Background: Quality control indicators for mass lesion in TBI use the delay between emergency department (ED) and OR arrival to measure quality of care. It does not provide the timing of brain decompression. The goals of this study are to observe step by step where delays occur from hospital admission until effective decompression of the brain. Methods: A prospective observational data collection of timing from ED admission to decompression was conducted for all emergency trauma craniotomies over a period of 15 months. Results: Sixty-five patients were included. Doing a CT at the outside institution instead of transferring the patient prior to CT resulted in a 112min delay in care. Neurosurgery team notification prior to patient’s arrival to ED shortened delivery of care by 51min. The time elapsed between OR arrival and brain decompression was 50min: anesthesia time 3min, surgical positioning/preparation 29min and surgical time 17min. Burrhole decompression followed by craniotomy (9min) shortened the decompression time by 17min compared to standard 4 holes craniotomy approach (26min). Conclusions: Benchmark for trauma system performance in emergency craniotomies should be door to decompression time. Bypassing CT in local hospitals, pre-alerting neurosurgeons, and burrhole decompression followed by standard craniotomy significantly decrease door to decompression time.

Background: The ketogenic diet (KD) is used to treat severe childhood-onset epileptic encephalopathies, such as Infantile Spasms (IS). Unfortunately, limited resources for KD initiation result in treatment delays. We ask if earlier KD treatment of early-onset drug-resistant epilepsy results in better seizure outcomes. Methods: Children who started KD before age 4 years between 2000-present at SickKids Hospital were identified. Six-month seizure outcome was calculated as percent of pre-diet baseline seizure frequency (BSF). Results: 67 children were identified. 30 (44.8%) started KD <2 years old, 37 (55.2%) started KD 2-4 years old. Among <2 years old group, 83.3% achieved 50% reduction in BSF and 36.7% achieved 90% reduction. Among 2-4 year old group, 62.2% achieved 50% reduction in BSF and 24.3% achieved 90% reduction. 38 children had a history of IS; 17 with IS at diet initiation and 21 with past history of IS. 41.2% of the spasms cohort achieved 90% reduction in BSF, compared to 23.8% of the post-spasms cohort. Conclusions: KD was more effective when started before age 2 years than 2-4 years, and more effective in children with IS than in children with past history of IS. A rapid protocol for KD initiation in young infants and children may improve long-term outcomes

Background: Sturge Weber Syndrome (SWS) patients at risk of epilepsy are often not identified before their first seizure which leads to unnecessary follow up of many patients with facial angioma. Methods: The medical photography database of our institution has been reviewed to identify SWS patients followed between 1993 and 2013. Patients with isolated glaucoma were compared to patients with epilepsy regarding the location of the facial angioma, the presence of asymmetrical background activity on EEG done prior epilepsy onset and cerebral imaging. Logistical regression tests and a p-value of 0.05 were used. Results: 21 patients with SWS have been identified. No significant difference was noted when patients were compared based on the laterality of the lesion (p=0.169), or the location of the facial angioma (p = 0.314 to 0.999). Only 2 epileptic patients had digital EEG done prior the onset of epilepsy and only 2 patients with glaucoma had digital EEG done during their follow up. No significant difference was noted between EEG background activities in the two groups (p= 0.514). The presence of venous drainage anomalies (VDA) predicted (p = 0.004) the onset of epilepsy. Conclusions: Cerebral VDA increases the risk of epilepsy in SWS patients. Since they can be detected at birth, they might guide the management.

Background: Down syndrome (DS) is the primary genetic cause of mental retardation and seizures are present in an estimated 5-13% of cases. One-third of seizures in DS are infantile spasms (IS). Hypsarrythmia (HS) is the cardinal electroencephalogram (EEG) feature of IS and has been found to affect cognition; however, its effect on DS patients is inconclusively reported. This study assesses the correlation of HS with cognitive outcomes in DS using the largest sample size to date. Methods: Retrospective study of medical records of children with DS [0-18yrs] at SickKids Hospital in Toronto, from 1990-2013. Seizure history, EEG findings, comorbities, and pharmacological treatments were identified. Developmental outcomes were also assessed from physician comments on motor, verbal and cognitive abilities. The cognitive outcomes of DS patients with and without HS were compared. Results: 70 [male=40] patients with DS and seizures were included. Among 31 (44.2%) patients with DS and IS, 27 had HS. Chi-square analysis showed a significant difference [P=0.007] in prevalence of severe developmental delay in patients with IS and HS versus all other seizure types. Conclusions: The developmental outcome of patients with Down syndrome appears to worsen when IS and HS had occurred in the first year of life.

Background: NCSE dramatically increases morbidity and mortality. It is clinically subtle, which makes it difficult to diagnose without EEG. StatNet EEG provides a quick alternative to conventional EEG, which is often unavailable after hours. Methods: Each patient received a StatNet EEG by a neurology resident and a conventional EEG by an EEG technologist. Blinded studies were compared for delay between the studies, setup time, artifact, and abnormality detection using conventional EEG as controls. Nonparametric Mann-Whitney two-sample T-test was used. Results are expressed in mean minutes +/- SD. Kappa score assessed inter-observer reliability. Results: 19 patients were collected. Two StatNet EEGs were not interpretable and excluded. The mean delay between studies was 26h53min. Electrode placement is significantly shorter: 13:14±5:24 StatNet EEG vs 18:07±5:35 conventional EEG (p=0.02).

Conclusions: There is high inter-rater reliability between the conventional and StatNet EEG groups (table 1) demonstrating that StatNet is a reliable and effective tool, aiding in early recognition and management of NCSE.

Background: Leucine-rich glioma inactived-1 (LGI1) antibodies are associated with limbic encephalitis and distinctive seizure types, which are typically immunotherapy-responsive. While nonspecific EEG abnormalities are commonly seen, specific EEG characteristics are not currently understood to be useful for suspecting the clinical diagnosis. Based on initial observations in two patients, we analyzed the EEG recordings in a larger series of patients and describe a novel ictal pattern that can suggest the diagnosis of LGI1-antibody mediated encephalitis, even in the absence of common clinical features. Methods: Clinical and EEG data were collected in nine patients with LGI1 antibodies. Results: Psychiatric and cognitive symptoms were common, as were tonic seizures associated with EEG electrodecremental events (often with the so-called faciobrachial dystonic semiology). A rarity or absence of interictal epileptiform discharges contrasted with frequent subclinical temporal lobe seizures in some patients, which at times showed characteristics similar to subclinical rhythmic electrographic discharges of adults (SREDA), including sensitivity to hyperventilation. Conclusions: LGI1-antibody mediated encephalitis may be associated with tonic seizures and corresponding electrodecremental events, as well as an unusual SREDA-like pattern of frequent subclinical temporal lobe seizures, which may be triggered by hyperventilation, all in the setting of rare interictal epileptiform discharges.

Background: Exploration of the insular cortex is now commonly considered in patients with refractory epilepsy requiring invasive EEG investigations. The safety and yield of routine insular exploration is uncertain. Methods: All patients (pediatric and adult) who had invasive EEG (iEEG) with insular depth electrode placement, either through SEEG or open implantation, were reviewed. Ictal insular involvement was characterized as primary, secondary or not involved. Results of insular resections were recorded. Results: A total of 173 patients had iEEG of which 26 included insular electrodes (SEEG-18, Open - 8). No complications of placement were identified. Insular involvement was seen in 20 (76%) patients. Primary ictal involvement was identified in 9 (33 %) patients, while secondary spread was noted in 11 (42 %) patients. Six patients went on to have resections including the insular cortex of which 5 patients achieved good seizure control (Engle class I/II). Conclusions: Insular depth electrode placement is a safe and effective adjunct to invasive EEG investigations. Ictal involvement of the insular cortex was commonly identified in our series leading to inclusion of the insula in cortical resections with good seizure control, which may not have been considered without iEEG evidence.

Background: There are currently no national standards for clinical electromyography (EMG) training for residents in neurology and physiatry in Canada. The purpose of this study was to obtain demographic and qualitative data pertaining to EMG residency training in Canada, with the goal of facilitating discourse that could lead to national standards for EMG training. Methods: An online survey was distributed to senior neurology and physiatry residents (post-graduate years 3-5), at seven tertiary Canadian centres. The study authors, who are trainees and consultants with a broad range of EMG expertise (junior and senior resident, clinical neuromuscular fellows, senior physiatrist and neuromuscular neurologists), developed pertinent demographic and qualitative questions. Results: Thirty-eight residents completed the survey (23 neurology, 15 physiatry). There was inter-program variation in quantity of the training experience, content of the curriculum, access to expertise (including technologists) and goals for future training and practice. Similarly, differences were identified between the training experiences of neurology and physiatry residents. Conclusions: Inter-program variability in EMG training was identified. Additionally, differences were identified between neurology and physiatry resident training. This data provides evidence of training discrepancies across the country and can be used to establish national training standards for EMG in Canada.

Background: Ataluren is the first drug to treat the underlying cause of nmDMD. Methods: ACT DMD is a Phase 3, randomized, double-blind study. Males 7-16 years with nmDMD and a screening six-minute walk distance (6MWD) ≥150 m and <80%-predicted were randomized to ataluren 40 mg/kg/day or placebo for 48 weeks. A pre-specified subgroup included patients with baseline 6MWD 300-400 m. A meta-analysis of the overall ACT DMD population and the ‘ambulatory decline phase’ subgroup of the Phase 2b study (those patients meeting ACT DMD entry criteria) was pre-specified in the statistical plan. Results: In the overall ACT DMD population (N=228), changes in TFTs favored ataluren over placebo: 10-meter walk/run, -1.2s (p=0.117); 4-stair climb, -1.8s (p=0.058); 4-stair descend, -1.8s (p=0.012). In the pre-specified subgroup (n=99), these differences increased to -2.1s, -3.6s, and -4.3s, respectively, and were statistically significant (p<0.01) for 4-stair climb and descend. Results are supported by the meta-analysis (N=291), which demonstrated significant differences (p<0.05) in 10-meter walk/run, 4-stair climb, 4-stair descend. Conclusions: TFT results showed a benefit for ataluren in ACT DMD, and a larger treatment effect in the pre-specified baseline 6MWD 300-400 m subgroup as well as the pre-specified meta-analysis of ACT DMD and the Phase 2b study decline subgroup.

Supported By: PTC Therapeutics Inc.

Background: Improved respiratory care of Duchenne muscular dystrophy (DMD) patients has unmasked cardiomyopathy as a major source of morbidity and mortality. There is currently no consensus regarding the management of DMD-associated cardiomyopathy (DMD-CM). The objective of this systematic review was to evaluate the efficacy of pharmacological therapies for prevention and management of DMD-CM, and determine the optimal timing to commence these interventions. Methods: A systematic search was conducted in October 2015 and updated in January 2016 using MEDLINE, EMBASE and CINAHL databases and 9 grey literature sources for studies evaluating the use of angiotensin-converting enzyme inhibitors (ACEi), angiotensin receptor blockers (ARB), beta-blockers (BB) or aldosterone antagonists (AA) in DMD patients. References of retrieved records were searched. Quality assessment was conducted using the Downs and Black Quality Assessment Checklist. PRISMA reporting guidelines were used. Results: The 11 included studies were of low methodological quality. However, the use of an ACEi, ARB, BB and AA tended to improve or preserve left ventricular systolic function and delay the progression of cardiomyopathy. Conclusions: While there is evidence supporting the use of heart failure medication in patients with DMD-CM, data regarding these interventions for delaying the onset of DMD-CM and when to initiate therapy is lacking.

Background: Acute flaccid paralysis (AFP) is notifiable in Canada with a differential diagnosis that includes a number of conditions. This analysis describes the epidemiology of AFP in Canadian youth less than 15 years old. Methods: Monthly active surveillance for AFP was conducted as part of the Canadian AFP Surveillance System. Results: From 1996 to 2014, 850 cases of AFP were reported, representing an average annual crude incidence rate of 0.77 cases per 100,000 youth less than 15 years old. The mean age of cases was 6.8 years (median 5.9 years). Nine percent had an abnormal neurological history and 53% had an acute respiratory illness within 30 days of onset. Fever occurred in 23% of cases, 96% experienced bilateral weakness, 21% had respiratory muscle involvement, and 26% had cranial nerve involvement. The average hospital length of stay was 13.5 days. The most common diagnoses were Guillain-Barré Syndrome (GBS) or a variant (70%), and transverse myelitis (TM, 14%). At the time of the initial report, 14% had fully recovered. Conclusions: Our AFP surveillance system provides a baseline for AFP and its causes in the Canadian paediatric population. While rare, AFP is associated with severe morbidity in youth. GBS and TM were the most common diagnoses.

Background: Cerebral Palsy (CP) is a neurological condition arising from a perinatal or intra-uterine stroke. In the past 25 years there has been a revolution in neonatal care. For over 40 years children with CP in and around Saskatoon have been treated through the Kinsman Childrens’ Centre (KCC). This is a unique population database covering all CP patients in the region. We analyzed the KCC database to determine if the recent changes in neonatal care were correlated with the incidence of CP co-morbidities. Methods: A retrospective study using a Saskatchewan database of cerebral palsy data from the last four decades. Results: Over the last 40 years the incidence of visual disturbance and diagnoses of epilepsy in children with CP have remained stable regardless of advances in neonatal care. However, incidences of spine and hip issues requiring orthopedic intervention have halved. Conclusions: We hypothesize that advances in neonatal care have been successful in decreasing the incidence of gross motor impairments however have yet to significantly impact impairments relating to cortical network function. Although improvements in care have resulted in a decreased burden of disability, there remains opportunity for further improvements, especially in the settings of epilepsy and long-term visual function.

Background: Stroke leads to a substantial societal economic burden. We aimed to characterize productivity and factors associated with employability in Canadian stroke survivors. Methods: We used the Canadian Community Health Survey (CCHS) 2010-2011 to identify stroke survivors and employment status. We used multivariable models to determine the impact of stroke on employment and factors associated with employability. We used the Heckman model to estimate the effect of stroke on productivity (number of hours worked/week and hourly wages). Results: We included data from 91,633 respondents between 18 and 70 years and identified 923 (1%) stroke survivors. Stroke survivors were less likely to be employed (adjusted Odds Ratio 0.39, 95% CI 0.33 to 0.46) and had hourly wages 17.7% (95% CI 8.3% to 27.1%) lower compared to the general population, although there was no association between work hours and being a stroke survivor. Older age, being single and having medical comorbidities were associated with lower odds of employment in stroke survivors. Conclusions: Stroke survivors are less likely to be employed and earn a lower hourly wage than the general population. Interventions such as dedicated vocational rehabilitation and policies around return to work could be considered to address this lost productivity among stroke survivors.

Background: The Saskatoon stroke program participated in the ESCAPE trial looking at rapid endovascular revascularization for large vessel occlusion. Improvements were necessary to meet the timelines mandates in ESCAPE and to comply with Canadian Best Practice Guidelines. Methods: Retrospective chart review and prospective gathering of key metrics was performed using REDCap (Research Electronic Data Capture) software. Changes adapted from Canadian Best Practice Recommendations for Stroke Care, the ESCAPE protocol, and the Calgary stroke program HASTE project were implemented. Results: Changes implemented included increasing ambulance bypass window to 12 hours, FAST stroke assessment, emergency department pre-notification and registration, stroke alert protocol, team swarm of the patient, administration of tPA in the computed tomography (CT) room, and rapid access to the endovascular suite. Total number of patients between the years 2012 and 2014 was 287, and of those, 93 received tPA. Door-to-CT times decreased from 40 minutes to 21 minutes from 2012 to 2014; and Door-to-Needle (tPA) decreased from 62 minutes to 46 minutes from 2012 to 2014. Conclusions: By following Canadian best practice recommendations for stroke care, the ESCAPE protocol, and adaptation of Calgary stroke program HASTE project, our stroke program implemented changes to reduce treatment times for patients experiencing stroke in our province.

Background: Vasospasm causing delayed ischemic neurologic deficit (DIND) remains a leading cause of devastating outcome after aneurysmal subarachnoid hemorrhage (aSAH). Therapy using intravenous milrinone (IVM) and intra-arterial milrinone (IAM) has been described. We report our results using IAM in patients with refractory and super refractory vasospasm (RV and SRV respectively). Methods: Retrospective single center study of all adult patients treated with IAM between 2006 and 2016 inclusively. IAM was used as part of the Montreal Neurological Hospital Protocol when the patients’ symptoms failed to respond to initial and higher IVM doses. We report their clinical outcomes. Results: IAM was used in 19 patients. The median loading dose was 8 mg and average maintenance dose was 0.78 mcg/kg/min. Angiographic improvement was seen in 15 (79%) and clinical improvement - within the first 48 hours - was seen in all patients. The median mRS was 3 at time of discharge and 1 three months later. Five patients lost follow up. Conclusions: IAM appears to be safe and effective in this small retrospective series of RV and SRV complicating aSAH. Angiographic and clinical improvements were observed. Further prospective studies are warranted to confirm these findings.

Background: The cause of ICU delirium is unknown. We used near infrared spectroscopy (NIRS) to measure brain tissue oxygenation (BtO2) in critically ill patients, to test the hypothesis that poor cerebral oxygen delivery contributes to ICU delirium. Methods: Adult patients were enrolled if they required mechanical ventilation for >24 hours, and/or vasoactive agents. Patients were excluded if they had previous cognitive dysfunction, brain injury on admission, or a life expectancy <24 hours. BtO2 was measured for the first 24 hours of ICU admission. The confusion assessment method-ICU (CAM-ICU) was used to screen for delirium. Participants were designated to one of three groups on the basis of their predominant neurological status (comatose, delirious, or intact). Results: To date, 47 patients have been recruited. Both delirious and comatose patients’ had significantly lower BtO2 levels compared to intact patients (P<0.001). There was a significant correlation between hemoglobin and BtO2 (R2=0.347, P<0.01). However, when correlation analysis was conducted separately amongst the three groups, the delirious patients (R2=0.485, P<0.05) were the strongest contributors to this positive correlation. Conclusions: Delirious patients exhibited the lowest BtO2 recordings and demonstrated a significant association between Hb and BtO2. This study offers potential insight into the pathophysiology of ICU delirium.

Background: Graduating neurology residents require general palliative care skills. This study aims to develop an evidence-based palliative care curriculum to provide neurology residents with the general palliative care skills required for providing patient care along the continuum of life. Methods: A needs assessment of the palliative skills necessary for a neurology resident was performed. Focus groups were held with physicians, allied health care and senior residents. Semi-structured interviews were held with patients and their caregivers. Interviews analysed using qualitative thematic analysis techniques. The Kolb learning style inventory will determine the learning style of neurology residents and inform the curricular design. Results: Qualitative analysis identified 3 overarching challenges for neurology residents: 1) uncertainty regarding disease trajectory in neurology and timing of palliative care discussions; 2) cohesiveness of the health care team regarding end of life issues; 3) the role of the resident in initiating palliative care. Other principals identified for inclusion were: symptom management, communication, psychosocial aspects of care, care coordination and access, and myths and pitfalls in palliative care. Conclusions: This project will identify the current best evidence and expert opinion in palliative care neurology. The data will be used to develop a novel Canadian neurological palliative care curriculum.

Background: Few evidence-based disease-modifying treatments exist for progressive multiple sclerosis (MS). How can neurologists best care for patients with advanced MS? Little is known about how patients with progressive MS view their relationship with their treating neurologist, and if the role of the neurologist matches their needs and preferences. Methods: A qualitative cross-sectional analysis of patient preferences regarding the role of the neurologist in their care. Patients with progressive MS and an EDSS score of 6 or more were invited to participate. Patients and caregivers completed separate written questionnaires and were then interviewed by one of the authors. Data were subjected to thematic coding to group common themes and the distribution of themes among different disability sub-groups was analyzed. Results: Full results will be available at the time of the conference. Preliminary results suggest that the neurologist has an important role in updating patients on the progress of their disease and responding to questions. Patients are fearful of becoming dependent on others for their care. The concept of palliative care is unfamiliar to most patients. Conclusions: Despite a lack of disease-modifying treatments for progressive multiple sclerosis, patients believe that the neurologist has an important role in their care.

Background: Background: High-dose intravenous immunoglobulin (IV-Ig) is an evidence-based treatment for chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). Recently, subcutaneous Ig (SC-Ig) has received increasing attention. We performed a meta-analysis to assess the efficacy of SC-Ig versus IV-Ig. Methods: Methods: We searched PubMed, Embase, and Scopus from January, 1990 to December, 2015 for publications comparing IV-Ig vs. SC-Ig in patients with CIDP or MMN. We performed fixed-effects meta-analyses for strength changes as measured by the Medical Research Council sum score changes (MRC-SS). Results: Results: A total of 8 studies comprising 138 patients (88 with CIDP and 50 with MMN) were included in the meta-analysis. Considering the total population the use of SC-Ig showed slightly better results for MRC-SS (ES=-1.78, 95%CI=-3.45 to -0.11, I2<0.001%). However, when CIDP and MMN were compared separately, there were no differences between treatments (CIDP: ES=-0.28, 95%CI=-0.57 to 0.02, I2<0.001%; MMN: ES=-0.34, 95%CI=-3.99 to 3.31, I2<0.001%). Conclusions: Conclusions: We found comparable efficacy between SC and IV-Ig administrations for CIDP and MMN. These results suggest that SC-Ig is a suitable alternative treatment method, especially when other situations (e.g. convenience, safety profile) warrant its use. Further studies are needed to explore the efficacy of SC-Ig for CIDP and MMN.

Background: Several studies have demonstrated the safety and efficacy of endovascular therapy for patients with acute ischemic stroke. However, patient, imaging and treatment factors associated with the optimal functional outcome require better definition. Methods: We pooled data from 8 randomized controlled trials (SYNTHESIS, MR RESCUE, IMS III, MR CLEAN, ESCAPE, EXTEND-IA, SWIFT-PRIME, and REVASCAT). We conducted subgroup and sensitivity analyses to evaluate predictors of optimal functional results (modified Rankin scale, mRS) at 90 days. Results: Meta-analysis of 8 trials including 2,423 patients yielded that endovascular therapy resulted in 44.6% functional independence (mRS 0-2) versus 31.8% in the usual care group (OR 1.71, 95% CI 1.18-2.49, P=0.005). This treatment effect was significantly greater among patients with confirmed angiographic imaging of proximal arterial occlusion (OR 2.24, 95% CI 1.72-2.90, P<0.001), in patients who received the combined therapy of intravenous tPA and endovascular intervention (OR 2.07, 95% CI 1.46-2.92, P<0.001), and when using stent retriever for mechanical thrombectomy (OR 2.39, 95% CI 1.88-3.04, P<0.001). Conclusions: The relative functional benefit associated with endovascular therapy among patients with acute ischemic stroke was increased when combined with intravenous tPA, with confirmed proximal arterial occlusion on angiographic imaging, and with use of stent retrievers for mechanical thrombectomy.