{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T14:16:58Z","timestamp":1789827418922,"version":"4.0.1"},"reference-count":47,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2023,7,5]],"date-time":"2023-07-05T00:00:00Z","timestamp":1688515200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100014076","name":"Stichting ALS Nederland","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100014076","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and purpose<\/jats:title>\n                    <jats:p>The lack of reliable early biomarkers still causes substantial diagnostic delays in amyotrophic lateral sclerosis (ALS). The aim was to assess the diagnostic accuracy of a novel electrophysiological protocol in patients with suspected motor neuron disease (MND).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Consecutive patients with suspected MND were prospectively recruited at our tertiary referral centre for MND in Utrecht, The Netherlands. Procedures were performed in accordance with the Standards for Reporting of Diagnostic Accuracy. In addition to the standard diagnostic workup, an electrophysiological protocol of compound muscle action potential (CMAP) scans and nerve excitability tests was performed on patients' thenar muscles. The combined diagnostic yield of nerve excitability and CMAP scan based motor unit number estimation was compared to the Awaji and Gold Coast criteria and their added value was determined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>In all, 153 ALS or progressive muscular atrophy patients, 63 disease controls and 43 healthy controls were included. Our electrophysiological protocol had high diagnostic accuracy (area under the curve [AUC] 0.85, 95% confidence interval [95% CI] 0.80\u20130.90), even in muscles with undetectable axon loss (AUC 0.78, 95% CI 0.70\u20130.85) and in bulbar\u2010onset patients (AUC 0.85, 95% CI 0.73\u20130.95). Twenty\u2010four of 33 (73%) ALS patients who could not be diagnosed during the same visit were correctly identified, as well as 8\/13 (62%) ALS patients not meeting the Gold Coast criteria and 49\/59 (83%) ALS patients not meeting the Awaji criteria during this first visit.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Our practical and non\u2010invasive electrophysiological protocol may improve early diagnosis in clinically challenging patients with suspected ALS. Routine incorporation may boost early diagnosis, enhance patient selection and generate baseline measures for clinical trials.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/ene.15954","type":"journal-article","created":{"date-parts":[[2023,6,24]],"date-time":"2023-06-24T21:43:27Z","timestamp":1687643007000},"page":"3068-3078","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Diagnostic accuracy of nerve excitability and compound muscle action potential scan derived biomarkers in amyotrophic lateral sclerosis"],"prefix":"10.1111","volume":"30","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3984-1635","authenticated-orcid":false,"given":"D. J. L.","family":"Stikvoort\u00a0Garc\u00eda","sequence":"first","affiliation":[{"name":"Department of Neurology, Brain Center Utrecht University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6618-3573","authenticated-orcid":false,"given":"B.T.H.M.","family":"Sleutjes","sequence":"additional","affiliation":[{"name":"Department of Neurology, Brain Center Utrecht University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L. J.","family":"van Schelven","sequence":"additional","affiliation":[{"name":"Department of Medical Technology and Clinical Physics University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H. S.","family":"Goedee","sequence":"additional","affiliation":[{"name":"Department of Neurology, Brain Center Utrecht University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L. H.","family":"van den Berg","sequence":"additional","affiliation":[{"name":"Department of Neurology, Brain Center Utrecht University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2023,7,5]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(17)31287-4"},{"key":"e_1_2_9_3_1","first-page":"176","article-title":"Diagnosing ALS: the Gold Coast criteria and the role of EMG","volume":"22","author":"Turner MR","year":"2022","journal-title":"Pract Neurol"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.3109\/21678421.2014.903974"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.34.2.121"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(08)70293-X"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1080\/21678421.2019.1587634"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2017.03.045"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26304"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.24945"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2013.07.016"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2014-308589"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2017-315574"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2006.04.016"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2019.07.023"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/118.1.217"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awl024"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1113\/JP276624"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0158596"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2012.06.020"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/121.5.851"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2011.244939"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25960"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2020.08.004"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2020-324006"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-510X(99)00210-5"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajem.2018.01.008"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26621"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1002\/cpt.1096"},{"key":"e_1_2_9_30_1","doi-asserted-by":"crossref","unstructured":"LuggA SchindleM SivakA TankisiH JonesKE.Nerve excitability as a biomarker for amyotrophic lateral sclerosis: a systematic review and meta\u2010analysis.medRxiv. 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