{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T19:32:13Z","timestamp":1786476733074,"version":"build-2736575974"},"reference-count":13,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2006,6,5]],"date-time":"2006-06-05T00:00:00Z","timestamp":1149465600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2006,6,5]],"date-time":"2006-06-05T00:00:00Z","timestamp":1149465600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2006,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>We here describe a patient with late\u2010infantile Niemann\u2013Pick disease type C (NPC) presenting with worsening myoclonus, seizures, cerebellar symptoms, mild mental impairment, and gaze palsy. Electroencephalographic (EEG) \u2013polymyographic examinations showed abnormally high and diffuse background alpha\u2010activity, enhanced by intermittent photic stimulation. The electromyographic (EMG) showed quasirhythmic myoclonic jerks during motor activation. EEG\u2013EMG frequency analysis (better than jerk\u2010locked back\u2010averaging) demonstrated the cortical origin of the myoclonus. Our observations indicate that cortical myoclonus may occur as the main symptom of NPC. \u00a9 2006 Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.20984","type":"journal-article","created":{"date-parts":[[2006,6,5]],"date-time":"2006-06-05T17:00:20Z","timestamp":1149526820000},"page":"1453-1456","source":"Crossref","is-referenced-by-count":24,"title":["Rhythmic cortical myoclonus in Niemann\u2013Pick disease type C"],"prefix":"10.1002","volume":"21","author":[{"given":"Laura","family":"Canafoglia","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marianna","family":"Bugiani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graziella","family":"Uziel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernardo","family":"Dalla Bernardina","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Ciano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vidmer","family":"Scaioli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuliano","family":"Avanzini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvana","family":"Franceschetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ferruccio","family":"Panzica","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2006,6,5]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-0004.2003.00147.x"},{"key":"e_1_2_6_3_2","first-page":"151","article-title":"Sphingomyelin storage in a patient with myoclonus epilepsy as a main clinical symptom\u2013a variant of Niemann\u2010Pick disease Type C?","volume":"49","author":"Kunishita T","year":"1979","journal-title":"Jpn J Exp Med"},{"key":"e_1_2_6_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/S1388-2457(03)00066-X"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00713855"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410280129"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1097\/00004691-199107010-00003"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/S0167-8760(97)00754-X"},{"key":"e_1_2_6_9_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.28.10.1037"},{"key":"e_1_2_6_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/122.3.461"},{"key":"e_1_2_6_11_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awg043"},{"key":"e_1_2_6_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10584"},{"key":"e_1_2_6_13_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.40.10.1561"},{"key":"e_1_2_6_14_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.2005.00385.x"}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.20984","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.20984","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mds.20984","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.20984","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,11]],"date-time":"2026-08-11T19:12:52Z","timestamp":1786475572000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.20984"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2006,6,5]]},"references-count":13,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2006,9]]}},"alternative-id":["10.1002\/mds.20984"],"URL":"https:\/\/doi.org\/10.1002\/mds.20984","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"value":"0885-3185","type":"print"},{"value":"1531-8257","type":"electronic"}],"subject":[],"published":{"date-parts":[[2006,6,5]]}}}