Background: The purpose of this study was to: 1) describe current non-invasive ventilation (NIV) usage patterns amongst Canadian ALS healthcare providers; 2) compare/contrast with previous practice patterns; and 3) explore barriers to NIV access encountered by current practitioners. Methods: Healthcare professionals (including physicians, respiratory therapists, and nurses) at major Canadian ALS care centres were sent a web-based survey. Participants were asked to provide input on practice demographics, access and initiation of NIV, and follow-up of NIV. Quantitative data were analyzed with descriptive and comparative statistics, while qualitative data were analyzed using interpretative phenomenological analysis method to identify emergent themes. Results: 26 participants responded. Median NIV usage was 39% (range 10-100%), about double of what was previously reported (18%). Mean times from referral to routine and urgent NIV initiation were 13 (95% CI 9-17) and 5 (95% CI 3-7) days respectively. NIV was most commonly initiated in clinic (68%), while 38% report having access to home-NIV initiation. Lack of social support (62%) and cognitive impairment (46%) were the most common deterrents to initiating NIV. Similar to what is previously reported, barriers to access can be stratified to patient, clinical, institutional, and regional levels. Conclusions: Despite increased usage and improved access, there remain considerable barriers for ALS patients to receive NIV.

Background: Spinal Cord Injury Without Radiological Abnormality (SCIWORA) is underreported and poorly recognized in adults. This entity is an important subtype of spinal cord injury (SCI) with relatively good outcomes. Despite this, few studies have been performed to determine specific imaging-related prognostic factors. Methods: A retrospective review of adult patients with cervical SCI admitted to two University hospitals from January 2000 to December 2010 was performed. Only patients with an MRI performed within 72 hours after trauma were included. All patients with bony injury or traumatic malalignment were excluded. Data gathered on the remaining patients included demographics, mechanism of injury, severity of SCI, long-term patient outcome, improvement in neurological condition and MRI results. Results: 49 patients selected. Patients with extramedullary hemorrhage showed worse neurological status at initial examination. Disruption of either the anterior longitudinal ligament or ligamentum flavum was associated with worse outcomes at initial examination and at 1-year follow up. Lesion length was also significantly associated with outcomes at 1 year evaluation and initial evaluation. Conclusions: Early MRI has an important prognostic value in patients suffering SCIWORA. Lesion length is a powerful predictor of outcome. Soft tissue injury and spinal cord changes play a role in the severity of injury as well as the ability to recover.

Background: The incidence of brain metastases is increasing with the development of improved systemic therapies with limited impact on intracranial disease. The purpose of this study was to determine if there is a threshold tumor size below which local control (LC) rates approach 100% after stereotactic radiosurgery (SRS). Methods: 200 patients with 1237 tumors were identified from a prospective registry of patients having undergone SRS between 2012-2014. Histology consisted predominantly of non-small cell lung cancer (NSCLC), melanoma and breast cancer. Results: The median tumor size was 6 mm in diameter or 70 mm3 and most commonly NSCLC. Thirty-three tumors had local progression at a median time of 8.8 months. The 1- and 2-year actuarial LC for all tumors were 97% and 93%. LC of 100% was seen for intracranial metastases less than 100 mm3 or 6 mm in diameter, independent of histology. Total tumor volume was an independent predictor of overall survival, after adjusting for age, KPS and extracranial disease status. Conclusions: SRS can achieve LC rates approaching 100% for subcentimeter metastases. The earlier detection and prompt treatment of small intracranial metastases may prevent the development of neurological symptoms, the need for surgical resection, and potentially improve overall survival. The results of this study would favour the implementation of routine staging MRIs.

Background: Delayed facial palsy (DFP) after resection of vestibular schwannomas (VS) is described as worsening of facial nerve function after a normal postoperative result. Several mechanisms have been postulated to explain this phenomenon, although none satisfactorily explain all of its features. Furthermore, systematic documentation of recovery rates is lacking. Methods: 403 consecutive cases of VS resection between 2001 and 2015 were reviewed. Patients with preoperative facial palsy were excluded. Patients developing significant facial palsy (HB grade ≥ 3) were categorized into groups based on timing of onset: immediate facial palsy (IFP), “early-onset” DFP (within 48 h), and “late-onset” DFP (after 48 h). IFP patients were subdivided into “minor” (HB grade 3) and “major” (HB grade≥4) groups. These groups were compared with respect to demographics, intraoperative data, and recovery. Results: The late-onset DFP group demonstrated the quickest recovery to HB≤2 (2.9 weeks), followed by the minor IFP group (8.5 weeks), then the early-onset DFP group (53 weeks). Major IFP group exhibited the poorest recovery with only 32% recovering to HB grade≤2 within one year. Conclusions: The bimodal distribution in recovery time in delayed facial palsy patients implies separate underlying phenomena. We propose that a delayed demyelination of the facial nerve occurs in late-onset DFP, and best explains the uniformly rapid recovery observed.

Background: The Flow diversion in the treatment of Intracranial Aneurysm (FIAT) trial was designed to guide the clinical use of flow diversion. Methods: FIAT proposed randomized allocation flow diversion or standard management (observation, coiling, parent vessel occlusion, or clipping), and a registry of non-randomized patients treated with flow diversion. Primary safety outcome was death or dependency (mRS > 2) at 3 months. Primary efficacy outcome was angiographic occlusion at 3-12 months combined with independent clinical outcome. Results: Of 112 participating patients recruited, 78 were randomized, and 34 received flow diversion within the registry. The study was halted for safety concerns. Twelve of 73 patients (16.4%; CI [9.7% -26.7%]) who were allocated or received flow diversion at any time were dead (n=8) or dependent (n=4) at 3 months or more, crossing a predefined safety boundary. Death or dependency occurred in 5 of 36 patients randomly allocated flow diversion and in 5 of 36 patients allocated standard treatment (13.9%; [6.1%-28.7%]). Efficacy was below hypothesized expectations: 15 of 36 patients (41.7%; [27.1%-57.8%]) randomly allocated flow diversion failed to reach the primary outcome, as compared to 11 of 36 patients allocated standard treatment (30.1%; [18.0%-46.9%]). Conclusions: Flow diversion was not as safe and effective as hypothesized. More randomized trials are needed.

Background: Brain arteriovenous malformations (AVM’s) are abnormal connections between arteries and veins. Endovascular glue embolization with N-butyl cyanoacrylate (NBCA) is an accepted form of treatment, with most complications related to timing of polymerization. Current literature reports a wide range of polymerization times with large discrepancies between in-vivo and in-vitro results. Methods: Polymerization time was measured for mixtures of lipiodol/NBCA of 50/50, 60/40, 70/30. The influence of pH, temperature and presence of biological catalysts on polymerization rate was investigated in-vivo using submerged droplet tests. PVA-C, silicone and endothelium surfaces were compared and contact angles were measured to assess physical interaction with NBCA. High-speed video of glue injection through a microcatheter was captured to characterize coaxial flow. Results: Polymerization rate increases with pH and temperature. A hydrophilic substrate such as PVA-C provides surface properties that are most similar to endothelium. Endothelium provides a catalytic surface that increases the rate of polymerization. Blood products further increase the polymerization rate with RBC’s providing almost instantaneous polymerization of NBCA upon contact. Characterization of coaxial flow shows dripping to jetting transition with significant wall effect. Conclusions: We have successfully deconstructed and characterized the dynamic behavior of NBCA embolization. A refined understanding of NBCA behavior could help reduce embolization-related complications.

Background: Neurosurgery patients are at higher risk of falls given the morbidity associated with their neurological disease. We present our department’s experience with in-patient falls. Methods: We analyzed our hospital’s database for Neurosurgery in-patient falls from January 1st till December 31st, 2015. Results: Of 1,317 patients admitted under Neurosurgery, 5% (n=63) had in-patient falls. CT head was done in 24% (n=15) of patients who had a fall and 93% (n=14) of the CT head post-fall was reported as no significant interval change. The combined cost of repeat CT imaging reporting no interval changes was approximately $ 7,000. One CT head post-fall showed worsening midline shift but did not impact management. One of the 78% (n=48) post-fall patients who did not get a CT head progressed to coma requiring emergent surgery and another patient suffered an isolated hip fracture requiring operation. 41% (n=26) of falls were from bed and 37% (n=22) were while ambulating. Leading diagnosis of in-patient falls was subdural hematoma (33%, n=21) and tumour (32%, n=20). Conclusions: Identification of risk factors for in-patient falls can reduce hospitalization costs. The highest number of in-patient falls occurs in patients with subdural hematoma and are likely to occur from a patient’s bed.

Background: Treatment of adult patients with hydrocephalus is often undertaken with a ventriculoperitoneal shunt (VPS). Failure rates have been reported as high as 50% in the first year. Methods: A Quality Improvement (QI) model was used to evaluate and modify VPS-insertion techniques to improve outcome. Malfunction was defined as a change in neurological shunt-related function with correlated diagnostic imaging studies. Prospectively collected data from 2012-2015 was reviewed. Results: 146 patients underwent a new VPS insertion. Diagnoses were: normal pressure hydrocephalus 101 patients, acquired hydrocephalus 28 patients and chronic-congenital hydrocephalus 17 patients. 103 patients had traditional insertion of a ventricular catheter using surface landmarks with 2 catheter misplacements requiring surgery. Image guidance with electromagnetic tracking was instituted with 0 catheter misplacements in 43 consecutive patients. 121 patients had traditional minilaparotomy/trocar placement of the peritoneal catheter with 59/121 (49%) experiencing shunt malfunction and 35/59 (59%) experiencing a second malfunction requiring surgery. Laparoscopic insertion of the peritoneal catheter was instituted in 25 consecutive patients with 3 (12%) distal obstructions. Laparoscopy was also used in 13 patients undergoing VPS revision with 2 (15%) experiencing subsequent malfunction. Conclusions: Changes to standard VPS surgical treatment including the addition of image-guidance and laparoscopic surgical techniques were associated with a significant decrease in shunt malfunction requiring surgery.

Background: The aim of this study was to assess the outcomes of surgery for multiple sclerosis-related trigeminal neuralgia (MS-TN). Methods: All Manitobans undergoing first surgery for medically refractory MS-TN between 2000 and 2014 were identified. The time interval until additional surgeries were required for recurrent pain, defined as the time to fail (TTF), was determined from a retrospective chart review. Kaplan-Meier analyses were performed and outcomes compared. Results: Twenty-one patients (26 sides) underwent first rhizotomy by GammaKnife (GK, 13), glycerol injection (PGR, 10) or balloon compression (BCR, 3). Second procedures were required in 88% at 15±13 months, including GK (24), PGR (19), BCR (25), microvascular decompression (2) and open surgical partial rhizotomy (Dandy, 4) for an overall total of 99 surgeries (1-12 per side). The additional GK, PGR, and BCR eventually failed and required further surgeries in 40%, 60% and 70% at 1, 2, and 3 years respectively with a trend to longer TTF compared to first surgeries (ns). Follow up of Dandy procedures, however, identified no pain recurrence at 4 to 110 months. Conclusions: The minimally invasive rhizotomies for MS-TN were associated with high rates of recurrence and reoperation. Long term pain relief was best achieved with a Dandy procedure, even after multiple prior rhizotomies.

Background: We retrospectively review benign peripheral nerve sheath tumours (BPNST) managed surgically at the Toronto Western Hospital. The incidence of BPNST is classified by anatomic location and predisposition syndrome. Independent predictors of tumour recurrence and symptom resolution are identified. Methods: 175 patients with 201 tumours were eligible for analysis. Data was collected on patient age, gender, diagnosis of neurofibromatosis (NF), tumour histopathology, tumour location, tumour volume, and extent of resection. Postoperative motor, sensory and pain outcomes were dichotomized as stable/improved or worse than preoperative scores. Relationships between tumour recurrence, or symptom resolution, and predictor variables were assessed with univariate and multiple logistic regression models. Results: Among Schwannomas, subtotal resection, a diagnosis of Schwannomatosis, and larger tumour volume were associated with recurrence (p=0.012, p=0.048, p=0.049, respectively); for neurofibromas, subtotal resection and a diagnosis of NF1 were associated with recurrence (p=0.036, p=0.022, respectively). Multivariate analyses revealed subtotal resection as an independent predictor of recurrence for BPNSTs (p=0.007, OR=13.16, 95%-CI 2.34-52.63). Gross-total resection (p=0.023, OR=4.01, 95%-CI 1.21-13.22) and presence of a preoperative motor deficit (p=0.038, OR=8.06, 95%-CI 4.65-90.91) were independent predictors of stable/improved postoperative motor function for BPNSTs. Conclusions: Gross-total resection is associated with both reduced recurrence and improved postoperative motor function, and should be attempted for all eligible BPNSTs.

Background: Accurate prediction of length of stay (LOS) following elective lumbar fusion may help optimize the utilization of resources and may assist with physician and patient expectations. Methods: Outcomes were collected prospectively among patients undergoing elective lumbar fusion. Prolonged LOS was defined as ≥5 days. The influence of baseline and peri-operative characteristics on the odds of prolonged LOS was assessed by a multivariate logistic regression model. Results: 150 patients underwent elective lumbar fusion surgery. Patient characteristics were as follows: average age was 61.9, average pre-operative back pain measured by the visual analogue scale was 54.3, and 36.5% of patients were identified as having severe disability, defined by an Oswestry disability index over 40. The average LOS was 4.9 days, with 28% having a prolonged LOS. Majority of patients had one level fused (69.7%). The odds of prolonged LOS were increased by severe disability (odds ratio [OR] 3.58, p<0.005), levels fused (OR 2.52, p<0.005), greater than 70 years of age (OR 3.81, p<0.005), and any treatment related adverse event (OR 4.32, p <0.02). There was no significant influence of prolonged surgery (p=0.3) or pre-operative back pain (p=0.23) on LOS. Conclusions: Prolonged length of stay was significantly influenced by severe disability, levels fused, age > 70, and any adverse events.

Background: Approximately 95,000 Ontarians, including nearly 15,000 children, have a diagnosis of epilepsy (CCSO, 2015). Management is typically with medication, though surgical resection offers permanent cure in a subsection of patients. A 2012 Health Quality Ontario study estimated a potential 9,300 patients as surgical candidates, but only 150 operations are performed annually, suggesting the surgery option is underutilized. This study attempts to identify reasons for non-referral in a small epilepsy practice. Methods: Evidence based guidelines (Jette, N et al, CMAJ 2014) were used to define eligibility for surgical referral in 274 children with epilepsy. The presence of referral criteria was analyzed. Results: 22 children had clear evidence of drug resistance and one other criteria for referral for epilepsy surgery. 10 referrals had been made. Complex syndromes were present in 40% of the referrals, and 10% of the unreferred. Surgical lesions were present on MRI in 60% of the referrals and 14% of the unreferred. The majority of the unreferred have refractory focal epilepsy but no known surgical abnormality on MRI. Conclusions: In our practice there is a referral bias towards patients with MRI lesions, whereas those without MRI findings tend not to be referred, despite being refractory.

Background: Brain imaging in asphyxiated newborns treated with hypothermia has shown that brain injury can be identified as early as day 2 of life and continue to evolve over the first month of life. Methods: Asphyxiated newborns treated with hypothermia were enrolled prospectively. Apparent diffusion coefficient (ADC) and fractional anisotropy (FA) were measured on magnetic resonance imaging (MRI) performed over the first month of life. Neurodevelopment was evaluated around 2 years of age. Results: Twenty-six asphyxiated newborns treated with hypothermia were enrolled. In asphyxiated newborns treated with hypothermia, who developed cerebral palsy, ADC values were significantly decreased on day 1 of life, on day 2-3 and around day 10 of life in the thalamus. In the same newborns, the FA values were significantly decreased on day 10 of life. Conclusions: Early MRI measurements permitted to identify the newborns developing cerebral palsy as early as on day 1 of life and could thus be used in the future to predict the long-term neurodevelopmental outcome asphyxiated newborns treated with hypothermia.

Background: Genetic leukoencephalopathies are a group of neurodegenerative diseases imposing a great burden on patients and families. There is no previous systematic study looking at the impacts of these diseases. Methods: HRQOL was assessed using the Pediatric Quality of Life Inventory (PedsQL) model. A total of 24 patients with genetically determined leukoencephalopathies and their family members completed the PedsQL questionnaires. Detailed clinical assessments were performed at the time the questionnaires were filled. HRQOL results were correlated with the severity of the clinical features and the presence vs. absence of a definitive molecular diagnosis. Results: Preliminary results show lower PedsQL total scores for patients without compared to with a molecular diagnosis. Emotional and physical functioning scores were significantly impaired in patients without a molecular diagnosis. Lower total scores were obtained for patients who presented more severe clinical features such as lost ambulatory functions and dysphagia. Conclusions: Overall, our preliminary results indicate that patients without a molecular diagnosis have an impaired HRQOL and that more severely affected patients have a poorer HRQOL. Further analyses and studies on a larger population of patients in a prospective fashion are required to assess the burden of these diseases and identify potential modifiable factors.

Background: 4H or POLR3-related leukodystrophy is an autosomal recessive disorder characterized by hypomyelination, hypodontia and hypogonadotropic hypogonadism caused by mutations in POLR3A, POLR3B and POLR1C. The endocrine abnormalities have never been systematically studied. Methods: A cross sectional international multicenter study was performed and the following variables were assessed: weight, height, head circumference, pubertal history, hormone levels and neurological and non-neurological features. Data was analyzed to determine whether there was a correlation between the presence of endocrine abnormalities and mutations in a specific gene and/or the presence of specific symptoms such as other non-neurological symptoms. Results: Data was collected on 156 patients. Endocrine data were available for 144 patients. The most common endocrine abnormalities seen in this cohort were short stature (54/90 patients (60%)) and delayed puberty (53/70 patients (76%)). 13 of the 58 patients tested (22%) had abnormal thyroid function. Patients with POLR3A mutations were more likely to have endocrine abnormalities. Conclusions: Our results confirm that the most common endocrine features in 4H leukodystrophy are short stature and pubertal abnormalities. However, the other potential endocrine abnormalities are typically under-investigated in this patient population. A prospective study is required to investigate the extent and severity of the endocrine abnormalities in 4H leukodystrophy.

Background: Sarcoidosis is a multiorgan autoimmune disease characterized by the presence of non-caseating granulomas. The diagnosis can be difficult, particularly with central nervous system (CNS) involvement, and pathology outside of the CNS has to be carefully evaluated. Early and correct diagnosis is crucial for appropriate management particularly in children where sarcoidosis and neurosarcoidosis are rare. Methods: We describe a 16 year old previously healthy boy who presented with progressive pyramidal neurological signs and symptoms localizable primarily to the brain stem. Results: Initial imaging revealed striking brainstem, as well as cerebral, cerebellar and spinal cord perivascular enhancement. Lung involvement was subclinical with a miliary pattern on chest imaging and needle biopsy revealed an interstitial lymphocytic infiltration. Extensive serum and CSF rheumatological, autoimmune and infectious investigations were noncontributory. Serum ACE levels were at first within normal limits. Steroid treatment stabilized symptoms and perhaps coincidentally, separate rituximab treatments were followed within days by vertigo (with a new pontine lesion) or a respiratory decompensation. A wedge lung biopsy revealed granulomatosis. Current treatment consists of mycophenolate, methotrexate with a prednisone wean. Conclusions: This case report reinforces the varied manifestations and mimics of sarcoidosis (including CLIPPERS) and highlights the need for a high index of suspicion despite apparently negative investigations.

Background: We investigated motor function associations with age, gender, and D4Z4 fragment size among participants with infantile FSHD. Methods: We collected standardized motor assessments including goniometry, manual muscle testing (MMT), quantitative muscle testing (QMT), and FSHD clinical severity scores (CSS) at 12 CINRG sites. To measure associations, we used linear regression models adjusted for age at enrollment, onset of weakness, gender, and D4Z4 repeats. Results: 53 participants (59% female, mean age 23.1±14.6 years) were enrolled. Weakness was most pronounced at the shoulder girdle and rectus abdominis (median MMT 30-38% of normal). Older enrollment age was associated with greater CSS (p=0.005) and reduced range of motion in shoulder abduction, shoulder flexion, elbow flexion, and ankle dorsiflexion (all p<0.01). Females and participants with larger D4Z4 repeats had milder shoulder/arm weakness and lesser disease severity (all p<0.05). Increased age at onset of facial weakness was significantly associated with greater total muscle strength, as measured by QMT and MMT (both p=0.002). Conclusions: We confirm the descending pattern of muscle involvement and milder disease severity in females or those with larger D4Z4 repeats. Furthermore, earlier age at onset of facial weakness was associated with greater muscle weakness. Future longitudinal assessments will describe rates of disease progression in this population.

Background: The Supreme Court of Canada (SCC) recently rendered a decision striking down the Criminal Code absolute prohibition on providing assisted dying. End of life decisions are commonly encountered by neurosurgeons due to the nature of their practice. Neurosurgeons will be faced with patients requesting PAD in the near future. Methods: The recent SCC ruling heralds a change that will radically alter a most basic tenet that has historically guided physicians and surgeons. A subcommittee of the Canadian Neurosurgical Society (CNSS) was formed to generate a position statement to reflect the interests of both neurosurgeons and their patients. Results: Fundamental issues regarding the implementation of PAD identified include:

Clarity of legislation

The creation of an independent, third party referral service

Effective safeguards and oversight of the entire process

-The right to “conscientious objection” on the part of hysicians who do not wish to be involved in PAD

Conclusions: The CNSS urges clarity in legislation regarding PAD and strict oversight in its implementation to reduce potential harm. We also support the creation of an independent, third party referral service which would serve to respect the conscience of those health care providers who do not wish to actively participate in PAD.

Background: The goals of evidence-based neurosurgery are to improve surgical outcomes, reduce complications, and provide an objective basis for altering practice. The need for higher quality studies, typically prospective and multicentre, has been growing especially in light of the evolving complexity of neurosurgical interventions and heterogeneity of patient populations. In the United Kingdom (UK), trainee-led research collaboratives have been established to tackle this problem. Therefore, we sought to evaluate the potential role for a resident-led research collaborative in neurosurgery in Canada based on the UK experience. Methods: A literature review of trainee-led collaboratives was conducted utilizing PubMed and Medline. Identified articles were reviewed for study quality and clinical relevance to explore the potential benefits of collaboratives. Results: In the UK, 27 collaboratives have been established in various specialties by trainees. Some published high quality trials with implications on their clinical fields. Evidence suggests that such endeavors improves trainees’ research skills and may help cultivate a research culture tailored towards clinical trials. Conclusions: Given the growing evidence for research collaboratives in the UK, we propose launching the Canadian Neurosurgery Research Collaborative (CNRC) which currently represents 12 out of 14 neurosurgery programs in Canada, and planning its first multicenter prospective study.

Background: In contrast with 56% of US medical schools, most Canadian medical schools do not offer a required clerkship neurology rotation. This study aims to assess the need for additional clinical neurology training in Quebec medical schools. Methods: Third and fourth year medical students from the province of Quebec completed surveys inquiring about accumulated theoretical teaching time, clinical neurology exposure, self-reported neurological examination proficiency and interest in additional training. Results: 66 students answered the survey. 43% were from Université de Montréal, 18 % from McGill University, 14% from Université Laval and 24% from Université de Sherbrooke. For theoretical teaching, 44% reported at least 60 hours (h) of teaching, 44% reported 40 to 60 h and 23% reported 10 to 40 h. For clinical exposure, 24% reported at least 60 h, 8% reported 40 to 60 h, 40% reported 10 to 40 h and 29% reported less than 10 h. Most students reported being comfortable with their neurological examination skills (58%) but still 41% were uncertain or felt uncomfortable. 80% indicated interest in receiving additional clinical exposure. Conclusions: Amongst Quebec medical students, clinical neurology exposure is likely insufficient. An important proportion of students remain uncomfortable with the neurological examination and most students are interested in additional neurological training.