bluuebunny/crossref_metadata_2025_split huggingface dataset:
E-Selectin Expression Increased in Human Ruptured Cerebral Aneurysm Tissues
Authors: Jia Wenqing , Wang Rong , Zhao Jizong , Liu Isabelle Yisha , Zhang Dong , Wang Xuejiang , Han Xiaodi 
Published: December 2014

Objectives:The purpose of the present study is to investigate the expression of inflammation factor endothelial-leukocyte adhesion molecule (E-selectin, CD62E) in cerebral aneurysm walls and its relationship with aneurysm rupture.Methods:Cerebral aneurysm tissue samples were collected at the time of surgical clipping of nine patients with history of subarachnoid hemorrhage, and then compared with control artery tissues from the superficial temporal arteries (STA) of five patients with intracranial tumors. Immunohistochemistry (IHC) was performed to reveal and localize E-selectin expression in the aneurysms and artery tissues. Western blot analysis was used to relatively quantify the level of E-selectine protein expression in cerebral aneurysms when compared with normal arteries.Results:E-selectin was detected in the wall of all the aneurysm tissue samples and was rarely found in normal control arteries by IHC, and it was concentrated in proliferating and disorganized epithelia cells. Moreover, with the Western blot method, the E-selectin protein level increased significantly in aneurysm tissues compared to normal STA.Conclusions:E-selectin might be an important factor involved in the process of cerebral aneurysm formation and rupture, by promoting inflammation and weakening cerebral artery walls.
TGF-beta 1 Codon 10 Polymorphism is Associated with Cerebral SVD
Authors: Tao Hong-miao , Chen Guo-zhong , Lu Xiao-dong , Hu Xiao-gang , Chen Gan-ping , Shao Bei 
Published: December 2014

Background:To clarify the role of inflammation in the pathogenesis of cerebral small vessel disease (SVD), we investigated whether the gene encoding transforming growth factor-beta 1(TGF-beta 1) is a risk factor for cerebral SVD as a whole, and for two different SVD subtypes.Methods:TGF-beta 1 codon10 (T+29C) genotype was determined in 441 Chinese patients (313 male and 128 female) with cerebral SVD and 450 control subjects (326 male and 124 female). Cerebral SVD patients were retrospectively classified into two groups based on neuroimaging findings: lacunar infarction group with 112 patients and ischaemic leukoaraiosis group with 329 patients.Results:Subjects carrying TT homozygote were susceptible to cerebral SVD [adjusted odds ratio (OR) =1.44, 95% confidence interval (CI), 1.05-1.98; P=0.026]. Further analysis of SVD subtypes revealed a moderate association with the ischaemic leukoaraiosis group [OR= 1.60, 95% CI, 1.14-2.25; P=0.007].Conclusions:Codon 10 of TGF-beta 1 might be a risk factor for SVD, specifically in ischaemic leukoaraiosis phenotype.
Elevated Free Fatty Acid is Associated with Cardioembolic Stroke Subtype
Authors: Seo Woo-Keun , Kim Juyeon , Kim Yoo Hwan , Kim Ji Hyun , Oh Kyungmi , Koh Seong-Beom , Seo Hong Seok 
Published: December 2014

Background and Objectives:Free fatty acids (FFAs), an important energy substrate, have an association with cardiovascular diseases, such as atherosclerosis, myocardial dysfunction and abnormal cardiac rhythm. However, limited reports are available on the association between FFAs and ischemic stroke. We hypothesized that plasma FFA concentration could be associated with an ischemic stroke, emphasizing the relationship between FFA and subtypes of ischemic stroke.Methods:A cross-sectional study examined the association between FFA concentration and subtypes of stroke and cerebral atherosclerosis from a hospital-based acute stroke registry.Results:Data of 715 stroke patients were analyzed. The concentration of FFA was highest in the cardioembolic stroke subtype compared with the other stroke subtypes. Logistic regression analysis revealed that an increase in FFA concentration was significantly associated with the cardioembolic subtype after the adjustment of covariates. FFA concentration was also higher in patients with atrial fibrillation (AF) than those without AF. According to the presence of atherosclerotic stenosis, no significantly difference of FFA concentration was found for intracranial and extracranial cerebral arterial atherosclerosis.Conclusion:Here we report a significant association between fasting FFA concentration and the cardioembolic stroke subtype. AF is suggested as the mediating factor between FFA and the cardioembolic stroke subtype.
Biomarkers of Oxidative Stress in Vascular Dementia Patients
Authors: Shi Guang-Xia , Liu Cun-Zhi , Wang Lin-Peng , Guan Li-Ping , Li Si-Qian 
Published: December 2014

Objective:Little is known about the role of oxidative stress in the pathogenesis of vascular dementia (VaD). The aim of this study was to investigate the biomarkers of oxidative stress in urine, as reflected by 8-hydroxydeoxyguanosine (8-OHdG), 8-isoprostaglandin F2a (8-isoPGF2a) and nitrotyrosine (NT) levels, in a group of well characterized VaD patients and in two control groups of Vascular Not Demented (VaND) patients and healthy subjects.Methods:Ninety-six subjects from the Tianjin municipality in China were recruited. Forty-six patients were in the VaD group, 24 patients with VaND and 26 persons with no signs of cognitive disorder were employed as control groups. Urinary 8-OHdG and 8-isoPGF2a was performed using enzyme-linked immunosorbent assay (ELISA), and urinary NT levels were measured by chemiluminescence detection.Results:Significantly higher urinary 8-OHdG levels were detected in VaD patients compared to VaND patients and healthy control subjects. In contrast, urinary 8-isoPGF2a levels were significantly lower in VaD patients compared with two control groups. For NT levels, no statistically significant differences were observed among the three groups.Conclusion:Increased urinary 8-OHdG level was a potential marker of oxidative stress in VaD patients. Furthermore, it is also important to take into account potential confounders in order to improve the identification of changes in the status of oxidative stress as related to VaD.
Ethical Challenges with Awake Craniotomy for Tumor
Authors: Kirsch Brandon , Bernstein Mark 
Published: December 2014

Background:Awake brain surgery is useful for the treatment of a number of conditions such as epilepsy and brain tumor, as well as in functional neurosurgery. Several studies have been published regarding clinical results and outcomes of patients who have undergone awake craniotomy but few have dealt with related ethical issues.Objective:The authors undertake to explore broadly the ethical issues surrounding awake brain surgery for tumor resection to encourage further consideration and discussion.Methods:Based on a review of the literature related to awake craniotomy and in part from the personal experience of the senior author, we conducted an assessment of the ethical issues associated with awake brain tumor surgery.Results:The major ethical issues identified relate to: (1) lack of data; (2) utilization; (3) conflict of interest; (4) informed consent; (5) surgical innovation; and (6) surgical training.Conclusion:The authors respectfully suggest that the selection of patients for awake craniotomy needs to be monitored according to more consistent, objective standards in order to avoid conflicts of interest and potential harm to patients.
Improving the Neurological Exam Skills of Medical Students
Authors: Moore Fraser G. A. , Chalk Colin 
Published: December 2014

Objective:Determine if distributed practice of neurological exam (NE) skills in first year medical school produces sustained improvements in the skills of second year students.Methods:A prospective, controlled, non-blinded study conducted at McGill University (class size = 180 students). Expanded teaching of muscle stretch reflexes was provided to first year medical students. A structured examination of muscle stretch reflexes (max score = 100) was administered in second year medical school after a required two week rotation in Neurology. Results for class A (received the intervention in first year) were compared to the results for the preceding class B (had not received the intervention).Results:77 of 177 (44%) eligible students in class A and 69 of 166 (42%) eligible students in class B participated. Results were analyzed separately for each of two examiners. Mean (SD) scores were 95.2 (5.6) for class A (intervention) and 81.7 (11.1) for class B (control) for the first examiner and 90.4 (8.2) for class A and 83.8 (11.7) for class B for the second examiner. Results were statistically significant (Mann-Whitney test z = 5.27, p < 0.0001 first examiner and z = 2.67, p < 0.0038 second examiner).Conclusions:Distributed practice of muscle stretch reflexes during first year medical school results in improved performance by second year medical students after their mandatory clinical rotation in neurology, even when examined up to 14 months after the intervention. This finding has implications for the teaching of the NE.
Genetic Testing of Epileptic Encephalopathies of Infancy: An Approach
Authors: Sharma Suvasini , Prasad Asuri N. 
Published: December 2014

Abstract:The epileptic encephalopathies of infancy are a group of disorders characterized by intractable seizures, persistent abnormality of cortical function documented on EEG, and consequently impaired neuro-developmental outcomes. The etiologies vary and include; structural brain malformations, acquired brain insults, and inborn errors of metabolism in the majority of the affected patients. In a proportion of these cases no obvious etiology is identifiable on investigation. Recent advances in molecular diagnostics have led to the discovery of a number of gene defects that may be causal in many epileptic encephalopathies. Identification of the causative mutation is important for prognostic and genetic counseling, and may also carry treatment implications. The recently described genes include; Cyclin-Dependent Kinase-Like 5 gene (CDKL5), Protocadherin 19 (PCDH19), Sodium channel neuronal type 1a subunit gene (SCN1A), Aristaless-Related Homeobox Gene (ARX), and Syntaxin binding protein 1 gene (STXBP1), amongst others. Distinct electro-clinical syndromes are increasingly being identified amongst patients carrying the various mutations. In this review, we outline the approach to clinical evaluation and genetic testing of epileptic encephalopathies in infancy.
When Dementia is in the House: Needs Assessment Survey for Young Caregivers
Authors: Nichols Katherine R. , Fam David , Cook Cheryl , Pearce Michelle , Elliot Gail , Baago Sylvia , Rockwood Kenneth , Chow Tiffany W. 
Published: December 2014

Abstract:Objective:To learn more about the needs and experiences of young carers for patients of frontotemporal dementia (FTD) in order to create a relevant support website for young caregivers to dementia patients.Methods:Two focus groups were held with a total of fourteen young carers aged 11-18. The data corpus was collected through a semi-structured interview facilitated by a medical journalist who had prior experience as a caregiver to a patient with FTD. The transcripts were narrowed to a dataset for descriptive analysis using a coding scheme to reveal the main themes of their responses.Results:Seven overlapping theme areas were: emotional impact of living with a parent with FTD, caregiving, coping, symptoms, diagnosis, relationships, and support. Based on the participants' responses, a website was launched providing supportive information and counsel for young carers.Conclusion:Young carers saw the experience of caring for a parent with early-onset dementia as positive overall, but identified opportunities for professionals to assist them in overcoming stigma and the challenge of balancing childhood and adolescent development within this context.
Establishing a Canadian Registry of Patients with Amyotrophic Lateral Sclerosis
Authors: Korngut L. , Genge A. , Johnston M. , Benstead T. , Bourque P. , Briemberg H. , Casey A. , D'Amour M. , Dupré N. , Figlewicz D. , Hader W. , Johnston W. , Kalra S. , Melanson M. , O'Connell C. , Rouleau G. , Shoesmith C. , Wee J. , Zinman L. 
Published: December 2014

Abstract:Background:Amyotrophic lateral sclerosis (ALS) is a devastating cause of progressive weakness, respiratory failure and death. To date there is no effective therapy to meaningfully extend survival but continuously emerging targets and putative treatments are studied in clinical trials. Canadian epidemiological data on ALS is scarce and the socioeconomic impact of ALS on Canadian society is unclear. The Canadian Neuromuscular Disease Registry (CNDR) is a national clinic-based registry of patients with neuromuscular diseases with the goal of facilitating the design and execution of clinical research.Methods:We conducted a national stakeholder survey to assess interest for a Canadian ALS registry and an assessment of expected case ascertainment. A dataset derivation meeting was held to establish the registry medical dataset.Results:We report the results of the national stakeholder survey, case ascertainment assessment, and the derived dataset that have resulted in the current implementation of a Canadian registry of patients with ALS.Conclusions:The development of this long sought-after resource is a significant step forward for the Canadian ALS patient and research communities that will result in more efficient clinical trial recruitment and advancements in our understanding of ALS in Canada.
Prevalence of Lifestyle Risk Factors in Myotonic Dystrophy Type 1
Authors: Gagnon Cynthia , Chouinard Maud-Christine , Laberge Luc , Brisson Diane , Gaudet Daniel , Lavoie Mélissa , Leclerc Nadine , Mathieu Jean 
Published: December 2014

Abstract:Background:The prevalence of unhealthy lifestyle habits such as smoking has seldom been described in neuromuscular disorders, including myotonic dystrophy type 1 (DM1). However, it is essential to document the unhealthy lifestyle habits as they can exacerbate existing impairments and disabilities. The objectives are: 1) To determine the prevalence of risk factors among individuals with DM1; 2) To compare the prevalence among classic and mild phenotypes.Method:A survey was done on a sample of two-hundred (200) patients with DM1 as part of a larger study. Lifestyle risk factors included being overweight or obese, tobacco smoking, illicit drug use, excessive alcohol consumption and physical inactivity. A registered nurse administered the validated public health survey. Categorization of risk factors were based on national standards and compared with provincial and regional prevalences.Results:50% of DM1 patients were overweight or obese, 23.6% were regular smokers, and 76% were physically inactive. Except for overweight and obesity, significant differences were observed between patients with classic and mild phenotypes for all the other lifestyle risk factors: those with the classic phenotype being more often regular smokers, consuming more often illicit drugs and being less physically active.Conclusions:The results of this study will provide guidance for the development of better adapted and focussed health promotion interventions in the future.
Diversity of ARSACS Mutations in French-Canadians
Authors: Thiffault I. , Dicaire M.J. , Tetreault M. , Huang K.N. , Demers-Lamarche J. , Bernard G. , Duquette A. , Larivière R. , Gehring K. , Montpetit A. , McPherson P.S. , Richter A. , Montermini L. , Mercier J. , Mitchell G.A. , Dupré N. , Prévost C. , Bouchard J.P. , Mathieu J. , Brais B. 
Published: December 2014

Abstract:Background:The growing number of spastic ataxia of Charlevoix-Saguenay (SACS) gene mutations reported worldwide has broadened the clinical phenotype of autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). The identification of Quebec ARSACS cases without two knownSACSmutation led to the development of a multi-modal genomic strategy to uncover mutations in this large gene and explore phenotype variability.Methods:Search forSACSmutations by combining various methods on 20 cases with a classical French-Canadian ARSACS phenotype without two mutations and a group of 104 sporadic or recessive spastic ataxia cases of unknown cause. Western blot on lymphoblast protein from cases with different genotypes was probed to establish if they still expressed sacsin.Results:A total of 12 mutations, including 7 novels, were uncovered in Quebec ARSACS cases. The screening of 104 spastic ataxia cases of unknown cause for 98SACSmutations did not uncover carriers of two mutations. Compounds heterozygotes for one missenseSACSmutation were found to minimally express sacsin.Conclusions:The large number ofSACSmutations present even in Quebec suggests that the size of the gene alone may explain the great genotypic diversity. This study does not support an expanding ARSACS phenotype in the French-Canadian population. Most mutations lead to loss of function, though phenotypic variability in other populations may reflect partial loss of function with preservation of some sacsin expression. Our results also highlight the challenge ofSACSmutation screening and the necessity to develop new generation sequencing methods to ensure low cost complete gene sequencing.
Association of Serum Bilirubin with Stroke Severity and Clinical Outcomes
Authors: Xu Tian , Zhang Jintao , Xu Tan , Liu Wenqing , Kong Yan , Zhang Yonghong 
Published: December 2014

Objective:The aim of the study is to explore the association of serum bilirubin levels with admission severity and short term clinical outcomes among acute ischemic stroke patients.Methods:Data were collected from 2361 acute ischemic stroke patients in four hospitals of Shangdong Province during January 2006 and December 2008. National Institutes of Health Stroke Scale (NIHSS) was used to assess admission and discharge severity. NIHSS≥10 at discharge or in-hospital death was defined as short-term clinical outcomes. Logistic regression and trend test were used to examine the association of serum bilirubin levels with admission severity and short term clinical outcomes.Results:Serum bilirubin levels were significantly and positively associated with admission severity (P for trend <0.05). The age-sex adjusted odds ratios (95% confidential intervals) of NIHSS≥10 associated with the second, third and fourth quartile of total bilirubin/direct bilirubin were 1.245 (0.873, 1.777)/1.276 (0.895, 1.818), 1.484 (1.048, 2.102)/1.628 (1.158, 2.289) and 2.869 (2.076, 3.966)/2.765 (1.996, 3.828), respectively, compared with the lowest quartile; the multivariate adjusted odds ratios of NIHSS≥10 associated with the second, third and fourth quartile of total bilirubin/direct bilirubin were 1.088(0.711, 1.665)/1.436(0.94, 2.193), 1.328(0.877, 2.011)/1.647(1.092, 2.485) and 2.336(1.579, 3.458)/3.079 (2.049, 4.623), respectively, compared with the lowest quartile. However, no association between serum bilirubin levels and short-term clinical outcomes was observed in our study.Conclusion:Serum bilirubin levels were associated with initial stroke severity closely. Nevertheless, there is no significant relationship between serum bilirubin levels and short-term clinical outcomes among acute ischemic stroke patients.
Iron Deficiency Anemia Prevalence at First Stroke or Transient Ischemic Attack
Authors: Dubyk Melanie D. , Card Robert T. , Whiting Susan J. , Boyle Carol A. J. , Zlotkin Stanley H. , Paterson Phyllis G. 
Published: December 2014

Background:Iron deficiency anemia (IDA) has been implicated in the etiology of transient ischemic attack and ischemic stroke. This study aimed to: 1) document IDA prevalence in patients ≥ 65 years of age admitted to hospital with transient ischemic attack or first ischemic stroke, and 2) investigate dietary intake as a predictor of iron status.Methods:Ninety-four patients were enrolled. An algorithm containing values for hemoglobin, ferritin, total iron binding capacity, transferrin saturation, and serum transferrin receptor measured at admission was used to identify IDA. Usual dietary intake was assessed with the Clue II food frequency questionnaire.Results:Prevalence estimates were 6.4% for IDA, 2.1% for iron deficiency without anemia, and 6.4% for anemia from other causes. IDA prevalence was significantly higher than published National Health and Nutrition Examination Survey III (NHANES III) estimates for gender-specific age groups ≥ 70 years (One-Sample Proportion Test; males p = 0.038 [n= 37]; females p = 0.002 [n=44]). A comparison of IDA prevalence against selected controls from the NHANES III database yielded an odds ratio (OR) of 6.3, 95% confidence interval (CI) 0.8 to 53.7, which was not statistically significant (Fisher's Exact Test; n=94; p = 0.118). Multivariate linear regression analysis of dietary intake with indicators of iron status (n=58) revealed only iron supplements (p = 0.013) and heme iron intake (p = 0.038) as negative predictors of total iron binding capacity (p<0.05).Conclusions:These findings support the initiation of a prospective case control study to investigate IDA as a risk factor for ischemic stroke in elderly patients.
Treatment Comfort of Adult Neurologists in Childhood Onset Conditions
Authors: Oskoui M. , Wolfson C. 
Published: December 2014

Background:The majority of children with chronic neurodevelopmental disabilities are surviving to adulthood. Our goal was to assess how prepared and comfortable adult neurologists are in treating young adults with childhood onset chronic neurological conditions and evaluate the difficulty pediatric neurologists experience when transferring these patients to adult care.Methods:We conducted a cross-sectional study using two postal surveys of all pediatric and adult neurologists in the province of Quebec, Canada.Results:The response rate was 51.5%, with 119 neurologists completing the survey. Half of neurologists agreed that adult neurologists may not have adequate training in childhood onset disorders to prepare them to manage the disorders in adulthood, and 60% of pediatric neurologists reported having difficulty finding an adult provider for their patients. Adult neurologists were least comfortable treating patients with autism, chromosomal or metabolic disorders, and cognitive or behavioral disorders.Conclusion:Almost half of those surveyed believed that adult neurologists are not adequately trained to care for this growing patient population. Improving treatment comfort and knowledge among adult neurologists in childhood onset chronic neurological conditions may smooth the transition of these young adults from pediatric to adult care.