bluuebunny/crossref_metadata_2025_split huggingface dataset:
Third Nerve Palsy: Analysis of 1400 Personally-examined Inpatients
Authors: Keane James R. 
Published: December 2014

Background:Most studies of third nerve palsy (TNP) antedate computerized imaging and focus primarily on chart review of referral outpatients.Methods:To compare a large contrasting population, I reviewed 1400 personally-examined municipal hospital inpatients with TNPs seen over 37 years.Results:TNPs were bilateral in 11%, complete in 33%, without other neurological signs (isolated) in 36%, and associated with recurrent cranial neuropathies in 7%. Third nerve damage occurred in the subarachnoid space in 32%, the cavernous sinus in 23%, the brainstem in 14%, as a nonlocalized peripheral neuropathy in 18% and at an uncertain location in 13%. Causes were trauma (26%), tumor (12%), diabetes (11%), aneurysm (10%), surgery (10%), stroke (8%), infection (5%), Guillain-Barre and Fisher syndromes (5%), idiopathic cavernous sinusitis (3%), benign self-limited (2%), miscellaneous (4%), and unknown (3%). Local causes, besides an abundance of trauma, included six cases involving cysticercosis, four with wound botulism, and one with coccidiomycotic meningitis. Of 234 patients with diabetes, microvascular ischemia was the cause of TNP in only two-thirds (five had aneurysms) and 53% of those with diabetic microvascular ischemia had pupillary involvement—often bilateral, suggesting concomitant autonomic neuropathy. Only 2% of aneurysms spared the pupil. Apainful onset occurred with 94% of aneurysm and 69% of diabetic cases.Conclusions:Bilateral TNPs, multiple cranial neuropathies, and accompanying neurological signs were common among our inpatients, as were causes rare in outpatient settings such as severe trauma, transtentorial herniation, midbrain strokes, and the Guillain-Barre syndrome. Few cases remained undiagnosed and nondiabetic ischemia was rare.
Functional Impairment in Patients with Sporadic Inclusion Body Myositis
Authors: Dunlap Heather V. , MacNeil Lauren G. , Tarnopolsky Mark A. 
Published: December 2014

Introduction:We conducted a retrospective chart review of 53 patients diagnosed with sporadic Inclusion Body Myositis (sIBM) who have been followed at the McMaster Neuromuscular Clinic since 1996.Objectives:We reviewed patient medical histories in order to compare our findings with similar cohorts, and analyzed quantitative strength data to determine functionality in guiding decisions related to gait assistive devices.Methods:Patient information was acquired through retrospective clinic chart review.Results:Our study found knee extension strength decreased significantly as patients transitioned to using more supportive gait assistive devices (P < 0.05). A decline to below 30 Nm was particularly indicative of the need for a preliminary device (i.e. cane)(P < 0.05). Falls and fear of falling poses a significant threat to patient physical well-being. The prevalence of dysphagia increased as patients required more supportive gait devices, and finally a significant negative correlation was found between time after onset and creatine kinase (CK) levels (P < 0.01).Conclusion:This study supports that knee extension strength may be a useful tool in advising patients concerning ambulatory assistance. Further investigations concerning gait assistive device use and patient history of falling would be beneficial in preventing future falls and improving long-term patient outcomes.
Referrals, Wait Times and Diagnoses at an Urgent Neurology Clinic over 10 Years
Authors: Wile D.J. , Warner J. , Murphy W. , Lafontaine A.L. , Hanson A. , Furtado S. 
Published: December 2014

Background:An urgent neurology assessment clinic was created at our institution to improve access to prompt neurological assessment, and has been in operation for over a decade. We assessed its timeliness and impact.Methods:The clinic database was examined retrospectively for trends in the volume and waiting time to assessments, neurologic diagnoses, and whether neurologic assessment changed patients’ diagnoses. Before and after implementation, the frequency of emergency department neurology assessments and hospital admissions for neurological investigation were compared.Results:In the first decade, 25145 referrals were received; 12460 patients were accepted and assessed within an average of 3.8 working days. The most common problems seen included headache and seizure (20.2% each). Overall, 44.6% of assessments resulted in a change to the referring diagnosis; this proportion varied by the type of problem seen (from 10.5% for seizures to 92.5% for psychiatric disturbances). From the pre- to post-opening periods, there were fewer emergency room neurological assessments (35.7% reduction) and fewer hospital admissions for neurological investigation (4.4/week to 2.2/week, 50% reduction).Conclusions:The urgent neurology clinic model at our institution has provided excellent service, including wait times of a few days, to a catchment of over two million Canadians for over a decade; clinic assessments have affected diagnoses and patient care.
Decompressive Craniectomy: Contralateral Lesions and Metabolic Abnormalities
Authors: Zeiler F.A. , West M. 
Published: December 2014

Objective:To define the incidence of new contralateral intracranial lesions following decompressive hemicraniectomy for blunt traumatic brain injury, and explore the potential association with metabolic factors that contribute to coagulopathy.Methods:We retrospectively reviewed the records and imaging of all patients treated with hemicraniectomy for blunt traumatic brain injury at our institution from May 2007 up to and including January 2012.Results:Twenty patients were identified during the time period to have undergone decompressive craniectomy for blunt head injury. The average age and Glasgow Coma Scale on presentation was 44.1 years (range: 19 – 72 years) and 6.5 (range: 3 – 14) respectively. All but one patient presented with an extra-axial hematoma as their surgical indication for craniectomy. Seven patients (35.0%) developed new contralateral lesions post-craniectomy. The average peri-operative pH, bicarbonate (HCO3) and hematocrit (HCT) levels for those with new contralateral lesions were lower than those without new lesions. Five of the seven patients (71.4%) with new lesions had abnormalities on their laboratory results that have been know to be attributable to coagulopathy, with four (57.1%) having two or more abnormal results. Eight of 13 (61.5%) patients without new lesion had laboratory abnormalites, with five (38.5%) having two or more abnormalities identified.Conclusions:The incidence of new contralateral lesions post-craniectomy for blunt head injury is 35.0% in our experience. There is an association between the metabolic derangements linked to trauma related coagulopathy and the formation of new lesions.
Wilson Disease in the South Chinese Han Population
Authors: Cheng Nan , Wang Kai , Hu Wenbin , Sun Daoyin , Wang Xun , Hu Jiyuan , Yang Renmin , Han Yongzhu 
Published: December 2014

Purpose:To prospectively investigate the incidence and prevalence of Wilson disease (WD) in Chinese Han population in Anhui Province, to analyze the genetic mutations in individuals with WD, and to provide basic epidemiological data regarding WD in this Chinese Han population.Methods:Between November 2008 and June 2010, individuals aged from 7 to 75 years were screened for the cornea K-F ring in both eyes using slit lamp examination and random sampling methods based on age stratification and cluster level 1. The participants were from Anhui Province's Hanshan County, Jinzhai County, and Lixin County. The clinical manifestations of the brain, liver, kidney, skin, and other organs in each individual were also determined. Individuals with positive K-F rings and clinical manifestations indicative of WD underwent copper biochemistry evaluations, abdominal ultrasound testing, and ATP7B gene mutation screening to confirm or exclude the diagnosis of WD.Results:Of 153,370 individuals investigated in this study, nine were diagnosed with WD. In these WD individuals, three cases had neurological symptoms, one has hepatic symptoms, one was hepatic and neurological combined, and the other four cases were presymptomatic. Of the eight individuals in whom genetic mutations were detected, seven individuals had mutations in the ATP7B gene. The other individual had no ATP7B gene mutations but her copper biochemical test results met the diagnostic criteria for WD. The incidence and prevalence of WD in this population were approximately 1.96/100,000 and 5.87/100,000 respectively.Conclusions:The Chinese Han population had a higher average prevalence of WD than the populations of the United States or Europe.
Canadian Epileptologists’ Counseling of Drivers Amidst Guideline Inconsistencies
Authors: Jirsch Jeffrey , Gross Donald W. , Jette Nathalie , Federico Paolo , Dubeau Francois , Tellez-Zenteno Jose F. , Pohlmann-Eden Bernd , Burneo Jorge G. , McLachlan Richard , Ahmed Syed N. , Deacon Charles , Javidan Manouchehr , Nguyen Dang K. , Sadler R. Mark , Spiller Allison , Townsend Tiffany , Veilleux Martin , Wennberg Richard A. , Wiebe Samuel , Yankovsky Alexei 
Published: December 2014

Background:Epilepsy is a common medical condition for which physicians perform driver fitness assessments. The Canadian Medical association (CMA) and the Canadian Council of Motor transportation administrators (CCMTA) publish documents to guide Canadian physicians’ driver fitness assessments.Objectives:We aimed to measure the consistency of driver fitness counseling among epileptologists in Canada, and to determine whether inconsistencies between national guidelines are associated with greater variability in counseling instructions.Methods:We surveyed 35 epileptologists in Canada (response rate 71%) using a questionnaire that explored physicians’ philosophies about driver fitness assessments and counseling practices of seizure patients in common clinical scenarios. Of the nine scenarios, CCMTA and CMA recommendations were concordant for only two. Cumulative agreement for all scenarios was calculated using Kappa statistic. Agreement for concordant (two) vs. discordant (seven) scenarios were split at the median and analyzed using the Wilcoxon signed rank sum test.Results:Overall the agreement between respondents for the clinical scenarios was not acceptable (Kappa=0.28). For the two scenarios where CMa and CCMta guidelines were concordant, specialists had high levels of agreement with recommendations (89% each). A majority of specialists disagreed with CMa recommendations in three of seven discordant scenarios. The lack of consistency in respondents’ agreement attained statistical significance (p<0.001).Conclusions:Canadian epileptologists have variable counseling practices about driving, and this may be attributable to inconsistencies between CMa and CCMta medical fitness guidelines. This study highlights the need to harmonize driving recommendations in order to prevent physician and patient confusion about driving fitness in Canada.
HOXA1 Mutations are Not Commonly Associated with Non-Syndromic Deafness
Authors: Abu-Amero Khaled K. , Hagr Abdulrahman al , Almomani Murad O. , Azad Taif Anwar , Alorainy Ibrahim A. , Oystreck Darren T. , Bosley Thomas M. 
Published: December 2014

Objective:Homozygous homeobox A1 (HOXA1) mutations cause a spectrum of abnormalities in humans including bilateral profound deafness. This study evaluates the possible role ofHOXA1mutations in familial, non-syndromic sensorineural deafness.Methods:Forty-eight unrelated Middle Eastern families with either consanguinity or familial deafness were identified in a large deafness clinic, and the proband from each family was evaluated by chart review, audiogram, neuroimaging, andHOXA1sequencing.Results:All 48 probands had normal neuro-ophthalmologic and general medical examinations except for refractive errors. All had congenital non-syndromic sensorineural hearing loss that was symmetric bilaterally and profound (>90 dBHL) in 33 individuals and varied from 40 to 90 dBHL in the remainder. Thirty-nine of these individuals had neuroimaging studies, all documenting normal internal carotid arteries and normal 6th, 7th, and 8th cranial nerves bilaterally. Of these, 27 had normal internal ear structures with the remaining 12 having mild to modest developmental abnormalities of the cochlea, semicircular canals, and/or vestibular aqueduct. No patient had homozygousHOXA1mutations.Conclusions:None of these patients with non-syndromic deafness hadHOXA1mutations. None had major inner ear anomalies, obvious cerebrovascular defects, or recognized congenital heart disease.HOXA1is likely not a common cause of non-syndromic deafness in this Middle Eastern population.
The Effect of tPA on Inpatient Rehabilitation after Stroke: A Cost Comparison
Authors: Richardson Marina , Meyer Matthew , Teasell Robert 
Published: December 2014

Background:Tissue plasminogen activator has been found to significantly improve patient outcomes post stroke. Previous economic evaluations have adjusted for fewer admissions to inpatient rehabilitation but not for decreased length of stay in rehabilitation. Our objective was to estimate the potential cost savings associated with a decreased length of stay in inpatient rehabilitation for patients who receive tissue plasminogen activator compared to those who do not, in a Canadian context.Methods:Decreased length of stay in inpatient rehabilitation for patients who received tissue plasminogen activator compared to controls was reported previously in a population of 1962 patients admitted to hospital with an ischemic stroke in Ontario between July 1, 2003 and March 31, 2008. Average per diem cost savings associated with the use of tissue plasminogen activator were calculated using a literature based cost estimate. Sensitivity analysis varying the length of stay in inpatient rehabilitation was performed.Results:The estimated mean per diem cost of inpatient rehabilitation derived from the literature was $626. Based on previously reported estimates for reduced length of stay, receipt of tissue plasminogen activator was estimated to result in savings of $939 per patient during inpatient rehabilitation. Sensitivity analysis suggested that these cost savings could range from $501 to $1377 per patient on average.Conclusions:Future economic evaluations of tissue plasminogen activator should consider adjusting for shortened length of stay in inpatient rehabilitation for patients who receive tissue plasminogen activator.
White Matter Repair: Skin-Derived Precursors as a Source of Myelinating Cells
Authors: Biernaskie Jeff , Miller Freda D. 
Published: December 2014

ABSTRACT:Stem cell based therapies hold great promise for repair and functional restoration following neurological injury and disease. Skin-derived precursors (or “SKPs”) are a novel, multipotent somatic stem cell that resides within the mammalian dermis. SKPs persist within the skin throughout adulthood and yet intriguingly, exhibit many similarities to embryonic neural crest stem cells (NCSCs). For example, SKPs give rise to both neural and mesodermal cell types, and the former appear biased to peripheral nervous system fates. As such, SKPs are capable of generating Schwann cells, the myelinating glial cell of the peripheral nervous system. Here we discuss our current understanding of the biological origin of SKPs and specifically the potential therapeutic utility of SKPs as a highly accessible and autologous source of Schwann cells for remyelination and repair of the injured or diseased nervous system.
New Directions in Multiple Sclerosis Therapy: Matching Therapy with Pathogenesis
Authors: Antel Jack 
Published: December 2014

ABSTRACT:All currently approved therapies for multiple sclerosis (MS) modulate systemic immune components prior to their entry into the central nervous system (CNS). Available data indicate they lack impact on the progressive phases of disease; the more potent systemic immune-directed agents predispose to development of infectious or neoplastic disorders. Development of new agents that enhance disease stage related efficacy and limit systemic toxicity will need to consider the underlying mechanisms related to each phase of the clinical disorder, namely relapses, remission, and progression. This report focuses on disease related mechanisms ongoing within the CNS that contribute to the different phases of MS and how these may serve as potential therapeutic targets. Such mechanisms include CNS compartment specific immunologic properties especially as related to the innate immune system and neural cell-related properties that are determinants of the extent of actual tissue injury and repair (or lack thereof).
The effects of extracellular ATP and its receptor antagonists on pig oocytes during in vitro maturation
Authors: Wakizoe Erika , Ashizawa Koji , Sakamoto Shinsuke H. , Hemmi Koichiro , Kobayashi Ikuo , Tsuzuki Yasuhiro 
Published: December 2014

SummaryWe measured the ATP concentrations in the porcine follicular fluid derived from three sizes of follicles (small: <3 mm, medium: 3–6 mm, large: >6 mm in diameter). Then, the effects of pre-treatment (100 μM each for 30 min before maturation) with antagonists for extracellular ATP receptor P2X or P2Y on the nuclear maturation rate of cumulus-cell-enclosed (COs) or -denuded oocytes (DOs) up to the preovulatory stage in the presence or absence of 20 nM ATP (a similar concentration to that of medium-sized follicle fluid) were investigated. The antagonists used were pyridoxalphosphate-6-azophenyl-2′,4′-disulfonic acid (PPADS) or reactive blue 2 (RB2), for extracellular ATP receptor P2X and P2Y, respectively. In addition, the embryonic development rates of COs pre-treated with RB2 were also evaluated. It was found that when the follicular sizes increased, the ATP concentrations significantly decreased (P < 0.05). No differences were observed in the nuclear maturation rates among all COs, regardless of pre-treatment with (+) or without (–) PPADS and in the presence (+) or absence (–) of ATP during maturation. In contrast, the nuclear maturation rate of the COs, but not DOs, in the ATP(–) RB2(+) group was significantly lower (P < 0.05) than that of the ATP(–) RB2(–) and ATP(+)RB2(–) groups. The pronuclear formation and blastocyst formation rates by parthenogenetic activation in the ATP(–) RB2(+) and ATP(+) RB2(+) groups were significantly lower (P < 0.05) than those in the ATP(–) RB2(–) group. In conclusion, it is suggested that the nuclear maturation of porcine oocytes may be influenced by the ATP receptor P2Y present in the cumulus cells.
High speed and adaptable error correction for megabit/s rate quantum key distribution
Authors: Dixon A. R. , Sato H. 
Published: December 2014

AbstractQuantum Key Distribution is moving from its theoretical foundation of unconditional security to rapidly approaching real world installations. A significant part of this move is the orders of magnitude increases in the rate at which secure key bits are distributed. However, these advances have mostly been confined to the physical hardware stage of QKD, with software post-processing often being unable to support the high raw bit rates. In a complete implementation this leads to a bottleneck limiting the final secure key rate of the system unnecessarily. Here we report details of equally high rate error correction which is further adaptable to maximise the secure key rate under a range of different operating conditions. The error correction is implemented both in CPU and GPU using a bi-directional LDPC approach and can provide 90–94% of the ideal secure key rate over all fibre distances from 0–80 km.
Tandem Mass Spectrometry of Lipids
Authors: Murphy Robert C 
Published: December 2014

The emerging field of lipidomics has been made possible because of advances in mass spectrometry, and in particular tandem mass spectrometry of lipid ions generated by electrospray ionization. The ability to carry out basic biochemical studies of lipids using electrospray ionization is predicated upon understanding the behaviour of lipid derived ions following collision induced decomposition and mechanisms of product ion formation. During the past 20 years, a wealth of information has been generated about lipid molecules that are now analysed by mass spectrometry, however there is no central source where one can obtain basic information about how these very diverse biomolecules behave following collisional activation.
This book brings together, in one volume, this information so that investigators considering using tandem mass spectrometry to structurally characterize lipids or to quantitate their occurrence in a biological matrix, will have a convenient source to review mechanism of decomposition reactions related to the diversity of lipid structures.
A separate chapter is devoted to each of seven major lipid classes including fatty acids, eicosanoids and bioactive lipid mediators, fatty acyl esters and amides, glycerol esters, glycerophospholipids, sphingolipids, and steroids. Mechanistic details are provided for understanding the pathways of formation of major product ions and ions used for structural characterization. In most cases specific ancillary information has been critical to understand the pathways, including isotope labeling and high resolution analysis of precursor and product ions. For a few specific examples such data is missing and pathways are proposed as a means to initiate further mass spectral experiments to prove or disprove pathway hypotheses. While this work largely centres on the lipid biochemistry of animal (mammalian) systems, general principles can be taken from the specific examples and applied to lipid biochemistry found in plants, fungi, prokaryotes and archeal organisms.
Gcn1 contacts the small ribosomal protein Rps10, which is required for full activation of the protein kinase Gcn2
Authors: Lee Su Jung , Swanson Mark J. , Sattlegger Evelyn 
Published: December 2014

In eukaryotes, amino acid deprivation leads to the accumulation of uncharged tRNAs that are detected by Gcn2 (general control non-derepressible 2), which in turn phosphorylates eIF2α (α-subunit of eukaryotic translation initiation factor 2), an essential process for overcoming starvation. In Saccharomyces cerevisiae, sensing amino acid shortages requires that Gcn2 binds directly to its effector protein Gcn1 and both must associate with the ribosome. Our hypothesis is that uncharged tRNAs occur in the ribosomal A-site and that Gcn1 is directly involved in transfer of this starvation signal to Gcn2. In the present paper, we provide evidence that Gcn1 directly contacts the small ribosomal protein S10 (Rps10). Gcn1 residues 1060–1777 showed a yeast two-hybrid (Y2H) interaction with Rps10A. In vitro, Rps10A or Rps10B co-precipitated Gcn1[1060–1777] in an RNA-independent manner. rps10AΔ or rps10BΔ strains showed reduced eIF2α phosphorylation under replete conditions and shortly after onset of starvation, suggesting that Gcn1-mediated Gcn2 activation was impaired. Overexpression of GST-tagged Rps10 reduced growth under amino acid starvation and this was exacerbated by the Gcn1–M7A mutation known to impair Gcn1–ribosome interaction and Gcn2 activity. Under amino acid starvation, eEF3 (eukaryotic translation elongation factor 3) overexpression, known to weaken Gcn1 function on the ribosome, exacerbated the growth defect of rps10AΔ or rps10BΔ strains. Taken together, these data support the idea that Gcn1 contacts ribosome-bound Rps10 to efficiently mediate Gcn2 activation.