Eleonora Colombo1, Alberto Doretti1, Francesco Scheveger1,2, Alessio Maranzano1,2, Giulia Pata1, Delia Gagliardi3, Megi Meneri3,4, Stefano Messina1, Federico Verde1,4, Claudia Morelli1, Stefania Corti3,4, Luca Maderna1, Vincenzo Silani1,4, Nicola Ticozzi5,6. 1. Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, P.le Brescia 20, 20149, Milan, Italy. 2. Neurology Residency Program, Università degli Studi di Milano, Via Festa del Perdono 7, 20122, Milan, Italy. 3. Neurology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via Francesco Sforza 35, 20122, Milan, Italy. 4. Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Via Festa del Perdono 7, 20122, Milan, Italy. 5. Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, P.le Brescia 20, 20149, Milan, Italy. n.ticozzi@auxologico.it. 6. Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Via Festa del Perdono 7, 20122, Milan, Italy. n.ticozzi@auxologico.it.
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.
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.
Authors: B Johnsen; K Pugdahl; A Fuglsang-Frederiksen; K Kollewe; L Paracka; R Dengler; J P Camdessanché; W Nix; R Liguori; I Schofield; L Maderna; D Czell; C Neuwirth; M Weber; V E Drory; A Abraham; M Swash; M de Carvalho Journal: Clin Neurophysiol Date: 2018-12-09 Impact factor: 3.708
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Authors: Matthew S Devine; Emma Ballard; Peter O'Rourke; Matthew C Kiernan; Pamela A Mccombe; Robert D Henderson Journal: Amyotroph Lateral Scler Frontotemporal Degener Date: 2015-12-24 Impact factor: 4.092