bluuebunny/crossref_metadata_2025_split huggingface dataset:
075 A case of spontaneous CSF rhinorrhea in a patient with marfan syndrome
Authors: Upadhyay Viral , Khan Salman 
Published: July 2019

IntroductionSpontaneous cerebrospinal fluid leak is uncommon condition and frequently associated with hereditary disorders of connective tissues. Nasal CSF leakage is extremely rare.1Methods and resultsWe present the case of a 40-year-old woman presented to hospital for few days history of postural headache associated with clear intermittent discharge from right nostril without any signs of meningism. There was no history of trauma. She has a background history of Marfan syndrome with associated complications of ASD repair at age 2, mechanical Aortic and Mitral valve replacement, aortic root repair, previous ST elevation MI with LV dysfunction, automated implantable cardioverter-defibrillator in situ, atrial fibrillation, and Hashimoto’s thyroiditis. Her regular medications are warfarin, bisoprolol and thyroxine. The clear nasal discharge was positive for β-2 transferrin confirming cerebrospinal fluid. Her CT Brain did not reveal any clear site of CSF leak. She had a flexible nasendoscopy which showed normal nasal passageway, no defect in nasal mucosa and no active CSF leakage. She was managed conservatively with strict bed rest and advised to minimise strenuous activity and heavy lifting.ConclusionSpontaneous cerebrospinal fluid leak is uncommon condition and frequently associated with hereditary disorders of connective tissues. Nasal CSF leakage is extremely rare.1 Testing β-2 transferrin has high sensitivity and specificity.2Initial treatment may include bed rest, oral or intravenous hydration, oral caffeine or corticosteroids.3 4 If conservative therapy fails, surgical repair with nasal endoscopic approach is recommended.2 5ReferencesOmmaya A, Di Chiro G, Baldwin M, Pennybacker J. Non-traumatic cerebrospinal fluid rhinorrhoea. Journal of Neurology, Neurosurgery & Psychiatry 1968;31(3):214-–225.Wang E, Vandergrift W, Schlosser R. Spontaneous CSF Leaks. Otolaryngologic Clinics of North America 2011;44(4):845–856.Milledge J, Ades L, Cooper M, Jaumees A, Onikul E. Severe spontaneous intracranial hypotension and Marfan syndrome in an adolescent. Journal of Paediatrics and Child Health 2005;41(1–2):68–71.Placantonakis D, Bassani L, Graffeo C, Behrooz N, Tyagi V, Wilson T, et al. Noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with marfan syndrome: Case Report and Review of the Literature. Surgical Neurology International 2014;5(1):8.Spontaneous cerebrospinal fluid rhinorrhea. Medicine 2018;97(7):e9954.
076 A rare case of anti-glutamate decarboxylase antibody syndrome presenting with recurrent vomiting
Authors: Dal Shoaib , O’Brien Bill 
Published: July 2019

IntroductionAnti-glutamte decarboxylase antibody (anti-GAD) has been linked with various neurological syndromes including stiff-person syndrome, limbic encephalopathy, cerebellar ataxia, eye movement disorders and epilepsy (collectively known as ‘anti-GAD positive neurological syndromes’).1 We describe a very atypical phenotypic presentation of anti-GAD syndrome with unexplained vomiting and weight loss.CaseA 46 years old lady with no past medical or family history of note, presented with 6 months history of severe headaches and recurrent attacks of episodic vomiting (4–6 episodes of multiple vomiting daily) with no identified precipitant and complete normality in between the episodes with no other associated symptoms. She reported 15 kg of unintentional weight loss. Neurological examination and investigations including MRI brain, CT angiogram and liver enzymes, immunoglobulins, thyroid function, vasculitic screen were normal. Upper GI endoscopy, gastric emptying studies, CT imaging of chest, abdomen and pelvis and whole body PET scan were unremarkable. Serum autoimmune antibody screen was positive with high titre of anti-GAD antibody (1200 kU/liter). The cerebrospinal fluid anti-GAD antibody titre was raised at 103.7 kU/liter with otherwise normal parameters including negative oligoclonal bands. The nerve conduction studies did not show continuous motor activity or spasmodic reflex myoclonus (seen in stiff-person syndrome).2 A therapeutic trial of immunosuppression was introduced with moderate improvement in symptoms.ConclusionAnti-GAD neurological syndromes are rare and this is a unique presentation of the same. It is not completely understood why the presence of one antibody causes varied syndromes. The hypothesis is that the recurrent vomiting is possibly due to diaphragmatic spasms.ReferencesSaiz A, Blanco Y, Sabater L, et al. Spectrum of neurological syndromes associated with glutamic acid decarboxylase antibodies: diagnostic clues for this association. Brain 2008;131:2553–2563.Buechner S, Florio I, Capone L. Stiff person syndrome: A rare neurological disorder, heterogeneous in clinical presentation and not easy to treat. Case Rep Neurol Med2015; 2015:278065.
083 Novel wireless S – patch device may efficiently detect atrial fibrillation in patients with ischaemic stroke
Authors: Lazarovska Suzana , Tie Hui , Hopkins Andrew , Dimitri Hany , Premawardhana Upul , Mcdougall Alan , Gopinath Sumana , Chow Josephine , Rajaratnam Rohan 
Published: July 2019

IntroductionAtrial fibrillation (AF) is the most common atrial arrhythmia leading to increased risk of thromboembolic events. Ward telemetry (WT) has a low detection rate for AF in stroke patients and often patients are not appropriately treated with anticoagulation. This study compares conventional 2-day WT versus 4-day wireless S-patch monitoring to detect AF.MethodsProspective case series.Results51 patients admitted for stroke workup were recruited across 2 major tertiary centre’s in Sydney to compare WT monitoring for 2 days versus S-patch monitoring for 4 days in the detection of AF. The efficacy to detect AF using both technologies across 76 hours of telemetry was assessed via data extractions and Cardiologist review. A matrix was used to measure nursing/patient satisfaction and setup/resource times were assessed.84–94% of patients and 75–95% of nursing preferred the S-Patch. Non-parametric tests indicate significant time saving for removal of S-Patch versus WT [2.2 mins vs 5.1 mins (p=0.00)]. Efficacy of S-Patch to detect AF following Cardiologist review was greater than WT, with 7 patients identified with AF by S-Patch versus 1 using WT. The S-patch had a false positive rate of 78%.ConclusionThe S-patch had a higher detection rate of AF compared to WT. This allows patients to be anticoagulated appropriately for the prevention of further stroke. Analysis shows patients and staff overwhelmingly prefer the S-Patch. The S-Patch is sensitive in the detection of AF however it showed a high false positive rate. We are confident that further refinement and advances will provide a novel device in the detection of AF.
092 Motor neuron disease with malignancy: clinical and pathophysiological insights
Authors: Higashihara Mana , Menon Parvathi , Geevasinga Nimeshan , Bos Mehdi AJVan den , Kiernan Matthew C , Vucic Steve 
Published: July 2019

IntroductionWhile some regard an association between motor neuron disease (MND) and malignancy as co-incidental, others have argued that it could represent a distinct clinical entity. The present study undertook in depth phenotyping along with assessment of cortical function to further explore disease pathophysiology in MND with malignancy (MND-M) patients.MethodsClinical features along with assessment of peripheral and cortical function was undertaken in 13 MND-M and results were compared to sporadic and familial MND cohorts.ResultsFrom a cohort 13 patients (10 males; aged 65.2±2.0 years), 30.8% were diagnosed with a haematological malignancy. The lower motor neuron phenotype predominated in the in the MND-M patients (χ2=10.8, P<0.01), with the upper motor neuron (UMN) score being significantly reduced in MND-M patients compared to sporadic and familial MND cohorts (χ2=6.84, P<0.01). The neurological deficits did not respond to treatment of the underlying malignancy in the majority of MND-M (92%) patients, and as such there were no significant differences in survival between the cohorts. Despite a paucity of UMN signs, cortical hyperexcitability was evident in MND-M patients, as indicated by reduction in short interval intracortical inhibition (P<0.01) and increase in motor evoked potential amplitude (P<0.01), that were similar to findings in sporadic and familial MND cohorts.ConclusionsThe present study suggests that MND-M falls within the spectrum of MND. A co-incidental association between MND and malignancy is underscored by cortical dysfunction and clinical findings which seems within the spectrum of abnormality evident in classical MND phenotypes.
009 Axonal excitability properties in dravet’s syndrome reflect effect of loss of sodium channels
Authors: Farrar Michelle A , Tomlinson Susan E , Howells James , Lin Cindy Shin-YI , Carey Kate , Park Susanna B , Hollingsworth Georgina , Lawson John A , Kiernan Matthew C , Berkovic Sam F , Burke David , Scheffer Ingrid E 
Published: July 2019

IntroductionMutations in SCN1A encoding the Nav1.1 subunit of the neuronal sodium channel underlie the devastating epilepsy of Dravet’s syndrome.1 The mechanism by which Nav1.1 dysfunction causes seizures is not clear. In vitro and in silico channel evaluation can support mutation pathogenicity but cannot define the in vivo impact of channel dysfunction. Axonal excitability studies can show the pattern of single-channel dysfunction in disorders where the channel is peripherally expressed.2 This study was undertaken to determine whether axonal excitability studies could detect changes in Dravet’s patients related to the condition or due to medication effect.MethodsPatients with Dravet’s syndrome were recruited from clinics in Sydney and Melbourne and axonal excitability studies were performed. Excitability results were analysed in 3 age groups and compared to age-matched normal controls.ResultsTwenty six patients (ages 2–46) were studied. Findings were most pronounced in patients aged 20–46 (n=7) with 6.9% greater increase in threshold during hyperpolarization(p=0.1), 7.6% greater threshold decrease on depolarization(p=0.005) and, in the recovery cycle, 19.7% reduction in superexcitability(p=0.002) and 26% reduction in subexcitability(p=0.03). Axonal excitability studies resembled previously published changes seen in patients with sodium channel blockade caused by acute tetrodotoxin poisoning.3ConclusionsChanges in excitability of axonal membrane in Dravet’s syndrome are consistent with a decrease in sodium channel function. As the affected channel in Dravet’s syndrome is not peripherally expressed, the effect seen is likely due to the heavy anticonvulsant regime required to control epilepsy, combined with a progressive loss of sodium channel function that occurs with age.ReferencesMeisler MH, O’Brien JE, Sharkey LM. Sodium channel gene family: epilepsy mutations, gene interactions and modifier effects. J Physiol 2010;588:1841–1848.Tomlinson SE, Howells J, Burke D. In vivo assessment of neurological channelopathies: Application of peripheral nerve excitability studies. Neuropharmacology. 2018 Apr;132:98–107.Kiernan MC, Isbister GK, Lin CS, Burke D, Bostock H. Acute tetrodotoxin-induced neurotoxicity after ingestion of puffer fish. Ann Neurol 2005;57:339–48.
103 New zealand hospital stroke service provision: a national survey
Authors: Thompson Stephanie , Douwes Jeroen , Barber Alan , Cadilhac Dominique , McNaughton Harry , Gommans John , Fink John , Davis Alan , Feigin Valery , Ranta Anna 
Published: July 2019

IntroductionThe REGIONS Care study assesses stroke care throughout New Zealand. It includes a national audit and here we present organisational survey results assessing current availability of interventions and management approaches in each hospital.MethodsAll 20 District Health Boards were invited to complete a survey about patients managed per annum, care setting, and service provision. Results were grouped into rural and urban groups to assess for geographic differences.ResultsAll NZ hospitals managing stroke patients completed the survey. Results found that of these hospitals 92% have an acute stroke unit, 85% have a TIA pathway, but only 58% offer rapid access specialist TIA services. 100% offer thrombolysis, 92% use pre-hospital notification and rapid ED triage system, and 73% an in-hospital ‘code stroke’ alert. 50% have access to thrombectomy. Only 32% offer a dedicated inpatient stroke rehabilitation unit. While 92% provide community rehabilitation, only 11% offer early supported discharge programmes. 87% routinely provide stroke patient education, but only 54% provide individualised stroke care plans at discharge. Only 28% have access to a psychologist. In-hospital stroke alerts (p=0.014), access to thrombectomy (p=0.016), access to stroke unit care (p=0.027), and routine referral to stroke foundation (p=0.049) were more common in urban areas.ConclusionThe results of this organisational survey indicates that stroke care provision has improved since the last audit in 2009, but important gaps remain. These results will help services focus on specific areas for improvement, some of which such as pre-hospital alerts should be relatively easy to address.
Pressure effects on mechanical and electronic properties of metallic C14 by first-principles calculation
Authors: Zhou Yingjiao , Wei Qun , Wei Bing , Yang Ruike , Cheng Ke , Zhang Meiguang , Yan Haiyan , Chen Qinghua , Zhang Junqin 
Published: July 2019

The elastic constants and phonon dispersion of metallic C[Formula: see text] are calculated by first-principles calculations. The results show that the metallic C[Formula: see text] is mechanically and dynamically stable under high pressure. The variations of G/B ratio, Poisson’s ratio, elastic anisotropy, acoustic velocity and Debye temperature at the pressure range from 0 GPa to 100 GPa are analyzed. The results reveal that by adjusting the pressures the elastic anisotropy and thermodynamic properties could be improved for better applicability.
Interferometric Fringe Visibility Null as a Function of Spatial Frequency: A Probe of Stellar Atmospheres
Authors: Armstrong J. T. , Jorgensen A. M. , Mozurkewich D. , Neilson H. R. , Baines E. K. , Schmitt H. R. , van Belle G. T. 
Published: July 2019

We introduce an observational tool based on visibility nulls in optical spectro-interferometry fringe data to probe the structure of stellar atmospheres. In a preliminary demonstration, we use both Navy Precision Optical Interferometer (NPOI) data and stellar atmosphere models to show that this tool can be used, for example, to investigate limb darkening. Using bootstrapping with either multiple linked baselines or multiple wavelengths in optical and infrared spectro-interferometric observations of stars makes it possible to measure the spatial frequency [Formula: see text] at which the real part of the fringe visibility [Formula: see text] vanishes. That spatial frequency is determined by [Formula: see text], where [Formula: see text] is the projected baseline length, and [Formula: see text] is the wavelength at which the null is observed. Since [Formula: see text] changes with the Earth’s rotation, [Formula: see text] also changes. If [Formula: see text] is constant with wavelength, [Formula: see text] varies in direct proportion to [Formula: see text]. Any departure from that proportionality indicates that the brightness distribution across the stellar disk varies with wavelength via variations in limb darkening, in the angular size of the disk, or both. In this paper, we introduce the use of variations of [Formula: see text] with [Formula: see text] as a means of probing the structure of stellar atmospheres. Using the equivalent uniform disk diameter [Formula: see text], given by [Formula: see text], as a convenient and intuitive parameterization of [Formula: see text], we demonstrate this concept by using model atmospheres to calculate the brightness distribution for [Formula: see text] Ophiuchi and to predict [Formula: see text], and then comparing the predictions to coherently averaged data from observations taken with the NPOI.
Fairness in responsive parallelism
Authors: Muller Stefan K. , Westrick Sam , Acar Umut A. 
Published: July 2019

Research on parallel computing has historically revolved around compute-intensive applications drawn from traditional areas such as high-performance computing. With the growing availability and usage of multicore chips, applications of parallel computing now include interactive parallel applications that mix compute-intensive tasks with interaction, e.g., with the user or more generally with the external world. Recent theoretical work on responsive parallelism presents abstract cost models and type systems for ensuring and reasoning about responsiveness and throughput of such interactive parallel programs.
In this paper, we extend prior work by considering a crucial metric: fairness. To express rich interactive parallel programs, we allow programmers to assign priorities to threads and instruct the scheduler to obey a notion of fairness. We then propose the fairly prompt scheduling principle for executing such programs; the principle specifies the schedule for multithreaded programs on multiple processors. For such schedules, we prove theoretical bounds on the execution and response times of jobs of various priorities. In particular, we bound the amount, i.e., stretch, by which a low-priority job can be delayed by higher-priority work. We also present an algorithm designed to approximate the fairly prompt scheduling principle on multicore computers, implement the algorithm by extending the Standard ML language, and present an empirical evaluation.
The next 700 compiler correctness theorems (functional pearl)
Authors: Patterson Daniel , Ahmed Amal 
Published: July 2019

Compiler correctness is an old problem, with results stretching back beyond the last half-century. Founding the field, John McCarthy and James Painter set out to build a "completely trustworthy compiler". And yet, until quite recently, even despite truly impressive verification efforts, the theorems being proved were only about the compilation of whole programs, a theoretically quite appealing but practically unrealistic simplification. For a compiler correctness theorem to assure complete trust, the theorem must reflect the reality of how the compiler will be used.
There has been much recent work on more realistic "compositional" compiler correctness aimed at proving correct compilation of components while supporting linking with components compiled from different languages using different compilers. However, the variety of theorems, stated in remarkably different ways, raises questions about what researchers even mean by a "compiler is correct." In this pearl, we develop a new framework with which to understand compiler correctness theorems in the presence of linking, and apply it to understanding and comparing this diversity of results. In doing so, not only are we better able to assess their relative strengths and weaknesses, but gain insight into what we as a community should expect from compiler correctness theorems of the future.
Relational cost analysis for functional-imperative programs
Authors: Qu Weihao , Gaboardi Marco , Garg Deepak 
Published: July 2019

Relational cost analysis aims at formally establishing bounds on the difference in the evaluation costs of two programs. As a particular case, one can also use relational cost analysis to establish bounds on the difference in the evaluation cost of the same program on two different inputs. One way to perform relational cost analysis is to use a relational type-and-effect system that supports reasoning about relations between two executions of two programs.
Building on this basic idea, we present a type-and-effect system, called ARel, for reasoning about the relative cost of array-manipulating, higher-order functional-imperative programs. The key ingredient of our approach is a new lightweight type refinement discipline that we use to track relations (differences) between two mutable arrays. This discipline combined with Hoare-style triples built into the types allows us to express and establish precise relative costs of several interesting programs which imperatively update their data. We have implemented ARel using ideas from bidirectional type checking.
Synthesizing symmetric lenses
Authors: Miltner Anders , Maina Solomon , Fisher Kathleen , Pierce Benjamin C. , Walker David , Zdancewic Steve 
Published: July 2019

Lenses are programs that can be run both "front to back" and "back to front," allowing updates to either their source or their target data to be transferred in both directions. Since their introduction by Foster et al., lenses have been extensively studied, extended, and applied. Recent work has also demonstrated how techniques from type-directed program synthesis can be used to efficiently synthesize a simple class of lenses---so-called bijective lenses over string data---given a pair of types (regular expressions) and a small number of examples.
We extend this synthesis algorithm to a much broader class of lenses, called simple symmetric lenses, including all bijective lenses, all of the popular category of "asymmetric" lenses, and a rich subset of the more powerful "symmetric lenses" proposed by Hofmann et al. Intuitively, simple symmetric lenses allow some information to be present on one side but not the other and vice versa. They are of independent theoretical interest, being the largest class of symmetric lenses that do not rely on persistent internal state.
Synthesizing simple symmetric lenses is substantially more challenging than synthesizing bijective lenses: Since some of the information on each side can be "disconnected" from the other side, there will, in general, be many lenses that agree with a given example. To guide the search process, we use stochastic regular expressions and ideas from information theory to estimate the amount of information propagated by a candidate lens, generally preferring lenses that propagate more information, as well as user annotations marking parts of the source and target data structures as either irrelevant or essential.
We describe an implementation of simple symmetric lenses and our synthesis procedure as extensions to the Boomerang language. We evaluate its performance on 48 benchmark examples drawn from Flash Fill, Augeas, the bidirectional programming literature, and electronic file format synchronization tasks. Our implementation can synthesize each of these lenses in under 30 seconds.