0000000000764764

AUTHOR

E Maestri

showing 3 related works from this author

Randomized double-blind placebo-controlled trial of acetyl-L-carnitine for ALS.

2013

Our objective was to assess the effects of acetyl-L-carnitine (ALC) with riluzole on disability and mortality of amyotrophic lateral sclerosis (ALS). Definite/probable ALS patients, 40-70 years of age, duration 6-24 months, self-sufficient (i.e. able to swallow, cut food/handle utensils, and walk), and with forced vital capacity (FVC) > 80% entered a pilot double-blind, placebo-controlled, parallel group trial and were followed for 48 weeks. ALC or placebo 3 g/day was added to riluzole 100 mg/day. Primary endpoint: number of patients no longer self-sufficient. Secondary endpoints: changes in ALSFRS-R, MRC, FVC and McGill Quality of Life (QoL) scores. Analysis was made in the intention-to-tr…

Maleamyotrophic lateral sclerosisVital CapacityPlacebo-controlled studyPilot ProjectsGastroenterologylaw.inventionRandomized controlled triallawAcetyl-L-carnitineamyotrophic lateral sclerosis; motor neuron disease; randomized trial; acetyl-l-carnitinerandomized trialAmyotrophic lateral sclerosisAcetylcarnitineALS acetyl-L-carnitineNootropic AgentsRiluzoleMiddle AgedRiluzoleTreatment OutcomeNeurologyCombinationDisease Progressionmotor neuron diseaseDrug Therapy CombinationSettore MED/26 - NeurologiaFemaleAcetylcarnitinemedicine.drugAdultmedicine.medical_specialtyAcetyl-L-carnitine amyotrophic lateral sclerosis motor neuron disease randomized trialDouble blindDouble-Blind MethodDrug TherapyInternal medicinemedicineHumansAgedMED/26 - NEUROLOGIAbusiness.industryDisease progressionmedicine.diseaseAcetyl-L-carnitineSurgeryQuality of LifeAcetylcarnitine; Adult; Aged; Amyotrophic Lateral Sclerosis; Disease Progression; Double-Blind Method; Drug Therapy Combination; Excitatory Amino Acid Antagonists; Female; Humans; Male; Middle Aged; Nootropic Agents; Pilot Projects; Quality of Life; Riluzole; Treatment Outcome; Vital CapacityNeurology (clinical)businessExcitatory Amino Acid Antagonists
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CHCH10 mutations in an Italian cohort of familial and sporadic amyotrophic lateral sclerosis patients

2015

Mutations in CHCHD10 have recently been described as a cause of frontotemporal dementia (FTD) comorbid with amyotrophic lateral sclerosis (ALS). The aim of this study was to assess the frequency and clinical characteristics of CHCHD10 mutations in Italian patients diagnosed with familial (n= 64) and apparently sporadic ALS (n= 224). Three apparently sporadic patients were found to carry c.100C>T (p.Pro34Ser) heterozygous variant in the exon 2 of CHCHD10. This mutation had been previously described in 2 unrelated French patients with FTD-ALS. However, our patients had a typical ALS, without evidence of FTD, cerebellar or extrapyramidal signs, or sensorineural deficits. We confirm that CHC…

MaleAgingPediatricsmedicine.medical_specialtyPathologyAmyotrophic lateral sclerosis; CHCHD10; Familial; Sporadic; Aged; Amyotrophic Lateral Sclerosis; Cohort Studies; Female; Frontotemporal Dementia; Genetic Predisposition to Disease; Humans; Italy; Male; Middle Aged; Mitochondrial Proteins; Genetic Association Studies; MutationGenetic Association StudieDiseaseSettore MED/03 - GENETICA MEDICAmedicine.disease_causeCohort StudiesMitochondrial ProteinsExonFamilialmental disordersmedicineHumansMitochondrial ProteinDementiaGenetic Predisposition to DiseaseAmyotrophic lateral sclerosisAmyotrophic lateral sclerosis; CHCHD10; Familial; Sporadic; Aged; Amyotrophic Lateral Sclerosis; Cohort Studies; Female; Frontotemporal Dementia; Genetic Predisposition to Disease; Humans; Italy; Male; Middle Aged; Mitochondrial Proteins; Genetic Association Studies; Mutation; Neurology (clinical); Neuroscience (all); Aging; Developmental Biology; Geriatrics and GerontologyGenetic Association StudiesAmyotrophic lateral sclerosiAgedMutationNeuroscience (all)business.industryGeneral NeuroscienceMiddle AgedAmyotrophic lateral sclerosisSporadicmedicine.disease3. Good healthAmyotrophic lateral sclerosis; CHCHD10; Familial; SporadicCHCHD10ItalyFrontotemporal DementiaMutationCohortFemaleNeurology (clinical)Cohort StudieGeriatrics and GerontologybusinessHumanDevelopmental BiologyFrontotemporal dementiaCohort studyNeurobiology of Aging
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Erythropoietin in amyotrophic lateral sclerosis: a multicentre, randomized, double blind, placebo controlled, phase III study.

2015

Objective To assess the efficacy of recombinant human erythropoietin (rhEPO) in amyotrophic lateral sclerosis (ALS). Methods Patients with probable laboratory-supported, probable or definite ALS were enrolled by 25 Italian centres and randomly assigned (1:1) to receive intravenous rhEPO 40 000 IU or placebo fortnightly as add-on treatment to riluzole 100 mg daily for 12 months. The primary composite outcome was survival, tracheotomy or >23 h non-invasive ventilation (NIV). Secondary outcomes were ALSFRS-R, slow vital capacity (sVC) and quality of life (ALSAQ-40) decline. Tolerability was evaluated analysing adverse events (AEs) causing withdrawal. The randomisation sequence was computer-…

MaleGastroenterologylaw.inventionRandomized controlled triallaw1506Amyotrophic lateral sclerosisMOTOR NEURON DISEASEeducation.field_of_studyRecombinant ProteinMiddle AgedRecombinant ProteinsTreatment OutcomePsychiatry and Mental HealthNeuromuscularSettore MED/26 - NeurologiaFemaleerythropoietyn clinical trialmedicine.drugHumanALS; MOTOR NEURON DISEASE; Adult; Aged; Amyotrophic Lateral Sclerosis; Double-Blind Method; Erythropoietin; Female; Humans; Male; Middle Aged; Recombinant Proteins; Treatment OutcomeAdultmedicine.medical_specialtyPopulationSocio-culturalePlaceboDouble blindALS; erythropoietyn clinical trialDouble-Blind MethodArts and Humanities (miscellaneous)ALS; MOTOR NEURON DISEASE; Adult; Aged; Amyotrophic Lateral Sclerosis; Double-Blind Method; Epoetin Alfa; Erythropoietin; Female; Humans; Male; Middle Aged; Recombinant Proteins; Treatment Outcome; Neurology (clinical); Psychiatry and Mental Health; Surgery; Arts and Humanities (miscellaneous)Internal medicinemedicineALS; MOTOR NEURON DISEASEHumanseducationErythropoietinAgedbusiness.industryAmyotrophic Lateral SclerosisEpoetin alfamedicine.diseaseSurgeryClinical trialEpoetin AlfaErythropoietinSurgeryNeurology (clinical)ALSbusinessAmyotrophic Lateral Sclerosi
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