{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T02:25:04Z","timestamp":1784859904012,"version":"3.55.0"},"reference-count":29,"publisher":"Wiley","issue":"16","license":[{"start":{"date-parts":[[2010,10,11]],"date-time":"2010-10-11T00:00:00Z","timestamp":1286755200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2010,12,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We used electroencephalography (EEG)\u2010polygraphic recordings to classify myoclonus in 109 patients with Creutzfeldt\u2010Jakob disease (CJD) on the basis of its electromyography (EMG) pattern, time course, distribution, and EEG correlates. We recorded myoclonic jerks in 55 patients (50.4%), and we classified them as periodic myoclonus in 28, rhythmic in 13, and irregular in 20 (6 patients showed two types of myoclonus). Myoclonus occurred as a prominently negative event (interrupting the EMG discharge) in 10. Periodic sharp\u2010wave complexes (PSWCs) were present in all but one patient with myoclonic jerks but were time\u2010locked with EMG\u2010bursts only in case of periodic myoclonus. Jerk\u2010locked back averaging revealed a variable EEG\u2010EMG transfer\u2010time commonly exceeding that characterizing cortical myoclonus. Myoclonus was frequently associated with Met\/Met polymorphism at codon 129 of the prion protein gene, but it was also observed in association with Met\/Val or Val\/Val polymorphisms provided that the EEG showed the presence of the PSWC pattern. The presence of enlarged somatosensory evoked potentials significantly correlated with the myoclonic presentation, as did MR signal hyperintensity involving the cortical mantle. Our observations on the basis of standard polygraphic criteria suggest that CJD associates with a remarkable variety of myoclonic jerks, and therefore different brain structures are probably involved as generators. The significant association between the presence of all myoclonus types with PSWCs suggests that hyperexcitable corticosubcortical loops are always required to generate (or allow) both myoclonus and the EEG complexes, either they are time locked or not. \u00a9 2010 Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.23397","type":"journal-article","created":{"date-parts":[[2010,10,11]],"date-time":"2010-10-11T14:52:19Z","timestamp":1286808739000},"page":"2818-2827","source":"Crossref","is-referenced-by-count":37,"title":["Myoclonus in Creutzfeldt\u2010Jakob disease: Polygraphic and video\u2010electroencephalography assessment of 109 patients"],"prefix":"10.1002","volume":"25","author":[{"given":"Simona","family":"Binelli","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pamela","family":"Agazzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Canafoglia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vidmer","family":"Scaioli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ferruccio","family":"Panzica","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Visani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Di Fede","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giorgio","family":"Giaccone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Bizzi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Orso","family":"Bugiani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guiliano","family":"Avanzini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabrizio","family":"Tagliavini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvana","family":"Franceschetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,10,11]]},"reference":[{"key":"e_1_2_7_2_2","unstructured":"WHO. Manual for surveillance of human transmissible spongiform encephalopathies including variant Creutzfeldt\u2010Jakob disease. 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