Assessment of individuals at risk for falling entails comprehensive neurological and vestibular examinations. Chronic limitation in cervical mobility reduces gaze accuracy, potentially impairing navigation through complex visual environments. Additionally, humans with scoliosis have altered otolithic vestibular responses, causing imbalance. We sought to determine whether dynamic cervical mobility restrictions or static cervicothoracic impairments are also fall risk factors.

We examined 435 patients referred for soft-tissue musculoskeletal complaints; 376 met criteria for inclusion (mean age 52; 266 women). Patients were divided into nonfallers, single fallers, and multiple fallers, less or greater than 65 years old. Subject characteristics, dynamic cervical rotations, and static cervicothoracic axial measurements were compared between groups. Fear of falling was evaluated using the Falls Efficacy Scale-International questionnaire.

Long-standing cervicothoracic pain and stiffness conferred increased risk of falling. Neck rotation amplitudes decreased with longer duration musculoskeletal symptoms and were significantly more restricted in fallers, doubling the risk of falling and contributing to increased fear of falling. Mid-thoracic scoliosis amplitudes increased over time, but static axial abnormalities were not greater among fallers, although thoracic kyphoscoliosis heightened fear of falling.

In patients at fall risk, thoracic kyphoscoliosis and dynamic neck movements should be assessed, in addition to standard vestibular and neurological evaluations. Additionally, patients with soft tissue cervicothoracic pain and restricted mobility have increased fall frequency and fear of falling, independent of other fall risk factors and should undergo complete fall risk appraisal.

Pituitary adenomas (PAs) are common and often require complex multidisciplinary care with multiple specialists. This may result in a healthcare system that is challenging for patients to navigate. Audits of care at our institution revealed opportunities for improvement to better align care with patients’ needs.

A quality improvement initiative that incorporated a patient advisory committee of patients who had received treatment for PA at our center and their family members were used to help identify opportunities for improvement. The patient-identified gaps in care included the need to coordinate and minimize appointments and the desire for better communication and education. Based on this information, changes were implemented to the pituitary program, including increasing access to the Multidisciplinary Clinic and developing a standardized and centralized triage process.

A pre- and post-intervention analysis consisting of retrospective chart reviews revealed that these changes had an impact on wait times for first assessment, and a significant shift in the location of this first visit – with a larger proportion of patients being seen in the Multidisciplinary Clinic after an intervention.

We demonstrate that patient involvement, beyond individual patient–physician interactions, can lead to meaningful and observable changes, and can improve the quality of care for PA.

Retrospective observational study to determine diagnostic yield and utility of genetic testing in children with epilepsy attending the Epilepsy Clinic at Children’s Hospital, London, Ontario, Canada.

Children (birth–18 years) with epilepsy, who were seen in a 10-year period (January 1, 2008–March 31, 2018), were selected using defined inclusion criteria and by combining clinic datasets and laboratory records.

In total, 105 children (52.38% male and 47.61% female) with a variety of seizures were included in the analysis. Developmental delay was documented in the majority (83; 79.04%). Overall, a genetic diagnosis was established in 24 (22.85%) children. The diagnostic yield was highest for whole-exome sequencing (WES), at 35.71%. The yield from microarray was 8.33%. Yields of single-gene testing (18.60%) and targeted multigene panel testing (19.23%) were very similar. Several likely pathogenic and pathogenic variants not previously reported were identified and categorized using ACMG criteria. All diagnosed patients underwent a review of anti-seizure medication management and received counseling on natural history of their disease, possible complications, recurrence risks, and possibilities of preimplantation or prenatal genetic diagnosis.

Our study confirms the multiple benefits of detecting a genetic etiology in children with epilepsy. Similar yields in single versus multigene testing underscore the importance of accurate clinical phenotyping. Patients with epilepsy and their caregivers in Ontario would undoubtedly benefit from repatriation of multigene panels and WES to the province.

Symptoms of cervical dystonia (CD) can vary in severity and cause significant pain. OnabotulinumtoxinA is an approved treatment for CD. This study assessed health-related quality of life (HRQoL) in patients with CD who received multiple onabotulinumtoxinA treatments.

This prospective, observational standard-of-care study was conducted at multiple neurology centers in Québec, Canada. Patients reported the health impact of CD using the Cervical Dystonia Impact Profile (CDIP)-58, before and after up to eight onabotulinumtoxinA treatments. Other measures included the Cervical Dystonia Severity Rating Scale by physician, employment status using the Work Productivity Questionnaire and pain using the Pain Numeric Rating Scale (PNRS). Adverse events (AEs) were recorded.

Sixty-two patients were enrolled (safety population, n = 61; modified efficacy population, n = 58). Participants were mostly females who were employed; most (79.3%) had torticollis. In all, 21/62 patients (33.9%) discontinued the study. At the final visit, there was a statistically significant (p < 0.001) improvement in all eight CDIP-58 subscales, particularly head and neck symptoms (−31.0) and psychosocial functioning (−28.2). Employment increased from baseline (55%) to the end of the study (64%), and there was improvement in work productivity. There was a significant (p < 0.0001) reduction in pain measured by the PNRS, from −0.5 post-treatment 1 to −2.4 at end of study. AEs (neck pain, muscular weakness, dysphagia, nausea) were consistent with onabotulinumtoxinA use.

These real-world data indicate that after repeated, long-term use, onabotulinumtoxinA continues to be a safe and effective treatment for CD, improving HRQoL and work productivity.

We investigated the impact of regionally imposed social and healthcare restrictions due to coronavirus disease 2019 (COVID-19) to the time metrics in the management of acute ischemic stroke patients admitted at the regional stroke referral site for Central South Ontario, Canada.

We compared relevant time metrics between patients with acute ischemic stroke receiving intravenous tissue plasminogen activator (tPA) and/or endovascular thrombectomy (EVT) before and after the declared restrictions and state of emergency imposed in our region (March 17, 2020).

We identified a significant increase in the median door-to-CT times for patients receiving intravenous tPA (19 min, interquartile range (IQR): 14–27 min vs. 13 min, IQR: 9–17 min, p = 0.008) and/or EVT (20 min, IQR: 15–33 min vs. 11 min, IQR: 5–20 min, p = 0.035) after the start of social and healthcare restrictions in our region compared to the previous 12 months. For patients receiving intravenous tPA treatment, we also found a significant increase (p = 0.005) in the median door-to-needle time (61 min, IQR: 46–72 min vs. 37 min, IQR: 30–50 min). No delays in the time from symptom onset to hospital presentation were uncovered for patients receiving tPA and/or endovascular reperfusion treatments in the first 1.5 months after the establishment of regional and institutional restrictions due to the COVID-19 pandemic.

We detected an increase in our institutional time to treatment metrics for acute ischemic stroke patients receiving tPA and/or endovascular reperfusion therapies, related to delays from hospital presentation to the acquisition of cranial CT imaging for both tPA- and EVT-treated patients, and an added delay to treatment with tPA.

Glucose Transporter-1 (GLUT1) Deficiency Syndrome (GLUT1DS) is caused by defective transport of glucose across the blood–brain barrier into brain cells resulting in hypoglycorrhachia due to the heterozygous pathogenic variants in SLC2A1. We report on the phenotypic spectrum of patients with pediatric GLUT1DS as well as their diagnostic methods from a single center in Canada.

We reviewed patient charts for clinical features, biochemical and molecular genetic investigations, neuroimaging, treatment modalities, and outcomes of patients with GLUT1DS at our institution.

There were 13 patients. The most common initial symptom was seizures, with the most common seizure type being absence seizures (85%). Seventy-seven percent of the patients had movement disorders, and dystonia and ataxia were the most common movement disorders. Fifty-four percent of the patients did not have a history of developmental delay during their initial presentation, whereas all patients had developmental delay, intellectual disability, or cognitive dysfunction during their follow-up. All patients had a pathogenic or likely pathogenic variant in SLC2A1 and missense variants were the most common variant type.

We present 13 patients with GLUT1DS in the pediatric patient population. Atypical clinical features such as hemiplegia and hemiplegic migraine were present in an infant; there was a high prevalence of absence seizures and movement disorders in our patient population. We report an increased number of patients with GLUT1DS since the introduction of next-generation sequencing in the clinical settings. We believe that GLUT1DS should be included in the differential diagnosis of seizures, movement disorders, and hemiplegic migraine.

This presentation will enable the learner to:

1. 1. Discuss cellular proliferation in the pathogenesis of the DRG lesion in Friedreich ataxia

AHK is a consultant to PTC Therapeutics of South Plainfield, NJ USA. SP and CS are majority owners of Kinexus.

Falls are a growing concern in seniors (≥65 yrs). Cognitive impairment (CI) and vestibular impairment (VI) increase fall risk. The aim of this study is to assess the prevalence of CI and VI in seniors experiencing falls.

Participants (≥65 yrs) with falls were recruited from Falls Prevention Programs (FPPs) and a Memory Clinic (MC). CI was assessed using the Montreal Cognitive Assessment at FPPs. VI was assessed at an MC and FFPs using the Head Impulse- (video + bedside), Headshake-, Dix-Hallpike test, and test of sensory interaction in balance. Questionnaires included Dizziness Handicap Inventory (DHI) and Activities-specific Balance Confidence Scale (ABC).

Of 41 participants (29 FPPs, 12 MC); mean age was 80.1 ± 7.1 years, and 58.5% were female. Overall, 82.9% had VI. At FPPs, 76.0% had CI, and 72.3% had CI + VI. Bilateral vestibular hypofunction (BVH) was more common than unilateral vestibular hypofunction (UVH) (70.6% vs. 29.4%); p = 0.016. Dizziness Handicap (DHI) was not different between those with a VI (23.5 ± 23.9) versus without VI [PVI + no impairment] (10.0 ± 15.4); p = 0.160. Balance confidence (ABC) was lowest in VI but not significantly different between those with a VI (63.4 ± 27.3) versus without VI [PVI + no impairment] (85.0 ± 16.5); p = 0.053.

VI and CI are prevalent in seniors experiencing falls. For seniors with history of falls, both cognitive and vestibular functions should be considered in the assessment and subsequent treatment. Screening enables earlier detection, targeted interventions, and prevention, reducing the clinical and financial impact.

Growing evidence showed that coronavirus disease 2019 (COVID-19) infection may present with neurological manifestations. This review aimed to determine the neurological manifestations and complications in COVID-19.

We conducted a systematic review and meta-analysis that included cohort and case series/reports involving a population of patients confirmed with COVID-19 infection and their neurologic manifestations. We searched the following electronic databases until April 18, 2020: PubMed, Embase, Scopus, and World Health Organization database (PROSPERO registration number: CRD42020180658).

From 403 articles identified, 49 studies involving a total of 6,335 confirmed COVID-19 cases were included. The random-effects modeling analysis for each neurological symptom showed the following proportional point estimates with 95% confidence intervals: “headache” (0.12; 0.10–0.14; I2 = 77%), “dizziness” (0.08; 0.05–0.12; I2 = 82%), “headache and dizziness” (0.09; 0.06–0.13; I2 = 0%), “nausea” (0.07; 0.04–0.11; I2 = 79%), “vomiting” (0.05; 0.03–0.08; I2 = 74%), “nausea and vomiting” (0.06; 0.03–0.11; I2 = 83%), “confusion” (0.05; 0.02–0.14; I2 = 86%), and “myalgia” (0.21; 0.18–0.25; I2 = 85%). The most common neurological complication associated with COVID-19 infection was vascular disorders (n = 23); other associated conditions were encephalopathy (n = 3), encephalitis (n = 1), oculomotor nerve palsy (n = 1), isolated sudden-onset anosmia (n = 1), Guillain–Barré syndrome (n = 1), and Miller–Fisher syndrome (n = 2). Most patients with neurological complications survived (n = 14); a considerable number of patients died (n = 7); and the rest had unclear outcomes (n = 12).

This review revealed that neurologic involvement may manifest in COVID-19 infection. What has initially been thought of as a primarily respiratory illness has evolved into a wide-ranging multi-organ disease.

In Ontario, there are approximately 66,000 adults living with a diagnosis of intellectual and developmental disabilities (IDD). These individuals are nearly twice as likely to experience an injury compared to the general population. Falls are an important contributor to injuries in persons with IDD and in the general population, and are consistently found to be the leading cause of traumatic brain injury (TBI). There is currently no literature that quantitatively examines TBI among persons with IDD. The purpose of this study was to compare the risk of TBI for adults with and without IDD in Ontario over time and by demographic information.

Using administrative health databases, two main cohorts were identified: (1) adults with IDD, and (2) a random 10% sample of adults without IDD. Within each cohort, annual crude and adjusted incidence of TBI were calculated among unique individuals for each fiscal year from April 1, 2002 to March 31, 2017.

Over the 15-year study period, the average annual adjusted incidence of TBI was approximately 2.8 new cases per 1000 among Ontario adults with IDD, compared to approximately 1.53 per 1000 among those without IDD. In both cohorts, a higher proportion of TBI cases were younger (19–29 years) and male.

During the study period, persons with IDD experienced a significantly higher risk of TBI compared to the general population indicating the possibility, and need, for targeted TBI prevention.

Contemporary data on temporal trends in acute stroke incidence, specific to stroke type and age, are lacking. We sought to evaluate temporal trends in incidence of ischemic stroke and intracerebral hemorrhage over 15 years in a large population.

We used linked administrative data to identify all emergency department visits and hospital admissions for first-ever ischemic stroke or intracerebral hemorrhage in Ontario, Canada from 2003–2017. We evaluated annual age-/sex-standardized incidence per 100,000 person-years for ischemic stroke and intracerebral hemorrhage across the study period. We used negative binomial regression to determine incidence rate ratios for each year compared to 2003, with assessment of modification by age, sex, or stroke type.

Our cohort had 163,574 people with stroke (88% ischemic stroke). For ischemic stroke and intracerebral hemorrhage combined, age-/sex-standardized incidence decreased between 2003 and 2011 (standardized rate 109.4 to 85.8 per 100,000; 22%), then increased until 2017 (standardized rate 96.8 per 100,000; 13%). The pattern of change was similar for ischemic stroke and intracerebral hemorrhage, and for men and women, but was modified by age. For those aged 60 and above, adjusted incidence rate ratios decreased from 2003 to 2011 then subsequently increased, whereas for those aged <60 years incidence rate ratios increased throughout the entire study time period, particularly after 2011.

Acute stroke incidence decreased from 2003 to 2011 but subsequently increased until 2017. Among those aged <60, incidence increased continuously from 2003 to 2017 but especially after 2011. The underlying reasons for these changes should be determined.

Childhood acute arterial ischemic stroke (AIS) is diagnosed at a median of 23 hours post-symptom onset, delaying treatment. Pediatric stroke pathways can expedite diagnosis. Our goal was to understand the similarities and differences between Canadian pediatric stroke protocols with the aim of optimizing AIS management.

We contacted neurologists at all 16 Canadian pediatric hospitals regarding AIS management. Established protocols were analyzed for similarities and differences in eight domains.

Response rate was 100%. Seven (44%) centers have an established AIS protocol and two (13%) have a protocol under development. Seven centers do not have a protocol; two redirect patients to adult neurology, five rely on a case-by-case approach for management. Analysis of the seven protocols revealed differences in: 1) IV-tPA dosage: age-dependent 0.75–0.9 mg/kg (N = 1) versus age-independent 0.9 mg/kg (N = 6), with maximum doses of 75 mg (N = 1) or 90 mg (N = 6); 2) IV-tPA lower age cut-off: 2 years (N = 5) versus 3 or 10 years (each N = 1); 3) IV-tPA exclusion criteria: PedNIHSS score <4 (N = 3), <5 (N = 1), <6 (N = 3); 4) first choice of pre-treatment neuroimaging: computed tomography (CT) (N = 3), magnetic resonance imaging (MRI) (N = 2) or either (N = 2); 5) intra-arterial tPA use (N = 3) and; 6) mechanical thrombectomy timeframe: <6 hour (N = 3), <24 hour (N = 2), unspecified (N = 2).

Although 44% of Canadian pediatric hospitals have established AIS management pathways, several differences remain among centers. Some criteria (dosage, imaging) reflect adult AIS literature. Canadian expert consensus regarding IV-tPA and endovascular treatment should be established to standardize and implement AIS protocols across Canada.

Idiopathic normal pressure hydrocephalus (iNPH) is characterized by the clinical triad of gait disturbance, urinary incontinence, and memory impairment with normal cerebrospinal fluid (CSF) pressure. Transcranial magnetic stimulation (TMS) has been used to assess the corticospinal motor pathways in patients with iNPH with conflicting results.

Our study included 11 patients with iNPH and 13 healthy controls. All the subjects underwent TMS and resting motor threshold (RMT), central motor conduction time (CMCT), short-interval intracortical inhibition (SICI), intracortical facilitation, and silent period (SP) were recorded in the upper limb. Besides, RMT and CMCT in lower limb were also recorded. Cognitive assessments were done using mini-mental status examination, Montreal cognitive assessment (MoCA), and Addenbrooke’s cognitive evaluation III (ACE III). Same parameters were recorded 24 h of CSF (lumbar puncture, LP) drainage.

Mean age of the iNPH patients was 69.00 ± 6.71 years with age at onset being 66.64 ± 7.10 years. Duration of disease was 1.80 ± 1.25 years. A significant difference was noted in CMCT for the lower limb (CMCT-LL), SICI, and ipsilateral SP between pre-LP NPH and controls. Also, there was a significant difference in MoCA and ACE III between pre-LP NPH and controls. A significant reduction was observed in lower limb RMT between pre- and post-LP NPH patients. Post LP, there was a reduction in the lower limb CMCT and improvement in SICI.

A significant prolongation of CMCT-LL was observed in NPH patients. Lumbar CSF drainage in them resulted in a significant reduction in lower limb RMT thereby suggesting an increase in cortical excitability.

To compare hospitalizations among children with cerebral palsy (CP) and healthy controls and to identify factors associated with hospitalizations in children with CP.

This retrospective cohort study linked data from a provincial CP Registry and administrative health databases. The CP cohort was comprised of children born from 1999 to 2002. Age, sex, and region-matched controls were identified from administrative health databases. Mean differences, relative risk (RR), and 95% confidence intervals (CIs) were calculated.

A total of 301 children with CP were linked to administrative health data and matched to 6040 controls. Mean hospitalizations per child during the study period were higher in children with CP compared to controls (raw mean difference (RMD) 5.0 95% CI 4.7 to 5.2) with longer length of stay (RMD 2.8 95% CI 1.8 to 3.8) and number of diagnoses per hospitalization (RMD 1.6 95% CI 1.4 to 1.8). Increased risk of hospitalization was observed in non-ambulant children with CP (RR 1.12 95% CI 1.01 to 1.22) compared to ambulant children and among those with spastic tri/quadriplegic CP compared to other CP subtypes (RR 1.15, 95% CI 1.05 to 1.27). Feeding difficulties (RR 1.20 95% CI 1.13 to 1.27), cortical visual (RR 1.22 95% CI 1.13 to 1.32), cognitive (RR 1.16 95% CI 1.04 to 1.30), and communication impairment (RR 1.26 95% CI 1.10 to 1.44) were associated with increased hospitalizations.

Children with CP face more frequent, longer hospital stays than peers, especially those with a more severe CP profile. Coordinated interdisciplinary care is needed in school-aged children with CP and medical complexity.

Decisions to treat large-vessel occlusion with endovascular therapy (EVT) or intravenous alteplase depend on how physicians weigh benefits against risks when considering patients’ comorbidities. We explored EVT/alteplase decision-making by stroke experts in the setting of comorbidity/disability.

In an international multi-disciplinary survey, experts chose treatment approaches under current resources and under assumed ideal conditions for 10 of 22 randomly assigned case scenarios. Five included comorbidities (cancer, cardiac/respiratory/renal disease, mild cognitive impairment [MCI], physical dependence). We examined scenario/respondent characteristics associated with EVT/alteplase decisions using multivariable logistic regressions.

Among 607 physicians (38 countries), EVT was chosen less often in comorbidity-related scenarios (79.6% under current resources, 82.7% assuming ideal conditions) versus six “level-1A” scenarios for which EVT/alteplase was clearly indicated by current guidelines (91.1% and 95.1%, respectively, odds ratio [OR] [current resources]: 0.38, 95% confidence interval 0.31–0.47). However, EVT was chosen more often in comorbidity-related scenarios compared to all other 17 scenarios (79.6% versus 74.4% under current resources, OR: 1.34, 1.17–1.54). Responses favoring alteplase for comorbidity-related scenarios (e.g. 75.0% under current resources) were comparable to level-1A scenarios (72.2%) and higher than all others (60.4%). No comorbidity independently diminished EVT odds when considering all scenarios. MCI and dependence carried higher alteplase odds; cancer and cardiac/respiratory/renal disease had lower odds. Being older/female carried lower EVT odds. Relevant respondent characteristics included performing more EVT cases/year (higher EVT-, lower alteplase odds), practicing in East Asia (higher EVT odds), and in interventional neuroradiology (lower alteplase odds vs neurology).

Moderate-to-severe comorbidities did not consistently deter experts from EVT, suggesting equipoise about withholding EVT based on comorbidities. However, alteplase was often foregone when respondents chose EVT. Differences in decision-making by patient age/sex merit further study.

This presentation will enable the learner to:

1. 1. Understand the current spectrum of practice of Neuropathologists across Canada

2. 2. Describe the patterns of employment and anticipated retirements of Canadian Neuropathologists