Explanipedia

Polyneuropathy in Wild‐Type Transthyretin Amyloidosis Open

Maria Ausilia Sciarrone, Francesca Vitali, Valeria Guglielmino, Angela Romano, Giovanni Siconolfi , et al. · 2025

Introduction Transthyretin amyloidosis (ATTR) is a systemic disorder characterized by the extracellular accumulation of amyloid fibrils, classified as either mutant (ATTRv, v for variant) or wild‐type (ATTRwt), based on the genetic sequenc…

Twitch force in human Amyotrophic Lateral Sclerosis Open

Laura Libonati, Chiara Cambieri, Marco Ceccanti, Federica Moret, M. Di Giulio , et al. · 2025

Introduction This study investigated differences in muscle twitch force between slow and fast progressors of amyotrophic lateral sclerosis (ALS) to better understand disease heterogeneity and identify potential biomarkers of disease progre…

Exploring Cardiac Sympathetic Denervation in Transthyretin-Mediated Hereditary Amyloidosis (ATTRv): Insights from 123I-mIBG Scintigraphy Open

Maria Silvia De Feo, Chiara Cambieri, Eleonora Galosi, Viviana Frantellizzi, Cristina Chimenti , et al. · 2025

Background/Objectives: Hereditary transthyretin-mediated amyloidosis (ATTRv) is a rare disease characterized by the deposition of amyloid in the heart and peripheral nerves, particularly affecting small fibers. This study aims to evaluate …

Assessment of upper GI motor activity and GI symptoms in patients with amyotrophic lateral sclerosis: an observational study Open

Emanuela Ribichini, Nadia Pallotta, Danilo Badiali, M. Carlucci, Marco Ceccanti , et al. · 2025

Background/aims Oro-pharyngeal dysfunction has been reported in Amyotrophic Lateral Sclerosis (ALS). We aimed to assess ALS patients upper gastrointestinal (GI) motor activity and GI symptoms according to bulbar and spinal onset and severi…

GDF15-GFRAL signaling drives weight loss and lipid metabolism in mouse model of amyotrophic lateral sclerosis Open

Germana Cocozza, Ludovica Maria Busdraghi, Giuseppina Chece, Anna Menini, Marco Ceccanti , et al. · 2024

Weight loss is a common early sign in amyotrophic lateral sclerosis (ALS) patients and negatively correlates with survival. In different cancers and metabolic disorders, high levels of serum growth differentiation factor 15 (GDF15) contrib…

Oxidative Stress in Transthyretin-Mediated Amyloidosis: An Exploratory Study Open

Marco Fiore, Chiara Cambieri, Laura Libonati, Federica Moret, Edoardo D’Andrea , et al. · 2024

Transthyretin-mediated amyloidosis (ATTR) is a systemic disease with protein precipitation in many tissues, mainly the peripheral nerve and heart. Both genetic (ATTRv, “v” for variant) and wild-type (ATTRwt) forms are known. Beyond the ste…

Emotion recognition in amyotrophic lateral sclerosis in a dynamic environment Open

Marco Ceccanti, Laura Libonati, Federica Moret, Edoardo D'Andrea, Maria Cristina Gori , et al. · 2024

MiR206 and 423-3p Are Differently Modulated in Fast and Slow-Progressing Amyotrophic Lateral Sclerosis Patients Open

Antonio Musarò, Gabriella Dobrowolny, Chiara Cambieri, Laura Libonati, Federica Moret , et al. · 2024

Chronic inflammatory demyelinating polyneuropathy and HEV antibody status: A case-control study from Lazio, Italy Open

Federica Moret, Enea Spada, Marco Ceccanti, Laura Libonati, Edoardo D'Andrea , et al. · 2024

Correction to: Retrospective observational study on the use of acetyl-l-carnitine in ALS Open

S Sassi, Elisa Bianchi, Luca Diamanti, Danilo Tornabene, Elisabetta Sette , et al. · 2023

Withdrawal of mechanical ventilation in amyotrophic lateral sclerosis patients: a multicenter Italian survey Open

Cristina Moglia, Francesca Palumbo, Simone Veronese, Stefania Angelocola, Paolo Barone , et al. · 2023

Retrospective observational study on the use of acetyl-l-carnitine in ALS Open

S Sassi, Elisa Bianchi, Luca Diamanti, Danilo Tornabene, Elisabetta Sette , et al. · 2023

ALCAR (Acetyl-L-carnitine) is a donor of acetyl groups and increases the intracellular levels of carnitine, the primary transporter of fatty acids across the mitochondrial membranes. In vivo studies showed that ALCAR decrease oxidative str…

Does Patisiran Reduce Ocular Transthyretin Synthesis? A Pilot Study ofTwo Cases Open

Chiara Cambieri, Marco Marenco, Tania Colasanti, Carmine Mancone, Alessandro Corsi , et al. · 2023

Background: Variant transthyretin-mediated amyloidosis (ATTR-v) is a well-characterized disease affecting the neurologic and cardiovascular systems. Patisiran has been approved for neurologic involvement as it reduces hepatic synthesis of …

Exploring the use of synthetic placebo populations in ALS randomized clinical trials Open

Harry Bowles, Sarah Opie-Martin, Ali Shojaie, Laura Libonati, Alfredo Iacoangeli , et al. · 2022

Objectives The use of synthetic data to supplement clinical trial placebo groups or for trial planning is rapidly gaining interest. However, there is not yet an established framework for generating synthetic data for these purposes. In thi…

The Silent Period for Small Fiber Sensory Neuropathy Assessment in a Mixed Cohort of Transthyretin-Mediated Amyloidosis Open

Chiara Cambieri, Laura Libonati, Federica Moret, Giorgio Tartaglia, Matteo Garibaldi , et al. · 2022

Background: Transthyretin-mediated amyloidosis (ATTR) is a rare multisystemic disease involving the peripheral nervous system and heart. Autonomic and small fiber involvement is one of the hallmarks of ATTR, and many tools have been propos…

Effects of 3,4-diaminopyridine on myasthenia gravis: Preliminary results of an open-label study Open

Marco Ceccanti, Laura Libonati, Gabriele Ruffolo, Pierangelo Cifelli, Federica Moret , et al. · 2022

Background: 3,4-diaminopyridine (3,4-DAP) can lead to clinical and electrophysiological improvement in myasthenic syndrome; it may thus represent a valuable therapeutic option for patients intolerant to pyridostigmine. Objective: to assess…

Classical and Unexpected Effects of Ultra-Micronized PEA in Neuromuscular Function Open

Pierangelo Cifelli, Gabriele Ruffolo, Marco Ceccanti, Chiara Cambieri, Laura Libonati , et al. · 2022

Recently, the endocannabinoid system has attracted growing attention from the scientific community for its involvement in homeostatic and pathological processes as they pertains to human physiology. Among the constituents of the endocannab…

Effects of Skin Stimulation on Sensory-Motor Networks Excitability: Possible Implications for Physical Training in Amyotrophic Lateral Sclerosis Open

Marco Ceccanti, Chiara Cambieri, Laura Libonati, Giorgio Tartaglia, Federica Moret , et al. · 2022

Background Many different trials were assessed for rehabilitation of patients with amyotrophic lateral sclerosis (ALS), with non-unique results. Beside the effects on muscle trophism, some of the encouraging results of physical training co…

Validation of the DYALS (dysphagia in amyotrophic lateral sclerosis) questionnaire for the evaluation of dysphagia in ALS patients Open

Luca Diamanti, Paola Borrelli, Raffaele Dubbioso, Margherita Capasso, Claudia Morelli , et al. · 2021

Creatine Kinase and Progression Rate in Amyotrophic Lateral Sclerosis Open

Marco Ceccanti, Valeria Pozzilli, Chiara Cambieri, Laura Libonati, Emanuela Onesti , et al. · 2020

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with no recognized clinical prognostic factor. Creatinine kinase (CK) increase in these patients is already described with conflicting results on prognosis and survival. In…

A case of motor neuron involvement in Gaucher disease Open

Valeria Pozzilli, Fiorina Giona, Marco Ceccanti, Chiara Cambieri, Vittorio Frasca , et al. · 2019

Heteronymous H reflex in temporal muscle as sign of hyperexcitability in ALS patients Open

Laura Libonati, Tommaso Francesco Barone, Marco Ceccanti, Chiara Cambieri, Giorgio Tartaglia , et al. · 2019

Vitamin D in amyotrophic lateral sclerosis Open

Laura Libonati · 2017

Vitamin D supplementation has been proposed as a potential treatment to delay amyotrophic lateral sclerosis (ALS) progression. The aims of this study were to compare retrospectively vitamin D blood levels in ALS patients with those in heal…

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