{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T14:45:22Z","timestamp":1786632322351,"version":"build-2736575974"},"reference-count":59,"publisher":"Wiley","issue":"13","license":[{"start":{"date-parts":[[2013,8,7]],"date-time":"2013-08-07T00:00:00Z","timestamp":1375833600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2013,8,7]],"date-time":"2013-08-07T00:00:00Z","timestamp":1375833600000},"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":[[2013,11]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>\n                    Unverricht\u2010Lundborg disease is the most common form of progressive myoclonus epilepsies. In addition to generalized seizures, it is characterized by myoclonus, which usually is the most disabling feature of the disease. Classically, the myoclonus has been attributed to increased excitability of the primary motor cortex. However, inhibitory cortical phenomena have also been described along with anatomical alterations. We aimed to characterize the relationship between the excitability and anatomy of the motor cortex and their association with the severity of the clinical symptoms. Seventy genetically verified patients were compared with forty healthy controls. The symptoms were evaluated with the Unified Myoclonus Rating Scale. Navigated transcranial magnetic stimulation was applied to characterize the excitability of the primary motor cortex by determining the motor thresholds and cortical silent periods. In addition, the induced cortical electric fields were estimated using individual scalp\u2010to\u2010cortex distances measured from MRIs. A cortical thickness analysis was performed to elucidate possible disease\u2010related anatomical alterations. The motor thresholds, cortical electric fields, and silent periods were significantly increased in the patients (\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.01). The silent periods correlated with the myoclonus scores (\n                    <jats:italic>r<\/jats:italic>\n                    = 0.48 to\n                    <jats:italic>r<\/jats:italic>\n                    = 0.49,\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.001). The scalp\u2010to\u2010cortex distance increased significantly with disease duration (\n                    <jats:italic>r<\/jats:italic>\n                    = 0.56,\n                    <jats:italic>P<\/jats:italic>\n                    &lt; 0.001) and correlated inversely with cortical thickness. The results may reflect the refractory nature of the myoclonus and indicate a possible reactive cortical inhibitory mechanism to the underlying disease process. This is the largest clinical series on Unverricht\u2010Lundborg disease and the first study describing parallel pathophysiological and structural alterations associated with the severity of the symptoms. \u00a9 2013 International Parkinson and Movement Disorder Society\n                  <\/jats:p>","DOI":"10.1002\/mds.25615","type":"journal-article","created":{"date-parts":[[2013,8,7]],"date-time":"2013-08-07T16:32:35Z","timestamp":1375893155000},"page":"1860-1867","source":"Crossref","is-referenced-by-count":19,"title":["Alterations of motor cortical excitability and anatomy in Unverricht\u2010Lundborg disease"],"prefix":"10.1002","volume":"28","author":[{"given":"Nils","family":"Danner","sequence":"first","affiliation":[{"name":"Institute of Clinical Medicine, School of Medicine, Faculty of Health Sciences University of Eastern Finland Kuopio Finland"},{"name":"Department of Clinical Neurophysiology Kuopio University Hospital Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Petro","family":"Julkunen","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurophysiology Kuopio University Hospital Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jelena","family":"Hypp\u00f6nen","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurophysiology Kuopio University Hospital Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eini","family":"Niskanen","sequence":"additional","affiliation":[{"name":"Department of Clinical Radiology Kuopio University Hospital Kuopio Finland"},{"name":"Department of Applied Physics University of Eastern Finland Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"S\u00e4is\u00e4nen","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine, School of Medicine, Faculty of Health Sciences University of Eastern Finland Kuopio Finland"},{"name":"Department of Clinical Neurophysiology Kuopio University Hospital Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mervi","family":"K\u00f6n\u00f6nen","sequence":"additional","affiliation":[{"name":"Department of Clinical Neurophysiology Kuopio University Hospital Kuopio Finland"},{"name":"Department of Clinical Radiology Kuopio University Hospital Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P\u00e4ivi","family":"Koskenkorva","sequence":"additional","affiliation":[{"name":"Department of Clinical Radiology Kuopio University Hospital Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ritva","family":"Vanninen","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine, School of Medicine, Faculty of Health Sciences University of Eastern Finland Kuopio Finland"},{"name":"Department of Clinical Radiology Kuopio University Hospital Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Reetta","family":"K\u00e4lvi\u00e4inen","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine, School of Medicine, Faculty of Health Sciences University of Eastern Finland Kuopio Finland"},{"name":"Department of Neurology Kuopio University Hospital Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Esa","family":"Mervaala","sequence":"additional","affiliation":[{"name":"Institute of Clinical Medicine, School of Medicine, Faculty of Health Sciences University of Eastern Finland Kuopio Finland"},{"name":"Department of Clinical Neurophysiology Kuopio University Hospital Kuopio Finland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,8,7]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(05)70043-0"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1528-1157.44.s11.4.x"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/386847a0"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng0497-393"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.271.5256.1731"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1600-0404.1974.tb02782.x"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1979.tb01770.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2008.01546.x"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2006.00553.x"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20234"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neucli.2006.10.001"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2010.01.006"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.57.10.1793"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.43.1_Part_1.186"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2009.02.015"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0013-4694(98)00115-1"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.23813"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3181b3888b"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A2882"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1097\/WNP.0b013e31818e7944"},{"key":"e_1_2_8_22_1","first-page":"361","article-title":"The Unified Myoclonus Rating Scale","volume":"89","author":"Frucht SJ","year":"2002","journal-title":"Adv Neurol."},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneumeth.2011.10.004"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2004.07.045"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1098\/rstb.2001.0915"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1109\/42.668698"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1002\/hbm.10062"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2004.05.007"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2005.03.036"},{"key":"e_1_2_8_30_1","unstructured":"ChungMK TaylorJ.Diffusion smoothing on brain surface via finite element method. IEEE International Symposium on Biomedical Engineering: From Nano to Macro.2004;1:432\u2013435."},{"key":"e_1_2_8_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2009.08.016"},{"key":"e_1_2_8_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneumeth.2008.08.023"},{"key":"e_1_2_8_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneumeth.2007.12.005"},{"key":"e_1_2_8_34_1","doi-asserted-by":"publisher","DOI":"10.1006\/nimg.2001.1037"},{"key":"e_1_2_8_35_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-010-0738-2"},{"key":"e_1_2_8_36_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neucli.2006.12.008"},{"key":"e_1_2_8_37_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-3223(00)01039-8"},{"key":"e_1_2_8_38_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.brainres.2006.04.094"},{"key":"e_1_2_8_39_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1528-1157.2002.09902.x"},{"key":"e_1_2_8_40_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2008.01562.x"},{"key":"e_1_2_8_41_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1157.1999.tb00711.x"},{"key":"e_1_2_8_42_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2004.03.006"},{"key":"e_1_2_8_43_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410340308"},{"key":"e_1_2_8_44_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00702-009-0195-z"},{"key":"e_1_2_8_45_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00213-010-2126-y"},{"key":"e_1_2_8_46_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00221-006-0506-7"},{"key":"e_1_2_8_47_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.eplepsyres.2004.08.001"},{"key":"e_1_2_8_48_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2008.02110.x"},{"key":"e_1_2_8_49_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.1993.sp019732"},{"key":"e_1_2_8_50_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00221-004-2134-4"},{"key":"e_1_2_8_51_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2010.02557.x"},{"key":"e_1_2_8_52_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2012.04.005"},{"key":"e_1_2_8_53_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.WNL.0000147475.71345.AA"},{"key":"e_1_2_8_54_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.58.11.1686"},{"key":"e_1_2_8_55_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2007.01118.x"},{"key":"e_1_2_8_56_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.40.1.53"},{"key":"e_1_2_8_57_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1388-2457(02)00196-7"},{"key":"e_1_2_8_58_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuroimage.2006.06.036"},{"key":"e_1_2_8_59_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2012.11.011"},{"key":"e_1_2_8_60_1","article-title":"Simultaneous EEG\u2010fMRI in patients with Unverricht\u2010Lundborg disease: event\u2010related desynchronization\/synchronization and hemodynamic response analysis","volume":"164278","author":"Visani E","year":"2010","journal-title":"Comput Intell Neurosci."}],"container-title":["Movement Disorders"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.25615","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fmds.25615","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.25615","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mds.25615","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mds.25615","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T13:54:39Z","timestamp":1786629279000},"score":1,"resource":{"primary":{"URL":"https:\/\/movementdisorders.onlinelibrary.wiley.com\/doi\/10.1002\/mds.25615"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,8,7]]},"references-count":59,"journal-issue":{"issue":"13","published-print":{"date-parts":[[2013,11]]}},"alternative-id":["10.1002\/mds.25615"],"URL":"https:\/\/doi.org\/10.1002\/mds.25615","archive":["Portico"],"relation":{},"ISSN":["0885-3185","1531-8257"],"issn-type":[{"value":"0885-3185","type":"print"},{"value":"1531-8257","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,8,7]]}}}