bluuebunny/crossref_metadata_2025_split huggingface dataset:
Free Vibration of Rectangular Plates with Attached Discrete Sprung Masses
Authors: Zhou Ding , Ji Tianjian 
Published: January 2014

A direct approach is used to derive the exact solution for the free vibration of thin rectangular plates with discrete sprung masses attached. The plate is simply supported along two opposite edges and elastically supported along the two other edges. The elastic support can represent a range of boundary conditions from free to clamped supports. Considering only the compatibility of the internal forces between the plate and the sprung masses, the equations of the coupled vibration of the plate-spring-mass system are derived. The exact expressions for mode and frequency equations of the coupled vibration of the plate and sprung masses are determined. The solutions converge steadily and monotonically to exact values. The correctness and accuracy of the solutions are demonstrated through comparison with published results. A parametric study is undertaken focusing on the plate with one or two sprung masses. The results can be used as a benchmark for further investigation.The solution provided in the paper is general and includes several special cases, such as the plate with classical boundary conditions, the plate attached with discrete rigid masses, the plate supported by discrete springs and the plate restricted by rigid vertical point-supports.
The Cognitive and Behavioural Profile of Amyotrophic Lateral Sclerosis: Application of the Consensus Criteria
Authors: Consonni Monica , Iannaccone Sandro , Cerami Chiara , Frasson Paola , Lacerenza Marco , Lunetta Christian , Corbo Massimo , Cappa Stefano F. 
Published: January 2014

Objective:The study aims to assess the spectrum of cognitive and behavioural disorders in patients affected by Amyotrophic Lateral Sclerosis (ALS) according to the recent consensus criteria [9]. The study also intends to assess the impact of physical disability on cognitive and behavioural abnormalities.Methods:Detailed neurological, neuropsychological and neurobehavioral evaluations were administered to 23 ALS patients, 11 Lower Motor Neuron Disease (LMND) patients and 39 healthy controls. Strong et al.’s criteria [9] were applied to diagnose the presence of cognitive/behavioural impairment. Clinical and neuropsychological scores were used for group comparisons and correlation analyses.Results:In comparison with LMND and controls, a subgroup of ALS patients (∼30%) manifested executive dysfunction, which was severe enough to classify them as cognitively impaired. Action naming difficulties and short-term memory deficits were also observed. Aspontaneity, disorganization and mental rigidity reached clinical relevance in 20% of ALS patients. A small percentage of ALS patients (13%) also had comorbid dementia. The cognitive or behavioural status was not related to the clinical features of ALS.Conclusion:The use of consensus criteria for cognitive and behavioural impairment and the comparison with the LMND group proved useful in defining the spectrum of non-motor manifestations of ALS.
The Gilles De La Tourette Syndrome-Quality of Life Scale for Children and Adolescents (C&A-GTS-QOL): Development and Validation of the Italian Version
Authors: Cavanna Andrea E. , Luoni Chiara , Selvini Claudia , Blangiardo Rosanna , Eddy Clare M. , Silvestri Paola R. , Calì Paola V. , Seri Stefano , Balottin Umberto , Cardona Francesco , Rizzo Renata , Termine Cristiano 
Published: January 2014

Background:Gilles de la Tourette syndrome (GTS) is a chronic childhood-onset neuropsychiatric disorder with a significant impact on patients’ health-related quality of life (HR-QOL). Cavanna et al. (Neurology 2008; 71: 1410–1416) developed and validated the first disease-specific HR-QOL assessment tool for adults with GTS (Gilles de la Tourette Syndrome-Quality of Life Scale, GTS-QOL). This paper presents the translation, adaptation and validation of the GTS-QOL for young Italian patients with GTS.Methods:A three-stage process involving 75 patients with GTS recruited through three Departments of Child and Adolescent Neuropsychiatry in Italy led to the development of a 27-item instrument (Gilles de la Tourette Syndrome-Quality of Life Scale in children and adolescents, C&A-GTS-QOL) for the assessment of HR-QOL through a clinician-rated interview for 6–12 year-olds and a self-report questionnaire for 13–18 year-olds.Results:The C&A-GTS-QOL demonstrated satisfactory scaling assumptions and acceptability. Internal consistency reliability was high (Cronbach’s alpha > 0.7) and validity was supported by interscale correlations (range 0.4–0.7), principal-component factor analysis and correlations with other rating scales and clinical variables.Conclusions:The present version of the C&A-GTS-QOL is the first disease-specific HR-QOL tool for Italian young patients with GTS, satisfying criteria for acceptability, reliability and validity.
Motor Extinction in Distinct Reference Frames: A Double Dissociation
Authors: Heidler-Gary Jennifer , Pawlak Mikolaj , Herskovits Edward H. , Newhart Melissa , Davis Cameron , Trupe Lydia A. , Hillis Argye E. 
Published: January 2014

Objective:Test the hypothesis that right hemisphere stroke can cause extinction of left hand movements or movements of either hand held in left space, when both are used simultaneously, possibly depending on lesion site.Methods:93 non-hemiplegic patients with acute right hemisphere stroke were tested for motor extinction by pressing a counter rapidly for one minute with the right hand, left hand, or both simultaneously with their hands held at their sides, or crossed over midline.Results:We identified two distinct types of motor extinction in separate patients; 20 patients extinguished left hand movements held in left or right space (left canonical body extinction); the most significantly associated voxel cluster of ischemic tissue was in the right temporal white matter. Seven patients extinguished either hand held in left space (left space extinction), and the most significantly associated voxel cluster of ischemic tissue was in right parietal white matter.Conclusions:There was a double dissociation between left canonical body extinction and left space motor extinction. Left canonical body extinction seems to be associated with more dorsal (parietal) ischemia, and left canonical body extinction seems to be associated with more ventral (temporal) ischemia.
Verbal and Nonverbal Memory in Primary Progressive Aphasia: The Three Words-Three Shapes Test
Authors: Weintraub Sandra , Rogalski Emily , Shaw Emily , Sawlani Sabrina , Rademaker Alfred , Wieneke Christina , Mesulam M.-Marsel 
Published: January 2014

Objectives:To investigate cognitive components and mechanisms of learning and memory in primary progressive aphasia (PPA) using a simple clinical measure, the Three Words Three Shapes Test (3W3S).Background:PPA patients can complain of memory loss and may perform poorly in standard tests of memory. The extent to which these signs and symptoms reflect dysfunction of the left hemisphere language versus limbic memory network remains unknown.Methods:3W3S data from 26 patients with a clinical diagnosis of PPA were compared with previously published data from patients with typical dementia of the Alzheimer type (DAT) and cognitively healthy elders.Results:PPA patients showed two bottlenecks in new learning. First, they were impaired in the effortless (but not effortful) on-line encoding of verbal (but not non-verbal) items. Second, they were impaired in the retrieval (but not retention) of verbal (but not non-verbal) items. In contrast, DAT patients had impairments also in effortful on-line encoding and retention of verbal and nonverbal items.Conclusions:PPA selectively interferes with spontaneous on-line encoding and subsequent retrieval of verbal information. This combination may underlie poor memory test performance and is likely to reflect the dysfunction of the left hemisphere language rather than medial temporal memory network.
Change of Accent as an Atypical Onset of non Fluent Primary Progressive Aphasia
Authors: Paolini Susy , Paciaroni Lucia , Manca Antonio , Rossi Roberto , Fornarelli Daniela , Cappa Stefano F. , Abbatecola Angela M. , Scarpino Osvaldo 
Published: January 2014

Language disorders can be the first symptom of many neurodegenerative diseases, including Alzheimer's disease (AD) and primary progressive aphasia (PPA). The main variants of PPA are: the non-fluent/agrammatic variant, the semantic variant and the logopenic variant.Several additional variants of PPA, however, have been described and are considered as atypical presentations.We describe the case of a woman presenting a progressive isolated language disturbance, characterized by an early dysprosodia, phonological and semantic paraphasias, agrammatism, impairment in repetition, writing of non-words and sentence comprehension. This clinical picture pointed to an atypical presentation of the non-fluent variety. The frequent symptom overlap between the different variants of PPA, most likely reflecting differences in the topography of the pathological changes, needs to be considered in the definition of diagnostic criteria.
Neural Plasticity in Functional and Anatomical MRI Studies of Children with Tourette Syndrome
Authors: Eichele Heike , Plessen Kerstin J. 
Published: January 2014

Background:Tourette syndrome (TS) is a neuropsychiatric disorder with childhood onset characterized by chronic motor and vocal tics. The typical clinical course of an attenuation of symptoms during adolescence in parallel with the emerging self-regulatory control during development suggests that plastic processes may play an important role in the development of tic symptoms.Methods:We conducted a systematic search to identify existing imaging studies (both anatomical and functional magnetic resonance imaging [fMRI]) in young persons under the age of 19 years with TS.Results:The final search resulted in 13 original studies, which were reviewed with a focus on findings suggesting adaptive processes (using fMRI) and plasticity (using anatomical MRI). Differences in brain activation compared to healthy controls during tasks that require overriding of prepotent responses help to understand compensatory pathways in children with TS. Along with alterations in regions putatively representing the origin of tics, deviations in several other regions most likely represent an activity-dependent neural plasticity that help to modulate tic severity, such as the prefrontal cortex, but also in the corpus callosum and the limbic system.Discussion:Factors that potentially influence the development of adaptive changes in the brain of children with TS are age, comorbidity with other developmental disorders, medication use, IQ along with study-design or MRI techniques for acquisition, and analysis of data. The most prominent limitation of all studies is their cross-sectional design. Longitudinal studies extending to younger age groups and to children at risk for developing TS hopefully will confirm findings of neural plasticity in future investigations.
Functional Neuroanatomy and Behavioural Correlates of the Basal Ganglia: Evidence from Lesion Studies
Authors: Ward Peter , Seri Stefano , Cavanna Andrea Eugenio 
Published: January 2014

Introduction:The basal ganglia are interconnected with cortical areas involved in behavioural, cognitive and emotional processes, in addition to movement regulation. Little is known about which of these functions are associated with individual basal ganglia substructures.Methods:Pubmed was searched for literature related to behavioural, cognitive and emotional symptoms associated with focal lesions to basal ganglia structures in humans.Results:Six case-control studies and two case reports were identified as relevant. Lesion sites included the caudate nucleus, putamen and globus pallidus. These were associated with a spectrum of behavioural and cognitive symptoms, including abulia, poor working memory and deficits in emotional recognition.Discussion:It is often difficult to precisely map associations between cognitive, emotional or behavioural functions and particular basal ganglia substructures, due to the non-specific nature of the lesions. However, evidence from lesion studies shows that most symptoms correspond with established non-motor frontal-subcortical circuits.
Trials of Pharmacological Interventions for Tourette Syndrome: A Systematic Review
Authors: Waldon Karen , Hill Jonathan , Termine Cristiano , Balottin Umberto , Cavanna Andrea Eugenio 
Published: January 2014

Introduction:Gilles de la Tourette Syndrome (GTS) is a childhood-onset hyperkinetic movement disorder defined by the chronic presence of multiple motor tics and at least one vocal tic and often complicated by co-morbid behavioural problems. The pharmacological treatment of GTS focuses on the modulation of monoaminergic pathways within the cortico-striato-thalamo-cortical circuitry. This paper aims to evaluate the efficacy and safety profiles of pharmacological agents used in the treatment of tics in patients with GTS, in order to provide clinicians with an evidence-based rationale for the pharmacological treatment in GTS.Method:In order to ascertain the best level of evidence, we conducted a systematic literature review to identify double-blind randomised controlled trials of medications in GTS populations.Results:We identified a large number of pharmacological agents as potentially effective in improving tic symptoms. The alpha-2 agonist Clonidine is amongst the agents with the most favourable efficacy-versus-adverse events ratio, especially in patients with co-morbid attention-deficit hyperactivity disorder, although effect sizes vary evidence-based studies.Discussion:Our results are in line with the findings of uncontrolled open-label studies. However, most trials have low statistical power due to the small sample sizes, and newer agents, such as Aripiprazole, have not been formally tested in double-blind randomised controlled trials. Further research should focus on better outcome measures, including Quality of Life instruments.
Decrease of Mutual Information in Brain Electrical Activity of Patients with Relapsing-Remitting Multiple Sclerosis
Authors: Lenne Bruno , Blanc Jean-Luc , Nandrino Jean-Louis , Gallois Philippe , Hautecæur Patrick , Pezard Laurent 
Published: January 2014

The disturbance of cortical communication has been hypothesized as an important factor in the appearance of cognitive impairment in (MS). Cortical communication is quantified here in control subjects and patients with relapsing-remitting multiple sclerosis (RRMS) on the basis of mean coherence in theδ,θ,α,βandγbands and using mutual information computed between pairs of bipolar EEG signals recorded during resting condition. Each patient received also a cognitive assessment using a battery of neuropsychological tests specific to cognitive deficits in MS.No difference was observed for the coherence indices whereas inter-hemispheric and right hemisphere mutual information is significantly lower in patients with MS than in control subjects. Moreover, inter-hemispheric mutual information decrease significantly with illness duration and right mutual information differentiate cognitively deficient and non-deficient patients.Mutual information allows to quantify the cortical communication in patients with RRMS and is related to clinical characteristics.Cortical communication quantified in a resting state might be a potential marker for the neurological damage induced by RRMS.
Visual Hallucinations in PD and Lewy Body Dementias: Old and New Hypotheses
Authors: Onofrj M. , Taylor J. P. , Monaco D. , Franciotti R. , Anzellotti F. , Bonanni L. , Onofrj V. , Thomas A. 
Published: January 2014

Visual Hallucinations (VH) are a common non-motor symptom of Parkinson’s Disease (PD) and the Lewy body dementias (LBD) of Parkinson's disease with dementia (PDD) and Dementia with Lewy Bodies (DLB). The origin of VH in PD and LBD is debated: earlier studies considered a number of different possible mechanisms underlying VH including visual disorders, Rapid Eye Movement (REM) Sleep Intrusions, dysfunctions of top down or bottom up visual pathways, and neurotransmitter imbalance.More recently newer hypotheses introduce, among the possible mechanisms of VH, the role of attention networks (ventral and dorsal) and of the Default Mode Network (DMN) a network that is inhibited during attentional tasks and becomes active during rest and self referential imagery.Persistent DMN activity during active tasks with dysfunctional imbalance of dorsal and ventral attentional networks represents a new hypothesis on the mechanism of VH.We review the different methods used to classify VH and discuss reports supporting or challenging the different hypothetical mechanisms of VH.
Patterns of Cognitive Dysfunction in Progressive MS
Authors: Connick Peter , Chandran Siddharthan , Bak Thomas H. 
Published: January 2014

Background:Progressive MS is associated with a high frequency of cognitive impairment. However, it is not clear to what extent this reflects global dysfunction, or independent deficits in specific functions.Objective:To characterise patterns of cognitive impairment in progressive MS on a multi-dimensional cognitive assessment tool well established in neurodegenerative diseases.Methods:Patients with secondary (SPMS;n= 60) and primary progressive MS (PPMS;n= 28) were assessed using the Addenbrooke’s Cognitive Examination-Revised (ACE-R) multi-dimensional cognitive assessment scale. Independent dimensions of impairment and their relative contribution to the overall burden of cognitive dysfunction were then determined by factor analysis.Results:Two independent dimensions of impairment were seen: frontal-executive (attention, verbal fluency, recall) on one hand, and language and visuospatial functions on the other. These accounted for 55% and 45% respectively of the variance not explained by a global influence (14.2% and 11.6% respectively of total variance). Isolated language and visuospatial dysfunction was seen in both groups, whereas isolated impairment in frontal-executive functions was underrepresented in SPMS (p= 0.001) and not seen in PPMS patients (p= 0.040).Conclusions:In addition to a prominent global influence on cognitive performance, patients with progressive MS commonly exhibit language and visuospatial deficits. Evaluation of these abilities should therefore be included in clinical assessment of cognition in progressive MS.
Neuropsychological and Emotional Correlates of Personality Traits in Parkinson’s Disease
Authors: Koerts Janneke , Tucha Lara , Leenders Klaus L. , Tucha Oliver 
Published: January 2014

Parkinson’s disease (PD) is, apart from the well-known motor symptoms, also characterized by neuropsychological and emotional disturbances. However, patients also often present with a personality profile of low Novelty Seeking and high Harm Avoidance. This profile can be identified as the disease emerges, which raises the question whether these traits correlate with more fundamental neuropsychological and emotional disturbances. This study determined the neuropsychological and emotional correlates of Novelty Seeking, Harm Avoidance and two other personality traits that are often considered in PD, i.e. Reward Dependence and Persistence.Forty-three patients and 25 healthy participants were assessed with the Temperament and Character Inventory, a symptoms of depression questionnaire and neuropsychological tests.PD patients showed a higher Harm Avoidance than healthy participants, which was predicted by symptoms of depression. Groups did not differ regarding Novelty Seeking, Reward Dependence and Persistence. While cognitive flexibility was a predictor of Reward Dependence, Persistence was predicted by divergent thinking and inhibition. Novelty Seeking was not predicted by cognition or emotion.In conclusion, cognition and emotion are selectively related to personality traits in PD. Whereas Harm Avoidance covaries with emotional symptoms, Persistence and Reward Dependence are related to cognition. Alterations in personality, cognition and emotion in PD are thus not independent from each other.
Overexpression of the Transcription Factor MEF2D in Hepatocellular Carcinoma Sustains Malignant Character by Suppressing G2–M Transition Genes
Authors: Ma Leina , Liu Jia , Liu Limei , Duan Guangjie , Wang Qingliang , Xu Yanmin , Xia Feng , Shan Juanjuan , Shen Junjie , Yang Zhi , Bie Ping , Cui Youhong , Bian Xiu-Wu , Prieto Jesus , Avila Matías A. , Qian Cheng 
Published: January 2014

Abstract
The underlying molecular pathogenesis in hepatocellular carcinoma remains poorly understood. The transcription factor MEF2D promotes survival in various cell types and it seems to function as an oncogene in leukemia. However, its potential contributions to solid cancers have not been explored. In this study, we investigated MEF2D expression and function in hepatocellular carcinoma, finding that MEF2D elevation in hepatocellular carcinoma clinical specimens was associated with poor prognosis. MEF2D-positive primary hepatocellular carcinoma cells displayed a faster proliferation rate compared with MEF2D-negative cells, and silencing or promoting MEF2D expression in these settings limited or accelerated cell proliferation, respectively. Notably, MEF2D-silencing abolished hepatocellular carcinoma tumorigenicity in mouse xenograft models. Mechanistic investigations revealed that MEF2D-silencing triggered G2–M arrest in a manner associated with direct downregulation of the cell-cycle regulatory genes RPRM, GADD45A, GADD45B, and CDKN1A. Furthermore, we identified MEF2D as an authentic target of miR-122, the reduced expression of which in hepatocellular carcinoma may be responsible for MEF2D upregulation. Together, our results identify MEF2D as a candidate oncogene in hepatocellular carcinoma and a potential target for hepatocellular carcinoma therapy. Cancer Res; 74(5); 1452–62. ©2014 AACR.
Bufalin Is a Potent Small-Molecule Inhibitor of the Steroid Receptor Coactivators SRC-3 and SRC-1
Authors: Wang Ying , Lonard David M. , Yu Yang , Chow Dar-Chone , Palzkill Timothy G. , Wang Jin , Qi Ruogu , Matzuk Alexander J. , Song Xianzhou , Madoux Franck , Hodder Peter , Chase Peter , Griffin Patrick R. , Zhou Suoling , Liao Lan , Xu Jianming , O'Malley Bert W. 
Published: January 2014

Abstract
Virtually all transcription factors partner with coactivators that recruit chromatin remodeling factors and interact with the basal transcription machinery. Coactivators have been implicated in cancer cell proliferation, invasion, and metastasis, including the p160 steroid receptor coactivator (SRC) family composed of SRC-1 (NCOA1), SRC-2 (TIF2/GRIP1/NCOA2), and SRC-3 (AIB1/ACTR/NCOA3). Given their broad involvement in many cancers, they represent candidate molecular targets for new chemotherapeutics. Here, we report on the results of a high-throughput screening effort that identified the cardiac glycoside bufalin as a potent small-molecule inhibitor for SRC-3 and SRC-1. Bufalin strongly promoted SRC-3 protein degradation and was able to block cancer cell growth at nanomolar concentrations. When incorporated into a nanoparticle delivery system, bufalin was able to reduce tumor growth in a mouse xenograft model of breast cancer. Our work identifies bufalin as a potentially broad-spectrum small-molecule inhibitor for cancer. Cancer Res; 74(5); 1506–17. ©2014 AACR.