Background: Myotonic dystrophy is an autosomal dominant condition affecting distal hand strength, energy and cognition. There is a neuromuscular patient portal under development that has the potential to give voices and resource access to patients, regardless of location via the internet and social media. The current state of access to technology and underlying factors affecting use and interest were explored. Methods: Surveys were mailed to 156 participants with myotonic dystrophy type 1 (DM1) through the Canadian Neuromuscular Disease Registry. The survey questions touched on demographics, technology use, internet use, and interest in the portal. Results: Seventy-two participants (43 female) responded so far and 50% were younger than 46 years. Most (62/72) used the internet and 94% of participants had access to an internet-connected device. Almost half of the responders (34/72) used social media. The complexity and cost of technology were commonly cited reasons not to use technology. The majority of responders (79%) were interested in a myotonic dystrophy patient portal. Conclusions: DM1 patients have access to and use technology such as computers and mobile phones. They expressed interest in a portal that would provide them with access to relevant information such as guidelines, self-management modules, educational videos, and support groups.

Background: The distal hereditary motor neuropathies (dHMN) have been characterized through case reports and family studies. Their genetic characterization remains a work in progress. An appreciation of the genetic underpinnings may lead to treatment options in the future. Hence reports, like this one, which add to this understanding, remain extremely important. Methods: The clinical presentations, electrophysiology and genetics of two patients with the dHMN I phenotype are described. Results: A mother and son presented with slowly progressive distal weakness of the lower extremities with onset in the first and second decades. Distal weakness of the upper extremities developed later. Examination 20 and 50 years after onset revealed wasting and weakness of distal upper and lower extremity muscles with absent distal and preserved proximal deep tendon reflexes. Sensory examination was normal. Electrodiagnostic studies demonstrated a motor axonopathy. Genetics testing revealed a missense mutation on chromosome 7, exon 11 of the GARS gene: c.1415A>G (p.His472Arg). Conclusions: GARS mutations have been identified in patients with the dHMN I (juvenile onset distal weakness and wasting) and dHMN V (upper limb predominant) phenotypes. However, this mutation has not previously been directly linked to the dHMN I phenotype.

Background: Length-Dependent Sensorimotor Peripheral Neuropathy (LDSMPN) affects the longest nerve fibers in the body. Less well-appreciated, and absent from the current literature, is that LDSMPN affecting thoracic segments gives rise to ventral abdominal sensory loss on clinical exam. Methods: Consecutive patients seen for LDSMPN (n=30) were evaluated prospectively for the presence or absence of ventral abdominal sensory loss. Demographic variables, symptoms, quantitative neurologic findings (Neuropathy Impairment Score [NIS]) and final diagnosis were examined using descriptive statistics. Results: Ventral abdominal sensory loss was documented in 20/30 LDSMPN patients (66.7%), mean age was 64.1 years (range 33-81), M:F gender ratio was 19:11, mean NIS was 21.4 (range 0-77). NCS/EMG abnormalities were found in 25/30 patients, with 5/30 having a clinical exam and/or other electrophysiological evidence convincing for LDSMPN. LDSMPN patients without ventral abdominal sensory loss (n=10) had a mean age of 61.2 (range 45-73), M:F of 7:3, and mean NIS of 20.9 (range 0-54). Conclusions: 1) Ventral abdominal sensory loss appears to be common in patients diagnosed with LDSMPN of a variety of causes; 2) in addition to those innervating distal limb territories, distal sensory fibers from the thoracic region represent another category of length dependent involvement in LDSMPN; 3) the clinical examination of LDSMPN should include the ventral abdomen.

Background: Mononeuritis multiplex is a painful, asymmetrical peripheral neuropathy involving motor and sensory nerves. This neurological condition is classically associated with systemic diseases such as connective tissue disorders, vasculitis, hematologic diseases including cryoglobulinaemia and amyloidosis. It has also been reported infrequently as a paraneoplastic or post infectious disorder. Methods: Case report Results: We are reporting a case of a 35-year-old man who presented with mononeuritis multiplex following an infectious mononucleosis associated with a mixed cryoglobulinemia. He was treated with IVIG, IV pulse steroid and a prednisone taper over 7 months. Later on, he had a nerve transfer from FCR (flexor carpi radialis) to ECRB (extensor carpi radialis brevis) and PIN (posterior interosseous nerve) due to complete denervation of the PIN without evidence of spontaneous recovery. Conclusions: Acute EBV infection should be suspected in the setting of mononeuritis multiplex. This is the first reported case of nerve transfer for this type of nerve injury.

Background: Effects of stroke at the cellular and sub-cellular level remain poorly understood by conventional techniques. We use synchrotron-based imaging techniques to study elemental and biochemical changes in the infarct and penumbra after stroke in an experimental model. Methods: Ischemic stroke is induced in mice using the previously validated photothrombotic model. Animals are sacrificed at various time-points after stroke. Fourier transform infrared spectroscopic imaging (FTIRI) is used to gather sub-cellular (<1 µm spatial resolution) imaging data of lipid oxidation and protein aggregation in the areas of interest. X-ray fluorescence (XRF) imaging is used to image the distribution of bio-important elements at the cellular and sub-cellular spatial resolutions. Routine histology and immunohistochemistry are used to co-localize cell-types to areas of interest. Results: Preliminary XRF results indicate significant reduction in the concentration of multiple elements in the infarct, compared to the penumbra, at day 1 post-stroke. Some elements begin to return to normal concentration in the penumbra at day 3. FTIRI data shows that lipid and total protein levels decrease, while aggregate protein levels increase in the penumbra. Conclusions: Multi-modality synchrotron imaging can be used to map elements as well as bio-molecules in a stroke model. A better understanding of these changes can guide therapeutic interventions after stroke.

Background: Faster administration of tissue plasminogen activator (tPA) for patients with acute ischaemic stroke yields greater clinical benefits. We implemented door-to-needle (DTN) time reduction strategies at our centre and evaluated their short- and long-term effects on in-hospital treatment delays and clinical outcomes. Methods: Stroke team pre-notification, direct computed tomography (CT) transfer, not routinely waiting for labs and tPA delivery on CT table were implemented in June 2013. We included all thrombolysed patients admitted directly to our hospital between January 2012 and March 2015. In-hospital delays and clinical outcomes (Modified Rankin scale, mRS) at 3 months were compared between patients pre- and post-modification, and the latter period was divided into early and late phases to assess the durability of our modifications. Results: Forty-eight individuals were treated pre-modification, compared to 58 post-modification. The median DTN time was reduced from 75 (interquartile range: 60-93) minutes to 46 (33-59) minutes (p<0.0001). The median DTN time in the early and late post-modification phases was not different (41 versus 46 minutes, p=0.4085). Functional outcome at 3 months was not different in the two groups (proportion of mRS≤1: 34% versus 28%, p=0.882). Conclusions: We were able to decrease our DTN time for treatment of acute stroke by implementing simple modifications and these improvements persisted over time.

Background: The Canadian Stroke Best Practice recommends admission of patients to a specialised stroke unit within three hours. We aimed at assessing delays in our emergency department (ED) and correlating these with medical complications and clinical outcomes. Methods: Predictors and outcomes This is a retrospective review of patients (n=353) admitted with ischemic strokes (January 2011-March 2014). We assessed the length of stay in ED, medical complications in ED and in the stroke unit, functional status (modified Rankin Scale) at discharge and survival. Results: The median delay in ED was 13.8 hours. The rate of medical complications in the ED was 14% (most common being delirium), compared to the stroke unit with 46.7% (most common being pneumonia). Worse functional outcome was correlated with diagnosis of pneumonia (standardised β coefficient=0.2, p=0.001) and presence of brain oedema in the stroke unit (standardised β coefficient=0.2, p<0.01). Increased risk of death was correlated with brain oedema (OR=649.2, 95%CI=19-2184, p<0.01) and sepsis in the stroke unit (OR=26.8, 95%CI=2.1-339, p<0.01). Conclusions: We found a significant delay in the admission of our patients from the ED to the stroke unit, which is not in keeping with the present guidelines. Medical complications were correlated with worse outcomes. Future analyses will correlate ED delays with clinical outcomes.

Background: Approximately, one-third of TIA clinics use the ABCD2 score to triage referrals. However, the usefulness of the score is limited because of its low specificity for non-cerebrovascular/mimic conditions. Timely access of referred patients to specialized TIA clinics may reduce recurrent stroke. Methods: The SpecTRA project implemented a novel electronic triage system in the TIA clinic that services Vancouver Island (BC), which replaced the existing ABCD2 triage model. A clinical classifier generating an ACVS probability score was calculated on the basis of the clinic referral form information. Next, a time-varying ABCD2-based risk score derived from Johnston et al. (2007) was calculated, which is then weighted by the ACVS probability score to produce a finalized triage score. Time-to-care was compared pre- (2013/14) and post- (2014/15) implementation. Results: One year results show a statistically significant improvement in that time-to-care for ACVS patients (ABCD2 4/5) was one day earlier with the new triage system (median= 4days since symptom onset; N=250) compared to the previous year (median=5days; N=255) (Mann-Whitney U=38130, p< 0.001). No difference in unit arrival times (median= 5days) for non-cerebrovascular patients was observed (Mann-Whitney U=5563, p= 0.15). Conclusions: The performance of our ACVS triage system highlights quality improvement potential in time-to-care for outpatient TIA clinics.

Background: Bedside dysphagia screening is recommended for all patients with acute ischemic stroke, in order to detect swallowing impairment early and prevent complications. However, limited data are available on outcomes associated with failing a dysphagia screen. Methods: We used the Ontario Stroke Registry to identify patients who were admitted to Regional Stroke Centres from 2010-2013 and received a dysphagia screen within 72 hours. We used multivariable regression to determine outcomes of patients who failed the dysphagia screen. Results: Among 5145 patients who underwent dysphagia screening, 2458 (47.8%) failed and 2687 (52.2%) passed. Patients who failed had more co-morbidities and presented with more severe strokes (mean NIHSS 11.0 vs. 5.4). Among those who failed, 9% required permanent feeding tubes, versus 0.1% among those who passed. After controlling for age, co-morbidities, and stroke severity, failing a bedside swallowing screen remained highly predictive of poor outcomes, including decubitus ulcer (adjusted odds ratio aOR 10.5), pneumonia (aOR 4.6), discharge to long-term care (aOR 4.1) and 30-day mortality (aOR 4.5; 16.6% vs. 2.2%). *All p <0.0001 Conclusions: Patients who failed a dysphagia screen on admission had dramatically worse outcomes after controlling for baseline factors. A bedside dysphagia screen provides immediate risk stratification for acute stroke patients and can be used to guide appropriate care.

Background: Stroke alerts are used to triage patients with acute neurologic change for rapid imaging evaluation. CTA has been advocated to rapidly triage stroke patients for endovascular therapy. However, the yield of this approach is not well established. We evaluated the stroke alert yield in a non-teaching hospital system. Methods: A retrospective review of radiology reports for stroke alerts using PACS archive. Cases were then followed for 72 hours to determine the types of advanced imaging obtained and the findings of those studies. Results: From January to March 2014, 269 stroke alert head CTs were performed. Subsequent imaging included 128 MRIs (48%), 25 CTAs (9%) and 2 angiograms (0.7%). There were 58 (22%) tissue-defined strokes and 16 were non-lacunar (6% stroke alerts). 61% of stroke alert head CTs were negative or reported microvascular change. Other findings included large vessel occlusion (5%), intracranial stenosis (1.5%), extracranial stenosis(1.5 %), intracranial hemorrhage (9%) and masses (13%). Conclusions: Most stroke alerts were negative for tissue-defined stroke. Based on this data, universal use of CTA in the ER to triage patients with acute neurologic symptoms may not be appropriate. An updated triage system to facilitate endovascular rescue is being analyzed for changes to advanced imaging utilization and yield.

Background: Electroencephalogram (EEG) is used in evaluating thalamocortical function in comatose patients. EEG reactivity is increasingly being recognized as a potentially important predictor of outcome in comatose patients. There are no existing guidelines or standardized testing for EEG reactivity assessment. We will report the use of a clinically implemented standardized reactivity testing protocol in comatose patients to determine accurate prognosis. Methods: In this retrospective study we report results from standardized reactivity testing from January 2016 to May 2016. Five stimuli (Calling name, clapping, nasal tickle, noxious stimulus, tracheal suctioning) were applied at one minute intervals in comatose patients of all etiologies. The EEG background reactivity will be analyzed by two independent electroencephalographers ad correlated to clinical outcome. Results: The methods for establishing EEG reactivity and the inter-rater reliability in determining EEG reactivity will be reported. Conclusions: EEG background reactivity is likely beneficial in determining prognosis. However, reliable methods for eliciting and determining EEG reactivity in comatose patients are necessary.

Background: Psychiatrists commonly use electroencephalogram (EEG) to rule out epilepsy as a cause of psychiatric symptoms. A large number of these studies are normal. Our study aims to identify the predictive factors of an EEG with epileptiform activity in these patients. Methods: We performed a retrospective study of the EEG results and chart reviews of the 208 psychiatric patients at Royal University Hospital in Saskatoon, Saskatchewan from 2013-2015. The EEG results were correlated with several factors known to increase the probability of an abnormal recording including history of seizures, previously abnormal EEGs, imaging abnormalities, medications known to cause epileptiform discharges, electroconvulsive therapy, prematurity, brain infection, childhood febrile seizures, head trauma, and family history. Results: Of the 208 EEGs performed, 176 (84%) were normal (77%) or essentially normal (7%). Epileptiform activity was found in 13 EEGs (6.3%), of which 9 (4.3%) had a previous EEG with epileptiform activity. Focal slowing appeared in 12 EEGs (5.8%), two of which had previous abnormal EEGs. Generalized slowing was found in 7 EEGs (3.4%). Conclusions: We conclude that the majority of EEGs in patients with psychiatric manifestations are normal. The most predictive factor for epileptiform activity in this population is a previous EEG with epileptiform discharges. Other predictive factors are under review.

Background: Donation after circulatory death (DCD) is a recently accepted source for solid organ donation in Canada. Standards for circulatory death vary within and between countries and little is known about neurologic function at the time of circulatory death. This pilot study will explore the electrical physiology of the brain during the dying process following withdrawal of life support and at the time of declaration of circulatory death. Methods: This single-centre pilot study will build on preliminary data from the DDePICt (Death Determination Practices in Intensive Care) research program. With institutional approval and signed consent from the substitute decision maker, participants will undergo continuous 10-20 EEG (cEEG) monitoring in addition to monitoring vital signs during the dying process and for 30 minutes after the declaration of death. Results: Preliminary results including cEEG, blood pressure with arterial wave forms, EKG activity and oxygen saturations are currently under analysis and will be presented. Conclusions: It is feasible to study neurologic function during withdrawal of life support and these results will allow us to further understand the electrical activity of the brain during the dying process.

Background: NMDA receptor encephalitis (NMDA-RE) is an autoimmune disorder caused by antibodies to the NR1-NR2B heterodimer of the NMDA receptor. Currently, disease status is tracked primarily by the presence of auto-antibodies in the cerebrospinal fluid (CSF) and serum. Using serological and CSF markers along with clinical parameters to track disease progress can be challenging since patient symptoms and disease progress can vary widely. Methods: EEGs were reviewed in a 31 year old male patient with proven NMDA-RE. EEG data were sampled from various times before and after diagnosis, as well as during various stages of treatment. All analyses were performed using Matlab (Mathworks). Results: We showed that using a simple 1/f model of spectral behaviour (Buzsaki and Draguhn, 2004), we could fit the power spectra of the raw data at various instances during routine EEGs. We have demonstrated that the values of specific fitting parameters vary in relationship to the patient’s clinical status across various stages of illness. Conclusions: The aim of this project was to explore the potential utility of EEG as a complement to the usual clinical metrics used in monitoring NMDA-RE. The analysis techniques presented here highlight the use of EEG as a practical, minimaly-invasive tool to monitor progress and potentially aid in clinical decision making.

Background: Transient osteoporosis is an infrequent musculoskeletal condition most often seen in middle-aged males, but can rarely present during late pregnancy in females. Patients present with sudden onset of severe joint pain. MRI typically shows T2 hypersignals and bone marrow edema. Abnormal neurophysiological findings have only rarely been described in the literature. Methods: Case report Results: A 35-year old patient presented at 34 weeks of pregnancy with acute onset of right hip and leg pain. MRI showed marked T2 hypersignals in the head of the femur with distal extension and bone marrow edema pathognomonic of transient osteoporosis. Neurophysiological studies showed normal nerve conductions of the lower extremities, but fibrillation potentials and positive sharp waves were found acutely in proximal muscles of the affected extremity and not limited to a single nerve territory. Inflammatory markers were unremarkable. Treatment was conservative with rest and reduction of weight bearing and pain resolved in the hip, but recurred in the ipsilateral knee several months after delivery suggesting evolution toward regional migratory osteoporosis. Conclusions: Transient osteoporosis of the hip may be associated with EMG signs of denervation that could suggest a contributing neurogenic mechanism. Their prevalence may be underestimated and we suggest considering serial EMG studies in its investigation.

Background: Postoperative infection is a significant cause of morbidity and mortality in traumatic brain injury (TBI) patients who undergo craniotomy and/or craniectomy. Data on the rates of infections associated with these procedures are limited. We present a single-center retrospective study on the rates of infection in post-traumatic craniotomies, craniectomies and cranioplasties. Methods: Data on 100 TBI adult patients who underwent a craniotomy, craniectomy and/or cranioplasty from 2011-2015 will be analyzed. Demographic and perioperative data including open/closed TBI, peri/postoperative infections, duration of procedure, type and mode of bone flap preservation will be retrieved. Results: Following our data collection (to be completed by the end of February), we expect infection rates of 3-20% in our study. Upon instituting a protocol similar to the Hydrocephalus Clinical Research Network’s (HCRN) ventriculoperitoneal shunt (VP) protocol, we hope to reduce our post-TBI craniotomy/craniectomy/cranioplasty infections rates to less than 10%. Our projection is based on the HCRN protocol’s 3.15% absolute risk reduction of VP shunt infections. Conclusions: The results of this study will emphasize the need for instituting robust perioperative protocols to reduce infections. Further research will be pursued following this study to establish a protocol similar to the VP shunt protocol from the HCRN, in an attempt to reduce perioperative rates of infection.

Background: Characteristics of traumatic spinal cord injury (tSCI) patients admitted to the Saint John Regional Hospital and the Stan Cassidy Center for Rehabilitation from 2011 to 2014 were examined. Methods: Demographic, neurological and functional outcome data for 18 patients, who had consented to participate in a database for tSCI in Canada, was obtained. Results: The majority of patients were male (88.9%), with a mean age of 41. 33 (SD=17.17). The most common causes of tSCI were motor vehicle accidents (41.2%) and falls (29.4%). Cervical spine injuries (70.6%) and an ASIA impairment scale classification of D (38.9%) predominated. The median latency from injury to surgery was 22.67 hours. Functional independence Measure scores (M=64.17, SD=25.84) indicated that motor/functional independence was impaired (M=32.44, SD=19.15) relative to cognitive independence (M=31.83, SD=4.07). Conclusions: The results suggest that characteristics of tSCI patients in New Brunswick are similar to the Canadian tSCI patient population. Emergency care appears to be delivered in a timely fashion. Both centers participate in research registries focused on collecting data related to tSCI, surgical interventions, and patient outcomes. Registries are valuable research tools that allow for an alternative way to examine the quality of care their patients receive.

Background: Research has suggested that female athletes have a higher incidence of concussion compared to their male counterparts. As such, programs designed to improve knowledge and attitudes of concussion should target this high-risk population. Previous work demonstrated the effect of a novel Concussion-U educational presentation on knowledge and attitudes of concussion amongst male Bantam and Midget AAA hockey players. The objective of this study was to determine if the same presentation was effective in improving the knowledge and attitudes of concussion in a cohort of elite female hockey players. Methods: 26 elite female high-school aged (14-17) hockey players from the province of New Brunswick consented to participate in the study. Each participant completed a modified version of Rosenbaum and Arnett’s Concussion Knowledge and Attitudes Survey questionnaire immediately before and after a Concussion-U educational presentation. Results were compared across the two time-points to assess the effectiveness of the presentation. Results: Concussion knowledge and attitude scores significantly (p<.001) increased from pre-presentation to post-presentation by 12.5% and 13.4%, respectively. Conclusions: A Concussion-U educational presentation resulted in increased knowledge and improved attitudes towards concussion in elite female hockey players. Future research should examine the long-term retention of these improvements.

Background: Indigenous populations are disproportionately affected by traumatic brain injury (TBI). These populations rely on large jurisdiction surveillance efforts to inform their prevention strategies, which may not address their needs. This study describes the TBI determinants of a Quebec indigenous population, the Cree served by the Terres-Cries-de-la-Baie-James health region, and compares them to the determinants of two neighbouring health regions and the entire Province of Quebec. Methods: We conducted a retrospective population-based cohort study of incident TBI hospitalizations, stratified by the aforementioned health regions, in Quebec from 2000-2012. MED-éCHO administrative data were used for case finding. A sub-analysis of the Terres-Cries-de-la-Baie-James adults was completed to assess for determinants of TBI severity and outcomes. Regression models, multiple imputations and a sensitivity analysis were used to account for biased associations. Results: 172 incident TBI hospitalizations occurred in the Terres-Cries-de-la-Baie-James region from 2000-2012. The incidence rate was 92.1 per 100,000 person-years and the adjusted IRR was 1.86 (95% CI 1.56-2.17) when compared to the entire province. Determinants of TBI for the Terres-Cries-de-la-Baie-James were significantly different from those of neighboring populations and the entire province. Conclusions: TBI surveillance information from large jurisdiction initiatives can be misleading for indigenous communities. Community-based surveillance provides evidence that these populations should use to prioritize prevention strategies.

Background: Penetrating brain injury is a rare entity. Furthermore, clinical presentation can be highly variable and management difficult, especially when the foreign body is retain in the skull Methods: We present a serie of three recent cases of penetrating brain injury that happened at our institutionm including clinical and radiological data. We discuss management of those challenging cases and present a brief review of the literature. Results: Our cases (3) encompassed different mechanisms: bilateral nail gun injury, knife and aircraft propeller. All patients were male, with a median age of 37 years old. Work-up was negative for intracranial vascular injury. All patients were treated with initial craniectomy (bilateral in one case) and a course of antibiotics. Cranioplasty was later performed. All patients survived and evolved in favorable fashion. Conclusions: Penetrating brain injury is a rare injury, requiring individualized surgical and medical management. A few recommendations may be found in the literature but are often based on literature from blunt traumatic brain injury or war-related injuries.