Rituximab in Severe Seronegative Juvenile Myasthenia Gravis: Review of the Literature
Abstract Myasthenia gravis is an autoimmune neuromuscular disorder caused by circulating antibodies that block acetylcholine receptors at the postsynaptic neuromuscular junction. A wide range of symptomatic therapy with acetylcholinesterase inhibitors and immunotherapy such as corticosteroids, azath...
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Veröffentlicht in: | Pediatric neurology 2012-09, Vol.47 (3), p.209-212 |
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description | Abstract Myasthenia gravis is an autoimmune neuromuscular disorder caused by circulating antibodies that block acetylcholine receptors at the postsynaptic neuromuscular junction. A wide range of symptomatic therapy with acetylcholinesterase inhibitors and immunotherapy such as corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, plasmapheresis, and intravenous immunoglobulin has been used in the treatment of myasthenia gravis, with variable responses. However, most modalities of treatment involve delayed onset of action. We describe a child with severe, life-threatening seronegative myasthenia gravis who repeatedly failed extubation and responded dramatically to rituximab. She achieved complete and sustained remission for more than 9 months, with gradual reduction in steroid dose without any side effects. Advances in the treatment of myasthenia gravis have reduced mortality and morbidity and improved the quality of life in these patients. |
doi_str_mv | 10.1016/j.pediatrneurol.2012.05.017 |
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A wide range of symptomatic therapy with acetylcholinesterase inhibitors and immunotherapy such as corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, plasmapheresis, and intravenous immunoglobulin has been used in the treatment of myasthenia gravis, with variable responses. However, most modalities of treatment involve delayed onset of action. We describe a child with severe, life-threatening seronegative myasthenia gravis who repeatedly failed extubation and responded dramatically to rituximab. She achieved complete and sustained remission for more than 9 months, with gradual reduction in steroid dose without any side effects. Advances in the treatment of myasthenia gravis have reduced mortality and morbidity and improved the quality of life in these patients.</description><identifier>ISSN: 0887-8994</identifier><identifier>EISSN: 1873-5150</identifier><identifier>DOI: 10.1016/j.pediatrneurol.2012.05.017</identifier><identifier>PMID: 22883288</identifier><language>eng</language><publisher>New York, NY: Elsevier Inc</publisher><subject>Acetylcholine receptors ; Acetylcholinesterase ; Airway Extubation ; Anti-Inflammatory Agents - administration & dosage ; Anti-Inflammatory Agents - therapeutic use ; Antibodies, Monoclonal, Murine-Derived - adverse effects ; Antibodies, Monoclonal, Murine-Derived - therapeutic use ; Antigens, CD19 - analysis ; Antirheumatic Agents - adverse effects ; Antirheumatic Agents - therapeutic use ; azathioprine ; Biological and medical sciences ; Blepharoptosis - etiology ; Child, Preschool ; Corticoids ; Cyclosporins ; Diplopia - etiology ; Diseases of striated muscles. Neuromuscular diseases ; Female ; Humans ; Immunoglobulins ; Immunotherapy ; Intravenous administration ; Literature reviews ; Lymphocyte Count ; Medical sciences ; Morbidity ; Mortality ; Myasthenia gravis ; Myasthenia Gravis - drug therapy ; Neurology ; Neuromuscular junctions ; neuromuscular system ; Pediatrics ; Plasmapheresis ; Prednisone - administration & dosage ; Prednisone - therapeutic use ; Quality of life ; Remission ; Respiration, Artificial ; Rituximab ; Side effects</subject><ispartof>Pediatric neurology, 2012-09, Vol.47 (3), p.209-212</ispartof><rights>Elsevier Inc.</rights><rights>2012 Elsevier Inc.</rights><rights>2015 INIST-CNRS</rights><rights>Copyright © 2012 Elsevier Inc. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c501t-aaad00b1510dd02b21e9d22fe5e9e9a3c542f0a18bca1ab86a63cecbb4220adf3</citedby><cites>FETCH-LOGICAL-c501t-aaad00b1510dd02b21e9d22fe5e9e9a3c542f0a18bca1ab86a63cecbb4220adf3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S088789941200255X$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3537,27901,27902,65306</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=26280960$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22883288$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Koul, Roshan, MD</creatorcontrib><creatorcontrib>Al Futaisi, Amna, MD</creatorcontrib><creatorcontrib>Abdwani, Reem, MD</creatorcontrib><title>Rituximab in Severe Seronegative Juvenile Myasthenia Gravis: Review of the Literature</title><title>Pediatric neurology</title><addtitle>Pediatr Neurol</addtitle><description>Abstract Myasthenia gravis is an autoimmune neuromuscular disorder caused by circulating antibodies that block acetylcholine receptors at the postsynaptic neuromuscular junction. A wide range of symptomatic therapy with acetylcholinesterase inhibitors and immunotherapy such as corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, plasmapheresis, and intravenous immunoglobulin has been used in the treatment of myasthenia gravis, with variable responses. However, most modalities of treatment involve delayed onset of action. We describe a child with severe, life-threatening seronegative myasthenia gravis who repeatedly failed extubation and responded dramatically to rituximab. She achieved complete and sustained remission for more than 9 months, with gradual reduction in steroid dose without any side effects. Advances in the treatment of myasthenia gravis have reduced mortality and morbidity and improved the quality of life in these patients.</description><subject>Acetylcholine receptors</subject><subject>Acetylcholinesterase</subject><subject>Airway Extubation</subject><subject>Anti-Inflammatory Agents - administration & dosage</subject><subject>Anti-Inflammatory Agents - therapeutic use</subject><subject>Antibodies, Monoclonal, Murine-Derived - adverse effects</subject><subject>Antibodies, Monoclonal, Murine-Derived - therapeutic use</subject><subject>Antigens, CD19 - analysis</subject><subject>Antirheumatic Agents - adverse effects</subject><subject>Antirheumatic Agents - therapeutic use</subject><subject>azathioprine</subject><subject>Biological and medical sciences</subject><subject>Blepharoptosis - etiology</subject><subject>Child, Preschool</subject><subject>Corticoids</subject><subject>Cyclosporins</subject><subject>Diplopia - etiology</subject><subject>Diseases of striated muscles. Neuromuscular diseases</subject><subject>Female</subject><subject>Humans</subject><subject>Immunoglobulins</subject><subject>Immunotherapy</subject><subject>Intravenous administration</subject><subject>Literature reviews</subject><subject>Lymphocyte Count</subject><subject>Medical sciences</subject><subject>Morbidity</subject><subject>Mortality</subject><subject>Myasthenia gravis</subject><subject>Myasthenia Gravis - drug therapy</subject><subject>Neurology</subject><subject>Neuromuscular junctions</subject><subject>neuromuscular system</subject><subject>Pediatrics</subject><subject>Plasmapheresis</subject><subject>Prednisone - administration & dosage</subject><subject>Prednisone - therapeutic use</subject><subject>Quality of life</subject><subject>Remission</subject><subject>Respiration, Artificial</subject><subject>Rituximab</subject><subject>Side effects</subject><issn>0887-8994</issn><issn>1873-5150</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2012</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNktGL1DAQxoMo3nr6L0hABF9aJ0nTTRUEOe5OZUW488C3ME2nmrXbrklbb_97s-yq6NM9hAnkN_OF7xvGngnIBYjy5TrfUuNxDD1NYehyCULmoHMQy3tsIcxSZVpouM8WYMwyM1VVnLBHMa4BQFeyeMhOpDRGpbNgN1d-nG79Bmvue35NMwVKJQw9fcXRz8Q_TDP1viP-cYdx_JbuyC8Dzj6-4lc0e_rJh5anB77yIwUcp0CP2YMWu0hPjvWU3Vycfz57l60-Xb4_e7vKnAYxZojYANRCC2gakLUUVDVStqSpogqV04VsAYWpHQqsTYmlcuTqupASsGnVKXtxmLsNw4-J4mg3PjrqOuxpmKIVoEwpyhLEXVBVaFmpZUJfH1AXhhgDtXYbkkNhlyC7j8Cu7T8R2H0EFrRNEaTup0ehqd5Q86f3t-cJeH4EMDrs2oC98_EvV0oDVQmJOz9wlBxMPgcbnafeJeVAbrTN4O_4oTf_zXGd732S_k47iuthCn0KyQobU4-93m_NfmmEBJBaf1G_ABNYwrQ</recordid><startdate>20120901</startdate><enddate>20120901</enddate><creator>Koul, Roshan, MD</creator><creator>Al Futaisi, Amna, MD</creator><creator>Abdwani, Reem, MD</creator><general>Elsevier Inc</general><general>Elsevier</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope><scope>7TK</scope></search><sort><creationdate>20120901</creationdate><title>Rituximab in Severe Seronegative Juvenile Myasthenia Gravis: Review of the Literature</title><author>Koul, Roshan, MD ; Al Futaisi, Amna, MD ; Abdwani, Reem, MD</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c501t-aaad00b1510dd02b21e9d22fe5e9e9a3c542f0a18bca1ab86a63cecbb4220adf3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2012</creationdate><topic>Acetylcholine receptors</topic><topic>Acetylcholinesterase</topic><topic>Airway Extubation</topic><topic>Anti-Inflammatory Agents - administration & dosage</topic><topic>Anti-Inflammatory Agents - therapeutic use</topic><topic>Antibodies, Monoclonal, Murine-Derived - adverse effects</topic><topic>Antibodies, Monoclonal, Murine-Derived - therapeutic use</topic><topic>Antigens, CD19 - analysis</topic><topic>Antirheumatic Agents - adverse effects</topic><topic>Antirheumatic Agents - therapeutic use</topic><topic>azathioprine</topic><topic>Biological and medical sciences</topic><topic>Blepharoptosis - etiology</topic><topic>Child, Preschool</topic><topic>Corticoids</topic><topic>Cyclosporins</topic><topic>Diplopia - etiology</topic><topic>Diseases of striated muscles. Neuromuscular diseases</topic><topic>Female</topic><topic>Humans</topic><topic>Immunoglobulins</topic><topic>Immunotherapy</topic><topic>Intravenous administration</topic><topic>Literature reviews</topic><topic>Lymphocyte Count</topic><topic>Medical sciences</topic><topic>Morbidity</topic><topic>Mortality</topic><topic>Myasthenia gravis</topic><topic>Myasthenia Gravis - drug therapy</topic><topic>Neurology</topic><topic>Neuromuscular junctions</topic><topic>neuromuscular system</topic><topic>Pediatrics</topic><topic>Plasmapheresis</topic><topic>Prednisone - administration & dosage</topic><topic>Prednisone - therapeutic use</topic><topic>Quality of life</topic><topic>Remission</topic><topic>Respiration, Artificial</topic><topic>Rituximab</topic><topic>Side effects</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Koul, Roshan, MD</creatorcontrib><creatorcontrib>Al Futaisi, Amna, MD</creatorcontrib><creatorcontrib>Abdwani, Reem, MD</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><collection>Neurosciences Abstracts</collection><jtitle>Pediatric neurology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Koul, Roshan, MD</au><au>Al Futaisi, Amna, MD</au><au>Abdwani, Reem, MD</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Rituximab in Severe Seronegative Juvenile Myasthenia Gravis: Review of the Literature</atitle><jtitle>Pediatric neurology</jtitle><addtitle>Pediatr Neurol</addtitle><date>2012-09-01</date><risdate>2012</risdate><volume>47</volume><issue>3</issue><spage>209</spage><epage>212</epage><pages>209-212</pages><issn>0887-8994</issn><eissn>1873-5150</eissn><abstract>Abstract Myasthenia gravis is an autoimmune neuromuscular disorder caused by circulating antibodies that block acetylcholine receptors at the postsynaptic neuromuscular junction. A wide range of symptomatic therapy with acetylcholinesterase inhibitors and immunotherapy such as corticosteroids, azathioprine, cyclosporine, mycophenolate mofetil, plasmapheresis, and intravenous immunoglobulin has been used in the treatment of myasthenia gravis, with variable responses. However, most modalities of treatment involve delayed onset of action. We describe a child with severe, life-threatening seronegative myasthenia gravis who repeatedly failed extubation and responded dramatically to rituximab. She achieved complete and sustained remission for more than 9 months, with gradual reduction in steroid dose without any side effects. Advances in the treatment of myasthenia gravis have reduced mortality and morbidity and improved the quality of life in these patients.</abstract><cop>New York, NY</cop><pub>Elsevier Inc</pub><pmid>22883288</pmid><doi>10.1016/j.pediatrneurol.2012.05.017</doi><tpages>4</tpages></addata></record> |
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subjects | Acetylcholine receptors Acetylcholinesterase Airway Extubation Anti-Inflammatory Agents - administration & dosage Anti-Inflammatory Agents - therapeutic use Antibodies, Monoclonal, Murine-Derived - adverse effects Antibodies, Monoclonal, Murine-Derived - therapeutic use Antigens, CD19 - analysis Antirheumatic Agents - adverse effects Antirheumatic Agents - therapeutic use azathioprine Biological and medical sciences Blepharoptosis - etiology Child, Preschool Corticoids Cyclosporins Diplopia - etiology Diseases of striated muscles. Neuromuscular diseases Female Humans Immunoglobulins Immunotherapy Intravenous administration Literature reviews Lymphocyte Count Medical sciences Morbidity Mortality Myasthenia gravis Myasthenia Gravis - drug therapy Neurology Neuromuscular junctions neuromuscular system Pediatrics Plasmapheresis Prednisone - administration & dosage Prednisone - therapeutic use Quality of life Remission Respiration, Artificial Rituximab Side effects |
title | Rituximab in Severe Seronegative Juvenile Myasthenia Gravis: Review of the Literature |
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