AChR myasthenia gravis switching to MuSK or double antibody positive myasthenia gravis in two children and literature review
•Coexistence of AChR antibody and MuSK antibody is rare in children MG.•AChR–MG children can switch to MuSK–MG or double antibody positive MG.•The serological switch is accompanied by a switch in clinical phenotype to that of MuSK–MG. Muscle–specific tyrosine kinase antibody (MuSK–Ab) and acetylchol...
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Veröffentlicht in: | Neuromuscular disorders : NMD 2020-07, Vol.30 (7), p.534-538 |
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creator | Lu, Yaru Ran, Hao Yang, Wenhao Ma, Qian Qiu, Li Ou, Changyi Chen, Pei Lin, Zhongqiang Liu, Weibin |
description | •Coexistence of AChR antibody and MuSK antibody is rare in children MG.•AChR–MG children can switch to MuSK–MG or double antibody positive MG.•The serological switch is accompanied by a switch in clinical phenotype to that of MuSK–MG.
Muscle–specific tyrosine kinase antibody (MuSK–Ab) and acetylcholine receptor antibody (AChR–Ab) coexistence in myasthenia gravis (MG) is very rare. In this report, two children with AChR–Ab switching to double antibody positive MG (DP–MG) or MuSK–Ab positive MG (MuSK–MG) are described. Six similar cases were found in the literature via online database search. Therefore, this study describes eight patients in total, six female and two male. The average age of onset was 7.25 ± 5.95 years. Four AChR–MG patients switched to DP–MG with no known precipitating factor and four switched after thymectomy (two to MuSK–MG and two to DP–MG). After the serological switch, the patients transitioned to the phenotype of MuSK–MG and responded poorly to cholinesterase inhibitors and well to corticosteroids and plasma exchange. |
doi_str_mv | 10.1016/j.nmd.2020.03.012 |
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Muscle–specific tyrosine kinase antibody (MuSK–Ab) and acetylcholine receptor antibody (AChR–Ab) coexistence in myasthenia gravis (MG) is very rare. In this report, two children with AChR–Ab switching to double antibody positive MG (DP–MG) or MuSK–Ab positive MG (MuSK–MG) are described. Six similar cases were found in the literature via online database search. Therefore, this study describes eight patients in total, six female and two male. The average age of onset was 7.25 ± 5.95 years. Four AChR–MG patients switched to DP–MG with no known precipitating factor and four switched after thymectomy (two to MuSK–MG and two to DP–MG). After the serological switch, the patients transitioned to the phenotype of MuSK–MG and responded poorly to cholinesterase inhibitors and well to corticosteroids and plasma exchange.</description><identifier>ISSN: 0960-8966</identifier><identifier>EISSN: 1873-2364</identifier><identifier>DOI: 10.1016/j.nmd.2020.03.012</identifier><identifier>PMID: 32387283</identifier><language>eng</language><publisher>England: Elsevier B.V</publisher><subject>Achr antibody ; Children ; Musk antibody ; Myasthenia gravis</subject><ispartof>Neuromuscular disorders : NMD, 2020-07, Vol.30 (7), p.534-538</ispartof><rights>2020 Elsevier B.V.</rights><rights>Copyright © 2020 Elsevier B.V. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c419t-b10b74d1f713ebedcc829e52f7f5d0d7ed71850026139334217678796a7aecb43</citedby><cites>FETCH-LOGICAL-c419t-b10b74d1f713ebedcc829e52f7f5d0d7ed71850026139334217678796a7aecb43</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.nmd.2020.03.012$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,27924,27925,45995</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/32387283$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Lu, Yaru</creatorcontrib><creatorcontrib>Ran, Hao</creatorcontrib><creatorcontrib>Yang, Wenhao</creatorcontrib><creatorcontrib>Ma, Qian</creatorcontrib><creatorcontrib>Qiu, Li</creatorcontrib><creatorcontrib>Ou, Changyi</creatorcontrib><creatorcontrib>Chen, Pei</creatorcontrib><creatorcontrib>Lin, Zhongqiang</creatorcontrib><creatorcontrib>Liu, Weibin</creatorcontrib><title>AChR myasthenia gravis switching to MuSK or double antibody positive myasthenia gravis in two children and literature review</title><title>Neuromuscular disorders : NMD</title><addtitle>Neuromuscul Disord</addtitle><description>•Coexistence of AChR antibody and MuSK antibody is rare in children MG.•AChR–MG children can switch to MuSK–MG or double antibody positive MG.•The serological switch is accompanied by a switch in clinical phenotype to that of MuSK–MG.
Muscle–specific tyrosine kinase antibody (MuSK–Ab) and acetylcholine receptor antibody (AChR–Ab) coexistence in myasthenia gravis (MG) is very rare. In this report, two children with AChR–Ab switching to double antibody positive MG (DP–MG) or MuSK–Ab positive MG (MuSK–MG) are described. Six similar cases were found in the literature via online database search. Therefore, this study describes eight patients in total, six female and two male. The average age of onset was 7.25 ± 5.95 years. Four AChR–MG patients switched to DP–MG with no known precipitating factor and four switched after thymectomy (two to MuSK–MG and two to DP–MG). After the serological switch, the patients transitioned to the phenotype of MuSK–MG and responded poorly to cholinesterase inhibitors and well to corticosteroids and plasma exchange.</description><subject>Achr antibody</subject><subject>Children</subject><subject>Musk antibody</subject><subject>Myasthenia gravis</subject><issn>0960-8966</issn><issn>1873-2364</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2020</creationdate><recordtype>article</recordtype><recordid>eNp9kE1v1DAURSMEotPCD2CDvGST8J6diROxqkYFKoqQ-Fhbjv3S8SiJB9uZ0Uj8eFxNYYVY3c29R7qnKF4hVAjYvN1V82QrDhwqEBUgf1KssJWi5KKpnxYr6Boo265pLorLGHcAuJaNfF5cCC5ayVuxKn5db7Zf2XTSMW1pdprdB31wkcWjS2br5nuWPPu8fPvEfGDWL_1ITM_J9d6e2N5Hl9yB_rF3M0tHzzJitIHmvLFsdImCTksgFujg6PiieDboMdLLx7wqfry_-b75WN59-XC7ub4rTY1dKnuEXtYWB4mCerLGtLyjNR_ksLZgJVmJ7RqANyg6IWqO-WUru0ZLTaavxVXx5szdB_9zoZjU5KKhcdQz-SUqXgMiAkfIVTxXTfAxBhrUPrhJh5NCUA_S1U5l6epBugKhsvS8ef2IX_qJ7N_FH8u58O5coHwyHw8qGkezIesCmaSsd__B_wbMWZO7</recordid><startdate>20200701</startdate><enddate>20200701</enddate><creator>Lu, Yaru</creator><creator>Ran, Hao</creator><creator>Yang, Wenhao</creator><creator>Ma, Qian</creator><creator>Qiu, Li</creator><creator>Ou, Changyi</creator><creator>Chen, Pei</creator><creator>Lin, Zhongqiang</creator><creator>Liu, Weibin</creator><general>Elsevier B.V</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>20200701</creationdate><title>AChR myasthenia gravis switching to MuSK or double antibody positive myasthenia gravis in two children and literature review</title><author>Lu, Yaru ; Ran, Hao ; Yang, Wenhao ; Ma, Qian ; Qiu, Li ; Ou, Changyi ; Chen, Pei ; Lin, Zhongqiang ; Liu, Weibin</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c419t-b10b74d1f713ebedcc829e52f7f5d0d7ed71850026139334217678796a7aecb43</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2020</creationdate><topic>Achr antibody</topic><topic>Children</topic><topic>Musk antibody</topic><topic>Myasthenia gravis</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Lu, Yaru</creatorcontrib><creatorcontrib>Ran, Hao</creatorcontrib><creatorcontrib>Yang, Wenhao</creatorcontrib><creatorcontrib>Ma, Qian</creatorcontrib><creatorcontrib>Qiu, Li</creatorcontrib><creatorcontrib>Ou, Changyi</creatorcontrib><creatorcontrib>Chen, Pei</creatorcontrib><creatorcontrib>Lin, Zhongqiang</creatorcontrib><creatorcontrib>Liu, Weibin</creatorcontrib><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Neuromuscular disorders : NMD</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Lu, Yaru</au><au>Ran, Hao</au><au>Yang, Wenhao</au><au>Ma, Qian</au><au>Qiu, Li</au><au>Ou, Changyi</au><au>Chen, Pei</au><au>Lin, Zhongqiang</au><au>Liu, Weibin</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>AChR myasthenia gravis switching to MuSK or double antibody positive myasthenia gravis in two children and literature review</atitle><jtitle>Neuromuscular disorders : NMD</jtitle><addtitle>Neuromuscul Disord</addtitle><date>2020-07-01</date><risdate>2020</risdate><volume>30</volume><issue>7</issue><spage>534</spage><epage>538</epage><pages>534-538</pages><issn>0960-8966</issn><eissn>1873-2364</eissn><abstract>•Coexistence of AChR antibody and MuSK antibody is rare in children MG.•AChR–MG children can switch to MuSK–MG or double antibody positive MG.•The serological switch is accompanied by a switch in clinical phenotype to that of MuSK–MG.
Muscle–specific tyrosine kinase antibody (MuSK–Ab) and acetylcholine receptor antibody (AChR–Ab) coexistence in myasthenia gravis (MG) is very rare. In this report, two children with AChR–Ab switching to double antibody positive MG (DP–MG) or MuSK–Ab positive MG (MuSK–MG) are described. Six similar cases were found in the literature via online database search. Therefore, this study describes eight patients in total, six female and two male. The average age of onset was 7.25 ± 5.95 years. Four AChR–MG patients switched to DP–MG with no known precipitating factor and four switched after thymectomy (two to MuSK–MG and two to DP–MG). After the serological switch, the patients transitioned to the phenotype of MuSK–MG and responded poorly to cholinesterase inhibitors and well to corticosteroids and plasma exchange.</abstract><cop>England</cop><pub>Elsevier B.V</pub><pmid>32387283</pmid><doi>10.1016/j.nmd.2020.03.012</doi><tpages>5</tpages></addata></record> |
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subjects | Achr antibody Children Musk antibody Myasthenia gravis |
title | AChR myasthenia gravis switching to MuSK or double antibody positive myasthenia gravis in two children and literature review |
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