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The role of basal ganglia dysfunction in the pathogenesis is unknown.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Pallidal neuronal activity was recorded in six myoclonus\u2013dystonia and six primary generalized dystonia patients operated on for internal globus pallidus deep brain stimulation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In myoclonus\u2013dystonia patients compared with primary\u2010dystonia patients, internal pallidum neurons showed higher burst frequency, lower mean burst, and pause durations. External pallidum neurons showed higher mean pause frequency. Oscillatory activity was present in 33% and 35% of internal pallidum neurons in myoclonus\u2013dystonia and primary\u2010dystonia patients, respectively, predominantly in the theta frequency band (3\u20108 Hz). In myoclonus\u2013dystonia patients with more severe myoclonus, internal pallidum neurons exhibited a higher bursting activity with high intraburst frequency and lower oscillatory activity frequency.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Myoclonus\u2013dystonia appears to be related to specific changes in internal pallidum activity, leading to disruption in striato\u2010pallido\u2010thalamo\u2010cortical circuits. \u00a9 2015 International Parkinson and Movement Disorder Society<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.26244","type":"journal-article","created":{"date-parts":[[2015,4,16]],"date-time":"2015-04-16T07:51:32Z","timestamp":1429170692000},"page":"992-996","source":"Crossref","is-referenced-by-count":38,"title":["Pallidal activity in myoclonus dystonia correlates with motor signs"],"prefix":"10.1002","volume":"30","author":[{"given":"Marie\u2010Laure","family":"Welter","sequence":"first","affiliation":[{"name":"Universit\u00e9 Pierre et 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