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
Hauptverfasser: Koul, Roshan, MD, Al Futaisi, Amna, MD, Abdwani, Reem, MD
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container_title Pediatric neurology
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creator Koul, Roshan, MD
Al Futaisi, Amna, MD
Abdwani, Reem, MD
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. 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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 &amp; 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. 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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. 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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 &amp; 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 &amp; 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. 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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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