 patients and review of the literature.

BACKGROUND: KBG syndrome is caused by haploinsufficiency of ANKRD11 and is characterised by macrodontia of upper central incisors, distinctive facial features, short stature, skeletal anomalies, developmental delay, brain malformations and seizures. The central nervous system (CNS) and skeletal features remain poorly defined. METHODS: CNS and/or skeletal imaging were collected from molecularly confirmed individuals with KBG syndrome through an international network. We evaluated the original imaging and compared our results with data in the literature. RESULTS: We identified 53 individuals, 44 with CNS and 40 with skeletal imaging. Common CNS findings included incomplete hippocampal inversion and posterior fossa malformations; these were significantly more common than previously reported (63.4% and 65.9% vs 1.1% and 24.7%, respectively). Additional features included patulous internal auditory canal, never described before in KBG syndrome, and the recurrence of ventriculomegaly, encephalic cysts, empty sella and low-lying conus medullaris. We found no correlation between these structural anomalies and epilepsy or intellectual disability. Prevalent skeletal findings comprised abnormalities of the spine including scoliosis, coccygeal anomalies and cervical ribs. Hand X-rays revealed frequent abnormalities of carpal bone morphology and maturation, including a greater delay in ossification compared with metacarpal/phalanx bones. CONCLUSION: This cohort enabled us to describe the prevalence of very heterogeneous neuroradiological and skeletal anomalies in KBG syndrome. Knowledge of the spectrum of such anomalies will aid diagnostic accuracy, improve patient care and provide a reference for future research on the effects of ANKRD11 variants in skeletal and brain development.

Quick, Effective Screening Tasks Identify Children With Medical Conditions or Disabilities Needing Physical Literacy Support.

PURPOSE: This study evaluated screening tasks able to identify children with medical conditions or disabilities who may benefit from physical literacy. METHOD: Children completed ≤20 screening tasks during their clinic visit and then the Canadian Assessment of Physical Literacy (2nd edition) at a separate visit. Total Canadian Assessment of Physical Literacy scores <30th percentile were categorized as potentially needing physical literacy support. Receiver operator characteristic curves identified assessment cut points with 80% sensitivity and 40% specificity relative to total physical literacy scores. RESULTS: 223 children (97 girls; 10.1 [2.6] y) participated. Physical activity adequacy, predilection, and physical competence achieved ≥80% sensitivity and ≥40% specificity in both data sets. Adequacy ≤ 6.5 had 86% to 100% sensitivity and 48% to 49% specificity. Daily screen time >4.9 hours combined with Adequacy ≤6.15 had 88% to 10% sensitivity and 53% to 56% specificity. CONCLUSIONS: Activity adequacy, alone or with screen time, most effectively identified children likely to benefit from physical literacy support. Adequacy and screen time questionnaires are suitable for clinical use. Similar results regardless of diagnosis suggest physical competence deficits are not primary determinants of active lifestyles. Research to enhance screening specificity is required.

The mitochondrial tRNA MT-TW m.5537_5538insT variant presents with significant intra-familial clinical variability.

Mitochondrial disorders can present with a wide range of clinical and biochemical phenotypes. Mitochondrial DNA variants may be influenced by factors such as degree of heteroplasmy and tissue distribution. We present a four-generation family in which 10 individuals carry a pathogenic mitochondrial variant (m.5537_5538insT, MT-TW gene) with differing levels of heteroplasmy and clinical features. This genetic variant has been documented in two prior reports, both in individuals with Leigh syndrome. In the current family, three individuals have severe mitochondrial symptoms including Leigh syndrome (patient 1, 100% in blood), MELAS (patient 2, 97% heteroplasmy in muscle), and MELAS-like syndrome (patient 3, 50% heteroplasmy in blood and 100% in urine). Two individuals have mild mitochondrial symptoms (patient 4, 50% in blood and 67% in urine and patient 5, 50% heteroplasmy in blood and 30% in urine). We observe that this variant is associated with multiple mitochondrial presentations and phenotypes, including MELAS syndrome for which this variant has not previously been reported. We also demonstrate that the level of heteroplasmy of the mitochondrial DNA variant correlates with the severity of clinical presentation; however, not with the specific mitochondrial syndrome.

Barriers and facilitators to program directors' use of the medical education literature: a qualitative study.

BACKGROUND: It is unclear how often frontline clinical teachers are using this literature and its evidence base in teaching and assessment. Our study purpose was to examine postgraduate program director perspectives on the utilization and integration of evidence-based medical education literature in their teaching and assessment practices. METHODS: The authors conducted semi-structured telephone interviews with a convenience sample of current and former program directors from across Canada. Interviews were transcribed and analyzed inductively to distil pertinent themes. RESULTS: In 2017, 11 former and current program directors participated in interviews. Major themes uncovered included the desire for time-efficient and easily adaptable teaching and assessment tools. Participants reported insufficient time to examine the medical education literature, and preferred that it be 'synthesized for them'. (i.e., Best evidence guidelines). Participants recognised continuing professional development and peer to peer sharing as useful means of education about evidence-based tools. Barriers to the integration of the literature in practice included inadequate time, lack of financial compensation for teaching and assessment, and the perception that teaching and assessment of trainees was not valued in academic promotion. DISCUSSION: Faculty development offices should consider the time constraints of clinical teachers when planning programming on teaching and assessment. To enhance uptake, medical education publications need to consider approaches that best meet the needs of a targeted audiences, including frontline clinical teachers. This may involve novel methods and formats that render evidence and findings from their studies more easily 'digestible' by clinical teachers to narrow the knowledge to practice gap.

KMT2B-related disorders: expansion of the phenotypic spectrum and long-term efficacy of deep brain stimulation.

Heterozygous mutations in KMT2B are associated with an early-onset, progressive and often complex dystonia (DYT28). Key characteristics of typical disease include focal motor features at disease presentation, evolving through a caudocranial pattern into generalized dystonia, with prominent oromandibular, laryngeal and cervical involvement. Although KMT2B-related disease is emerging as one of the most common causes of early-onset genetic dystonia, much remains to be understood about the full spectrum of the disease. We describe a cohort of 53 patients with KMT2B mutations, with detailed delineation of their clinical phenotype and molecular genetic features. We report new disease presentations, including atypical patterns of dystonia evolution and a subgroup of patients with a non-dystonic neurodevelopmental phenotype. In addition to the previously reported systemic features, our study has identified co-morbidities, including the risk of status dystonicus, intrauterine growth retardation, and endocrinopathies. Analysis of this study cohort (n = 53) in tandem with published cases (n = 80) revealed that patients with chromosomal deletions and protein truncating variants had a significantly higher burden of systemic disease (with earlier onset of dystonia) than those with missense variants. Eighteen individuals had detailed longitudinal data available after insertion of deep brain stimulation for medically refractory dystonia. Median age at deep brain stimulation was 11.5 years (range: 4.5-37.0 years). Follow-up after deep brain stimulation ranged from 0.25 to 22 years. Significant improvement of motor function and disability (as assessed by the Burke Fahn Marsden's Dystonia Rating Scales, BFMDRS-M and BFMDRS-D) was evident at 6 months, 1 year and last follow-up (motor, P = 0.001, P = 0.004, and P = 0.012; disability, P = 0.009, P = 0.002 and P = 0.012). At 1 year post-deep brain stimulation, >50% of subjects showed BFMDRS-M and BFMDRS-D improvements of >30%. In the long-term deep brain stimulation cohort (deep brain stimulation inserted for >5 years, n = 8), improvement of >30% was maintained in 5/8 and 3/8 subjects for the BFMDRS-M and BFMDRS-D, respectively. The greatest BFMDRS-M improvements were observed for trunk (53.2%) and cervical (50.5%) dystonia, with less clinical impact on laryngeal dystonia. Improvements in gait dystonia decreased from 20.9% at 1 year to 16.2% at last assessment; no patient maintained a fully independent gait. Reduction of BFMDRS-D was maintained for swallowing (52.9%). Five patients developed mild parkinsonism following deep brain stimulation. KMT2B-related disease comprises an expanding continuum from infancy to adulthood, with early evidence of genotype-phenotype correlations. Except for laryngeal dysphonia, deep brain stimulation provides a significant improvement in quality of life and function with sustained clinical benefit depending on symptoms distribution.

Physical activity, sleep and neuropsychiatric symptom severity in children with tourette syndrome.

The purpose of this study was to examine associations between physical activity, sleep and symptom severity in children with tic disorders. Children with tic disorders wore the GeneActiv device, a wrist-worn accelerometer that measures physical activity intensity and sleep/wake parameters continuously for seven days, and completed questionnaires on sleep quality, exercise and severity of tics, ADHD, obsessive-compulsive behaviours, anxiety and depression. 110 children participated in the study. Children with more severe tics had significantly more frequent comorbid diagnoses, greater impairment in subjective sleep measures, greater sedentary activity time and less light, moderate and vigorous activity time (all p < 0.05). There was a significant negative correlation between light, moderate and vigorous physical activity and the severity of tics (- 0.22, p = 0.04), obsessive compulsive behaviours (- 0.22, p = 0.03), anxiety (- 0.35, p = 0.0005) and depression (- 0.23, p = 0.03). There was no correlation between objective sleep time, sleep efficiency and symptom severity. Subjective sleep quality was positively correlated with all symptom severity measures, with the strongest correlation with ADHD severity (0.42, p < 0.00001). The results of this observational study indicate a small, but significant relationship between activity and sleep measures and the severity of the main symptom domains present in tic disorders.

Implementation and evaluation of a curriculum on the assessment and treatment of disruptive behaviour disorders.

Disruptive behaviour disorders (DBDs)-which can include or be comorbid with disorders such as attention-deficit hyperactivity disorder, oppositional defiant disorder, conduct disorder and disruptive mood dysregulation disorder-are commonly seen in paediatric practice. Given increases in the prescribing of atypical antipsychotics for children and youth, it is imperative that paediatric trainees in Canada receive adequate education on the optimal treatment of DBDs. We describe the development, dissemination, and evaluation of a novel paediatric resident curriculum for the assessment and treatment of DBDs in children and adolescents. Pre-post-evaluation of the curriculum showed improved knowledge in participants.

Compound heterozygous mutations in the gene PIGP are associated with early infantile epileptic encephalopathy.

There are over 150 known human proteins which are tethered to the cell surface via glycosylphosphatidylinositol (GPI) anchors. These proteins play a variety of important roles in development, and particularly in neurogenesis. Not surprisingly, mutations in the GPI anchor biosynthesis and remodeling pathway cause a number of developmental disorders. This group of conditions has been termed inherited GPI deficiencies (IGDs), a subgroup of congenital disorders of glycosylation; they present with variable phenotypes, often including seizures, hypotonia and intellectual disability. Here, we report two siblings with compound heterozygous variants in the gene phosphatidylinositol glycan anchor biosynthesis, class P (PIGP) (NM_153681.2: c.74T > C;p.Met25Thr and c.456delA;p.Glu153AsnFs*34). PIGP encodes a subunit of the enzyme that catalyzes the first step of GPI anchor biosynthesis. Both children presented with early-onset refractory seizures, hypotonia, and profound global developmental delay, reminiscent of other IGD phenotypes. Functional studies with patient cells showed reduced PIGP mRNA levels, and an associated reduction of GPI-anchored cell surface proteins, which was rescued by exogenous expression of wild-type PIGP. This work associates mutations in the PIGP gene with a novel autosomal recessive IGD, and expands our knowledge of the role of PIG genes in human development.

Pediatric Neurology Workforce in Canada: A 5-Year Update.

BACKGROUND: In 2013, a task force was developed to discuss the future of the Canadian pediatric neurology workforce. The consensus was that there was no indication to reduce the number of training positions, but that the issue required continued surveillance. The current study provides a 5-year update on Canadian pediatric neurology workforce data. METHODS: Names, practice types, number of weekly outpatient clinics, and dates of certification of all physicians currently practicing pediatric neurology in Canada were obtained. International data were used to compute comparisons between countries. National data sets were used to provide information about the number of residency positions available and the number of Canadian graduates per year. Models for future projections were developed based on published projected population data and trends from the past decade. RESULTS: The number of pediatric neurologists practicing in Canada has increased 165% since 1994. During this period, wait times have not significantly shortened. There are regional discrepancies in access to child neurologists. The Canadian pediatric neurology workforce available to see outpatient consultations is proportionally less than that of USA. After accounting for retirements and emigrations, the number of child neurologists being added to the workforce each year is 4.9. This will result in an expected 10-year increase in Canadian pediatric neurologists from 151 to 200. CONCLUSIONS: Despite an increase in the number of Canadian child neurologists over the last two decades, we do not predict that there will be problems with underemployment over the next decade.

Les effectifs en neurologie pédiatrique au Canada : une mise à jour tenant compte des cinq dernières années.Contexte:En 2013, un comité de réflexion a été mis sur pied afin de discuter de l'avenir des effectifs canadiens en neurologie pédiatrique. Un consensus s'est alors dessiné : bien que rien n'indique qu'il faille réduire le nombre de places disponibles dans ce domaine de formation, cette question nécessite toutefois un suivi continu. La présente étude vise ainsi à offrir une mise à jour des données qui concernent ces effectifs en tenant compte des cinq dernières années.Méthodes:Les données suivantes ont été obtenues : noms des praticiens, types de pratique, nombre de consultations hebdomadaires en clinique externe et dates de certification de tous les médecins pratiquant actuellement la neurologie pédiatrique au Canada. Des données internationales ont également été utilisées pour effectuer des comparaisons entre divers pays. Des données au niveau national ont été rassemblées pour établir le nombre de places disponibles en résidence et le nombre de diplômés canadiens par année. Des modèles de projection ont aussi été élaborés en se fondant sur des projections de population déjà publiées et sur les tendances ayant marqué la décennie précédente.Résultats:Le nombre de neurologues pédiatriques qui pratiquent au Canada a augmenté de 165 % depuis 1994. Fait à noter, les temps d'attente n'ont pas été notablement raccourcis durant cette période. Il existe aussi des disparités régionales en matière d'accès à cette spécialité de la neurologie. Les effectifs canadiens en neurologie pédiatrique disponibles pour des consultations en clinique externe demeurent proportionnellement moins importants qu'aux États-Unis. Après avoir tenu compte des départs à la retraite et des apports de l'extérieur, le nombre de neurologues pédiatriques ajoutés chaque année a été en moyenne de 4,9. Pendant une période de 10 ans, on anticipe donc qu'on devrait passer de 151 à 200 neurologues pédiatriques canadiens.Conclusions:En dépit d'une hausse du nombre de neurologues pédiatriques canadiens au cours des deux dernières décennies, nous ne prédisons pas de problèmes de sous-emploi au cours de la prochaine décennie.

Perceptions of competency-based medical education from medical student discussion forums.

BACKGROUND: Competency-based medical education (CBME) is becoming widely implemented in medical education. Trainees' perceptions of CBME are important factors in the implementation and acceptance of CBME. Online discussion groups allow unique insight into trainees' perceptions of CBME during residency training. METHODS: We analysed 867 posts from 20 discussion threads in Premed 101 (Canadian) and 2756 posts from 50 threads in Student Doctor Network (SDN) (American) using NVivo 11. Inductive content analysis was used to develop a data-driven coding scheme that evolved throughout the analysis. Measures were taken to ensure the trustworthiness of findings, including co-coding of a subsample of 600 posts, peer debriefing, consensus-based analytical decision making and the maintenance of an audit trial. RESULTS: Medical residents and students participating in the discussion forums emphasised select themes regarding the implementation of CBME in residency training. Concerns about CBME in Canada primarily involved its implications for the length of residency and post-residency opportunities. Posts on the American forum had a prominent focus on differing areas, such as the subjectivity in the assessment of core competencies and the role of CBME in termination of a resident's position. CONCLUSIONS: Online discussion groups have the potential to provide unique insight into perceptions of CBME. The presented concerns may have implications for refining the model of CBME and illustrate the importance of providing clarification for trainees regarding length of training and evaluation structures from those involved in designing of CBME programmes.

Observations of the hidden curriculum on a paediatrics tertiary care clinical teaching unit.

OBJECTIVE: The hidden curriculum is a set of influences that function at the level of organizational structure and culture which fall outside the formal curriculum, and are often unarticulated or unexplored. Learning associated with the hidden curriculum may have negative consequences on trainee educational development. The study goal was to examine how aspects of the hidden curriculum were enacted during ward rounds on a tertiary care general paediatrics clinical teaching unit (CTU). METHODS: We conducted an ethnographic case study on a CTU of a tertiary care paediatric hospital and observed interactions between medical students, residents and attending staff on rounds. Detailed field notes were collected and inductive analysis was used to inform descriptions and identify themes regarding the hidden curriculum. RESULTS: Twenty-two hours of observation were performed. Two major themes emerged: the hierarchical nature of team dynamics and engagement mediated by perceptions of relevance of the rotation. The hierarchical nature of team dynamics reflected the reinforcement of the rules of rank and 'who to respect'; it contained three subthemes including 'rules of the game', positioning and questioning order. Engagement mediated by perceptions of relevance of the rotation concerned the finding that active participation and attentiveness during rounds varied by home discipline. CONCLUSIONS: We were able to identify representations of the hidden curriculum during observations on a tertiary care paediatric CTU. Interest appears to be a determinant in student level of engagement and observed hierarchies have the potential to impact this engagement either positively or negatively.

Expansion of the clinical phenotype of the distal 10q26.3 deletion syndrome to include ataxia and hyperemia of the hands and feet.

Distal deletion of the long arm of chromosome 10 is associated with a dysmorphic craniofacial appearance, microcephaly, behavioral issues, developmental delay, intellectual disability, and ocular, urogenital, and limb abnormalities. Herein, we present clinical, molecular, and cytogenetic investigations of four patients, including two siblings, with nearly identical terminal deletions of 10q26.3, all of whom have an atypical presentation of this syndrome. Their prominent features include ataxia, mild-to-moderate intellectual disability, and hyperemia of the hands and feet, and they do not display many of the other features commonly associated with deletions of this region. These results point to a novel gene locus associated with ataxia and highlight the variability of the clinical presentation of patients with deletions of this region.

What is the Future of Pediatric Neurology in Canada? Resident and Faculty Perceptions of Training and Workforce Issues.

BACKGROUND: Pediatric neurology trainee numbers have grown considerably in Canada; recent research, however, has shown that the number of pediatric neurology graduates is outpacing the need for future pediatric neurologists. The purpose of this study was to seek the opinion of pediatric neurology program directors and trainees regarding possible solutions for this issue. METHODS: Two focus groups were convened during the Canadian Neurological Sciences Federation annual congress in June 2012; one consisted of current and former program directors, and the other of current pediatric neurology trainees. Groups were asked for their perceptions regarding child neurology manpower issues in Canada as well as possible solutions. Focus groups were audio-recorded and transcribed for analysis. Theme-based qualitative analysis was used to analyze the transcripts. RESULTS: Major themes emerging from both focus groups included the emphasis on community pediatric neurology as a viable option for trainees, including the need for community mentors; recognizing the needs of underserviced areas; and establishing academic positions for community preceptors. The need for career mentoring and support structures during residency training was another major theme which arose. Program directors and trainees also gave examples of ways to reduce the current oversupply of trainees in Canada, including limiting the number of trainees entering programs, as well as creating a long-term vision of child neurology in Canada. CONCLUSIONS: A nationwide dialogue to discuss the supply and demand of manpower in academic and community pediatric neurology is essential. Career guidance options for pediatric neurology trainees across the country merit further strengthening.

Canadian Paediatric Neurology Workforce Survey and Consensus Statement.

BACKGROUND: Little knowledge exists on the availability of academic and community paediatric neurology positions. This knowledge is crucial for making workforce decisions. Our study aimed to: 1) obtain information regarding the availability of positions for paediatric neurologists in academic centres; 2) survey paediatric neurology trainees regarding their perceptions of employment issues and career plans; 3) survey practicing community paediatric neurologists 4) convene a group of paediatric neurologists to develop consensus regarding how to address these workforce issues. METHODS: Surveys addressing workforce issues regarding paediatric neurology in Canada were sent to: 1) all paediatric neurology program directors in Canada (n=9) who then solicited information from division heads and from paediatric neurologists in surrounding areas; 2) paediatric neurology trainees in Canada (n=57) and; 3) community paediatric neurologists (n=27). A meeting was held with relevant stakeholders to develop a consensus on how to approach employment issues. RESULTS: The response rate was 100% from program directors, 57.9% from residents and 44% from community paediatric neurologists. We found that the number of projected positions in academic paediatric neurology is fewer than the number of paediatric neurologists that are being trained over the next five to ten years, despite a clinical need for paediatric neurologists. Paediatric neurology residents are concerned about job availability and desire more career counselling. CONCLUSIONS: There is a current and projected clinical demand for paediatric neurologists despite a lack of academic positions. Training programs should focus on community neurology as a viable career option.

The hidden and informal curriculum across the continuum of training: A cross-sectional qualitative study.

PURPOSE: The hidden and informal curricula refer to learning in response to unarticulated processes and constraints, falling outside the formal medical curriculum. The hidden curriculum has been identified as requiring attention across all levels of learning. We sought to assess the knowledge and perceptions of the hidden and informal curricula across the continuum of learning at a single institution. METHODS: Focus groups were held with undergraduate and postgraduate learners and faculty to explore knowledge and perceptions relating to the hidden and informal curricula. Thematic analysis was conducted both inductively by research team members and deductively using questions structured by the existing literature. RESULTS: Participants highlighted several themes related to the presence of the hidden and informal curricula in medical training and practice, including: the privileging of some specialties over others; the reinforcement of hierarchies within medicine; and a culture of tolerance towards unprofessional behaviors. Participants acknowledged the importance of role modeling in the development of professional identities and discussed the deterioration in idealism that occurs. CONCLUSIONS: Common issues pertaining to the hidden curriculum exist across all levels of learners, including faculty. Increased awareness of these issues could allow for the further development of methods to address learning within the hidden curriculum.

The hidden and informal curriculum across the continuum of training: A cross-sectional qualitative study.

PURPOSE: The hidden and informal curricula refer to learning in response to unarticulated processes and constraints, falling outside the formal medical curriculum. The hidden curriculum has been identified as requiring attention across all levels of learning. We sought to assess the knowledge and perceptions of the hidden and informal curricula across the continuum of learning at a single institution. METHODS: Focus groups were held with undergraduate and postgraduate learners and faculty to explore knowledge and perceptions relating to the hidden and informal curricula. Thematic analysis was conducted both inductively by research team members and deductively using questions structured by the existing literature. RESULTS: Participants highlighted several themes related to the presence of the hidden and informal curricula in medical training and practice, including: the privileging of some specialties over others; the reinforcement of hierarchies within medicine; and a culture of tolerance towards unprofessional behaviors. Participants acknowledged the importance of role modeling in the development of professional identities and discussed the deterioration in idealism that occurs. CONCLUSIONS: Common issues pertaining to the hidden curriculum exist across all levels of learners, including faculty. Increased awareness of these issues could allow for the further development of methods to address learning within the hidden curriculum.

Lumbar puncture simulation in pediatric residency training: improving procedural competence and decreasing anxiety.

BACKGROUND: Pediatric residents must become proficient with performing a lumbar puncture (LP) during training. Residents have traditionally acquired LP skills by observing the procedure performed by a more senior resident or staff physician and then attempting the procedure themselves. This process can result in variable procedural skill acquisition and trainee discomfort. This study assessed changes in resident procedural skill and self-reported anxiety when residents were provided with an opportunity to participate in an interactive training session and practice LPs using a simulator. METHODS: All pediatric residents at our institution were invited to participate. Residents were asked to report their post-graduate year (PGY), prior LP attempts and self-reported anxiety scores as measured by the standardized State-Trait Anxiety Inventory - State Anxiety Scale (STAI-S) prior to completing an observed pre-test using an infant-sized LP simulator. Staff physicians observed and scored each resident's procedural skill using a previously published 21-point scoring system. Residents then participated in an interactive lecture on LP technique and were given an opportunity for staff-supervised, small group simulator-based practice within 1 month of the pre-test. Repeat post-test was performed within 4 months. RESULTS: Of the pediatric residents who completed the pre-test (N = 20), 16/20 (80 %) completed both the training session and post-test. Their PGY training level was: PGY1 (38 %), PGY2 (25 %), PGY3 (25 %) or PGY4 (12 %). Procedural skill improved in 15/16 residents (paired t-test; p < 0.001), driven by a significant improvement in skill for residents in PGY1 (P = 0.015) and PGY2 (p = 0.003) but not PGY3 or PGY4. Overall anxiety scores were higher at baseline than at post testing (mean ± SD; 44.8 ± 12.1 vs 39.7 ± 9.4; NS) however only PGY1 residents experienced a significant reduction in anxiety (paired t-test, p = 0.04). CONCLUSION: LP simulation training combined with an interactive training session may be a useful tool for improving procedural competence and decreasing anxiety levels, particularly among those at an earlier stage of residency training.

Life imitating art: depictions of the hidden curriculum in medical television programs.

BACKGROUND: The hidden curriculum represents influences occurring within the culture of medicine that indirectly alter medical professionals' interactions, beliefs and clinical practices throughout their training. One approach to increase medical student awareness of the hidden curriculum is to provide them with readily available examples of how it is enacted in medicine; as such the purpose of this study was to examine depictions of the hidden curriculum in popular medical television programs. METHODS: One full season of ER, Grey's Anatomy and Scrubs were selected for review. A summative content analysis was performed to ascertain the presence of depictions of the hidden curriculum, as well as to record the type, frequency and quality of examples. A second reviewer also viewed a random selection of episodes from each series to establish coding reliability. RESULTS: The most prevalent themes across all television programs were: the hierarchical nature of medicine; challenges during transitional stages in medicine; the importance of role modeling; patient dehumanization; faking or overstating one's capabilities; unprofessionalism; the loss of idealism; and difficulties with work-life balance. CONCLUSIONS: The hidden curriculum is frequently depicted in popular medical television shows. These examples of the hidden curriculum could serve as a valuable teaching resource in undergraduate medical programs.

Central nervous system venulitis presenting as migraine.

OBJECTIVE: To describe a case of pediatric central nervous system (CNS) venulitis. BACKGROUND: Primary angiitis of the CNS is a rare but increasingly well-recognized cause of morbidity in children. It primarily involves the arteries and arterioles of the CNS, with only 1 published case of a pediatric patient found to have isolated CNS venulitis on brain biopsy. CASE REPORT: A 17-year-old female with a 4-year history of migraines presented with increasingly frequent migraines and right-sided hemiplegia. Infectious, hematologic, and rheumatologic work-ups were negative. Brain magnetic resonance imaging showed multiple rim-enhancing lesions consistent with calcifications affecting the deep left white matter. On brain biopsy, there was evidence of an inflammatory process involving small veins and venules. The patient displayed clinical improvement with a course of high-dose steroids and 6 monthly cyclophosphamide infusions followed by maintenance therapy with mycophenolate mofetil. DISCUSSION: We describe a case of pediatric CNS venulitis presenting with migraine.