Background: Curriculum maps outline the content of an educational program identifying links between targeted outcomes, educational opportunities, and assessments. The transition to Competence by Design (CBD) in Canadian specialty residency programs requires thoughtful reorganization of educational programming. A curriculum map may assist with understanding the existing curriculum and thereby facilitate planning for CBD. Methods: A map of the pediatric neurology residency curriculum at the University of Calgary was constructed by linking objectives with related learning activities and assessments. Qualitative line-by-line analysis was then conducted to identify gaps in the existing curriculum. The map was used as a framework to plot CBD outcomes and curricular structure as these were established. Results: Generating the traditional curriculum map was time-consuming, requiring 48 hours. Careful review identified several objectives that did not link to formal learning activities or assessments. Many such gaps were recognized to link to non-clinical activities. Using the scaffold of the traditional curriculum reduced the time required for mapping the planned CBD curriculum to 4 hours. Conclusions: The creation of a curriculum map prior to transition to CBD improved understanding of the existing curriculum and will facilitate transition to CBD. Ongoing evaluation of the fit of our predicted CBD map will support effective implementation.

Background: Improved understanding of factors predictive of emergency department (ED) visits in children with cerebral palsy (CP) can help optimize healthcare use. We sought to identify the pattern of ED consultations in these children. Methods: Data from the Registre de paralysie cérébrale du Québec and provincial administrative databases were linked. The CP cohort was comprised of children born between 1999 and 2002. Data pertaining to ED presentations between 1999 and 2012 were obtained. Relative risks were calculated to identify factors associated with increased ED visits. Peers without CP were selected from administrative databases and matched in a 20:1 ratio. Chi-square tests and Student’s T-tests were used to compare the two cohorts. Results: 301 children with CP and 6040 peer controls were selected. Ninety-two percent (92%) of the CP cohort had at least one ED visit, compared to 74% amongst controls. Children with CP had an increased risk of high ED use compared to peers (RR 1.40 95% CI 1.30-1.52). Factors predictive of high ED use were comorbid epilepsy, severe motor impairment and low socioeconomic status. Conclusions: Children with CP have a higher need for urgent health assessments than their peers, resulting in increased use of ED services. System factors and barriers should be investigated.

Background: Children with pathogenic variations in SCN8A can present with early infantile epileptic encephalopathy-13, benign familial infantile seizures-5 or intellectual disability alone without epilepsy. In this case series, we discuss six children with variants in SCN8A managed at BC Children’s Hospital. Methods: We describe clinical and genetic results on six individuals with SCN8A variants identified via clinical or research next-generation sequencing. Functional consequences of two SCN8A variants were assessed using electrophysiological analyses in transfected cells. Results: Clinical findings ranged from normal development with well-controlled epilepsy to significant developmental delay with treatment-resistant epilepsy. Phenotypes and genotypes in our cohort are described in the table below. Functional analysis supported gain-of-function in P2 and loss-of-function in P4. Conclusions: Our cohort expands the clinical and genotypic spectrum of SCN8A-related disorders. We establish functional evidence for two missense variants in SCN8A, including LoF variant in a patient with intellectual disability, and autism spectrum disorder without seizures.

Table for P.120

Background: Children and Adolescents with rare neurogenetic disorders often have no known cure or disease modifying treatments. Recent advancements in treatments are offering much needed hope to these patients and families. However, these treatments are extremely costly, have complex administration requirements and have many unknown long-term risks and outcomes. Methods: In this presentation, we will discuss our experiences with the implementation process, including developing intricate care pathways, collaborating with multiple disciplines and services, supporting and advocating for our patients and families, and interacting with government agencies and pharmaceutical companies. Case studies will highlight the positive impact these treatments are making on the lives of children and adolescents with rare neurological disorders. Results: Spinal muscular atrophy and Neuronal Ceroid Lipofuscinosis Type 2 are both rare and devastating neurodegenerative conditions with significant morbidity and mortality. Health Canada and government funding agencies recently approved Nusinersen, Onasemnogene abeparvovec for the treatment of SMA and Cerliponase alfa for the treatment of CLN2, leading us to swiftly integrate these treatments into our standard of care. Conclusions: While implementing these novel therapies into clinical practice can be both challenging and rewarding, neuroscience nurses are positioned at the forefront to be leaders in this process at both organizational, national, and international levels.

Background: We describe an infant with a diagnosis of GM3 synthase deficiency, presenting with severe neuroirritability from birth. He required multiple admissions due to extreme agitation and caregiver burnout. Multiple pharmacological agents were tried, and the effect of each medication was modest and short-lasting at best. The literature on the management of neuroirritability in children with progressive genetic and metabolic conditions is sparse, and a neuroirritability management protocol has yet to be developed at our institution. Methods: We searched for relevant primary research and articles on PubMed. We reviewed the evidence of each pharmacological agent and added non-pharmacological strategies. We developed management guidelines for neuroirritability at our hospital. This protocol was reviewed by several pediatric neurologists and pediatric palliative care specialists at the Stollery and SickKids Hospitals. Results: We present the Pediatric Neuroirritability Management Protocol for the Stollery Children’s Hospital. Conclusions: Further study is required to assess whether this protocol can be adapted to treat irritability in the context of other neurological conditions such as hypoxic-ischemic encephalopathy and non-accidental injury. In addition, we will expand our guidelines to include other symptoms such as spasticity, dystonia, and autonomic dysfunction.

Background: Patients with DNM1-encephalopathy almost exclusively have missense variants, mostly in the GTPase domain of DNM1. Delayed myelination has been reported in at least three patients with DNM1-encephalopathy, all with missense mutations in the DNM1 central domain. Only one DNM1 splice-site variant has previously been reported, and the authors questioned whether the variant accounted for all aspects of the patient’s phenotype. Methods: Case-Report. Results: Our patient had hypotonia and brief multifocal tonic seizures from age-1-month. He still has profound global developmental delay, daily seizures and microcephaly. MRI-Brain at age-21-months showed T2 hyperintensity in the bilateral periventricular and subcortical white matter; spectroscopy showed a questionable lactate peak and an elevated choline peak relative to N-acetylaspartate. Clinical gene-panel identified a heterozygous de novo pathogenic variant in intron 9 of DNM1 (c.1197-8G > A; IVS9- 8G>A). In-silico tools categorized this variant as deleterious secondary to a splicing defect. RT-PCR analysis on peripheral blood was unsuccessful as DNM1 expression is extremely low outside of the brain. Conclusions: Our patient carried the same DNM1 variant previously reported, indicating this is a recurrent pathogenic splice-site variant. The spectroscopic abnormalities suggest a possible element of demyelination in DNM1 variants of the central domain, though the mechanism remains unclear.

Background: Attention and executive function (EF) deficits in children negatively impact academics, social interactions, and overall quality of life. Children with other brain-based disorders are at high risk for attention and EF concerns, but the effects of these impairments are not well studied in the literature. The Complex Attention and Executive Function Clinic at the Alberta Children’s Hospital collected baseline data on patients referred for concerns of attention deficits co-occurring with diagnosed neurologic illness/injury, or neurodevelopmental disorder (NDD). Methods: The Behaviour Rating Inventory of Executive Function (BRIEF-2), Behaviour Assessment System for Children (BASC-3), Parenting Stress Index (PSI-4) and medical and past treatment information were collected on initial clinic visit for patients aged 5-15 years. Results: BRIEF-2 Global Executive Composite demonstrated 88.9% of children had clinically elevated scores. Clinically significant scores were observed in 55.5% for BASC-3 Adaptive Skills index and 40% of parents in PSI-4 Total Stress scores. Conclusions: Children with neurologic illness/NDDs are at high risk of clinical impairments in attention and EF. In children referred for attention and behavioural regulation, there is clinically significant increased reporting of executive function impairment out of proportion to other behavioural difficulties. The clinic aims to improve overall functioning through treatment of unmanaged attention and EF deficits.

Background: Interictal epileptiform discharges (IEDs) are known as epilepsy biomarkers for seizure detection, and It is essential for clinicians to detect them from from physiological events with similar temporal frequency characteristics. Methods: We analyzed the SEEG recordings obtained from patients with medically-resistant epilepsy (MRE) implanted with DE at the Western University Hospital Epilepsy Unit. The data were cleaned, denoised, montaged and segmented based on the clinical annotations, such as sleep intervals and observed Ictals. For event detection, the signal waveform and its power were extracted symmetrically in non-overlapping intervals of 500 ms. Each waveform’s power across all detected spikes was computed and clustered based on their energy distributions. Results: The recordings included thirteen sessions of 24 hours of extracellular recordings from two patients, with 312 hours extracted from four hippocampus electrodes anterior and posterior hippocampus. Our results indicate IEDs carrying the most different characteristics in the bands [25-75] Hz; SWR, on the other hand, are distributed between [80-170] Hz. Conclusions: Our algorithm detected and successfully distinguished IED from SWRs based on their carrying energy during non-sleep periods. Also, the most powerful spectral features that they were distinguished from occur in [15-30] Hz and [75-90] Hz.

Background: For over 40 years, the objective structured clinical examination (OSCE) has been a part of medical education, eventually finding its way into most aspects of clinical training and evaluation. However, the EEG/epilepsy fellowship training has not classically involved OSCE evaluations. Methods: We designed and implemented a formative OSCE for pediatric and adult EEG/epilepsy fellows in Montreal, Quebec, Canada. The examination was offered in French and English. Stations included: technical issues, short cases, a long case, and communication. We solicited post-examination feedback from all participants via anonymous electronic survey after they had completed the Canadian Society for Clinical Neurophysiology (CSCN) EEG examination. We asked questions surrounding utility of the examination, areas for improvement, and whether the participant had been successful in passing the CSCN examination. Results: Six fellows took the initial formative OSCE. All six reported passing the subsequent CSCN examination. All participants reported the OSCE as useful in examination preparation. The communication station was consistently ranked as the least useful station, an unsurprising finding given that the CSCN examination does not involve a communication component. Conclusions: OSCE is an effective tool in assessment of the level of competence of EEG/epilepsy fellows, and as preparation for the CSCN EEG examination.

Background: Assessment of patients for temporal lobe epilepsy (TLE) surgery requires multimodality input, including EEG to ensure optimal surgical planning. Often EEG demonstrates abnormal foci not detected on clinical MRI. 7T MRI provides improved resolution and we investigated its utility to detect potential abnormalities associated with EEG. Methods: Images were acquired on 7T MRI scanner (N=13) in oatients with TLE. Evaluation of 7T imaging for focal abnormalities was performed. Correlation of 7T MRI findings with EEG of focal slowing or interictal epileptic spikes (IEDs) and seizures was performed. Results: Assessment of 7T MRI demonstrated concordance with TLE in 8/13 cases. Three cases exhibited abnormal 7T MRI abnormalities not detected by 1.5 T MRI. Eleven out of 13 cases had EEG findings without anatomic correlates on MRI, with IEDs localizing to contralateral temporal, frontal, and parieto-occipital lobes. 7T images did not reveal focal anatomical abnormalities to account for the EEG findings in these patients. Conclusions: To our knowledge, this is the first study to investigate the role of 7T MRI in relation to EEG abnormalities. 7T RI findings show concordance with clinical data. 7T MRI did not reveal anatomical findings to account for EEG abnormalities, suggesting that such changes may be functional rather than anatomical.

Background: Rapid eye movement sleep (REM) is divided into phasic and tonic microstates. Phasic REM is defined by presence of REMs with reportedly greater antiepileptic effect. We assessed whether quantitative EEG (QEEG) software can detect REM microstates. Methods: We applied artifact reduction and detection trends from QEEG software (Persyst 14) on 18 patients undergoing 30 day-night high density EEG recordings in the epilepsy monitoring unit. We identified phasic REM as 10-second epochs of previously human-scored REM that demonstrated presence of either vertical or horizontal eye movements on the QEEG artifact detection panel. Remaining epochs were identified as tonic REM. Results: Out of 91.2 average minutes of REM (range 24.5-167.5) per recording, a mean of 2.5% (range 0-18.9%) demonstrated eye movements intensive enough for QEEG artifact detection to be identified as phasic REM. On average, only 40% (range 0-500%) of eye movements per recording was flagged as vertical. Conclusions: These findings provide proof-of-concept that QEEG can automatically assess REM microstructure by readily detecting phasic and tonic REM. These findings also confirm that most REMs are horizontal. Having the ability to easily and automatically detect phasic versus tonic REM can help further future studies examining the antiepileptic effect of REM sleep.

Background: Background: Responsive Neurostimulation (RNS) has proven efficacy in treating medically resistant epilepsy as an intracranial system detecting, recording and treating seizures automatically. No information exists pertaining to artifact characteristics of RNS findings in scalp EEG. Methods: A 30 year-old female was diagnosed using intracranial electroencephalography(iEEG), with bi-insular epilepsy, of unknown cause. She presented large number of focal unaware non-motor seizures and seizures with progression to bilateral tonic-clonic. She was implanted with bi-insular RNS. Results: During scalp EEG recordings, a prominent artifact was seen corresponding to an automatized discharge suspectedly evoked by the RNS trying to minimize the frequent epileptiform activity in her case. Figure 1 and 2 depict these findings. Conclusions: Artifact seen by the RNS in scalp EEG has not been previously described in scientific literature. These findings must be identified to better characterize the role of the RNS in EEG and treatment of seizure activity visible on scalp recordings.

Background: Standard of care treatment for adult intracranial ependymoma patients includes maximal safe surgical resection, while the role for adjuvant radiotherapy remains unclear with existing data from small retrospective series’. Accordingly, we built a multi-institutional cohort to assess the prognostic value of adjuvant radiotherapy and other clinical factors in these patients. Methods: Patients managed for adult intracranial ependymomas from 1968 onwards within the University Health Network in Toronto, The University of Oklahoma Health Sciences Center, and The Ottawa Hospital were identified. Multivariate models assessing the prognostic value of clinical factors were built using variables with known prognostic value and/or significance in univariate Cox models. Results: Of 122 ependymomas, 71% were infratentorial, 78% grade two, 55% gross/near-totally resected (GTR/NTR), and 65% treated with adjuvant radiotherapy. Multivariate analyses identified GTR/NTR (vs. subtotal resection; HR=0.2, 95%CI=0.1–0.4, p<0.0001) and cranial (HR=0.5, 95%CI=0.2–1.1) or craniospinal (HR=0.2, 95%CI=0.04–0.5) radiotherapy receipt (p=0.01) as independent statistically significant predictors of more favourable PFS. Grade II pathology (vs. grade III; HR=0.2, 95%CI=0.05–0.6, p=0.006) and GTR/NTR (vs. subtotal resection; HR=0.1, 95%CI=0.03–0.3, p=0.0001) were independent statistically significant predictors of better OS. Conclusions: This work confirms the importance of maximal safe resection for adult intracranial ependymomas and establishes that adjuvant radiotherapy improves progression-free survival in these patients.

Background: Nusinersen is approved for spinal muscular amyotrophy type I,II,III and is available for adult and pediatric populations since January 2019 at the CHU de Québec-UL. Methods: Patients who received at least one dose of nusinersen between January 2019 and March 2021 were included. Required information was gathered from patients’ charts. Results: Ten adults (70 injections) and 23 infants and children (202 injections) were included. No child but two adults stopped the therapy for personal reasons. Tests were performed at 0, 6, 18, 24 months when possible to assess efficacy, tolerability and quality of life perception (Table 1). Preliminary data shows stabilization or improvement for many tests. Conclusions: Nusinersen is well tolerated. Favorable effects were observed in both populations. A monitoring is still imperative for an objective assessment and for evaluation of the most relevant tests. Long term benefits remain to be demonstrated but results are encouraging.

Background: Simulation-based educations’ prevalence within clinical neuroscience is on the rise, however investigation into what environment is most conducive to optimizing learning performance is limited. We aimed to determine whether training a simple-to-complex (progressive) sequence would result in superior learning compared to complex-to-simple (mixed) or complex-only sequences. Methods: A three-arm, prospective, randomised experiment was conducted to determine the effects on novice learner LP performance and cognitive load during learning and a very complex simulated reality assessment test 9-11 days later. Results: During learning, sterility breaches decreased linearly over time (p<.01) with no group differences, and accuracy was higher in the progressive group compared to complex-only (p<.01) and trended in the mixed group (p<.09). Across the learning phase cognitive load increased in the progressive group (p<.01) and decreased across the mixed group (p<.01). At assessment, there were no group differences in number of sterility breaches (p=.66), needle passes (p=.68) or cognitive load (p=.25). Conclusions: Contrary to our hypothesis, equivocal assessment performance was found across groups. Our results suggest that successive progression in complexity of simulation does not increase novice learner outcomes. Further, a “one-size fits all” approach to simulated environment complexity in clinical neurosciences education may be warranted given equivocal learning and less resources necessary.

Background: Rotating internal medicine (IM) residents do not feel adequately prepared to approach patients with neurologic issues. The purpose of this project was to conduct a needs assessment to determine the optimal components and delivery of a neurology curriculum for internal medicine residents. Methods: We utilized a mixed-methods design and recruited participants through a combination of purposive and convenience sampling. We conducted interviews with IM residents (n=12) and focus groups with neurology residents (n=7) and neurology staff (n=8). IM residents completed entry- and post-call surveys while on a neurology rotation. Results: Themes according to Kern’s framework for curriculum development: 1. Problem: Discomfort and perception of under-preparedness amongst IM trainees 2. Needs Assessment: What the learners (stakeholders) think they need to know vs. what their teachers want them to know vs external requirements (Royal College) 3. Goals/objectives: What content is relevant for clinical requirements vs assessments? 4. Methods and setting: Didactic vs bedside vs virtual 5. Implementation of the curriculum 6. Evaluation and feedback Conclusions: Our findings illustrate a possible mismatch between internal medicine residents’ needs and neurologist teachers’ expectations in teaching neurology. Addressing learners’ needs could enhance neurology knowledge and sense of preparedness when encountering patients with neurologic issues.

Background: Mounting evidence supports aerobic exercise as a promising treatment option for individuals experiencing persistent post-concussion symptoms beyond four weeks. The purpose of this review was to determine whether initiating structured aerobic exercise within the early period (first two weeks) following concussion affects symptom recovery compared to standard care. Methods: A systematic literature search was performed using MEDLINE, Embase, PsycINFO and CENTRAL databases, combining keywords: (brain concussion, post-concussion syndrome, mild traumatic brain injury) AND (exercise, exercise therapy, physical activity, kinesiotherapy). Results were limited to Randomized Controlled Trials (RCTs). All selected articles underwent quality assessment. Results: The search generated 112 unique abstracts, of which 5 met inclusion criteria. Sample sizes ranged from 16 to 103 participants. Of the four studies that were able to analyze between-group differences, one showed significantly faster recovery in the early aerobic exercise group compared to control, while three showed no significant difference. Two studies demonstrated a trend toward faster initial symptom resolution in the early exercise group. Conclusions: Preliminary RCT evidence suggests that aerobic exercise initiated in the early postconcussion period does not exacerbate symptoms or prolong recovery time, and may potentially hasten recovery. Larger, more rigorous RCTs are required to define the optimal exercise parameters to facilitate symptom recovery.

Background: The circle of Willis (CoW) and cervical carotid arteries are important sources of collateral flow during acute large vessel occlusion (LVO) in the anterior circulation. We examined the anatomical components of the circle and the cervical carotid arteries to determine relationship to acute stroke severity. Methods: Consecutive patients with acute LVO who underwent EVT were assessed. Measurements were made of the luminal diameters of 16 anatomical vascular components. Admission NIHSS, ASPECTS and mCTA collateral scores were statistically analyzed for any relationship to vascular measurements. Results: 100 patients were studied. No relationship was found between the collateral Willisian pathways and measures of stroke severity. However, the ophthalmic arteries exhibited a relationship to stroke severity. In adjusted analysis, 1-mm increases in the ipsilateral and contralateral ophthalmic artery diameter were independently associated with a 4.80-point decrease and a 6.31-point increase in the NIHSS scale, respectively. Similarly, a 1.53-point increase and a 2.62-point decrease in the ASPECTS. In the neck a majority showed 0-55% stenosis, with no stenosis between 55% and 95%, and 14% at 95% to 100%. Conclusions: Stroke severity and collateral during LVO is unrelated to Willisian collateral. Ophthalmic artery calibers are related. Acute progression of 55-95% stenoses to complete occlusion occurs in LVO stroke

Background: Predicting outcomes after endovascular treatment (EVT) for acute ischemic stroke with baseline variables remains a challenge. We assessed the performance of stroke outcome prediction models for EVT in acute ischemic stroke in an iterative fashion using baseline, treatment-related and post-treatment variables. Methods: Data from the ESCAPE-NA1 trial were used to build 4 outcome prediction models using multi-variable logistic regression: Model 1 included baseline variables only that are available prior to treatment decision-making, model 2 included additional treatment-related variables, model 3 additional early post-treatment variables, and model 4 additional late post-treatment variables. The primary outcome was 90-day modified Rankin Scale score 0-2. Model performance was compared using the area under the curve (AUC). Results: Among 1,105 patients, good outcome was achieved by 666 (60.3%). When using baseline variables only (model 1), the AUC was 0.74 (95%CI:0.71-0.77); this iteratively improved when treatment and post-treatment variables were added to the models (model 2: AUC 0.77,95%CI: 0.74-0.80, model 3: AUC 0.80,95%CI:0.77-0.83, model 4: AUC 0.82, 95%CI:0.79-0.85). Conclusions: Predicting EVT outcomes using baseline variables alone is inaccurate in one in four patients, and may be inappropriate for patient selection. Even the most comprehensive models with treatment-related and post-treatment factors involve considerable uncertainty.

Background: MAiD became legal in Canada in 2015, with Bill C-14 delineating eligibility criteria and access. Previous research found families are intimately involved with decision-making, with conflicting perspectives on how they cope.

Our study sought to learn about the experiences of family members, and determine what supports might be beneficial to improve MAiD delivery and aftercare. Methods: We conducted hour-long semi-structured interviews with 20 family members of individuals who had MAiD. Interviews took place by telephone or virtually via MS Teams, and transcripts were analyzed using an iterative coding process and thematic analysis. Results: Prominent themes emphasized the importance of respecting autonomy, decision-making, and allowing people to regain a sense of control, particularly with so much taken away.

The death itself was described as peaceful. Interviewees were overwhelmingly filled with relief and gratitude for being able to respect the individual’s wishes.

Interviewees spoke of importance of support for themselves, and the desire to build a network of individuals with similar experiences; to share their stories, grieve together, and support the next generation. Conclusions: These results will help improve MAiD delivery and aftercare in Nova Scotia, by informing, developing and enabling access to resources for individuals who accompany a family member on their end-of-life journey.