0000000000862254

AUTHOR

Enrico Granieri

showing 4 related works from this author

Bovine spongiform encephalopathy and Creutzfeldt-Jakob disease: facts and uncertainties underlying the causal link between animal and human diseases

2004

Following an outbreak of bovine spongiform encephalopathy (BSE) in dairy cows in the United Kingdom (UK), 153 definite and probable human cases of new variant Creutzfeldt-Jakob disease (nvCJD) have been reported, almost exclusively in the UK. Although exposure to the BSE agent is the most plausible interpretation for the occurrence of nvCJD, the causal link between the BSE prion and nvCJD is still debated. This review discusses the pros and cons of nvCJD as a separate nosographic entity, the scientific basis for a correlation between BSE and nvCJD, the validity of the current diagnostic criteria for CJD and nvCJD, the contribution of epidemiology to the detection of a causal relation betwee…

Pediatricsmedicine.medical_specialtyMeatEncephalopathy Bovine Spongiform Encephalopathy Bovine SpongiformBovine spongiform encephalopathyEncephalopathyDermatologyDiseaseCreutzfeldt-Jakob SyndromeAnimalsHumansMedicinebovine spongiform encephalopathybusiness.industryCausal relationsGeneral MedicineNew variantmedicine.diseaseVirologyCreutzfeldt-Jakob diseaseEncephalopathy Bovine SpongiformPsychiatry and Mental healthCattleCausal linkepidemiologyNeurology (clinical)business
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Encoding of human action in Broca's area.

2009

International audience; Broca's area has been considered, for over a century, as the brain centre responsible for speech production. Modern neuroimaging and neuropsychological evidence have suggested a wider functional role is played by this area. In addition to the evidence that it is involved in syntactical analysis, mathematical calculation and music processing, it has recently been shown that Broca's area may play some role in language comprehension and, more generally, in understanding actions of other individuals. As shown by functional magnetic resonance imaging, Broca's area is one of the cortical areas activated by hand/mouth action observation and it has been proposed that it may …

AdultMaleDissociation (neuropsychology)Neuropsychological Testsmotor syntaxApraxia050105 experimental psychology03 medical and health sciences0302 clinical medicineBroca's areaAphasiamirror-neuron systemmedicineHumans0501 psychology and cognitive sciencesLanguage disorderBroca's areaMirror neuronLanguageAphasia BrocaBrain Mappingaction recognition[SCCO.NEUR]Cognitive science/Neuroscience05 social sciencesNeuropsychologyBrainCognitionMiddle Agedaction recognition; Broca's area; frontal aphasia; mirror-neuron system; motor syntax;medicine.diseaseMagnetic Resonance ImagingFrontal Lobefrontal aphasiaFemaleNeurology (clinical)medicine.symptomComprehensionPsychologyNeurosciencePhotic Stimulation030217 neurology & neurosurgeryCognitive psychology
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Protein misfolding, amyotrophic lateral sclerosis and guanabenz: Protocol for a phase II RCT with futility design (ProMISe trial)

2017

IntroductionRecent studies suggest that endoplasmic reticulum stress may play a critical role in the pathogenesis of amyotrophic lateral sclerosis (ALS) through an altered regulation of the proteostasis, the cellular pathway-balancing protein synthesis and degradation. A key mechanism is thought to be the dephosphorylation of eIF2α, a factor involved in the initiation of protein translation. Guanabenz is an alpha-2-adrenergic receptor agonist safely used in past to treat mild hypertension and is now an orphan drug. A pharmacological action recently discovered is its ability to modulate the synthesis of proteins by the activation of translational factors preventing misfolded protein accumula…

0301 basic medicineOncologyPathologyamyotrophic lateral sclerosisamyotrophic lateral sclerosis; motor neurone disease; neuromuscular disease; randomized clinical trial guanabenz; unfolded protein response; adrenergic alpha-2 receptor agonist s; age of onset; amyotrophic lateral sclerosis; disease progression; double-blind method; endoplasmic reticulum stress; guanabenz; humans; italy; medical futility; neuroprotective agents; proteostasis deficienciesamyotrophic lateral sclerosis; motor neurone disease; neuromuscular disease; randomized clinical trial guanabenz; unfolded protein response; Medicine (all)randomized clinical trial guanabenzHelsinki declaration0302 clinical medicineProtocolAdrenergic alpha-2 Receptor Agonists1506Amyotrophic lateral sclerosisAge of OnsetGuanabenzMedicine (all)amyotrophic lateral sclerosis; motor neurone disease; neuromuscular disease; randomized clinical trial guanabenz; unfolded protein responseNeurodegenerationamyotrophic lateral sclerosis; motor neurone disease; neuromuscular disease; randomized clinical trial guanabenz; unfolded protein response;amyotrophic lateral sclerosis; guanabenz; motor neurone disease; neuromuscular disease; randomized clinical trial; unfolded protein response; Adrenergic alpha-2 Receptor Agonists; Age of Onset; Amyotrophic Lateral Sclerosis; Disease Progression; Double-Blind Method; Endoplasmic Reticulum Stress; Guanabenz; Humans; Italy; Medical Futility; Neuroprotective Agents; Proteostasis DeficienciesGeneral Medicineunfolded protein responseEndoplasmic Reticulum StressRiluzoleNeuroprotective AgentsNeurologyTolerabilityItalyDisease Progression1713GuanabenzMedical Futilitymedicine.drugmedicine.medical_specialtyamyotrophic lateral sclerosis; motor neurone disease; neuromuscular disease; randomized clinical trial guanabenz; unfolded protein response; Adrenergic alpha-2 Receptor Agonists; Age of Onset; Amyotrophic Lateral Sclerosis; Disease Progression; Double-Blind Method; Endoplasmic Reticulum Stress; Guanabenz; Humans; Italy; Medical Futility; Neuroprotective Agents; Proteostasis Deficiencies; Medicine (all)Neuroprotection03 medical and health sciencesmotor neurone diseaseDouble-Blind MethodInternal medicinemedicineHumansProteostasis Deficienciesbusiness.industryAmbientaleneuromuscular diseaserandomized clinical trialmedicine.diseaseClinical trial030104 developmental biologybusiness030217 neurology & neurosurgery
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Prognostic indicators in pediatric clinically isolated syndrome

2017

To assess prognostic factors for a second clinical attack and a first disability worsening event in pediatric clinically isolated syndrome (pCIS) suggestive of Multiple Sclerosis (MS) patients. Objective: To assess prognostic factors for a second clinical attack and a first disability-worsening event in pediatric clinically isolated syndrome (pCIS) suggestive of multiple sclerosis (MS) patients. Methods: A cohort of 770 pCIS patients was followed up for at least 10 years. Cox proportional hazard models and Recursive Partitioning and Amalgamation (RECPAM) tree-regression were used to analyze data. Results: In pCIS, female sex and a multifocal onset were risk factors for a second clinical att…

RegistrieMaleMultiple SclerosisAdolescentAdolescent; Age of Onset; Child; Demyelinating Diseases; Female; Follow-Up Studies; Humans; Male; Multiple Sclerosis; Prognosis; Retrospective Studies; Risk Factors; Disease Progression; Registries; Neurology; Neurology (clinical)PrognosiONSET MULTIPLE-SCLEROSISCHILDHOODCHILDRENPARACLINICAL FEATURESDISABILITY PROGRESSIONNOFollow-Up StudieRisk FactorsRetrospective Studieprognostic indicatorsMultiple Sclerosipediatric multiple sclerosis prognosis indicatorsHumansRegistriesAge of OnsetChildOPTIC NEURITISRetrospective StudiesRisk FactorDemyelinating DiseaseNATURAL-HISTORYPrognosismultiple sclerosis clinically isolated syndrome prognostic indicatorsNeurologyTRANSVERSE MYELITISclinically isolated syndromeINTERFERON BETA-1BDisease ProgressionSettore MED/26 - NeurologiaFemaleNeurology (clinical)FOLLOW-UPDemyelinating DiseasesFollow-Up StudiesHuman
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