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showing 10 items of 7948 documents

The EGR2 gene is involved in axonal Charcot-Marie-Tooth disease

2015

Background and purpose A three-generation family affected by axonal Charcot−Marie−Tooth disease (CMT) was investigated with the aim of discovering genetic defects and to further characterize the phenotype. Methods The clinical, nerve conduction studies and muscle magnetic resonance images of the patients were reviewed. A whole exome sequencing was performed and the changes were investigated by genetic studies, in silico analysis and luciferase reporter assays. Results A novel c.1226G>A change (p.R409Q) in the EGR2 gene was identified. Patients presented with a typical, late-onset axonal CMT phenotype with variable severity that was confirmed in the ancillary tests. The in silico studies sho…

AdultMaleEarly Growth Response Protein 2In silicomedicine.disease_causeCharcot-Marie-Tooth diseaseSeverity of Illness Indexhereditary motor sensory neuropathywhole exome sequencingYoung AdultCharcot-Marie-Tooth DiseasemedicineEGR2 geneHumansExomeeducationGeneExomeExome sequencingEarly Growth Response Protein 2Genetic testingAgedGeneticsAged 80 and overeducation.field_of_studyMutationmedicine.diagnostic_testbusiness.industryMiddle AgedPhenotypeAxonsPedigreePhenotypeNeurologyMutationFemaleNeurology (clinical)business
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'Prefrontal' cognitive performance of healthy subjects positively correlates with cerebral FDOPA influx: an exploratory [18F]-fluoro-L-DOPA-PET inves…

2006

Dopamine neurotransmission influences those cognitive processes, which are generally regarded as prefrontal cortical functions. In previous positron‐emission‐tomography (PET) studies, net blood‐brain clearance of [(18)F]‐fluoro‐l‐DOPA (FDOPA) correlated with impaired cognitive performance in patients with Parkinson's disease or schizophrenia. We hypothesized that FDOPA influx also correlates with performance of cognitive tasks associated with prefrontal functioning in healthy volunteers. The net blood‐brain clearance of FDOPA (K [Formula: see text]) was mapped in a group of 11 healthy volunteers and calculated in striatal volumes‐of‐interest. The Wisconsin‐Card‐Sorting‐Test (WCST), Stroop‐T…

AdultMaleElementary cognitive taskMetabolic Clearance RateDopamineCaudate nucleusPrefrontal CortexStriatumNeuropsychological Testsbehavioral disciplines and activitiesSynaptic TransmissionBasal GangliaFunctional LateralityCognitionMesencephalonReference ValuesBasal gangliaNeural PathwaysReaction TimeHumansRadiology Nuclear Medicine and imagingPrefrontal cortexResearch ArticlesBrain MappingRadiological and Ultrasound TechnologyReceptors Dopamine D2PutamenCognitive flexibilityPutamenMiddle AgedDihydroxyphenylalanineNeurologyBlood-Brain BarrierPositron-Emission TomographyNeurology (clinical)AnatomyCaudate NucleusPsychologyNeuroscienceStroop effectHuman brain mapping
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Methylphenidate, modafinil, and caffeine for cognitive enhancement in chess: A double-blind, randomised controlled trial.

2017

Stimulants and caffeine have been proposed for cognitive enhancement by healthy subjects. This study investigated whether performance in chess - a competitive mind game requiring highly complex cognitive skills - can be enhanced by methylphenidate, modafinil or caffeine. In a phase IV, randomized, double-blind, placebo-controlled trial, 39 male chess players received 2×200mg modafinil, 2×20mg methylphenidate, and 2×200mg caffeine or placebo in a 4×4 crossover design. They played twenty 15-minute games during two sessions against a chess program (Fritz 12; adapted to players' strength) and completed several neuropsychological tests. Marked substance effects were observed since all three subs…

AdultMaleElementary cognitive taskmedicine.medical_specialtyModafinilAudiologyNeuropsychological TestsPlacebolaw.inventionDevelopmental psychology03 medical and health sciences0302 clinical medicineCognitionRandomized controlled trialDouble-Blind MethodlawCaffeinemedicineHumansPharmacology (medical)Benzhydryl CompoundsBiological PsychiatryRetrospective StudiesPharmacologyAnalysis of VarianceMethylphenidateModafinilNeuropsychologyCognitionWakefulness-Promoting AgentsMiddle AgedCrossover study030227 psychiatryPsychiatry and Mental healthCross-Sectional StudiesNeurologyMethylphenidateCentral Nervous System StimulantsFemaleNeurology (clinical)Psychologyhuman activities030217 neurology & neurosurgerymedicine.drugEuropean neuropsychopharmacology : the journal of the European College of Neuropsychopharmacology
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Cognitive and social cognitive functioning in spinocerebellar ataxia : a preliminary characterization

2006

INTRODUCTION : The spinocerebellar ataxias (SCAs), are rare neurodegenerative disorders caused by distinct genetic mutations. Clinically, the SCAs are characterised by progressive ataxia and a variety of other features, including cognitive dysfunction. The latter is consistent with a growing body of evidence supporting a cognitive as well as motor role for the cerebellum. Recent suggestions of cerebellar involvement in social cognition have not been extensively explored in these conditions. The availability of definitive molecular diagnosis allows genetically defined subgroups of SCA patients, with distinct patterns of cerebellar and extracerebellar involvement, to be tested comparatively u…

AdultMaleEmotionsNeuropsychological TestsSocial Environmentcognitive functioningDisability EvaluationCognitionSocial cognitionCerebellumTheory of mindmedicineHumansSpinocerebellar AtaxiasCognitive skillSocial BehaviorAgedIntelligence TestsVerbal BehaviorCognitive disorderNeuropsychologyRecognition PsychologyCognitionMachado-Joseph DiseaseMiddle Agedmedicine.diseaseNeurologyMental RecallSpinocerebellar ataxiaAutismFemaleAtaxiaNeurology (clinical)PsychologyNeurosciencePsychomotor Performance
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The importance of impulsivity and attention switching deficits in perpetrators convicted for intimate partner violence.

2018

It has been stated that Attention deficit hyperactivity disorder (ADHD) increases the likelihood of risky behavior such as intimate partner violence (IPV), but the cognitive mechanisms that facilitate or underlie these types of behavior remain unexplained. In this regard, several authors have established that impulsivity and inattentive symptoms might affect basic processes such as emotional decoding and set-shifting abilities, which are important processes for emotional and behavioral regulation. Hence, these symptoms entail a reduction in sensitivity to key contextual stimuli. Accordingly, the main aim of this study was to examine the involvement of impulsivity (assessed by self-reports) …

AdultMaleEmotionsPoison controlIntimate Partner Violence050109 social psychologyImpulsivityAffect (psychology)behavioral disciplines and activitiesCognitionArts and Humanities (miscellaneous)Wisconsin Card Sorting TestRisk FactorsDevelopmental and Educational PsychologymedicineAttention deficit hyperactivity disorderHumans0501 psychology and cognitive sciencesCognitive rehabilitation therapyGeneral Psychology05 social sciencesCognitive flexibilityCognitionmedicine.diseaseAttention Deficit Disorder with HyperactivityImpulsive BehaviorFemalemedicine.symptomPsychology050104 developmental & child psychologyClinical psychologyAggressive behavior
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A preliminary characterisation of cognition and social cognition in spinocerebellar ataxia types 2, 1, and 7.

2010

Over the last decade, studies have implicated the cerebellum not only in motor functioning, but also in cognition and social cognition. Although some aspects of cognition have been explored in the five most common forms of Spinocerebellar Ataxia (SCA), social cognition in these patients has rarely been examined. The present study provides a preliminary characterisation of the severity of cognitive and social cognitive impairments in patients with SCA2, SCA1 and SCA7 using an identical battery to the one previously used in SCA3 and SCA6 patients for comparison. The cognitive profiles of SCA1 and SCA7 patients were comparable to that of SCA6 patients; SCA1 patients had relatively intact profi…

AdultMaleEmotionsTheory of MindemotionNeurosciences. Biological psychiatry. NeuropsychiatryGeneral MedicineMiddle AgedNeuropsychological TestsNeuropsychology and Physiological PsychologyCognitionNeurologySocial PerceptionHumansSpinocerebellar AtaxiasSpinocerebellar ataxia (SCA)FemaleOtherNeurology (clinical)EmpathyErratumSocial BehaviorPsychomotor PerformanceRC321-571Behavioural neurology
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Neuronal and Behavioral Correlates of Health Anxiety: Results of an Illness-Related Emotional Stroop Task

2011

<b><i>Background:</i></b> Health anxiety (HA) is defined as the objectively unfounded fear or conviction of suffering from a severe illness. Predominant attention allocation to illness-related information is regarded as a central process in the development and maintenance of HA, yet little is known about the neuronal correlates of this attentional bias. <b><i>Methods:</i></b> An emotional Stroop task with body symptom, illness, and neutral words was employed to elicit emotional interference in healthy participants with high (HA+, n = 12) and low (HA–, n = 12) HA during functional magnetic resonance imaging. <b><i>Results:</i>…

AdultMaleEmotionsmacromolecular substancesAnxietyNeuropsychological TestsAttentional biasVocabularyYoung AdultImage Processing Computer-AssistedReaction TimemedicineHumansBiological PsychiatryAnterior cingulate cortexAnalysis of VarianceBrain Mappingmedicine.diagnostic_testBrainMagnetic Resonance ImagingSemanticsOxygenPsychiatry and Mental healthNeuropsychology and Physiological Psychologymedicine.anatomical_structureConvictionAnxietyFemaleSelf Reportmedicine.symptomPsychologyFunctional magnetic resonance imagingPhotic StimulationStroop effectCognitive psychologyNeuropsychobiology
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Endemic hepatitis C virus infection in a Sicilian town: Further evidence for iatrogenic transmission

2002

The prevalence of and risk factors for HCV and HBV infections in the general population and the predictive value of ALT screening in identifying anti-HCV positive subjects have been evaluated in a small Sicilian town. A random 1:4 sampling from the census of the general population was performed. Anti-HCV, HCV-RNA, HCV genotype, HBsAg, and anti-HBc were tested. The linkage between HCV infection and potential risk factors was evaluated by multiple logistic regression analysis. Among 721 subjects studied, 75 (10.4%) were anti-HCV positive. The HCV infection rate increased from 0.4% in subjects 10-29 years of age to 34% in those > 60 years of age. Among the 75 anti-HCV positive subjects, 66.7% …

AdultMaleEndemic DiseasesAdolescentEpidemiologyIatrogenic DiseasePredictive Value of TestEndemic DiseaseHepacivirusAge DistributionPredictive Value of TestsRisk FactorsVirologyPrevalenceHumansChildSicilyAgedAged 80 and overHepaciviruRisk FactorGeneral populationAlanine TransaminaseHepatitis B viruHepatitis C AntibodiesMiddle AgedHepatitis BHepatitis CPopulation SurveillanceFemaleHepatitis C viruHepatitis C AntibodieHuman
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Skin conductance rises in preparation and recovery to psychosocial stress and its relationship with impulsivity and testosterone in intimate partner …

2013

Intimate partner violence (IPV) perpetrators were categorized into 2 groups using Gottman et al.'s (1995) typology depending on their skin conductance (SC) reactivity to stress. Overall, type I perpetrators tend to show autonomic underarousal, whereas type II perpetrators present a preparatory hyperreactivity to confront stress. Moreover, impulsivity traits and testosterone (T) levels may modulate SC responses to increase the risk of proneness to violence. In this study, SC response to stress was assessed by comparing IPV perpetrators with non-violent controls while performing a modified version of the Trier Social Stress Test (TSST). Subjects with a history of IPV demonstrated higher non-s…

AdultMaleEnzyme-Linked Immunosorbent AssayViolenceImpulsivityStatistics NonparametricDevelopmental psychologyInterpersonal relationshipPhysiology (medical)mental disordersmedicineTrier social stress testHumansInterpersonal RelationsTestosteroneReactivity (psychology)RecidivismGeneral NeurosciencePrisonersTestosterone (patch)social sciencesGalvanic Skin ResponseMiddle AgedNeuropsychology and Physiological PsychologyImpulsive BehaviorDomestic violencemedicine.symptomSkin conductancePsychologyStress PsychologicalInternational journal of psychophysiology : official journal of the International Organization of Psychophysiology
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A new method to calculate external mechanical work using force-platform data in ecological situations in humans: Application to Parkinson's disease

2016

Abstract Background and aim To accurately quantify the cost of physical activity and to evaluate the different components of energy expenditure in humans, it is necessary to evaluate external mechanical work ( W EXT ). Large platform systems surpass other currently used techniques. Here, we describe a calculation method for force-platforms to calculate long-term W EXT . Methods Each force-platform (2.46 × 1.60 m and 3.80 × 2.48 m) rests on 4 piezoelectric sensors. During long periods of recording, a drift in the speed of displacement of the center of mass (necessary to calculate W EXT ) is generated. To suppress this drift, wavelet decomposition is used to low-pass filter the source signal.…

AdultMaleExternal mechanical workPiezoelectric sensorComputer scienceParkinson's diseaseBiophysicsMédecine humaine et pathologieModels BiologicalSignalDisplacement (vector)Human physical activity03 medical and health sciences0302 clinical medicine[SDV.MHEP.PHY]Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO]HumansOrthopedics and Sports MedicineForce platformPhysiologieSimulationenergy expenditure;external mechanical work;human physical activity;parkinson's disease;work efficiencySignal processing[ SDV.MHEP.PHY ] Life Sciences [q-bio]/Human health and pathology/Tissues and Organs [q-bio.TO][SCCO.NEUR]Cognitive science/NeuroscienceRehabilitationWork (physics)PendulumParkinson DiseaseWork efficiency030229 sport sciencesFilter (signal processing)Biomechanical PhenomenaCase-Control Studiesphysiology[ SCCO.NEUR ] Cognitive science/NeuroscienceExercise TestHuman health and pathologyEnergy expenditureEnergy Metabolism030217 neurology & neurosurgery
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