Motor unit action potentials analysis in patients with myopathies with a new wireless portable and multichannel Surface EMG device (WPM-SEMG)
Prof. MD PhD Alain Kälin, Neurocentro della Svizzera Italiana, Lugano
Abstract (Lay summary see below)
Background
Current standard diagnostic functional tools for neuromuscular disorders are nerve conductions and needle EMG studies. Needle electrodes are invasive, painful, and often difficult in paediatric patients. A wireless portable and multi-channel surface EMG device (WPM-SEMG) has been recently developed by our group to analyse motor unit action potentials (MUPs) and measure muscle fiber conduction velocity (MFCV) in patients suffering from myopathies. MFCV is a reliable diagnostic tool, because conduction velocity correlates with muscle fiber size variability, a common structural change occurring in most myopathies. However, so far most studies validated this parameter in myopathies only applying invasive procedures (needle electrodes).
Aims
To investigate whether WPM-SEMG is a reliable non-invasive tool to diagnose myopathies, alone or in combination with standard routine investigations (needle EMG, MRI, muscle biopsy)
Methods
In phase 1 (6 months), the technical liability of the WPM-SEMG device will be investigated on 10 healthy volunteers, studying 3 selected muscles (abductor digiti minimi, biceps brachii, vastus lateralis). In phase 2 (1 year), 20 prospectively selected patients suspected for myopathy (metabolic, inflammatory etc.) will receive MRI and both standard needle EMG and WPM-SEMG studies. The same three selected muscles (abductor digiti minimi, biceps brachii, vastus lateralis) will be analysed and the MFCV will be calculated for each muscle. Moreover, patients will receive muscle biopsy and biochemical analysis when clinically needed. The histological data obtained from biopsies will be compared to neurophysiological findings, in particular MFCV values. MUPS characteristics and interference pattern obtained by needle EMG and WPM-SEMG will be compared.
Significance
We prospectively investigate the potential use of non-invasive WPM-SEMG as a new diagnostic tool in myopathies.
Lay Summary
Un nuovo elettrodo multicanale per la diagnosi delle miopatie
L’eletromiografia ad ago è la tecnica standard da molti anni per studiare lo stato funzionale dei muscoli nelle malattie neuromuscolari. Con un ago-elettrodo, vengono registrati i potenziali che risultano patologici in queste malattie consentendo una corretta diagnosi. Poiché tramite questa tecnica si analizzano i potenziali solo in un’area ristretta, per una corretta diagnosi si rende necessario lo spostamento dell’ago in diversi punti e diversi muscoli. L’ esame risulta pertanto doloroso e stressante per il paziente, in particolare ne limita l’utilizzo nei bambini
Tale limitazione sarebbe superata dall’utilizzo di elettrodi cutanei di superficie. Tuttavia, nessuno dei metodi studiati fino a oggi ha dimostrato una qualità sufficiente per sostituire l’esame ad ago. Il nostro gruppo multidisciplinare ha recentemente sviluppato un nuovo elettrodo multicanale che utilizza la tecnologia più moderna, sia per l’acquisizione dei segnali su aree più estese, sia per la loro analisi. Infatti, questo nuovo elettrodo permette di misurare con precisione la velocità di conduzione delle fibre muscolari, che non viene solitamente fatto con l’esame tradizionale. Infine questo nuovo sistema è “wireless”, funziona in assenza di cavi; rendendolo più duttile alla pratica clinica soprattutto nei bambini e per gli studi dinamici.
Lo scopo di questo studio è dunque di paragonare l’efficienza del nuovo sistema multicanale wireless all’esame EMGrafico ad ago in venti soggetti adulti. Inoltre valuteremo per la prima volta l’utilità della velocità di conduzione delle fibre muscolari tramite elettrodi di superficie per la diagnosi di miopatie.
Se questa prima analisi avrà esito positivo, ci proponiamo di estendere lo studio anche alla popolazione pediatrica.
Progetti
- Nuovi progetti di ricerca dal 2024
- L'importanza della ricerca
- Progetti finanziati
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- Rapid Exploratory Imaging for High-resolution and Whole Extremity Coverage in MR Neurography
- Deciphering novel mechanisms and effectors contributing to muscle dysfunction in Myotonic Dystrophy Type I
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- Identification of the critical regulators of protein synthesis and degradation in human muscle atrophy
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- Targeting NADPH oxidase 4 in models of Duchenne muscular dystrophy
- Characterizing the role of ER stress in C9orf72-linked ALS pathology
- Inducing mitophagy with Urolithin A to restore mitochondrial and muscle function in muscular dystrophy
- Motor unit action potentials analysis in patients with myopathies with a new wireless portable and multichannel Surface EMG device (WPM-SEMG)
- Role and therapeutic potential of PLIN3 in neuromuscular diseases
- Changes in ventilation distribution in children with neuromuscular disease using the insufflator/exsufflator technique: An observational study
- Mechanism and function of genome organization in muscle development and integrity
- Role and therapeutic potential of NADPH oxidases in a mouse model of Duchenne Muscular Dystrophy
- Characterization of pathological pathways activated in muscles of patients with congenital myopathies with disturbed Ca2+ homeostasis
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- Understanding the pathomechanisms leading to muscle alterations in Myotonic Dystrophy type I
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- Protective effects and mechanisms of action of tamoxifen in mice with severe muscular diseases
- Role of the receptor FgfrL1 in the development of slow muscle fibers
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- Generation of uncommitted human IPSC derived muscle stem cells for therapeutic applications
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- Deciphering the pathogenic mechanisms of C9ORF72 ALS
- Enhancing estrogenic signalling to fight muscular dystrophies: Mechanisms of action and repurposing clinically approved drugs
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- Improving cellular therapies of muscle dystrophies by uncovering epigenetic and signaling pathways of muscle formation
- Protein engineering in an attempt to increase the mechanical, integrin dependent cytoskeleton-matrix linkage in muscle fibers
- Muscle velocity recovery cycles: a new tool for characterization of muscle disease in vivo
- Excessive neurotrypsin activation and agrin cleavage-a pathogenic condition leading to sarcopenia-like muscle atrophy?
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- Restoration of autophagy as a new strategy for the treatment of congenital muscular dystrophies
- Development of magnetic resonance methods for functional imaging of the skeletal muscle
- Targeting ER stress response: a potential mechanism for neuroprotection in Amyotrophic Lateral Sclerosis
- Generation of uncommitted human IPSC derived muscle stem cells for therapeutic applications
- Opuscolo progetti
- SEAL Therapeutics AG
- Meeting e workshop
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- La rete Myosuisse
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