Background: Under Competence by Design (CBD), there are required training experiences (TEs) and entrustable professional activities (EPAs) in the Transition to Practice (TTP) stage. Limited literature exists to support an evidence-based approach to its implementation and evaluation. We created a novel outpatient rotation for PGY5 neurology residents, simulating independent practice and addressing the TTP TEs. Methods: We conducted a needs assessment with informal interviews of senior residents, the program director, and program administrator of our neurology residency program. Guided by Royal College requirements, and available TTP-focused literature, we designed a general neurology clinic run by PGY5 neurology residents. Focuses included increased independence and efficiency, longitudinal follow-up, and applied principles of practice management. Results: Go-live was August 1, 2024. Eight PGY5 residents completed one block, with a second scheduled later in the academic year. Eleven supervisors participated across two sites. Surveys and structured interviews will be used for both groups to evaluate the program, based on the Kirkpatrick Model. Conclusions: Development of a dedicated clinic addressing the TTP TEs in CBD is feasible. Iterative evaluation of the structure, delivery and outcomes of this required TE is critical to ensure that objectives are met and value is added to the residency curriculum.

Background: Identifying white matter abnormalities after acute concussion is challenging due to variable microstructural changes and individual imaging limitations. Combining diffusion tensor imaging (DTI) and neurite orientation dispersion and density imaging (NODDI) improves sensitivity to alterations. This study integrates neuroimaging and behavioural assessments to improve detection and characterization of abnormalities for clinical management. Methods: We recruited 12 recently concussed athletes (21 ± 2.1 years, 7 ± 4.6 days post-injury; 9 completed behavioural testing) and 24 controls. All participants underwent DTI and NODDI to assess white matter integrity. Kinematic performance was evaluated using the Kinarm exoskeleton robot’s Reverse Visually Guided Reaching (RVGR) task. Group differences in imaging and kinematic metrics were analyzed using permutation-based and parametric tests, controlling for age and sex. Results: Concussed athletes had elevated fractional anisotropy, reduced mean and radial diffusivity, and lower isotropic volume fraction in affected tracts. However, no group differences emerged in RVGR parameters, indicating intact sensorimotor function despite imaging abnormalities. Conclusions: Our findings reveal that acute concussion leads to measurable microstructural changes without corresponding functional deficits on a cognitive inhibition task. These findings highlight the clinical utility of neuroimaging for early and precise diagnosis, emphasizing its sensitivity over behavioural measures to detect subtle impairment for acute concussion management.

Background: Studies have found similar rates of functional independence for men and women after endovascular thrombectomy (EVT). Less is known regarding EVT-related procedural complications and symptomatic intracerebral hemorrhage (sICH) between sexes. Methods: Using the OPTIMISE registry including data from 20 comprehensive stroke centers across Canada between 1/1/2018 and 12/31/2022, we performed a retrospective descriptive analysis of patients divided between men and women. Hemorrhagic transformation on follow-up imaging with associated clinical deterioration was required to define sICH. Results: 3631 patients were included (1778 men and 1853 women) for analysis. Female patients were older (71.8±14.6 vs 68.0±13.1 years, p<0.001). There were no differences in sICH rates (2.5% men vs. 2% women, p= 0.388}. Procedural complication rates were not different between men and women (5.8 vs 5.6% p=0.76): dissection {26 (1.5%) vs. 30 (1.6%), p=0.804}, perforation {11 (0.6%) vs. 7 (0.4%), p=0.426}, embolization {25 (1.4%) vs. 25 (1.3%), p=0.996} and arterial access complications {45 (2.5%) vs. 43 (2.3%), p=0.761}. Conclusions: In this large multicentre registry of stroke patients undergoing EVT, men and women had similarly low and reassuring rates of sICH and procedural complications. This complements previous data showing similar functional outcomes for men and women after EVT.

Background: The use of Tenecteplase (TNK) in Extended Time Window (ETW) for Acute Ischemic Stroke (AIS) remains an ongoing debate. Methods: Systematic review of 3 Randomized controlled trials (RCTs)- TIMELESS, TRACE 3, CHABLIS-T II was conducted. Results: 1198 patients were enrolled: 603 received TNK, while 595 were controls. All 3 trials included patients with Internal Carotid and/ or Proximal Middle Cerebral Artery Occlusions; however, in TRACE 3, patients did not have access to endovascular thrombectomy. TIMELESS and CHABLIS-T II showed better recanalization in the TNK group but the median Modified Rankin Score was 3 at 90 days in both groups, demonstrating no benefit in clinical outcomes. Symptomatic Intracranial hemorrhage (sICH) was similar in the two groups. In TRACE 3, there was an improvement in functional outcomes at 90 days in the TNK group (33.0% vs. 24.2%), but the incidence of sICH was also higher (3.0% and 0.8%, respectively). Conclusions: Better recanalization rates are seen with TNK in ETW, but may not be associated with improved functional outcomes at 90 days compared to medical management. Incidence of sICH also remains largely favorable, except in TRACE 3, which showed a higher incidence in the TNK group. There remains a need for more RCTs in this population.

Background: Spinal cord injury (SCI) is a central nervous system injury that often leads to motor, sensory and autonomic dysfunction. Non-invasive trans-spinal electrical stimulation (ts-ES) has been shown to activate neural networks below the injury and improve motor function recovery after SCI. The objective of this study was to compare changes in motor and autonomic function attributable to ts-ES in individuals with incomplete SCI after 4 weeks of personalized training. Methods: Participants received 4 weeks of treadmill training with personalized step-cycle based PNS and FES with and without non-invasive lumbar ts-ES. Clinical outcome measures of motor function (2-minute walk test, Berg Balance and modified SCIM-Mobility) and metabolic analysis (heart rate and rate of oxygen consumption (VO2 sub-max)) were assessed before and after training. Non-invasive electromyography (EMG) and kinematic data assessed motor function. Results: Based on participant feedback and data, ts-ES with PNS/FES during training was tolerable, improved leg movement and facilitated muscle activity in knee extensors with 10-25% increased RMS amplitude of pre-training EMG activity during both forward and backward walking. Moreover, ts-ES tended to increase HR and VO2 sub-max within one session. Conclusions: Personalized rehabilitation strategies combining ts-ES with traditional physiotherapy exercises and locomotor training have the potential to improve recovery after SCI.

Background: COMISA (comorbid insomnia and obstructive sleep apnea) is associated with daytime functioning and cognitive impairments. This post hoc analysis assessed the impact of lemborexant (LEM), a dual-orexin-receptor-antagonist approved to treat insomnia in adults, on morning sleepiness/alertness in participants with COMISA. Methods: Of the overall population (n=1006), 410 (40.8%) adults (≥55 years) with comorbid insomnia disorder and mild obstructive sleep apnea (apnea-hypopnea-index, 5–≤15 events/h) from Study E2006-G000-304 (NCT02783729), a 1-month, randomized, placebo- and active-controlled study, were analyzed. Participants received placebo (PBO), LEM 5mg (LEM5), LEM 10mg (LEM10), or zolpidem tartrate 6.25mg (not reported). A daily sleep diary assessed morning sleepiness/alertness (1, extremely sleepy to 9, extremely alert). Participants (%) shifting from baseline mild/moderate sleepiness (≤3) towards greater alertness (4, 5, or >5) during the first/last 7 mornings of the study were analyzed. Results: At baseline, 17/75 (22.7%), 36/112 (32.1%), and 28/104 (26.9%) participants with COMISA receiving PBO, LEM5, or LEM10, respectively, reported mild/moderate sleepiness. Across the first/last 7 mornings, more participants shifted from mild/moderate sleepiness towards alertness with LEM5 (66.7%, 82.9%) and LEM10 (64.3%, 75.0%) versus PBO (47.1%, 64.7%), respectively. Conclusions: A greater percentage of participants with COMISA experienced improvements in morning sleepiness across the treatment period with LEM versus PBO.

Background: The consistency of effects of lemborexant (LEM), a dual orexin-receptor antagonist, on sleep maintenance variables across 2 phase 3 studies with contrasting populations was compared. Methods: E2006-G000-304 (Study 304; NCT02783729) and E2006-J086-311 (Study 311; NCT04549168) were 1-month, randomized, double-blind, placebo (PBO)-controlled studies evaluating LEM 10mg (LEM10) in adults with insomnia disorder. Global Study 304 (N=1006; PBO, n=208; LEM10, n=269) enrolled participants of any race (≥55y); Study 311 (N=193; PBO, n=100; LEM10, n=93) participants were exclusively Chinese (≥18y). Pairs of polysomnograms were conducted at baseline and after the first/last 2 doses of the 1-month treatment. Change from baseline in sleep efficiency (SE [%]), wake-after-sleep-onset (WASO [min]), and total-sleep-time (TST [min]) were analyzed. Results: Mean baseline sleep parameters: Study 304: SE, 67.9–68.9; WASO, 111.8–114.8; TST, 325.1–330.7; Study 311: SE, 69.4–70.3; WASO, 79.3-–85.8; TST, 333.2–336.7. Least squares mean [standard error] increases from baseline were significantly larger with LEM10 vs PBO (P<0.001) for SE (Study 304, 8.0 [0.7]; Study 311, 7.1 [1.4]) and TST (38.9 [3.7]; 32.8 [6.9]), as were decreases in WASO (-25.4 [3.1]; -17.8 [4.8]). Most treatment-emergent adverse events were mild–moderate. Conclusions: Short-term LEM10 treatment consistently improved objective sleep maintenance in patients with insomnia of different races.

Background: Accessible ambulatory neurology care can reduce the need for inpatient evaluation. Aligning patient demand (service requests) with provider and space resources can optimize ambulatory clinic flow. In response to increasing referral volumes and wait times for neurologist access, a quality improvement initiative was undertaken to address demand. Methods: Process mapping and root cause analysis demonstrated access challenges and referral processing errors. Audit of 968 accepted referrals revealed variation in triage processes and decisions for referral questions. Neurologists defined inclusion criteria to specialty programs, based on referral questions. Referral management transitioned to a central intake model, reducing intra- and inter-clinic triage variability. Guidelines were established to prevent triage duplication and standardize appointment management. The primary outcome was accepted referrals per month. Secondary outcomes were referral rejection rate and neurology wait times. Results: Significantly more referrals were received per month post intervention (987 vs. 859, p<0.000). The number of accepted referrals did not change (p=0.147). Referral rejection rate increased from 21% to 31 % (p<0.000). Wait times increased by 16% (p=0.003). Conclusions: Referral management helped respond to increased referral requests. Despite no change in accepted referrals, wait times increased, suggesting a significant capacity problem and focus for further work.

Background: Efgartigimod, a human immunoglobulin (Ig)G1 antibody Fc fragment, blocks the neonatal Fc receptor, reducing IgGs involved in chronic inflammatory demyelinating polyneuropathy (CIDP). The multi-stage, double-blinded, placebo-controlled ADHERE (NCT04281472) and open-label extension ADHERE+ (NCT04280718) trials (interim analysis cutoff: February 16, 2024) assessed efgartigimod PH20 SC in participants with CIDP. Methods: Participants with active CIDP received open-label, weekly efgartigimod PH20 SC 1000 mg during ≤12-week run-in (stage-A). Responders were randomized (1:1) to efgartigimod or placebo for ≤48 weeks (stage-B). Participants with clinical deterioration in stage-B or who completed ADHERE entered ADHERE+. Week 36 changes from run-in baseline (CFB) in adjusted Inflammatory Neuropathy Cause and Treatment (aINCAT), Inflammatory Rasch-built Overall Disability Scale (I-RODS), and grip strength scores were evaluated. Results: Of 322 stage-A participants, 221 were randomized and treated in stage-B, and 99% entered ADHERE+. Mean CFB (SE) in aINCAT, I-RODS, and grip strength scores were -1.2 (0.15) and 8.8 (1.46) and 17.5 (2.02), respectively, at ADHERE+ Week 36 (N=150). Half the participants with clinical deterioration during ADHERE stage-B restabilized on efgartigimod from ADHERE+ Week 4. Conclusions: Interim results from ADHERE+ indicate long-term effectiveness of efgartigimod PH20 SC in clinical outcomes in participants with CIDP.

Background: Neurofibromatosis 1 (NF1) is a multisystem neurocutaneous disorder. Treatment involves multiple specialists. There are currently no multidisciplinary clinics for adults with NF1 in BC, which impacts communication between subspecialties. We sought perspectives of patients and providers to identify the impact of and solutions to gaps in care. Methods: Focus groups with patients (2 groups; 9 patients) and physicians (10) who see people with NF1 were conducted. Thematic content analysis was applied to the data to derive major themes. Concurrently, quarterly NF multidisciplinary rounds were initiated to enhance coordination of care. Results: Major themes emerged around the need for increased coordination and communication amongst providers. Specifically, physicians identified working in “siloed care structures”, and patients and providers identified lack of awareness of expertise and barriers to accessing care. Conclusions: Focus groups enable inclusion of patient and provider perspectives in developing solutions to gaps in care. The importance of supporting interdisciplinary communication in caring for NF1 patients was confirmed in focus groups. To date, we have held multidisciplinary NF rounds, with 12 cases discussed. Disciplines represented include neurology, pediatrics, radiology, neuro-ophthalmology, neuro-otology, pathology, orthopedic plastic and neurosurgery, medical and radiation oncology, and the hereditary cancer program. Telehealth format enables participation from distributed centres across BC.

Background: Informed consent is not always possible in emergency research particularly during life threatening situations. Deferral of consent is an acceptable method in consenting patients; however, it is underutilized. We aim to share our experience with deferred consent. Methods: Participants in two prospective studies underwent a CT-Perfusion scan (intervention) at the time of first hospital imaging, in order not to impact clinical treatment. Deferred consent was then obtained. The primary outcome was the rate of deferred consent. The number of days to obtain consent, refusal rate, and waiver of consent rate was also reported. Results: A total of 291 patients (200 severe traumatic brain injury [TBI] and 91 out-of-hospital cardiac arrest) were enrolled between the two emergency CT-perfusion studies. Some (34/291[11.9%]) could not be reached; waiver of consent was granted by our ethics board. Deferred consent was obtained in 252/291(86.6%). The majority were consented by the partner/spouse (25.2%) and most consents took place within 7-days (76.0%) of enrollment. Five (1.7%) refused consent. Deferred consent rates were higher in the cardiac arrest population (97.8%) compared to the severe TBI population (83.7%). Conclusions: Deferred consent is an acceptable method of obtaining consent in emergency research when the intervention risk is low.

Background: Postural Orthostatic Tachycardia Syndrome (POTS) is the increase in heart rate (HR) by ≥30 bpm within 10 minutes of upright posture without orthostatic hypotension (OH). This study specifcally investigated patients who develop delayed symptomatic orthostatic tachycardia (i.e. after >10 minutes) without OH. Methods: Clinical histories and laboratory tests of patients assessed for orthostatic intolerance (OI) during last 10 years were reviewed. All patients underwent autonomic tests (sweat test, heart rate variability to deep breathing/Valsalva, 45-minute head up tilt table test (TTT) and quantitative sensory testing. Results: Among 974 patients with OI, 43% (419/974) were diagnosed with POTS whereas 12.4% (121/974) had delayed orthostatic symptoms on TTT alongwith delayed orthostatic tachycardia (DOT; x¯ = 30.3 minutes) without OH. In this cohort, mean HR increase was 51.7 bpm (range: 40-104 bpm). Other findings were significant narrowing of pulse pressure (PP) i.e. ≤25% of systolic pressure (x¯ = 16.2%, range: 6.4% -24.3%), excessive BP oscillations and syncope (9.9%). About 1/3 (37.2%) with DOT had definite small fiber/autonomic neuropathy. Conclusions: Patients with OI may manifest delayed symptoms/DOT and maybe missed on a 10-minute TTT. The marked reduction in PP observed in these pateints signifies reduced cardiac output, possibly from peripheral blood pooling due to small fiber/autonomic neuropathy.

Background: Glial fibrillary acidic protein (GFAP), a brain-specific biomarker, shows promise in differentiating intracerebral hemorrhage (ICH) from acute ischemic stroke (IS) and stroke mimics (SM). A novel point-of-care platform measures GFAP in minutes, yet requires centrifugation to obtain plasma. We aim to determine whether participants recruited in an ongoing prospective biomarker study (during working hours) differ from non-recruited patients. Methods: An exploratory analysis of undifferentiated stroke <24h from onset, where plasma GFAP levels (pg/ml) are measured (i-STAT Alinity) at hospital arrival. Clinical characteristics are compared among recruited and non-recruited patients. Results: Among the first 101 patients recruited, mean (±SD) age (70.8±14.5 years), % females (48%), and median (IQR) NIHSS (9(3-20) were similar to the 270 non-recruited patients (70.3±16.3 years, 51% females, NIHSS 7 (3-17), respectively) in the same time period. Median ASPECTS was slightly lower in recruited patients (10(9-10) vs (10(10-10)) (p=0.03). ICH and SM were more common among non-recruited (52% IS/13% ICH/32% SM) compared to recruited patients (67% IS/5% ICH/29% SM, p=0.002), while large-vessel occlusion was more common among those recruited (44% vs 19%, p=0.001). Conclusions: Clinical characteristics do not differ among recruited vs. non-recruited patients in an ongoing biomarker study, yet sampling bias exists regarding underlying stroke condition, with efforts to mitigate this going forward.

Background: Presence of right-to-left shunt has been proposed as a mechanism of paradoxical embolism in patients with active cancer. Our study thus aims to investigate the role of shunting in stroke occurrence among cancer patients. Methods: This is a retrospective study with our population consisting of patients presenting to the Ottawa Hospital with ischemic stroke between January 01, 2020, and December 31, 2022. Presence of right-to-left shunting is identified in patients with and without cancer diagnosis within one year of ischemic stroke. The prevalence of shunt is assessed using 95% confidence intervals (CI). Results: Among654 patients, 495 (37% female, median age 53 years) were included in the study, in which 47 (9.5%) had active cancer, with 12 patients (25.5%, 95% CI 14 - 40) diagnosed with a shunt. In contrast, among 448 patients (90.5%) without active cancer, 133 patients (30%, 95% CI 25 - 34) were found to have a shunt. Conclusions: The prevalence of right-to-left shunting tends to be lower in patients with ischemic stroke and active cancer diagnosis. Our results are similar to a recent study indicating a higher rate of shunt among patients without cancer. Our finding does not support the hypothesis that cancer-associated stroke is related to right-to-left shunting.

Background: Telemedicine evaluation for treatment of acute stroke patients with IV thrombolysis has been shown to be beneficial. Its usefulness for the evaluation of patients transferred from a primary stroke centre (PSC) to a comprehensive stroke centre (CSC) for endovascular thrombectomy (EVT) is less well defined. Methods: We retrospectively analyzed the Canadian OPTIMISE registry which included data from 20 comprehensive stroke centers across Canada between January 1, 2018, and December 31, 2022 to compare treatment metrics and early outcomes between two groups: patients evaluated by telemedicine (TM) and patients evaluated in person (non-TM) at the PSC prior to CSC transfer. Results: We included 3317 patients who were transferred from a PSC to a CSC for: 888 TM and 2429 non-TM. There were no major differences in baseline characteristics, including intravenous thrombolysis administration, though the TM group included more men. TM patients had longer onset-to-puncture times (441 vs 403 minutes, p<0.001) and higher symptomatic intracerebral hemorrhage (sICH) rates (7.4% vs 3.7%, p<0.001), but CSC door-to-puncture times and successful recanalization rates did not differ. Conclusions: Patients transferred to a CSC for EVT first evaluated by TM had similar characteristics to those evaluated in person at the PSC, but longer onset-to-puncture times and higher sICH rates.

Background: Referrals to the Stroke prevention clinic with incomplete preliminary investigations decrease clinic capacity due to additional workload and the need for follow-ups. We aimed to improve the efficacy of the initial visit by increasing the completion rate of vascular imaging. Methods: Pre-post quasi-experimental study with three phases: Phase 1: Surveillance; Phase 2: Stakeholder feedback-informed intervention development (physicians and clinic staff); and Phase 3: Implementation. Interventions included a new referral order within the provincial EMR; a specific physician triage form listing required investigations (brain imaging, vascular imaging, cardiac tracing); and a nurse-led pre-visit via telephone. The primary outcome measure was the completion of vascular imaging - assessed with multivariable logistic regression Results: The study’s inclusion criteria were met by 383 patients, mean age of 67.6±13.2 years; 49% were female, 62.5% were diagnosed with vascular events. An increase in vascular imaging before the initial visit was found in Phase 3 (139/184, 75.5%) compared to Phase 1 (121/198, 61.1%, Odds ratio 1.96 95% CI 1.3-3.1; p=0.003). Fewer follow-up visits were required in Phase 3 (22.8%) compared to Phase 1 (31.8%, p=0.049). Conclusions: A uniform referral process, a standard triage process, and a nurse-led pre-visit may improve the completion of essential investigations before the patient visit.

Background: Tenecteplase is a genetically-modified variant of the tissue plasminogen activator alteplase, with increased fibrin-specificity, administered as a more convenient intravenous bolus. Recent data, from the AcT trial, have shown tenecteplase to be non-inferior to alteplase in patients with acute ischemic stroke (AIS) treated within 4.5 hours from symptom onset, the direction of effect favoring tenecteplase. As a result, the Heart and Stroke Foundation of Canada has added tenecteplase to the Stroke Best Practice Recommendations. However, its cost-effectiveness in the Canadian setting remains unknown. Methods: An analysis was performed to estimate the cost-effectiveness of tenecteplase compared to alteplase in the AIS population. The model structure combines a decision tree for the first 90 days post index stroke, where the 7 modified Rankin Scale (mRS) states are informed by the AcT trial, and a Markov model for the remainder of the lifetime horizon. Cost and utility values were derived from the literature and public sources. Canadian health care system and hospital perspectives were used. Results: This economic analysis demonstrates that tenecteplase is dominant compared to alteplase, providing more quality-adjusted life years at lower costs. Conclusions: Adding tenecteplase to hospital formularies for AIS would generate savings for the health care system while providing more benefits.

Background: Ischemic stroke often results in long-term motor impairments due to disrupted corticospinal pathways. Transcranial magnetic stimulation (TMS) motor mapping is a non-invasive technique used to assess corticospinal integrity by measuring motor evoked potentials (MEPs). This study investigates whether MEP amplitudes can predict impairment severity and functional performance in chronic stroke. Methods: Four non-human primates (NHPs) with chronic stroke (> six months) following transient right middle cerebral artery occlusion underwent TMS motor mapping using neuronavigation under ketamine anesthesia. Single pulses of TMS (50-70% of maximum stimulator output) were applied to the affected and contralesional primary motor cortices to elicit MEPs and assess cortical excitability. Intramuscular electromyography recorded muscle responses from the biceps, extensor digitorum longus, and abductor pollicis brevis. Neurological dysfunction was evaluated daily for three weeks using the NHP Stroke Scale, NHP Upper Extremity Motor Dysfunction Scale, and the primate Rankin Scale. Results: MEPs were present in NHP1, NHP3, and NHP4 but absent in NHP2. Stronger MEPs correlated with lower impairment severity and better functional performance, while NHP2 exhibited higher impairment and poorer performance. Conclusions: MEP presence and strength can serve as biomarkers of motor recovery potential, highlighting their role in assessing corticospinal integrity and functional outcomes.

Background: Absence epilepsy is a common epilepsy syndrome in children. This can have a negative impact on the cognitive abilities of preschool and school-age children. The objective was to study in the Guinean context, the epidemiological, clinical, electrophysiological, therapeutic and evolutionary aspects of this syndrome. Methods: The study included all children diagnosed with absence epilepsy based on evidence obtained from history, clinical, and electroencephalogram. Results: The cohort was made up of 41 girls and 28 boys with a sex ratio (F/M) equal to 1.46. The mean age was 8 ± 2 years with extremes of 2 and 14 years. The simple absences were observed in 42.02% of cases. The components : tonic was associated in 11.59%, clonic in 10.14%, atonic in 13.04%, automatisms in 15.94% and vegetative in 7.25%. EEG was typical in 75.36%. As monotherapy, sodium valproate was used in 92.75% and ethosuximide in 2.9%. The evolution was marked by a remission of seizures in 85.51%. During follow-up, the appearance of tonic-clonic convulsions was noted in 4.3%, myoclonus in 2.9%, a combination of myoclonus and tonic-clonic convulsions noted in 4.3%. Conclusions: Effective and efficient collaboration between stakeholders is essential for the best overall management of this syndrome with serious cognitive repercussions in children.

Background: Pathogenic variants in SYNGAP1causedevelopmental and epileptic encephalopathy (DEE) and intellectual disability. Seizures are medically refractory and there is limited evidence on the use of corpus callosotomy (CC) and vagal nerve stimulation (VNS). Methods: A retrospective study was completed examining the effectiveness of VNS and CC in children with SYNGAP1-DEE using the SynGAP Research Database and an additional child followed at our centre. Results: Fifteen patients from the SynGAP Database were included. Of those who had VNS (n=11), 7 children had an >50% reduction in seizure frequency (n=7/11, 64%), 2 had worsening (n=2/11, 18%), 1 had no change (n=1/11, 9%), and 1 had an unknown response (n=1/11, 9%). Two children had CC only, 1 had complete seizure freedom, and 1 had a >50% reduction. Two children underwent VNS and CC, 1 had a >50% reduction in seizure frequency and the other had no change. One child followed at our centre experienced a sustained >80% reduction in seizure frequency following CC (i.e., after 1.5 years). Conclusions: We provide the first in-depth description of the response to VNS and CC in children with SYNGAP1-DEE, and provide insight into the use of of palliative surgical procedures in this population.