bluuebunny/crossref_metadata_2025_split huggingface dataset:
VERY EARLY ONSET MULTIPLE SCLEROSIS
Authors: Cole G. F. , Auchterlonie L A. , Best P. V. 
Published: July 2010

SUMMARYThe authors report two cases of multiple sclerosis occurring in very young children. Age at onset in one child was 13 months; he had the primary progressive form of the condition and died at nine years. Age at onset of the other child was two years; he has the classical relapsing‐remitting form and is well at 15 years. These children illustrate the broad clinical spectrum of the condition even in very‐early‐onset disease.RÉSUMÉDébut trés précoce de sclérose en plaque.Les auteurs rapportent deux cas de sclérose en plaque apparus chez de trés jeuncs enfants. Lâge de début pour L'un des enfants était de 13 mois; il présentait une forme ďemblée progressive et mourut á lâge de neuf ans. ľâge de début pour ľautre enfant fut de deux ans; il présenta la forme classique avec des rémissions et rechute, et il est en bon état à 15 ans. Ces deux cas illustrent le large spectre clinique de ľaffection, même en cas de début très précoce.ZUSAMMENFASSUNGMultiple Sklerose mil sehr frühem BeginnDie Autoren beriehten über zwei Fälle, bei denen die Multiple Sklerose sehr früh auftrat. Das Alter des einen Kindes war 13 Monate; es hatte die ehronisch progrediente Form und starb mit neun Jahren. Das Alter des anderen Kindes betrug zwei Jahre; dieses hat die klassische in Schüben verlaufende form mit Remissionen und ist mit 15 Jahren wohlauf. Diese Kinder zeigen das breite klinische Spektrum der Erkrankung, selbst wenn sie sehr früh beginnt.RESUMENEsclerosois múltiple de inicio muy precoz: dos casos que ilustran el amplio espectro de la gravedad clínicaLos autores aportan dos casos de esclerosis múltiple aparecida en niños muy pequeños. La edad de inicio en uno era de 13 meses; tovo la primera forma progresiva de la enfermedad y murió a los nueve años. La edad de inicio en el otro niño fue de dos años. Sufrió la forma clásica de broteremisión y se encuentra bien a los 15 años. Estos casos ilustran el amplio espectro clínico de la enfermedad. incluyendo un inicio muy precoz de la misma.
IQ AND ITS PREDICTORS IN CHILDHOOD HEMIPLEGIA
Authors: Goodman Robert , Yude Carole 
Published: July 2010

SUMMARYIn a population‐based sample of 149 children with hemiplegia. IQ was highly correlated with a simple‐to‐calculate index of neurological severity. Performance IQ was an averâge of 13 points lower then verbal IQ ‐ a difference that seemed to reflect specific visuospatial difficulties rather than an effect of the motor disability itself. Side of lesion was not significantly related to either IQ or verbal‐performance discrepancy. Neurological factors and social class had additive effects on IQ, suggesting that neurological damâge neither amplified nor obliterated the impact of ordinary psychosocial and genetic factors on IQ.RÉSUMÉLe QI et sa valeur predictive dans I'hemiplegie infantileSur un échantillon de population de 149 enfants hémiplégiques, le QI était hautement corrélé avee un index de simple calcul de la gravite neurologiquc. Le QI de performance etait en moyenne de 13 points inferieur au QI verbal, une difference qui semblait traduire des diffcultes visuo‐spatiales specifiques plutot que l'incapacitd motrice par ellc‐meme. Le cote de la lesion n'etait relie significativement, ni aux QI verbal ou performance, ni a la difference verbal‐performance. Les facteurs ncurologiqucs et la classe sociale avaient des effcts additionnels sur le QI, suggerant que le dommâge neurologique n'amplifie ni masque l'impact des facteurs psychosociaux et genetiques habitucls sur le QLZUSAMMENFASSUNGIQ und seine prognostischen Faktdren bei Kindern mil HemiplegieBei einer Gruppe von 149 Kindern mit Hemiplegie bestand eine deutliche (Correlation zwischen IQ und einem emfach zu berechnenden Index für den neurologischen Schweregrad. Der Handlungs‐IQ war im Durchschnitt 13 Punkte niedriger als der verbale IQ ‐ ein Unterschied, der cher Ausdruck speziellcr visuell‐riiumlicher Storungen als der motorischen Stoning selbst war. Die Seite der Lasion spielte keine signifikante Rolle, weder für den IQ noch für die Diskrcpanz zwischen verbalem ‐ und Handlungs‐IQ. Neurologische Faktoren und sozialer Status hatten einen additiven Effeckt auf den IQ. was vcrmuten laBt, daß die neurologische Schadigung das Gewicht der allgemeinen psychosozialen und genetischen Faktoren auf den IQ weder verstarkt noch vermindert.RESUMEN CI y sus predictores en la hemiplegia infantilEn una poblacion de 149 niños con hemiplegia, el CI estaba altamente correlacionado con el indice de gravedad neurologica, facilmente calculable. El CI de realizacion era de un promedio de 13 puntos por debajo del verbal. Esta diferencia parecc reflejar dificultades visuo‐espaciales espeefficas, mas que un efecto de la disfuncion motora en si. El lado de la lesi6n no estaba significativamente en relacion con el CI o con la discrepancia cntre el CI verbal y el de realizacion. Los factores neurologicos y la clase social tenian efectos aditivos sobre cl CI, lo que sugiere que el dafio neurologico ni amplificaba ni obliteraba el impacto de los factores ordinaries psicosociales y geneticos sobre el Cl.
Cortical Dysgenesis in a Patient with Turner Mosaicism
Authors: Terao Yasuo , Hashimoto Kouichi , Nukina Nobuyuki , Mannen Toru , Shinohara Tamiko 
Published: July 2010

SUMMARYIn a patient with Turner mosaicism who had mental retardation, epilepsy and cerebellar ataxia, MRI showed cerebellar atrophy and a bizarre cortical dysgenesis of the cerebrum, which was considered to comprise a mixture of relatively normal gyri and structures resembling pachygyria and Iissencephaly. The karyotype of the patient was 45.X/47.XXX, but the brain dysgenesis could not be explained solely on the basis of this mosaicism, which is rarely associated with a gross abnormality in brain pathology. Abnormality of the X chromosome seems to have some potential for inducing cortical dysgenesis, and this case may be partially attributable to an abnormal locus on the X chromosome.RÉSUMÉDysgénésie corticale chez un sujei avec un Turner mosaïqueChez un patient avec Turner mosaïque présentant un retard mental, une épilepsie et une ataxie cérébelleuse, l'IRM révéla une atrophie cérébelleuse et une dysgénésie corticale bizarre du cerveau, qui fut considérée comme un mélange d'un gyrus relativement normal et de structures ressemblant à une pachygyrie et une lissencéphalie. Le caryotype du patient était 45,X/47,XXX, mais la dysgénésie cérébrale ne pouvait pas étre expliquée seulement par la mosaïque, rarement associée à une anomalie sévère dans la pathologie cérébrale. L'anomalie du chromosome X semblc présenter un potentiel d'induction de la dysgénésie corticale, et ce cas peut étre partiellement attribuable à un locus anormal du chromosome X.ZUSAMMENFASSUNGKortikale Dysgenesie bei einem Patienten mil Turner MosaikBei einem Patienten mit Turner Mosaik mit geistiger Retardierung, Epilepsie und cerebellärer Ataxie fand sich im MRI eine cerebelläre Atrophie und cine seltsame kortikale Dysgenesie des Gehirns, was als eine Mischung aus relativ normalen Gyri und Strukturen wie bei der Pachgyrie und Lissenzephalie angesehen wurde. Der Karyotyp des Patienten war 45, X / 47, XXX, aber die Dysgenesie ließ sich nicht nur auf der Basis dieses Mosaiks erklären, da bei diesem selten auffällige Anomalien in der Hirnpathologie auftreten. Durch eine Anomalie des X Chromosoms könnte eine kortikale Dysgenesie hervorgerufen werden, und dieser Fall könnte einem abnormen Locus auf dem X Chromosom zugeschrieben werden.RESUMENDysgenesia conical en un paciente con mosaicismo de TurnerEn un caso de mosaicismo de Turner con retraso mental, epilepsia y ataxia cerebclosa. la IRM mostró una atrofia cercbelosa y una disgenesia cortical rara que comprendía una mezcla de circulvoluciones relativamerite normales y estructural que semejaban paquigiria y lisencefalia. El cariotipo del caso era 45.X/47.XXX, pero la disgenesia cerebral no podía ser expresada nicamente sobre la base del mosaicismo, que raramente va asociado a anomalias groseras del cerebro. La anomalia del cromosoma X parece tener una capacidad para inducir una disgenesia cortical y este caso puede ser parcialmente atribuible a un locus anomalo en el cromosoma X.
Book Reviews
Authors: Jan J E , Wiggs Luci 
Published: July 2010

Book reviewed in this article:The Developing Visual Brain By Janette Atkinson Oxford University Press: Oxford Psychology Series No 32, First published 2000. First published in paperback 2002, pp 211Awakening and Sleep‐Wake Cycle Across Development Edited by Piero Salzarulo and Gianluca Ficca Amsterdam, John Benjamins Publishing Company, 2002, pp 299
Spatial layout of letters in nonwords affects visual short‐term memory load: Evidence from human electrophysiology
Authors: Prime David , Dell'acqua Roberto , Arguin Martin , Gosselin Frédéric , Jolicœur Pierre 
Published: July 2010

AbstractThe sustained posterior contralateral negativity (SPCN) was used to investigate the effect of spatial layout on the maintenance of letters in VSTM. SPCN amplitude was measured for words, nonwords, and scrambled nonwords. We reexamined the effects of spatial layout of letters on SPCN amplitude in a design that equated the mean frequency of use of each position. Scrambled letters that did not form words elicited a larger SPCN than either words or nonwords, indicating lower VSTM load for nonwords presented in a typical horizontal array than the load observed for the same letters presented in spatially scrambled locations. In contrast, prior research has shown that the spatial extent of arrays of simple stimuli did not influence the amplitude of the SPCN. Thus, the present results indicate the existence of encoding and VSTM maintenance mechanisms specific to letter and word processing.
Activity screening and structure–activity relationship of the hit compounds targeting APN/CD13
Authors: Wang Xuejian , Jing Fanbo , Zhu Huawei , Fang Hao , Zhang Jian , Xu Wenfang 
Published: July 2010

AbstractAminopeptidase N (APN) plays an important role in tumor progression, which participates in the progress such as proliferation, attachment, angiogenesis, and tumor invasion. All of this makes APN as a good chemical therapeutic anti‐tumor target. In this study, a series of chemically synthesized APN inhibitors were tested for the anti‐tumor activities, and three most effective compounds were chosen according to the MTT assay. Then, the enzyme inhibitory, anti‐tumor, specificity, angiogenesis, and invasion were determined to evaluate the activity of these three compounds. All compounds can markedly inhibit the enzyme activity of APN, angiogenesis of endothelial cells, and the invasion of ES‐2 cells. And it had little effect on the viability of K562 which express low level of APN. This data indicated that the tested compounds were APN hit compounds. We also did kinetic assay to determine the inhibition constant and constructed a three‐dimensional quantitative structure–activity relationship model to analyze the structure–activity relationship to direct the further design of novel APN inhibitors as anti‐tumor agents. These data demonstrate that the tested compounds can be developed as novel candidates of anticancer agent.
Differential Effect of Helicobacter pylori Eradication on Time‐Trends in Brady/Hypokinesia and Rigidity in Idiopathic Parkinsonism
Authors: Dobbs Sylvia M. , Dobbs R. John , Weller Clive , Charlett André , Bjarnason Ingvar T. , Lawson Andrew J. , Letley Darren , Harbin Lucy , Price Ashley B. , Ibrahim Mohammad A. A. , Oxlade Norman L. , Bowthorpe James , Leckstroem Daniel , Smee Cori , Plant J. Malcolm , Peterson Dale W. 
Published: July 2010

AbstractBackground:  We examine the effect of eradicating Helicobacter in idiopathic parkinsonism (IP). Marked deterioration, where eradication‐therapy failed, prompted an interim report in the first 20 probands to reach de‐blinding. The null‐hypothesis, “eradication has no effect on principal outcome, mean stride length at free‐walking speed,” was rejected. We report on study completion in all 30 who had commenced post‐treatment assessments.Methods:  This is a randomized, placebo‐controlled, parallel‐group efficacy study of eradicating biopsy‐proven (culture and/or organism on histopathology) Helicobacter pylori infection on the time course of facets of IP, in probands taking no, or stable long‐t½, anti‐parkinsonian medication. Persistent infection at de‐blinding (scheduled 1‐year post‐treatment) led to open active eradication‐treatment.Results:  Stride length improved (73 (95% CI 14–131) mm/year, p = .01) in favor of “successful” blinded active over placebo, irrespective of anti‐parkinsonian medication, and despite worsening upper limb flexor rigidity (237 (57–416) Nm × 10−3/year, p = .01). This differential effect was echoed following open active, post‐placebo. Gait did not deteriorate in year 2 and 3 post‐eradication. Anti‐nuclear antibody was present in all four proven (two by molecular microbiology only) eradication failures. In the remainder, it marked poorer response during the year after eradication therapy, possibly indicating residual “low‐density” infection.We illustrate the importance of eradicating low‐density infection, detected only by molecular microbiology, in a proband not receiving anti‐parkinsonian medication. Stride length improved (424 (379–468) mm for 15 months post‐eradication, p = .001), correction of deficit continuing to 3.4 years. Flexor rigidity increased before hydrogen‐breath‐test positivity for small intestinal bacterial overgrowth (208 (28–388) Nm × 10−3, p = .02), increased further during (171 (67–274), p = .001) (15–31 months), and decreased (136 (6–267), p = .04) after restoration of negativity (32–41 months).Conclusion:  Helicobacter is an arbiter of progression, independent of infection‐load.
Long‐term efficacy and safety of eslicarbazepine acetate: Results of a 1‐year open‐label extension study in partial‐onset seizures in adults with epilepsy
Authors: Halász Peter , Cramer Joyce A. , Hodoba Danilo , Członkowska Anna , Guekht Alla , Maia Joana , Elger Christian , Almeida Luis , Soares‐da‐Silva Patricio ,   on behalf of the BIA‐2093‐301 Study Group
Published: July 2010

SummaryPurpose:  To evaluate the long‐term efficacy and safety of once daily eslicarbazepine acetate (ESL) as adjunctive therapy for partial‐onset seizures in adults with epilepsy.Methods:  One‐year open‐label treatment extension with ESL in patients who completed a placebo‐controlled pivotal study (Epilepsia 2009; 50: 454–463). Starting dose was 800 mg once daily, for 4 weeks; thereafter, dose could be titrated up or down. Doses of concomitant antiepileptic drugs were to be kept stable.Results:  Overall, 314 patients were enrolled. The intent‐to‐treat population consisted of 312 patients, and 239 (76.6%) completed 1 year of treatment. ESL median dose was 800 mg once daily. Compared to baseline, median seizure frequency decreased by 39% during the first 4 weeks and between 48% and 56% thereafter. The responder rate (≥50% seizure reduction) was 41% during weeks 1–4 and, thereafter ranged between 48% and 53%. The proportion of seizure‐free patients per 12‐week interval ranged between 8.7% and 12.5%. Quality of life, as measured by the Quality of Life in Epilepsy Inventory‐31 (QOLIE‐31), and depressive symptoms, as measured by the Montgomery Asberg Depression Rating Scale (MADRS), improved significantly compared to baseline. Treatment‐emergent adverse events (TEAEs) were reported by 51% of patients. The most frequent TEAEs were headache (10%), dizziness (10%), diplopia (5%), and nasopharyngitis (5%). TEAEs were mostly (97%) of mild to moderate intensity. Eleven patients (3.5%) discontinued due to TEAEs. There were no results of laboratory tests raising safety concerns.Discussion:  Sustained therapeutic effect, favorable tolerability and safety, and an improvement in quality of life and depressive symptoms were observed during long‐term add‐on treatment of partial‐onset seizures in adults with once daily ESL.
Therapeutic time window of low‐frequency stimulation at entorhinal cortex for amygdaloid‐kindling seizures in rats
Authors: Xu Zheng‐Hao , Wu Deng‐Chang , Fang Qi , Zhong Kai , Wang Shuang , Sun Hong‐Liu , Zhang Shi‐Hong , Chen Zhong 
Published: July 2010

SummaryThe present study was designed to determine whether low‐frequency stimulation (LFS) of the entorhinal cortex (EC) has an anticonvulsive effect, and whether LFS delivered at different times plays different roles. We found that LFS of the EC immediately or 4 s after kindling stimulation had an anticonvulsive effect, and that the latter had a better effect on both kindling and kindled seizures. However, LFS delivered after the cessation of afterdischarge or 10 s after the kindling stimulation, augmented the epileptic activity. So the EC is a potential target for LFS to interfere with epilepsy. Our findings suggest that even in the duration of afterdischarge, there exists a “time window” for LFS treatment, indicating that the time delay of closed‐loop stimulation is crucial for LFS treatment.
Catheter Ablation of Electrical Storm Due to Monomorphic Ventricular Tachycardia in Patients with Nonischemic Cardiomyopathy: Acute Results and Its Effect on Long‐Term Survival
Authors: ARYA ARASH , BODE KERSTIN , PIORKOWSKI CHRISTOPHER , BOLLMANN ANDREAS , SOMMER PHILIPP , GASPAR THOMAS , WETZEL ULRIKE , HUSSER DANIELA , KOTTKAMP HANS , HINDRICKS GERHARD 
Published: July 2010

Background: Electrical storm due to recurrent ventricular tachycardia (VT) in patients with implantable cardioverter defibrillator (ICD) can adversely affect their long‐term survival. This study evaluates the efficiency of the radiofrequency catheter ablation of electrical storm due to monomorphic VT in patients with idiopathic dilated cardiomyopathy (DCM) and assesses its long‐term effects on survival.
Methods and Results: Between April 2004 and October 2008, 13 consecutive patients (nine men, mean age 56.8 ± 17.8 years) with DCM and electrical storm due to monomorphic VT who had ICD underwent 17 catheter ablation procedures, including four epicardial, at our center. Acute complete success was defined as the lack of inducibility of any VT at the end of procedure during programmed right ventricular stimulation and was achieved in eight patients (61.5%). During a median follow‐up of 23 months (range 3–63 months) nine patients (69%) were alive and eight patients (61.5%) were free from VT recurrence. Among those with acute complete (n = 8) and partial (n = 5) success, seven patients (87.5%) and one patient (20%) were free from any VT recurrence and ICD therapy, respectively (P = 0.025). Among those with acute complete and partial success, seven patients (87.5%) and two patients (40%) were alive, respectively (Mantel‐Cox test P = 0.082). Among those who had an initially failed endocardial ablation (n = 8), four underwent further epicardial ablation that was completely successful in three patients (75%).
Conclusion: Catheter ablation in patients with DCM and electrical storm due to monomorphic VT who had an ICD prevents further VT recurrence in 61.5% of the patients. Complete successful catheter ablation may play a protective role and was associated with reduced mortality during the follow‐up period. More aggressive ablation strategies in patients with initially failed endocardial ablation might improve the long‐term survival of these patients; however, further studies are needed to clarify this issue. (PACE 2010; 33:1504–1509)
ExHALED Study: Prevalence of smoking and harm levels in an emergency department cohort
Authors: Lynch Abigail , Quigley Paul 
Published: July 2010

AbstractObjective: To determine the prevalence of smoking among ED patients compared with the general New Zealand (NZ) smoking prevalence. Secondary outcomes were to determine smokers' level of nicotine dependence, readiness to quit and engagement with primary health care.Methods: This was a prospective, cross‐sectional prevalence study of ED patients seen consecutively over 6 days in Wellington Hospital, Wellington South, NZ. Medically stable patients ≥18 years were asked about their smoking habits by a closed‐question survey.Results: Five hundred and twenty‐eight patients comprised the study group. The ED smoking prevalence was 33.1% and higher than the general NZ smoking prevalence of 20.7%. Of those who smoked, 26.3% were ‘moderately’ to ‘very highly’ dependent on nicotine (Fagerstrom Test for Nicotine Dependence, FTND score ≥5). Of those who smoked, 74.9% stated they wanted to quit, 42.9% wanted to quit within the next month and 60.6% wanted an ED quit smoking pack. There were 13.6% of ED patients not registered with a general practitioner; of this, 61.1% were current smokers and 70.5% wanted to quit smoking.Conclusions: The prevalence rates of smoking are higher among patients attending Wellington Hospital ED than the general NZ population and the majority would like to quit smoking. One in four ED smokers have a high FTND score and are considered nicotine‐dependent. Many patients who were not registered with a general practitioner smoked, and the majority wanted to quit. Finally, there is significant interest from ED patients in receiving quit smoking packs from the ED.
Secular aberration drift and IAU definition of International Celestial Reference System
Authors: Titov Oleg 
Published: July 2010

Abstract
The gravitational attraction of the Galactic Centre leads to the centrifugal acceleration of the Solar system barycentre. It results in secular aberration drift which displaces the position of the distant radio sources. The effect should be accounted for in high-precision astrometric reductions as well as by the corresponding update of the International Celestial Reference System definition.
CHARACTERISTICS OF GELLAN GUM BASED FOOD GELS
Authors: SAHA DIPJYOTI , BHATTACHARYA SUVENDU 
Published: July 2010

ABSTRACT Fruit‐based gels with gellan gum as the gelling agent was prepared. Textural attributes of the gellan gum gels, formed with different concentrations of the gum (0.5–3.0%) and sugar and/or pineapple juice, were determined employing the methods of large‐deformation uniaxial compression and stress relaxation. Fracture stress/energy markedly increases with an increase in the concentration of gellan gum while fracture strain exhibits a marginal effect. The change in these compressive textural parameters is more pronounced for sugar added samples compared with gels without sugar. Marked decay in stress relaxation curves was observed; the extent of relaxation decreases marginally with an increase in gum content up to 2% but shows much lesser values beyond 2% addition. The sugar added samples exhibit lesser relaxation characteristics but higher relaxation times indicating elastic characteristics compared with samples without sugar. Use of gellan gum provides an innovative method for developing fruit juice based gels as a convenience food because of attractive transparent appearance and textural attributes. PRACTICAL APPLICATIONThe use of hydrocolloids like gellan gum can develop fabricated foods in the form of fruit juice based gels having unique textural properties. These properties can be altered to suit the consumer preference by varying the proportions of gellan gum, fruit juice and sugar. The results in the present investigation arising from compression and relaxation testing thus help in characterizing the developed gels.