bluuebunny/crossref_metadata_2025_split huggingface dataset:
MRI of Tibialis Anterior Skeletal Muscle in Myotonic Dystrophy Type 1
Authors: Coté Chantal , Hiba Bassem , Hebert Luc J. , Vial Christophe , Remec Jean François , Janier Marc , Puymirat Jack 
Published: December 2014

Abstract:Objective:The aim of this study was to evaluate whether magnetic resonance imaging (MRI) can be used as a noninvasive approach to assessment of disease severity and muscle damage in Myotonic Dystrophy type 1 (DM1).Methods:The MRI findings in legs of 41 patients with DM1 were evaluated with respect to the tibialis anterior (TA) skeletal muscle impairment. Magnetic resonance imaging findings were compared with TA strength measurements obtained by quantitative manual testing, duration of the disease and with the length of the CTG repeats.Results:Muscle MRI abnormalities were observed in 80% of DM1 patients, ranging from edema-like abnormalities alone to severe atrophy / fatty replacement. Edema-like abnormalities seem to be an earlier MRI marker of the disease. Fatty infiltration/atrophy correlated with the TA muscle force (r = 0.95), the severity (P = 0.00001) of the disease but not with the duration of the disease (P = 0.3) or the length of the CTG repeats (P > 0.10), measured in peripheral leukocytes. Evaluation of other muscles of the legs revealed that the medial gastrocnemius and soleus muscles were the most frequently and severely affected muscles, while tibialis posterior muscles were relatively spared. Edema-like abnormalities are most frequently observed in the skeletal muscles of the anterior compartment.Conclusion:Muscle MRI is helpful to depict muscle abnormalities but does not seem to be a reliable indicator of skeletal muscle involvement in DM1 since the decrease in TAmuscle force is not correlated with MRI abnormalities in some patients.
The Design of the Canadian UnRuptured Endovascular versus Surgery (CURES) Trial
Authors: Darsaut Tim E. , Findlay J. Max , Raymond Jean 
Published: December 2014

Background:Once a decision has been made to treat an intact aneurysm, the best treatment remains uncertain. Both surgical and endovascular management strategies are commonly performed for these lesions. Surgical clipping, for years the standard treatment, is gradually becoming supplanted by endovascular treatment. However, there is no randomized data available to compare the results of surgery versus endovascular treatment of unruptured aneurysms (UIAs).Methods:We report the design of the Canadian UnRuptured Endovascular versus Surgery (CURES) trial to compare angiographic and clinical outcomes following treatment of UIAs.Results:The Canadian pilot phase will serve two purposes: i) to determine feasibility of the pivotal international study, and ii) to determine the incidence of treatment failure, a composite primary end-point comprising the occurrence of either: failure to accomplish aneurysm obliteration with the initial treatment modality, a major saccular aneurysm remnant or recurrence, or intracranial hemorrhage following treatment at one year. The pivotal international study will address which strategy leads to the best overall clinical outcomes in terms of mortality, morbidity, and clinical efficacy. CURES is designed to be a pragmatic management trial with loose inclusion criteria. The pilot study plans to enroll 260 patients, a size sufficient (at 80% power and 0.05 significance) to detect a decrease in the incidence of treatment failure from 13% to 4%. The formulation of specific hypotheses for the pivotal phase awaits the preliminary CURES morbidity and mortality results.Conclusions:The CURES trial intends to test surgical versus endovascular management strategies for the treatment of unruptured intracranial aneurysms.
Web-Based Software to Assist in the Localization of Neuroanatomical Lesions
Authors: Lewis Evan Cole , Strike Melanie , Doja Asif , Ni Andy , Weber Jonathan , Wiper-Bergeron Nadine , Sell Erick 
Published: December 2014

Objective:To evaluate the educational effectiveness of a novel, web-based neuroanatomical localization application.Methods:A prototype version of a neuroanatomical localization application was developed, limited to lesions involving Cranial Nerve (CN) VII. Second year medical students at the University of Ottawa were recruited to participate in the study. Participants were exposed to a didactic teaching session on CN VII anatomy. They were subsequently randomized to two groups - one group was granted access to the localization application (the “intervention group”), while the other group was given a booklet of standard textbook resources (the “control group”). Participants then completed a case-based multiple choice test on localization of neurologic lesions associated with CN VII, followed by a questionnaire regarding the experience.Results:Thirty-nine students volunteered to participate. Twenty were randomized to the intervention group and 19 to the control group. There was a mean test score difference of 1.3 (CI.95 = 0.2, 2.3) that was significantly higher in the intervention group when compared to the control group. Significance was determined by aWilcoxon rank test (p = 0.028). Questionnaire results were similar for both groups, showing an overall favourable evaluation of the localization application.Conclusions:The results support our hypotheses that students using the application would perform better on the multiple choice question (MCQ) test and there would be an overall preference for its use. The demonstrated educational benefit of the application, in addition to the demand for such a resource expressed by the participants, warrant further investigation into the development of a neurological localization application.
Normal-Pressure Hydrocephalus: Is There a Genetic Predisposition?
Authors: Cusimano M. D. , Rewilak D. , Stuss D. T. , Barrera-Martinez J. C. , Salehi F. , Freedman M. 
Published: December 2014

Background:Normal-pressure hydrocephalus (NPH) is characterized by gait disturbance, cognitive impairment, with or without urinary incontinence, enlarged ventricles with or without cerebral atrophy and normal cerebrospinal fluid pressure.Methods:We report two sisters with NPH who lived together their entire lives and whose natural history might provide insights into genetic and environmental mechanisms underlying this disorder. Both patients were in their early seventies, single, had similar daily habits and hypertension. No other family members had NPH.Results:They both underwent shunt placement and showed improvement documented by history and neuropsychological assessment. Both showed a delayed deterioration due to vasculopathy. Both patients were homozygous for the apolipoprotein E (ApoE) e3 allele on chromosome 19. No environmental factors that might have influenced the development of NPH were identified.Conclusion:Our report of two sisters with NPH may indicate the presence of genetic predisposition and further studies involving genetics and environmental factors are necessary to elucidate their role in the pathogenesis of NPH.
The Neuropathies of Waldenström's Macroglobulinemia (WM) and IgM-MGUS
Authors: Klein Christopher J. , Moon Joon-Shik , Mauermann Michelle L. , Zeldenrust Steven R. , Wu Yanhong , Dispenzieri Angela , Dyck Peter J. 
Published: December 2014

Background:Neuropathy is common in Waldenström's macroglobulinemia (WM, an IgM-associated lymphoplasmacytic lymphoma) and in IgM-monoclonal gammopathy of undetermined significance (IgM-MGUS). Paraneoplastic or paraimmune mechanisms are thought to be involved in the pathogenesis of these neuropathies. Attempts at distinguishing WM and IgM-MGUS neuropathies are lacking especially among bone marrow (BM) confirmed patients.Methods:Retrospective analyses were performed on BM confirmed WM (N=30) and IgM-MGUS (N=73) neuropathy patients with neurologic assessments and hematologic features.Results:The presence of anemia and quantity of IgM monoclonal protein were significantly greater in WM. Based on multiple neurologic assessments differences were not found for: 1) length of time from neurologic symptom onset to evaluation; 2) chief complaint of painless loss of feeling in the feet, Romberg's sign and tremor; and 3) clinical motor, sensory and reflex abnormalities. Autonomic testing was normal in both diseases. Using nerve conduction (NCS) criteria for demyelination, 62% of IgM-MGUS and 27% of WM met this criteria (p=0.013). IgM MGUS patients had greater terminal conduction slowing by ulnar residual latency calculation (<0.01). The degree of axonal loss as measured by summated compound muscle action potentials and available nerve biopsy was not significantly different between diseases.Conclusion:Although WM and IgM-MGUS must be distinguished for hematologic prognosis and treatment, clinical neuropathy presentations of WM and IgM-MGUS are similar and likely related to comparable axonal loss in both conditions. Despite these similarities, evidence of demyelination was found by electrophysiologic studies much more commonly in IgM-MGUS. This difference may reflect varied immune mechanism(s) in the two disorders.
Variations in5-HT2AInfluence Spatial Cognitive Abilities and Working Memory
Authors: Gong Pingyuan , Li Jing , Wang Jian , Lei Xu , Chen Dongmei , Zhang Kejin , Zhang Wenjiang , Zhen Anyuan , Gao Xiaocai , Zhang Fuchang 
Published: December 2014

Background:5-hydroxytryptamine receptor 2A (5-HT2A) participates in diverse psychiatric disorders by regulating the activity of serotonin. Some previous studies have also suggested that the receptor is involved in cognitive abilities of disease groups. We hypothesize that some functional genetic variants in5-HT2Ahave certain specific influences on cognitive abilities in a normal population.Method:To confirm this hypothesis, two polymorphisms (rs6313 and rs4941573) in5-HT2Awere selected, and a population-based study was performed in a young healthy Chinese Han cohort.Results:The results indicated that the rs6313 and rs4941573 were associated with touching blocks and mental rotation-3D error ratio in males, and the rs4941573 was associated with visuo-spatial working memory in the whole cohort.Conclusion:All the findings suggest that5-HT2Aparticipates in human spatial cognitive abilities and spatial working memory.
Subcortical Hyperexcitability in Migraineurs: A High-Frequency Oscillation Study
Authors: Lai Kuan-Lin , Liao Kwong-Kum , Fuh Jong-Ling , Wang Shuu-Jiun 
Published: December 2014

Objective:An abnormal central nervous system excitability level was found in patients with migraine. Whether it is hyper- or hypo-excitable is still debated. This study aimed to compare the somatosensory high-frequency oscillations (HFOs), which reflected subcortical excitability (early phase) and intracortical inhibition (late phase), between patients with migraine and control subjects.Methods:HFOs were recorded from C3'-Fz, using a 500-1000 Hz frequency filter after stimulation at right median nerves at the wrists, and divided into early and late phases based on the N20 peak. Fifty-nine untreated patients (n=24 during ictal period; n=35, interictal) and 22 controls finished the study.Results:In early HFOs, patients both during ictal and interictal periods had higher maximal amplitudes (p =0.039) and area-under-curve (p =0.029) than those of the controls. Regarding the late HFOs, there were no significant differences among these groups.Conclusion:Our study suggests a hyper-excitable state in the subcortical regions in patients with migraine both during interictal and ictal periods.
Interprofessional Stroke Rehabilitation for Stroke Survivors Using Home Care
Authors: Markle-Reid Maureen , Orridge Camille , Weir Robin , Browne Gina , Gafni Amiram , Lewis Mary , Walsh Marian , Levy Charissa , Daub Stacey , Brien Heather , Roberts Jacqueline , Thabane Lehana 
Published: December 2014

Objective:To compare a specialized interprofessional team approach to community-based stroke rehabilitation with usual home care for stroke survivors using home care services.Methods:Randomized controlled trial of 101 community-living stroke survivors (<18 months post-stroke) using home care services. Subjects were randomized to intervention (n=52) or control (n=49) groups. The intervention was a 12-month specialized, evidence-based rehabilitation strategy involving an interprofessional team. The primary outcome was change in health-related quality of life and functioning (SF-36) from baseline to 12 months. Secondary outcomes were number of strokes during the 12-month follow-up, and changes in community reintegration (RNLI), perceived social support (PRQ85-Part 2), anxiety and depressive symptoms (Kessler-10), cognitive function (SPMSQ), and costs of use of health services from baseline to 12 months.Results:A total of 82 subjects completed the 12-month follow-up. Compared with the usual care group, stroke survivors in the intervention group showed clinically important (although not statistically significant) greater improvements from baseline in mean SF-36 physical functioning score (5.87, 95% CI -3.98 to 15.7; p=0.24) and social functioning score (9.03, CI-7.50 to 25.6; p=0.28). The groups did not differ for any of the secondary effectiveness outcomes. There was a higher total per-person costs of use of health services in the intervention group compared to usual home care although the difference was not statistically significant (p = 0.76).Conclusions:A 12-month specialized, interprofessional team is a feasible and acceptable approach to community-based stroke rehabilitation that produced greater improvements in quality of life compared to usual home care. Clinicaltrials.gov identifier: NCT00463229
Pediatric Traumatic Retroclival Epidural Hematoma
Authors: McDougall Cameron M. , Sankar Tejas , Mehta Vivek , Pugh Jeffrey A. 
Published: December 2014

A previously healthy ten-year-old girl was involved in a high speed motor vehicle accident (MVA) in which she was the belted front-seat passenger. The patient's teeth marks on the dashboard were evidence of a high-velocity impact. Her Glasgow Coma Score on scene was 13, and she was amnestic for the event. She was subsequently transported to the emergency room in full spinal precautions; on arrival she was hemodynamically stable with a Glasgow Coma Score of 14, complaining of neck and jaw pain. Neurological examination revealed impaired abduction of the right eye, with no other focal deficits. She had oral lacerations and bruising over the mandibles bilaterally.Computed tomogram (CT) scan of the head was initially interpreted as normal, but on closer inspection demonstrated a 4mm retroclival hematoma (Figure 1). Sagittal CT through the upper cervical spine revealed the hematoma was epidural, bounded by the spheno-occipital synchondrosis superiorly and by the mid-body of the axis inferiorly (Figure 2). The odontoid was retroflexed (Figure 2). Bilateral mandibular fractures were also uncovered on facial imaging.
Anatomical Variants of the Circle of Willis and Brain Lesions in Migraineurs
Authors: Cavestro C. , Richetta L. , L'Episcopo M. R. , Pedemonte E. , Duca S. , Di Pietrantonj C. 
Published: December 2014

Background:Some reports demonstrated vascular alterations in brain magnetic resonance imaging (MRI) in migraineurs and a relationship between circle of Willis (Circle) variants and lacunar brain infarcts. We examined anomalies of the whole circle of Willis and their relationship with vascular brain lesions in migraineurs, to identify any possible vascular mechanism in migraine.Methods:We studied, with a cohort controlled study, the circle of Willis in migraineurs seen consecutively in our Headache Center, and in non-headache controls, using angio-MRI of the brain. Statistical analysis used ANOVA, Scheffè's criterion, t-student test.Results:We recruited 270 migraineurs (204 without aura (MWOA), 66 with aura (MWA) and 159 controls. Migraineurs presented an anatomical variant in 108 (40%) cases with 34 controls (21.4%) presenting a variant. We found a significant association between MWOA and variants (OR=2.4 CI95% [1.5 to 3.9]) and between MWA and variants (OR=3.2 CI95% [1.6 to 4.1]). Unilateral posterior variants with basilar hypoplasia are statistically associated only with MWA compared to controls (OR=9.2, CI95% [2.3 to 37.2]). Thirty-three percent of MWOA and 24% of MWA sufferers present some kind of brain lesion, included 2% of infra-tentorial lesions. We did not find any statistical association between the presence of Circle variants and ischemic lesions on MRI (OR=1.5 CI95% [0.68; 1.94]), or with infratentorial lacunar lesions (OR=1.58 CI95% [0.48 to 5.24]).Conclusions:Anatomical variants of the Circle of Willis are significantly more frequent in migraineurs; posterior anomalies are more frequent in MWA, suggesting a vascular mechanism provoking changes in cerebral blood flow, thereby stimulating cortical spreading depression.
Meta-Analysis of Aldehyde Dehydrogenase 2 Gene Polymorphism and Alzheimer's Disease in East Asians
Authors: Hao Pan-Pan , Chen Yu-Guo , Wang Jia-Li , Wang Xing Li , Zhang Yun 
Published: December 2014

Background:The association of genetic polymorphism of mitochondrial aldehyde dehydrogenase 2 (ALDH2) and Alzheimer's disease (AD) has been controversial and has been investigated only in several small-sample studies. In the present study, we performed a meta-analysis to evaluate the cross-sectional association ofALDH2variants and AD risk in East Asian populations.Methods:Trials were retrieved through MEDLINE, EMBASE, J-STAGE and the China National Knowledge Internet databases (from January 1, 1994 to November 1, 2010) without any restriction on language. Data were abstracted by a standardized protocol.Results:We found four studies of 821AD patients and 1380 healthy controls that qualified for the analysis. The variantALDH2genotype GA/AA was not associated with increased AD risk (odds ratio (OR) = 1.35; 95% confidence interval (CI) = 0.75-2.42; p = 0.32), even after stratification for the status of apolipoprotein E epsilon 4 allele. However, in the subgroup analyses, the association was significant for men (OR = 1.72; 95% CI = 1.10-2.67; p = 0.02).Conclusions:This study adds to the evidence thatALDH2GA/AA genotype increases the risk of AD among East Asian men, although the effect size is moderate.
ARAC - The Montreal Jewish General Hospital Alzheimer Risk Assessment Clinic
Authors: Schipper Hyman M. , Liberman Adrienne , Kelner Nora , Babins Lennie , Fried Lynda , Bilbul Melanie , Goodman Rachel 
Published: December 2014

Abstract:Introduction:In parallel with robust efforts world-wide to develop effective neuroprotection for established disease, resources are being mobilized to delineate risk factors and implement preventive measures in a concerted effort to forestall the anticipated Alzheimer disease (AD) epidemic. A review of heritable and ‘acquired’ dementia risk factors, many operating at midlife, is presented in a companion paper.Objectives:In 2009, an Alzheimer Risk Assessment Clinic (ARAC) was established at the Jewish General Hospital (Montreal) to address the concerns increasingly being voiced by active middle-aged individuals at risk for AD. A positive family history of AD and/or perceived changes in personal cognitive function (predominantly short-term memory) are main reasons for referral. The primary objectives of ARAC are to (i) ascertain, inform and mitigate the risks of developing AD in cognitively-healthy persons aged 40-65 based on best available medical and epidemiological evidence, (ii) conduct scientific research on midlife dementia risk and prevention in this population and (iii) provide instruction in dementia risk assessment and management to health professionals, clinical/research fellows, medical residents and students. ARAC infrastructure, evaluation protocol, risk profile classification scheme, interventions, knowledge dissemination program, case vignettes, and seminal research projects are described.Conclusions:It is hoped that ARAC and similar initiatives will help prevent or delay dementia by innovating effective interventions based on increasingly nuanced estimation of modifiable AD risk in presymptomatic persons.
Primary or Secondary Decompressive Craniectomy: Different Indication and Outcome
Authors: Al-Jishi Ahmed , Saluja Rajeet Singh , Al-Jehani Hosam , Lamoureux Julie , Maleki Mohammad , Marcoux Judith 
Published: December 2014

Abstract:Background:Intracranial hypertension can cause secondary damage after a traumatic brain injury. Aggressive medical management might not be sufficient to alleviate the increasing intracranial pressure (ICP), and decompressive craniectomy (DC) can be considered. Decompressive craniectomy can be divided into categories, according to the timing and rationale for performing the procedure: primary (done at the time of mass lesion evacuation) and secondary craniectomy (done to treat refractory ICP). Most studies analyze primary and secondary DC together. Our hypothesis is that these two groups are distinct and the aim of this retrospective study is to evaluate the differences in order to better predict outcome after DC.Methods:Seventy patients had DC over a period of four years at our center. They were divided into two groups based on the timing of the DC. Primary DC (44 patients) was done within 24 hours of the injury for mass lesion evacuation. Secondary DC (26 patients) was done after 24 hours and purely for the treatment of refractory ICP. Pre-op characteristics and post-op outcomes were compared between the two groups.Results:There was a significant difference in the mechanism of injury, the pupil abnormalities and Marshall grade between primary and secondary DC. There was also a significant difference in outcome with primary DC showing 45.5% good outcome and 40.9% mortality and secondary DC showing 73.1% good outcome and 15.4% mortality.Conclusions:Primary and secondary DC have different indications and patients characteristics. Outcome prediction following DC should be adjusted according to the surgical indication.
2-Methoxyestradiol Attenuates Autophagy Activation After Global Ischemia
Authors: Xin Xiao-Yu , Pan Jing , Wang Xiao-Qiang , Ma Jian-Fang , Ding Jian-Qing , Yang Guo-Yuan , Chen Sheng-Di 
Published: December 2014

Background:Hypoxia inducible factor 1 (HIF-1) is a key transcriptional factor activated during cerebral ischemia, which regulates a great number of downstream genes, including those associated with cell death. In the present study, we aimed to test the hypothesis that post-ischemic HIF-1α up-regulation might promote autophagy activation; thereby, HIF-1α inhibitor 2ME2 might prevent neurons from ischemic injury through inhibiting autophagy.Methods:Global ischemia was induced using the four-vessel occlusion model (4-VO) in Sprague-Dawley rats (male, 250-280g). 2-Methoxyestradiol (2ME2, 5mg/kg, i.p.) was administrated to down-regulate HIF-1α expression. Post-ischemic beclin-1 and LC3 protein expression was determined at different time points through Western blot assay. Neuronal injury was determined by cresyl violet staining and TUNEL staining in coronal histological sections.Results:The expression of beclin-1 and the ratio of LC3-II/LC3-I increased significantly at 12 and 24 h after ischemia. 2ME2 could remarkably inhibit the up-regulation of beclin-1 and the increase of LC3-II/LC3-I ratio during reperfusion. Moreover, 2ME2 and 3-MA exhibited powerful protective effects against ischemic/reperfusion induced neuronal injury.Conclusions:This study confirmed that autophagy participated in post-ischemic neuronal injury. 2ME2, a HIF-1α inhibitor, might significantly decrease autophagy activation after cerebral ischemia and relieve post-ischemic neuronal injury. Our findings demonstrate that autophagy could be a potential target for neuronal protection after cerebral ischemia.