Choosing Wisely Canada (CWC) is a national branch of a global campaign advocating for fewer unnecessary tests and for optimizing patient care. Professional societies representing physicians, pharmacists, and nurses participate by generating lists of recommendations meant to reduce patient harm and resource mismanagement in healthcare. The Canadian Neurological Society (CNS) plays an important role in advocating for quality patient care demonstrated by deriving specific recommendations. This process is described.

The CNS Choosing Wisely task force adapted 10 recommendations for Canadian neurology practice. These were approved by the CNS board, and subsequently ranked by CNS members.

Ten recommendations were brought forward and ranked in a survey completed by CNS members. Survey ranking is presented. The top five recommendations were selected and optimized, resulting in seven key recommendations.

The recommendations set forth by the CNS will help with resource stewardship and patient safety in the delivery of neurological care by healthcare providers in Canada.

In Canada, standard dementia workup consists of clinical, neurological, and cognitive evaluation, as well as structural brain imaging. For atypical dementia presentations, additional FDG-PET brain imaging is recommended. Cerebrospinal fluid (CSF) biomarkers have recently been proposed as the gold standard for in vivo detection of Alzheimer’s disease (AD) pathophysiology (NIA-AA research framework, 2018). As clinical implementation of CSF assessment is still limited in Canada, the present study assessed its impact on diagnostic accuracy in atypical neurodegenerative disorders in the clinical practice.

This retrospective clinical chart review included patients with cognitive complaints who underwent lumbar puncture (LP) in addition to the standard diagnostic workup. CSF analysis determined the presence of biological AD based on reduced amyloid-β42-to-total-tau index (ATI) and increased phosphorylated-tau (p-tau) levels. CSF-based diagnoses were compared to standard workup and FDG-PET-based diagnoses.

A total of 28 patients with atypical dementia presentations were included in the present study after evaluation for cognitive complaints at a specialized dementia clinic between November 2017 and July 2019. CSF analysis changed or better specified the initial clinical diagnosis in 43.0% of cases (alternative diagnosis revealed in 25% and excluded in 18%). In patients with additional FDG-PET imaging (n = 23), FDG-PET and CSF-based diagnosis did not correspond in 35% of patients, even though FDG-PET appeared to increase diagnostic accuracy compared to the initial clinical diagnosis.

CSF biomarkers improved diagnostic accuracy in atypical cognitively-impaired patients beyond standard workup and FDG-PET imaging. These results support CSF analysis implementation for atypical dementias in Canada, in addition to the standard diagnostic workup.

To describe clinical characteristics of Canadian patients with myelin-oligodendrocyte glycoprotein immunoglobulin-G optic neuritis (MOG-IgG ON).

Retrospective observational case series of MOG-IgG seropositive patients with ON referred to tertiary neuro-ophthalmology practices. Outcome measures included clinical characteristics, radiologic findings, and visual outcomes.

Forty-six eyes of 30 patients were included. Twenty-three (76.7%) were women, mean onset age was 40.7 years (range 16–77), and most were Caucasian. Seventeen (56.7%) presented with their first ON episode. Sixteen (53.3%) had bilateral eye involvement. Isolated ON without associated neurological symptoms occurred in 90.0%. In 22 patients with acute ON (seen within 1 month of onset), presenting mean visual acuity (VA) was 20/258 (logMAR 1.11), mean deviation (MD) on Humphrey visual fields was −16.90 ± 10.83 dB, and peripapillary retinal nerve fiber layer (RNFL) thickness on ocular coherence tomography (OCT) was 164.23 ± 46.53 um. Orbital magnetic resonance imaging (MRI) within 1 month of symptom onset for 19 patients demonstrated orbital optic nerve enhancement in 11 (57.9%) and perineural enhancement in 11 (57.9%). Brain MRI was normal in 28 (93.3%) patients. Twenty out of 22 patients with acute presentation were treated with high-dose glucocorticoids and 5 with plasma exchange in addition to corticosteroids. Long-term immunosuppression was utilized in 9 (30%) out of all 30 patients. Final VA was 20/30 (logMAR 0.18), MD was −7.17 ± 8.85 dB, and RNFL thickness was 72.15 ± 20.16 um.

MOG-IgG ON in Canada has a variable presentation with most patients having substantial initial vision loss with good recovery. This is the largest characterization of the disease in Canada to date.

Tuberous sclerosis complex (TSC) is a rare genetic disorder that commonly leads to drug-resistant epilepsy in affected patients. This study aimed to determine whether the underlying genetic mutation (TSC1 vs. TSC2) predicts seizure outcomes following surgical treatments for epilepsy.

We retrospectively assessed TSC patients using the TSC Natural History Database core registry. Data review focused on outcomes in patients treated with surgical resection or vagus nerve stimulation.

A total of 42 patients with a TSC1 mutation, and 145 patients with a TSC2 mutation, were identified. We observed a distinct clinical phenotype: children with TSC2 mutations tended to be diagnosed with TSC at a younger age than those with a TSC1 mutation (p < 0.001), were more likely to have infantile spasms (p < 0.001), and to get to surgery at a later age (p = 0.003). Among this TSC2 cohort, seizure control following resective epilepsy surgery was achieved in less than half (47%) the study sample. In contrast, patients with TSC1 mutations tended to have more favorable postsurgical outcomes; seizure control was achieved in 66% of this group.

TSC2 mutations result in a more severe epilepsy phenotype that is also less responsive to resective surgery. It is important to consider this distinct clinical disposition when counseling families preoperatively with respect to seizure freedom. Larger samples are required to better characterize the independent effects of genetic mutation, infantile spasms, and duration of epilepsy as they relate to seizure control following resective or neuromodulatory epilepsy surgery.

Improvements in management of transient ischemic attack (TIA) have decreased stroke and mortality post-TIA. Studies examining trends over time on a provincial level are limited. We analyzed whether efforts to improve management have decreased the rate of stroke and mortality after TIA from 2003 to 2015 across an entire province.

Using administrative data from the Canadian Institute for Health Information’s (CIHI) databases from 2003 to 2015, we identified a cohort of patients with a diagnosis of TIA upon discharge from the emergency department (ED). We examined stroke rates at Day 1, 2, 7, 30, 90, 180, and 365 post-TIA and 1-year mortality rates and compared trends over time between 2003 and 2015.

From 2003 to 2015 in Ontario, there were 61,710 patients with an ED diagnosis of TIA. Linear regressions of stroke after the index TIA showed a significant decline between 2003 and 2015, decreasing by 25% at Day 180 and 32% at 1 year (p < 0.01). The 1-year stroke rate decreased from 6.0% in 2003 to 3.4% in 2015. Early (within 48 h) stroke after TIA continued to represent approximately half of the 1-year event rates. The 1-year mortality rate after ED discharge following a TIA decreased from 1.3% in 2003 to 0.3% in 2015 (p < 0.001).

At a province-wide level, 1-year rates of stroke and mortality after TIA have declined significantly between 2003 and 2015, suggesting that efforts to improve management may have contributed toward the decline in long-term risk of stroke and mortality. Continued efforts are needed to further reduce the immediate risk of stroke following a TIA.

A Canadian Stroke Best Practices consensus statement on Acute Stroke Management during pregnancy was published in 2018. The state of individual practice, however, is unknown.

A survey on treatment of acute stroke in pregnant and post-partum women was distributed via the Canadian Stroke Consortium email list. Descriptive statistics (frequencies and proportions) were calculated for demographic and response variables and free-text responses were coded for thematic content.

Thirty-five participants completed the survey; 12 had experience with intravenous tissue plasminogen activator (IV-tPA), endovascular therapy (EVT), or both in pregnant patients. None had treatment-related complications. The majority (92%) of those who had not yet encountered the issue in practice expressed some reservation about giving IV-tPA to an otherwise eligible pregnant woman. In a theoretical scenario where an otherwise eligible pregnant woman was a candidate for both IV-tPA and EVT, 58% of respondents would have opted for EVT alone. Amongst this cohort comprised mainly of stroke sub-specialists, more than a third had treated pregnant patients with reperfusion therapy.

The reported safety experience with both IV-tPA and EVT was reassuring. Overall, there was a hesitancy towards use of IV-tPA in pregnancy that is discordant with the recent consensus statement. Possible barriers to uptake identified through thematic analysis were concerns regarding risks of bleeding in the pregnant patient, presence of EVT as a perceived alternative, and the need for express consent from the patient and family.

The study was conducted to test the hypothesis that nitroglycerin (NTG) increases cerebral perfusion focally and globally in acute ischemic stroke patients, using serial perfusion-weighted imaging (PWI) magnetic resonance imaging measurements.

Thirty-five patients underwent PWI immediately before and 72 h after administration of a transdermal NTG patch or no treatment. Patients with baseline mean arterial pressure (MAP) > 100 mmHg (NTG group, n = 20) were treated with transdermal NTG (0.2 mg/h) for 72 h, without a nitrate-free interval. Patients with MAP ≤ 100 mmHg (untreated group, n = 15) were not treated. The primary outcome measure was absolute cerebral blood flow (CBF) in the hypoperfused region at 72 h.

The mean baseline absolute CBF in the hypoperfused region was similar in the NTG group (33.3 ± 10.2 ml/100 g/min) and untreated (32.7 ± 8.4 ml/100 g/min, p = 0.4) groups. The median (IQR) baseline infarct volume was 10.4 (2.5–49.3) ml in the NTG group and 32.6 (8.6–96.7) ml in the untreated group (p = 0.09). MAP change in the NTG group was 1.2 ± 12.6 and 8 ± 20.7 mmHg at 2 h and 72 h, respectively. Mean absolute CBF in the hypoperfused region at 72 h was similar in the NTG (29.9 ± 12 ml/100 g/min) and untreated groups (24.1 ± 10 ml/100 g/min, p = 0.8). The median infarct volume increased in untreated (11.8 (5.7–44.2) ml) than the NTG group (3.2 (0.5–16.5) ml; p = 0.033) on univariate analysis, however, there was no difference on regression analysis.

NTG was not associated with improvement in cerebral perfusion in acute ischemic stroke patients.

In young patients, the cause of ischemic stroke (IS) remains often cryptogenic despite presence of traditional vascular risk factors (VRFs). Since arterial hypertension (AH) is considered the most important one, we aimed to evaluate the impact of AH and blood pressure (BP) levels after discharge on risk of recurrent IS (RIS) in young patients.

The study set consisted of acute IS patients < 50 years of age enrolled in the prospective Heart and Ischemic STrOke Relationship studY registered on ClinicalTrials.gov (NCT01541163). Cause of IS was assessed according to the ASCOD classification.

Out of 319 enrolled patients <50 years of age (179 males, mean age 41.1 ± 7.8 years), AH was present in 120 (37.6%) of them. No difference was found in the rates of etiological subtypes of IS between patients with and without AH. Patients with AH were older, had more VRF, used more frequently antiplatelets prior IS, and had more RIS (10 vs. 1%, p = 0.002) during a follow-up (FUP) with median of 25 months. Multivariate logistic regression stepwise model showed the prior use of antiplatelets as only predictor of RIS (p = 0.011, OR: 6.125; 95% CI: 1.510–24.837). Patients with elevated BP levels on BP Holter 1 month after discharge did not have increased rate of RIS during FUP (3.8 vs. 1.7%, p = 1.000).

AH occurred in 37.6% of young IS patients. Patients with AH had more frequently RIS. Prior use of antiplatelets was found only predictor of RIS in young IS patients with AH.

Venolymphatic malformations are rare benign vascular lesions of the head and neck. Sclerotherapy has become the first-line therapy of these lesions with bleomycin being a sclerosing agent commonly used.

To perform a systematic review of the published literature to synthesize evidence on the safety and efficacy of bleomycin for the treatment of head and neck venolymphatic malformations.

A systematic review of the literature (January 1995–May 2019) was performed in PubMed, Embase, and Cochrane Library databases to identify studies on sclerotherapy of venolymphatic malformations of the head and neck.

A total of 32 studies with participants met the inclusion criteria among which 1121 patients were included in the systematic review.

Two reviewers independently screened and extracted data and assessed the risk of bias. The primary outcome was the subjective or objective reduction of lesion size as well as minor and major complications.

The bleomycin/pingyangmycin sclerotherapy achieved subjective or objective lesion size reduction in 96.3% (95% CI 94.1%–98.5%) of patients. Minor complications were observed in 16.2% and major complications in 1.1%.

Bleomycin is a highly effective treatment of venolymphatic malformations of the head and neck with a low rate of major adverse events. This study represents an update on the “available” evidence, but only low-to-moderate quality studies were available.

This study reviewed 32 studies performed in different parts of the world, but there was heterogeneity of the study designs and interventions.

To compare long-term survival of Parkinson’s disease (PD) patients with deep brain stimulation (DBS) to matched controls, and examine whether DBS was associated with differences in injurious falls, long-term care, and home care.

Using administrative health data (Ontario, Canada), we examined DBS outcomes within a cohort of individuals diagnosed with PD between 1997 and 2012. Patients receiving DBS were matched with non-DBS controls by age, sex, PD diagnosis date, time with PD, and a propensity score. Survival between groups was compared using the log-rank test and marginal Cox proportional hazards regression. Cumulative incidence function curves and marginal subdistribution hazard models were used to assess effects of DBS on falls, long-term care admission, and home care use, with death as a competing risk.

There were 260 DBS recipients matched with 551 controls. Patients undergoing DBS did not experience a significant survival advantage compared to controls (log-rank test p = 0.50; HR: 0.89, 95% CI: 0.65–1.22). Among patients <65 years of age, DBS recipients had a significantly reduced risk of death (HR: 0.49, 95% CI: 0.28–0.84). Patients receiving DBS were more likely than controls to receive care for falls (HR: 1.56, 95% CI: 1.19–2.05) and home care (HR: 1.59, 95% CI: 1.32–1.90), while long-term care admission was similar between groups.

Receiving DBS may increase survival for younger PD patients who undergo DBS. Future studies should examine whether survival benefits may be attributed to effects on PD or the absence of comorbidities that influence mortality.

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.

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.

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.

To describe complementary and alternative medicine (CAM) use amongst children with cerebral palsy (CP) in Canada and to identify factors associated with CAM use.

We conducted a cross-sectional study, utilising data from the Canadian CP Registry. We explored the association between CAM use and regional, socioeconomic and CP phenotypic variables, and parental perception of the family-centredness of clinical care using the Measures of Process of Care-56 (MPOC-56). Chi-square analyses were performed, and odds ratios (OR) and 95% confidence intervals (CI) were obtained. Mann–Whitney U tests were used to compare MPOC-56 scores between CAM users and non-CAM users.

The study sample consisted of 313 families of which 27% reported CAM use in the past year. Children with CP using CAM were more likely to reside in Western Canada (OR 3.3, 95% CI 1.6–6.7), live in a two-parent household (OR 3.5, 95% CI 1.5–8.4), have an ataxic/hypotonic or dyskinetic CP subtype (OR 3.0, 95% CI 1.5–6.1) and have a greater motor impairment (OR 2.8, 95% CI 1.7–4.9). MPOC-56 subscale scores were not significantly associated with CAM use.

Physicians need to be aware of existing CAM therapies, the level of evidence supporting their efficacy (beneficence), their associated risks of adverse events (non-maleficence) and enable fair access to care that may be of benefit to each child.