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Open Access 02-10-2022 | Amyotrophic Lateral Sclerosis | Original Communication

Correlation between clinical phenotype and electromyographic parameters in amyotrophic lateral sclerosis

Authors: Eleonora Colombo, Alberto Doretti, Francesco Scheveger, Alessio Maranzano, Giulia Pata, Delia Gagliardi, Megi Meneri, Stefano Messina, Federico Verde, Claudia Morelli, Stefania Corti, Luca Maderna, Vincenzo Silani, Nicola Ticozzi

Published in: Journal of Neurology | Issue 1/2023

Abstract

Introduction

Even if electromyography (EMG) is routinely used to confirm the diagnosis of amyotrophic lateral sclerosis (ALS), few studies have analysed the correlation between electrophysiological parameters and clinical characteristics of ALS. We assessed if the quantification of active denervation (AD) and chronic denervation (CD) provides clinicians with information about phenotype, disease progression and survival in ALS patients.

Methods

We studied a cohort of 689 ALS patients recording the following parameters: age and site of onset, survival, MRC scale for muscle strength evaluation, burden of upper and lower motor signs as measured with specific scales (PUMNS and LMNS, respectively), ALSFRS-R, progression rate (ΔFS), MITOS and King’s Staging systems (KSS). We performed EMG on 11 muscles, and calculated semiquantitative AD and CD scores for each limb, as well as for the bulbar and spinal regions.

Results

We found a positive correlation between AD and CD scores with LMNS (respectively p = 4.4 × 10^–37 and p = 2.8 × 10^–45) and a negative correlation with MRC (respectively p = 4.5 × 10^–35 and p = 3.0 × 10^–35). Furthermore, patients with higher spinal AD and CD scores had significantly lower ALSFRS-R scores, and higher KSS and MITOS stages. Conversely, only AD was associated to higher ΔFS (p = 1.0 × 10^–6) and shorter survival (p = 1.1 × 10^–5).

Conclusion

Our results confirmed that EMG examination represents not only a diagnostic instrument, but also a prognostic tool. In this context, AD seems to be a reliable predictor of disease’s progression and survival while CD better describes functional disability.

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Title: Correlation between clinical phenotype and electromyographic parameters in amyotrophic lateral sclerosis
Authors: Eleonora Colombo
Alberto Doretti
Francesco Scheveger
Alessio Maranzano
Giulia Pata
Delia Gagliardi
Megi Meneri
Stefano Messina
Federico Verde
Claudia Morelli
Stefania Corti
Luca Maderna
Vincenzo Silani
Nicola Ticozzi
Publication date: 02-10-2022
Publisher: Springer Berlin Heidelberg
Keywords: Amyotrophic Lateral Sclerosis
Neurotomy
Published in: Journal of Neurology / Issue 1/2023
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-022-11404-4

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Correlation between clinical phenotype and electromyographic parameters in amyotrophic lateral sclerosis

Abstract

Introduction

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Methods

Results

Conclusion

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