{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T12:13:10Z","timestamp":1790770390121,"version":"4.1.0"},"reference-count":18,"publisher":"S. Karger AG","issue":"6","license":[{"start":{"date-parts":[[2008,1,1]],"date-time":"2008-01-01T00:00:00Z","timestamp":1199145600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"},{"start":{"date-parts":[[2008,1,1]],"date-time":"2008-01-01T00:00:00Z","timestamp":1199145600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.karger.com\/Services\/SiteLicenses"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neurodegener Dis"],"published-print":{"date-parts":[[2008]]},"abstract":"<i>Background and Purpose:<\/i> Myoclonus-dystonia (MD) is a rare movement disorder characterized by myoclonic jerks, dystonia and a variety of psychiatric symptoms. Neuroimaging and electrophysiologic studies have not been able to detect any specific central nervous system abnormality. We report for the first time a well-characterized case with MD and abnormal brain perfusion imaging using single photon emission computed tomography (SPECT) with <sup>99m<\/sup>Tc-ethyl cysteinate dimer (ECD). A review of the literature on the phenotypic and pathogenetic considerations for MD is also presented. <i>Methods:<\/i> To better define the functional regional central nervous system involvement in MD, we conducted a brain perfusion SPECT with <sup>99m<\/sup>Tc-ECD in a patient diagnosed with typical disease. <i>Results:<\/i> Analysis of the SPECT data revealed significantly reduced regional cerebral blood flow (rCBF) in both temporal lobes (left > right and medial > lateral). Reduced rCBF was also observed in both frontal lobes and the right caudate nucleus. <i>Conclusions:<\/i> Our findings of reduced frontotemporal and striatal rCBF in the absence of other neuroimaging and electrophysiologic findings correlate well with the clinical manifestations in our patient and suggest possible functional\/metabolic involvement of these areas in the etiopathogenesis of MD.<\/jats:p>","DOI":"10.1159\/000119458","type":"journal-article","created":{"date-parts":[[2008,2,29]],"date-time":"2008-02-29T11:12:53Z","timestamp":1204283573000},"page":"355-358","source":"Crossref","is-referenced-by-count":10,"title":["Frontotemporal and Striatal SPECT Abnormalities in Myoclonus-Dystonia: Phenotypic and Pathogenetic Considerations"],"prefix":"10.1159","volume":"5","author":[{"given":"Spiridon","family":"Papapetropoulos","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas A.","family":"Argyriou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Panagiotis","family":"Polychronopoulos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Trifonas","family":"Spyridonidis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippos","family":"Gourzis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisabeth","family":"Chroni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"127","published-online":{"date-parts":[[2008,2,29]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","DOI":"10.1002%2Fmds.870110202"},{"key":"ref2","doi-asserted-by":"publisher","DOI":"10.1086%2F344531"},{"key":"ref3","doi-asserted-by":"publisher","DOI":"10.1007%2Fs00259-002-0814-5"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1002%2F1531-8249%28200101%2949%3A1%3C121%3A%3AAID-ANA20%3E3.0.CO%3B2-8"},{"key":"ref5","doi-asserted-by":"publisher","DOI":"10.1002%2F1531-8249%28199911%2946%3A5%3C794%3A%3AAID-ANA19%3E3.0.CO%3B2-2"},{"key":"ref6","doi-asserted-by":"publisher","DOI":"10.1073%2Fpnas.96.9.5173"},{"key":"ref7","doi-asserted-by":"publisher","DOI":"10.1001%2Farchneur.59.8.1244"},{"key":"ref8","doi-asserted-by":"publisher","DOI":"10.1016%2F0387-7604%2893%2990027-6"},{"key":"ref9","doi-asserted-by":"publisher","DOI":"10.1016%2F0022-3999%2892%2990128-O"},{"key":"ref10","doi-asserted-by":"publisher","DOI":"10.1016%2F0304-3959%2886%2990207-1"},{"key":"ref11","doi-asserted-by":"publisher","DOI":"10.1016%2FS0165-0327%2899%2900110-X"},{"key":"ref12","doi-asserted-by":"publisher","DOI":"10.1159%2F000067220"},{"key":"ref13","doi-asserted-by":"publisher","DOI":"10.1038%2Fng1194-236"},{"key":"ref14","doi-asserted-by":"publisher","DOI":"10.1093%2Fhmg%2F9.9.1403"},{"key":"ref15","doi-asserted-by":"publisher","DOI":"10.1002%2F1531-8249%28200007%2948%3A1%3C127%3A%3AAID-ANA24%3E3.0.CO%3B2-D"},{"key":"ref16","doi-asserted-by":"publisher","DOI":"10.1016%2Fj.neuroimage.2003.10.019"},{"key":"ref17","doi-asserted-by":"publisher","DOI":"10.1002%2Fmds.21284"},{"key":"ref18","doi-asserted-by":"publisher","DOI":"10.1097%2F00003072-200102000-00020"}],"container-title":["Neurodegenerative Diseases"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/119458","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.karger.com\/Article\/Pdf\/119458","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,4,24]],"date-time":"2025-04-24T02:16:33Z","timestamp":1745460993000},"score":1,"resource":{"primary":{"URL":"https:\/\/karger.com\/article\/doi\/10.1159\/000119458"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2008]]},"references-count":18,"journal-issue":{"issue":"6","published-online":{"date-parts":[[2008,9,1]]}},"URL":"https:\/\/doi.org\/10.1159\/000119458","archive":["Portico"],"relation":{},"ISSN":["1660-2854","1660-2862"],"issn-type":[{"value":"1660-2854","type":"print"},{"value":"1660-2862","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008]]}}}