{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T05:02:09Z","timestamp":1777006929800,"version":"3.51.4"},"reference-count":33,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T00:00:00Z","timestamp":1776988800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Neurol."],"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Hyperkinetic movement disorders (MDs) in children, including tremor, myoclonus, dystonia, chorea, and tics, often present with overlapping or evolving clinical features, making classification based on observation alone challenging. Neurophysiological techniques such as video-EEG polymyography (P-VEEG) and somatosensory evoked potentials (SEPs) may provide objective markers to improve diagnostic accuracy, yet their application in pediatric populations remains limited.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>To describe the polygraphic characteristics of hyperkinetic MDs in a large pediatric cohort and to assess whether specific EMG patterns can support movement disorder classification beyond clinical inspection.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Seventy-three patients with pediatric-onset hyperkinetic MDs underwent standardized P-VEEG recordings and SEPs at Besta Neurological Institute between January and October 2024. EMG activity was recorded from antagonist muscle pairs during rest, posture maintenance, and specific tasks according to age. Polygraphic findings were compared with clinical diagnoses. Associations between EMG patterns and clinical variables were analyzed using Chi-square tests and regression analyses.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Mean age at MD onset was 9.4\u202f\u00b1\u202f6.1\u202fyears and mean age at recording was 13.5\u202f\u00b1\u202f5.1\u202fyears. Polygraphic analysis identified four distinct EMG patterns: (i) regular rhythmic oscillatory pattern (RRO), consistent with tremor, in 40 patients (56%); (ii) repetitive myoclonic pattern (RM) in 11 (15%); (iii) complex mixed repetitive pattern (CR) in 9 (12%); and (iv) complex non-rhythmic pattern (CNR), encompassing chorea, dystonia, tics and other dyskinesias, in 11 (15%). Significant differences among repetitive patterns were observed in rhythmicity (\n                      <jats:italic>p<\/jats:italic>\n                      \u202f&amp;lt;\u202f0.001), burst duration (\n                      <jats:italic>p<\/jats:italic>\n                      \u202f&amp;lt;\u202f0.001), agonist\u2013antagonist synchrony (\n                      <jats:italic>p<\/jats:italic>\n                      \u202f&amp;lt;\u202f0.001), and SEP hyperexcitability (\n                      <jats:italic>p<\/jats:italic>\n                      \u202f&amp;lt;\u202f0.03). RM was significantly associated with enhanced amplitude SEPs (\n                      <jats:italic>p<\/jats:italic>\n                      \u202f=\u202f0.023), supporting a cortical origin of myoclonus in most cases. A significant association between clinical diagnosis and EMG pattern was found (\n                      <jats:italic>p<\/jats:italic>\n                      \u202f=\u202f0.003), yet discrepancies between clinical and polygraphic classification was found in 22 cases (30%), particularly among tremor and myoclonus presentations. RM and CR were prevalent in older patients compared with CNR.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>P-VEEG proved to be an easily applicable tool providing valuable objective markers for the characterization of pediatric hyperkinetic MDs, revealing distinct EMG patterns that complement clinical evaluation and improve diagnostic precision. Systematic integration of neurophysiological assessment may enhance classification, diagnostic accuracy and help guide management in childhood movement disorders.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fneur.2026.1801440","type":"journal-article","created":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T04:30:08Z","timestamp":1777005008000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Video-EEG polygraphy in the clinical characterization of hyperkinetic movement disorders: a tertiary referral cohort"],"prefix":"10.3389","volume":"17","author":[{"given":"Davide","family":"Caputo","sequence":"first","affiliation":[{"name":"Pediatric Neuroscience Department, Fondazione IRCCS Istituto Neurologico \u201cC. Besta\u201d (full member of the European Reference Network EpiCARE)","place":["Milan, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brando","family":"Rasori","sequence":"additional","affiliation":[{"name":"Innovation Biomedicine section, Department of Engineering for Innovation Medicine, University of Verona","place":["Verona, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roberta","family":"Solazzi","sequence":"additional","affiliation":[{"name":"Pediatric Neuroscience Department, Fondazione IRCCS Istituto Neurologico \u201cC. Besta\u201d (full member of the European Reference Network EpiCARE)","place":["Milan, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Laura","family":"Canafoglia","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Technology, Fondazione IRCCS Istituto Neurologico \u201cC. Besta\u201d","place":["Milan, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elisa","family":"Visani","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Technology, Fondazione IRCCS Istituto Neurologico \u201cC. Besta\u201d","place":["Milan, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Davide Rossi","family":"Sebastiano","sequence":"additional","affiliation":[{"name":"Department of Diagnostic and Technology, Fondazione IRCCS Istituto Neurologico \u201cC. Besta\u201d","place":["Milan, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"Zibordi","sequence":"additional","affiliation":[{"name":"Pediatric Neuroscience Department, Fondazione IRCCS Istituto Neurologico \u201cC. Besta\u201d (full member of the European Reference Network EpiCARE)","place":["Milan, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"Graziola","sequence":"additional","affiliation":[{"name":"Pediatric Neuroscience Department, Fondazione IRCCS Istituto Neurologico \u201cC. Besta\u201d (full member of the European Reference Network EpiCARE)","place":["Milan, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gaetano","family":"Cantalupo","sequence":"additional","affiliation":[{"name":"Innovation Biomedicine section, Department of Engineering for Innovation Medicine, University of Verona","place":["Verona, Italy"]},{"name":"Child Neuropsychiatry Unit, University Hospital of Verona (full member of the European Reference Network EpiCARE)","place":["Verona, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tiziana","family":"Granata","sequence":"additional","affiliation":[{"name":"Pediatric Neuroscience Department, Fondazione IRCCS Istituto Neurologico \u201cC. Besta\u201d (full member of the European Reference Network EpiCARE)","place":["Milan, Italy"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanna","family":"Zorzi","sequence":"additional","affiliation":[{"name":"Pediatric Neuroscience Department, Fondazione IRCCS Istituto Neurologico \u201cC. 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myoclonus","volume":"39","author":"Callister","year":"2024","journal-title":"Mov Disord"},{"key":"ref21","doi-asserted-by":"publisher","first-page":"93","DOI":"10.1016\/j.clinph.2022.01.004","article-title":"The clinical and electrophysiological investigation of tremor","volume":"136","author":"Deuschl","year":"2022","journal-title":"Clini Neurophysiol"},{"key":"ref22","doi-asserted-by":"publisher","first-page":"51","DOI":"10.1016\/j.cnp.2021.12.002","article-title":"Electrophysiological testing aids the diagnosis of tremor and myoclonus in clinically challenging patients","volume":"7","author":"Everlo","year":"2022","journal-title":"Clin Neurophysiol Pract"},{"key":"ref23","doi-asserted-by":"publisher","first-page":"5553","DOI":"10.1007\/s10072-022-06236-z","article-title":"Significance and clinical suggestions for the somatosensory evoked potentials increased in amplitude revealed by a large sample of neurological patients","volume":"43","author":"Rossi 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subjects","volume":"67","author":"Caputo","year":"2025","journal-title":"Epilepsia"},{"key":"ref27","doi-asserted-by":"publisher","first-page":"563","DOI":"10.1093\/brain\/awaf297","article-title":"Molecular and clinical spectrum of epilepsy-dyskinesia syndromes: a cross-sectional study of 609 patients","volume":"149","author":"Quiroz","year":"2025","journal-title":"Brain"},{"key":"ref28","doi-asserted-by":"publisher","first-page":"2309","DOI":"10.1212\/01.wnl.0000147475.71345.aa","article-title":"Sensorimotor cortex excitability in Unverricht-Lundborg disease and Lafora body disease","volume":"63","author":"Canafoglia","year":"2004","journal-title":"Neurology"},{"key":"ref29","first-page":"405","article-title":"Progressive Myoclonic Epilepsies Definitive and Still Undetermined Causes","volume-title":"Neurology","author":"Franceschetti","year":"2014"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"1195","DOI":"10.1093\/brain\/121.7.1195","article-title":"The pathophysiology of primary dystonia","volume":"121","author":"Berardelli","year":"1998","journal-title":"Brain"},{"key":"ref31","doi-asserted-by":"publisher","first-page":"157","DOI":"10.1002\/mus.20234","article-title":"Electrophysiological studies of myoclonus","volume":"31","author":"Shibasaki","year":"2005","journal-title":"Muscle Nerve"},{"key":"ref32","doi-asserted-by":"publisher","first-page":"281","DOI":"10.1016\/j.neucli.2006.10.001","article-title":"Neurophysiology of myoclonus","volume":"36","author":"Cassim","year":"2006","journal-title":"Neurophysiol Clin"},{"key":"ref33","first-page":"E1884","article-title":"Movement Disorders in Patients with Genetic Developmental and Epileptic Encephalopathies","volume-title":"Neurology","author":"van der Veen","year":"2023"}],"container-title":["Frontiers in 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