Background: Patients with spontaneous intracerebral hemorrhage (ICH) suffer significant morbidity and mortality with lengthy critical care and hospital stays. Minimally invasive techniques for ICH removal have shown a positive relationship between hemorrhage volume reduction and patient outcome. We describe our single centre experience with endoscopic assisted, neuronavigation guided ICH evacuation using the Apollo system. Methods: Patients with ICH treated with the Apollo system since October 2014 were included in this retrospective review. ICH volume, clot reduction, midline shift, ICU and hospital length of stay, discharge disposition and last known functional outcome were assessed. Results: 58 patients were treated, mean age 54.1 years. Starting clot volume was 55.1±30.5cc, which was reduced to 10.2±12cc post-operatively, an average reduction of 80.6±25%. Midline shift improved from 7.1±4.5mm to 4.4±3.2mm. Length of ICU stay was 10.2±7.6 days. Covariate analysis showed greater relative reduction in ICH volume correlated with shorter ICU stay (p=0.01). In-hospital mortality was 27.3%; 29.1% of patients were discharged home either form hospital directly, or after a period of short-stay rehab. Conclusions: Significant hematoma volume reduction and improvement in midline shift is possible with the Apollo system. Degree of reduction of hematoma volume was associated with a shorter ICU Stay. Randomized controlled studies will be required to determine long term clinical benefit.

Background: The Canadian Neurosurgery Research Collaborative (CNRC) was founded in November 2015 as a resident-led national network for multicentre research. We present an annual report of our activities. Methods: CNRC meetings and publications were reviewed and summarized. The status of ongoing and future studies was collected from project leaders. Results: In its first year, the CNRC produced two papers accepted for publication in the Canadian Journal of Neurological Sciences: A CNRC launch letter and a study of operative volume at Canadian neurosurgery residency programs. Three manuscripts are in preparation: 1) a study of the demographics of Canadian neurosurgery residents, 2) an assessment of mobile devices usage patterns and 3) a validation study of the most utilized neurosurgery mobile apps. In addition, protocols for two multi-centre studies are currently undergoing national Research Ethics Board review: A retrospective study of the incidence and predictors of cerebellar mutism and a prospective registry of external ventricular drain procedures and complications. The network is now a registered not-for-profit organization endorsed by the Canadian Neurosurgical Society. Conclusions: The CNRC is a feasibile, relevant and productive resident-led national research network. As the CNRC matures, we look forward to expanding the scope and impact of its projects.

Background: Historically, description of patient visual acuity and visual field changes following intracranial procedures has been very rudimentary. Clinicians and researchers have relied on the use of vague descriptions like “improved”, “worsened”, and “unchanged” to describe outcomes following resections of tumours affecting the optic apparatus, which are difficult to quantify in a clinical setting. Methods: We present a novel way to describe a patient’s visual function as a combination of visual acuity and visual field assessment that is simple to use and can be used by surgeons, and researchers to gauge visual outcomes following tumour resection. Results: With our scale we were able to capture the overall visual change while being sensitive enough to define the overall quantity of improvement or worsening quantitatively, using categories that are clinically relevant and understandable. Conclusions: The implementation of pre- and post- operative assessment provides clinically relevant information for surgeons and is robust for routine use.

Background: Occasionally low grade gliomas (LGGs) are identified incidentally while asymptomatic. The diagnosis of incidental LGGs has become more frequent due to increase in access to medical imaging. While management of these lesions remains controversial, early surgery has been suggested to improve outcome. Methods: All LGGs treated between 2004 and 2016 at our institution were reviewed. Patients with incidentally discovered glioma were identified and retrospectively reviewed. “Incidental” was defined as an abnormality on imaging that was obtained for a reason not attributable to the glioma. Outcomes were measured by overall survival, progression free survival and malignant progression free survival. Results: Thirty-four out of 501 adult patients who were treated for low grade glioma were discovered incidentally. Headache (26%, n=9) and screening (21%, n=7) were the most common indications for brain imaging. The mean duration follow up was 5 years. Twelve patients had disease progression, 5 cases of malignant progression and 4 deaths. Oligodendroglioma was diagnosed in 16 and astrocytoma in 15 patients. Twenty-five (74%) patients had IDH1 mutation and demonstrated prolonged survival. Conclusions: This retrospective cohort of incidentally discovered LGGs were surgically removed with minimal surgical risk. There is improved overall survival likely attributable to the underlying favorable biology of the disease indicated by the presence of IDH1 mutation.

Background: Neurocysticercosis is the world’s leading cause of epilepsy and the most common helminthic disease affecting the human nervous system. It is relatively rarely seen in developed nations, and usually presents clinically with seizures. Methods: This case report was prepared using the patient’s hospital chart, and a review of the literature was undertaken using PubMed. This case was subsequently compared and contrasted to the known neurocysticercosis literature. Results: This is the case of an otherwise healthy 38 year old Nepalese female who presented with a history of headaches. Nonspecific in nature, they had worsened in the past couple of weeks, thus prompting appropriate imaging of the head. A large 4 cm ring enhancing lesion with edema and mass effect was discovered in the right anterior temporal lobe. No other neurological findings were found on exam. Pathological analysis confirmed a larval scolex of T. solium. Conclusions: Aside from being an unusual pathology to be seen in Manitoba, this case is unique in both its clinical and radiographic presentations. There were no seizures noted on presentation, and a significant amount of mass effect was seen around a large lesion, all unusual features for neurocysticercosis.

Background: Recurrent ophthalmoplegic cranial neuropathy (ROCN), previously called ophthalmoplegic migraine, is characterized by recurrent episodes of headache and ophthalmoplegia of unclear etiology. Characteristic neuroimaging findings can support the diagnosis. Methods: A case report and review of the literature. Results: We present a 6-year-old girl with a past history of migraine headaches associated with retroorbital pain since 4 years of age. Family history is positive for migraine. She presented with a half a day history of left eye ptosis, 10 days post a resolved gastroenteritis which was associated with headaches. Examination showed only a left eye ptosis, pupil-sparing with no exotropia or diplopia. There was no headache. The rest of the neurologic examination was normal. Investigations showed normal blood tests and lumbar puncture. MRI head showed on thin cuts asymmetric nodular thickening (4mm) of the origin of the cisternal segment of the left oculomotor nerve, with corresponding homogeneous enhancement post gadolinium infusion. Clinical resolution occurred spontaneously within 48 hours. A review of the literature highlights focal thickening and enhancement of the affected cranial nerve, with resolution of enhancement post-acute phase. Conclusions: Focal thickening and enhancement of affected cranial nerve is seen in the majority of pediatric ROCN. These findings are best seen with thin MRI cuts and gadolinium infusion.

Background: Stiff person syndrome (SPS) is a rare disorder presenting with progressive stiffness and spasms of the musculature of the trunk and limbs. SPS is reported very rarely in children and adolescents, with 5 cases over 25 years in a recent 99 patient cohort. Methods: Case Study Results: Herein we report a 15 year old female, presenting with acute onset of rapidly progressive spasticity of the lower extremities. Initial exam was remarkable for markedly limited left knee range of motion, in addition to asymmetrical knee spastic catch and hyper-reflexia. EMG revealed almost continuous motor unit activity which dissipated with voluntary muscle contraction. Diagnosis was confirmed by high titres of glutamate decarboxylase (GAD65) antibodies >25,000 units/ml. The patient was initially treated with IVIG, baclofen, and diazepam followed by IV methylprednisolone, with mild subjective improvements. One day following the first rituximab treatment, she achieved spontaneous knee flexion and regained the ability to ambulate independently. There is a residual spastic catch at the knees. Conclusions: This case highlights that SPS, albeit extremely rare, should be considered in the differential diagnosis of acquired spasticity in children. Also noteworthy is the relatively rapid resumption of function with aggressive immunomodulatory treatments in this historically devastating disorder.

Background: ETV/CPC has become an increasingly common technique for the treatment of infant hydrocephalus. Both flexible and rigid neuroendoscopy can be used, with little empirical evidence directly comparing the two. We, therefore, used a propensity-matched cohort and survival analysis to assess the comparative efficacy of flexible and rigid neuroendoscopy. Methods: Individual data were collected through retrospective review of infants < 2 years of age, treated at one of 2 hospitals: 1) Boston Children’s Hospital, exclusively utilizing flexible neuroendoscopy, and 2) Nicklaus Children’s Hospital, exclusively utilizing rigid neuroendoscopy. Patient characteristics and post-operative outcome were assessed. A propensity score (PS) model was developed to balance patient characteristics in the case mix. Results: A PS model was developed with 5 independent variables: chronological age, sex, hydrocephalus etiology, prior CSF diversion, and prepontine scarring. PS analysis revealed that compared to flexible neuroendoscopy, rigid neuroendoscopy had an ETV/CPC failure OR of 1.43 and 1.31 respectively, compared to unadjusted OR of 2.40. Furthermore, in a Cox regression analysis controlled by propensity score, rigid neuroendoscopy had a HR of 1.10, compared to unadjusted HR of 1.61. Conclusions: Much of the difference in ETV/CPC outcome between endoscopy types is attributed to the case mix. An observational study or randomized controlled trial is required to provide evidence-based guidelines.

Background: Pediatric hydrocephalus is one of the most common neurosurgical conditions and is a major contributor to the global burden of surgically treatable diseases. Methods: The authors conducted a literature review around the topic of pediatric hydrocephalus in the context of global surgery, the unique challenges to creating access to care in low-income countries, and current international efforts to address the problem. Results: Developing countries face the greatest burden of pediatric hydrocephalus due to high birth rates and greater risk of neonatal infections. This burden is related to more general global health challenges, including malnutrition, infectious diseases, maternal and perinatal risk factors, and education gaps. Unique challenges pertaining to the treatment of hydrocephalus in the developing world include a preponderance of postinfectious hydrocephalus, limited resources, and restricted access to neurosurgical care. In the 21st century, several organizations have established programs that provide hydrocephalus treatment and neurosurgical training in Africa, Central and South America, Haiti, and Southeast Asia. These international efforts have employed various models to achieve the goals of providing safe, sustainable, and cost-effective treatment. Conclusions: Broader commitment from the pediatric neurosurgery community, increased funding, public education, surgeon training, and ongoing surgical innovation will be needed to meaningfully address the global burden of untreated hydrocephalus.

Background: Absence epilepsy (AE) is believed to be generated by a thalamocortical network. Our laboratory showed that hippocampal neuronal firings were synchronous with the SWDs in the gamma butyrolactone (GBL) model of AE in rats. Here, we hypothesize that high frequency oscillations (HFOs) in the hippocampus and other parts of the limbic system were phase modulated by SWDs Methods: GBL (200 mg/kg i.p) was injected to induce SWDs in 6 male Long-Evans rats. Spontaneous local field potentials (LFPs) were recorded from electrodes implanted in the hippocampus and ventrolateral thalamus bilaterally and the right frontal cortex. For each LFP, modulation index (MI) gives the cross-frequency amplitude modulation of the HFOs (;90-250 Hz) by the phase of the SWD frequency at 2-8 Hz Results: Phase modulation of the HFOs by 2-8 Hz frequency increased for >45 min after GBL injection. MI increase was higher for hippocampal than thalamic LFPs, and not significant for frontal cortical LFP. MI for the nucleus accumbens LFP (N= 1 rat) also increased after GBL Conclusions: The modulation of HFOs (presumed local neural activity) by SWD frequency provides further support that the hippocampus and connected limbic system may become synchronous with the SWDs in AE

Background: Deep brain stimulation (DBS) is the application of electrical currents via depth electrodes to regulate neuronal activity. DBS has been shown as a productive intervention for seizure control in patients with drug-resistance. This case had both a failed response to antiepileptic drugs (AEDs) and a temporal lobectomy. Methods: This case details the evolution of epilepsy in a 29-year-old female with seizures since the age of around 10. The patient has been followed for 6 months to monitor the treatment effects. No medication changes were made post-procedure. Results: The patient experienced a seizure frequency of 2-5 events per month. The patient had a right temporal lobectomy at age 12, which led to only 3 years in remission. The events are complex partial seizures characterized by unresponsive staring, lip smacking, hand automatisms and confusion. The patient failed 7 AEDs. Intracranial recording showed the most frequent activity coming from the left anterior and posterior hippocampus. Two depth electrodes were implanted accordingly. Stimulation began in September 2016 and the patient has since had only 2 seizures. Conclusions: Deep brain stimulation provides extensive relief for this case of intractable epilepsy. The patient’s level of awareness, mood, and quality of life all improved significantly in response to treatment.

Background: Epilepsy surgery remains one of the most underused of all current medical interventions. It is estimated that less than 1% of patients with drug-resistant epilepsy are referred to specialized centers. The average delay from onset of seizures to surgical procedures is between 10 to 20 years Methods: In this study we describe the waiting times from the diagnosis of epilepsy to the assessment and epilepsy surgery at the epilepsy center of the University of Saskatchewan (SEP) Results: We included 70 patients assessed in the epilepy program between 2007 and 2015. Mean age of patients was 42.2+13. The time from the diagnosis of epilepsy to the referral was 16 years. The times for the for assesment and testing were the following: to the first consult with the epileptologist was 208 months, to the neuropsychological test was 201 months, to the video-EEG telemetry 219 months, to the first consult to neurosurgery 227 months and finally to the epilepsy surgery was 238 months. Conclusions: This study shows that patients with complex epilepsy in Canada are waiting significant time to be properly diagnosed and treated. Our study shows similar waiting times to otehrdeveloped countries suggesting a global problem in the reference of patients to epilepsy programs.

Background: Childhood epilepsy has increased in global incidence. Children with epilepsy require immediate healthcare evaluation and monitoring. Waiting times between first seizure onset and pediatric neurology assessment may impact seizure outcome at follow-up. Quality of medical care for children with first seizure onset will be assessed and the impact of pediatric neurology clinic waiting times on seizure outcomes will be determined Methods: This retrospective study, based on chart review, includes patients with first seizure evaluation at the Royal University Hospital in Saskatoon between January 2012 and December 2015. The interim period before first assessment and other factors were studied in relation to seizure outcome on follow-up. Results: 1158 patients were assessed. 378 (32.6%) patients had first seizure clinic assessment. 197 (52%) had epileptic events. 181 (48%) had non-epileptic events. The mean age of patients was 8.8 years. The mean waiting time for assessment by a pediatric neurologist was 4.33 months. The mean duration of follow-up was 20.9 months. At the last seizure assessment, 132 patients were free of seizures and 65 patients had a recurrence of seizures. Conclusions: First seizure assessment is crucial for management of children with epilepsy. Waiting time and other factors may influence seizure outcome, representing opportunities to improve standard medical care.

Background: To promote patient-centred care in epilepsy, it is essential to understand the issues most important to patients. Literature on patient perceptions of epilepsy treatments is sparse. One source of data is online patient support groups. Patients turn to social media for support from other patients and often express viewpoints not shared with healthcare providers. Methods: Using a qualitative content analysis approach, we analyzed major online epilepsy patient support groups. We initially selected a month-long discussion text across these forums, and further threads were sampled with maximum variation until theme saturation was reached. For data coding and analysis, we employed a combination of a priori codes and emergent codes, using NVivo 11 for data analysis. Results: In our preliminary analysis, we identified topics and categorized them into themes: 1) differential perceptions and understandings of epilepsy; (2) understanding treatment options; (3) experiences of physiological and psychological treatment side effects; (4) concerns about healthcare providers’ knowledge and communication regarding treatments. Conclusions: Preliminary results indicate a variety of patient perceptions and understandings of epilepsy and its treatments. Our findings also suggest that patient educational needs should be addressed by incorporating their understanding and concerns. Shared-decision making tools informed by patient perceptions could help healthcare providers better communicate treatment options with patients.

Background: Focal cortical dysplasia (FCD) refers to malformation of cortical development featuring abnormalities of cortical layering, neuronal differentiation and maturation. It is a common cause of medically refractory epilepsy. The coexistence of FCD and low-grade glial neoplasms such as ganglioglioma and dysembryoplastic neuroepithelial tumour is classified by the International League Against Epilepsy as “FCD Type IIIb”. We present a case of FCD Type IIIb associated with low grade oligodendroglioma (WHO grade II) in a seizure free patient. Methods: A 20-year-old male presented with suspected arteriovenous malformation of the right pinna. Imaging revealed an incidental right frontal lobe mass. Surgical resection was performed. Pathologic analysis revealed FCD Type IIIb associated with low grade oligodendroglioma (WHO grade II). Results: The patient recovered uneventfully. Only 4 prior cases of FCD Type IIIb associated with oligodendroglioma have been reported. This is the first reported case of FCD Type IIIb discovered incidentally in a seizure free patient. Conclusions: FCD Type IIIb associated with oligodendroglioma is rare. The mechanism(s) by which glioneuronal neoplasms and perilesional cortical tissue jointly contribute to epileptogenicity have not been clearly defined. There may be a reduced risk of seizures with oligodendroglioma rather than tumors with a neuronal component.

Background: Morphometry and connectivity studies targeting the thalamus have revealed specific patterns of atrophy and deafferentiation in patients with temporal lobe epilepsy (TLE). We used probabilistic tractography to investigate thalamic connectivity with respect to duration of epilepsy and surgical outcomes in TLE. Methods: Patients (N=20) with drug-resistant TLE (10 short duration (<15 years), 10 long duration (>15 years)) were scanned with multi-parametric 3T MRI and compared with 34 healthy controls. The Harvard-Oxford atlas was modified to create 14 target regions in the temporal lobes. Probabilistic tractography (FSL) was used to delineate thalamic sub-regions most connected to each target. The volume, mean T1, T2, FA and MD of each thalamic sub-region was quantified. Surgical success was quantified using Engel outcome scores. Results: Significant decreases in thalamic connected volumes to the hippocampus in patients with longer duration of TLE were revealed. Likewise, when stratified based on surgical success, significant differences in diffusion metrics to the hippocampus, parahippocampal gyrus, and temporal neocortex were found. Significant differences did not withstand false discovery rate (FDR) correction. Conclusions: These findings suggest ongoing connectivity changes dependent on epilepsy duration and promote further investigation into the use of thalamic connectivity data as biomarkers for predicting surgical outcomes in TLE patients.

Background: The KCNT1 gene encodes subunits of the Na+-activated K+ channel, widely expressed in the CNS. Mutations of this gene have been implicated in Malignant Migrating Partial Seizures of Infancy (MMPSI). This early-onset epileptic encephalopathy represents a challenge due to pharmacoresistance. The channel-specific mutation represents the potential for targeted pharmacotherapy. Quinidine is a partial antagonist of the KCNT1 encoded channel; patients with MMPSI have been reported to have responded to doses ranging 34.4/kg/d - 60mg/kg/d. We present a case of MMPSI with a KCNTI mutation (c.G1283A:p.R428Q) trialled on quinidine. Methods: Following ineffective trials of 6 anti-seizure medications, this patient was trialled on oral quinidine. This patient was titrated up to a dose of 52mg/kg/d. Twenty-four hour EEG monitoring prior to quinidine therapy, and at target dose were compared. Results: Prior to initiation of quinidine, this patient experienced 22 electrographic seizures over 24 hours. At target dose, this patient experienced greater than 70 seizures over 24 hours. Conclusions: Quinidine has previously been reported to be effective in patients with MMPSI with the same and different mutations. We report the second case of a patient with MMPSI and KCNT1 mutation R428Q with poor clinical response to quinidine.

Background: A significant part of the developmental delay (DD) population has epilepsy (26-70%) and live in an institution. These patients tend to have atypical presentation of epileptic seizures with higher risk of misdiagnosis. Distinguishing their ictal events from paroxysmal behaviors can be challenging.There often is a lack of description of the spells or inadequate history from the caregivers or the patients. These patients often have drug resistant epilepsy requiring polypharmacy with increased risk of morbidity and mortality. The aim of this study was to determine usefulness of Epilepsy Monitoring Unit (EMU) in diagnosis and management of these patients. Methods: This is a retrospective observational study of the patients with epilepsy and DD living in institutions that were admitted to the EMU. Results: Four patients met the inclusion criteria for this study. The mean age was 45(29-71), 3/4 (N=3) were male and 3/4 had focal epilepsy. All patients had mood disorders and 2 were taking antipsychotic medication. The mean admission-time was 6,25 days

(2-15) and there was a correlation with the events and seizures in 2/4 of the patients and the rest had a combination of behavioural-changes and seizures. Conclusions: EMU admission can provide an accurate diagnosis of spells in patients with DD and epilepsy, and improve their quality of life.

Background: Perampanel (PER) is a new anti-seizure medication that inhibits the α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid (AMPA) class of glutamate receptors. It is available in Canada for children since 2014. It is important for physicians to be aware of the efficacy and tolerability of drugs in the post-marketing phase. Methods: We did a retrospective review of our experience with PER at BC Children’s Hospital. Patients on PER were identified. Clinical data, including demographics, efficacy, tolerability, adverse effects (AE) and retention rates were obtained by review of clinical records. Results: Of 24 patients pediatric patients prescribed PER, 21 (87%) had focal and three had symptomatic generalized epilepsy. Ten (42%) had greater than 50% reduction in seizures. In fifteen patients, (63%) PER was discontinued due to AE or poor response. Twelve (50%) had behavioral AE and eight (33%) had non-behavioral AE. PER was effective, at lower doses than required for adults. One third experienced serious AE. One patient experienced oculogyric crisis, not previously reported with PER. AE were not associated with high doses and were reversible. Possible risk factors for behavioral AE include behavioral problems with other medications and pre-existing behavioral co-morbidities. Conclusions: It is important for clinicians to be aware of and counsel patients about serious AE, particularly behavioral, when prescribing PER.

Background: Hemimegalencephaly (HME) is a hamartomatous malformation of one cerebral hemisphere, resulting in refractory epilepsy, intellectual disability, and autistic features. Hemispherectomy is the definitive treatment, but there is risk of high morbidity and mortality, especially when done in early infancy. Various preclinical studies have shown that dysregulation of the mTOR pathway has an integral role in the development of various epilepsy syndromes, including tuberous sclerosis complex (TSC), focal cortical dysplasia and HME. Recently, mTOR inhibitors were proven to be effective in treating seizures in TSC. Methods: We present a case of a 6 day old female with refractory epilepsy despite the trial of 9 anti-seizure medications and the ketogenic diet. As the patient was awaiting epilepsy surgery, an mTOR inhibitor, rapamycin was initiated. Results: After 1 week of the initiation, she had over a 50% reduction in seizures. At two weeks, the parents felt that for the first time, she was making developmental gains. She also appeared brighter and more interactive. Due to her response to treatment, her hemispherectomy was deferred to when she is older, so there will be a decreased risk of complications from the surgery. Conclusions: This case exemplifies how mTOR inhibitors should be considered as a treatment option for patients with HME and refractory epilepsy.