{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T13:59:54Z","timestamp":1762955994270,"version":"3.37.3"},"reference-count":12,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2018,11,12]],"date-time":"2018-11-12T00:00:00Z","timestamp":1541980800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100008537","name":"Benign Essential Blepharospasm Research Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008537","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2019,2]]},"abstract":"<jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>The clinical manifestation of dystonic spasms in blepharospasm (<jats:styled-content style=\"fixed-case\">BSP<\/jats:styled-content>) patients may be heterogeneous. Whether the varying phenomenology of eyelid spasms becomes manifest sequentially during the course of the disease or aggregates in separate clusters according to different disease courses is still unclear. For this purpose, the clinical features in <jats:styled-content style=\"fixed-case\">BSP<\/jats:styled-content> patients were evaluated longitudinally over a 5\u2010year period and also the blink reflex recovery cycle was tested in a subgroup of <jats:styled-content style=\"fixed-case\">BSP<\/jats:styled-content> patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Sixty <jats:styled-content style=\"fixed-case\">BSP<\/jats:styled-content> patients were videotaped at time 0 and after approximately 5\u00a0years of follow\u2010up. Two experts in movement disorders, who were blinded to the video order, reviewed the videotapes and scored the severity of <jats:styled-content style=\"fixed-case\">BSP<\/jats:styled-content> using the Blepharospasm Severity Rating Scale. Changes in the R2 recovery index were also evaluated in 18 patients twice, i.e. upon enrolment and at the follow\u2010up.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The severity of <jats:styled-content style=\"fixed-case\">BSP<\/jats:styled-content> worsened significantly over the 5\u2010year follow\u2010up period owing to the appearance or the increased duration and frequency of prolonged spasms. It was also found that the blink reflex recovery cycle worsened at follow\u2010up in comparison with the baseline.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study shows that the disease progression of <jats:styled-content style=\"fixed-case\">BSP<\/jats:styled-content> is characterized by the appearance or worsening of prolonged spasms. Prolonged spasms are accompanied by changes in the excitability of brainstem interneurons. Aging\u2010related effects may exacerbate the pathophysiological mechanisms underlying spasms.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.13832","type":"journal-article","created":{"date-parts":[[2018,10,11]],"date-time":"2018-10-11T22:20:50Z","timestamp":1539296450000},"page":"268-273","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Disease progression in blepharospasm: a 5\u2010year longitudinal study"],"prefix":"10.1111","volume":"26","author":[{"given":"G.","family":"Ferrazzano","sequence":"first","affiliation":[{"name":"IRCCS Neuromed Pozzilli Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Conte","sequence":"additional","affiliation":[{"name":"IRCCS Neuromed Pozzilli Italy"},{"name":"Department of Human Neurosciences Sapienza University of Rome Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"A.","family":"Gigante","sequence":"additional","affiliation":[{"name":"Department of Basic Medical Sciences, Neurosciences and Sensory Organs \u2018Aldo Moro\u2019, University of Bari Bari Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Defazio","sequence":"additional","affiliation":[{"name":"Department of Basic Medical Sciences, Neurosciences and Sensory Organs \u2018Aldo Moro\u2019, University of Bari Bari Italy"},{"name":"Department of Medical Sciences and Public Health University of Cagliari Monserrato Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3598-3142","authenticated-orcid":false,"given":"A.","family":"Berardelli","sequence":"additional","affiliation":[{"name":"IRCCS Neuromed Pozzilli Italy"},{"name":"Department of Human Neurosciences Sapienza University of Rome Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G.","family":"Fabbrini","sequence":"additional","affiliation":[{"name":"IRCCS Neuromed Pozzilli Italy"},{"name":"Department of Human Neurosciences Sapienza University of Rome Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,11,12]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e31829bfdf6"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.25567"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.26934"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.26156"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.parkreldis.2017.04.014"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e318296e99d"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1152\/jn.00199.2017"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00221-005-0300-y"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2007.124594"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.20919"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.25088"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000002560"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fene.13832","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.13832","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,14]],"date-time":"2023-09-14T11:35:14Z","timestamp":1694691314000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.13832"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,11,12]]},"references-count":12,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2019,2]]}},"alternative-id":["10.1111\/ene.13832"],"URL":"https:\/\/doi.org\/10.1111\/ene.13832","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"type":"print","value":"1351-5101"},{"type":"electronic","value":"1468-1331"}],"subject":[],"published":{"date-parts":[[2018,11,12]]},"assertion":[{"value":"2018-05-15","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-10-08","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-11-12","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}