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Garfield Weston Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000243","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100002347","name":"BMBF","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[2017,10]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To characterize neurophysiological subcortical abnormalities in myoclonus\u2013dystonia and their modulation by alcohol administration.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Cerebellar associative learning and basal ganglia\u2013brainstem interaction were investigated in 17 myoclonus\u2013dystonia patients with epsilon\u2010sarcoglycan (<jats:italic>SGCE<\/jats:italic>) gene mutation and 21 age\u2010 and sex\u2010matched healthy controls by means of classical eyeblink conditioning and blink reflex recovery cycle before and after alcohol intake resulting in a breath alcohol concentration of 0.08% (0.8g\/l). The alcohol responsiveness of clinical symptoms was evaluated by 3 blinded raters with a standardized video protocol and clinical rating scales including the Unified Myoclonus Rating Scale and the Burke\u2013Fahn\u2013Marsden Dystonia Rating Scale.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients showed a significantly reduced number of conditioned eyeblink responses before alcohol administration compared to controls. Whereas the conditioning response rate decreased under alcohol intake in controls, it increased in patients (analysis of variance: alcohol state\u2009\u00d7\u2009group, <jats:italic>p<\/jats:italic>\u2009=\u20090.004). Blink reflex recovery cycle before and after alcohol intake did not differ between groups. Myoclonus improved significantly after alcohol intake (<jats:italic>p<\/jats:italic>\u2009=\u20090.016). The severity of action myoclonus at baseline correlated negatively with the conditioning response in classical eyeblink conditioning in patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>The combination of findings of reduced baseline acquisition of conditioned eyeblink responses and normal blink reflex recovery cycle in patients who improved significantly with alcohol intake suggests a crucial role of cerebellar networks in the generation of symptoms in these patients. Ann Neurol 2017;82:543\u2013553<\/jats:p><\/jats:sec>","DOI":"10.1002\/ana.25035","type":"journal-article","created":{"date-parts":[[2017,9,4]],"date-time":"2017-09-04T15:32:37Z","timestamp":1504539157000},"page":"543-553","source":"Crossref","is-referenced-by-count":52,"title":["Alcohol improves cerebellar learning deficit in myoclonus\u2013dystonia: A clinical and electrophysiological investigation"],"prefix":"10.1002","volume":"82","author":[{"given":"Anne","family":"Weissbach","sequence":"first","affiliation":[{"name":"Institute of Neurogenetics University of L\u00fcbeck L\u00fcbeck Germany"},{"name":"Department of Neurology University of L\u00fcbeck L\u00fcbeck Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elisa","family":"Werner","sequence":"additional","affiliation":[{"name":"Institute of Neurogenetics University of L\u00fcbeck L\u00fcbeck Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julien F.","family":"Bally","sequence":"additional","affiliation":[{"name":"Morton and Gloria Shulman Movement Disorder Clinic and Edmond J. 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