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Individual annualized rates of change were calculated and longitudinal trends were assessed using Bayesian mixed models. Psychiatric features were evaluated cross\u2010sectionally in the Spanish cohort.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010eight patients (21 males, 17 females) were evaluated at a mean age (SD) of 10\u2009years (4\u2009years 7\u2009months; range 2\u201321\u2009years) and 14\u2009years 2\u2009months\u2009(4\u2009years 8\u2009months; range 4\u201325\u2009years). We observed a significant worsening of action myoclonus, global dystonia, and dystonia during writing (mean annual increases of 1.356, 0.226, and 0.518 in the Unified Myoclonus, Burke\u2013Fahn\u2013Marsden, and Writer's Cramp Rating Scales respectively). Accordingly, participants perceived a significant worsening in their speech, writing, and walking abilities. Twenty\u2010six of 32 participants suffered from anxiety (<jats:italic>n<\/jats:italic>\u2009=\u200913), obsessive\u2010compulsive disorder (<jats:italic>n<\/jats:italic>\u2009=\u20099), and attention\u2010deficit\/hyperactivity disorder (<jats:italic>n<\/jats:italic>\u2009=\u20098).<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>This study demonstrates that, unlike in the adult population, myoclonus dystonia syndrome in childhood and adolescence follows a progressive course that can be debilitating in the early stages of life. These findings, along with a high prevalence of psychiatric symptoms, highlight the need for early therapeutic interventions to prevent long\u2010term motor and psychological sequelae.<\/jats:p><\/jats:sec>","DOI":"10.1111\/dmcn.16214","type":"journal-article","created":{"date-parts":[[2024,12,16]],"date-time":"2024-12-16T13:49:45Z","timestamp":1734356985000},"page":"740-749","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Natural history of <scp><i>SGCE<\/i><\/scp>\u2010associated myoclonus dystonia in children and adolescents"],"prefix":"10.1111","volume":"67","author":[{"given":"Valeria","family":"De Francesch","sequence":"first","affiliation":[{"name":"Child and Adolescent Neuropsychiatry Unit, AULSS1 Dolomiti San Martino Hospital  Belluno Italy"},{"name":"Innovative Therapies in Pediatric Neurology Research Group Vall d'Hebron Research Institute  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana","family":"Cazurro\u2010Guti\u00e9rrez","sequence":"additional","affiliation":[{"name":"Innovative Therapies in Pediatric Neurology Research Group Vall d'Hebron Research Institute  Barcelona Spain"},{"name":"Department of Pediatrics Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elze R.","family":"Timmers","sequence":"additional","affiliation":[{"name":"Expertise Centre Movement Disorders Groningen, Department of Neurology University Medical Center Groningen, University of Groningen  Groningen the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gemma","family":"Espa\u00f1ol\u2010Mart\u00edn","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"},{"name":"Department of Pediatrics, Pediatric Neurology and Psychiatry Vall d'Hebron Hospital  Barcelona Spain"},{"name":"Biomedical Research Networking Center on Rare Diseases  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia","family":"Ferrero\u2010Turri\u00f3n","sequence":"additional","affiliation":[{"name":"Innovative Therapies in Pediatric Neurology Research Group Vall d'Hebron Research Institute  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"G\u00f3mez\u2010Andr\u00e9s","sequence":"additional","affiliation":[{"name":"Innovative Therapies in Pediatric Neurology Research Group Vall d'Hebron Research Institute  Barcelona Spain"},{"name":"Department of Pediatrics, Pediatric Neurology and Psychiatry Vall d'Hebron Hospital  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Marc\u00e9\u2010Grau","sequence":"additional","affiliation":[{"name":"Innovative Therapies in Pediatric Neurology Research Group Vall d'Hebron Research Institute  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luc\u00eda","family":"Dougherty\u2010de Miguel","sequence":"additional","affiliation":[{"name":"Innovative Therapies in Pediatric Neurology Research Group Vall d'Hebron Research Institute  Barcelona Spain"},{"name":"Department of Pediatrics, Pediatric Neurology and Psychiatry Vall d'Hebron Hospital  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victoria","family":"Gonz\u00e1lez","sequence":"additional","affiliation":[{"name":"Innovative Therapies in Pediatric Neurology Research Group Vall d'Hebron Research Institute  Barcelona Spain"},{"name":"Department of Pediatrics, Pediatric Neurology and Psychiatry Vall d'Hebron Hospital  Barcelona Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Moreno\u2010Gald\u00f3","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Universitat Aut\u00f2noma de Barcelona  Barcelona Spain"},{"name":"Department of Pediatrics, Pediatric Neurology and Psychiatry Vall d'Hebron Hospital  Barcelona Spain"},{"name":"Biomedical Research Networking Center on Mental Health  Madrid Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marina A. 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