{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,3]],"date-time":"2026-02-03T11:00:58Z","timestamp":1770116458145,"version":"3.49.0"},"reference-count":13,"publisher":"Georg Thieme Verlag KG","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Fortschr Neurol Psychiatr"],"abstract":"Zusammenfassung<\/jats:title>\n Myasthenia gravis (MG) und das Lambert-Eaton-Myasthenie-Syndrom (LEMS) sind\n seltene, autoimmun-vermittelte neuromuskul\u00e4re Erkrankungen, die mit\n belastungsabh\u00e4ngiger Muskelschw\u00e4che einhergehen. W\u00e4hrend f\u00fcr MG verschiedene\n Therapieoptionen existieren, l\u00e4sst sich eine Untergruppe der Patienten als\n Therapie-refrakt\u00e4r einstufen, wobei in der wissenschaftlichen Literatur\n unterschiedliche Definitionen dieser Therapie-Refrakt\u00e4rit\u00e4t existieren.<\/jats:p>\n In dieser monozentrischen, retrospektiven Kohortenstudie wurden 200\n MG-Patienten und 15 LEMS-Patienten untersucht. Es wurden Daten zu klinischen\n Merkmalen, Antik\u00f6rperstatus, Therapieansprechen und Begleiterkrankungen\n erfasst. Als Therapie-refrakt\u00e4r galten diejenigen MG-Patienten, die in ihrem\n Krankheitsverlauf mindestens drei verschiedene Immunsuppressiva erhielten.\n Statistische Analysen wurden mit SPSS durchgef\u00fchrt.<\/jats:p>\n 11,5% der MG-Patienten (n=23) wurden als Therapie-refrakt\u00e4r klassifiziert.\n Diese wiesen h\u00e4ufiger eine schwere Krankheitsauspr\u00e4gung gem\u00e4\u00df\n MGFA-Klassifikation, eine h\u00f6here Pr\u00e4valenz von MuSK-Antik\u00f6rpern sowie\n vermehrt kardiovaskul\u00e4re Komorbidit\u00e4ten auf.<\/jats:p>\n Die Ergebnisse zeigen, dass Patienten in der Therapie-refrakt\u00e4ren Gruppe\n h\u00e4ufiger schwere Krankheitsverl\u00e4ufe gem\u00e4\u00df der MGFA-Klassifikation aufwiesen\n und h\u00e4ufiger Eskalationstherapien ben\u00f6tigten. Eine einheitliche Definition\n des Begriffes der Therapie-refrakt\u00e4ren MG ist jedoch nicht verf\u00fcgbar.<\/jats:p>","DOI":"10.1055\/a-2777-1902","type":"journal-article","created":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T23:39:07Z","timestamp":1770075547000},"source":"Crossref","is-referenced-by-count":0,"title":["Myasthene Syndrome: Retrospektive Analyse zweier\n Patientenkohorten"],"prefix":"10.1055","author":[{"given":"Felix Maximilian Xaver","family":"Lorenz","sequence":"additional","affiliation":[{"name":"Friedrich-Baur-Institut, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen,\n M\u00fcnchen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2757-8131","authenticated-orcid":false,"given":"Benedikt","family":"Schoser","sequence":"additional","affiliation":[{"name":"Friedrich-Baur-Institut, Ludwig-Maximilians-Universit\u00e4t M\u00fcnchen,\n M\u00fcnchen, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2026,2,2]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"7","DOI":"10.25122\/jml-2020-0145","article-title":"The epidemiology of myasthenia gravis","volume":"14","author":"A M Bubuioc","year":"2021","journal-title":"J Med Life"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"976","DOI":"10.1016\/S1474-4422(17)30369-1","article-title":"Safety and efficacy of eculizumab in anti-acetylcholine receptor\n antibody-positive refractory generalised myasthenia gravis (REGAIN): a phase 3,\n randomised, double-blind, placebo-controlled, multicentre study","volume":"16","author":"J F Howard","year":"2017","journal-title":"Lancet Neurol"},{"key":"ref6","first-page":"305","volume-title":"Rebooting the immune system with high-dose cyclophosphamide for treatment of\n refractory myasthenia gravis","author":"D B Drachman","year":"2008"},{"key":"ref8","first-page":"Doc12","article-title":"Refractory myasthenia gravis \u2013 clinical profile, comorbidities and response to\n rituximab","volume":"14","author":"S R Sudulagunta","year":"2016","journal-title":"Ger Med Sci"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"511","DOI":"10.1056\/NEJMoa1602489","article-title":"Randomized Trial of Thymectomy in Myasthenia Gravis","volume":"375","author":"G I Wolfe","year":"2016","journal-title":"New England Journal of Medicine"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"48","DOI":"10.5152\/eurasianjmed.2017.17009","article-title":"Thymectomy in Myasthenia Gravis","volume":"49","author":"Y Aydin","year":"2017","journal-title":"Eurasian Journal of Medicine. AVES Ibrahim Kara"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1002\/acn3.51492","article-title":"Drug-refractory myasthenia gravis: Clinical characteristics, treatments, and\n outcome","volume":"9","author":"E Cort\u00e9s-Vicente","year":"2022","journal-title":"Ann Clin Transl Neurol"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1111\/ene.12599","article-title":"Myasthenia gravis and risks for comorbidity","volume":"22","author":"N E Gilhus","year":"2015","journal-title":"European Journal of Neurology. Blackwell Publishing\n Ltd"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1002\/jca.21868","article-title":"Treatment of refractory myasthenia gravis by double-filtration plasmapheresis\n and rituximab: A case series of nine patients and literature review","volume":"36","author":"H N Bennani","year":"2021","journal-title":"J Clin Apher"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"902","DOI":"10.1200\/JCO.2010.32.0440","article-title":"Clinical Dutch-English Lambert-Eaton Myasthenic Syndrome (LEMS) Tumor\n Association Prediction Score Accurately Predicts Small-Cell Lung Cancer in the\n LEMS","volume":"29","author":"M J Titulaer","year":"2011","journal-title":"Journal of Clinical Oncology"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1007\/s00415-003-1063-7","article-title":"The epidemiology of myasthenia gravis, Lambert-Eaton myasthenic syndrome and\n their associated tumours in the northern part of the province of South\n Holland","volume":"250","author":"P W Wirtz","year":"2003","journal-title":"J Neurol"},{"key":"ref20","first-page":"379","volume-title":"Lambert-Eaton Myasthenic Syndrome","author":"V G Kesner","year":"2018"},{"key":"ref22","volume-title":"When myasthenia gravis is deemed refractory: Clinical signposts and treatment\n strategies","author":"R Mantegazza","year":"2018"}],"container-title":["Fortschritte der Neurologie \u00b7 Psychiatrie"],"original-title":[],"language":"de","link":[{"URL":"http:\/\/www.thieme-connect.de\/products\/ejournals\/pdf\/10.1055\/a-2777-1902.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T23:39:11Z","timestamp":1770075551000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.thieme-connect.de\/DOI\/DOI?10.1055\/a-2777-1902"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,2]]},"references-count":13,"URL":"https:\/\/doi.org\/10.1055\/a-2777-1902","archive":["Portico","CLOCKSS"],"relation":{},"ISSN":["0720-4299","1439-3522"],"issn-type":[{"value":"0720-4299","type":"print"},{"value":"1439-3522","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,2]]}}}