bluuebunny/crossref_metadata_2025_split huggingface dataset:
Aspirin Treatment Increases the Risk of Cerebral Microbleeds
Authors: Ge Lihong , Niu Guangming , Han Xiaodong , Gao Yang , Wu Qiong , Wu Hui , Zhang Ying , Guo Dongling 
Published: December 2014

Background:The objective of the study was to determine the frequency of cerebral microbleeds (CMBs) by using phasesensitive imaging in patients with previous transient ischemic attack (TIA) or stroke who were receiving aspirin treatment.Methods:We retrospectively analyzed 300 outpatients with ischemic cerebrovascular disease: 150 had been receiving aspirin treatment for >1 year (patients), and 150 controls had not previously received aspirin. Cerebral microbleeds were defined by a trained observer (blinded to clinical details) according to results of T2-weighted, T1-weighted, diffusion-weighted, and phase-sensitive magnetic resonance imaging (MRI). Numerous vascular risk factors including white matter hyperintensity (WMH), duration of aspirin treatment, age, hypertension or diabetes mellitus were investigated for a possible association with the presence of CMBs in the two groups.Results:The frequency of CMBs (60/150 (40%) vs 18/150 (12%); odds ratio 4.899, p <0.0001) and intracerebral hemorrhage (ICH)(42/150 (28%) vs 2/150 (1%); odds ratio 28.778, p <0.0001) were significantly higher in the patients than in the controls. Among patients, those using aspirin for >5 years(42/68 (62%) showed a higher frequency of CMBs than those receiving aspirin for ≤ 5 years(18/82 (22%); odds ratio 5.744, p<0.0001). WMH (p=0.020/0.030, 0.007/0.000) age (p=0.007/0.000) and hypertension (p=0.000/0.033), in patients and controls respectively, were each associated with CMBs.Conclusions:There was a clear impact of aspirin treatment on CMBs associated with intracerebral hemorrhage in Chinese patients. The frequency of CMBs and hemorrhagic complications was higher in patients treated with long-term aspirin.
A Critical Look at Phenytoin Use for Early Post-Traumatic Seizure Prophylaxis
Authors: Debenham Sierra , Sabit Behzad , Saluja Rajeet S. , Lamoureux Julie , Bajsarowicz Paul , Maleki Mohammad , Marcoux Judith 
Published: December 2014

Backround:The American Academy of Neurology recommended using phenytoin or carbamazepine to prevent early post-traumatic seizures (PTS) in severe traumatic brain injuries (TBI). In this study, we examined the effects of using phenytoin prophylaxis on mild, moderate, and severe TBIs. There have been no studies looking at compliance rate and side effects of systematic use of phenytoin at a large population scale. The goal of this study is to determine 1) the proportion of TBI patients receiving phenytoin prophylaxis; 2) which parameters decided when to decide administer phenytoin; 3) prophylaxis efficacy and complication rate.Methods:We retrospectively studied all patients admitted with a TBI over a two year-period and collected the following information: age, GCS score, CT-scan Marshall grade, incidence of early PTS, incidence of phenytoin use and time delay, side effects, and incidence of over-dosage or under-dosage.Results:1008 patients were included. 5.4 % had early PTS, 2.3 % while on prophylaxis and 3.1% while not on prophylaxis, 1.9% before reaching the hospital and 1.2% prior to phenytoin administration while in hospital. Delay of administration was 5 hours. 64.8% received prophylaxis and physicians used positive CT scan as the primary decision-making parameter (p<.001). Compliance with guidelines was 99.7%. Adverse reactions occurred in 0.5%. Levels were drawn in 42.2% (52% therapeutic, 41% low, 7% high).Conclusions:Phenytoin is used according to guidelines, with CT scan being the main decision factor for its use. The frequency of early PTS rate is low and side effects are rare. However, earlier administration of phenytoin and adequate levels could further prevent early PTS.
Predictors of Survival in a Huntington's Disease Population from Southern Italy
Authors: Rinaldi Carlo , Salvatore Elena , Giordano Ilaria , De Matteis Sara , Tucci Tecla , Cinzia Valeria Russo , Rossi Fabiana , Castaldo Imma , Morra Vincenzo Brescia , Di Maio Luigi , Filla Alessandro , De Michele Giuseppe 
Published: December 2014

Background:The primary aim of the present study was to determine the survival rates and identify predictors of disease duration in a cohort of Huntington's disease (HD) patients from Southern Italy.Methods:All medical records of HD patients followed between 1977 and 2008 at the Department of Neurological Sciences of Federico II University in Naples were retrospectively reviewed and 135 patients were enrolled in the analysis. At the time of data collection, 41 patients were deceased (19 males and 22 females) with a mean ± SD age at death of 56.6 ± 14.9 years (range 18-83).Results:The median survival time was 20 years (95% CI: 18.3-21.7). Cox regression analysis showed that the number of CAG in the expanded allele (HR 1.09 for 1 point triplet increase, p=0.002) and age of onset (HR 1.05 for 1 point year increase, p=0.022) were independent and significant predictors of lower survival rates.Conclusions:We believe that these findings are important for a better understanding of the natural history of the disease and may be relevant in designing future therapeutic trials.
Recurrent Encephalopathy: NAGS (N-Acetylglutamate Synthase) Deficiency in Adults
Authors: Cartagena A. , Prasad A.N. , Rupar C.A. , Strong M. , Tuchman M. , Mew N. Ah , Prasad C. 
Published: December 2014

Abstract:N-acetyl-glutamate synthase (NAGS) deficiency is a rare autosomal recessive urea cycle disorder (UCD) that uncommonly presents in adulthood. Adult presentations of UCDs include; confusional episodes, neuropsychiatric symptoms and encephalopathy. To date, there have been no detailed neurological descriptions of an adult onset presentation of NAGS deficiency. In this review we examine the clinical presentation and management of UCDs with an emphasis on NAGS deficiency. An illustrative case is provided. Plasma ammonia levels should be measured in all adult patients with unexplained encephalopathy, as treatment can be potentially life-saving. Availability of N-carbamylglutamate (NCG; carglumic acid) has made protein restriction largely unnecessary in treatment regimens currently employed. Genetic counselling remains an essential component of management of NAGS.
Serum Urate and the Risk of Parkinson's Disease: Results From a Meta-Analysis
Authors: Shen Chunhong , Guo Yi , Luo Wei , Lin Chen , Ding Meiping 
Published: December 2014

Objective:Serum urate may exert protective effects against Parkinson's disease (PD) through its antioxidant capacities. In this article, we examine the hypothesis that high serum urate levels are associated with lower risk of PD.Methods:We searched NCBI (PubMed), ISI Web of Science and EMBASE for studies that reported the risk of PD associated with serum urate. Fixed or random effects meta-analysis was used to pool results across studies, and further analysis was used to assess the effects by gender.Results:Six studies met the inclusion criteria involving a total of 33 185 participants. Overall, we found a 33% reduction in PD incidence among persons with high serum urate level (relative risk [RR]=0.67; 95% confidence interval [CI], 0.50-0.91). Subgroup analysis was performed with 20 641 men and 12 544 women included, indicating statistically significant protective effects of serum urate in men (RR=0.60; 95% CI, 0.40-0.90) but not in women. A dose-response trend of serum urate to reduce PD risk was also observed involving 11 795 participants (RR=0.77; 95% CI, 0.68-0.88). Additionally, high serum urate levels seemed to slow the clinical decline of PD patients (RR=0.56; 95% CI, 0.43-0.72).Conclusions:In light of these findings, our study confirms previous findings of a robust association between high serum urate level and PD risk, especially in men. It also suggests that long-term exposure to high serum urate may be linked to the delay of PD progression, however more well-designed investigations are needed.
Patients Referred for TIA May Still Have Persisting Neurological Deficits
Authors: Moreau Francois , Jeerakathil Thomas , Coutts Shelagh B. ,   FRCPC for the ASPIRE investigators
Published: December 2014

Background:The presence of residual neurological deficits after neurological symptoms is important information for making a diagnosis of Transient Ischemic Attack (TIA) versus stroke. The purpose of this study was to establish the reliability of the referring physician (non neurologist) to report focal neurological deficits in the context of an urgent referral for TIA.Methods:Prospectively recorded urgent physician-to-physician phone referrals for TIA through the Southern Alberta TIA hotline from March 2009 to July 2010 were reviewed. “Has the neurological deficit completely resolved?” was asked to the referring physician (family or emergency room physician) and recorded prospectively as a yes/no response. Patients were included if a neurological examination was performed by a neurologist on the same day as referral. The neurologist's assessment of whether the deficit had resolved was compared to that of the referring physician.Results:78 patients were included in this study. 62 patients had resolved as per the referring physician's assessment. Of these 62 patients, 16 (25.8% 95%CI 16-38) had evidence of persisting neurological deficits on the neurologist's assessment. A wide variety of mild neurological deficits were identified. None of these deficits appeared to be explained by progression of symptoms.Conclusion:Physicians referring patients with TIA syndromes for emergent assessment do not reliably detect mild residual deficits in one-quarter of patients. We are questioning the validity of neurological deficit resolution as a triage rule. The findings suggest that studies of TIA likely include a proportion of minor stroke patients and this should be remembered when extrapolating the results to other populations.
Increased Intestinal Permeability and Parkinson Disease Patients: Chicken or Egg?
Authors: Salat-Foix D. , Tran K. , Ranawaya R. , Meddings J. , Suchowersky O. 
Published: December 2014

Gastrointestinal involvement is a frequent and early event in the course of Parkinson Disease (PD), and may have a prominent role in the early pathophysiology of the disease. On the other hand, derangement in intestinal permeability could also result from the involvement of the gastrointestinal tract over the course of the disease.Patients and methods:The intestinal permeability of 12 non-selected PD patients was studied using a validated, non-invasive test; these results were compared to predefined age-adjusted reference values.Results:4/12 PD patients had abnormal gastrointestinal permeability; two had both an abnormal lactulose/mannitol ratio and an abnormal sucrose concentration, and two an isolated abnormal result. An increased lactulose/mannitol ratio is consistent with defect of either the enterocytes or the tight junctions between them.Conclusion:Intestinal permeability is increased in a significant proportion of unselected PD patients with minimal gastrointestinal symptoms. The significance of this finding needs to be further evaluated.
Effects of Creatine and Exercise on Skeletal Muscle of FRG1-Transgenic Mice
Authors: Ogborn Daniel I. , Smith Katelyn J. , Crane Justin D. , Safdar Adeel , Hettinga Bart P. , Tupler Rossella , Tarnopolsky Mark A. 
Published: December 2014

Background:The FRG1-transgenic mouse displays muscle dysfunction and atrophy reminiscent of fascioscapulohumeral muscular dystrophy (FSHD) and could provide a model to determine potential therapeutic interventions.Methods:To determine if FRG1 mice benefit from treatments that improve muscle mass and function, mice were treated with creatine alone (Cr) or in combination with treadmill exercise (CrEX).Results:The CrEx treatment increased quadriceps weight, mitochondrial content (cytochome c oxidase (COX) activity, COX subunit one and four protein), and induced greater improvements in grip strength and rotarod fall speed. While Cr increased COX subunits one and four protein, no effect on muscle mass or performance was found. Since Cr resulted in no functional improvements, the benefits of CrEx may be mediated by exercise; however, the potential synergistic action of the combined treatment cannot be excluded.Conclusion:Treatment with CrEx attenuates atrophy and muscle dysfunction associated with FRG1 overexpression. These data suggest exercise and creatine supplementation may benefit individuals with FSHD.
Epileptiform Activity in Neurocritical Care Patients
Authors: Kramer Andreas H. , Jette Nathalie , Pillay Neelan , Federico Paolo , Zygun David A. 
Published: December 2014

Background:Non-convulsive seizures have been reported to be common in neurocritical care patients. Many jurisdictions do not have sufficient resources to enable routine continuous electroencephalography (cEEG) and instead use primarily intermittent EEG, for which the diagnostic yield remains uncertain. Determining risk factors for epileptiform activity and seizures could help identify patients who might particularly benefit from EEG monitoring.Methods:We performed a cohort study involving neurocritical care patients with admission Glascow Coma Scale (GCS) scores =≤ 12, who underwent ≥ 1 EEG. EEGs were reviewed for presence of interictal discharges, periodic epileptiform discharges (PEDs), and seizures. Multivariate analysis was used to identify predictors of these findings and to describe their prognostic implications.Results:393 patients met inclusion criteria. 34 underwent cEEG, usually because epileptiform activity was first detected on a routine EEG. The prevalence of PEDs or electrographic seizures was 13%, and was highest with anoxic encephalopathy and central nervous system infections. Other independent predictors for epileptiform activity included a history of convulsive seizure(s), increasing age, deeper coma, and female gender. Although patients with epileptiform activity had higher mortality, this association disappeared after adjustment for confounders.Conclusion:Approximately 7-8 neurocritical care patients must undergo intermittent EEG monitoring in order to diagnose one with PEDs or seizures. The predictors we identified could potentially help guide use of resources. Repeated intermittent studies, or cEEG, should be considered in patients with multiple risk factors, or when interictal discharges are identified on an initial EEG. It remains unclear whether aggressive prevention and treatment of electrographic seizures improves neurologic outcomes.
Concussion Knowledge among Medical Students and Neurology/Neurosurgery Residents
Authors: Boggild Miranda , Tator Charles H. 
Published: December 2014

Background and Objectives:Concussion is a prevalent brain injury in the community. While primary prevention strategies need to be enhanced, it is also important to diagnose and treat concussions expertly and expeditiously to prevent serious complications that may be life-threatening or long lasting. Therefore, physicians should be knowledgeable about the diagnosis and management of concussions. The present study assesses Ontario medical students’ and residents’ knowledge of concussion management.Methods:A survey to assess the knowledge and awareness of the diagnosis and treatment of concussions was developed and administered to graduating medical students (n= 222) and neurology and neurosurgery residents (n = 80) at the University of Toronto.Results:Residents answered correctly significantly more of the questions regarding the diagnosis and management of concussions than the medical students (mean = 5.8 vs 4.1, t= 4.48, p<0.01). Gender, participation in sports, and personal concussion history were not predictive of the number of questions answered correctly. Several knowledge gaps were identified in the sample population as a whole. Approximately half of the medical students and residents did not recognize chronic traumatic encephalopathy (n = 36) or the second impact syndrome (n = 44) as possible consequences of repetitive concussions. Twenty-four percent of the medical students (n = 18) did not think that “every concussed individual should see a physician” as part of management.Conclusions:A significant number of medical students and residents have incomplete knowledge about concussion diagnosis and management. This should be addressed by targeting this population during undergraduate medical education.
Diabetes, Intracranial Stenosis and Microemboli in Asymptomatic Carotid Stenosis
Authors: Lam Thach D. , Lammers Stephanie , Munoz Claudio , Tamayo Arturo , Spence J. David 
Published: December 2014

Background:The risk of stroke in patients with asymptomatic carotid stenosis (ACS) is now so low that it is important to have methods to identify those patients most likely to benefit from intervention, or who may require special consideration in choice of medical therapy. We studied the prediction of stroke, death or transient ischemic attacks (stroke/death/TIA) in patients with ACS by intracranial arterial stenosis, and microemboli on transcranial Doppler (TCD), and the effect of diabetes mellitus on microemboli, intracranial stenosis and risk of events.Methods:Patients with ACS > 60% by Doppler ultrasound were recruited from the Stroke Prevention Clinic of University Hospital, London, Canada. All 339 participants underwent TCD for detection of intracranial stenosis and detection of microemboli, and carotid ultrasound to measure extracranial stenosis and total carotid plaque area. Participants were followed for three years, to determine the risk of stroke/death/TIA.Results:Stroke/death/TIA occurred in 38% of patients with microemboli versus 10% without (p=0.0001), and in 18% of patients with intracranial stenosis, versus 10% without (p=0.042). Diabetics were significantly more likely to have intracranial stenosis (45% vs. 29%, p =0.014), microemboli (38% vs. 10%, p <0.0001), and had significantly higher risk of stroke/death/TIA over three years (21% vs. 11% without; p=0.024). Survival free of stroke, TIA or death was significantly better without microemboli or intracranial stenosis (p<0.0001).Conclusions:Diabetes, microemboli and intracranial stenosis predicted higher risk of stroke, death or TIA than did extracranial stenosis or total plaque area; diabetics may need more intensive therapy.
Long-term Results of Radiosurgery for Cerebral Arteriovenous Malformations
Authors: Lecavalier-Barsoum Magali , Roy Daniel , Doucet Robert , Fortin Bernard , Lambert Carole , Moumdjian Robert , Bahary Jean-Paul 
Published: December 2014

Background:Stereotactic radiosurgery (SRS) is known to safely result in a high obliteration rate for small and medium sized arteriovenous malformations (AVM).Objective:To evaluate the long-term outcome of patients treated with SRS, with special emphasis given to obliteration and toxicity rates.Methods:We performed a review of 43 cerebral AVM patients, treated from 1998 to 2008 with a single SRS dose ranging from 21-25 Gy. Of these, 37 had a minimal follow-up of one year. Medical files were reviewed to assess patient and AVM characteristics, the SRS treatment, therapy prior to SRS, the obliteration rate and toxicities. Whenever necessary, outcome data was supplemented by telephone interviews with the patient or treating physician.Results:AVM size was ≥3cm in diameter in 21% of patients. Five patients (11.6%) underwent surgery prior to SRS and 31 patients (72.1%) received one or more embolizations prior to SRS. Of the patients followed with angiography ≥1 year post-SRS, 89% (33/37) had a complete obliteration of the nidus, after a median time of 24.7 months post-treatment. Embolization prior to SRS was not predictive of outcome. One patient suffered a non-fatal haemorrhage between treatment and obliteration. The rate of symptomatic radiation-induced radiological changes was 8.1%.Conclusion:Our study shows both obliteration and complication rates in the upper limit of those reported in the literature. SRS seems an attractive treatment option for small AVMs. Unlike other reports, the prior use of embolization did not impact negatively on obliteration rates.
The Syndrome of Infantile-Onset Saccade Initiation Delay
Authors: Salman Michael S. , Ikeda Kristin M. 
Published: December 2014

Introduction:Infantile-onset saccade initiation delay (ISID), also known as congenital ocular motor apraxia, is characterized by the inability to initiate volitional horizontal saccades. Other abnormalities including developmental delay and ataxia have been reported. The frequency of these abnormalities is unknown. We performed a detailed review of the medical literature to quantify features of ISID.Methods:We searched the English medical literature for articles related to ISID from 1952 to 2010. Whenever possible, patients were excluded if they had acquired SID, Joubert syndrome or neurodegenerative conditions. The minimum prevalence was calculated for each abnormality.Results:Sixty-six articles with information on 288 patients were included in the analysis. Head thrusts were reported in 84.7%. Blinks without head thrusts were used to initiate saccades in 41%. The fast phases of the optokinetic response and vestibulo-ocular reflex were impaired in 69.8% and 34.4% respectively. Smooth ocular pursuit was abnormal in 33%. Global developmental delay occurred in 41.3%, speech or language delay in 36.5%, cognitive delay in 17%, hypotonia in 35.8%, motor delay in 48.6%, and ataxia/clumsiness in 49.3% of patients. Neuroimaging was performed on 197 patients and was normal in 39.1%. Abnormalities involved the cerebellum (24.9%), cerebrum (15.7%), other infratentorial structures (11.7%), and corpus callosum (6.1%).Conclusions:Infantile-onset saccade initiation delay is frequently associated with deficits in reflexive saccades and less frequently with impaired smooth ocular pursuit. Developmental delay, hypotonia, and ataxia occur frequently in ISID, suggesting more global brain impairment and not just a saccadic disorder.
Impact of Technique on Cushing Disease Outcome Using Strict Remission Criteria
Authors: Alahmadi Hussein , Cusimano Michael D. , Woo Kenneth , Mohammed Ameen A. , Goguen Jeannette , Smyth Harley S. , Macdonald Robert L. , Muller Paul J. , Horvath Eva , Kovacs Kalman 
Published: December 2014

Background:Cushing disease (CD) constitutes a challenging condition for the pituitary surgeon. Given the variety of factors affecting outcomes in CD, it is uncertain whether the newer endoscopic technique improves the results of surgery.Methods:A review was conducted of CD cases at our institution between 2000 and 2010. Analysis was done to: determine if surgical technique had an effect on outcome, identify the predictors of outcome and provide details of failed cases. Remission was defined as normal postoperative 24-hour urinary free cortisol (24-h UFC), suppression of morning serum cortisol to <50 nmol/L after 1mg of dexamethasone or being dependent on steroid replacement.Results:Forty-two patients met our inclusion criteria. Average follow-up period was 33 months. There were 15 macroadenomas and 27 microadenomas. Seventeen patients had an endoscopic transsphenoidal surgery and twenty-five patients had a microscopic transsphenoidal procedure. Long-term overall remission was achieved in 26 (62%) patients. There was no significant difference in remission rates between the two techniques (p value 0.757). Patient's subjective symptomatic improvement and drop of morning serum cortisol in the postoperative period to less than 100 nmol/L correlated with long-term remission (p value 0.0031and 0.0101, respectively) while repeat surgery was the only predictor of the lack of postoperative remission (p value 0.0008).Conclusions:Revision surgery predicted poor remission rate for CD. Within the power of our study size, there was no difference in outcome between the endoscopic and microscopic approaches. Surgical outcomes should be reviewed in association with remission criteria used in a study.