Background: Chronic Inflammatory demyelinating polyradiculoneuropathy is a rare disorder of the peripheral nerves. A disease affecting up to 8.9 out of 100,000 people, and a yearly incidence of 1.6/100,000 people, CIDP is a condition that is treatable but still relatively unknown outside of the neuromuscular community. The purpose of this research, initiated by the GBS/CIDP Foundation, is to better understand a patient’s journey living with the disease and identify unmet needs. Methods: The research consists of a mix of structured interviews, digital ethnography and patient records. A total of 10 Canadian patients living with CIDP and their caregivers, 7 Canadian neurologists and 3 Canadian neuroscience nurses will be the subjects for our research. Results: In order to identify key interactions between patients and the healthcare system, the report will map a patient’s experience on 4 distinct planes. Clinical journey (ex: first symptoms, diagnosis, disease progression), Patient emotional journey (the emotional states the patient undergoes throughout his/her journey), Caregiver emotional journey, and Outcomes (ex: delays in care, damaged relationships, commitment to therapy). The report will identify key areas along the patient journey where more intervention is possible and where more research may be needed. Conclusions: The research is expected to be completed by April 2019.

Background: The response of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) to Intravenous Immunoglobulins (IVIG) treatment is well established . However, determination if patients not responding to 2 IVIG treatments or those whose condition stabilizes (ICE Trial) may benefit from additional doses remains unclear. We aim to identify time period required to reach maximal strength gains from IVIG treatment. Methods: Retrospective chart review of 14 patients with CIDP was performed. Change in Grip strength (GS), Knee extension (KE), Elbow Flexion (EF) and Dorsflexion(DF) was analyzed with a dynamometer during IVIG therapy. Averages for : percent change from baseline(Max%Δ),cumulative grams(g) of IVIG and time in weeks(w) required for maximal strength recovery was determined per function (+/−SEM).Anciliary therapy for all patients was recorded. Results: Strongest improvement was observed for DF(124+/−30%,p<0.001), followed by KE(113+/−19%,p<0.01),GS(100+/−21%,p<0.001) and EF(98+/−14%p<0.05).GS improved the fastest(19.1+/−3w) followed by DF(29.5+/−7w),KE(29.6+/−4w) and EF(31+/−6w). Cumulative IVIG dose to reach Max%Δ was highest for EF(869+/−201g) and lowest for GS(573+/−78g). Conclusions: Our study has demonstrated effectiveness of multiple treatments with IVIG to reach significant improvement in strength. Different muscle groups manifested different time-dependency ,reflecting variable amounts of IVIG required. Improvement was identified to be present on a ongoing basis ,with therapy lasting between 19.1-31 weeks,requiring between 869-573g of IVIG.

Background: “Neurophobia” describes a fear of Neurology on the part of medical students. This contrasts with the “neurophilia” that exists in society with increasing awareness of disorders such as stroke and multiple sclerosis. Ideally, we should take advantage of “neurophilia” to promote our specialty’s strengths. One step would be to better understand what students learn from a Neurology elective. Methods: This was a qualitative study. Students completing an elective between September 2011 and March 2015 at the Jewish General Hospital (JGH) in Montreal completed written pre- and post-elective questionnaires. Results: 36 students participated; 15 from McGill, 11 from other Canadian medical schools, and 10 from International medical schools. Many students changed their opinion about Neurology, with fewer citing lack of treatments or poor patient prognoses as negatives after completing their elective. They valued knowledge acquired about the neurological exam and problem-solving, while the range of cases and subspecialties surprised them. Many would diversify the setting of their elective to better experience this variety. Conclusions: More diversified elective experiences could showcase the strengths of our specialty and the scope of neurological practice. Presenting Neurology as a challenging, intellectually stimulating specialty that emphasizes problem solving could increase student interest.

Background: This is an updated on-line survey of the awareness and knowledge of stroke and heart disease amongst Chinese-Canadians carried out in 2017. Methods: 1001 randomnly selected Chinese-Canadians from Toronto and Vancouver area. Results: 46% were > 45 years old and male to female ratio was: 49.3 : 50.8, with native language being Cantonese in 40%, Mandarin 24% and 31% English. 82% were Canadian citizens and 31% had been in Canada < 10 years. 44% were from Mainland China, 37% Hong Kong , 6% Taiwan and 12% were borned in Canada. 85% were able to name at least one symptom of heart attack (p=0.005) while 80% were able to name at least one symptom of stroke (p=0.0008). 85% would call 911 in response to symptoms of heart attack or stroke compared to only 20% in a previous 2004 telephone survey (p=0.00001) . Conclusions: There is a dramatic improvement in the awareness and knowledge of stroke and heart disease amongst Chinese-Canadians compared to a previous telephone survey in 2004. This significant change could be due to difference in survey technique, but these improvements could also be due to the ongoing health promotion efforts by the Chinese-Canadian Council in support of the Heart & Stroke Foundation.

Background: Flow diversion is an established endovascular method for the treatment of intracranial aneurysms. The Pipeline Embolization Device (PED) remains the only FDA-approved stent available in USA and Canada since 2011. Stent position plays an important role in determining long-term success. The Leonardo Workstation(Siemens) is used for planning the ideal stent size and post-deployment destination. This first-ever study evaluates the accuracy of modeling software in predicting PED location post-deployment. Methods: 48 PED-assisted cases were performed 2012-2018 at the University of Alberta Hospital. 20 fit our preliminary inclusion criteria (single stents, simple anatomy). The proximal and distal landing zones were used to model the ideal stent using Leonardo. Accuracy was measured by comparing the Leonardo-predicted stent length vs known length. Results modeling against the dimensions predicted by AngioSuite, an app-based interface designed for use in the planning stages. Results: Leonardo workstation is accurate within 5mm at predicting final length for stents oversized by ≥0.25 cm. The predicted difference by Leonardo workstation & AngioSuite did not demonstrate statistical significance (P=0.36, P=0.24 respectively). Conclusions: Current angiographic planning tools are accurate at predicting PED deployment within 5mm. Complex vascular anatomy and deployment of multiple stents make prediction challenging. Analysis of these complex cases is currently underway.

Background: Telestroke can improve ischemic stroke patient outcomes by improving access to physicians specialized in stroke care and increasing the rate of thrombolysis. The aim of this study to assess the effect of the newly implemented Telestroke service on ischemic stroke patient outcomes in New Brunswick, Canada, a province with a high rural population. Methods: By means of a retrospective chart review, data for 366 adult acute ischemic stroke patients (Telestroke = 15.3%; non-Telestroke = 84.7%) were collected from emergency departmentsspanning five sites in the province. Outcomes included home discharge rates, complications (i.e., hemorrhage,angioedema), mortality, rate of thrombolysis and time to treatment. Results: No significant differences emerged for home discharge rates, complications, mortality or door-to-needle time. Telestroke patients had a significantly greater rate of thrombolysis treatment (51.8% vs 6.1%) and significantly less door-to-CT time (M= 27.63 min vs M= 100.78 min) compared to the non-Telestroke group. Conclusions: Overall, both groups had similar outcomes with some trends toward improvements for patients utilizing Telestroke.

Background: There are only few studies approaching the prevalence and cause of stroke in children and adults with Down Syndrome (DS). Methods: We did a retrospective study of our cohort of 4962 patients of Jerome Lejeune Institute since 2007. We collected age of stroke, clinical presentation, cause (TOAST classification), treatment and clinical course. Results: We identified 20 patients from 6 to 56 years old .In all cases, it was a stroke of ischemic origin.: 8 children with a prevalence of 0.33%. 4 had a cardio-embolic origin, 3 secondary to Moya-Moya syndrome and one of undetermined origin. 12 adults (21 to 52 years old) with a prevalence of 0.46%.The majority of the causes of these ischemic strokes are indeterminate (9 of 12), Conclusions: We found a low prevalence and an ischemic cause in all cases of stroke, which differs from the general population. For pediatric stroke, the causes are expected thromboembolic in a context of heart disease most often or secondary to a Moya-Moya syndrome. For adult strokes, the average age is younger than that in the general population and the cause is indeterminate in most cases. We must better explore our patients to identify the risk factors in DS population.

Background: The Canadian Stroke Best Practice Recommendations target a median door-to-needle time of 30 minutes. However, brain tissue becomes damaged with any delay from symptom onset. Efficiencies may be gained prior to hospital arrival, by evaluating the timeliness of patient access to hospital from symptom onset, as well as by improving healthcare provider communication, prior to arrival of the patient. Methods: We engaged with hospital administration, paramedic services, allied health colleagues, physicians, and engineers, to develop Kairos, a secure online platform that healthcare providers can utilize to track progress en route to hospital, as well as to share pertinent stroke patient information, prior to arrival. Results: Kairos is built on React Native, allowing users to access it on android or iOS devices. Paramedics select patient identification, symptom onset time, and associated symptoms. The receiving hospital can add doctors to the patient’s thread, and the stroke team can prepare for patient arrival. Conclusions: We plan to measure the median symptom onset-to-hospital time in patients with strokes, and monitor the change in door-to-needle time following implementation at an Ontario Regional Stroke Centre.

Background: This is the first report of Heparin induced thrombocytopenia (HIT) presenting as bilateral carotid thrombi and multiple cerebral infarcts. Methods: 54 year old woman presented with sudden onset of right arm numbness and weakness two days after discharge from hospital. During her hospitalization 9 days prior, she underwent colovesicular fistula repair, received heparin subcutaneously for DVT prophylaxis and had normal platelet counts. Results: On this admission, MRI Brain showed scattered multiple acute infarcts within the cortex of bilateral cerebral hemispheres. CT angiography head /neck showed non-occlusive thrombi at the carotid bifurcations bilaterally. Platelet count on admission was 267 K/uL q which decreased to 125 K/uL the next day, after which heparin was started for the carotid thrombi. The platelet count rapidly decreased further to 79 K/uL leading to suspicion for HIT and switching to Argatroban. HIT and serotonin release assay were positive confirming the diagnosis of HIT. CT chest and tranthoracic echocardiogram was normal. Venous Duplex of bilateral upper and lower extremities were negative for DVTs.Hypercoaguable evaluation was negative. Conclusions: This case highlights the importance of identifying HIT as a cause of arterial thrombosis and stroke even with normal platelet counts in the clinical setting of recent heparin use.

Background: Many guidelines in neurology encompass the principles of Choosing Wisely Canada (CWC): resource stewardship, patient safety, and high value care. There are currently 49 medical societies with CWC recommendations excluding the Canadian Neurologic Society (CNS). Methods: A descriptive process for list generation is outlined. A review of the American Choosing Wisely recommendations was undertaken to generate an adapted list of ten recommendations. CNS board members vetted this list and an online survey was sent to each CNS member. Results: A short list of recommendations endorsed by the CNS membership at large will be presented according to the survey results. CWC promotion of the list will take place to reach specialists, primary care providers, and trainees to ensure high value neurological care delivery is the standard across Canada. Conclusions: The process to delineate CWC recommendations for neurology is outlined. Participating in the CWC movement is an important leadership initiative for the CNS. It demonstrates the commitment of Canadian neurologists to the principles of high value patient care in neurology.

Background: Postural Orthostatic Tachycardia Syndrome (POTS) causes excessive heart rate and orthostatic intolerance on standing. About 25% patients have refractory POTS. Saline infusions reduce improve quality of life in such patients. Intravenous albumin expands circulatory volume by increasing plasma oncotic pressure. Efficacy of albumin infusions in POTS has not been studied. Methods: To assess the efficacy of albumin infusions in refractory POTS we treated patients with weekly or biweekly intravenous infusions of either 5% albumin in normal saline (n=16) or normal saline alone (n=7) in this open label comparative study. Most patients had failed multiple treatments. Serial clinical evaluations with individual symptom scores were the primary outcome measure of efficacy. Results: Mean follow up was 2 years (range 4 weeks - 5 years). 14/16 patients on albumin and 4/7 patients on saline infusions improved. Significantly more patients (7 vs. 1) on albumin showed marked improvement from baseline with more prominent reduction in orthostatic heart rate (mean reduction 19 vs. 14 beats minute). Albumin was well tolerated. More patients on saline (3/7 vs. 2/16) discontinued infusions due to lack of efficacy. Some patients required a permanent venous catheter. Conclusions: Intravenous albumin infusions are well tolerated and more effective than normal saline in refractory POTS.

Background: Calcium (Ca) and magnesium (Mg) are crucial in metabolism, excitability and neuroglial plasticity. Our aim was to evaluate whether Mg (20 mg/kg) or Ca (100 mg/kg) could improve the memory prognosis in the kainic model of mesial temporal epilepsy. Methods: Seizures were induced by systemic injection of kainate (8mg/kg) and mice were then treated by ions every 48 hours. A placebo (physiological solution) replaced kainate or ions in specific groups. Six cohorts were studied for seven weeks: control group (G0: no kainate and no ion, only placebo); untreated reference group (GR: kainate and then placebo); G1 groups were treated from the third day (G1m, G1c: kainate and then Mg/Ca); G2 groups were treated from the third week (G2m, G2c: kainate and then Mg/Ca). Radial maze and a classic maze were used for cognition evaluation. Results: The memory (short/long term) was differently affected by kainate or improved by Mg/Ca. The treated groups performed better than GR mice, but Mg was more effective. In addition, Mg demonstrated an increasing therapeutic effect over time while Ca showed an acute and apparently decreasing action in the G1c group. Conclusions: Mg should be considered for a clinical evaluation of its effect on epileptic disorders.

Background: There are few published reports on the safety and efficacy of stereoelectroencephalography (SEEG) in the presurgical evaluation of pediatric drug-resistant epilepsy. Our objective was to describe institutional experience with pediatric SEEG in terms of (1) insertional complications, (2) identification of the epileptogenic zone and (3) seizure outcome following SEEG-tailored resections. Methods: Retrospective review of 29 patients pediatric drug resistant epilepsy patients who underwent presurgical SEEG between 2005 – 2018. Results: 29 pediatric SEEG patients (15 male; 12.4 ± 4.6 years old) were included in this study with mean follow-up of 6.0 ± 4.1 years. SEEG-related complications occurred in 1/29 (3%)—neurogenic pulmonary edema. A total of 190 multi-contact electrodes (mean of 7.0 ± 2.5per patient) were implanted across 30 insertions which captured 437 electrographic seizures (mean 17.5 ± 27.6 per patient). The most common rationale for SEEG was normal MRI with surface EEG that failed to identify the EZ (16/29; 55%). SEEG-tailored resections were performed in 24/29 (83%). Engel I outcome was achieved following resections in 19/24 cases (79%) with 5.9 ± 4.0 years of post-operative follow-up. Conclusions: Stereoelectroencephalography in presurgical evaluation of pediatric drug-resistant epilepsy is a safe and effective way to identify the epileptogenic zone permitting SEEG-tailored resection.

Background: Cannabis has been shown to be an effective therapy for epilepsy in children with Dravet and Lennox-Gastaut syndrome. Despite the fact that many pediatric epilepsy patients across Canada are currently being treated with cannabis, little is known about pediatric neurologists’ attitudes towards it. Methods: A 26-item online survey was distributed to 148 pediatric neurologists across Canada. Results: 56/148 neurologists responded and reported that over 600 children with epilepsy are currently taking cannabinoids. 34% of neurologists authorized cannabis to children, 38% referred children for authorization, and 29% did not authorize or refer their patients. Of those neurologists who referred, 76% referred to a community-based non-neurologist. The majority of physicians authorized cannabis to patients with Dravet syndrome (68%) and Lennox-Gastaut syndrome (64%). Cannabis was never authorized as a first-line treatment. 54% of neurologists stated that their patients were taking CBD alone, despite this option not being available in Canada. All physicians reported having at least one hesitation regarding cannabis, the most common ones being poor evidence (66%), poor quality control (52%), and cost (50%). Conclusions: The majority of Canadian pediatric neurologists use cannabis as a treatment for epilepsy in children. However, there appear to be knowledge gaps and hesitations.

Background: The classic ketogenic diet is the main non-pharmacological treatment for refractory epilepsy; however, adherence is often challenging. The low glycemic index diet (LGID) is less strict, almost equally effective, and associated with improved adherence. Little is known about the quality of life of children treated with LGID. The objective of this study was to explore changes in the quality of life of children with epilepsy transitioning to the LGID. Methods: Patients on LGID and their parents filled out Pediatric Quality of Life Epilepsy Module questionnaires; one while being on the LGID, and one retrospectively for the time prior to starting the LGID. Results: Data was collected from five children ages 3-13 and their parents. Complete seizure control was seen in two children, >50% seizure reduction in one, and no change in two children. Parental reported quality of life while on the LGID increased with two participants but decreased in all child self reports. Conclusions: Although the LGID led to improved seizure control in three out of five patients, the child-reported quality of life decreased in all children. Larger prospective studies are warranted to reliably assess the impact of the LGID on the quality of life in children with epilepsy.

Background: Topiramate is an antiepileptic frequently used in pediatrics with multiple mechanisms of action. This includes carbonic anhydrase inhibition, which has unclear relevance to its antiepileptic effect. Metabolic acidosis, hypocitraturia and nephrolithiasis are known side-effects of carbonic anhydrase inhibition and can limit therapeutic effect. Alkali therapy may normalize acidosis, increase urinary citrate, and reduce nephrolithiasis risk. We hypothesize that provision of sodium bicarbonate supplementation to patients with topiramate-induced acidosis will mitigate these side-effects without worsening seizure frequency or severity. Methods: Pediatric patients on antiepileptic therapy with topiramate are being recruited from outpatient pediatric neurology clinics at McMaster Children’s Hospital. We aim to recruit 20 patients with metabolic acidosis and 20 control patients. Measures include blood gas, electrolytes, urine electrolytes and citrate. Patients with metabolic acidosis will be given daily sodium bicarbonate for one month, followed by repeat bloodwork. Seizure frequency will be prospectively documented in all participants throughout the three-month period. Results: Recruitment is ongoing, and three patients (1 with acidosis) have been recruited thus far. Results will be analyzed with chi-squared and paired T tests. Conclusions: This pilot study is the first to evaluate the safety and efficacy of sodium bicarbonate supplementation in patients receiving topiramate for seizure control.

Background: Non-convulsive seizures are common in critically ill patients and are best detected by continuous EEG (cEEG) monitoring. A recent consensus statement from the American Clinical Neurophysiology Society (ACNS) outlines the indications for EEG monitoring in critically ill patients. Our aim was to assess adherence to these indications, barriers to cEEG utilization as well as to optimize cEEG monitoring in critically ill children. Methods: We conducted a retrospective review of electronic medical records, analyzing patients admitted to the PICU from January 1st until June 23rd 2018, followed by an 8-week mentorship period, consisting of educational interventions as well as daily patient rounds to help identify patients meeting cEEG monitoring criteria. Results: Prevalence of patients meeting cEEG monitoring indications were similar in both the retrospective and mentorship period (18% vs. 23%). During the retrospective period, 23% of patients received cEEG monitoring, reaching 100% at the end of the mentorship period. The median delay for initiation of monitoring was 17 hours, largely due to restrictions in the availability of technologists. All cEEGs performed informed anti-convulsive management. Conclusions: An educational intervention was effective in increasing PICU cEEG monitoring. However, limited hours of technologist availability represented the largest barrier to timely cEEG monitoring.

Background: When compared to the general population, researchers have reported elevated rates of mental health issues in the pediatric epilepsy population. These issues have been found to be especially problematic around the time of transition from pediatric to adult care. This is significant because depression and/or anxiety have been found to be directly related to worsened seizure outcomes and quality of life. Despite this, no known Canadian pediatric epilepsy centers have integrated mental health assessment into mainstream practice. Methods: To explore the importance of mental health assessments, we looked at the prevalence rates of both depression and anxiety in 91 adolescents with epilepsy aged 14 to 18 (M=16.3, 51 males, 41 females) enrolled into an epilepsy transition clinic. Results: 58.3% of adolescents showed signs of depression (28.6% mild, 21.4% moderate, 6.0% moderately-severe, 2.4% severe), and 51.8% of adolescents showed signs of anxiety (31.8% mild, 10.6% moderate, 9.4% severe). Remarkably, 54.8% of patients presenting with moderate to severe depression and/or anxiety had not been previously identified Conclusions: These results suggest that in order to ensure the best possible outcomes for patients, mental health assessments should be integrated into the standard model of care for transition-aged adolescents with epilepsy.

Background: Focal cortical dysplasias (FCDs) are congenital structural abnormalities of the brain, and represent the most common cause of medication-resistant focal epilepsy in children and adults. Recent studies have shown that somatic mutations (i.e. mutations arising in the embryo) in mTOR pathway genes underlie some FCD cases. Specific therapies targeting the mTOR pathway are available. However, testing for somatic mTOR pathway mutations in FCD tissue is not performed on a clinical basis, and the contribution of such mutations to the pathogenesis of FCD remains unknown. Aim: To investigate the feasibility of screening for somatic mutations in resected FCD tissue and determine the proportion and spatial distribution of FCDs which are due to low-level somatic mTOR pathway mutations. Methods: We performed ultra-deep sequencing of 13 mTOR pathway genes using a custom HaloPlexHS target enrichment kit (Agilent Technologies) in 16 resected histologically-confirmed FCD specimens. Results: We identified causal variants in 62.5% (10/16) of patients at an alternate allele frequency of 0.75–33.7%. The spatial mutation frequency correlated with the FCD lesion’s size and severity. Conclusions: Screening FCD tissue using a custom panel results in a high yield, and should be considered clinically given the important potential implications regarding surgical resection, medical management and genetic counselling.

Background: Childhood absence epilepsy is a common generalized epilepsy in pediatric patients. Although this was considered a “benign” syndrome, new data suggests there are associated neurocognitive effects. This is the first study comparing quality of life and social functioning in those with absence epilepsy to those with other types of epilepsy. Methods: This observational study recruited patients from six Canadian academic centers. 106 patients had absence seizures, and 219 had other seizures. Established measures of depression, anxiety, social skills, social support, participation, quality of life, and epilepsy severity were assessed. MANCOVA was used to evaluate differences in social function, quality of life, and epilepsy severity measures, while accounting for age and gender. Results: This yielded a statistically significant result (Wilk’s lambda <0.05), with partial eta squared of 0.163. Follow up of between subjects tests revealed lower health related quality of life interpersonal/ social subscale and close friend social support scores in those with absence epilepsy, while other measures were not significant. Conclusions: Children with absence epilepsy have similar social function, quality of life and epilepsy severity measures compared to those with other types of epilepsy. This indicates that any dysfunction in these domains is similar to those with other types of epilepsy.