Background: Tuberous sclerosis complex (TSC) is a neurocutaneous disorder with a wide spectrum of manifestations. Recent consensus recommendations stress the importance of multidisciplinary management of children with TSC. The objective of this study was to examine the manifestations of TSC at a large referral centre to determine the care needs of this population. Methods: A retrospective, systematic chart review was performed of children with TSC managed at British Columbia Children’s Hospital. Patients were identified through epilepsy and clinical neurophysiology databases. Results: The study population comprised 81 patients, born between 1987 and 2014, who were a median of 10 years (range, 0.2-23.2) at most recent follow-up. Epilepsy occurred in 91% of patients, including 32% with a history of infantile spasms. Nineteen patients underwent epilepsy surgery, nine (47%) of whom were seizure-free at most recent follow-up. Overall, 61% of epilepsy patients had been seizure-free for at least 1 year at the time of last follow-up. Neuropsychiatric disorders were diagnosed in 49% of children, with autism (25%), attention deficit hyperactivity order (19%) and anxiety (16%) being the most common. Cardiac rhabdomyomata occurred in 35% of children and renal angiomyolipomas were seen in 43%. A total of 91% had skin manifestations. Conclusion: This study outlines the multisystem manifestations of TSC, observed through a large pediatric referral center. Epilepsy and neuropsychiatric disorders are the major source of morbidity in this age group and provide many challenges to the treating clinician. Because a subset of the study population is still quite young, the prevalence of neuropsychiatric disorders is likely underestimated.

Background: Perinatal stroke is the most common cause of hemiparetic cerebral palsy. Post-stroke plasticity is well studied in adults, but mechanisms in children are poorly understood. To better understand the relationship between functional connectivity and disability, we used rsfMRI to compare connectivity with sensorimotor dysfunction. Methods: Subjects with periventricular venous infarction were compared to controls. Resting-state BOLD signal was acquired on 3T MRI and analyzed using SPM12. Functional connectivity was computed between S1 and M1 of the left/non-lesioned and right/ lesioned hemisphere. Primary outcome was connectivity expressed as a Pearson correlation coefficient. Motor function was measured using the Assisting Hand Assessment (AHA), and Melbourne Assessment (MA). Proprioceptive function was measured using a robotic position matching task (VarXY). Results: Subjects included 17 PVI and 21 controls. AHA and MA in patients were negatively correlated with connectivity (increased connectivity=poorer performance). Correlations between AHA and connectivity between non-lesioned M1 to bilateral S1s were significant. VarXY in PVI was inversely correlated with connectivity (increased connectivity=improved performance), significantly between non-lesioned S1 and bilateral M1s. Control VarXY was positively correlated with connectivity between non-dominant S1 to bilateral M1s. Conclusions: We demonstrated significant correlations between connectivity and motor/sensory function in PVI patients. Greater insight into understanding reorganization of brain networks following perinatal stroke may facilitate personalized rehabilitation.

Objectives: To compare growth patterns of nonfunctioning and prolactin-producing pituitary macroadenomas, and to find whether their specific growth patterns are associated with clinically significant effects on vision. Materials and Methods: From our comprehensive provincial neuropituitary registry, we retrospectively identified 35 randomly selected patients each with nonfunctioning adenomas and prolactinomas >10 mm in any dimension. MRI scans were analyzed to determine the superior and inferior growth, volume, and maximum craniocaudal height of the adenomas. Patients underwent visual field testing at diagnosis. Continuous variables were compared using Student’s t test, the Mann–Whitney U test, and ANOVA. Categorical variables were compared using the chi-square test. Results: The mean height of prolactinomas (23.2±11.3 mm) was similar to nonfunctioning adenomas (22.3±9.3 mm, p=0.8), and so were mean tumor volumes (prolactinoma=5.9±8 ml vs. nonfunctioning adenoma=4.8±5 ml, p=0.47). However, the mean suprasellar growth for prolactinomas was 2.9±5.3 mm and 7.3±4.7 mm for nonfunctioning adenomas (p<0.001), and the mean infrasellar growth was 10.2±8.0 and 5.0±6.6 mm, respectively (p=0.04). The inferior growth pattern of prolactinomas was associated with a significantly lower likelihood of having visual field abnormalities (11.4 vs. 57.1%, p<0.001). Conclusions: Prolactinomas have predominantly inferior growth compared to nonfunctioning adenomas and are less likely to cause vision changes.

Background: Health administrative data are a common population-based data source for traumatic brain injury (TBI) surveillance and research; however, before using these data for surveillance, it is important to develop a validated case definition. The objective of this study was to identify the optimal International Classification of Disease , edition 10 (ICD-10), case definition to ascertain children with TBI in emergency room (ER) or hospital administrative data. We tested multiple case definitions. Methods: Children who visited the ER were identified from the Regional Emergency Department Information System at Alberta Children’s Hospital. Secondary data were collected for children with trauma, musculoskeletal, or central nervous system complaints who visited the ER between October 5, 2005, and June 6, 2007. TBI status was determined based on chart review. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated for each case definition. Results: Of 6639 patients, 1343 had a TBI. The best case definition was, “1 hospital or 1 ER encounter coded with an ICD-10 code for TBI in 1 year” (sensitivity 69.8% [95% confidence interval (CI), 67.3-72.2], specificity 96.7% [95% CI, 96.2-97.2], PPV 84.2% [95% CI 82.0-86.3], NPV 92.7% [95% CI, 92.0-93.3]). The nonspecific code S09.9 identified >80% of TBI cases in our study. Conclusions: The optimal ICD-10–based case definition for pediatric TBI in this study is valid and should be considered for future pediatric TBI surveillance studies. However, external validation is recommended before use in other jurisdictions, particularly because it is plausible that a larger proportion of patients in our cohort had milder injuries.

Background: Recurrent ataxia is encountered infrequently in clinical pediatric neurology practise and presents with diagnostic challenges. It is caused by several disorders. Our aims were to describe the epidemiology and clinical features in children with recurrent ataxia. Materials and Methods: A retrospective review was undertaken in 185 children with chronic ataxia, who presented during 1991 to 2008. Several databases were searched to ensure optimum ascertainment. Patients with brain tumors or isolated disorders of the peripheral nerves or vestibular system were excluded. Results: Recurrent ataxia was reported in 21 patients. Their age range was between 6 and 32.75 years (males=12). The crude period prevalence rate for the 18-year study period was 7.44/100,000. Eight patients had episodic ataxia and seven had inflammatory and metabolic disorders. In the rest the etiology was unknown. Many patients presented with ataxia, dizziness, and vertigo. The frequency and duration of the ataxic episodes varied from several per day to one every few months. Other clinical features included developmental delay and seizures. Neuroimaging in episodic ataxia was normal and abnormal in inflammatory or metabolic disorders. Acetazolamide provided symptomatic relief in patients with episodic ataxia, while steroids were beneficial in patients with an inflammatory etiology. One child with a metabolic disorder died. Conclusions: Recurrent ataxia is an uncommon presentation in children and mortality is rare. Genetic, metabolic, and inflammatory disorders should be considered in these patients. Neuroimaging is essential. Acetazolamide in selected patients provides good symptomatic relief.

Background: The current study involves a national survey of healthcare providers who offer services for individuals with a variety of neurological conditions. It aims to describe the provision of health and community-based services as well as the admission criteria, waitlist practices, and referral sources of these services. Methods: An online survey was directed at administrators/managers from publicly funded hospital programs, long-term care homes, and community-based healthcare provider agencies that were believed to be providing information and/or services to patients with a variety of neurological conditions. Results: Approximately 60% (n=254) of respondents reported providing services in either urban/suburban areas or rural/remote areas only, whereas the remaining 40% (n=172) provided services regardless of patient location. A small proportion of respondents reported providing services for individuals with dystonia (28%), Tourette syndrome (17%), and Rett syndrome (13%). There was also a paucity of diverse healthcare professionals across all institutions, but particularly mental healthcare professionals in hospitals. Lastly, the majority of respondents reported numerous exclusion criteria with regard to service provision, including prevalent comorbid conditions. Conclusions: If the few services provided for these neurological patient populations exclude common comorbidities, it is likely that there will be no other place for these individuals to seek care.

Introduction: Apathy is highly prevalent in Alzheimer’s disease (AD), but whether pharmacotherapy is effective in managing apathy is unclear. Methods: To assess the efficacy of pharmacotherapy for apathy in AD we searched for randomized controlled trials (RCT) and aggregate data reporting on apathy in several search engines, reference lists of articles, and reviews. Demographic characteristics and relevant data were extracted to assess apathy. Results: Fifteen RCTs’ were examined, and 11 were used in aggregate meta-analytic statistics. Drugs included were cholinesterase inhibitors, memantine, and psycho-stimulants. We found no significant treatment effect in favour of any of the drugs, and the effect-size estimates under a random effect model were heterogeneous. Most RCTs had a high attrition rate and used the NPI apathy subscale to measure apathy. Conclusion: The lack of an effect could be explained by methodological limitations, publication bias, and heterogeneity.

Background: Standardized data collection for traumatic brain injury (TBI) (including concussion) using common data elements (CDEs) has strengthened clinical care and research capacity in the United States and Europe. Currently, Ontario healthcare providers do not collect uniform data on adult patients diagnosed with concussion. Objective: The Ontario Concussion Care Strategy (OCCS) is a collaborative network of multidisciplinary healthcare providers, brain injury advocacy groups, patient representatives, and researchers with a shared vision to improve concussion care across the province, starting with the collection of standardized data. Methods: The International Framework of Functioning Disability and Health was selected as the conceptual framework to inform the selection of CDEs. The CDEs recommended by the OCCS were identified using key literature, including the National Institute of Neurological Disorders and Stroke–Zurich Consensus Statements for concussion in sport and the Ontario Neurotrauma Foundation Concussion/mTBI clinical guidelines. Results: The OCCS has recommended and piloted CDEs for Ontario that are readily available at no cost, clinically relevant, patient friendly, easy to interpret, and recognized by the international scientific community. Conclusions: The implementation of CDEs can help to shift Ontario toward internationally recognized standard data collection, and in so doing yield a more comprehensive evidence-based approach to care while also supporting rigorous research.

Background: Fatigue was recently reported to be the largest contributor to poor health-related quality of life (HRQOL) in paediatric Duchenne muscular dystrophy (DMD). Additional studies are necessary to confirm the generalizability of this finding. Our objective was to explore the longitudinal relationship between fatigue and HRQOL in an additional cohort of DMD patients. Methods: We performed a secondary analysis of data from a clinical trial (NCT00592553), which enrolled patients with nonsense mutation DMD, aged 5–20 years, from 37 sites in 11 countries (N=174). Fatigue and HRQOL were assessed using the PedsQLTM Multidimensional Fatigue Scale and Generic Core Scales, respectively, by patient- and parent-report at baseline and over 48 weeks. Results: Patients reported greater fatigue than healthy controls from published data. There was no significant difference between patient- and parent-reported fatigue. Fatigue was significantly correlated with worse HRQOL at baseline, by patient-report (r=0.70, P<0.001) and parent-report (r=0.70, P<0.001); and at 48 weeks, by patient-report (r=0.79, P<0.001) and parent-report (r=0.74, P<0.001). Change in fatigue was significantly correlated with change in HRQOL over 48 weeks, by patient-report (r=0.64, P<0.001) and parent-report (r=0.67, P<0.001). Conclusions: Fatigue is a major contributor to HRQOL in DMD. The strong association between fatigue and HRQOL corroborates previous studies, and suggests that reducing fatigue may improve HRQOL.

Background: Mild traumatic brain injury (mTBI) is a significant public health concern. Research has shown that mTBI is associated with persistent physical, cognitive, and behavioural symptoms, leading to significant direct and indirect medical costs. Our objective was to determine if age impacts the type and severity of post-mTBI symptoms experienced. Methods: Retrospective analysis of prospectively collected data at a level 1 tertiary care outpatient head injury clinic. Participants (N=167) were patients seen at the clinic following an mTBI. The Rivermead Post-Concussion Symptoms Questionnaire was used to assess symptom severity. Results: In our sample, the mean age was 44±16 years with 51% males. Compared with other age groups, patients >66 years of age were significantly more likely to report an mTBI between 6 AM to 12 PM (69%). Middle-aged patients (36-55 years) were more likely to report higher severity of certain post-mTBI symptoms (headache, nausea and vomiting, irritability, poor concentration, sleep disturbance, blurry vision, light sensitivity, and taking longer to think) compared with patients >66 years of age. Conclusions: In general, middle-aged patients reported higher severity of post-mTBI symptoms compared with the oldest patients. Thus, there was a significant association between age and the severity of specific mTBI symptoms, which highlights the need for targeted management. Additional research is needed to understand the mechanisms that could be contributing to the higher symptom severity experienced by the middle-aged group.

Background: Lack of a health advocacy curriculum and clarity are obstacles for effectively teaching neurology health advocacy (NHA) to neurology residents. Our purpose is to assess the need and develop content for a NHA curriculum and to describe its underlying components. Methods: This is a cross-sectional study with two steps. In step one, neurologists and neurology residents at University of Calgary were surveyed about their perception of teaching NHA and asked to rank 56 neurological diseases on a Likert scale based on how well they lend themselves to teaching health advocacy. In step two, curricular items were developed for the top five neurological diseases, using a modified Delphi procedure. The reliability of the survey instrument was determined by Cronbach’s alpha. Exploratory factor analysis was used to identify the underlying components of NHA. Results: Forty-six neurologists and 14 neurology residents were surveyed, with a response rate of 88.33%. Fifty-six percent of neurologists and 85% of residents believe that NHA curriculum is needed. The top five neurological presentations, that lend themselves easily to teaching NHA were: stroke/transient ischemic attacks, alcoholism, epilepsy, Alzheimer’s disease, and multiple sclerosis. The survey instrument reliability was 0.97. Exploratory factor analysis revealed four factors that can explain the variability in the survey instrument: multidisciplinary approach to neurological disorders, prevention of recurrence of neurological disease, collaboration with other medical subspecialties, and communication with professions outside the medical field. Conclusion: Neurologists’ and residents’ responses support that NHA curriculum is needed. Four components of NHA were identified that can be used for teaching NHA as well as health advocacy in general practice.

Objective: To explore the relationship between migraine and anxiety disorders, mood disorders and perceived mental health in a population-based sample of adolescents. Methods: The Canadian Community Health Survey (CCHS) is a cross-sectional health survey sampling a nationally representative group of Canadians. In this observational study, data on all 61,375 participants aged 12-19 years from six survey cycles were analyzed. The relationships between self-reported migraine, perceived mental health, and mood/anxiety disorders were modeled using univariate and multivariate logistic regression. The migraine–depression association was also explored in a subset of participants using the Composite International Diagnostic Interview–Short Form (CIDI–SF) depression scale. Results: The odds of migraine were higher among those with mood disorders, with the strongest association in 2011-2 (adjusted odds ratio [aOR]=4.59; 95% confidence interval [CI95%]=3.44-6.12), and the weakest in 2009-10 (aOR=3.06, CI95%=2.06-4.55). The migraine–mood disorders association was also significant throughout all cycles, other than 2011-2, when the CIDI–SF depression scale was employed. The odds of migraine were higher among those with anxiety disorders, with the strongest association in 2011-2 (aOR=4.21, CI95%=3.31-5.35) and the weakest in 2010 (aOR=1.87, CI95%=1.10-3.37). The inverse association between high perceived mental health and the odds of migraine was observed in all CCHS cycles, with the strongest association in 2011-2 (aOR=0.58, CI95%=0.48-0.69) and the weakest in 2003-4 (aOR=0.75, CI95%=0.62-0.91). Conclusions: This study provides evidence, derived from a large population-based sample of adolescents, for a link between migraine and mood/anxiety disorders.

Objective: Multiple system atrophy (MSA) is an incurable neurodegenerative illness in which progressive symptoms, including stridor and acute laryngeal obstruction, occur. Advanced care planning and palliative care discussions in people living with MSA are not well defined. The aim of the present study is to evaluate advanced care planning and current practices in palliative care in MSA to identify opportunities for improving quality of care. Methods: The study is a retrospective chart review assessing the focus and timing of palliative care discussions in people living with MSA. Some 22 charts were reviewed. Results: A total of 22 patients were included. The most common symptoms were parkinsonism, orthostatic hypotension, GI/GU dysfunction, ataxia and gait impairment. Six patients had stridor. Of the palliative care discussions that took place, the most common topics were diagnosis, symptoms or symptom management, and prognosis. In the majority of patients who died and who had a do-not-attempt-resuscitation order, discussions surrounding resuscitation and goals of care took place only hours before death. Conclusions: There is no standard approach to advanced care planning and palliative care discussions in people living with MSA. We propose a framework to guide advanced care planning and palliative care discussions in MSA.

Objectives: Intrathecal morphine (ITM) is an efficacious method of providing postoperative analgesia and reducing pain associated complications. Despite adoption in many surgical fields, ITM has yet to become a standard of care in lumbar spine surgery. Spine surgeons’ reticence to make use of the technique may in part be attributed to concerns of precipitating a cerebrospinal fluid (CSF) leak. Methods: Herein we describe a method for oblique intrathecal injection during lumbar spine surgery to minimize risk of CSF leak. The dural sac is penetrated obliquely at a 30° angle to offset dural and arachnoid puncture sites. Oblique injection in instances of limited dural exposure is made possible by introducing a 60° bend to a standard 30-gauge needle. Results: The technique was applied for injection of ITM or placebo in 104 cases of lumbar surgery in the setting of a randomized controlled trial. Injection was not performed in two cases (2/104, 1.9%) following preinjection dural tear. In the remaining 102 cases no instances of postoperative CSF leakage attributable to oblique intrathecal injection occurred. Three cases (3/102, 2.9%) of transient CSF leakage were observed immediately following intrathecal injection with no associated sequelae or requirement for postsurgical intervention. In two cases, the observed leak was repaired by sealing with fibrin glue, whereas in a single case the leak was self-limited requiring no intervention. Conclusions: Oblique dural puncture was not associated with increased incidence of postoperative CSF leakage. This safe and reliable method of delivery of ITM should therefore be routinely considered in lumbar spine surgery.

Background: The proposed implementation of work hour restrictions has presented a significant challenge of maintaining the quality of resident education and ensuring adequate hands-on experience that is essential for novice surgeons. To maintain the level of resident surgical competency, revision of the apprentice model of surgical education to include supplementary educational methods, such as laboratory and virtual reality (VR) simulations, have become frequent topics of discussion. We aimed to better understand the role of supplementary educational methods in Canadian neurosurgery residency training. Methods: An online survey was sent to program directors of all 14 Canadian neurosurgical residency programs and active resident members of the Canadian Neurosurgical Society (N=85). We asked 16 questions focusing on topics of surgeon perception, current implementation and barriers to supplementary educational models. Results: Of the 99 surveys sent, 8 out of 14 (57%) program directors and 37 out of 85 (44%) residents completed the survey. Of the 14 neurosurgery residency programs across Canada, 7 reported utilizing laboratory-based teaching within their educational plan, while only 3 programs reported using VR simulation as a supplementary teaching method. The biggest barriers to implementing supplementary educational methods were resident availability, lack of resources, and cost. Conclusions: Work-hour restrictions threaten to compromise the traditional apprentice model of surgical training. The potential value of supplementary educational methods for surgical education is evident, as reported by both program directors and residents across Canada. However, availability and utilization of laboratory and VR simulations are limited by numerous factors such as time constrains and lack of resources.

Purpose: To examine the safety and tolerability of clinical graded aerobic treadmill testing in recovering adolescent moderate and severe traumatic brain injury (TBI) patients referred to a multidisciplinary pediatric concussion program. Methods: We completed a retrospective case series of two moderate and five severe TBI patients (mean age, 17.3 years) who underwent initial Buffalo Concussion Treadmill Testing at a mean time of 71.6 days (range, 55-87) postinjury. Results: Six patients completed one graded aerobic treadmill test each and one patient underwent initial and repeat testing. There were no complications. Five initial treadmill tests were completely tolerated and allowed an accurate assessment of exercise tolerance. Two initial tests were terminated early by the treatment team because of neurological and cardiorespiratory limitations. As a result of testing, two patients were cleared for aerobic exercise as tolerated and four patients were treated with individually tailored submaximal aerobic exercise programs resulting in subjective improvement in residual symptoms and/or exercise tolerance. Repeat treadmill testing in one patient performed after 1 month of treatment with submaximal aerobic exercise prescription was suggestive of improved exercise tolerance. One patient was able to tolerate aerobic exercise following surgery for posterior glottic stenosis. Conclusions: Preliminary results suggest that graded aerobic treadmill testing is a safe, well tolerated, and clinically useful tool to assess exercise tolerance in appropriately selected adolescent patients with TBI. Future prospective studies are needed to evaluate the effect of tailored submaximal aerobic exercise prescription on exercise tolerance and patient outcomes in recovering adolescent moderate and severe TBI patients.

Objectives: Work-related traumatic brain injuries (TBIs) are not well documented in the literature. Published studies mostly rely on worker databases that fail to provide clinically relevant information. Our objective is to describe the characteristics of hospitalized patients and their work-related TBI. Methods: We used the Québec provincial trauma and TBI program databases to identify all patients with a diagnosis of work-related TBI admitted to the Montreal General Hospital, a level 1 trauma center, between 2000 and 2014. Data from their medical records were extracted using a predetermined information sheet. Simple descriptive statistics (means and percentages) were used to summarize the data. Results: A total of 285 cases were analyzed. Workplace TBI patients were middle-aged (mean, 43.62 years), overwhelmingly male (male:female 18:1), mostly healthy, and had completed a high school level education. Most workers were from the construction industry; falling was the most common mechanism of injury. The majority of patients (76.8%) presented with a mild TBI; only a minority (14%) required neurosurgery. The most common finding on computed tomography was skull fracture. The median length of hospitalization was 7 days, after which most patients were discharged directly home. A total of 8.1% died of their injuries. Conclusions: Our study found that most hospitalized victims of work-related TBI had mild injury; however, some required neurosurgical intervention and a non-negligible proportion died of their injury. Improving fall prevention, accurately document helmet use and increasing the safety practice in the construction industry may help decrease work-related TBI burden.