{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,31]],"date-time":"2026-10-31T13:43:17Z","timestamp":1793454197026,"version":"4.5.0"},"reference-count":71,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,12,21]],"date-time":"2017-12-21T00:00:00Z","timestamp":1513814400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of the New York Academy of Sciences"],"published-print":{"date-parts":[[2018,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Myasthenia gravis with antibodies to the muscle\u2010specific tyrosine kinase (MuSK<jats:sup>+<\/jats:sup> MG) is a rare disease with distinctive pathogenic mechanisms and clinical features. An acute onset and predominant bulbar muscle weakness are very common and highly suggestive of the disease. On the other hand, a more indolent course, atypical ocular presentation, and signs of cholinergic hyperactivity may complicate the diagnosis. Though MuSK<jats:sup>+<\/jats:sup> MG is still a severe disease, over the years we have observed a steady reduction in the rate of respiratory crisis and a significant improvement in the clinical outcome, both likely related to earlier diagnosis and timely treatment. Despite the improved management, MuSK<jats:sup>+<\/jats:sup> MG patients tend to remain dependent on long\u2010term immunosuppressive treatment and may develop permanent disabling weakness. In uncontrolled studies, B cell depletion with rituximab proved effective in most patients with refractory disease, inducing prolonged clinical responses associated with a sustained reduction of serum antibody levels. Promising results from experimental studies and case reports suggest that both 3,4\u2010diaminopyridine and albuterol may be effective as symptomatic agents.<\/jats:p>","DOI":"10.1111\/nyas.13518","type":"journal-article","created":{"date-parts":[[2017,12,21]],"date-time":"2017-12-21T15:50:38Z","timestamp":1513871438000},"page":"82-89","source":"Crossref","is-referenced-by-count":143,"title":["Myasthenia gravis with antibodies to MuSK: an update"],"prefix":"10.1111","volume":"1412","author":[{"given":"Amelia","family":"Evoli","sequence":"first","affiliation":[{"name":"Institute of Neurology, Catholic University Fondazione Policlinico Gemelli  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo E.","family":"Alboini","sequence":"additional","affiliation":[{"name":"Institute of Neurology, Catholic University Fondazione Policlinico Gemelli  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valentina","family":"Damato","sequence":"additional","affiliation":[{"name":"Institute of Neurology, Catholic University Fondazione Policlinico Gemelli  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raffaele","family":"Iorio","sequence":"additional","affiliation":[{"name":"Institute of Neurology, Catholic University Fondazione Policlinico Gemelli  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlo","family":"Provenzano","sequence":"additional","affiliation":[{"name":"Institute of General Pathology, Catholic University Fondazione Policlinico Gemelli  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emanuela","family":"Bartoccioni","sequence":"additional","affiliation":[{"name":"Institute of General Pathology, Catholic University Fondazione Policlinico Gemelli  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mariapaola","family":"Marino","sequence":"additional","affiliation":[{"name":"Institute of General Pathology, Catholic University Fondazione Policlinico Gemelli  Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,12,21]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1101\/cshperspect.a009167"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms18040896"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature11348"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2016.11.005"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.1313944110"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cbi.2016.04.019"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-015-7963-5"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2008.01.013"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2015.12.016"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2013.12.005"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000077"},{"key":"e_1_2_7_13_1","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.jaut.2013.12.007","article-title":"T cell repertoire in DQ5\u2010positive MuSK\u2010positive myasthenia gravis patients","volume":"52","author":"Marino M.P.","year":"2015","journal-title":"J. Autoimmun."},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.22006"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1468-1331.2009.02624.x"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2006.102517"},{"key":"e_1_2_7_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2013.07.010"},{"key":"e_1_2_7_18_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2005.075812"},{"key":"e_1_2_7_19_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2017-315782"},{"key":"e_1_2_7_20_1","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.54.4645"},{"key":"e_1_2_7_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2016.04.004"},{"key":"e_1_2_7_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23907"},{"key":"e_1_2_7_23_1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000357"},{"key":"e_1_2_7_24_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2015.0203"},{"key":"e_1_2_7_25_1","doi-asserted-by":"crossref","first-page":"2304","DOI":"10.1093\/brain\/awg223","article-title":"Clinical correlates with anti\u2010MuSK antibodies in generalized seronegative myasthenia gravis","volume":"156","author":"Evoli A.","year":"2003","journal-title":"Brain"},{"key":"e_1_2_7_26_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2008.05.032"},{"key":"e_1_2_7_27_1","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1136\/bjo.2006.108498","article-title":"Ocular myasthenia: a rare presentation with MuSK antibody and bilateral extraocular muscle atrophy","volume":"91","author":"Chan J.W.","year":"2006","journal-title":"Br. J. Ophthalmol."},{"key":"e_1_2_7_28_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clinph.2006.10.004"},{"key":"e_1_2_7_29_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13760-014-0364-1"},{"key":"e_1_2_7_30_1","doi-asserted-by":"publisher","DOI":"10.1196\/annals.1405.021"},{"key":"e_1_2_7_31_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2016.08.005"},{"key":"e_1_2_7_32_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1552-6569.2009.00456.x"},{"key":"e_1_2_7_33_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2990.2008.00965.x"},{"key":"e_1_2_7_34_1","doi-asserted-by":"crossref","first-page":"359","DOI":"10.5414\/NP300875","article-title":"Reduced muscle mitochondrial enzyme activity in MuSK\u2010immunized mice","volume":"34","author":"\u00d6zk\u00f6k E.","year":"2015","journal-title":"Clin. Neuropathol."},{"key":"e_1_2_7_35_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.55.1.16"},{"key":"e_1_2_7_36_1","doi-asserted-by":"publisher","DOI":"10.1196\/annals.1405.012"},{"key":"e_1_2_7_37_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.2012.06781.x"},{"key":"e_1_2_7_38_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0b013e32833c0982"},{"key":"e_1_2_7_39_1","doi-asserted-by":"publisher","DOI":"10.1097\/WCO.0000000000000136"},{"key":"e_1_2_7_40_1","first-page":"255","article-title":"Clinical characteristics of refractory myasthenia gravis patients","volume":"86","author":"Suh J.","year":"2013","journal-title":"Yale J. Biol. Med."},{"key":"e_1_2_7_41_1","doi-asserted-by":"publisher","DOI":"10.1097\/CND.0000000000000034"},{"key":"e_1_2_7_42_1","article-title":"Refractory myasthenia gravis\u2014clinical profile, comorbidities and response to rituximab","volume":"14","author":"Sudulagunta S.R.","year":"2016","journal-title":"Ger. Med. Sci."},{"key":"e_1_2_7_43_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3182407982"},{"key":"e_1_2_7_44_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.314"},{"key":"e_1_2_7_45_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-014-7532-3"},{"key":"e_1_2_7_46_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25597"},{"key":"e_1_2_7_47_1","doi-asserted-by":"crossref","first-page":"4419","DOI":"10.1212\/WNL.0000000000002790","article-title":"International consensus guidance for management of myasthenia gravis: executive summary","volume":"87","author":"Sanders D.B.","year":"2016","journal-title":"Neurology"},{"key":"e_1_2_7_48_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23951"},{"key":"e_1_2_7_49_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.39132"},{"key":"e_1_2_7_50_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2016.07.009"},{"key":"e_1_2_7_51_1","doi-asserted-by":"publisher","DOI":"10.1212\/CPJ.0000000000000202"},{"key":"e_1_2_7_52_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2016.12.004"},{"key":"e_1_2_7_53_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.24063"},{"key":"e_1_2_7_54_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11940-014-0283-8"},{"key":"e_1_2_7_55_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23839"},{"key":"e_1_2_7_56_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25458"},{"key":"e_1_2_7_57_1","doi-asserted-by":"publisher","DOI":"10.1007\/s40265-017-0701-9"},{"key":"e_1_2_7_58_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000183145.91579.74"},{"key":"e_1_2_7_59_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ajpath.2011.10.031"},{"key":"e_1_2_7_60_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2013.251827"},{"key":"e_1_2_7_61_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0087840"},{"key":"e_1_2_7_62_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000002466"},{"key":"e_1_2_7_63_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2015.09.019"},{"key":"e_1_2_7_64_1","doi-asserted-by":"publisher","DOI":"10.1097\/CND.0000000000000160"},{"key":"e_1_2_7_65_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nmd.2013.08.002"},{"key":"e_1_2_7_66_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2012.06.038"},{"key":"e_1_2_7_67_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1602678"},{"key":"e_1_2_7_68_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.12169"},{"key":"e_1_2_7_69_1","doi-asserted-by":"publisher","DOI":"10.3109\/08916934.2015.1031890"},{"key":"e_1_2_7_70_1","article-title":"IgG4\u2010related disease: a complex under\u2010diagnosed clinical entity","author":"Yadlapati S.","year":"2017","journal-title":"Rheumatol. Int."},{"key":"e_1_2_7_71_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jneuroim.2017.02.004"},{"key":"e_1_2_7_72_1","doi-asserted-by":"publisher","DOI":"10.1111\/ene.12758"}],"container-title":["Annals of the New York Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fnyas.13518","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/nyaspubs.onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/nyas.13518","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,12]],"date-time":"2023-09-12T14:15:58Z","timestamp":1694528158000},"score":1,"resource":{"primary":{"URL":"https:\/\/nyaspubs.onlinelibrary.wiley.com\/doi\/10.1111\/nyas.13518"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,12,21]]},"references-count":71,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2018,1]]}},"alternative-id":["10.1111\/nyas.13518"],"URL":"https:\/\/doi.org\/10.1111\/nyas.13518","archive":["Portico"],"relation":{},"ISSN":["0077-8923","1749-6632"],"issn-type":[{"value":"0077-8923","type":"print"},{"value":"1749-6632","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,12,21]]}}}