{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T02:37:51Z","timestamp":1777516671752,"version":"3.51.4"},"reference-count":11,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2021,1,1]],"date-time":"2021-01-01T00:00:00Z","timestamp":1609459200000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["J Cent Nerv Syst Dis"],"published-print":{"date-parts":[[2021,1]]},"abstract":"<jats:sec>\n                    <jats:title>Background and purpose:<\/jats:title>\n                    <jats:p>In 2017, eculizumab has been approved for treatment-refractory generalised myasthenia gravis (TRgMG). The German Myasthenia Foundation has published a consensus statement on the use of eculizumab, with a recent update. However, a treatment-refractory state is still ill-defined and the term warrants further clarification. We aimed at developing a sum score to operationalise the definition of a TRgMG status, which is easy- to-handle in clinical decision making.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>We established a structured consensus process according to the Delphi consensus methodology, with 12 members of the medical advisory board of the German Myasthenia Foundation. Accordingly, 4 consensus rounds were accomplished. Additionally, a literature survey covering the years 2004-2020 was done and relevant information offered to the consensus group. Consensus criteria were predefined. In the consensus process the relative importance of scoring items were to be consented, with a sum score of 20 and above indicating a TRgMG status.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>The sum score considers the categories disease severity, inefficiency of antecedent therapies, cessation of therapies due to side effects, and long term stay on the intensive care unit. Categories were specified by a total of 13 scoring items. Eventually, the Delphi process developed an unanimous scoring consensus.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>We suggest a sum score to define treatment refractory state in generalised myasthenia gravis. Beyond clarifying the indication of eculizumab, this easy-to-handle score facilitates clinical decision making and offers new inclusion criteria for clinical studies that explore new therapeutic perspectives in myasthenia gravis treatment.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/1179573521989151","type":"journal-article","created":{"date-parts":[[2021,2,2]],"date-time":"2021-02-02T00:34:01Z","timestamp":1612226041000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":9,"title":["A Sum Score to Define Therapy-Refractory Myasthenia Gravis: A German Consensus"],"prefix":"10.1177","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8441-4793","authenticated-orcid":false,"given":"Michael","family":"Schroeter","sequence":"first","affiliation":[{"name":"Department of Neurology, University Cologne and University Hospital, Cologne, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Benjamin","family":"Berger","sequence":"additional","affiliation":[{"name":"Clinic of Neurology and Neurophysiology, Medical Center \u2013 University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg im Breisgau, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Franz","family":"Blaes","sequence":"additional","affiliation":[{"name":"Department of Neurology, Gummersbach Hospital, Gummersbach, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3631-3450","authenticated-orcid":false,"given":"Tim","family":"Hagenacker","sequence":"additional","affiliation":[{"name":"Department of Neurology, University Hospital Essen, Essen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sebastian","family":"Jander","sequence":"additional","affiliation":[{"name":"Department of Neurology, Heinrich-Heine-University, Medical Faculty, D\u00fcsseldorf, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Julia","family":"Kaiser","sequence":"additional","affiliation":[{"name":"Department of Neurology, LVR-Klinik, Bonn, Nordrhein-Westfalen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Petra","family":"Kalischewski","sequence":"additional","affiliation":[{"name":"Neurological outpatient clinic Drs. Kalischewski & Spiegel-Meixensberger, Leipzig, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"De-Hyung","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of Regensburg, Regensburg, Bayern, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tobias","family":"Ruck","sequence":"additional","affiliation":[{"name":"Department of Neurology with Institute of Translational Neurology, University M\u00fcnster, M\u00fcnster, Nordrhein-Westfalen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ulrike","family":"Schara","sequence":"additional","affiliation":[{"name":"Department of Pediatric Neurology, University Clinic Essen, University of Duisburg-Essen, UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peter","family":"Urban","sequence":"additional","affiliation":[{"name":"Department of Neurology, Asklepios Klinik Barmbek, Hamburg, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7233-5342","authenticated-orcid":false,"given":"Andreas","family":"Meisel","sequence":"additional","affiliation":[{"name":"Department of Neurology with Experimental Neurology, Integrated Myasthenia gravis Center, Neurocure Clinical Research Center, Center for Stroke Research Berlin Charit\u00e9 \u2013 Universit\u00e4tsmedizin, corporate member of Freie Universit\u00e4t Berlin, Humboldt-Universit\u00e4t zu Berlin and Berlin Institute of Health, Berlin, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2021,2,1]]},"reference":[{"key":"bibr1-1179573521989151","unstructured":"Schroeter M, Meisel A, Schalke B, et al. Stellungnahme zur Therapie mit Eculizumab im Erwachsenenalter. Accessed August 29, 2020. https:\/\/www.dgn.org\/images\/200525_Stellungnahme_DMG_A%CC%88B_Eculizumab.pdf"},{"key":"bibr2-1179573521989151","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23839"},{"key":"bibr3-1179573521989151","doi-asserted-by":"publisher","DOI":"10.1177\/1756285617749134"},{"key":"bibr4-1179573521989151","doi-asserted-by":"publisher","DOI":"10.1177\/1756286419832242"},{"key":"bibr5-1179573521989151","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25529"},{"key":"bibr6-1179573521989151","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26114"},{"key":"bibr7-1179573521989151","doi-asserted-by":"publisher","DOI":"10.1196\/annals.1405.012"},{"key":"bibr8-1179573521989151","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2020.0851"},{"key":"bibr9-1179573521989151","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-019-09191-6"},{"key":"bibr10-1179573521989151","doi-asserted-by":"publisher","DOI":"10.1097\/CND.0000000000000257"},{"key":"bibr11-1179573521989151","doi-asserted-by":"publisher","DOI":"10.1196\/annals.1254.056"}],"container-title":["Journal of Central Nervous System Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/1179573521989151","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/full-xml\/10.1177\/1179573521989151","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.sagepub.com\/doi\/pdf\/10.1177\/1179573521989151","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T15:59:31Z","timestamp":1777391971000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.sagepub.com\/doi\/10.1177\/1179573521989151"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,1]]},"references-count":11,"alternative-id":["10.1177\/1179573521989151"],"URL":"https:\/\/doi.org\/10.1177\/1179573521989151","relation":{},"ISSN":["1179-5735","1179-5735"],"issn-type":[{"value":"1179-5735","type":"print"},{"value":"1179-5735","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,1]]},"article-number":"1179573521989151"}}