Publications by authors named "Daniela Pohl"

69 Publications

Can serum glial fibrillary acidic protein (GFAP) solve the longstanding problem of diagnosis and monitoring progressive multiple sclerosis.

Mult Scler Relat Disord 2021 05 31;50:102931. Epub 2021 Mar 31.

Department of Neurology, Queen Mary University London, Neuroscience Centre. Electronic address:

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http://dx.doi.org/10.1016/j.msard.2021.102931DOI Listing
May 2021

B cell therapy and the use of RNA-based COVID-19 vaccines.

Mult Scler Relat Disord 2021 04 8;49:102887. Epub 2021 Mar 8.

Blizard Institute, Barts and The London School of Medicine and Dentistry, 4 Newark Street, London, E1 2AT.

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http://dx.doi.org/10.1016/j.msard.2021.102887DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7938733PMC
April 2021

Characterization of physical literacy in children with chronic medical conditions compared to healthy controls: a cross-sectional study.

Appl Physiol Nutr Metab 2021 Mar 9. Epub 2021 Mar 9.

Children's Hospital of Eastern Ontario Research Institute, Ottawa, Canada.

To determine the physical literacy, defined as the capability for a physically active lifestyle, of children with medical conditions compared with healthy peers, this multicenter cross-sectional study recruited children with medical conditions from cardiology, neurology (including concussion), rheumatology, mental health, respirology, oncology, hematology, and rehabilitation (including cerebral palsy) clinics. Participants aged 8-12 years (N=130; mean age: 10.0±1.44 years; 44% female) were randomly matched to three healthy peers from a normative database, based on age, sex, and month of testing. Total physical literacy was assessed by the Canadian Assessment of Physical Literacy, a validated assessment of physical literacy measuring physical competence, daily behavior, knowledge/understanding, and motivation/confidence. Total physical literacy mean scores(/100) did not differ (t(498)=-0.67; p=0.44) between participants (61.0±14.2) and matched healthy peers (62.0±10.7). Children with medical conditions had lower mean physical competence scores (/30; -6.5 [-7.44, -5.51]; p<0.001) but higher mean motivation/confidence scores (/30; 2.6 [1.67, 3.63]; p<0.001). Mean daily behavior and knowledge/understanding scores did not differ from matches (/30; 1.8 [0.26, 3.33]; p=0.02; /10; -0.04 [-0.38, 0.30]; p=0.81; respectively). Children with medical conditions are motivated to be physically active but demonstrate impaired movement skills and fitness, suggesting the need for targeted interventions to improve their physical competence. Novelty bullets: • Physical literacy in children with diverse chronic medical conditions is similar to healthy peers • Children with medical conditions have lower physical competence than healthy peers, but higher motivation and confidence • Physical competence (motor skill, fitness) interventions, rather than motivation or education, are needed for these youth.
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http://dx.doi.org/10.1139/apnm-2020-0957DOI Listing
March 2021

Is multiple sclerosis overdiagnosed?

Mult Scler Relat Disord 2021 Jan 28;47:102721. Epub 2020 Dec 28.

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http://dx.doi.org/10.1016/j.msard.2020.102721DOI Listing
January 2021

Air pollution and multiple sclerosis risk.

Mult Scler Relat Disord 2021 02 30;48:102797. Epub 2021 Jan 30.

Blizard Institute, Barts and The London School of Medicine and Dentistry, 4 Newark Street, London, E1 2AT.

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http://dx.doi.org/10.1016/j.msard.2021.102797DOI Listing
February 2021

Pediatric Hyperacute Arterial Ischemic Stroke Pathways at Canadian Tertiary Care Hospitals.

Can J Neurol Sci 2021 Feb 11:1-8. Epub 2021 Feb 11.

University of Ottawa, Faculty of Medicine, Ottawa, ON, Canada.

Background: 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.

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

Results: 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).

Conclusions: 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.
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http://dx.doi.org/10.1017/cjn.2021.27DOI Listing
February 2021

Poor adherence to sleep and physical activity guidelines among children with epilepsy.

Epilepsy Behav 2021 02 12;115:107722. Epub 2021 Jan 12.

Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada; Division of Neurology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada; Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada. Electronic address:

Objective: To assess physical activity and sleep rates in a cohort of children with epilepsy (CWE) and determine if there is a relationship between physical activity and sleep time.

Methods: Children aged 8-14 years with a diagnosis of epilepsy and at least one seizure in the past 12 months were monitored via a wrist-worn activity tracker for 16 weeks, to objectively measure daily physical activity, as assessed by step counts, and sleep time. Adherence to physical activity (≥12,000 steps/day) and sleep recommendations (≥9 h for children aged 8-12 years, or ≥8 h for children aged 13-15 years) was determined. To predict daily activity or nightly sleep, a series of multivariable models incorporating age, sex, day-type (all combinations of weekday or weekend and summer holiday or school), participant (as a random effect), daily physical activity (for models predicting sleep), nightly sleep (for models predicting physical activity), and autoregressive terms of previous sleep or physical activity were constructed, and the best-performing models were selected with Akaike information criterion analysis.

Results: Twenty-two children with mild to moderate epilepsy were recruited (54.5% female, median (IQR) age 11 (10, 13) years) and monitored for 16 weeks. They met the recommended level of physical activity only in 38.0% (21.7%, 59.4%), and sleep in 49.1% (30.0%, 68.5%) of days. They met both physical activity and sleep guidelines on the same day in only 17.8% (95% CI 7.1%, 38.0%). There was no association between meeting the recommended levels of daily physical activity and sleep time (p = 0.86, ρ = 0.03). In the best-performing model, age, sex, day type, and participant explained 28.9% of the variance in daily physical activity, with no additional insight provided by measures of sleep time. Age, sex, day type, participant, and daily physical activity explained 17.3% of the variance in nightly sleep time, with a statistically discernable but small association between physical activity and sleep time (1.79 ± 0.53, p = 0.001).

Conclusion: Our cohort of children with mild to moderate epilepsy showed poor adherence to sleep and physical activity guidelines. There was no clinically relevant association between daily physical activity and sleep among these children who were similarly active to healthy peers. Future studies should assess the effect of increased sleep hygiene and physical activity on overall well-being and seizure control in CWE.
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http://dx.doi.org/10.1016/j.yebeh.2020.107722DOI Listing
February 2021

Cost-effectiveness of fingolimod versus interferon-β1a for the treatment of pediatric-onset multiple sclerosis in Canada.

J Med Econ 2020 Dec 12;23(12):1525-1533. Epub 2020 Nov 12.

Novartis Pharma AG, Basel, Switzerland.

Aims: To evaluate the cost-effectiveness of fingolimod versus interferon (IFN)-β1a at a dose of 30 μg per week for the treatment of relapsing pediatric-onset multiple sclerosis (POMS) in Canada.

Material And Methods: A discrete-time Markov model was developed to compare fingolimod with IFN β-1a over a time horizon of two years representing patients followed up to mean age of 18 years from a Canadian health care system perspective. Twenty-one health states based on the Expanded Disability Status Scale (EDSS) were considered: EDSS 0‒9 for relapsing multiple sclerosis (MS), EDSS 0‒9 for secondary progressive MS, and "Death." Relative treatment efficacy for fingolimod versus IFN-β1a was estimated from the PARADIGMS study. Costs and resource use were obtained from published literature and Canadian sources. Utilities were estimated by mapping the Pediatric Quality of Life inventory data onto the Child Health Utility Index-9 Dimension using a published mapping algorithm. Future costs and benefits were discounted at 1.5% per annum.

Results: Compared with IFN β-1a, fingolimod led to an increase in quality-adjusted life-years (QALYs) (0.125) with incremental costs (Canadian dollars [CAD] 2,977) and to an incremental cost-effectiveness ratio (ICER) of CAD 23,886/QALY over a time horizon of two years representing patients followed up to mean age of 18 years. The monetary benefits of fingolimod treatment versus IFN β-1a at a willingness-to-pay (WTP) threshold of CAD 50,000 per QALY gained were higher than the costs. One-way sensitivity analysis and probabilistic sensitivity analysis (PSA) both confirmed the robustness of the results.

Limitations: The main limitations of this analysis primarily stem from the limited data availability in POMS.

Conclusions: Fingolimod is cost effective compared with IFN β-1a for the treatment of POMS over a time horizon of two years representing patients followed up to a mean age of 18 years in Canada.
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http://dx.doi.org/10.1080/13696998.2020.1840138DOI Listing
December 2020

Association of outcomes in acute flaccid myelitis with identification of enterovirus at presentation: a Canadian, nationwide, longitudinal study.

Lancet Child Adolesc Health 2020 11;4(11):828-836

SickKids Research Institute, Neuroscience and Mental Health Program, The Hospital for Sick Children, Toronto, ON, Canada; Division of Neurology, The Hospital for Sick Children, Toronto, ON, Canada; Department of Pediatrics, University of Toronto, Toronto, ON, Canada. Electronic address:

Background: Acute flaccid myelitis (AFM) is characterised by rapid onset of limb weakness with spinal cord grey-matter abnormalities on MRI scan. We aimed to assess whether detection of enterovirus in respiratory or other specimens can help predict prognosis in children with AFM.

Methods: In this nationwide, longitudinal study, we evaluated the significance of detection of enterovirus in any sample in predicting outcomes in a cohort of Canadian children younger than 18 years presenting with AFM to tertiary paediatric hospitals in Canada in 2014 and 2018. All patients fulfilled the 2015 US Centers for Disease Control and Prevention case definition for definite AFM or probable AFM. Clinical data, laboratory findings, treatment, and neuroimaging results were collected (follow up period up to 5 years). We assessed neurological function and motor outcomes using Kurtzke's Expanded Disability Status Scale (EDSS) and a Weakest Limb Score.

Findings: 58 children with AFM (median age 5·1 years, IQR 3·8-8·3) were identified across five of Canada's ten provinces and three territories. 25 (43%) children had enterovirus detected in at least one specimen: 16 (64%) with EV-D68, two (8%) with EV-A71, two (8%) with coxsackievirus, 10 (40%) with untyped enterovirus. Children who were enterovirus positive were more likely than those that were negative to have had quadriparesis (12 [48%] of 25 vs four [13%] of 30; p=0·028), bulbar weakness (11 [44%] of 25 vs two [7%] of 30; p=0·028), bowel or bladder dysfunction (14 [56%] of 25 vs seven [23%] of 30; p=0·040), cardiovascular instability (nine [36%] of 25 vs one [3%] of 30; p=0·028), and were more likely to require intensive care unit admission (13 [52%] of 25 vs 5 [17%] of 30; p=0·028). On MRI, most children who were enterovirus positive showed brainstem pontine lesions (14 [61%] of 23), while other MRI parameters did not correlate with enterovirus status. Median EDSS of enterovirus positive (EV+) and enterovirus negative (EV-) groups was significantly different at all timepoints: baseline (EDSS 8·5, IQR 4·1-9·5 vs EDSS 4·0, IQR 3·0-6·0; p=0·0067), 3 months (EDSS 4·0, IQR 3·0-7·4 vs EDSS 3·0, IQR 1·5-4·3; p=0·0067), 6 months (EDSS 3·5, IQR 3·0-7·0 vs EDSS 3·0, IQR 1·0-4·0; p=0·029), and 12 months (EDSS 3·0, IQR 3·0-6·9 vs EDSS 2·5 IQR 0·3-3·0; p=0·0067). Kaplan-Meier survival analysis of a subgroup of patients showed significantly poorer motor recovery among children who tested positive for enterovirus than for those who tested negative (p=0·037).

Interpretation: Detection of enterovirus in specimens from non-sterile sites at presentation correlated with more severe acute motor weakness, worse overall outcomes and poorer trajectory for motor recovery. These results have implications for rehabilitation planning as well as counselling of families of children with these disorders. The findings of this study support the need for early testing for enterovirus in non-CNS sites in all cases of AFM.

Funding: None.
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http://dx.doi.org/10.1016/S2352-4642(20)30176-0DOI Listing
November 2020

Endocrine and Growth Abnormalities in 4H Leukodystrophy Caused by Variants in POLR3A, POLR3B, and POLR1C.

J Clin Endocrinol Metab 2021 Jan;106(2):e660-e674

Department of Child Neurology, University Children's Hospital Tübingen, Tübingen, Germany.

Context: 4H or POLR3-related leukodystrophy is an autosomal recessive disorder typically characterized by hypomyelination, hypodontia, and hypogonadotropic hypogonadism, caused by biallelic pathogenic variants in POLR3A, POLR3B, POLR1C, and POLR3K. The endocrine and growth abnormalities associated with this disorder have not been thoroughly investigated to date.

Objective: To systematically characterize endocrine abnormalities of patients with 4H leukodystrophy.

Design: An international cross-sectional study was performed on 150 patients with genetically confirmed 4H leukodystrophy between 2015 and 2016. Endocrine and growth abnormalities were evaluated, and neurological and other non-neurological features were reviewed. Potential genotype/phenotype associations were also investigated.

Setting: This was a multicenter retrospective study using information collected from 3 predominant centers.

Patients: A total of 150 patients with 4H leukodystrophy and pathogenic variants in POLR3A, POLR3B, or POLR1C were included.

Main Outcome Measures: Variables used to evaluate endocrine and growth abnormalities included pubertal history, hormone levels (estradiol, testosterone, stimulated LH and FSH, stimulated GH, IGF-I, prolactin, ACTH, cortisol, TSH, and T4), and height and head circumference charts.

Results: The most common endocrine abnormalities were delayed puberty (57/74; 77% overall, 64% in males, 89% in females) and short stature (57/93; 61%), when evaluated according to physician assessment. Abnormal thyroid function was reported in 22% (13/59) of patients.

Conclusions: Our results confirm pubertal abnormalities and short stature are the most common endocrine features seen in 4H leukodystrophy. However, we noted that endocrine abnormalities are typically underinvestigated in this patient population. A prospective study is required to formulate evidence-based recommendations for management of the endocrine manifestations of this disorder.
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http://dx.doi.org/10.1210/clinem/dgaa700DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7823228PMC
January 2021

Continuous EEG in a Pediatric Intensive Care Unit: Adherence to Monitoring Criteria and Barriers to Adequate Implementation.

Neurocrit Care 2021 Apr 21;34(2):519-528. Epub 2020 Jul 21.

Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada.

Background: Subclinical seizures are common in critically ill children and are best detected by continuous EEG (cEEG) monitoring. Timely detection of seizures requires pediatric intensive care unit (PICU) physicians to identify patients at risk of seizures and request cEEG monitoring. A recent consensus statement from the American Clinical Neurophysiology Society (ACNS) outlines the indications for cEEG monitoring in critically ill patients. However, adherence to these cEEG monitoring criteria among PICU physicians is unknown. Our project had two goals: 1. To assess adherence to cEEG monitoring indications and barriers toward their implementation; 2. To improve compliance with the ACNS cEEG monitoring criteria in our PICU.

Methods: This is a single-institution study. A total of 234 PICU admissions (183 unique patients) were studied. A 6-month retrospective chart review identified PICU patients meeting ACNS criteria for cEEG monitoring, and patients for whom monitoring was requested. This was followed by an 8-week quality improvement project. During this mentorship period, a didactic 15-min lecture and summary handouts regarding the ACNS indications for cEEG monitoring were provided to all PICU physicians. Requests for cEEG monitoring during the mentorship period were compared to baseline adherence to cEEG monitoring recommendations, and barriers toward timely cEEG monitoring were assessed.

Results: Nearly every fifth PICU patient met cEEG monitoring indications, and prevalences of patients meeting those indications were similar in the retrospective and the prospective mentorship period (18% vs. 19%). Almost all patients (98%) requiring cEEG as per ACNS criteria met the indication for monitoring already at the time of their PICU admission. During the retrospective period, 23% of patients meeting ACNS criteria had a request for cEEG monitoring, which increased to 83% during the mentorship period. The median delay to cEEG initiation was 16.7 h during the mentorship period, largely due to limited hours of EEG technician availability. Electrographic seizures were identified in 36% of patients monitored, all within the first 120 min of cEEG recording. The majority (79%) of cEEGs informed clinical management.

Conclusions: A brief teaching intervention supplemented by pictographic handouts significantly increased adherence to cEEG monitoring recommendations, and cEEGs guided clinical management. However, there were long delays to cEEG initiation. In order to promptly recognize subclinical seizures in critically ill children, we strongly advocate for a routine screening for cEEG monitoring indications as part of the PICU admission process, and a care model allowing for cEEG initiation around-the-clock.
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http://dx.doi.org/10.1007/s12028-020-01053-8DOI Listing
April 2021

Expanding the phenotypic and molecular spectrum of RNA polymerase III-related leukodystrophy.

Neurol Genet 2020 Jun 11;6(3):e425. Epub 2020 May 11.

Department of Neurology and Neurosurgery (S.P., L.G., M.A.M.-R., L.T.T., K.G., L.D., M. Srour, K.P., G.B.), McGill University; Child Health and Human Development Program (S.P., M.A.M.-R., L.T.T., K.G., L.D., M. Srour, G.B.), Research Institute of the McGill University Health Centre; Department of Pediatrics (L.G., L.T.T., K.G., L.D., M. Srour, G.B.), McGill University, Montreal, Quebec, Canada; Division of Clinical and Metabolic Genetics (L.G.), Division of Neurology, the Hospital for Sick Children, University of Toronto, Ontario, Canada; Department of Pathology (C.F.-B.), CHU Sainte-Justine, Université de Montreal, Quebec, Canada; Division of Pathology and Laboratory Medicine (M.K.D.), Phoenix Children's Hospital, AZ; Department of Human Genetics (L.T.T., K.G., L.D., G.B.), McGill University, Montreal, Quebec, Canada; McGill University (K.P.), Brain Tumour Research Center Montreal Neurological Institute and Hospital, Quebec, Canada; Department of Neurology (D.L.R.), Department of Clinical Genomics, Department of Pediatrics, Mayo Clinic, Rochester, MN; Department of Pediatrics (M. Saito), University of California Riverside School of Medicine, Riverside Medical Clinic, CA; Department of Pediatrics (S.C.), Beaver Medical Group, Redlands, CA; Division of Pediatric Neurology (S.L.), Department of Pediatrics, Klinikum Dritter Orden, Munich, Germany; Institute of Human Genetics (B.A., T.B.H.), Technische Universität München, Munich, Germany; Institute of Medical Genetics and Applied Genomics (T.B.H.), University of Tübingen, Germany; Department of Neurology (I.T.-M., F.I.M., N.R.-E.), Hospital Universitario Nuestra Señora de Candelaria, Santa Cruz de Tenerife, Canary Islands, Spain; Department of Neurology (D.P.), Children's Hospital of Eastern Ontario, University of Ottawa, Ontario, Canada; Department of Pediatrics (S.N.) and Department of Neurology (A.G.), Wake Forest School of Medicine, Winston-Salem, NC; Adult and Paediatric National Metabolic Service (E.G.), Starship Children's Hospital, Auckland, New Zealand; and Division of Medical Genetics (G.B.), Department of Specialized Medicine, Montreal Children's Hospital and McGill University Health Centre, Quebec, Canada.

Objective: To expand the phenotypic spectrum of severity of POLR3-related leukodystrophy and identify genotype-phenotype correlations through study of patients with extremely severe phenotypes.

Methods: We performed an international cross-sectional study on patients with genetically proven POLR3-related leukodystrophy and atypical phenotypes to identify 6 children, 3 males and 3 females, with an extremely severe phenotype compared with that typically reported. Clinical, radiologic, and molecular features were evaluated for all patients, and functional and neuropathologic studies were performed on 1 patient.

Results: Each patient presented between 1 and 3 months of age with failure to thrive, severe dysphagia, and developmental delay. Four of the 6 children died before age 3 years. MRI of all patients revealed a novel pattern with atypical characteristics, including progressive basal ganglia and thalami abnormalities. Neuropathologic studies revealed patchy areas of decreased myelin in the cerebral hemispheres, cerebellum, brainstem, and spinal cord, with astrocytic gliosis in the white matter and microglial activation. Cellular vacuolization was observed in the thalamus and basal ganglia, and neuronal loss was evident in the putamen and caudate. Genotypic similarities were also present between all 6 patients, with one allele containing a variant causing a premature stop codon and the other containing a specific intronic splicing variant (c.1771-7C>G), which produces 2 aberrant transcripts along with some wild-type transcript.

Conclusions: We describe genotype-phenotype correlations at the extreme end of severity of the POLR3-related leukodystrophy spectrum and shed light on the complex disease pathophysiology.
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http://dx.doi.org/10.1212/NXG.0000000000000425DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7238899PMC
June 2020

4H leukodystrophy: Mild clinical phenotype and comorbidity with multiple sclerosis.

Neurol Genet 2020 Apr 11;6(2):e409. Epub 2020 Mar 11.

Faculty of Medicine (S.M.D., D.P.), University of Ottawa, ON, Canada; Departments of Neurology and Neurosurgery, Pediatrics and Human Genetics (G.B.), McGill University; Department Specialized Medicine (G.B.), Division of Medical Genetics, McGill University Health Center; Child Health and Human Development Program (G.B.), Research Institute of the McGill University Health Center; MyeliNeuroGene Laboratory (G.B.), Research Institute of the McGill University Health Center, Montreal, Quebec, Canada; Department of Pediatric Neurology (N.I.W.), Emma Children's Hospital, Amsterdam, Netherlands; Amsterdam Neuroscience (N.I.W.), Vrije Universiteit, Netherlands; and Department of Medical Imaging (E.M.) and Division of Neurology (D.P.), CHEO, University of Ottawa, ON, Canada.

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http://dx.doi.org/10.1212/NXG.0000000000000409DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7164972PMC
April 2020

Cannabis Treatment in Children with Epilepsy: Practices of Canadian Neurologists.

Can J Neurol Sci 2020 07 4;47(4):511-518. Epub 2020 Mar 4.

Division of Neurology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada.

Background: Medical cannabis has recently emerged as a treatment option for children with drug-resistant epilepsy. Despite the fact that many pediatric epilepsy patients across Canada are currently being treated with cannabis, little is known about the attitudes of neurologists toward cannabinoid treatment of children with epilepsy.

Methods: A 21-item online survey was distributed via email to 148 pediatric neurologists working in hospitals and community clinics across Canada. Questions were related to clinical practice and demographics.

Results: This survey achieved a response rate of 38% (56 Canadian neurologists). These neurologists were treating 668 pediatric epilepsy patients with cannabinoids. While 29% of neurologists did not support cannabis treatment in their patients, 34% prescribed cannabis, and 38% referred to another authorizing physician, mostly to community-based non-neurologists. The majority of neurologists considered cannabis for patients with Dravet syndrome (68%) and Lennox-Gastaut syndrome (64%) after an average of three failed anticonvulsants. Twenty-seven percent considered it for patients with idiopathic generalized epilepsy, and 18% for focal epilepsy. No neurologist used cannabis as a first-line treatment. All neurologists had at least one hesitation regarding cannabis treatment in pediatric epilepsy. The most common one was poor evidence (66%), followed by poor quality control (52%) and high cost (50%).

Conclusions: The majority of Canadian pediatric neurologists consider using cannabis as a treatment for epilepsy in children. With many gaps in evidence and high patient-driven demand for cannabis therapy, this survey provides immediate information from the "wisdom of the crowd," to aid neurologists until further evidence is available.
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http://dx.doi.org/10.1017/cjn.2020.50DOI Listing
July 2020

Correction to: Long-Term Effect of Immediate Versus Delayed Fingolimod Treatment in Young Adult Patients with Relapsing-Remitting Multiple Sclerosis: Pooled Analysis from the FREEDOMS/FREEDOMSII Trials.

Neurol Ther 2020 Jun;9(1):193-195

Division of Neurology, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.

Figures 1a, 1b and 3 were published incorrectly in the original version. The corrected images are given as follows.
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http://dx.doi.org/10.1007/s40120-020-00178-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7229072PMC
June 2020

Physically active children with epilepsy have good objective sleep duration and efficiency despite subjective reports of fatigue and sleep problems.

Epilepsy Behav 2020 03 17;104(Pt A):106853. Epub 2020 Jan 17.

Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON, Canada; Division of Neurology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada; Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada. Electronic address:

Objective: The aim of this study was to longitudinally characterize in children with epilepsy the objective and subjective sleep quality and the relationship between increased physical activity and sleep as well as measures of psychosocial well-being.

Methods: Baseline physical activity and sleep were established in children with epilepsy over four weeks, prior to a 12-week exercise intervention (weekly meeting with exercise counselor). Participants continuously wore a wrist pedometer (Fitbit Flex®) to capture daily number of steps, sleep efficiency, and total sleep time. The Early Childhood Epilepsy Severity Scale (E-Chess) assessed baseline epilepsy severity. Subjective sleep quality (Children's Sleep Habits Questionnaire, CSHQ), quality of life (KIDSCREEN-27; Pediatric Quality of Life Inventory, PedsQL™, 4.0 Core), fatigue (PedsQL™ Multidimensional Fatigue Scale), depression (Children's Depression Inventory-Short), and anxiety (Multidimensional Anxiety Scale for Children) were assessed pre- and post-interventions.

Results: Our cohort of 22 children with epilepsy aged 8-14 years was similarly active to peers (11,271 ± 3189 mean steps per day) and displayed normal sleeping patterns (mean sleep efficiency: 87.4% ± 3.08 and mean total sleep time: 521 ± 30.4). Epilepsy severity assessed by E-Chess was low to moderate (median baseline E-Chess score of 6, interquartile range: 5-7). Study outcomes did not change with the intervention. Older children and those with lower baseline activity were more likely to increase their activity during the intervention. Changes in physical activity were not associated with changes in sleep outcomes when accounting for age, sex, and baseline E-Chess score. Subjective sleep quality marginally improved with the intervention (CSHQ total score: 44.5 ± 5.8 at baseline and 41.6 ± 7.2 at the end of study, p = 0.05). Quality of life, fatigue, depression, and anxiety did not change with the intervention (p = 0.55, 0.60, 0.12, and 0.69, respectively).

Significance: Children with epilepsy who are as active as peers without epilepsy have good objective measures of sleep despite self-reported fatigue and parent-reported sleep problems. The physical activity of initially less active and older children with epilepsy may benefit from an exercise counseling intervention.
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http://dx.doi.org/10.1016/j.yebeh.2019.106853DOI Listing
March 2020

No improvement in quality of life in children with epilepsy treated with the low glycemic index diet.

Epilepsy Behav 2020 03 17;104(Pt A):106664. Epub 2020 Jan 17.

Faculty of Medicine, University of Ottawa, Ottawa, ON, Canada; Division of Neurology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, ON, Canada. Electronic address:

Background: Up to 30% of children with epilepsy show a poor therapeutic response to pharmacologic treatment. Ketogenic diets, including the less strict low glycemic index treatment (LGIT), may improve seizure control in pharmacoresistant epilepsy. However, little is known about the quality of life (QoL) in children on LGIT. To explore psychosocial implications of the LGIT on pediatric patients and their caregivers, we have conducted a pilot study to explore the QoL of children and adolescents on the diet.

Methods: Pediatric patients on LGIT and their parents completed standardized, validated QoL questionnaires (Pediatric Quality of Life Epilepsy Module), one retrospectively and one while being on LGIT. An additional questionnaire included two open-ended questions in order to gain a better understanding of personal experiences of families.

Results: We enrolled six patients with epilepsy on LGIT between the age of 3 and 13 years. Self-reported QoL decreased in all adolescents, regardless of improvement in seizure control. Parent-reported QoL improved in three of six participants, remained stable in one, and decreased in two patients (both displayed no seizure improvement). Parents and adolescents reported positive experiences of trying new foods and being more health conscious, as well as negative themes such as social isolation and meal preparation difficulties.

Conclusions: The lack of improvement in patient-reported QoL points towards an overall negative impact of the LGIT on patient well-being, despite positive effects on seizure control. Our preliminary results indicate that the benefits of seizure control may subjectively be outweighed by adverse social effects of the LGIT. Families should be made aware of psychosocial risks of the diet. Whenever possible, children should be part of the therapeutic decision-making process. Larger prospective studies are required to fully assess the overall impact of the LGIT.
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http://dx.doi.org/10.1016/j.yebeh.2019.106664DOI Listing
March 2020

Benign spasms of infancy: a mimicker of infantile epileptic disorders.

Epileptic Disord 2019 Dec;21(6):585-589

University of Ottawa, Faculty of Medicine,, Children's Hospital of Eastern Ontario, Ottawa, Ontario, Canada.

Benign spasms of infancy (BSI), previously described as benign non-epileptic infantile spasms or benign myoclonus of early infancy, are non-epileptic movements manifesting during the first year of life and spontaneously resolving in the second year of life. BSI are characterized by spasms typically lasting 1-2 seconds, involving, to varying degrees, the head, neck, trunk, shoulders and upper extremities. Ictal and interictal EEG recordings are normal. BSI are not associated with developmental regression and do not require treatment. Distinction between BSI and infantile epileptic disorders, such as epileptic spasms or myoclonic epilepsy of infancy, can be challenging given the clinical similarities. Moreover, interictal EEGs can be normal in all conditions. Epileptic spasms and myoclonic epilepsy require timely treatment to improve neurodevelopmental outcomes. We describe a six-month-old infant presenting with spasm-like movements. His paroxysms as well as a positive family history for epileptic spasms were in keeping with a likely diagnosis of West syndrome. Surprisingly, ictal video-EEG did not reveal epileptiform activity, and suggested a diagnosis of BSI. We emphasize that ictal video-EEG is the gold standard for classification of infantile paroxysms as epileptic or non-epileptic, thereby avoiding over-treatment for BSI and facilitating timely targeted treatment of infantile epilepsies. [Published with video sequences].
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http://dx.doi.org/10.1684/epd.2019.1116DOI Listing
December 2019

Long-Term Effect of Immediate Versus Delayed Fingolimod Treatment in Young Adult Patients with Relapsing-Remitting Multiple Sclerosis: Pooled Analysis from the FREEDOMS/FREEDOMS II Trials.

Neurol Ther 2019 Dec 19;8(2):461-475. Epub 2019 Jul 19.

Division of Neurology, Children's Hospital of Eastern Ontario, Ottawa, ON, Canada.

Introduction: Fingolimod has demonstrated clinical and MRI benefits versus placebo/interferon β-1a in young adults with multiple sclerosis (MS). Here we report the long-term effects of fingolimod 0.5 mg on clinical and MRI outcomes in young adults with MS aged ≤ 30 years followed up for up to 8 years (96 months).

Methods: This post hoc analysis of pooled FREEDOMS/FREEDOMS II studies included patients who either received fingolimod 0.5 mg from randomization (immediate; N = 163) or switched from placebo to fingolimod at month (M) 24 (delayed; N = 147). The 6-month confirmed disability improvement [6m-CDI: based on Expanded Disability Status Scale (EDSS)], 6m-CDI-plus (6m-CDI+; EDSS, 9-Hole Peg Test, Timed 25-Foot Walk Test), 6-month confirmed disability progression (6m-CDP), time to EDSS score ≥ 4, annualized relapse rates (ARRs), new/newly enlarging T2 (neT2) lesions, and annual rate of brain volume loss (BVL) were analyzed from baseline to M24, M48, and M96. Cox regression and negative binomial regression models were used to analyze measured outcomes.

Results: At baseline, more than two-thirds of young adult patients were treatment naïve, had more than two relapses in the previous 2 years, and EDSS score < 2. From M0 to M96, a significantly higher proportion of young adult patients in the immediate group (vs. delayed group) achieved 6m-CDI (58.2% vs. 30.5%, p = 0.0206) and 6m-CDI+ (70.6% vs. 42.3%, p = 0.0149); significantly fewer patients reached 6m-CDP (20.1% vs. 34.7%, p = 0.0058) and EDSS ≥ 4 (24.1% vs. 34.1%, p = 0.0041). Up to M96, young adults in the immediate versus delayed group had lower ARRs (0.16 vs. 0.38, p < 0.0001) and a higher proportion of patients were free of neT2 lesions at M48 (31.0% vs. 5.0%, p = 0.0011).

Conclusion: In young adult patients with MS, immediate versus delayed fingolimod treatment was associated with improved disease outcomes and greater long-term benefits in both disease activity and disability progression.

Funding: Novartis Pharma AG.
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http://dx.doi.org/10.1007/s40120-019-0146-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6858894PMC
December 2019

Infantile Idiopathic Intracranial Hypertension: A Case Study and Review of the Literature.

J Child Neurol 2019 11 16;34(13):806-814. Epub 2019 Jul 16.

Division of Neurology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, Ontario, Canada.

Idiopathic intracranial hypertension, or pseudotumor cerebri, is an increase in cerebrospinal fluid pressure of unknown etiology. It is mostly seen in adults, less frequently in adolescents, rarely in younger children. Only 5 infants meeting idiopathic intracranial hypertension criteria have been mentioned in the literature. We report a case of a previously healthy 9-month-old boy who presented with irritability, decreased appetite, and a bulging fontanelle. Computed tomography (CT) head imaging and cerebrospinal fluid studies revealed normal results. The patient's symptoms transiently resolved after the initial lumbar puncture, but 11 days later, his fontanelle bulged again. A second lumbar puncture revealed an elevated opening pressure of 35 cmHO and led to a diagnosis of idiopathic intracranial hypertension in accordance with the modified Dandy Criteria. Treatment with acetazolamide at a dose of 25 mg/kg/d was initiated and the patient remained symptom-free for 6 weeks, followed by another relapse. His acetazolamide dose was increased to 37 mg/kg/d, with no further relapses to date. A diagnosis of idiopathic intracranial hypertension is challenging in infants, because the patients cannot yet verbalize typical idiopathic intracranial hypertension-related symptoms such as positional headaches, diplopia, or pulsatile tinnitus. Furthermore, it is more difficult to assess papilledema in that age group. If undetected and untreated, idiopathic intracranial hypertension may result in permanent visual deficits. Little is known about idiopathic intracranial hypertension in infants, and age-specific treatment guidelines are lacking. We discuss this rare case of infantile idiopathic intracranial hypertension and provide a review of the literature, including an overview of disease characteristics and outcomes of idiopathic intracranial hypertension in this very young age group.
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http://dx.doi.org/10.1177/0883073819860393DOI Listing
November 2019

Continuous Electroencephalography Monitoring for Critically Ill Neonates: A Canadian Perspective.

Can J Neurol Sci 2019 07 29;46(4):394-402. Epub 2019 Apr 29.

Division of Neurology,Children's Hospital of Eastern Ontario,Ottawa, Ontario,Canada.

Background: Continuous EEG monitoring, in the form of amplitude-integrated (aEEG) or conventional EEG (cEEG), is used in the neonatal intensive care unit (NICU) to detect subclinical central nervous system pathologies, inform management, and prognosticate neurodevelopmental outcomes. To learn more about provider attitudes and current practices in Canada, we evaluated neurologist and neonatologist opinions regarding NICU EEG monitoring.

Methods: A 15-item electronic questionnaire was distributed to 114 pediatric neurologists and 176 neonatologists working across 25 sites.

Results: The survey was completed by 87 of 290 physicians. Continuous EEG monitoring is utilized by 97% of pediatric neurologists and 92% of neonatologists. Neurologists and neonatologists differ in their EEG monitoring preferences. For seizure detection and diagnosis of encephalopathy, significantly more neonatologists favor aEEG alone or in combination with cEEG, whereas most neurologists prefer cEEG (p = 0.047, 0.001). There is a significant difference in the perceived gaps in monitoring patients with cEEG between neonatologists (13% would monitor more) and neurologists (41% would monitor more) (p = 0.007). Half of all respondents (53%) reported that they would be interested in attending an education session on neonatal EEG monitoring.

Conclusions: Canadian neurologists and neonatologists do not agree on the best monitoring approach for critically ill neonates. Furthermore, neonatologists perceive a smaller cEEG monitoring gap as compared with neurologists. However, many participants from both specialties would like to increase long-term EEG monitoring in the NICU setting. Facilitating access to EEG monitoring and enhancing education may help to address these needs.
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http://dx.doi.org/10.1017/cjn.2019.36DOI Listing
July 2019

Can behavioral strategies increase physical activity and influence depressive symptoms and quality of life among children with epilepsy? Results of a randomized controlled trial.

Epilepsy Behav 2019 05 30;94:158-166. Epub 2019 Mar 30.

Department of Pediatrics, McMaster University, 1400 Main Street W, Institute for Applied Health Sciences, Room 408, Hamilton, ON L8S 1C7, Canada. Electronic address:

Purpose: This study examined whether increasing physical activity (PA) through 6 months of behavioral counseling positively influenced depressive symptoms and quality of life (QoL) over 12 months among children with epilepsy (CWE).

Methods: A longitudinal multisite randomized controlled trial (RCT) was conducted with 8-14-year-old children with active epilepsy. Participants wore a pedometer to track daily PA and completed 3 measures at 4 time points to examine depressive symptoms and QoL. Stratified by site and activity level, participants were randomized to an intervention or control group. The 6-month intervention included 11 behavioral counseling sessions targeting self-regulation of PA. To assess the associations among PA, depression scores, and QoL, primary analysis involved mixed-effects models.

Results: We recruited 122 CWE, of whom 115 were randomized (M = 11 ± 2; 50% female) and included in the analysis. The intervention did not increase PA in the treatment compared with the control group. No differences were found between groups over time during the subsequent 6 months, where PA decreased among all participants. Results did not show differences between the groups and over time for measures of depressive symptoms and QoL.

Significance: The intervention did not improve or sustain PA levels over 12 months. Both groups demonstrated declines in PA over one year, but there were no changes in depression scores or QoL. As most participants were already nearly reaching the Canadian average of step counts of children their age, with a baseline daily step count of over 9000, there may be a challenge for further increasing PA over a longer period.
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http://dx.doi.org/10.1016/j.yebeh.2019.03.011DOI Listing
May 2019

Oligoclonal bands increase the specificity of MRI criteria to predict multiple sclerosis in children with radiologically isolated syndrome.

Mult Scler J Exp Transl Clin 2019 Jan-Mar;5(1):2055217319836664. Epub 2019 Mar 20.

Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, USA.

Background: Steps towards the development of diagnostic criteria are needed for children with the radiologically isolated syndrome to identify children at risk of clinical demyelination.

Objectives: To evaluate the 2005 and 2016 MAGNIMS magnetic resonance imaging criteria for dissemination in space for multiple sclerosis, both alone and with oligoclonal bands in cerebrospinal fluid added, as predictors of a first clinical event consistent with central nervous system demyelination in children with radiologically isolated syndrome.

Methods: We analysed an international historical cohort of 61 children with radiologically isolated syndrome (≤18 years), defined using the 2010 magnetic resonance imaging dissemination in space criteria (Ped-RIS) who were followed longitudinally (mean 4.2 ± 4.7 years). All index scans also met the 2017 magnetic resonance imaging dissemination in space criteria.

Results: Diagnostic indices (95% confidence intervals) for the 2005 dissemination in space criteria, with and without oligoclonal bands, were: sensitivity 66.7% (38.4-88.2%) versus 72.7% (49.8-89.3%); specificity 83.3% (58.6-96.4%) versus 53.9% (37.2-69.9%). For the 2016 MAGNIMS dissemination in space criteria diagnostic indices were: sensitivity 76.5% (50.1-93.2%) versus 100% (84.6-100%); specificity 72.7% (49.8-89.3%) versus 25.6% (13.0-42.1%).

Conclusions: Oligoclonal bands increased the specificity of magnetic resonance imaging criteria in children with Ped-RIS. Clinicians should consider testing cerebrospinal fluid to improve diagnostic certainty. There is rationale to include cerebrospinal fluid analysis for biomarkers including oligoclonal bands in planned prospective studies to develop optimal diagnostic criteria for radiologically isolated syndrome in children.
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http://dx.doi.org/10.1177/2055217319836664DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6429663PMC
March 2019

Neonatal Bicycling Movements Associated With a Basal Ganglia Stroke.

Mov Disord Clin Pract 2019 Feb 16;6(2):176-178. Epub 2019 Jan 16.

Division of Pediatric Neurology Children's Hospital of Eastern Ontario, University of Ottawa Ottawa Ontario Canada.

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http://dx.doi.org/10.1002/mdc3.12713DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6384175PMC
February 2019

Combined Conventional and Amplitude-Integrated EEG Monitoring in Neonates: A Prospective Study.

J Child Neurol 2019 05 14;34(6):313-320. Epub 2019 Feb 14.

1 Division of Neurology, Children's Hospital of Eastern Ontario, University of Ottawa, Ottawa, Ontario, Canada.

Background/objective: Seizure monitoring via amplitude-integrated EEG is standard of care in many neonatal intensive care units; however, conventional EEG is the gold standard for seizure detection. We compared the diagnostic yield of amplitude-integrated EEG interpreted at the bedside, amplitude-integrated EEG interpreted by an expert, and conventional EEG.

Methods: Neonates requiring seizure monitoring received amplitude-integrated EEG and conventional EEG in parallel. Clinical events and amplitude-integrated EEG were interpreted at bedside. Subsequently, amplitude-integrated EEG and conventional EEG were independently analyzed by experienced neonatology and neurology readers. Sensitivity and specificity of bedside amplitude-integrated EEG as compared to expert amplitude-integrated EEG interpretation and conventional EEG were evaluated.

Results: Thirteen neonates were monitored for an average duration of 33 hours (range 15-94, SD 25). Fourteen seizure-like events were detected by clinical observation, and 12 others by bedside amplitude-integrated EEG analysis. One of the clinical, and none of the bedside amplitude-integrated EEG events were confirmed as seizures on conventional EEG. Post hoc expert amplitude-integrated EEG interpretation revealed eight suspected seizures, all different from the ones detected by the bedside amplitude-integrated EEG team, of which one was confirmed via conventional EEG. Eight seizures were recorded on conventional EEG. Expert amplitude-integrated EEG interpretation had a sensitivity of 13% with 46% specificity for individual seizure detection, and a sensitivity of 50% with 46% specificity for detecting patients with seizures.

Conclusion: Real-world bedside amplitude-integrated EEG monitoring failed to detect all seizures evidenced via conventional EEG, while misclassifying other events as seizures. Even post hoc expert amplitude-integrated EEG interpretation provided limited sensitivity and specificity. Considering the poor sensitivity and specificity of bedside amplitude-integrated EEG interpretation, combined monitoring may provide limited clinical benefit.
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http://dx.doi.org/10.1177/0883073819829256DOI Listing
May 2019

Sensitivity, specificity, and reliability of the Get Active Questionnaire for identifying children with medically necessary special considerations for physical activity.

Appl Physiol Nutr Metab 2019 Jul 30;44(7):736-743. Epub 2018 Nov 30.

a Children's Hospital of Eastern Ontario Research Institute, Ottawa, ON K1H 8L1, Canada.

Physical activity is promoted for optimal health but may carry risks for children who require medically necessary activity restrictions. The sensitivity, specificity, and reliability of the Get Active Questionnaire (GAQ) for identifying children needing special considerations during physical activity was evaluated among parents of 207 children aged 3 to 14 years (97 (47%) female, mean age of 8.4 ± 3.7 years). GAQ responses were compared with reports obtained directly from the treating physician ( = 192/207) and information in the medical chart (clinic notes/physician letter, = 111/207). Parent GAQ responses (either "No to all questions" or "Yes to 1 or more questions") agreed with physician (κ = 0.16, = 0.003) and medical record (κ = 0.15, = 0.003) reports regarding the need for special consideration during physical activity (Yes/No). Sensitivity was 71% (20/28) and specificity was 59% (96/164), with few false-negative responses. The GAQ was most effective for rheumatology and cardiology patients. False positives were 29% to 46%, except among chronic pain (80%) and rehabilitation (75%) patients. Test-retest reliability was moderate (Cronbach's α = 0.70) among 57 parents who repeated the GAQ 1 week later. The GAQ effectively identified children not requiring physical activity restrictions and those with medical conditions similar to those of concern among adults. Additional questions from a qualified exercise professional, as recommended for a "Yes" response on the GAQ, should reduce the false-positive burden. Indicating the timeframe of reference for each question and including an option to describe other special considerations (e.g., medication, supervision) are recommended.
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http://dx.doi.org/10.1139/apnm-2018-0314DOI Listing
July 2019

Relationship Between Physical Activity, Tic Severity and Quality of Life in Children with Tourette Syndrome.

J Can Acad Child Adolesc Psychiatry 2018 Nov 1;27(4):222-227. Epub 2018 Nov 1.

Faculty of Medicine, University of Ottawa, Ottawa, Ontario.

Objective: To examine the relationship between physical activity, tic severity and quality of life (QoL) in children and adolescents with persistent tic disorder and Tourette Syndrome.

Method: Baseline data was examined from a larger randomized controlled trial (Clinicaltrials.gov NCT02153463). Physical activity was assessed via pedometers with daily step count recorded. Tic severity (assessed via Yale Global Tic Severity Scale or YGTSS) and QoL (assessed via PEDs QL 4.0) were compared between those more physically active (≥12,000 steps/day) and less physically active (<12,000 steps/day).

Results: Thirteen children participated; four had ≥12,000 steps/day and nine had <12,000 steps/day. The active group had a lower total tic severity (p = 0.02), and total YGTSS score (p=0.01). The vocal tic severity score was lower in the active group (p=0.02). Motor tic severity was not different amongst the two groups. For Peds QL scores, the active group performed better in physical functioning (p=0.01), social functioning (p=0.03), school functioning (p=0.02), psychosocial functioning (p=0.03) and total PEDs QL score (p=0.01).

Conclusions: Higher physical activity levels are associated with lower vocal tic severity and improved aspects of quality of life. Further research is needed to determine the utility of physical activity as therapy for tics.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6254264PMC
November 2018

Higher screen time, lower muscular endurance, and decreased agility limit the physical literacy of children with epilepsy.

Epilepsy Behav 2019 01 19;90:260-265. Epub 2018 Oct 19.

Children's Hospital of Eastern Ontario Research Institute, 401 Smyth Road, Ottawa, Ontario K1H 8L1, Canada; University of Ottawa, Faculty of Medicine, 401 Smyth Road, Ottawa, Ontario K1H 8L1, Canada.

Objective: The objective of this study was to determine the physical literacy (the motivation, confidence, physical competence, and knowledge contributing to the capacity for physical activity) of children with epilepsy, as compared with that of their healthy peers.

Methods: Patients age 8-12 years with epilepsy, without any disabilities interfering with their ability to answer questionnaires and perform vigorous physical activity, were recruited from the Neurology Clinic at the time of visits. They completed the Canadian Assessment of Physical Literacy (CAPL), a comprehensive battery of tests reflecting the primary domains of physical literacy (motivation/confidence, physical competence, knowledge/understanding, and daily behavior). Daily behavior was assessed by pedometer step counts, as well as self-reported moderate-to-vigorous physical activity and screen time. Physical competence included agility and movement skill measures as well as physical fitness. Children with epilepsy were matched with healthy peers from a large research database of over 6000 Canadian children.

Results: We tested 35 children with epilepsy, divided into those with presumed self-limiting forms of epilepsy (49%) and those with chronic disease (51%). Only a small proportion of participants (23%) were taking more than one antiepileptic medication, and only one patient was taking three anticonvulsants. Children with epilepsy including those with self-limiting forms had significantly lower total physical literacy scores, lower agility and movement skills, and lower muscular endurance, and reported more screen time than their healthy peers. Only 11% of the children with epilepsy achieved the recommended level of physical literacy. However, the children with epilepsy were knowledgeable about and highly motivated to participate in a physically active lifestyle.

Conclusions: Children with epilepsy demonstrate poor physical literacy levels, with potential immediate and long-lasting negative impacts on general health and psychosocial well-being. Programs promoting physical literacy in children with epilepsy should be encouraged, specifically interventions decreasing screen time and enhancing muscular endurance and motor skills, thereby facilitating healthier lifestyles.
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http://dx.doi.org/10.1016/j.yebeh.2018.05.010DOI Listing
January 2019

Health-Related Quality of Life for Patients With Genetically Determined Leukoencephalopathy.

Pediatr Neurol 2018 07 9;84:21-26. Epub 2018 Apr 9.

Department of Medical Genetics, Montreal Children's Hospital, McGill University Health Centre, Montreal, Quebec, Canada; Department of Neurology and Neurosurgery, McGill University, Montreal, Quebec, Canada; Department of Pediatrics, McGill University, Montreal, Quebec, Canada; Child Health and Human Development Program, Research Institute of the McGill University Health Centre, Montreal, Quebec, Canada. Electronic address:

Background: We attempted to characterize the health-related quality of life in patients with genetically determined leukoencephalopathies as it relates to the severity of clinical features and the presence or absence of a precise molecular diagnosis.

Methods: Health-related quality of life was assessed using the Pediatric Quality of Life Inventory model (Pediatric Quality of Life Inventory 4.0 Self- and Proxy-reports) on 59 patients diagnosed with genetically determined leukoencephalopathies. In total, 38 male and 21 female patients ranging from one to 32 years of age (mean nine years), as well as their parents, completed the Pediatric Quality of Life Inventory health-related quality of life measures. In addition, participants completed detailed standardized clinical assessments or questionnaires. The correlation between health-related quality of life results and the severity of the clinical features, as well as the presence or absence of a molecular diagnosis, were analyzed.

Results: Patients with more severe clinical features showed statistically significant lower total Pediatric Quality of Life Inventory scores. More specifically, lower health-related quality of life was noted in children with sialorrhea, gastrostomy, and dystonia and in children who use a wheelchair.

Conclusions: Patients with more severe clinical features experience a lower quality of life. Our study further highlights the importance of addressing both physical and psychosocial issues and discussing perception of quality of life with both parents and children. A larger multicenter prospective study will be needed to further define the burden of these diseases and to identify modifiable factors.
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http://dx.doi.org/10.1016/j.pediatrneurol.2018.03.015DOI Listing
July 2018

Relapse Rate and MRI Activity in Young Adult Patients With Multiple Sclerosis: A Post Hoc Analysis of Phase 3 Fingolimod Trials.

Mult Scler J Exp Transl Clin 2018 Apr-Jun;4(2):2055217318778610. Epub 2018 May 25.

Novartis Pharma AG, Switzerland.

Background: Disease activity differs in young patients with multiple sclerosis (MS) compared with the overall adult MS population.

Objective: The objective of this paper is to evaluate the effect of fingolimod 0.5 mg on disease activity in young adults with MS from three randomized, double-blind Phase 3 trials.

Methods: Annualized relapse rate (ARR), number of new/newly enlarging T2 lesions (neT2), and no evidence of disease activity (NEDA-3) were estimated in the intent-to-treat population at age 20 (youngest) and 30 (young) and compared to the overall population. Models used included a negative binomial regression (ARR/neT2) and a logistic regression (NEDA), with age at baseline as a continuous covariate.

Results: ARRs were higher in younger patients (all  < 0.05), and significantly reduced with fingolimod versus placebo or interferon beta-1a (IFN β-1a), with the percentage reduction inversely proportional to age. Fingolimod was significantly associated with a lower number of neT2 lesions versus placebo/IFN in all age groups except versus IFN in the youngest patients. Regardless of age, fingolimod-treated patients were more likely to achieve NEDA-3 versus placebo/IFN β-1a, with strongest benefits in the youngest patients (all  < 0.05).

Conclusions: Young adults show higher levels of MS disease activity, and may particularly benefit from fingolimod treatment compared with the overall study population.
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http://dx.doi.org/10.1177/2055217318778610DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5971396PMC
May 2018