Abnormal fetal corticogenesis results in malformations of cortical development (MCD). Abnormal cell proliferation leads to microcephaly or megalencephaly, incomplete neuronal migration results in heterotopia and lissencephaly, neuronal overmigration manifests as cobblestone malformations, and anomalous postmigrational cortical organization is responsible for polymicrogyria and focal cortical dysplasias. MCD comprises various congenital brain disorders, caused by different genetic, infectious, or vascular etiologies and is associated with significant neurological morbidity. Although MCD are rarely diagnosed prenatally, both dedicated multiplanar neurosonography and magnetic resonance imaging enable good demonstration of fetal cortical development. The imaging signs of fetal MCD are: delayed or absent cerebral sulcation; premature abnormal sulci; thin and irregular hemispheric parenchyma; wide abnormal overdeveloped gyri; wide opening of isolated sulci; nodular bulging into the lateral ventricles; cortical clefts; intraparenchymal echogenic nodules; and cortical thickening. The postnatal and prenatal imaging features of four main malformations of cortical development—lissencephaly, cobblestone malformations, periventricular nodular heterotopia, and polymicrogyria—are described.

The knowledge to action (KTA) process proposed by Graham et al (2006) is a framework to facilitate the development and application of research evidence into clinical practice. The KTA process consists of the knowledge creation cycle and the action cycle. The Evidence Based Review of Stroke Rehabilitation is a foundational part of the knowledge creation cycle and has helped guide the development of best practice recommendations in stroke. The Rehabilitation Knowledge to Action Project is an audit-feedback process for the clinical implementation of best practice guidelines, which follows the action cycle. The objective of this review was to: (1) contextualize the Evidence Based Review of Stroke Rehabilitation and Rehabilitation Knowledge to Action Project within the KTA model and (2) show how this process led to improved evidence-based practice in stroke rehabilitation. Through this process, a single centre was able to change clinical practice and promote a culture that supports the use of evidence-based practices in stroke rehabilitation.

In this review, we have gathered all the available evidence to guide medication management after deep brain stimulation (DBS) in Parkinson’s disease (PD). Surprisingly, we found that almost no study addressed drug-based management in the postoperative period. Dopaminergic medications are usually reduced, but whether the levodopa or dopamine agonist is to be reduced is left to the personal preference of the treating physician. We have summarized the pros and cons of both approaches. No study on the management of cognitive problems after DBS has been done, and only a few studies have explored the pharmacological management of such DBS-resistant symptoms as voice (amantadine), balance (donepezil) or gait disorders (amantadine, methylphenidate). As for the psychiatric problems so frequently reported in PD patients, researchers have directed their attention to the complex interplay between stimulation and reduction of dopaminergic drugs only recently. In conclusion, studies addressing medical management following DBS are still needed and will certainly contribute to the ultimate success of DBS procedures.

Lambert–Eaton syndrome (LES) is a rare immune-mediated disorder characterized by proximal leg weakness, autonomic symptoms and hypoactive tendon reflexes. The paraneoplastic form is associated with small-cell lung cancer in 50-60% of cases, whereas the remaining cases are found in younger adults with a higher likelihood of coexisting autoimmune disease. The early recognition of LES is crucial for improving clinical outcomes but remains a major challenge. In this review, we analyze the clinical characteristics and diagnostic considerations in treating LES through a series of three case studies, one of which showed definitive response to pyridostigmine and corticosteroid combination therapy, followed by spontaneous remission. Patients were assessed by image-based screening, serological testing and electrophysiological evaluations, which included respiratory and autonomic testing. A better understanding of the common pitfalls in the clinical, serological and neurophysiologic diagnosis of LES through assessment of typical LES dysfunction throughout the nervous system should enable improved recognition and treatment of this syndrome.

Background: For optimal stroke prevention, best practices guidelines recommend carotid endarterectomy (CEA) for symptomatic patients within two weeks; however, 2013 Ontario data indicated that only 9% of eligible patients from outpatient Stroke Prevention Clinics (SPCs) achieved this target. The goal of our study was to identify modifiable system factors that could enhance the quality and timeliness of care among patients needing urgent CEA. Methods: We conducted a retrospective chart review of transient ischemic attack/stroke patients assessed in Champlain Local Health Integrated Network SPCs between 2011 and 2014 who subsequently underwent CEA. Descriptive statistics were used to define patient characteristics, timelines from symptom onset to CEA, and system factors that contributed to delays or improvements in care. Multivariate analysis was used to determine statistically significant variations between groups. Results: Seventy-five records were eligible for study inclusion. Median time from initial symptoms to CEA was 31 days, with 21.3% of patients undergoing surgery within 2 weeks. Significant delays were common in patient presentation and assessment following symptom onset, wait times for vascular imaging and neurological assessment, and time from surgical assessment to CEA completion. Rapid testing and triage, coupled with collaborative initiatives among SPC, surgical, and radiology teams were associated with significantly improved timelines. Conclusions: Success factors for rapid CEA are multifaceted, including system changes that address public awareness of stroke and 911 response, improvements in vascular imaging access, and redesign of clinical services to promote collaboration and fast-tracking of care. Implementation of performance measures to monitor and guide clinical innovations is recommended.

Background Recent studies have strongly indicated the benefits of endovascular therapy for acute ischemic stroke, but what remains a continued debate is the role for general anaesthesia versus conscious sedation (CS) for such procedures. Retrospective studies have found poorer neurological outcomes in patients who underwent general anesthesia (GA); however, some have revealed worse baseline stroke severity in these patients.Methods This study is a retrospective cohort study aimed at comparing mortality and morbidity of GA versus CS in patients treated with endovascular intervention in acute ischemic stroke. Chi-square and t-test analyses were used. Results Patients in the GA (n=42) group were more likely to be deceased than those in the CS (n=67) group at hospital discharge, 3 months, and 6 months poststroke onset. Morbidity, as defined by modified Rankin Score, was significantly greater in the GA group at hospital discharge, and a similar trend was seen in morbidity at 3 months postdischarge. Conclusion General anesthesia for endovascular intervention in acute ischemic stroke was associated with increased mortality and poorer neurological incomes compared with conscious sedation. In our study, age, gender, history of hypertension, history of diabetes, and baseline National Institute of Health Stroke Scale were not significantly different between the groups. Although the need for a randomized, prospective study on this topic is clear, our study represents further corroboration of the safety and efficacy of conscious sedation in these procedures.

Background: The diagnosis of a sports-related concussion is often dependent on the athlete self-reporting their symptoms. It has been suggested that improving youth athlete knowledge and attitudes toward concussion may increase self-reporting behaviour. The objective of this study was to determine if a novel Concussion-U educational program improves knowledge of and attitudes about concussion among a cohort of elite male Bantam and Midget AAA hockey players. Methods: Fifty-seven male Bantam and Midget AAA-level hockey players (mean age=14.52±1.13 years) were recruited from the local community. Each participant completed a modified version of the Rosenbaum Concussion Knowledge and Attitudes Survey–Student Version immediately before and after a Concussion-U educational presentation. Follow-up sessions were arranged 4 to 6 months after the presentation, and assessed retention of knowledge and attitude changes. Results: Forty-three players completed all three surveys. Concussion knowledge and attitude scores significantly (p<0.01) increased from pre- to post-presentation by 12.79 and 8.41%, respectively. At long-term follow-up, knowledge levels remained significantly (p<0.01) higher than baseline by 8.49%. Mean attitude scores were also increased at follow-up; however, this increase was not statistically significant. Conclusions: A Concussion-U educational program led to an immediate improvement in concussion knowledge and attitudes among elite male Bantam and Midget AAA hockey players. Increased knowledge was maintained at long-term follow-up, but improved attitude was not. Future studies should investigate whether similar educational programs influence symptom reporting and concussion incidence. In addition, they should focus on how to maintain improved concussion attitudes.

Objective background: The Montreal Cognitive Assessment (MoCA) is a questionnaire that has been developed to help physicians around the world diagnose a patient’s cognitive ability. Available in multiple languages and for use in multiple countries worldwide, the goal of this study was to validate the alternate versions 2 and 3 of the French MoCA test to assist physicians in the detection of mild cognitive impairment (MCI), while decreasing the learning effect upon frequent testing. Methods: A validation study was conducted at the MoCA Clinic and Institute in Québec, Canada. The subject population consisted of 25 patients diagnosed with MCI meeting Petersen criteria and 25 healthy subjects serving as the normal control (NC) group. Three MoCA test versions were administered in the French language in random order within one session. Scores obtained in all three versions in MCI and NC groups were assessed for reliability and consistency from one version to the next. Results: On average, scores obtained in each subject group (MCI and NC) fell within their corresponding diagnostic ranges (score above 26 points for NC patients versus scores below 26 points for MCI patients). Difference in scores observed between the original French MoCA version and the two alternate versions in each subject cohort were minimal and not considered clinically significant. Conclusions: All three test versions of the French MoCA are considered equivalent in diagnostic reliability and consistency and contribute to decreasing the potential learning effect when patients are required to repeat the test frequently.

Background: Approximately 30% of patients with epilepsy have medically intractable seizures, and a proportion of them are candidates for surgical treatment. The efficacy and safety of epilepsy surgery have been supported by a large number of studies, yet only a small minority of such patients in Ontario receive surgery. Methods: Family physicians in Ontario were surveyed regarding demographics, referral practices and general knowledge about epilepsy surgery. Four hundred surveys were mailed to randomly selected family physicians using contact information from the College of Physicians and Surgeons of Ontario website. Results: The response rate was 50%. The majority of family physicians (81%) always refer patients with epilepsy, most often to neurologists. General knowledge of epilepsy was mixed, with 53.7% feeling that surgery should be considered in selected cases for the treatment of epilepsy, though 53.2% did not know what type of epilepsy could be surgically treated. Conclusions: The results suggest a relatively low level of knowledge among family physicians in terms of when surgery ought to be considered, the types of epilepsy that are amenable to surgical treatment and the risks and benefits of epilepsy surgery. A lack of knowledge in these areas may partly underlie the low referral rates of epilepsy patients, though the results show that the majority of family physicians refer their patients with epilepsy to neurologists. Other factors must be considered, such as access to neurologists, epileptologists and surgical resources. Education campaigns directed at family physicians may improve knowledge and change referral practices. Future studies need to examine these possibilities.

Background: Patient-centered care for individuals with myotonic dystrophy (DM1) and Huntington’s disease (HD)—chronic, progressive, and life-limiting neurological conditions—may be challenged by patients’ cognitive and behavioral impairments. However, no research has explored health care providers’ (HCPs’) perspectives about patient-centered care provision for these patients along their disease trajectory. Methods: Constructivist grounded theory informed the iterative data collection and analysis process. Eleven DM1 or HD HCPs participated in semistructured interviews, and three stages of coding were used to analyze their interview transcripts. Codes were collapsed into themes and categories.Results: Three categories including an evolving care approach, fluid roles, and making a difference were identified. Participants described that their clinical care approach evolved depending on the patient’s disease stage and caregivers’ degree of involvement. HCPs described that their main goal was to provide hope to patients and caregivers through medical management, crisis prevention, support, and advocacy. Despite the lack of curative treatments, HCPs perceived that patients benefited from ongoing clinical care provided by proactive clinicians. Conclusions: Providing care for individuals with DM1 and HD is a balancing act. HCPs must strike a balance between (1) the frustrations and rewards of patient-centered care provision, (2) addressing symptoms and preventing and managing crises while focusing on patients’ and caregivers’ quality of life concerns, and (3) advocating for patients while addressing caregivers’ needs. This raises important questions: Is patient-centered care possible for patients with cognitive decline? Does chronic neurological care need to evolve to better address patients’ and caregivers’ complex needs?

Background: OnabotulinumtoxinA is an efficacious treatment option for patients with various conditions. Although studies have reported on the efficacy of onabotulinumtoxinA, quality of life (QoL) data are limited. This study evaluated QoL in patients treated with onabotulinumtoxinA across various therapeutic indications. Methods: MDs on BOTOX Utility (MOBILITY) was a prospective, multicenter, observational Canadian study in patients initiating (naïve) or receiving ongoing (maintenance) onabotulinumtoxinA treatment. Health utility was the primary outcome measure and was obtained from the Short Form-12 Health Survey using the Short Form-6D at baseline, week 4 posttreatment, and up to five subsequent treatment visits. The safety cohort included patients who received ≥1 onabotulinumtoxinA treatment. Results: The efficacy cohort included 1062 patients; the majority were Caucasian, female, and on maintenance onabotulinumtoxinA treatment. Adult focal spasticity (n=398), blepharospasm (n=81), cerebral palsy (n=22), cervical dystonia (n=234), hemifacial spasm (n=116), and hyperhidrosis (n=211) patients were included. Baseline health utility was generally higher in maintenance versus naïve patients; however, naïve patients showed the greatest improvements over time. Health utility was generally maintained or trended toward improvement across all cohorts, including maintenance patients who had been treated for up to 22 years before study entry. Eighteen of 1222 patients (2%) in the safety cohort reported 28 treatment-related adverse events; eight were serious in four patients. Conclusion: MOBILITY is the largest prospective study to date to provide QoL data over a variety of therapeutic indications following treatment with onabotulinumtoxinA. Although the QoL burden varies by disease, data suggest that long-term treatment may help improve or maintain QoL over time.

Background: Parkinson’s disease (PD) and other parkinsonian syndromes are chronic, progressive neurodegenerative diseases. With advancing disease, both motor and non-motor symptoms represent a considerable burden and symptom relief and quality of life improvement become the main goal of treatment. Botulinum toxins (BTX) are an effective treatment modality for many neurological conditions. Methods: To understand the potential usefulness of BTX in this population, we performed a retrospective chart review of all patients with a clinical diagnosis of idiopathic PD and atypical parkinsonism who received treatment with BTX injections in our center from 1995 to 2014 for a variety of symptoms. Response to BTX was assessed using a subjective Clinical Global Impression. Results: Records of 160 patients were reviewed. Probable idiopathic PD was the diagnosis in 117 patients (73.1%). The main indication for BTX treatment was pain (50.6% of cases). Other indications were the treatment of functional impairment resulting from dystonia (26.25%), sialorrhea (18.75%), freezing of gait, and camptocormia. Considering pain as indication, 81% of all patients with PD reported benefits after the first BTX injections. This benefit was maintained after the last recorded visit without significant difference in outcome compared with the first injection (p=0.067). Similar results were observed in patients with atypical parkinsonism. Conclusions: Our results confirm the safety and efficacy of different uses of BTX in the symptomatic treatment of patients with parkinsonism even in advanced stages of the disease, and suggest BTX treatment could have a safe and useful role in the treatment of pain in this population.

Objective: Oxidative stress is involved in the pathogenesis of multiple system atrophy (MSA). The aim of this study is to examine oxidant biomarkers including homocysteine (Hcys), bilirubin, uric acid, lipids, and potential environmental risk factors and to ascertain whether these data correlate with MSA in a Chinese population. Methods: In this study, serum levels of Hcys, bilirubin, uric acid, and lipids were studied in 55 MSA patients and 76 healthy controls (HCs). Education, anti-parkinsonian agent usage, smoking, drinking, farming, and living area of the subjects also were analyzed. The Unified MSA Rating Scale (UMSARS), Hoehn & Yahr stage, International Cooperative Ataxia Rating Scale, and Mini-Mental State Examination were used to assess the disease severity, the parkinsonism, ataxia, and the cognitive ability of MSA, respectively. Results: The levels of Hcys were higher (p<0.001) and those of total bilirubin (p=0.007), indirect bilirubin (p=0.011), and total cholesterol (p=0.046) were lower in MSA patients than in healthy controls, whereas uric acid levels did not differ significantly between MSA and healthy controls. Moreover, Hcys levels in MSA patients had positive correlations with illness duration (rs=0.422, p=0.001) and UMSARS-I (rs=0.555, p<0.001), respectively. High-density lipoprotein cholesterol levels were negatively correlated with UMSARS-I (rs=−0.325, p=0.015). Farming was more frequent in MSA patients (1-20 years: odds ratio, 6.36; p<0.001; >20 years: odds ratio, 10.26; p=0.001), whereas current smoking was less frequent (odds ratio, 0.13, p=0.002). Conclusions: Elevated Hcys and decreased high-density lipoprotein cholesterol may be associated with the disease severity of MSA. Environmental exposures such as farming and smoking may contribute to the occurrence but not the progression of MSA.

Classical neurosurgical teaching suggests that corticosteroid administration reduces the diagnostic yield of stereotactic brain biopsy for primary central nervous system lymphoma (PCNSL). In a single-center series spanning 6 years, we reviewed 155 consecutive biopsy patients, 135 treated with prebiopsy corticosteroids. PCNSL was correctly diagnosed on initial biopsy in 15 of 16 steroid-treated patients; in the single nondiagnostic specimen, polymerase chain reaction reanalysis by an outside institution showed evidence of lymphoproliferative disease consistent with PCNSL. Our data challenge the notion that it is necessary to withhold corticosteroid therapy for cerebral edema in patients awaiting stereotactic biopsy for suspected PCNSL.