Background: Dementia is more prevalent in women. Sex differences exist as the disease progresses (e.g. males are more likely to become aggressive). In many medical illnesses (e.g. cardiac disease), there are differences in presentation between men and women. The current study explores sex differences at the patients’ initial presentation to the Rural and Remote Memory Clinic (RRMC). Methods: Patients were referred to the RRMC in Saskatoon, Saskatchewan. Cognitive and demographic data were collected. Questionnaires included cognitive (e.g. Mini-Mental Status Examination) and daily living (e.g. Instrumental Activities of Daily Living) assessments. Results: Three hundred and seventy-five (159 male, 216 female) patients participated. Of these patients, 146 (49 male, 97 female) were diagnosed with Alzheimer’s disease. Males and females presented to the clinic at similar ages. Females were more likely to have a son or daughter caregiver and to live alone. Males were more likely to be currently working and to be a former smoker. No statistically significant differences were found for cognitive assessment scores. Conclusions: Analysis of the initial presentation of patients to the RRMC revealed females and males had similar presentation in measures of cognitive impairment. This may be reassuring for patients and their families knowing their family member, regardless of sex, is receiving equivalent referral to receive care.

Background: With a growing and aging population, the number of individuals with AD and dementias and their associated costs are expected to increase in Canada. Up to now, no national mechanism was in place to monitor the epidemiological burden of AD and dementias. This presentation will showcase the first CCDSS data available on these conditions. Methods: Through the CCDSS, a Federal/Provincial/Territorial partnership, health administrative databases are linked to collect data on chronic conditions. Using selected ICD-9(CM)/ICD-10 codes for AD and dementias, the validated case definition implemented to identify relevant cases aged 65+ is:

1+ hospitalizations; or

3+ physician claims within 2 years, with a 30-day-gap between each claim; or

1+ anti-dementia drug prescriptions.

Prevalence and incidence rates will be presented by 5-year age group, sex, province/territory, and fiscal year. Results: Overall, incidence and prevalence rates were higher in women. The prevalence rate approximately doubled between 5-year age groups and sex differences tended to widen with age. While aged-standardised data show increasing prevalence rates over time, incidence rates fluctuated but suggest a decline since 2009/10. Conclusions: CCDSS data can be used to monitor the burden of AD and dementias in Canada. This information is important for the assessment of prevention actions and the planning of health care resources.

Background: ILI has been in use in Canada since 2011 to treat advanced PD. We review the benefits and complications of ILI for PD in a tertiary movement disorders center in Canada. Methods: Detailed chart review of patients treated with ILI at including motor UPDRS scores, ILI pump and PEG-J tube complications. Patients and caregivers were interviewed at regular clinic follow up about their experience with ILI. Results: 13 patients received ILI [10M, 3F; mean age 65.6 yrs, range (51.8-79.5); PD duration 14.2 yrs, range (9.1-22.0); mean follow-up 1.8 yrs, range (0.2-4.8)]. Patients reported improvement in motor function, decreased dyskinesias and ‘OFF times’ [mean motor UPDRS: pre-ILI 37.1, 1-6months post-ILI 27.5]. Common complications included dislodgement, knotting or blockage of the jejunal tube extension requiring endoscopic re- insertion (29 incidents in 6 patients over 5 yrs). Four patients discontinued Duodopa treatment, for reasons of declining cognition, inability to care for the pump, and/or minimal benefit. Conclusions: ILI is useful for the treatment of advanced PD, in patients that can care for the pump apparatus.

Background: AHEM is a rare form of acute disseminated encephalomyelitis (ADEM) characterized by fulminant encephalopathy with hemorrhagic necrosis and most often fatal outcome. Methods: A case report and review of literature. Results: A 6-year-old girl known SCD presented an acute demyelinating syndrome (ADS) with diplopia due to unilateral fourth nerve palsy. She received (20mg/kg/day for 5 days) of IVMP (intravenous methylprednisolone). Two weeks after steroid weaning, she presented right hemiplegia. Brain MRI showed a left frontal necrotico-hemorrhagic lesion and new areas of demyelination. She showed signs of herniation and underwent craniotomy. Investigations ruled out vascular and infectious process in both events. The deterioration stopped concomitantly to the initiation of IVMP. She was discharged with a longer oral wean. Later, she was diagnosed with Crohn’s disease and primary sclerosing cholangitis. We considered AHEM as the most plausible diagnosis based on the clinical and radiological presentation, the preceding ADS, the exclusion of other etiologies, and the response to IVMP including resolution of non-necrotic lesions. Similar - but fatal - AHEM has been reported in 2 SCD patients. Conclusions: In any ADS occurring in the context of SCD and/or auto-immune condition, we recommend to slowly wean steroids, and to closely monitor the patient after weaning to quickly treat any recurrence with IVMP.

Background: The Gilenya® Go ProgramTM offers education and support services, including coordination of first dose observation (FDO) and follow-up contact to reinforce monitoring recommendations and compliance in fingolimod-treated relapsing-remitting multiple sclerosis (RRMS) patients. Methods: Data were analyzed for patients enrolled in the Canadian Gilenya® Go ProgramTM from March 2011 to January 2016. The retention to fingolimod therapy, reasons for treatment discontinuation and incidence of adverse events (AEs) during treatment are reported. Results: At data cut-off, 3956 patients had completed FDO; 3201 patients were being actively treated. Mean age at enrolment was 41.0 years; 74.9% patients were female. The overall fingolimod exposure was 7869 patient-years. Most recent previous therapies (n=3746) included interferons (43.3%) and glatiramer acetate (29.6%). Most common reasons for switching to fingolimod (n=3674) was lack of efficacy (31.8%). Retention to therapy at data cut-off was 81.3%. AEs (45.2%) were the most common reason (n=334) for treatment discontinuation and included low lymphocyte count/abnormal hematology values (13.8%), gastrointestinal disturbances (6.9%), and elevated liver enzyme levels (7.8%). Adherence to recommended ophthalmic examination was 92.4%. Conclusions: In real-world clinical practice in Canada, adherence to both fingolimod treatment and monitoring was high. The Gilenya® Go Program™ helps to meet the safety monitoring recommendations for fingolimod-treated RRMS patients.

Background: As therapy for MS has expanded to include multiple immunomodulatory and immunosuppressive therapies, the need to consider patient vaccination status has emerged as a salient issue in the treatment of MS. Unfortunately, there is little research or consensus about how vaccination in these patients should be addressed. Methods: A search of primary literature on the topic of potential pathogens and available vaccinations in immunosuppressed patients was performed. We reviewed the limited available information in the MS, gastroenterology, and rheumatology literature. As well, the current Canadian immunization guide was referred to along with expert opinion from public health nurses and infectious disease specialists. Results: There is currently little consensus about vaccination protocols for patients initiating immunosuppressive therapy. We integrated information from all of our sources to create a preliminary protocol for the vaccination of MS patients prior to initiation of immunosuppressive therapy. Conclusions: More work needs to be done to create standardized vaccination protocols for MS patients who will be undergoing immunosuppressive therapy. We have created a preliminary protocol in conjunction with public health to standardize the vaccinations that MS patients receive. We hope that this will streamline immunization of patients immediately after diagnosis of MS so that initiation of immunosuppressive therapy will not be delayed in the future.

Background: Multiple sclerosis (MS) is a neurological disease which is highly prevalent in Canada. To date limited data exists on the characteristics of this population in Ontario. Methods: A retrospective chart review was conducted of initial patient presentations to a MS clinic in 2011. Initial and follow-up consult notes were reviewed. Patients with a previous MS diagnosis were excluded. Results: 81 patients presented to the clinic for the first time in 2011. 41 were given alternative diagnoses (non-MS). Of the remaining 40 patients (MS group), 9 had clinically or radiologically isolated syndrome and 8 were in a progressive phase of MS. The mean age of presentation was 22 (MS group) and 47 (non-MS group). The most common initial symptom in both groups was a sensory disturbance. The mean initial EDSS in the MS group was 1.75 (0-6.5). In the MS group only 35% were put on disease modifying treatments. The most common reasons for exclusion of treatment were progressive disease phase, clinically or radiologically isolated syndrome, and unclear diagnosis. In the non-MS group, the most common diagnoses were non-specific MRI findings, transverse myelitis and peripheral nerve or muscular diagnoses. Conclusions: This retrospective review has outlined the characteristics of a MS population in Ontario.

Background: MRI criteria are used to support multiple sclerosis diagnosis and evolution. However, normal age-related lesions (ARLs) can be cofounded with MS white matter lesion (MSL). Methods: Two Multiparametric 7T MRI scans 4 motnhs apart from 5 relapsing MS (RMS) patients were analyzed and compared to 5 matched healthy controls (HC) aiming to differentiate MSLs from ARLs. Six-echo GRE, FLAIR and MPRAGE sequences were acquired. Results: Average size of ARLs was 51 mm3 and of MSLs was 69 mm3 (p=0.27). Both have the same general appearance on FLAIR and MPRAGE contrasts, but different contrast on the R2* and QS maps. Inter-visit variation on MPRAGE was significantly higher in MSLs. Inter-visit signal change in the other contrasts (QSM, R2* and FLAIR) was not significant. Conclusions: R2*, QS maps and inter-visit variation using MPRAGE allowed differentiating MSLs from ARLs in 5 RMS with mean long term disease duration. This could improve correct early diagnosis and accurate lesion load accumulation evolution.

Background: Preliminary studies have supported the utility of exercise as a treatment for Charcot-Marie-Tooth disease (CMT) patients. Despite being the most common inherited neuropathy, there remains a paucity of guidelines for CMT management. Methods: A retrospective chart review was performed on 297 CMT patients. Self-reported exercise and strength results from standardized dynamometer testing were obtained from adult patients’ first visits. Values were converted and analyzed based on previously reported age and sex matched normative values. Results: Participants with CMT2 were stronger than CMT1 in hand grip, elbow flexion, and dorsiflexion (p<0.05). CMT1A participants were weaker than those with CMT1B/D. Participants with CMT1 and CMT2 who exercised were statistically significantly stronger in elbow flexion and dorsiflexion than those who did not exercise. Conclusions: These preliminary results suggest that self-directed exercise is associated with greater strength in patients with CMT. Furthermore, they support the evidence that the dysmyelinating process in CMT1 may lead to greater loss of strength compared to the axonal degeneration in CMT2, and that exercise may benefit both subtypes. Self-directed exercise may be a convenient, sustainable, and effective method of improving strength and decreasing disability in these individuals. Future research should explore the type of exercise prescription that best addresses the needs of the CMT population.

Background: Fatigue is frequent and disabling in adults with neuromuscular disorders, but not well characterized in paediatric neuromuscular disorders. Recently, fatigue was reported to be associated with poor health-related quality of life in children with Duchenne muscular dystrophy (DMD). Determinants of fatigue—a modifiable symptom—have not been studied in DMD. Our objective was to explore risk factors for fatigue in children with DMD. Methods: Patients aged 4–17 years identified via the Canadian Neuromuscular Disease Registry received mailed questionnaires. Fatigue was assessed using the PedsQLTM Multidimensional Fatigue Scale (patient- and parent-report). Standardized measures for depressive symptoms, sleep disturbances, functional ability and physical activity were used. Spearman’s correlations and Wilcoxon rank-sum tests were computed. Results: Of 194 eligible patients, 64 have responded to date. DMD patients reported greater fatigue than healthy controls from published data. Depressive symptoms were associated with greater fatigue, by patient-report (ρ=-0.44, P<0.001) and parent-report (ρ=-0.40, P=0.002). Sleep disturbances were associated with greater fatigue, by patient-report (ρ=-0.41, P=0.007) and parent-report (ρ=-0.51, P<0.001). Greater functional ability was associated with less fatigue, by parent-report (ρ=0.30, P=0.02). Physical activity and ambulatory status were not associated with fatigue. Conclusions: Fatigue is a significant issue in DMD. Depressive symptoms and sleep disturbances are associated with fatigue, warranting attention in therapeutic strategies to reduce fatigue.

Background: Mowat-Wilson Syndrome (MWS) is a genetic syndrome (ZEB2, OMIM: 235730) that occurs in 1 in 50000 births. It is characterized by microcephaly, intellectual disability, dysmorphisms (prominent chin, cupped ears, broad nasal bridge) and Hirschsprung’s disease. Although motor delay and hypotonia are common components, a myopathy has not been described in MWS literature. A childhood case with myopathic features prompted further study of this rare disease. Methods: Patients were recruited from the Mowat-Wilson Foundation via email or social media to complete a survey. Results: Thirteen surveys were returned to date. Although 54% of the patients reported motor delay, none of the patients had myopathy investigations. The index patient, presented at 1 year old, with hypotonia and developmental delay. Pregnancy and family history were unremarkable. Investigations revealed high CK levels (range 300 to 500 U/L), EMG confirmed myopathic motor units, and muscle biopsy showed type 1 fibre predominance. Single gene sequencing revealed pathogenic mutations of ZEB2, confirming a diagnosis of MWS. Conclusions: The description of myopathic features expands the spectrum of this rare syndrome and adds to the differential diagnosis of hyperCKemia in early childhood.

Background: Few studies have explored the effects of anti-epileptic drugs (AEDs) on electroencephalograph (EEG) findings during the assessment of seizure management. Although a patient may reach seizure freedom, EEG results may continue to be abnormal. Further information is required to understand the trend of EEG findings during seizure treatment. Methods: This is a retrospective study based on chart reviews. Patients who had epilepsy evaluations at the Royal University Hospital in Saskatoon between January 2012 and December 2015, were selected. The relationships among time of initiating AEDs, EEG findings, and seizure outcome on follow-ups, have been evaluated. Results: 151 patients had first seizure clinic assessments, in which 75 patients had an EEG before starting AEDs. Among the 75 patients, 54 (72%) had abnormal EEGs. From those, 38 (70.3%) patient’s EEGs became normal and 16 (29.7%) patients continued to have abnormal EEGs after the introduction of AEDs. The seizure freedom was 81.5% among those who had normal EEG on follow-up, and 43.7% of those who continued to have abnormal EEGs. Conclusions: Although patients with normal EEGs after starting AEDs may encounter a higher chance of seizure freedom, the seizure free patients with abnormal EEGs indicate that EEG is not completely sufficient in predicting seizure status.

Background: Burgeoning EEG demand has largely gone unmet with insufficient supply of manpower and equipment. Quantitative EEG (QEEG) may help compress large volumes of data for expedited review. We sought to determine the current use of QEEG in Canada through a national EEG technologist survey. Methods: A 10-item questionnaire was administered to participants at the 2016 meeting of the Canadian Association of Electroneurophysiology Technologists, which occurred in parallel with the Canadian Neurological Sciences Federation meeting. Results: A response rate of 63% (14/22) represented 12 institutions (11 adult, 6 paediatric) over six provinces with 73% of the national population. Only academic institutions (9/12) used QEEG, representing five provinces with 70% of the national population. Most institutions generated QEEG either real-time or retrospectively in the critical care and epilepsy monitoring units for long-term monitoring and automated seizure detection. The most used trends were spectrographic, seizure detection, and artifact detection. Montage use, QEEG duration, and timebase settings were highly variable. Conclusions: QEEG is in surprisingly frequent use across Canada. There is no consensus on optimal QEEG use, which mirrors uncertainty in the literature. The relative ubiquity of QEEG in Canada offers promise for collaborative multicentre research into unlocking the full potential of QEEG in enhancing patient care.

Background: Intermittent EEG attenuations have relatively clear significance in pediatric populations, but a consistent clinical correlation has not been identified in adults. While generally seen in metabolic encephalopathies, the specific clinical correlates and prognostic value have not been determined. Methods: We prospectively collected 22 consecutive EEGs noted to have intermittent generalized attenuations. Baseline and discharge modified Rankin Scale (mRS), diagnosis at discharge, EEG altering medications, ICU admissions, relevant imaging, mental status, the location the patient was discharged to, and pertinent lab values were assessed. Results: Mean patient age was 73.7 (SD=11.0) at admission. Twelve of the twenty-two patients (55%) died during their course in hospital. Four patients (18.2%) did not have a change in mRS score from baseline to discharge, while most had an increase in their mRS scores reflecting increased disability. Twelve patients (55%) were admitted to the ICU or CCU during their time in hospital. The most common etiologies were metabolic encephalopathies, and often associated with triphasic waves. Conclusions: Intermittent generalized EEG attenuations in adults are associated with severe metabolic encephalopathies and poor outcome including high association with mortality. The physiologic mechanism of generalized attenuations in poorly understood. Patients with this pattern should be suspected of having a severe metabolic encephalopathy and treated accordingly.

Background: Diaphragm pacing (DP) is an experimental ALS treatment, available through a compassionate use program. Eligibility requires forced vital capacity (FVC) between 45-50% predicted and phrenic nerve conduction study (NCS) evidence showing the diaphragm can be electrically stimulated. Diaphragm ultrasound (DU) also evaluates diaphragm function by demonstrating thickening with inspiration. Methods: A 63 year old man with advanced ALS requested DP as his respiratory functions worsened. He was wheelchair bound and had severe dysarthria and dysphagia. He had exertional dyspnea and used CPAP at night for obstructive apnea. Results: FVC was 47% predicted. Initial phrenic NCS showed a normal response on the right but no response on the left, making him ineligible for DP. Diaphragm function was further assessed with DU. This showed normal thickening with inspiration bilaterally. The DU result prompted repeating the right phrenic NCS which then showed a normal response. He successfully completed surgical implantation of diaphragm leads for DP. At surgery both diaphragms showed good responses to electrical stimulation. Conclusions: Phrenic NCS can be technically challenging and yield a false positive (absent) result. In this patient, DU indicated good diaphragm function, which prompted repeating phrenic NCS. The normal phrenic NCS allowed the patient to pursue DP.

Background: Metallic susceptibility artifact (MSA) on brain MRI has been described rarely in adult population as an incidental finding in patients undergone cardiac surgery, catheterization or prosthetic heart valve, but has not been described before in the pediatric population. Here we present two pediatric cases with MSA on brain MRI post-cardiac surgery. Case Series:Patient1: 13-month-old girl with Transposition of great arteries, aortic coarctation and multiple VSDs, who twice had open heart surgery requiring cardiac bypass. She presented with bilateral lower extremity paralysis secondary to spinal cord embolic infarction, Brain MRI showed an incidental finding of hundreds of diffuse foci of brain MSA. Patient2: 5-year-old boy with Trisomy21, Atrial-ventricular septal defect repaired at age one year. His brain MRI showed incidental finding of multiple, widespread foci of MSA during investigation for gait disturbance. Discussion: Here we present two pediatric patients post-cardiac surgery, found to have multiple scattered MSA in cerebral and cerebellar hemispheres and brain stem. Presumably, these are secondary to metallic microthrombi during cardiac surgery, however, neither had prosthetic material. Metallic microthrombi may be related to cardio-pulmonary bypass. Pediatric cardiac surgery patients should be studied, to understand the significance of these lesions and further distinguish the cause and association of these findings.

Background: Although surgery is the gold standard for treating brain arteriovenous malformations (AVMs), surgical techniques may not be suitable if the AVM is located in eloquent regions of the brain, such as the motor cortex. An alternative method for these cases is stereotactic radiosurgery. Localization of the motor cortex using functional magnetic resonance imaging (fMRI) is useful for helping the neurosurgeon determine which type of surgery is appropriate. We report a patient with a left frontal AVM near the motor cortex. fMRI was requested to localize motor functioning. Methods: The tasks included bilateral finger tapping, arm rubbing against the scanner, and abdominal tightening. All fMRI analyses were performed using BrainVoyager. Results: The fMRI results revealed that finger tapping and arm rubbing activated the precentral gyrus and supplementary motor area, and abdominal tightening activated the paracentral gyrus. These regions of activation were shown to be just posterior to the AVM and were mapped using neuronavigation during surgery. Conclusions: Given that the fMRI activation in the motor cortex was posterior to the AVM, the neurosurgeon felt confident that surgery could be performed. These findings elucidate the utility of fMRI for pre-surgical localization and for determining whether surgery or radiosurgery is appropriate in cases in which the AVM is near eloquent cortex.

Background: Functional magnetic resonance imaging (fMRI) and diffusion tensor imaging (DTI) are noninvasive and reliable tools for mapping eloquent cortex and white matter tracks prior to brain surgery. In this case, fMRI and DTI were used to inform the surgical approach in the resection of a deep cavernous malformation near the right lentiform nucleus. Post-surgery, the patient developed a fluid collection in the frontal cortex that applied pressure to M1, which led to reorganization of the motor cortex. Methods: The tasks included finger tapping, arm rubbing, and lip licking. All fMRI analyses were performed using BrainVoyager. Tensors were tracked from 20-direction diffusion MR images using DSIStudio. Results: An fMRI scan one-month pre-surgery revealed activation in M1 for the three tasks. A six-month follow-up scan revealed motor activation had been displaced by the fluid collection. A ten-month follow-up scan revealed that activation had shifted from its original location to more lateral and anterior regions. DTI revealed atrophy in the tracts through the insula, but increase in tracts through the lentiform nucleus. Conclusions: The results provide evidence that components of motor processing subserved by M1 can be taken over by adjacent regions, and that the rapid onset of pressure can lead to reorganization in a relatively short time period.

Background: Cerebral arteriovenous malformations (AVMs) are a type of vascular abnormality characterized by abnormal connections between arteries and veins without the normal interposed capillary bed. The gold standard for diagnosis is digital subtraction angiography (DSA). Functional MRI (fMRI), particularly with the increased sensitivity at ultra-high field (>=7T), may help to further characterize AVMs, but has not been performed in this population. Methods: We present a functional and structural neuroimaging analysis of an AVM at 7T. Resting-state fMRI was analyzed using independent components analysis (ICA) and compared to normal controls. Structural T1-weighted images were obtained at 1.5T and 7T. The patient also underwent DSA. Results: A 44 year-old, right handed man presented with a generalized tonic-clonic seizure. MRI at 1.5T and 7T revealed an AVM located in the pineal region measuring 3.2 cm. Multiple large feeder vessels were identified, and the AVM drained into the vein of Galen, clearly visualized on the 7T images. Functional imaging revealed an altered default mode network and ICA-identified vascular networks corresponding to the AVM. Conclusions: Imaging at 7T clearly delineates AVM structure. Functional connectivity is altered by the AVM. Vessel-specific independent components were identified that may be helpful for AVM characterization.

Background: Studies have placed the rate of infection associated with neuromodulation units to be up to 20%. We present our experience with the TYRX absorbable antibiotic envelope. Our length of follow-up adds to the body of evidence around the use of antibiotic envelops. Methods: We conducted a retrospective chart review of patients referred to our center for either new implantation or revision of neuromodulation units between July 2014 and September 2016. Consecutive cases were included for analysis. We included a control group of consecutive patients with neuromodulation units placed immediately prior to our experience with the TYRX envelopes for comparison Results: Between July 2014 and September 2016, 76 patients had 81 instances of neuromodulation unit insertion. All patients received the TYRX antibiotic envelope. There were no incidences of infection involving antibiotic envelope-containing implants over an average follow-up period of 11 months. In 77 consecutive cases of neuromodulation unit implantation prior to usage of the antibiotic pouch, there were 4 instances of infection (5.2%). Conclusions: Our single center experience demonstrates a significant drop in the rate of infections with the use of an antibiotic envelope for neuromodulation unit implantation. We consider the routine use of the envelope to be a cost-effective method of infection avoidance.