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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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>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 (\u0394FS), MITOS and King\u2019s 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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We found a positive correlation between AD and CD scores with LMNS (respectively<jats:italic>p\u2009=\u2009<\/jats:italic>4.4\u2009\u00d7\u200910<jats:sup>\u201337<\/jats:sup>and<jats:italic>p\u2009=\u2009<\/jats:italic>2.8\u2009\u00d7\u200910<jats:sup>\u201345<\/jats:sup>) and a negative correlation with MRC (respectively<jats:italic>p\u2009=\u2009<\/jats:italic>4.5\u2009\u00d7\u200910<jats:sup>\u201335<\/jats:sup>and<jats:italic>p\u2009=\u2009<\/jats:italic>3.0\u2009\u00d7\u200910<jats:sup>\u201335<\/jats:sup>). 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 \u0394FS (<jats:italic>p\u2009=\u2009<\/jats:italic>1.0\u2009\u00d7\u200910<jats:sup>\u20136<\/jats:sup>) and shorter survival (<jats:italic>p\u2009=\u2009<\/jats:italic>1.1\u2009\u00d7\u200910<jats:sup>\u20135<\/jats:sup>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>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\u2019s progression and survival while CD better describes functional disability.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s00415-022-11404-4","type":"journal-article","created":{"date-parts":[[2022,10,2]],"date-time":"2022-10-02T07:02:33Z","timestamp":1664694153000},"page":"511-518","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["Correlation between clinical phenotype and electromyographic parameters in amyotrophic lateral sclerosis"],"prefix":"10.1007","volume":"270","author":[{"given":"Eleonora","family":"Colombo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alberto","family":"Doretti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Francesco","family":"Scheveger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessio","family":"Maranzano","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giulia","family":"Pata","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Delia","family":"Gagliardi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Megi","family":"Meneri","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefano","family":"Messina","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federico","family":"Verde","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia","family":"Morelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefania","family":"Corti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luca","family":"Maderna","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vincenzo","family":"Silani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5963-7426","authenticated-orcid":false,"given":"Nicola","family":"Ticozzi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2022,10,2]]},"reference":[{"issue":"Suppl","key":"11404_CR1","doi-asserted-by":"publisher","first-page":"96","DOI":"10.1016\/0022-510X(94)90191-0","volume":"124","author":"BR Brooks","year":"1994","unstructured":"Brooks BR (1994) El Escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis. 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Scheveger, A. Maranzano, G. Pata, D. Gagliardi, M. Meneri, F. Verde, C. Morelli, S. Corti, L. Maderna report no disclosures. S. Messina received compensation for speaking activities from Eli Lilly and Novartis Pharma AG. V. Silani received compensation for consulting services and\/or speaking activities from AveXis, Cytokinetics, Italfarmaco, Liquidweb Srl, and Novartis Pharma AG. He is on the Editorial Board of Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, European Neurology, American Journal of Neurodegenerative Diseases, and Frontiers in Neurology. N. Ticozzi received compensation for consulting services from Amylyx Pharmaceuticals and Zambon Biotech SA. He is Associate Editor for Frontiers in Aging Neuroscience.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflicts of interest"}},{"value":"This study was approved by the Ethics Committee of IRCCS Istituto Auxologico Italiano (DAMARE 2021_05_18) and conducted according to the principles expressed in the Declaration of Helsinki.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical standard"}}]}}