Background: Visual impairment exists for an estimated 70% of individuals who have experienced a stroke. Identification and remediation of visual impairments can improve overall function and perceived quality of life. Our project aims to improve visual assessment and timely intervention for patients with post-stroke visual impairment (PSVI). Methods: We conducted a quality improvement initiative to create a standardized screening and referral process for patients with PSVI to access an orthoptist. Post-stroke visual impairment was assessed by way of the Visual Screen Assessment (VISA) tool, administered by an occupational therapist. Patients filled out a VFQ-25 questionnaire before and after orthoptic assessment and intervention. The VFQ-25 is a validated post-stroke survey assessing a patient’s perceived quality of life. Differences between pre- and post-orthoptic assessment scores will be evaluated. Results: Data collection currently ongoing.The benefits of a standardized screen for PSVI, standardized referral to, and experience with an orthoptist assessment will be determined. Learnings gained will also inform how we can expand the program to benefit a wider demographic of patients. Conclusions: The data gathered and the subsequent analysis will be instrumental in guiding ongoing improvement initiatives for patients with PSVI.

Background: Aβ-related angiitis (AβRA) is a rare presentation of cerebral amyloid angiopathy, where vasculitis results from an auto-immune reaction to amyloid deposits in leptomeningeal and cortical vessel walls. Anti-CD20 monoclonal antibodies, such as rituximab, have demonstrated efficacy in systemic small vessel vasculitides, particularly in refractory cases. The efficacy of rituximab in AβRA remains unknown. Methods: Patient chart, functional measures, and laboratory findings were reviewed from the time of patient admission until 12 months after discharge. Results: A 61-year-old man presented with headache and altered mental status. Brain MRI revealed multiple cortical infarcts, leptomeningeal enhancement, and cortical microbleeds, and brain biopsy ultimately confirmed the diagnosis of AβRA. The patient developed new ischemic lesions despite corticosteroid pulse, and intravenous cyclophosphamide was halted after four weeks due to iatrogenic acute hepatitis. Rituximab was initiated and led to sustained clinical improvement with no subsequent relapses. Maintenance therapy involved gradually tapered low-dose oral steroids and rituximab at 6- and 12-months post-induction. Conclusions: This report suggests that rituximab may be effective in inducing remission and preventing relapses in biopsy-proven case of AβRA. Controlled studies are needed to better assess the efficacy and tolerability of anti-CD20 antibodies in cerebral vasculitis.

Background: Fatal Familial Insomnia (FFI) is an autosomal dominant multisystem prion disease, with sleep disorders often being the first presentation. Although autonomic dysfunctions are key features, the frequency and timing vary between reports, and may accompany early insomnia. Moreover, endocrine changes are reported, but diabetes rarely is - with unclear timing of onset in relation to the insomnia. Methods: N/A Results: Here we present a 46-year-old previously healthy male, who within 22 months prior to the onset of sleep disturbances, developed hypertension and diabetes. Then within 3-4 months after onset of sleep disturbances development tachycardia and diaphoresis. His sleep continued to deteriorate, and later developed bulbar impairment, ataxia, diplopia, sleep apnea and cognitive decline. He passed away 20 months from onset of insomnia. Polysomnography showed status dissociates and central apnea. He had positive genetic testing, PRNP c.532G>A (p.Asp178Asn) and PRNP c.385A>G (pMet129Val), a pathological confirmation, and a positive family history Conclusions: Here diabetes and hypertension significantly preceded sleep disturbances, and tachycardia and diaphoresis developed shortly after. This illustrates that dysautonomia and endocrine dysfunction may be unrecognized prodromes in some cases of FFI, and could be an early marker of clinical disease onset and therapeutic interventions, especially in genetically confirmed asymptotic patients.

Background: Orbital apex syndrome (OAS) can be caused by a broad range of disorders. There are several challenges present in the evaluation of these patients and in reaching a final diagnosis. We report the case of a 69-year-old male who presented with OAS that was determined to be secondary to a rare malignancy (NK cell lymphoma, nasal type). Methods: We analyze the pitfalls and diagnostic delays in this patient’s evaluation. Furthermore we propose a work up for undifferentiated cases of OAS. Results: To accurately diagnose the underlying cause of OAS, a direct biopsy should be obtained whenever possible. The appropriate imaging sequences should be arranged as lesions in this region can be easily missed. Adjunct tests include assessment in the serum and CSF for granulomatous and infectious diseases, along with chest imaging. As many causes are PET enhancing, PET CT is a useful modality for identifying sites for biopsy. Conclusions: OAS can provide a diagnostic challenge for clinicians, however a systematic approach can help determine the underlying etiology.

Background: Parkinson Disease (PD) is a neurodegenerative disorder, resulting in a gradual decline in voluntary movement, where lifespan remains stable. Drosophila melanogaster offer comparable gene sequences to those targeted in PD; among them are two transcription factors, engrailed (en) and invected (inv). Methods: Wild-type homozygous allele Oregon-R(en+, inv+) was compared to heterozygous mutants of en1, en4, en7, en54, en58, invW, inv30, and Df (2R) enEinvE. Nine climbing and aging studies were executed from crosses with w1118(en+, inv+) as the maternal genotype. Results: Independent-samples t-tests were conducted to compare the percent survival (in days). No significant differences were observed between the experimental groups and the control group. A mixed Analysis of Variance was conducted to compare climbing behaviour over time (in weeks) for all nine groups. Both main effects (group, time), and the interaction (group x time) were significant. Post hoc Fisher’s Least Significant Difference tests revealed a significant difference between the control group and en1, en4, en54, invW, and Df (2R) enEinvE groups. Conclusions: These results support the hypothesis that mutations of en, inv, or both will result in a PD phenotype and consequent decreased motor function of D. melanogaster PD models, with or without a significant decrease in lifespan.

Background: Intravenous immunoglobulin (IVIG) may benefit many inflammatory central nervous system (CNS) disorders based on multiple immunomodulatory effects. IVIG is being used in inflammatory CNS conditions however robust evidence and guidelines are lacking in many disorders. Over the last 5 years, the percentage of IVIG used for CNS indications within neurology almost doubled in British Columbia (BC), Canada. Clear local guidelines may guide rational use. Methods: Consensus guidelines for IVIG use for CNS indications were developed by a panel of subspecialty neurologists and the Provincial Blood Coordinating Office, informed by focused literature review. Guidelines were structured similarly to existing BC peripheral nervous system guidelines and Australian Consensus Guidelines. Utilization and efficacy will be monitored provincewide on an ongoing basis. Results: Categories of conditions for Possible Indication (N=11) and Exceptional Circumstance Use (N=4) were created based on level of evidence for efficacy. Dosing and monitoring recommendations were made and outcomes measures defined. Rationale for Not Indicated conditions (N=3) was included. Guidelines will be distributed to BC neurologists for feedback and re-evaluated after 1 year. Conclusions: IVIG use in CNS inflammatory conditions has an emerging role. Guidelines for use and monitoring of outcomes will help improve resource utilization and provide further evidence regarding effectiveness.

Background: Aphasia is a life alerting deficit that affects up to 40% of people living with stroke. Barriers to communication ultimately impacts the care aphasic patients receive, as well as functional recovery. The Canadian Stroke Best Practice Recommendations suggest early and frequent language interventions to improve patients with aphasia quality of life, mood, and social outcomes. Methods: A supported conversation (SC) program (colloquially named The Aphasia Club) was implemented on the Acute Stroke Unit (ASU). The program included aphasia awareness and assessment training, as well as creation of an aphasia tool kit and discipline specific aphasia-friendly resources. Staff were encouraged to complete a 1-hour independent course on SC through the Aphasia Institute. Speech and language pathologists (SLP) offered an additional 30-minute in-person teaching session with interdisciplinary practice professionals. Following SLP assessment, personalized communication profiles were created for patients with aphasia to help staff understand the most useful strategies for communication. Results: More then 50 interprofessional staff members took SC training. Staff reported increased levels of knowledge and confidence when communicating with aphasic patients. Conclusions: A supported communication program was successfully implemented on an ASU. Planning appropriate communication interventions can assist interdisciplinary professionals in their ability to support patients through their stroke journey.

Background: The novel corona virus pandemic presented the Saskatoon Stroke Program with challenges related to patient- and caregiver-centered communication. Keeping all parties informed of a patient’s health status and plan of care in the setting of extreme visitation restrictions was difficult. Virtual interdisciplinary bedside rounds (VIDR) were introduced to enhance communication for stroke patients. Methods: A video conferencing application was adopted by the Saskatchewan Health Authority. Consent to participate was obtained by a social worker. Bedside nurses facilitated patient participation in VIDR on either a tablet or workstation on wheels, while caregivers were able to attend virtually. Each team member accessed the VIDR from an individual device to maintain social distancing. A structured questionnaire has been initiated to capture participant reported experiences and satisfaction with VIDR (data collection ongoing). Results: Most patients and caregivers were amiable to participate in VIDR. Challenges included: accessing appropriate technology for both family and staff members; rural and remote internet reliability; and maintaining a reasonable duration of rounds. There was overwhelming anecdotal positive feedback from participants. Conclusions: We implemented VIDR to enhance communication during the pandemic. Caregivers felt connected to the care team and up-to-date in the plan of care.

Background: The International League Against Epilepsy recommends patients with drug resistant epilepsy (DRE) be referred for surgical evaluation, however prior literature suggests this is an underutilized intervention. This study captures practices of North American pediatric neurologists regarding the management of DRE and factors which may promote or limit referrals for epilepsy surgical evaluation. Methods: A REDCap survey distributed via the Child Neurology Society mailing list to pediatric neurologists practicing in North America. “R” was used to conduct data analyses. Ethics approval from the CHEO REB was granted prior to the start of data collection. Results: 102 pediatric neurologists responded, 77% of whom currently practice in the United States. 73% of respondents reported they would refer a patient for surgical consultation after two failed medications. Of all potential predictors tested in a logistic regression model, low referral volume was the only predictor of whether participants refer patients after more than three failed medications. Conclusions: Pediatric neurologists demonstrate fair knowledge of formal recommendations to refer patients for surgical evaluation after two failed medication trials. Other modifiable factors reported, especially family perceptions of epilepsy surgery, should be prioritized when developing tools to enhance effective referrals and increase utilization of epilepsy surgery in the management of pediatric DRE.

Background: Pediatric occipital epileptiform discharges (OEDS) occur in various clinical settings, including benign and symptomatic epilepsies. The study objective is to determine electro-clinical predictors for aetiology and prognosis in children with OEDs. Methods: 205 patients with OEDs were classified into seizure groups: symptomatic (n=98), idiopathic focal (IF) (n=57), idiopathic generalized (IG) (n=18), no-seizures (n=27) and febrile seizures (n=5). Results: The median age of seizure onset was 3 years (range: 0-19). There was more EEG background slowing (P<0.05) in the symptomatic; photosensitivity (P<0.0001) and GSW (P<0.0001) in IG; and presence of consistent EEG spike dipole in IF group. The symptomatic had more DD (P< 0.0001), autism (P <0.019), and school difficulties (P<0.001) than the IF and IG groups, but not different from the no-seizure group. Conclusions: OEDs with consistent dipole spike is predictive of IF epilepsy. In contrast to frontal and temporal lobe epilepsy, only 30% with symptomatic epilepsy had occipital-predominant neuro-imaging abnormalities. Notably, neuro-psychiatric co-morbidities were similar between the symptomatic and no-seizure group.

Background: Newborns with hypoxic-ischemic encephalopathy (HIE) are at high risk for seizures, the majority of which have no clinical signs and therefore require continuous electroencephalographic (cEEG) monitoring for their detection. We sought to determine which neonates are at highest risk for seizures in order to optimize allocation of scarce cEEG resources. Methods: We identified term neonates diagnosed with HIE who underwent at least 24 hours of protocol-based cEEG monitoring between 2016 and 2019. We quantified seizure incidence, timing and burden, and correlated these with potential risk factors such as HIE severity, use of therapeutic hypothermia, preceding suspected clinical seizures, amplitude-integrated EEG (aEEG) background and patterns suspicious for seizures, and use of anti-seizure drugs. Results: cEEG monitoring was completed in 218 neonates with HIE, of whom 164 (75%) underwent therapeutic hypothermia. Preceding clinical/aEEG seizures occurred in 147 (67%), 99 (67%) of whom had been cooled but only 22 (10%) had cEEG-confirmed seizures. Characterization of seizure burden and correlation with potential risk factors is ongoing. Conclusions: Although seizures are commonly suspected in neonates with HIE, they are infrequently confirmed during cEEG monitoring, creating opportunities for more efficient risk-based allocation of cEEG resources.

Background: Hormonal therapy is a standard treatment for infantile spasms. The high doses given and long treatment duration expose patients to the risk of adrenal insufficiency (AI). This study aims to quantify the incidence of AI among children with infantile spasms treated with corticosteroids and/or adrenocorticotropic hormone (ACTH). Methods: A retrospective chart review of patients treated for infantile spasms was performed between January 2009 to March 2020 in one pediatric specialized hospital. Variables collected included patient and treatment characteristics, risk factors of AI and adrenal function testing. Analysis included descriptive statistics. Results: Thirty-one patients met the inclusion criteria and received a total of 33 separated courses of treatment. Adrenal function following each course of treatment was evaluated in all patients. AI occurred in 25/33 (76% [95CI 58-89]) children. There was no predictive factor of AI. No drug regimen was deemed safe. The two patients (6%) with an acute adrenal crisis were the youngest of the cohort. Conclusions: Adrenal suppression is frequent and can lead to adrenal crisis after standard hormonal therapy for infantile spasms. A routine laboratory assessment of adrenal function should be done for all patients. Hydrocortisone replacement therapy should be given until testing results are obtained, particularly for younger infants.

Background: Infantile spasms (IS) is an epileptic encephalopathy, characterized by epileptic spasms, hypsarrhythmia, and developmental regression. This is a retrospective case series detailing the experience in children with IS who have undergone epilepsy surgery at The Hospital for Sick Children (HSC). Methods: Records of 223 patients from HSC were reviewed. Patients were included if they had a current or previous history of IS with a lesion detected on MRI/PET scan who underwent epilepsy surgery. Results: Nineteen patients were included. The etiology of IS was encephalomalacia in six patients (32%), malformations of cortical development in 11 patients (58%), atypical hypoglycaemic injury in one patient (0.5%), and partial hemimegalencephaly in one patient (0.5%). The median age at the onset of IS was five months. The median age at surgery was 18 months. Nine patients (47%) underwent hemispherectomy and 10 patients (53%) underwent lobectomy/lesionectomy. Fifteen patients (79%) were considered ILAE Seizure Outcome Class 1. Developmental outcome was improved in 14/19 (74%) and stable in 5/19 (26%) patients. Conclusions: Even with a generalized EEG pattern such as hypsarrhythmia, patients should be considered for focal resective surgery. Early surgical intervention shortens the duration of active epilepsy thus limiting the potentially irreversible effects of on-going seizures.

Background: We aimed to study the proportion of patients with DRE and pre-existing VNS device, who show improvement of at least one class in McHugh seizure outcome classification at last follow up after generator replacement with cardiac based VNS device. Methods: We retrospectively reviewed children with DRE with the older VNS model (102) who underwent battery replacement with the AspireSR®, model 106 since September 2016 at our institution. We assessed the seizure outcomes since the first VNS device insertion till the last follow up after AspireSR® (with cardiac-based seizure detection) using McHugh seizure outcome classification. Results: The study population was comprised of 15 patients. The mean age at seizure onset was 2.7 years old, with mean age of initial VNS1 placement being 10.1 years and mean age of replacement with VNS2 being 14.9 years of age. Three of the fifteen patients had reported status epilepticus prior to initial VNS insertion, and none reported episodes following insertion. Two patients showed at least one class improvement in McHugh seizure outcomes at last follow up after VNS2. Conclusions: Through our preliminary data at the present time, we note that the majority of our patients maintains their seizure control following replacement with VNS2 with a few showing improvement.

Background: Epilepsy, a common neurologic condition, instigates a large number of emergency room (ER) visits annually. This project aims to retrospectively review the patterns and characteristics of Alberta ER visits by pediatric epilepsy patients. Methods: Methods: Alberta Health administrative databases, including the Inpatient Discharge Abstract Database, the National Ambulatory Care Reporting System, Diagnostic Imaging and Medical Laboratory, were used to identify ER utilization patterns among children with epilepsy in Alberta, Canada between 2012–2018. Results: Results: Of 5,419 pediatric epilepsy ER patients between 2012–2018 in Alberta, 59% were developmentally delayed children. Children in this particular group, when compared to developmentally normal children with epilepsy, had the following characteristics: they were significantly more likely to utilize ERs in children’s hospitals versus other hospitals; they presented at a significantly younger age; they had a significantly longer length of stay; they had higher triage scores; they were subjected to significantly more investigations; and they had significantly more hospital admissions for epilepsy. Conclusions: Discussion: This novel Alberta-wide study of resource utilization of pediatric epilepsy patients shows that developmentally delayed children with epilepsy use significantly higher resources compared to developmentally normal children with epilepsy. Whether this is justified or not requires further study.

Background: To assess the response to high dose daily nocturnal diazepam (HDD) in children with encephalopathy associated with electrical status epilepticus in sleep (ESES). Methods: A prospective cohort of patients (4-12 years), newly diagnosed with ESES, initiated on the first course HDD, was followed for ≤ 1-year. Sleep EEG scores (SES) pre and post HDD were evaluated. An EEG grading system based on both sleep spike wave index (sSWI) (Grade: 1-4) and distribution of epileptiform discharges (Grade: 0-4) was used and summed to yield an aggregate SES (ASES) (Grade: 1-8). Results: Eighteen eligible children (M:F 12:6; median age, 7.6 years) were initiated on first course HDD (median, 0.5 mg/kg/d). sSWI decreased from 85.7% (mean, SD 13.9) to 32.6% (mean, SD 37.1) at subsequent EEG (95% CI = -70.60- -35.62; p < 0.001). ASES decreased from 6.5 (SD 1.3) to 3.1 (SD 1.9) (95% CI = -4.17- -2.60; p < 0.001). EEG relapse after a period of improvement occurred in 10 children. Minimal response to HDD occurred in 2 children. Five patients manifested mild side effects; behavior (2), hyperactivity (2), and lethargy (1). Conclusions: HDD safely and significantly reduces both SWI and aggregate sleep EEG score in children with ESES.

Background: Seizures in childhood absence epilepsy (CAE) are usually easily controlled with anti-seizure medications (ASMs). Factors predictive of treatment resistance remain unclear. Our objectives were to assess prevalence of neuropsychiatric problems and factors influencing refractoriness in a cohort of CAE at a single centre. Methods: We retrospectively reviewed patients with CAE (ILAE 2017 classification) diagnosed between January 1999 and December 2016 with at least 1-year follow-up. Treatment resistance was defined as failure to respond to two or more appropriate ASMs. Exclusion criteria included eyelid myoclonia with absence, myoclonic absence, and generalized tonic-clonic (GTC) seizure before developing absences. Results: The study population comprised 164 patients (65 males) 6.25-year-old on average at absence onset. 22% had treatment-resistant seizures. The first ASM was Ethosuximide in 63.4%, Valproic acid in 23.2%, and Lamotrigine in 6.7%. Statistical differences between response groups included developing a second seizure type specifically GTC, the second and third ASM, and absence of EEG normalization. At last follow-up, 43.3% of children were seizure-free off ASMs. 32.9% of children had learning disabilities, 28% ADHD, and 12.8 % anxiety. Conclusions: 22% of children with CAE had treatment-resistant seizures. Photoparoxysmal response was not predictive of treatment resistance. Neuropsychiatric problems were common with learning disabilities increased with refractory absences.

Background: Epileptic discharges localized to the midline vertex are rare. However, they have been associated with intractable seizures and severe long-term consequences in the developing brain. Our study aimed to understand the etiology of pediatric midline seizures and define post-surgical seizure outcomes. Methods: We reviewed charts, electroencephalography (EEG), and neuroimaging studies of ten pediatric patients with epileptic discharges localized to the midline vertex in the Comprehensive Epilepsy Program. The seizures were classified according to the International League Against Epilepsy criteria, patient age, sex, neuroimaging results, seizure etiology and outcomes were obtained. Results: Age of seizure onset was within the first 10 years of life in 90% of patients, with focal seizures being the most prevalent. Focal cortical dysplasia (FCD) was the most common etiology present in 50% of patients. These children had normal neuroimaging studies and intractable epilepsy. However, seizure freedom was achieved following surgical resection of the epileptogenic zone. Conclusions: We demonstrated that patients with midline epileptic discharges are associated with intractable focal seizures and early seizure onset. Despite normal neuroimaging reports, FCD was the most common pathology. Thus our study suggests early localization and resection of the epileptogenic zone may be beneficial for achieving seizure freedom in children with this electroclinical syndrome.

Background: Lennox-Gastaut syndrome (LGS) is a severe form of pediatric epilepsy that is classically defined by a triad of drug-resistant seizures, characteristic EEG patterns, and intellectual disability. Long-term prognosis is generally poor with progressive intellectual deterioration and persistent seizures. At present, there are few reported cases of LGS and Trisomy 21 (T21) in the literature. To further delineate the spectrum of epilepsy in T21, we reviewed children with T21 and LGS at one center over 28 years. Methods: This is a retrospective case series. At our institution, all EEG results are entered into a database, which was queried for patients with T21 from 1992-2019. Pertinent electro-clinical data was obtained from medical records. Results: 63 patients with T21 and epilepsy, 6 (10%) had LGS and were included in the study. Four of the six patients were male and 5/6, had neuro-imaging, which was normal. Follow-up ranged from 3-20 years. Notably, 5/6 had predominant myoclonic seizures throughout the course of their epilepsy, associated with generalized spike-wave discharges. Conclusions: Myoclonic seizures appear to be a predominant seizure type in patients with T21, suggestive that T21 patients may have a unique pattern of LGS.

Background: Data on intravenous lacosamide use in young pediatric patients is scarce, especially of pre-school age. Methods: We retrospectively reviewed the medical records of all patients less than 6 years old who received intravenous lacosamide at our tertiary pediatric hospital. Data on dose, timing and order of administration was collected. Clinical and electrographic response was independently assessed with EEG interpretation blinded to time of administration. For adverse effects surveillance, heart rate was noted before and 1 hour after dose. Results: Eleven patients (8 boys), received lacosamide between 2013 and 2018. Mean age was 2 years (11 days – 5,3 years). Medical indications were: refractory status epilepticus (n=6), repetitive seizures (n=4), and inability to take oral lacosamide (n=1). On average, lacosamide was the fifth (1st-8th) IV antiepileptic drug administered 78 hours (SD 11 hours) after presentation. The most frequent dose was 5 mg/kg. Clinical response was confirmed in 7 patients, while electrographic response was proven in 3 patients. Seizure relapse at 24 hours was noted in 6 patients. No bradycardia occurred post-lacosamide. Conclusions: Although very safe, therapeutic response to lacosamide in young pediatric patients was inconclusive, mostly due to delay in administration, suboptimal dose, and high number of other IV antiepileptic drugs previously given.