{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T17:28:29Z","timestamp":1791912509977,"version":"4.3.4"},"reference-count":10,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2021,6,9]],"date-time":"2021-06-09T00:00:00Z","timestamp":1623196800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2021,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Eculizumab has been shown to be efficacious in acetylcholine receptor antibody\u2013positive (AChR<jats:sup>+<\/jats:sup>) Myasthenia Gravis Foundation of America (MGFA) class II, III, and IV generalized myasthenia gravis (gMG) patients. However, it has not been studied in MGFA class V gMG patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We report three AChR<jats:sup>+<\/jats:sup>, refractory, MGFA class V gMG patients treated with eculizumab. MGFA class, MG\u2010Composite (MGC) score and MG Activities of Daily Living (MG\u2010ADL) score were assessed before and after eculizumab.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Two of three gMG patients, refractory to intravenous immunoglobulin, plasmapheresis, prednisone, and (in one case) rituximab, showed a robust response to eculizumab with marked improvement in MGFA, MG\u2010ADL, and MGC measures. The third patient showed a partial response to eculizumab but remained on noninvasive ventilation and gastrostomy intubation. Patients 1 and 2 achieved minimal manifestation status at week 4 and week 6, respectively, and showed continued improvement on MG\u2010ADL and MGC scores through weeks 55 and 43, respectively, with eculizumab. The third patient showed a partial response at week 10, followed by a slight decline in his MG\u2010ADL score, but noted a slow but an incomplete improvement afterward on MG\u2010ADL and MGC scores, possibly due to delayed eculizumab infusion.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Eculizumab may play a role in the treatment of patients with MGFA class V, refractory gMG. Larger studies are required to provide further evidence.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27326","type":"journal-article","created":{"date-parts":[[2021,5,19]],"date-time":"2021-05-19T07:47:57Z","timestamp":1621410477000},"page":"212-215","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":38,"title":["The use of eculizumab in ventilator\u2010dependent myasthenia gravis patients"],"prefix":"10.1002","volume":"64","author":[{"given":"Uzma","family":"Usman","sequence":"first","affiliation":[{"name":"Department of Neurology Yale University  New Haven Connecticut USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christina","family":"Chrisman","sequence":"additional","affiliation":[{"name":"Department of Neurology Banner University Medical Centre, Phoenix, AZ  Phoenix Arizona USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Drew","family":"Houston","sequence":"additional","affiliation":[{"name":"Department of Neurology Barrow Neurological Institute. Phoenix. AZ  Phoenix Arizona USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clara Chow","family":"Haws","sequence":"additional","affiliation":[{"name":"Department of Neurology Phoenix Children Hospital  Phoenix Arizona USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Neurology Banner University Medical Centre, Phoenix, AZ  Phoenix Arizona USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suraj","family":"Muley","sequence":"additional","affiliation":[{"name":"Department of Neurology Barrow Neurological Institute. Phoenix. AZ  Phoenix Arizona USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,6,9]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(17)30369-1"},{"key":"e_1_2_8_3_1","first-page":"565","article-title":"Eculizumab treatment for refractory generalized myasthenia gravis","volume":"71","author":"Uzawa A","year":"2019","journal-title":"Brain Nerve."},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26447"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000011207"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1080\/25785826.2020.1724756"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26156"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000004341"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25597"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.3233\/JND-180300"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2016.4190"}],"container-title":["Muscle &amp; Nerve"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.27326","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/mus.27326","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/mus.27326","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T17:52:30Z","timestamp":1693331550000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/mus.27326"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6,9]]},"references-count":10,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2021,8]]}},"alternative-id":["10.1002\/mus.27326"],"URL":"https:\/\/doi.org\/10.1002\/mus.27326","archive":["Portico"],"relation":{},"ISSN":["0148-639X","1097-4598"],"issn-type":[{"value":"0148-639X","type":"print"},{"value":"1097-4598","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,6,9]]},"assertion":[{"value":"2020-11-18","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-05-16","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2021-06-09","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}