bluuebunny/crossref_metadata_2025_split huggingface dataset:
ANTI-VOLTAGE GATED POTASSIUM CHANNEL (VGKC) ANTIBODY POSITIVE ENCEPHALOPATHY
Authors: Kemp Michael , Kimber Jeff , Zhang Liqun 
Published: October 2015

Since autoimmune mediated encephalitis was recognised 15 years ago, several autoantibodies have been identified. We present 3 patients with positive VGKC antibodies to emphasise the spectrum of clinic presentation and value of antibody testing.A 61 yo male presented with cognitive decline and frequent right arm stiffening, which is recognised as faciobrachial dystonic seizure. VGKC/LGI1 antibody positive. Received IVIG treatment and rapidly recovered.A 69 yo women presented with acute personality change and poor short memory following a brief syncope. MRI showed bilateral hippocampus high T2/FLAIR signal. VGKC/LGI-1 antibody was 5800. She improved with IVIG and steroid treatment. However, seizures recurred when weaning from steroid at 4 months. Repeat titre of 1100. Steroid restarted until antibody negative.A 60 yo male presented with 8 months of personality change without myoclonus. VGKC antibody was positive with a titre of 169. Further testing LGI1 and CASPA2 were negative. He gradually recovered with 6 month steroid treatment. No specific cause was found with extensive investigations.The typical presentations of VGKC encephalitis have been widely recognised, however clinical vigilance is important for atypical presentations. Antibody titres are of great value following the treatment, while low positive cases prompt further investigations.
MANAGEMENT OF SUSPECTED ENCEPHALITIS IN A UK TEACHING HOSPITAL
Authors: Binks Sophie , Cooper Rebecca , Wood Ruth , Martin Llewelyn 
Published: October 2015

IntroductionThough infective encephalitis is relatively rare, prompt treatment significantly improves outcomes. In 2012, new national guidelines for management of suspected viral encephalitis were launched. We audited cases at Brighton and Sussex University Hospitals NHS Trust.MethodCases were identified retrospectively via medical coding and pharmacy records of patients to whom IV aciclovir was dispensed, between April 1 2012–June 30 2013. 42 cases were audited against the guidelines.ResultsIn 10 cases (24%), the final diagnosis was encephalitis but in only one was an organism confirmed (varicella). Time to suspecting encephalitis ranged from on admission to 27 days. No LP was performed in seven cases, and achieved in <6 hours in four (10%). Paired plasma glucose was not performed in 51% of LPs. Almost all patients (95%) started aciclovir, and 76% received MRI brain at a median of 3 days. HIV testing was performed in 60%. 26% were admitted to ITU and 24% died.ConclusionsEncephalitis is difficult to confirm and eventual differential of suspected cases is wide. The rate of ITU admission and death demonstrates the vulnerabilities of this cohort. While most patients received LP, aciclovir and MRI, there remains room for improvement in timing and completeness of investigations.
SPORADIC CREUTZFELDT-JAKOB DISEASE PRESENTING WITH A ‘JERKY ALIEN LIMB’
Authors: Vinjam Maruthi Ravi , Butterworth Steven , Davey Richard 
Published: October 2015

A 64 yr old right-handed mechanic presented with four weeks history of clumsy right hand with ongoing problems at work. He described stiffness and problems with co-ordination. At the time of presentation he was noted to be walking with his right arm behind him and not being aware of this. His initial examination showed Mini Mental Score of 26/30, with ongoing involuntary movements of his right arm (Video).Video shows spontaneous elevation of right arm with occasional myoclonic jerks. Video also demonstrates patient's difficulty in following simple motor tasks and his comments that his arm “has a mind of its own”.His MRI head (Figure 1) showed typical cortical ribboning pattern described in sporadic CJD and CSF Protein for 14-3-3, s100b and RT-QUIC findings were consistent with the diagnosis of CJD.Over next 4 weeks his cognitive function rapidly deteriorated with progressive worsening of his myoclonus. He died 4 weeks after his hospital discharge.There are three broad varieties of alien limb phenomenon (ALP) types described in the literature, frontal, callosal and sensory. Jerky (myoclonic) ALP is well described in patients with CJD, so CJD should be in the differentials in any patient presenting with sub-acute onset of ALP.
iPAD-BASED ASSESSMENT IN PARKINSON'S DISEASE
Authors: Noad Rupert , Newman Craig , Carroll Camille , Zajicek John 
Published: October 2015

BackgroundIn order to facilitate neuroprotective trials in Parkinson's disease (PD), there is a need for improved means of early disease detection and measuring disease progression. Computerised assessment may afford easier and increasingly accurate administration of motor and cognitive tests, as well as measurement of indices not readily accessible with standard testing paradigms.AimTo develop and validate iPad-based cognitive and motor measures in PD.Method62 PD patients and 42 age-matched controls completed traditional and iPad versions of the Trail Making Test (TMT) (executive function) and Knox Cube Test (visual memory), as well as a range of other measures.ResultsMedian age was 68 years; median MOCA score was 26. No participant had previously used an iPad. There was significant correlation between traditional and iPad measures: TMTa r=0.74, p<0.001; TMTb r=0.76, p<0.001; Knox r=0.63, p<0.001. Usability data were strong, 90% of participants providing positive feedback.ConclusionThis initial study has demonstrated that two iPad-based measures of cognition are acceptable to PD patients and perform similarly to traditional pen-and-paper tests. Further work will extend the analysis of the measured indices in longitudinal studies to determine correlation with disease progression, and extend the battery of iPad-based tests available for PD assessment.
ECG ABNORMALITIES IN ALTERNATING HEMIPLEGIA: A BROADENED PHENOTYPE
Authors: Jaffer Fatima , Avbersek Andreja , IAHRC  , ENRAH  , Hanna Michael G , Houlden Henry , Kaski Juan P , Cross Helen , Sisodiya Sanjay M 
Published: October 2015

IntroductionAlternating hemiplegia of Childhood (AH) caused by de novo mutations in ATP1A3, is characterised by recurrent plegic and dystonic episodes, seizures, non-paroxysmal neurological features, and dysautonomia. Premature mortality is observed and attributed to sudden unexpected death in epilepsy. A single case of asystole in AH is reported. Other neuronal channelopathies have cardiac involvement, and since ATP1A3 is expressed in cardiomyocytes, we hypothesised that ECG abnormalities are present in AH.Methods52 patients fulfilling the diagnostic criteria for AH were recruited, and screened for ATP1A3 mutations. ECGs were analysed for heart rate, cardiac axis, PR, QRS, RR, and QTc intervals, repolarisation patterns, and J-point abnormalities.Results53.8% exhibited abnormal resting 12-lead ECGs independent of seizures or AH episodes; 48.1% had repolarisation abnormalities. The proportion of intraventricular conduction delay or partial right bundle branch block was greater in this cohort compared to the general population. ECG abnormalities were significantly greater in patients >16 years than in those <16 years (p=0.0095), and also within the most common mutation group (D801N; p=0.045).ConclusionsThese ECG abnormalities reflect impaired repolarisation reserve in AH in an age-dependent manner, and may account for some cases of premature mortality observed. Long-term cardiac surveillance in AH is recommended.
DISCRIMINATION IN DYSTONIA: TIME FOR A RETHINK?
Authors: Sadnicka Anna , Daum Corinna , Manohar Sanjay , Rothwell John , Edwards Mark 
Published: October 2015

IntroductionAbnormal temporal discrimination thresholds (TDT) are thought to reflect abnormal temporal processing in dystonia. Despite widespread use of the TDT paradigm the task has been developed little since its inception; even though current paradigms may test additional factors unrelated to temporal processing.Method22 healthy subjects and 22 subjects with cervical dystonia (CD) were tested with two novel tasks. The first task ‘gap detection’ was a forced choice version of the TDT. Complicating spatial elements of the paradigm were removed and modelling data psychophysically increased the dimensionality of analysis informing on task performance. The second task ‘interval discrimination’ tested the ability to classify the length of two successive intervals.ResultsGap detection Equality of performance between groups was seen for general markers of performance such as hit rate, false positive rate and modelled psychometric function parameters. However, subjects with CD were significantly slower to make decisions. Interval discrimination No difference in the sensitivity between groups to classify differences in intervals was seen.ConclusionsThese results are surprising and challenge currently held concepts about the significance of previously documented abnormalities of TDTs in which a simple timing deficit is widely assumed. We hope that the ensuing discussion re-invigorates work to define the true biological correlate of TDT anomalies in dystonia as this will provide critical insight into its pathogenesis.
Optimizing Anti-EGFR Therapy in Colorectal Cancer
Authors: Salazar Ramon , Ciardiello Fortunato 
Published: October 2015

Abstract
Treatment with anti-EGFR monoclonal antibodies has been successfully integrated in the continuum of care for metastatic colorectal cancer. The major challenge is the identification of patients who would benefit from treatment. Currently, the best predictor of efficacy is the absence of mutations in KRAS and NRAS genes. Clin Cancer Res; 21(24); 5415–6. ©2015 AACR.
See related article by Peeters et al., p. 5469
Speculative Propositions: A Visual Pattern Sampler
Authors: Syjuco Stephanie 
Published: October 2015

Abstract
During World War I, a peculiar example of disruptive patterning was developed to adorn British and American battleships. “Dazzle camouflage” as it was known, did little to “hide” the vessels themselves. Rather, its function was to confuse enemy aim by utilizing chaotic black-and-white patterns. Vintage photographs of these ships provide startling visuals of a kind of graphical warfare. At first glance, the extreme angles and cutout shapes conjure everything from European Modernist abstraction, Russian Constructivism, and colonial ethnic and tribal patterning, to later forms of Op art and design. As an artist researching these images, I began speculating on the side-effects of their routes as global visual transmitters of conquest and empire. What if these patterns, likened to war paint and symbolic markers of dominance, were altered to show their transmission onto other forms, such as modern architecture, commodities, and trade/transit routes—a sort of cross-pollination of hybridity and influence across cultures and continents? How can these patterns be employed by unexpected “clients” of economic and cultural colonialism? How can colonized forms misappropriate this visual technique for themselves? Speculative Propositions: A Visual Pattern Sampler is the result of this exploration.
Quantifying dominance of adult female white-tailed deer in the presence of abundant food
Authors: Michel Eric S. , Demarais Stephen , Strickland Bronson K. , Belant Jerrold L. , Millspaugh Joshua J. 
Published: October 2015

Dominance is a behavioural mechanism that allows individuals to access and monopolize resources which should ultimately improve their fitness. Hierarchy strength should be strongest when resources are limited; however, this relationship is not consistent. We provided abundant food to assess whether hierarchy strength was consistent with resource abundance using 9 groups of captive female white-tailed deer (Odocoileus virginianus). We further assessed how body mass, age and testosterone levels were associated with rank position. Deer displayed a weak hierarchy with a mean linearity () of 0.39 (SD = 0.09) and a mean directional consistency index of 0.83 (SD = 0.06). Rank was related to body mass (, slope = 0.011), but not age or testosterone levels (). We demonstrate that hierarchy strength was weak in the presence of abundant food resources and suggest the possibility that dominance is a plastic behaviour that may vary with resource abundance.
The influence of storage time and temperature on the measurement of serum, plasma and urine osmolality
Authors: Bezuidenhout Karla , Rensburg Megan A , Hudson Careen L , Essack Younus , Davids M Razeen 
Published: October 2015

Background Many clinical laboratories require that specimens for serum and urine osmolality determination be processed within 3 h of sampling or need to arrive at the laboratory on ice. This protocol is based on the World Health Organization report on sample storage and stability, but the recommendation lacks good supporting data. We studied the effect of storage temperature and time on osmolality measurements. Methods Blood and urine samples were obtained from 16 patients and 25 healthy volunteers. Baseline serum, plasma and urine osmolality measurements were performed within 30 min. Measurements were then made at 3, 6, 12, 24 and 36 h on samples stored at 4–8℃ and room temperature. We compared baseline values with subsequent measurements and used difference plots to illustrate changes in osmolality. Results At 4–8℃, serum and plasma osmolality were stable for up to 36 h. At room temperature, serum and plasma osmolality were very stable for up to 12 h. At 24 and 36 h, changes from baseline osmolality were statistically significant and exceeded the total allowable error of 1.5% but not the reference change value of 4.1%. Urine osmolality was extremely stable at room temperature with a mean change of less than 1 mosmol/kg at 36 h. Conclusions Serum and plasma samples can be stored at room temperature for up to 36 h before measuring osmolality. Cooling samples to 4–8℃ may be useful when delays in measurement beyond 12 h are anticipated. Urine osmolality is extremely stable for up to 36 h at room temperature.
Optimization of scan timing for aortic computed tomographic angiography using the test bolus injection technique
Authors: Hoshino Takashi , Ichikawa Katsuhiro , Hara Takanori , Terakawa Shoichi , Hosomi Kazuhiro , Nishimura Kenji , Takayama Katsutoshi 
Published: October 2015

Background With fast computed tomography (CT), it is possible for the scanning to outpace the contrast medium bolus during aortic CT angiography (CTA). Purpose To evaluate the effectiveness of a new method for reducing the risk of outpacing in which the scan start timing ( ST) and speed can be estimated from the peak enhancement time measured at the femoral artery using a single test-bolus injection (femoral artery test injection method [FTI method]). Material and Methods In 30 cases of aortic CTA, we measured the time to peak enhancement at the femoral artery ( TPF) and the ascending aorta ( TPA) with test-bolus injection performed twice in each examination. From the resultant linear relationship between TPF and transit time ( TT =  TPF −  TPA), we developed a method for determining the ST and TT from TPF. One hundred patients were assigned to two groups: FTI and bolus tracking (BT), each with 50 patients. CT values were measured in main vessels (ascending aorta, descending aorta, femoral artery). The CT values of the vessels and the rate of cases with more than 300 HU (good cases) were compared between the two groups. Results The enhancement in the FTI method was significantly higher than that of the BT method (average CT values: FTI, 388.3 ± 52.4; BT, 281.2 ± 59.1; P < 0.001). The rates of good cases for FTI and BT were 86.0% and 46.0%, respectively. Conclusion The FTI method was very effective in reducing the risk of outpacing of the contrast medium transit in aortic CTA without the need for an additional contrast medium dose.
Male-Female Differences in Knee Laxity and Stiffness
Authors: Boguszewski Daniel V. , Cheung Edward C. , Joshi Nirav B. , Markolf Keith L. , McAllister David R. 
Published: October 2015

Background: It has been reported that over 70% of anterior cruciate ligament (ACL) injuries occur in noncontact situations and that females are at 2 to 8 times greater risk of ACL injury than males. Increased joint laxity and reduced knee stiffness in female knees have been suggested as possible explanations for the higher ACL injury rates in females. Hypothesis: Compared with male knees, female knees will demonstrate increased laxity and reduced stiffness along the anterior-posterior (AP), internal-external (IE), and varus-valgus (VV) directions. Study Design: Controlled laboratory study. Methods: Forty-seven fresh-frozen human cadaveric knees were tested (22 male and 25 female) by use of a robotic system. Mean ages were 34.6 years (range, 19-45 years) for males and 28.4 years (range, 16-42 years) for females. Joint laxity and stiffness were measured from force-vs-displacement or torque-vs-rotation curves recorded for 3 modes of testing: ±134 N AP force, ±5 N·m IE torque, and ±10 N·m VV moment. Results: Compared with male knees, female knees had greater internal laxity from 0° to 50° flexion ( P < .01; maximum difference of 8.3° at 50° of flexion) and greater valgus laxity from 0° to 50° of flexion ( P < .05; maximum difference of 1.6° at 50° of flexion). However, female knees exhibited greater anterior laxity only at 50° of flexion ( P < .03; difference of 1.3 mm). No significant male-female differences in anterior or posterior stiffness were found. Male knees had 42% greater internal stiffness from 0° to 30° of flexion ( P < .03), 35% greater valgus stiffness at 10° of flexion ( P < .03), and 19% greater varus stiffness at 50° of flexion ( P < .03). Conclusion: Female knees demonstrated significantly increased laxity and reduced stiffness compared with males. This finding was not uniform but was dependent on the direction tested and the knee flexion angle. Clinical Relevance: Understanding the risk factors for noncontact ACL injury is important for injury prevention. In combination with other female-specific risk factors, increased knee laxity may be a contributing factor associated with the higher rate of female ACL injuries.
Palliation of Truncus Arteriosus Associated With Complete Atrioventricular Canal—Results of Single Ventricle Palliation
Authors: He Dingchao , Olivieri Laura J. , Jonas Richard A. , Sinha Pranava 
Published: October 2015

Background: Truncus arteriosus associated with complete atrioventricular canal defect is rare and continues to be a surgical challenge with high morbidity and mortality. In the absence of extension of the ventricular septal defect to the outlet septum, biventricular repair is precluded, and single ventricle palliation remains the only option. We present our experience with five patients with truncus arteriosus and complete atrioventricular canal defect who underwent single ventricular palliation. Methods: Five patients with truncus arteriosus and complete atrioventricular canal defect managed along the single ventricle palliation pathway were retrospectively reviewed. Demographic, echocardiographic, cardiac catheterization, and perioperative data were analyzed. Results: All patients underwent neonatal palliation tailored to their anatomy (excision of pulmonary arteries from the common trunk and systemic to pulmonary artery shunt in two patients, excision of pulmonary artery and right ventricle to pulmonary artery conduit in two patients, and bilateral branch pulmonary artery bands in one patient). There were two early deaths after neonatal palliation. At a median follow-up of 210 days (interquartile range 1,210 days), all three survivors have undergone second-stage palliation. Of these patients, one is interstage II to III, and two patients have completed their Fontan procedure and are doing well. Conclusion: Truncus arteriosus with complete atrioventricular canal defect is a rare combination of defects. Single ventricle palliation pathway with a tailored neonatal approach may be employed for patients with uncommitted ventricular septal defects.
Are We Overdiagnosing Acute Kidney Injury in Pediatric Patients Following Cardiac Surgery?
Authors: Soni Meshal , Piggott Kurt D. , DeCampli William , Ramirez Jorge , Pourmoghadam Kamal , Fakioglu Harun , Blanco Carlos 
Published: October 2015

Background: The use of two diagnostic criteria in the current literature has led to some degree of ambiguity in the precise diagnosis of acute kidney injury in pediatric patients undergoing surgery for congenital heart disease. This study aims to determine which criteria is the most accurate diagnostic indicator of acute kidney injury and determine whether the incidence is being overestimated based on the current criteria. Methods: This retrospective study consisted of 389 patients with congenital heart disease from birth to 18 years, who underwent cardiac surgery. The statistical tests conducted were the student t test and chi-square test. Outcomes measured included hospital length of stay, duration of mechanical ventilation, and mortality. Results: The incidence rate of acute kidney injury diagnosed by the pediatric Risk, Injury, Failure, Loss, and End-Stage Renal Disease (RIFLE) criterion was 56% compared to 24.4% for the Acute Kidney Injury Network criterion. The pediatric RIFLE criterion consists of the following subsets: risk, injury, failure, loss, and end-stage renal disease. Patients classified in the “risk” subset of the pediatric RIFLE criterion who failed to meet Acute Kidney Injury Network criterion were compared to patients without acute kidney injury. Comparison of intensive care unit outcomes between these groups lacked statistical significance for all variables except the duration of mechanical ventilation postoperatively. Conclusion: Although recent research in this field identified the pediatric RIFLE criterion as the most sensitive indicator of acute kidney injury, the results of this study suggest the pediatric RIFLE criterion overestimates acute kidney injury incidence and that the Acute Kidney Injury Network criterion is the more accurate diagnostic indicator.
Gelation Properties of Aprotic Low-Molecular-Mass Organic Gelators Based on a Gemini 4-[2-(Perfluoroalkyl)ethylsulfanyl]phenoxy Derivative Unit
Authors: Yoshida Tomohiro , Hirakawa Tomomi , Nakamura Toru , Yamada Yasuhiro , Tatsuno Hiroko , Hirai Masayuki , Morita Yuki , Okamoto Hiroaki 
Published: October 2015

Abstract
Gelation properties of aprotic low-molecular-mass organic gelators (LMOGs) based on a gemini 4-[2-(perfluoroalkyl)ethylsulfanyl]phenoxy derivative unit are described. These LMOGs without hydrogen-bonding functional groups produced physical gels in several organic solvents such as ethanol, 1-octanol, acetonitrile, DMF, DMSO, propylene carbonate, and γ-butyrolactone at low concentration below 1 wt %. From the results of microscope observation and nuclear magnetic resonance spectroscopy, these aprotic LMOG molecules were aggregated into tape-shaped molecular nanofibers, and formed three-dimensional networks. In addition, the self-diffusion coefficient of the solvent molecules in these networks was similar to that of neat, although these solvent molecules have non-fluidity by gelation.
Selective Synthesis of Nanospheres and Nanosheets of Bismuth Subcarbonate
Authors: Jeon Seong Gi , Park Sun Hwa , Yu Jin , Song Jae Yong 
Published: October 2015

Abstract
Bismuth subcarbonate ((BiO)2CO3) nanospheres (150 nm in diameter) and nanosheets (5 nm thickness) were selectively synthesized in ultra-dilute bismuth nitrate solutions of 1 to 100 µM. Bismuth ions react with carbonic acid, which is formed by CO2 dissolved in water, and (BiO)2CO3 nanostructures are synthesized by the reaction of BiONO3 and carbonic acid. The morphologies of the nanostructures are controlled by the reaction kinetics, proportional to the concentration of reactant ions; lower concentration leads to slower reaction and isotropic growth of nanospheres, while higher concentration causes faster reaction in the direction of higher indexed plane, and nanosheets are synthesized.